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Emergency And Acute Medicine – Hemophilia
Basics And Description
Hemophilia is a hereditary bleeding disorder caused by deficiency of functional clotting factor VIII or factor IX, resulting in impaired activation of the coagulation cascade and defective hemostasis. Hemophilia A is due to factor VIII deficiency, while hemophilia B (Christmas disease) is due to factor IX deficiency. Clinical severity depends on residual factor activity. Mild disease (5–30% activity) presents with bleeding after major trauma or surgery. Moderate disease (1–5% activity) causes bleeding with trauma or surgery and occasional spontaneous hemarthroses. Severe disease (<1% activity) leads to spontaneous bleeding beginning in infancy, often occurring weekly and frequently requiring factor replacement. major complications include fatal hemorrhage, progressive joint destruction from recurrent hemarthroses, transfusion-transmitted infections (now rare with purified products), development of inhibitors—igg antibodies that neutralize clotting factors.< />pan>
Etiology
Hemophilia is inherited in an X-linked recessive pattern, although approximately one-third of cases arise from spontaneous mutations. Hemophilia A occurs in approximately 1 in 5,000–7,000 males, while hemophilia B occurs in about 1 in 30,000 males. Inhibitors develop in roughly 20% of patients with severe hemophilia A and fewer than 5% of those with severe hemophilia B.
Clinical Presentation And Symptoms
Bleeding manifestations include hemarthrosis, most commonly affecting the knee, followed by elbow, ankle, shoulder, and wrist. Other presentations include muscle hemorrhage, prolonged bleeding from soft tissue lacerations, post–dental extraction or oral mucosal bleeding, epistaxis in severe disease, hematuria, gastrointestinal bleeding, intracranial hemorrhage, and pseudotumors formed by encapsulated blood collections.
Essential Workup
Evaluation begins with a thorough history and physical examination, including bleeding pattern, known factor deficiency, baseline factor levels, inhibitor status, and prior treatments.
Diagnostic Tests And Interpretation
Laboratory findings typically show a normal platelet count, normal bleeding time, normal prothrombin time, and prolonged partial thromboplastin time. Urinalysis may reveal asymptomatic hematuria. Specific factor assays demonstrate decreased factor VIII or IX activity. Von Willebrand factor levels are normal, helping distinguish hemophilia from von Willebrand disease. Imaging is guided by clinical presentation and may include CT of the head for suspected intracranial hemorrhage, renal ultrasound or cystoscopy for persistent hematuria, or abdominal CT for suspected retroperitoneal bleeding.
Differential Diagnosis
Consider von Willebrand disease, anticoagulant or antiplatelet medication use, thrombocytopenia, and hepatic dysfunction.
Initial Stabilization And Therapy
Bleeding control begins immediately with proximal pressure when appropriate. Establish IV access and obtain type and screen. Packed red blood cells may be required for significant blood loss. Early factor replacement is critical and should not be delayed for imaging or consultation.
Emergency Department Management
Patients often know their diagnosis, factor type, dose, and inhibitor status. Emergency care should be coordinated with the patient’s hematologist whenever possible. Factor replacement should be initiated immediately for any suspected bleed.
Desired factor activity depends on bleeding severity. For low to moderate bleeding, target 30–50% activity. For moderate to severe bleeding, including gastrointestinal or genitourinary hemorrhage, target 50–100%. For life-threatening bleeding such as intracranial hemorrhage, major trauma, or airway compromise, target 100% activity.
Factor VIII dosing is calculated as body weight (kg) × 0.5 × desired percent activity, with dosing every 12 hours. Factor IX dosing is calculated as body weight (kg) × desired percent activity, with dosing every 24 hours.
Hemarthrosis is managed with splinting, compression, and ice; arthrocentesis is rarely indicated. Muscle hemorrhage requires monitoring for compartment syndrome, particularly in the forearm, calf, or iliopsoas. Oral and dental bleeding may benefit from local hemostatic agents and antifibrinolytics. Hematuria is usually mild and managed with hydration; antifibrinolytics and cryoprecipitate should be avoided. All head injuries are treated as significant, and therapy must precede imaging. Gastrointestinal bleeding requires factor replacement before endoscopic procedures.
Medications And Factor Therapy
First-line treatment for patients without inhibitors is recombinant factor VIII or IX concentrates. Plasma-derived factor concentrates are second-line options. Cryoprecipitate is reserved for hemophilia A only when factor concentrates are unavailable. Fresh frozen plasma may be used temporarily in life-threatening bleeding if specific factors are delayed.
Adjunctive therapies include desmopressin for mild hemophilia A, antifibrinolytics for mucosal bleeding, and topical thrombin for superficial wounds.
For patients with inhibitors, recombinant factor VIIa is first-line therapy, with activated prothrombin complex concentrates as second-line options. High-dose factor concentrates may be considered in low-titer inhibitor patients. Intramuscular injections and aspirin-containing products must be avoided.
Disposition And Follow-Up
There should be a low threshold for hospital admission. Indications include joint or muscle bleeds, internal hemorrhage, severe bleeding, need for repeated transfusions, complications, or any head trauma. Minor superficial bleeding with complete control may be discharged with close follow-up. All patients require hematology consultation and outpatient follow-up, with instructions to return immediately for recurrent or worsening bleeding.
Clinical Pearls And Common Errors
Never delay factor replacement while awaiting imaging in suspected serious bleeding, especially head trauma. In unknown factor levels, assume zero activity and treat as severe disease. Be familiar with management differences in patients with and without inhibitors. Replace factor before transfers, procedures, or consultations. Maintain a low threshold for admission and hematology involvement.
Basics And Description
Hemophilia is a hereditary bleeding disorder caused by deficiency of functional clotting factor VIII or factor IX, resulting in impaired activation of the coagulation cascade and defective hemostasis. Hemophilia A is due to factor VIII deficiency, while hemophilia B (Christmas disease) is due to factor IX deficiency. Clinical severity depends on residual factor activity. Mild disease (5–30% activity) presents with bleeding after major trauma or surgery. Moderate disease (1–5% activity) causes bleeding with trauma or surgery and occasional spontaneous hemarthroses. Severe disease (<1% activity) leads to spontaneous bleeding beginning in infancy, often occurring weekly and frequently requiring factor replacement. major complications include fatal hemorrhage, progressive joint destruction from recurrent hemarthroses, transfusion-transmitted infections (now rare with purified products), development of inhibitors—igg antibodies that neutralize clotting factors.< />pan>
Etiology
Hemophilia is inherited in an X-linked recessive pattern, although approximately one-third of cases arise from spontaneous mutations. Hemophilia A occurs in approximately 1 in 5,000–7,000 males, while hemophilia B occurs in about 1 in 30,000 males. Inhibitors develop in roughly 20% of patients with severe hemophilia A and fewer than 5% of those with severe hemophilia B.
Clinical Presentation And Symptoms
Bleeding manifestations include hemarthrosis, most commonly affecting the knee, followed by elbow, ankle, shoulder, and wrist. Other presentations include muscle hemorrhage, prolonged bleeding from soft tissue lacerations, post–dental extraction or oral mucosal bleeding, epistaxis in severe disease, hematuria, gastrointestinal bleeding, intracranial hemorrhage, and pseudotumors formed by encapsulated blood collections.
Essential Workup
Evaluation begins with a thorough history and physical examination, including bleeding pattern, known factor deficiency, baseline factor levels, inhibitor status, and prior treatments.
Diagnostic Tests And Interpretation
Laboratory findings typically show a normal platelet count, normal bleeding time, normal prothrombin time, and prolonged partial thromboplastin time. Urinalysis may reveal asymptomatic hematuria. Specific factor assays demonstrate decreased factor VIII or IX activity. Von Willebrand factor levels are normal, helping distinguish hemophilia from von Willebrand disease. Imaging is guided by clinical presentation and may include CT of the head for suspected intracranial hemorrhage, renal ultrasound or cystoscopy for persistent hematuria, or abdominal CT for suspected retroperitoneal bleeding.
Differential Diagnosis
Consider von Willebrand disease, anticoagulant or antiplatelet medication use, thrombocytopenia, and hepatic dysfunction.
Initial Stabilization And Therapy
Bleeding control begins immediately with proximal pressure when appropriate. Establish IV access and obtain type and screen. Packed red blood cells may be required for significant blood loss. Early factor replacement is critical and should not be delayed for imaging or consultation.
Emergency Department Management
Patients often know their diagnosis, factor type, dose, and inhibitor status. Emergency care should be coordinated with the patient’s hematologist whenever possible. Factor replacement should be initiated immediately for any suspected bleed.
Desired factor activity depends on bleeding severity. For low to moderate bleeding, target 30–50% activity. For moderate to severe bleeding, including gastrointestinal or genitourinary hemorrhage, target 50–100%. For life-threatening bleeding such as intracranial hemorrhage, major trauma, or airway compromise, target 100% activity.
Factor VIII dosing is calculated as body weight (kg) × 0.5 × desired percent activity, with dosing every 12 hours. Factor IX dosing is calculated as body weight (kg) × desired percent activity, with dosing every 24 hours.
Hemarthrosis is managed with splinting, compression, and ice; arthrocentesis is rarely indicated. Muscle hemorrhage requires monitoring for compartment syndrome, particularly in the forearm, calf, or iliopsoas. Oral and dental bleeding may benefit from local hemostatic agents and antifibrinolytics. Hematuria is usually mild and managed with hydration; antifibrinolytics and cryoprecipitate should be avoided. All head injuries are treated as significant, and therapy must precede imaging. Gastrointestinal bleeding requires factor replacement before endoscopic procedures.
Medications And Factor Therapy
First-line treatment for patients without inhibitors is recombinant factor VIII or IX concentrates. Plasma-derived factor concentrates are second-line options. Cryoprecipitate is reserved for hemophilia A only when factor concentrates are unavailable. Fresh frozen plasma may be used temporarily in life-threatening bleeding if specific factors are delayed.
Adjunctive therapies include desmopressin for mild hemophilia A, antifibrinolytics for mucosal bleeding, and topical thrombin for superficial wounds.
For patients with inhibitors, recombinant factor VIIa is first-line therapy, with activated prothrombin complex concentrates as second-line options. High-dose factor concentrates may be considered in low-titer inhibitor patients. Intramuscular injections and aspirin-containing products must be avoided.
Disposition And Follow-Up
There should be a low threshold for hospital admission. Indications include joint or muscle bleeds, internal hemorrhage, severe bleeding, need for repeated transfusions, complications, or any head trauma. Minor superficial bleeding with complete control may be discharged with close follow-up. All patients require hematology consultation and outpatient follow-up, with instructions to return immediately for recurrent or worsening bleeding.
Clinical Pearls And Common Errors
Never delay factor replacement while awaiting imaging in suspected serious bleeding, especially head trauma. In unknown factor levels, assume zero activity and treat as severe disease. Be familiar with management differences in patients with and without inhibitors. Replace factor before transfers, procedures, or consultations. Maintain a low threshold for admission and hematology involvement.
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Emergency And Acute Medicine – Hemorrhagic Fevers
Basics And Description
Hemorrhagic fever refers to a multisystem syndrome characterized by vasocapillary permeability, hemorrhage, and organ dysfunction. Viral hemorrhagic fevers (VHF) are caused by a distinct group of viruses and often begin with a nonspecific influenza-like illness. Hemorrhagic manifestations occur in a minority of patients and typically appear in later stages of disease.
Risk Factors
Travel to endemic regions
Exposure related to biologic warfare
Close contact with animals
Insect bites or ingestion
Pathophysiology
Viral hemorrhagic fevers cause endothelial injury leading to increased vascular permeability, hemorrhage, and progression to shock. The shock state is both hypovolemic and distributive and is often difficult to reverse. Rapid progression to hypotension indicates a very high mortality risk. Disseminated intravascular coagulation is commonly seen in Marburg and Crimean-Congo hemorrhagic fever but is less frequent in arenavirus infections. Dengue hemorrhagic fever is immune mediated and often results from secondary infection and is among the most common causes of VHF.
Etiology
Viral hemorrhagic fevers are caused by RNA viruses with zoonotic life cycles and short incubation periods of less than 10–21 days.
Filoviruses include Ebola and Marburg, with fruit bat reservoirs and transmission primarily in sub-Saharan Africa.
Arenaviruses include Lassa fever and South American hemorrhagic fevers, transmitted via aerosolized rodent excreta.
Flaviviruses include Dengue and Yellow fever, transmitted by mosquitoes, primarily in tropical regions.
Bunyaviridae include Rift Valley fever and Crimean-Congo hemorrhagic fever, transmitted by ticks or mosquitoes.
Hantaviridae include hemorrhagic fever with renal syndrome and hantavirus pulmonary syndrome, transmitted via aerosolized rodent excreta.
Alert
Viral hemorrhagic fevers represent a potential biowarfare threat. With the exception of dengue, most are transmissible via aerosols and body fluids. Some have very high morbidity and mortality, and many replicate efficiently in cell culture, allowing weaponization.
Diagnosis – Signs And Symptoms
Common symptoms include acute fever, malaise, headache, nausea, vomiting, flushing, diarrhea, abdominal pain, and myalgias.
Less common manifestations include gingival bleeding, conjunctival hemorrhage, petechiae, hematemesis, melena, epistaxis, and ecchymoses.
Hemorrhagic features usually appear after day three and may involve skin, IV sites, gums, lungs, gastrointestinal tract, or uterus. Diffuse alveolar hemorrhage and ARDS may occur, particularly in Ebola, Marburg, Lassa, Crimean-Congo hemorrhagic fever, and hantavirus infections.
Characteristic rashes include nonpruritic centripetal maculopapular eruptions in Ebola and Marburg, jaundice in Yellow fever, and blanching maculopapular truncal rash in Dengue.
Late-stage disease may progress to shock, seizures, coma, and death.
History And Physical Examination
History should assess recent travel, exposure to sick contacts, animal or insect exposure, and clustering of cases.
Strict protection of healthcare workers is essential, using universal blood and body fluid precautions.
Vital signs should be monitored closely, as narrowed pulse pressure may signal impending cardiovascular collapse.
Physical examination may reveal hemorrhage, rash, hepatomegaly, right upper quadrant tenderness, and abnormal lung findings.
Essential Workup
The primary goal is to distinguish VHF from other acute febrile illnesses, especially malaria, in returning travelers. Lung involvement suggests systemic disease and poorer prognosis. Consider the possibility of a biologic attack if multiple patients present with similar unusual findings.
Diagnostic Tests And Interpretation
Laboratory studies may show leukocytosis, leukopenia, thrombocytopenia, pancytopenia, or hemoconcentration. Elevated hematocrit suggests third spacing and impending shock. Renal and hepatic dysfunction are common. Coagulation studies may reveal DIC, particularly in Ebola, Marburg, and Crimean-Congo hemorrhagic fever.
Definitive diagnosis requires specialized biosafety level 4 laboratories using RT-PCR, viral isolation, or immunohistochemistry, coordinated through the CDC.
Imaging such as chest radiograph or CT may identify ARDS, pneumonia, or internal hemorrhage.
Differential Diagnosis
Malaria
Dengue fever
Rickettsial infections
Typhoid fever
Meningococcemia
Sepsis
Hemolytic uremic syndrome
Thrombotic thrombocytopenic purpura
Leukemia
Pit viper envenomation
Treatment – Prehospital And Initial Stabilization
Early recognition is critical due to increased globalization and imported cases. Suspected cases must be reported to the CDC immediately.
Patients should be isolated, and healthcare workers must use full protective equipment, including HEPA-filtered respirators during aerosol-generating procedures.
Emergency Department Management
Treatment is primarily supportive. Empiric antimalarial therapy should be initiated until malaria is excluded. Secondary infections should be treated aggressively.
Bleeding is usually mild; if severe, manage with blood products and clotting factors.
Fluid resuscitation must be cautious due to third spacing and risk of flash pulmonary edema. Colloids or blood products should be reserved for impending shock.
Ribavirin is effective against Lassa fever, South American hemorrhagic fever, Crimean-Congo hemorrhagic fever, and hantavirus infections but is ineffective against filoviruses.
Convalescent plasma may benefit South American hemorrhagic fever if administered early.
Medications
Ribavirin intravenous loading dose of 33 mg/kg, followed by 16 mg/kg every 6 hours for 4 days, then 8 mg/kg every 8 hours for 3 days.
Oral prophylaxis consists of 500 mg every 6 hours for 7 days.
Vaccines are available for Yellow fever, while others remain under development.
Disposition And Follow-Up
All suspected cases require admission with isolation precautions. ICU care is indicated for shock or multiorgan failure. Discharge is not appropriate when VHF is suspected.
Coordination with the CDC is mandatory for patient management and postexposure prophylaxis of contacts.
Clinical Pearls And Pitfalls
Use extreme caution with fluid resuscitation.
Hemoconcentration and pulmonary involvement are critical warning signs.
Always consider viral hemorrhagic fever in febrile patients returning from endemic regions.
Strict isolation and universal precautions are essential to prevent transmission.
Notify the CDC immediately for all suspected cases.
Basics And Description
Hemorrhagic fever refers to a multisystem syndrome characterized by vasocapillary permeability, hemorrhage, and organ dysfunction. Viral hemorrhagic fevers (VHF) are caused by a distinct group of viruses and often begin with a nonspecific influenza-like illness. Hemorrhagic manifestations occur in a minority of patients and typically appear in later stages of disease.
Risk Factors
Travel to endemic regions
Exposure related to biologic warfare
Close contact with animals
Insect bites or ingestion
Pathophysiology
Viral hemorrhagic fevers cause endothelial injury leading to increased vascular permeability, hemorrhage, and progression to shock. The shock state is both hypovolemic and distributive and is often difficult to reverse. Rapid progression to hypotension indicates a very high mortality risk. Disseminated intravascular coagulation is commonly seen in Marburg and Crimean-Congo hemorrhagic fever but is less frequent in arenavirus infections. Dengue hemorrhagic fever is immune mediated and often results from secondary infection and is among the most common causes of VHF.
Etiology
Viral hemorrhagic fevers are caused by RNA viruses with zoonotic life cycles and short incubation periods of less than 10–21 days.
Filoviruses include Ebola and Marburg, with fruit bat reservoirs and transmission primarily in sub-Saharan Africa.
Arenaviruses include Lassa fever and South American hemorrhagic fevers, transmitted via aerosolized rodent excreta.
Flaviviruses include Dengue and Yellow fever, transmitted by mosquitoes, primarily in tropical regions.
Bunyaviridae include Rift Valley fever and Crimean-Congo hemorrhagic fever, transmitted by ticks or mosquitoes.
Hantaviridae include hemorrhagic fever with renal syndrome and hantavirus pulmonary syndrome, transmitted via aerosolized rodent excreta.
Alert
Viral hemorrhagic fevers represent a potential biowarfare threat. With the exception of dengue, most are transmissible via aerosols and body fluids. Some have very high morbidity and mortality, and many replicate efficiently in cell culture, allowing weaponization.
Diagnosis – Signs And Symptoms
Common symptoms include acute fever, malaise, headache, nausea, vomiting, flushing, diarrhea, abdominal pain, and myalgias.
Less common manifestations include gingival bleeding, conjunctival hemorrhage, petechiae, hematemesis, melena, epistaxis, and ecchymoses.
Hemorrhagic features usually appear after day three and may involve skin, IV sites, gums, lungs, gastrointestinal tract, or uterus. Diffuse alveolar hemorrhage and ARDS may occur, particularly in Ebola, Marburg, Lassa, Crimean-Congo hemorrhagic fever, and hantavirus infections.
Characteristic rashes include nonpruritic centripetal maculopapular eruptions in Ebola and Marburg, jaundice in Yellow fever, and blanching maculopapular truncal rash in Dengue.
Late-stage disease may progress to shock, seizures, coma, and death.
History And Physical Examination
History should assess recent travel, exposure to sick contacts, animal or insect exposure, and clustering of cases.
Strict protection of healthcare workers is essential, using universal blood and body fluid precautions.
Vital signs should be monitored closely, as narrowed pulse pressure may signal impending cardiovascular collapse.
Physical examination may reveal hemorrhage, rash, hepatomegaly, right upper quadrant tenderness, and abnormal lung findings.
Essential Workup
The primary goal is to distinguish VHF from other acute febrile illnesses, especially malaria, in returning travelers. Lung involvement suggests systemic disease and poorer prognosis. Consider the possibility of a biologic attack if multiple patients present with similar unusual findings.
Diagnostic Tests And Interpretation
Laboratory studies may show leukocytosis, leukopenia, thrombocytopenia, pancytopenia, or hemoconcentration. Elevated hematocrit suggests third spacing and impending shock. Renal and hepatic dysfunction are common. Coagulation studies may reveal DIC, particularly in Ebola, Marburg, and Crimean-Congo hemorrhagic fever.
Definitive diagnosis requires specialized biosafety level 4 laboratories using RT-PCR, viral isolation, or immunohistochemistry, coordinated through the CDC.
Imaging such as chest radiograph or CT may identify ARDS, pneumonia, or internal hemorrhage.
Differential Diagnosis
Malaria
Dengue fever
Rickettsial infections
Typhoid fever
Meningococcemia
Sepsis
Hemolytic uremic syndrome
Thrombotic thrombocytopenic purpura
Leukemia
Pit viper envenomation
Treatment – Prehospital And Initial Stabilization
Early recognition is critical due to increased globalization and imported cases. Suspected cases must be reported to the CDC immediately.
Patients should be isolated, and healthcare workers must use full protective equipment, including HEPA-filtered respirators during aerosol-generating procedures.
Emergency Department Management
Treatment is primarily supportive. Empiric antimalarial therapy should be initiated until malaria is excluded. Secondary infections should be treated aggressively.
Bleeding is usually mild; if severe, manage with blood products and clotting factors.
Fluid resuscitation must be cautious due to third spacing and risk of flash pulmonary edema. Colloids or blood products should be reserved for impending shock.
Ribavirin is effective against Lassa fever, South American hemorrhagic fever, Crimean-Congo hemorrhagic fever, and hantavirus infections but is ineffective against filoviruses.
Convalescent plasma may benefit South American hemorrhagic fever if administered early.
Medications
Ribavirin intravenous loading dose of 33 mg/kg, followed by 16 mg/kg every 6 hours for 4 days, then 8 mg/kg every 8 hours for 3 days.
Oral prophylaxis consists of 500 mg every 6 hours for 7 days.
Vaccines are available for Yellow fever, while others remain under development.
Disposition And Follow-Up
All suspected cases require admission with isolation precautions. ICU care is indicated for shock or multiorgan failure. Discharge is not appropriate when VHF is suspected.
Coordination with the CDC is mandatory for patient management and postexposure prophylaxis of contacts.
Clinical Pearls And Pitfalls
Use extreme caution with fluid resuscitation.
Hemoconcentration and pulmonary involvement are critical warning signs.
Always consider viral hemorrhagic fever in febrile patients returning from endemic regions.
Strict isolation and universal precautions are essential to prevent transmission.
Notify the CDC immediately for all suspected cases.
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Emergency And Acute Medicine – Hemorrhoid
Basics And Description
Hemorrhoids are normal venous sinusoids of the distal rectum and proximal anal canal that function as vascular cushions contributing to anal continence. These cushions consist of thick submucosa containing blood vessels, smooth muscle, and elastic connective tissue. An arteriovenous shunt system at the level of the internal hemorrhoids explains the typical presentation of bright red blood per rectum. Hemorrhoid disease develops when these normal structures become symptomatic. Hemorrhoids are generally painless unless thrombosed or strangulated. Pathologically, disease occurs when portions of the anal canal lining slide downward.
External hemorrhoids are located below the dentate line, covered by skin or anoderm, and drain into the internal iliac venous system. Internal hemorrhoids arise from submucosal vessels above the dentate line, drain into the portal venous system, and are typically found at the left lateral, right posterolateral, and right anterolateral positions. Internal hemorrhoids are classified by degree of prolapse: grade 1 causes painless bleeding without prolapse; grade 2 prolapses with bowel movements but reduces spontaneously; grade 3 prolapses with bowel movements and requires manual reduction; grade 4 is chronically prolapsed and not reducible.
Etiology
The exact cause of hemorrhoid disease is unclear. Gravitational forces and increased abdominal pressure contribute to distention of venous sinusoids. It is commonly associated with straining and irregular bowel habits, including hard, bulky stools or diarrhea, which cause tenesmus and excessive straining. These forces weaken submucosal support tissue, promoting prolapse of anal cushions. Additional contributing factors include higher resting anal pressures related to erect posture, hereditary absence of venous valves, and chronically elevated intra-abdominal pressure from conditions such as pregnancy, ascites, and portal hypertension.
Diagnosis – Signs And Symptoms
Patients most commonly present with painless rectal bleeding during or after defecation. Blood is typically bright red and may appear on toilet paper, coat the stool, or drip into the toilet bowl. Rectal discomfort or a sensation of pressure may occur. Severe pain suggests thrombosed external hemorrhoids or prolapsed internal hemorrhoids that have become strangulated. Pruritus ani is common, and anal fissures may coexist.
History
Key historical features include duration and severity of bleeding, association with pain, presence of new perianal masses, stool consistency, prior anorectal disease, changes in stool caliber, and use of anticoagulants.
Physical Examination
Examination begins with inspection of the perianal area by gently separating the buttocks. A discrete, dark blue, tender mass covered by skin is consistent with a thrombosed external hemorrhoid and may coexist with an internal component. A purplish, tender, mucosa-covered mass suggests a prolapsed and strangulated internal hemorrhoid, often accompanied by thrombosed external hemorrhoids. Asking the patient to bear down may reveal prolapse. Digital rectal examination is mandatory to exclude malignancy. Anoscopy allows direct visualization of the anal canal and identification of bleeding internal hemorrhoids.
Essential Workup
Diagnosis is primarily clinical and based on a detailed history and thorough anorectal examination.
Diagnostic Tests And Interpretation
Laboratory testing is not routinely required. A complete blood count is indicated if there is significant bleeding to assess hemoglobin and hematocrit. Platelet count and coagulation studies (PT, PTT, INR) should be obtained in patients on anticoagulation or with significant comorbid disease.
Differential Diagnosis
Rectal prolapse, anal fissure, perirectal abscess or fistula, condyloma acuminata, anorectal carcinoma, and melanoma.
Treatment – Prehospital Care
Establish intravenous access in cases of severe bleeding.
Initial Stabilization And Therapy
Apply direct digital pressure to control active bleeding when present.
Emergency Department Treatment And Procedures
Conservative therapy is the foundation of treatment for all patients. This includes warm sitz baths for 15 minutes three times daily and after bowel movements, a high-fiber diet targeting approximately 30 g/day, increased oral hydration, stool softeners, bulk-forming laxatives, and analgesia with NSAIDs or acetaminophen.
Thrombosed external hemorrhoids causing severe pain may be excised if symptoms are present for fewer than five days and the clot has not begun to resolve. The procedure is performed with the patient in the prone jackknife or left lateral decubitus position. After local anesthesia with lidocaine containing epinephrine, an elliptical incision is made to excise the clot and overlying skin. Hemostasis may require silver nitrate. A small piece of Gelfoam or gauze is applied, and the dressing is removed at the first sitz bath approximately six hours later.
Nonthrombosed prolapsed internal hemorrhoids may be manually reduced, sometimes requiring topical anesthesia or an anal sphincter block. Bleeding internal hemorrhoids may be treated with sclerotherapy using agents such as sodium morrhuate or hypertonic saline, or with rubber band ligation of one or two hemorrhoids, which should be avoided in immunocompromised patients. Nonstrangulated, nonreducible hemorrhoids are managed conservatively with surgical referral, whereas strangulated internal hemorrhoids require immediate surgical consultation.
Pregnancy-associated hemorrhoids are common in the third trimester and are managed conservatively. Certain topical agents should be avoided due to fetal risk.
Medications
Analgesics include acetaminophen with or without codeine and NSAIDs such as ibuprofen. Stool regulation is achieved with fiber supplements, psyllium, and docusate sodium. Topical therapies include lidocaine-based anorectal creams, hydrocortisone-containing preparations, and 0.2% nitroglycerin ointment applied locally to reduce sphincter spasm and pain. Caution is required with topical anesthetics due to systemic absorption.
Follow-Up And Disposition
Admission is indicated for strangulated grade 4 hemorrhoids requiring urgent surgery, severe anemia due to bleeding, or significant bleeding in patients on anticoagulation or with portal hypertension. Most patients can be discharged with outpatient management.
Issues For Referral
Surgical referral is indicated for grade 3 or 4 internal hemorrhoids, suspected malignancy, inflammatory bowel disease, coagulopathy, pregnancy, or immunocompromised status.
Follow-Up Recommendations
Patients with advanced hemorrhoids or concern for neoplasia should have colorectal surgery follow-up. Uncomplicated cases should follow up with primary care. All patients with bright red blood per rectum should be referred for gastrointestinal or colorectal evaluation to exclude malignancy.
Key Clinical Insights And Common Pitfalls
Hemorrhoids are a common but not exclusive cause of anorectal bleeding and pain. Failure to evaluate for alternative diagnoses, including malignancy, is a frequent pitfall.
Basics And Description
Hemorrhoids are normal venous sinusoids of the distal rectum and proximal anal canal that function as vascular cushions contributing to anal continence. These cushions consist of thick submucosa containing blood vessels, smooth muscle, and elastic connective tissue. An arteriovenous shunt system at the level of the internal hemorrhoids explains the typical presentation of bright red blood per rectum. Hemorrhoid disease develops when these normal structures become symptomatic. Hemorrhoids are generally painless unless thrombosed or strangulated. Pathologically, disease occurs when portions of the anal canal lining slide downward.
External hemorrhoids are located below the dentate line, covered by skin or anoderm, and drain into the internal iliac venous system. Internal hemorrhoids arise from submucosal vessels above the dentate line, drain into the portal venous system, and are typically found at the left lateral, right posterolateral, and right anterolateral positions. Internal hemorrhoids are classified by degree of prolapse: grade 1 causes painless bleeding without prolapse; grade 2 prolapses with bowel movements but reduces spontaneously; grade 3 prolapses with bowel movements and requires manual reduction; grade 4 is chronically prolapsed and not reducible.
Etiology
The exact cause of hemorrhoid disease is unclear. Gravitational forces and increased abdominal pressure contribute to distention of venous sinusoids. It is commonly associated with straining and irregular bowel habits, including hard, bulky stools or diarrhea, which cause tenesmus and excessive straining. These forces weaken submucosal support tissue, promoting prolapse of anal cushions. Additional contributing factors include higher resting anal pressures related to erect posture, hereditary absence of venous valves, and chronically elevated intra-abdominal pressure from conditions such as pregnancy, ascites, and portal hypertension.
Diagnosis – Signs And Symptoms
Patients most commonly present with painless rectal bleeding during or after defecation. Blood is typically bright red and may appear on toilet paper, coat the stool, or drip into the toilet bowl. Rectal discomfort or a sensation of pressure may occur. Severe pain suggests thrombosed external hemorrhoids or prolapsed internal hemorrhoids that have become strangulated. Pruritus ani is common, and anal fissures may coexist.
History
Key historical features include duration and severity of bleeding, association with pain, presence of new perianal masses, stool consistency, prior anorectal disease, changes in stool caliber, and use of anticoagulants.
Physical Examination
Examination begins with inspection of the perianal area by gently separating the buttocks. A discrete, dark blue, tender mass covered by skin is consistent with a thrombosed external hemorrhoid and may coexist with an internal component. A purplish, tender, mucosa-covered mass suggests a prolapsed and strangulated internal hemorrhoid, often accompanied by thrombosed external hemorrhoids. Asking the patient to bear down may reveal prolapse. Digital rectal examination is mandatory to exclude malignancy. Anoscopy allows direct visualization of the anal canal and identification of bleeding internal hemorrhoids.
Essential Workup
Diagnosis is primarily clinical and based on a detailed history and thorough anorectal examination.
Diagnostic Tests And Interpretation
Laboratory testing is not routinely required. A complete blood count is indicated if there is significant bleeding to assess hemoglobin and hematocrit. Platelet count and coagulation studies (PT, PTT, INR) should be obtained in patients on anticoagulation or with significant comorbid disease.
Differential Diagnosis
Rectal prolapse, anal fissure, perirectal abscess or fistula, condyloma acuminata, anorectal carcinoma, and melanoma.
Treatment – Prehospital Care
Establish intravenous access in cases of severe bleeding.
Initial Stabilization And Therapy
Apply direct digital pressure to control active bleeding when present.
Emergency Department Treatment And Procedures
Conservative therapy is the foundation of treatment for all patients. This includes warm sitz baths for 15 minutes three times daily and after bowel movements, a high-fiber diet targeting approximately 30 g/day, increased oral hydration, stool softeners, bulk-forming laxatives, and analgesia with NSAIDs or acetaminophen.
Thrombosed external hemorrhoids causing severe pain may be excised if symptoms are present for fewer than five days and the clot has not begun to resolve. The procedure is performed with the patient in the prone jackknife or left lateral decubitus position. After local anesthesia with lidocaine containing epinephrine, an elliptical incision is made to excise the clot and overlying skin. Hemostasis may require silver nitrate. A small piece of Gelfoam or gauze is applied, and the dressing is removed at the first sitz bath approximately six hours later.
Nonthrombosed prolapsed internal hemorrhoids may be manually reduced, sometimes requiring topical anesthesia or an anal sphincter block. Bleeding internal hemorrhoids may be treated with sclerotherapy using agents such as sodium morrhuate or hypertonic saline, or with rubber band ligation of one or two hemorrhoids, which should be avoided in immunocompromised patients. Nonstrangulated, nonreducible hemorrhoids are managed conservatively with surgical referral, whereas strangulated internal hemorrhoids require immediate surgical consultation.
Pregnancy-associated hemorrhoids are common in the third trimester and are managed conservatively. Certain topical agents should be avoided due to fetal risk.
Medications
Analgesics include acetaminophen with or without codeine and NSAIDs such as ibuprofen. Stool regulation is achieved with fiber supplements, psyllium, and docusate sodium. Topical therapies include lidocaine-based anorectal creams, hydrocortisone-containing preparations, and 0.2% nitroglycerin ointment applied locally to reduce sphincter spasm and pain. Caution is required with topical anesthetics due to systemic absorption.
Follow-Up And Disposition
Admission is indicated for strangulated grade 4 hemorrhoids requiring urgent surgery, severe anemia due to bleeding, or significant bleeding in patients on anticoagulation or with portal hypertension. Most patients can be discharged with outpatient management.
Issues For Referral
Surgical referral is indicated for grade 3 or 4 internal hemorrhoids, suspected malignancy, inflammatory bowel disease, coagulopathy, pregnancy, or immunocompromised status.
Follow-Up Recommendations
Patients with advanced hemorrhoids or concern for neoplasia should have colorectal surgery follow-up. Uncomplicated cases should follow up with primary care. All patients with bright red blood per rectum should be referred for gastrointestinal or colorectal evaluation to exclude malignancy.
Key Clinical Insights And Common Pitfalls
Hemorrhoids are a common but not exclusive cause of anorectal bleeding and pain. Failure to evaluate for alternative diagnoses, including malignancy, is a frequent pitfall.
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Emergency And Acute Medicine – Hemothorax
Basics And Description
Hemothorax is the accumulation of blood within the intrapleural space, most commonly following blunt or penetrating chest trauma, though nontraumatic causes exist. Bleeding usually results from disruption of chest wall tissues, pleura, or intrathoracic vessels, leading to decreased vital capacity, hypoxia, and respiratory compromise. Significant intravascular blood loss may cause hemodynamic instability and hemorrhagic shock. Massive hemothorax can increase intrathoracic pressure, impair venous return, and reduce cardiac output. In blunt trauma, hemothorax is rarely isolated and is frequently associated with pneumothorax, extrathoracic injuries, and pulmonary contusion. Large collections may release anticoagulant substances, promoting continued bleeding. Untreated hemothorax can progress to empyema or fibrothorax due to pleural adhesions and lung trapping.
Etiology
Traumatic causes include injury to major thoracic vessels such as intercostal arteries, internal mammary arteries, pulmonary vessels, the aorta, vena cava, and the heart. Lung parenchymal injuries may also cause bleeding, which often stops spontaneously because of low pulmonary pressures and high thromboplastin levels, and are frequently associated with pneumothorax. Nontraumatic or spontaneous hemothorax is rare and should prompt evaluation for coagulation disorders, malignancy, vascular catastrophes such as aortic dissection or ruptured aneurysm, pulmonary embolism with infarction, tuberculosis, bullous emphysema, pulmonary arteriovenous malformations, or lobar sequestration. Hemothorax may also occur from torn pleural adhesions during spontaneous pneumothorax or following tube thoracostomy.
Diagnosis – Signs And Symptoms
Small hemothoraces, generally less than 400 mL, may cause minimal or no changes in vital signs or physical examination. Large hemothoraces, typically exceeding 1,000 mL, present with restlessness, anxiety, pallor, pleuritic chest pain, hemoptysis, dyspnea, and air hunger. Signs of hemorrhagic shock appear when blood loss reaches or exceeds 30% of circulating volume, including tachycardia, tachypnea, and hypotension. In cases with slow or insidious onset, such as malignancy-related hemothorax, dyspnea is often the predominant symptom without acute hemodynamic compromise.
History
Important historical features include recent blunt or penetrating chest trauma, rib fractures, flail chest, or delayed onset of symptoms hours to days after injury. Delayed hemothorax may result from rupture of chest wall hematomas or intercostal vessel injury from rib fracture movement. Additional history includes known malignancy, metastatic disease, or recent thoracic procedures such as thoracentesis or chest tube placement.
Physical Examination
Vital signs may reveal hypoxia, tachypnea, tachycardia, or hypotension depending on severity. Jugular venous distention may be present with increased intrathoracic pressure, and tracheal deviation can occur in massive collections. Chest inspection may show asymmetric expansion, deformity, contusions, abrasions, or paradoxical movement. Palpation may elicit rib tenderness, crepitus, or subcutaneous emphysema, while percussion typically reveals dullness over the affected hemithorax. Auscultation demonstrates decreased or absent breath sounds, best appreciated in the upright patient.
Essential Workup
Chest radiography is the primary diagnostic study. In stable patients, an upright posteroanterior film is ideal and can detect pleural fluid volumes greater than 200–300 mL, seen as meniscus formation or blunting of the costophrenic angle. In supine trauma patients, up to 1,000 mL of blood may be missed, appearing only as diffuse haziness of the hemithorax. Associated injuries such as pneumothorax, rib fractures, pulmonary contusions, or mediastinal widening should be assessed.
Diagnostic Tests And Interpretation
Laboratory evaluation includes hematocrit monitoring for trends, type and cross-match, pulse oximetry, and arterial blood gas analysis when indicated. Pleural fluid hematocrit exceeding 50% of peripheral blood hematocrit confirms hemothorax. Ultrasound, particularly extended FAST, is highly sensitive for detecting intrapleural fluid in trained hands. Computed tomography is useful for identifying small hemothoraces not visible on chest radiographs and for evaluating associated injuries.
Differential Diagnosis
Hemopneumothorax, pneumothorax, pulmonary contusion, pleural effusion, and empyema or pneumonia.
Treatment – Prehospital Care
Initial management includes assessment of vital signs and oxygen saturation, supplemental oxygen, intravenous access, and fluid resuscitation for hypotension. Clinical differentiation between hemothorax and pneumothorax may be difficult; subcutaneous emphysema suggests pneumothorax, while dullness to percussion favors hemothorax. In unstable patients with suspected tension pneumothorax, needle thoracostomy should be performed immediately.
Initial Stabilization And Therapy
Airway, breathing, and circulation are prioritized. Endotracheal intubation is indicated for impending respiratory failure. High-flow oxygen, two large-bore intravenous lines, and rapid fluid resuscitation are initiated. If chest tube equipment is not immediately available in an unstable patient, needle thoracostomy should not be delayed. Patients should be positioned upright when possible.
Emergency Department Treatment And Procedures
Definitive management involves evacuation of blood from the pleural space via tube thoracostomy. A large-bore chest tube, typically 36–40 French, is inserted in the 4th or 5th intercostal space at the mid-axillary line, directed posteriorly and superiorly, and connected to underwater seal drainage with suction. Post-procedure chest radiography confirms placement and lung re-expansion. Autotransfusion should be considered when available. Indications for operative thoracotomy include initial chest tube output greater than 20 mL/kg in children or approximately 1,000 mL in adults, persistent bleeding exceeding 7 mL/kg/hr or 200 mL/hr for four hours, enlarging hemothorax, refractory hypotension, or clinical deterioration after initial response. Emergency department thoracotomy may be indicated in select cases of penetrating trauma with recent arrest or profound shock, and rarely in blunt trauma under specialized circumstances.
Medications
Local anesthetics are used for chest tube placement in awake patients. Procedural sedation and analgesia with agents such as fentanyl and midazolam may be administered in stable patients. Additional sedatives may be used as clinically appropriate.
Follow-Up And Disposition
Patients requiring tube thoracostomy should be admitted to a trauma, cardiothoracic, or surgical service experienced in chest tube management. Small, isolated hemothoraces detected incidentally may be observed for 4–6 hours and discharged if asymptomatic, normoxic, and without evidence of ongoing bleeding. Asymptomatic blunt chest trauma patients with normal initial radiographs do not require repeat imaging prior to discharge.
Key Clinical Insights And Common Pitfalls
The pleural cavity can hold more than 4 liters of blood, allowing massive hemorrhage without external bleeding. In supine patients, physical findings may be subtle due to posterior blood distribution. Hemothorax may be misread as pneumonia in the absence of trauma history. Concurrent diaphragmatic injury raises concern for intra-abdominal bleeding. Early preparation for autotransfusion is important, as the greatest blood loss often occurs at initial chest tube placement. Improper chest tube positioning, especially too anterior or superior, can impair drainage; all fenestrations must lie within the thoracic cavity. Prophylactic antibiotics with chest tube placement do not reduce pneumonia or empyema risk.
Basics And Description
Hemothorax is the accumulation of blood within the intrapleural space, most commonly following blunt or penetrating chest trauma, though nontraumatic causes exist. Bleeding usually results from disruption of chest wall tissues, pleura, or intrathoracic vessels, leading to decreased vital capacity, hypoxia, and respiratory compromise. Significant intravascular blood loss may cause hemodynamic instability and hemorrhagic shock. Massive hemothorax can increase intrathoracic pressure, impair venous return, and reduce cardiac output. In blunt trauma, hemothorax is rarely isolated and is frequently associated with pneumothorax, extrathoracic injuries, and pulmonary contusion. Large collections may release anticoagulant substances, promoting continued bleeding. Untreated hemothorax can progress to empyema or fibrothorax due to pleural adhesions and lung trapping.
Etiology
Traumatic causes include injury to major thoracic vessels such as intercostal arteries, internal mammary arteries, pulmonary vessels, the aorta, vena cava, and the heart. Lung parenchymal injuries may also cause bleeding, which often stops spontaneously because of low pulmonary pressures and high thromboplastin levels, and are frequently associated with pneumothorax. Nontraumatic or spontaneous hemothorax is rare and should prompt evaluation for coagulation disorders, malignancy, vascular catastrophes such as aortic dissection or ruptured aneurysm, pulmonary embolism with infarction, tuberculosis, bullous emphysema, pulmonary arteriovenous malformations, or lobar sequestration. Hemothorax may also occur from torn pleural adhesions during spontaneous pneumothorax or following tube thoracostomy.
Diagnosis – Signs And Symptoms
Small hemothoraces, generally less than 400 mL, may cause minimal or no changes in vital signs or physical examination. Large hemothoraces, typically exceeding 1,000 mL, present with restlessness, anxiety, pallor, pleuritic chest pain, hemoptysis, dyspnea, and air hunger. Signs of hemorrhagic shock appear when blood loss reaches or exceeds 30% of circulating volume, including tachycardia, tachypnea, and hypotension. In cases with slow or insidious onset, such as malignancy-related hemothorax, dyspnea is often the predominant symptom without acute hemodynamic compromise.
History
Important historical features include recent blunt or penetrating chest trauma, rib fractures, flail chest, or delayed onset of symptoms hours to days after injury. Delayed hemothorax may result from rupture of chest wall hematomas or intercostal vessel injury from rib fracture movement. Additional history includes known malignancy, metastatic disease, or recent thoracic procedures such as thoracentesis or chest tube placement.
Physical Examination
Vital signs may reveal hypoxia, tachypnea, tachycardia, or hypotension depending on severity. Jugular venous distention may be present with increased intrathoracic pressure, and tracheal deviation can occur in massive collections. Chest inspection may show asymmetric expansion, deformity, contusions, abrasions, or paradoxical movement. Palpation may elicit rib tenderness, crepitus, or subcutaneous emphysema, while percussion typically reveals dullness over the affected hemithorax. Auscultation demonstrates decreased or absent breath sounds, best appreciated in the upright patient.
Essential Workup
Chest radiography is the primary diagnostic study. In stable patients, an upright posteroanterior film is ideal and can detect pleural fluid volumes greater than 200–300 mL, seen as meniscus formation or blunting of the costophrenic angle. In supine trauma patients, up to 1,000 mL of blood may be missed, appearing only as diffuse haziness of the hemithorax. Associated injuries such as pneumothorax, rib fractures, pulmonary contusions, or mediastinal widening should be assessed.
Diagnostic Tests And Interpretation
Laboratory evaluation includes hematocrit monitoring for trends, type and cross-match, pulse oximetry, and arterial blood gas analysis when indicated. Pleural fluid hematocrit exceeding 50% of peripheral blood hematocrit confirms hemothorax. Ultrasound, particularly extended FAST, is highly sensitive for detecting intrapleural fluid in trained hands. Computed tomography is useful for identifying small hemothoraces not visible on chest radiographs and for evaluating associated injuries.
Differential Diagnosis
Hemopneumothorax, pneumothorax, pulmonary contusion, pleural effusion, and empyema or pneumonia.
Treatment – Prehospital Care
Initial management includes assessment of vital signs and oxygen saturation, supplemental oxygen, intravenous access, and fluid resuscitation for hypotension. Clinical differentiation between hemothorax and pneumothorax may be difficult; subcutaneous emphysema suggests pneumothorax, while dullness to percussion favors hemothorax. In unstable patients with suspected tension pneumothorax, needle thoracostomy should be performed immediately.
Initial Stabilization And Therapy
Airway, breathing, and circulation are prioritized. Endotracheal intubation is indicated for impending respiratory failure. High-flow oxygen, two large-bore intravenous lines, and rapid fluid resuscitation are initiated. If chest tube equipment is not immediately available in an unstable patient, needle thoracostomy should not be delayed. Patients should be positioned upright when possible.
Emergency Department Treatment And Procedures
Definitive management involves evacuation of blood from the pleural space via tube thoracostomy. A large-bore chest tube, typically 36–40 French, is inserted in the 4th or 5th intercostal space at the mid-axillary line, directed posteriorly and superiorly, and connected to underwater seal drainage with suction. Post-procedure chest radiography confirms placement and lung re-expansion. Autotransfusion should be considered when available. Indications for operative thoracotomy include initial chest tube output greater than 20 mL/kg in children or approximately 1,000 mL in adults, persistent bleeding exceeding 7 mL/kg/hr or 200 mL/hr for four hours, enlarging hemothorax, refractory hypotension, or clinical deterioration after initial response. Emergency department thoracotomy may be indicated in select cases of penetrating trauma with recent arrest or profound shock, and rarely in blunt trauma under specialized circumstances.
Medications
Local anesthetics are used for chest tube placement in awake patients. Procedural sedation and analgesia with agents such as fentanyl and midazolam may be administered in stable patients. Additional sedatives may be used as clinically appropriate.
Follow-Up And Disposition
Patients requiring tube thoracostomy should be admitted to a trauma, cardiothoracic, or surgical service experienced in chest tube management. Small, isolated hemothoraces detected incidentally may be observed for 4–6 hours and discharged if asymptomatic, normoxic, and without evidence of ongoing bleeding. Asymptomatic blunt chest trauma patients with normal initial radiographs do not require repeat imaging prior to discharge.
Key Clinical Insights And Common Pitfalls
The pleural cavity can hold more than 4 liters of blood, allowing massive hemorrhage without external bleeding. In supine patients, physical findings may be subtle due to posterior blood distribution. Hemothorax may be misread as pneumonia in the absence of trauma history. Concurrent diaphragmatic injury raises concern for intra-abdominal bleeding. Early preparation for autotransfusion is important, as the greatest blood loss often occurs at initial chest tube placement. Improper chest tube positioning, especially too anterior or superior, can impair drainage; all fenestrations must lie within the thoracic cavity. Prophylactic antibiotics with chest tube placement do not reduce pneumonia or empyema risk.
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Emergency And Acute Medicine – Hepatic Encephalopathy
Basics And Description
Hepatic encephalopathy (HE) is a neuropsychiatric syndrome characterized by alterations in behavior, level of consciousness, and motor function resulting from hepatic insufficiency and the accumulation of substances normally detoxified by the liver. The condition arises from a multifactorial process, most notably the accumulation of ammonia produced from protein degradation by colonic bacteria and deamination of glutamine in the small bowel, kidneys, and muscle. Ammonia crosses the blood–brain barrier and is metabolized by astrocytes into glutamine, leading to astrocytic swelling, cerebral edema, and mitochondrial dysfunction. Additional neurotoxins such as short-chain fatty acids, manganese, neurosteroids, phenols, mercaptans, and amino acids including tryptophan also contribute. Neurotransmitter imbalance plays a role, with increased inhibitory neurotransmitters such as benzodiazepines, γ-aminobutyric acid, and serotonin, and decreased excitatory neurotransmitters including glutamate, dopamine, aspartate, and catecholamines. Other contributing factors include reduced cerebral blood flow and oxygen delivery, increased cerebral glucose utilization with potential hypoglycemia, zinc deficiency affecting urea cycle enzymes, and rare inherited urea cycle disorders.
Etiology
Hepatic encephalopathy is classified according to the 11th World Congress of Gastroenterology. Type A is associated with acute liver injury and fulminant hepatic failure. Type B is related to portosystemic shunting without intrinsic liver disease. Type C is associated with cirrhosis and portal hypertension. Common precipitating factors include gastrointestinal bleeding, electrolyte abnormalities such as hypokalemia and hyponatremia, metabolic alkalosis reducing renal ammonium excretion, sepsis including spontaneous bacterial peritonitis, constipation, noncompliance with treatment in chronic liver disease, high-protein diet, hypoglycemia, hypovolemia following large-volume paracentesis, azotemia from diuretics or diarrhea, sedatives or narcotics including alcohol, zinc deficiency, hepatocellular injury from viral or drug-induced hepatitis, and post portosystemic shunt placement. Recurrent episodes may occur even without identifiable triggers.
Diagnosis – Signs And Symptoms
Type A HE progresses rapidly and may lead to seizures, decerebrate rigidity, coma, and death from cerebral edema. Type B and C HE are typically chronic and may present as minimal or overt encephalopathy. Minimal HE manifests as impaired psychomotor speed, attention, concentration, visual perception, memory deficits, and slowed mental processing, detectable only with psychometric testing. Overt HE includes slow and monotonous speech, impaired fine motor skills, hyperreflexia, clonus, hyperventilation, extrapyramidal movements, seizures, confusion, coma, and abnormal posturing. Episodic HE is characterized by transient changes in consciousness over hours to days with return to baseline after treatment, whereas persistent HE does not fully resolve.
Clinical Grading (West Haven Criteria)
Stage 0 shows no overt clinical changes but abnormal neuropsychological testing and no asterixis. Stage I includes personality changes, irritability, depression or euphoria, sleep–wake reversal, impaired writing or calculations, and possible asterixis. Stage II is marked by inappropriate behavior, lethargy, slowed responses, asterixis, slurred speech, and ataxia. Stage III presents with disorientation, amnesia, paranoia, nystagmus, hypoactive reflexes, positive Babinski sign, and stupor. Stage IV is characterized by coma and dilated pupils.
Essential Workup
Evaluation should include a detailed history of liver disease and prior HE episodes, identification of precipitating factors, and assessment for electrolyte disturbances, as even mild abnormalities may cause significant clinical deterioration.
Diagnostic Tests And Interpretation
Ammonia levels correlate poorly with disease severity but may assist in evaluating unexplained altered mental status. Samples must be handled correctly and processed promptly. A normal ammonia level should prompt investigation for alternative diagnoses. Additional studies include fecal occult blood testing and nasogastric lavage to assess for gastrointestinal bleeding, complete blood count for anemia or infection, electrolytes, renal function, glucose, coagulation studies, and liver enzymes. Urinalysis and cultures of urine and ascitic fluid help identify infection. Toxicology screening may be required to exclude other causes of altered consciousness. Additional testing may include thyroid function, arterial blood gases, magnesium, and viral serologies. Imaging includes chest radiography for pneumonia or heart failure and head CT for new-onset altered mental status, focal deficits, trauma, or suspected cerebral edema. Brain MRI may aid diagnosis in patients with portosystemic shunts without intrinsic liver disease. ECG assesses arrhythmias and electrolyte effects. Lumbar puncture is indicated for unexplained or worsening symptoms, and paracentesis is required when spontaneous bacterial peritonitis is suspected. EEG typically shows generalized slowing.
Differential Diagnosis
Consider alcohol withdrawal, cerebrovascular accident, congestive heart failure, carbon dioxide narcosis, head trauma, electrolyte disturbances, hypoglycemia, meningitis, encephalitis, metabolic encephalopathies, psychiatric illness, drug or alcohol intoxication, uremia, and in children, Reye syndrome or fatty acid oxidation disorders.
Initial Stabilization And Therapy
Management includes supplemental oxygen, cardiac monitoring, and airway protection, with intubation required for grade III or IV encephalopathy. Propofol is preferred for sedation if needed. Standard initial altered mental status treatment includes naloxone, glucose administration, and thiamine.
Emergency Department Treatment And Procedures
Identification and correction of precipitating factors is central to management. Frequent glucose monitoring is essential due to the risk of hypoglycemia. Complications such as gastrointestinal bleeding, sepsis, electrolyte abnormalities, coagulopathy, and renal dysfunction must be treated promptly. Sedatives and narcotics should be avoided when possible; if required, agents with minimal hepatic metabolism are preferred. Ammonia reduction is achieved with nonabsorbable disaccharides such as lactulose, administered orally, via nasogastric tube, or as retention enemas in severe cases. Intestinal flora reduction may include rifaximin, neomycin, metronidazole, or vancomycin, often in combination with lactulose. Rifaximin is preferred for lactulose-resistant HE due to its efficacy and favorable safety profile. Cerebral edema should be treated promptly if present. Zinc deficiency should be corrected, and short-term protein restriction may be considered. Flumazenil may provide transient improvement in select patients but should be avoided in chronic benzodiazepine or alcohol users. Liver transplantation is the definitive therapy for severe or recurrent HE. Additional therapies under investigation include L-carnitine, albumin dialysis, N-acetylcysteine, probiotics, branched-chain amino acids, and manganese chelation.
Medications
Therapy may include intravenous dextrose for hypoglycemia, lactulose titrated to achieve two to three soft stools daily, rifaximin 550 mg twice daily, neomycin or metronidazole when indicated, naloxone for suspected opioid effect, thiamine supplementation, zinc acetate or sulfate, and mannitol for cerebral edema.
Follow-Up And Disposition
Patients with grade II to IV HE, type A HE of any grade, type B or C HE grade II or higher, associated complications such as sepsis or gastrointestinal bleeding, or diagnostic uncertainty require admission, often to the intensive care unit with gastroenterology consultation. Discharge may be considered for patients with known chronic HE presenting with grade 0 or I symptoms, a reversible cause, and reliable supervision. Discharged patients should receive lactulose therapy and dietary guidance.
Follow-Up Recommendations
Patients should realize the importance of dietary counseling, medication adherence, and alcohol cessation when appropriate. Referral to gastroenterology is recommended for recurrent episodes, and patients with type A HE should be evaluated for liver transplantation.
Clinical Insights And Common Errors
Rifaximin is effective for lactulose-resistant hepatic encephalopathy. Hypoglycemia is common and easily overlooked. Sedatives and narcotics can worsen encephalopathy and should be avoided whenever possible or replaced with agents not metabolized by the liver.
Basics And Description
Hepatic encephalopathy (HE) is a neuropsychiatric syndrome characterized by alterations in behavior, level of consciousness, and motor function resulting from hepatic insufficiency and the accumulation of substances normally detoxified by the liver. The condition arises from a multifactorial process, most notably the accumulation of ammonia produced from protein degradation by colonic bacteria and deamination of glutamine in the small bowel, kidneys, and muscle. Ammonia crosses the blood–brain barrier and is metabolized by astrocytes into glutamine, leading to astrocytic swelling, cerebral edema, and mitochondrial dysfunction. Additional neurotoxins such as short-chain fatty acids, manganese, neurosteroids, phenols, mercaptans, and amino acids including tryptophan also contribute. Neurotransmitter imbalance plays a role, with increased inhibitory neurotransmitters such as benzodiazepines, γ-aminobutyric acid, and serotonin, and decreased excitatory neurotransmitters including glutamate, dopamine, aspartate, and catecholamines. Other contributing factors include reduced cerebral blood flow and oxygen delivery, increased cerebral glucose utilization with potential hypoglycemia, zinc deficiency affecting urea cycle enzymes, and rare inherited urea cycle disorders.
Etiology
Hepatic encephalopathy is classified according to the 11th World Congress of Gastroenterology. Type A is associated with acute liver injury and fulminant hepatic failure. Type B is related to portosystemic shunting without intrinsic liver disease. Type C is associated with cirrhosis and portal hypertension. Common precipitating factors include gastrointestinal bleeding, electrolyte abnormalities such as hypokalemia and hyponatremia, metabolic alkalosis reducing renal ammonium excretion, sepsis including spontaneous bacterial peritonitis, constipation, noncompliance with treatment in chronic liver disease, high-protein diet, hypoglycemia, hypovolemia following large-volume paracentesis, azotemia from diuretics or diarrhea, sedatives or narcotics including alcohol, zinc deficiency, hepatocellular injury from viral or drug-induced hepatitis, and post portosystemic shunt placement. Recurrent episodes may occur even without identifiable triggers.
Diagnosis – Signs And Symptoms
Type A HE progresses rapidly and may lead to seizures, decerebrate rigidity, coma, and death from cerebral edema. Type B and C HE are typically chronic and may present as minimal or overt encephalopathy. Minimal HE manifests as impaired psychomotor speed, attention, concentration, visual perception, memory deficits, and slowed mental processing, detectable only with psychometric testing. Overt HE includes slow and monotonous speech, impaired fine motor skills, hyperreflexia, clonus, hyperventilation, extrapyramidal movements, seizures, confusion, coma, and abnormal posturing. Episodic HE is characterized by transient changes in consciousness over hours to days with return to baseline after treatment, whereas persistent HE does not fully resolve.
Clinical Grading (West Haven Criteria)
Stage 0 shows no overt clinical changes but abnormal neuropsychological testing and no asterixis. Stage I includes personality changes, irritability, depression or euphoria, sleep–wake reversal, impaired writing or calculations, and possible asterixis. Stage II is marked by inappropriate behavior, lethargy, slowed responses, asterixis, slurred speech, and ataxia. Stage III presents with disorientation, amnesia, paranoia, nystagmus, hypoactive reflexes, positive Babinski sign, and stupor. Stage IV is characterized by coma and dilated pupils.
Essential Workup
Evaluation should include a detailed history of liver disease and prior HE episodes, identification of precipitating factors, and assessment for electrolyte disturbances, as even mild abnormalities may cause significant clinical deterioration.
Diagnostic Tests And Interpretation
Ammonia levels correlate poorly with disease severity but may assist in evaluating unexplained altered mental status. Samples must be handled correctly and processed promptly. A normal ammonia level should prompt investigation for alternative diagnoses. Additional studies include fecal occult blood testing and nasogastric lavage to assess for gastrointestinal bleeding, complete blood count for anemia or infection, electrolytes, renal function, glucose, coagulation studies, and liver enzymes. Urinalysis and cultures of urine and ascitic fluid help identify infection. Toxicology screening may be required to exclude other causes of altered consciousness. Additional testing may include thyroid function, arterial blood gases, magnesium, and viral serologies. Imaging includes chest radiography for pneumonia or heart failure and head CT for new-onset altered mental status, focal deficits, trauma, or suspected cerebral edema. Brain MRI may aid diagnosis in patients with portosystemic shunts without intrinsic liver disease. ECG assesses arrhythmias and electrolyte effects. Lumbar puncture is indicated for unexplained or worsening symptoms, and paracentesis is required when spontaneous bacterial peritonitis is suspected. EEG typically shows generalized slowing.
Differential Diagnosis
Consider alcohol withdrawal, cerebrovascular accident, congestive heart failure, carbon dioxide narcosis, head trauma, electrolyte disturbances, hypoglycemia, meningitis, encephalitis, metabolic encephalopathies, psychiatric illness, drug or alcohol intoxication, uremia, and in children, Reye syndrome or fatty acid oxidation disorders.
Initial Stabilization And Therapy
Management includes supplemental oxygen, cardiac monitoring, and airway protection, with intubation required for grade III or IV encephalopathy. Propofol is preferred for sedation if needed. Standard initial altered mental status treatment includes naloxone, glucose administration, and thiamine.
Emergency Department Treatment And Procedures
Identification and correction of precipitating factors is central to management. Frequent glucose monitoring is essential due to the risk of hypoglycemia. Complications such as gastrointestinal bleeding, sepsis, electrolyte abnormalities, coagulopathy, and renal dysfunction must be treated promptly. Sedatives and narcotics should be avoided when possible; if required, agents with minimal hepatic metabolism are preferred. Ammonia reduction is achieved with nonabsorbable disaccharides such as lactulose, administered orally, via nasogastric tube, or as retention enemas in severe cases. Intestinal flora reduction may include rifaximin, neomycin, metronidazole, or vancomycin, often in combination with lactulose. Rifaximin is preferred for lactulose-resistant HE due to its efficacy and favorable safety profile. Cerebral edema should be treated promptly if present. Zinc deficiency should be corrected, and short-term protein restriction may be considered. Flumazenil may provide transient improvement in select patients but should be avoided in chronic benzodiazepine or alcohol users. Liver transplantation is the definitive therapy for severe or recurrent HE. Additional therapies under investigation include L-carnitine, albumin dialysis, N-acetylcysteine, probiotics, branched-chain amino acids, and manganese chelation.
Medications
Therapy may include intravenous dextrose for hypoglycemia, lactulose titrated to achieve two to three soft stools daily, rifaximin 550 mg twice daily, neomycin or metronidazole when indicated, naloxone for suspected opioid effect, thiamine supplementation, zinc acetate or sulfate, and mannitol for cerebral edema.
Follow-Up And Disposition
Patients with grade II to IV HE, type A HE of any grade, type B or C HE grade II or higher, associated complications such as sepsis or gastrointestinal bleeding, or diagnostic uncertainty require admission, often to the intensive care unit with gastroenterology consultation. Discharge may be considered for patients with known chronic HE presenting with grade 0 or I symptoms, a reversible cause, and reliable supervision. Discharged patients should receive lactulose therapy and dietary guidance.
Follow-Up Recommendations
Patients should realize the importance of dietary counseling, medication adherence, and alcohol cessation when appropriate. Referral to gastroenterology is recommended for recurrent episodes, and patients with type A HE should be evaluated for liver transplantation.
Clinical Insights And Common Errors
Rifaximin is effective for lactulose-resistant hepatic encephalopathy. Hypoglycemia is common and easily overlooked. Sedatives and narcotics can worsen encephalopathy and should be avoided whenever possible or replaced with agents not metabolized by the liver.
- Published on
Emergency And Acute Medicine – Henoch–Schönlein Purpura
Basics And Description
Henoch–Schönlein purpura is a small-vessel vasculitis characterized by IgA-mediated inflammation affecting the skin, gastrointestinal tract, kidneys, joints, and, less commonly, the central nervous system. Multisystem involvement may lead to acute, life-threatening complications or long-term sequelae, particularly renal disease. Although often self-limited, careful evaluation and follow-up are essential.
Etiology
The underlying mechanism involves increased serum IgA with formation of circulating IgA immune complexes and mesangial IgA deposition in the glomeruli. The exact cause is unknown, but multiple associations have been identified. These include infections such as group A streptococcus, Mycoplasma, and viral illnesses including varicella and Epstein–Barr virus. Drug associations include penicillin, tetracycline, aspirin, sulfonamides, and erythromycin. Allergens such as insect bites and certain foods including chocolate, milk, and wheat have also been implicated. The condition most commonly affects school-aged children and young adults, occurs more frequently in males than females, is more common in white populations, and has a higher incidence in winter and spring.
Diagnosis – Signs And Symptoms
Patients are often well appearing despite extensive rash. Constitutional symptoms may include malaise, low-grade fever, and recent or current upper respiratory infection. Hypertension may be present when renal involvement develops. Infants younger than 3 months may present with skin findings alone, while children under 2 years may present with isolated facial edema.
Skin Manifestations
A purpuric rash is the hallmark and presenting feature in approximately half of patients, with eventual development in nearly all cases. Lesions begin as blanching pink papules and evolve within 24 hours into palpable purpura measuring 2–3 cm. The rash is typically symmetric, involves gravity-dependent areas such as the lower extremities and buttocks, and may extend to the trunk or upper extremities. Facial involvement is rare. Recurrence occurs in up to 40% of patients, usually within six weeks.
Abdominal Involvement
Abdominal pain occurs in 70–80% of cases and may range from colicky to severe. Gastrointestinal symptoms may precede the rash by up to four weeks. Gastrointestinal bleeding is common and ranges from occult blood loss to severe hemorrhage. Intussusception, usually ileoileal or ileocolic, is a serious complication.
Renal And Genitourinary Involvement
Asymptomatic hematuria is common and occurs in up to 80% of patients. Proteinuria suggests glomerulonephritis. Other manifestations include scrotal pain, testicular swelling, and, in severe cases, renal failure.
Musculoskeletal Involvement
Arthritis or arthralgia occurs in 70–80% of patients, is typically migratory, periarticular, and most commonly affects the knees and ankles. Angioedema may also occur.
Neurologic Involvement
Less common manifestations include headache, seizures, altered mental status, focal neurologic deficits, and visual or speech disturbances. Intracranial hemorrhage is rare but serious.
History
Key elements include recent infections, onset and progression of rash, presence of abdominal pain, vomiting, edema, joint symptoms, and timing and progression of multisystem complaints.
Physical Examination
Assessment should include vital signs with attention to blood pressure, cardiovascular perfusion, abdominal tenderness or distention, palpable masses, evidence of gastrointestinal bleeding, genitourinary examination for testicular involvement, careful skin inspection for nonblanching purpura, and a focused neurologic examination.
Essential Workup
The primary goal is to exclude life-threatening causes of purpura, abdominal pain, hematuria, and neurologic findings.
Diagnostic Tests And Interpretation
Laboratory studies typically show a normal platelet count and may reveal leukocytosis. Coagulation studies are indicated if bleeding is present or diagnosis is uncertain. Electrolytes, blood urea nitrogen, and creatinine should be obtained if hypertension or abnormal urinalysis is present. Urinalysis frequently shows hematuria, and proteinuria suggests renal involvement. Cultures may be obtained to exclude infectious triggers. Abdominal imaging with ultrasound, contrast enema, or CT is indicated for abdominal pain or gastrointestinal bleeding to evaluate for intussusception. Testicular ultrasound is indicated for scrotal symptoms. Head CT is required for neurologic findings. Lumbar puncture is performed only when clinically indicated.
Differential Diagnosis
Important considerations include gastroenteritis, appendicitis, inflammatory bowel disease, Meckel diverticulum, acute glomerulonephritis, systemic lupus erythematosus, polyarteritis nodosa, meningococcemia, bacterial sepsis, Rocky Mountain spotted fever, viral exanthems, thrombocytopenia, drug reactions, testicular torsion, and incarcerated hernia.
Treatment – Prehospital Care
Stabilization should be provided as clinically indicated.
Initial Stabilization And Therapy
Intravenous fluids are administered for shock. Packed red blood cells are indicated for massive gastrointestinal hemorrhage.
Emergency Department Treatment And Procedures
Life-threatening complications require immediate intervention. NSAIDs such as ibuprofen are used for arthralgia. Corticosteroids, typically prednisone, are indicated for severe abdominal pain after exclusion of surgical causes, painful subcutaneous edema, arthritis, significant renal involvement, or central nervous system disease. Severe nephritis may require high-dose pulse methylprednisolone. Immunosuppressive agents and intravenous immunoglobulin may be considered in severe, life-threatening disease, though evidence is limited.
Medications
First-line therapy includes ibuprofen 600 mg orally every 6 hours, or 5–10 mg/kg per dose in children. Second-line therapy includes prednisone 60 mg daily in adults or 1–2 mg/kg/day in children for 5–7 days.
Follow-Up And Disposition
Admission is required for severe abdominal pain, gastrointestinal bleeding, intussusception, central nervous system involvement, or evidence of renal failure. Patients may be discharged if platelet count and renal function are normal and abdominal pain is minimal or absent. Patients started on steroids should have follow-up within 24 hours.
Follow-Up Recommendations
Close follow-up with a primary care physician is required, with regular monitoring of blood pressure and repeat urinalysis and laboratory studies as indicated. In children, monitoring is recommended for at least six months.
Clinical Insights And Common Errors
Always exclude life-threatening causes of purpura and abdominal pain. NSAIDs are sufficient for most patients. Systemic corticosteroids do not alter the long-term prognosis of Henoch–Schönlein purpura nephritis and should be reserved for specific indications.
Basics And Description
Henoch–Schönlein purpura is a small-vessel vasculitis characterized by IgA-mediated inflammation affecting the skin, gastrointestinal tract, kidneys, joints, and, less commonly, the central nervous system. Multisystem involvement may lead to acute, life-threatening complications or long-term sequelae, particularly renal disease. Although often self-limited, careful evaluation and follow-up are essential.
Etiology
The underlying mechanism involves increased serum IgA with formation of circulating IgA immune complexes and mesangial IgA deposition in the glomeruli. The exact cause is unknown, but multiple associations have been identified. These include infections such as group A streptococcus, Mycoplasma, and viral illnesses including varicella and Epstein–Barr virus. Drug associations include penicillin, tetracycline, aspirin, sulfonamides, and erythromycin. Allergens such as insect bites and certain foods including chocolate, milk, and wheat have also been implicated. The condition most commonly affects school-aged children and young adults, occurs more frequently in males than females, is more common in white populations, and has a higher incidence in winter and spring.
Diagnosis – Signs And Symptoms
Patients are often well appearing despite extensive rash. Constitutional symptoms may include malaise, low-grade fever, and recent or current upper respiratory infection. Hypertension may be present when renal involvement develops. Infants younger than 3 months may present with skin findings alone, while children under 2 years may present with isolated facial edema.
Skin Manifestations
A purpuric rash is the hallmark and presenting feature in approximately half of patients, with eventual development in nearly all cases. Lesions begin as blanching pink papules and evolve within 24 hours into palpable purpura measuring 2–3 cm. The rash is typically symmetric, involves gravity-dependent areas such as the lower extremities and buttocks, and may extend to the trunk or upper extremities. Facial involvement is rare. Recurrence occurs in up to 40% of patients, usually within six weeks.
Abdominal Involvement
Abdominal pain occurs in 70–80% of cases and may range from colicky to severe. Gastrointestinal symptoms may precede the rash by up to four weeks. Gastrointestinal bleeding is common and ranges from occult blood loss to severe hemorrhage. Intussusception, usually ileoileal or ileocolic, is a serious complication.
Renal And Genitourinary Involvement
Asymptomatic hematuria is common and occurs in up to 80% of patients. Proteinuria suggests glomerulonephritis. Other manifestations include scrotal pain, testicular swelling, and, in severe cases, renal failure.
Musculoskeletal Involvement
Arthritis or arthralgia occurs in 70–80% of patients, is typically migratory, periarticular, and most commonly affects the knees and ankles. Angioedema may also occur.
Neurologic Involvement
Less common manifestations include headache, seizures, altered mental status, focal neurologic deficits, and visual or speech disturbances. Intracranial hemorrhage is rare but serious.
History
Key elements include recent infections, onset and progression of rash, presence of abdominal pain, vomiting, edema, joint symptoms, and timing and progression of multisystem complaints.
Physical Examination
Assessment should include vital signs with attention to blood pressure, cardiovascular perfusion, abdominal tenderness or distention, palpable masses, evidence of gastrointestinal bleeding, genitourinary examination for testicular involvement, careful skin inspection for nonblanching purpura, and a focused neurologic examination.
Essential Workup
The primary goal is to exclude life-threatening causes of purpura, abdominal pain, hematuria, and neurologic findings.
Diagnostic Tests And Interpretation
Laboratory studies typically show a normal platelet count and may reveal leukocytosis. Coagulation studies are indicated if bleeding is present or diagnosis is uncertain. Electrolytes, blood urea nitrogen, and creatinine should be obtained if hypertension or abnormal urinalysis is present. Urinalysis frequently shows hematuria, and proteinuria suggests renal involvement. Cultures may be obtained to exclude infectious triggers. Abdominal imaging with ultrasound, contrast enema, or CT is indicated for abdominal pain or gastrointestinal bleeding to evaluate for intussusception. Testicular ultrasound is indicated for scrotal symptoms. Head CT is required for neurologic findings. Lumbar puncture is performed only when clinically indicated.
Differential Diagnosis
Important considerations include gastroenteritis, appendicitis, inflammatory bowel disease, Meckel diverticulum, acute glomerulonephritis, systemic lupus erythematosus, polyarteritis nodosa, meningococcemia, bacterial sepsis, Rocky Mountain spotted fever, viral exanthems, thrombocytopenia, drug reactions, testicular torsion, and incarcerated hernia.
Treatment – Prehospital Care
Stabilization should be provided as clinically indicated.
Initial Stabilization And Therapy
Intravenous fluids are administered for shock. Packed red blood cells are indicated for massive gastrointestinal hemorrhage.
Emergency Department Treatment And Procedures
Life-threatening complications require immediate intervention. NSAIDs such as ibuprofen are used for arthralgia. Corticosteroids, typically prednisone, are indicated for severe abdominal pain after exclusion of surgical causes, painful subcutaneous edema, arthritis, significant renal involvement, or central nervous system disease. Severe nephritis may require high-dose pulse methylprednisolone. Immunosuppressive agents and intravenous immunoglobulin may be considered in severe, life-threatening disease, though evidence is limited.
Medications
First-line therapy includes ibuprofen 600 mg orally every 6 hours, or 5–10 mg/kg per dose in children. Second-line therapy includes prednisone 60 mg daily in adults or 1–2 mg/kg/day in children for 5–7 days.
Follow-Up And Disposition
Admission is required for severe abdominal pain, gastrointestinal bleeding, intussusception, central nervous system involvement, or evidence of renal failure. Patients may be discharged if platelet count and renal function are normal and abdominal pain is minimal or absent. Patients started on steroids should have follow-up within 24 hours.
Follow-Up Recommendations
Close follow-up with a primary care physician is required, with regular monitoring of blood pressure and repeat urinalysis and laboratory studies as indicated. In children, monitoring is recommended for at least six months.
Clinical Insights And Common Errors
Always exclude life-threatening causes of purpura and abdominal pain. NSAIDs are sufficient for most patients. Systemic corticosteroids do not alter the long-term prognosis of Henoch–Schönlein purpura nephritis and should be reserved for specific indications.
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Emergency And Acute Medicine – Hepatorenal Syndrome
Overview And Definition
Hepatorenal syndrome is a form of renal failure that occurs in patients with advanced acute or chronic liver disease in the absence of intrinsic renal pathology. It reflects a severe reduction in renal perfusion caused by advanced hepatic dysfunction. The kidneys are structurally normal, and renal failure does not improve despite adequate volume resuscitation.
Disease Classification And Mechanism
Type I hepatorenal syndrome is an acute and rapidly progressive condition characterized by a sudden decline in renal function, defined by a doubling of serum creatinine or a 50% reduction in creatinine clearance within two weeks. It is commonly associated with acute liver failure or severe alcoholic hepatitis, often presents with oliguria or anuria, and carries a mortality rate approaching 90% within three months.
Type II hepatorenal syndrome has a more indolent course and typically occurs in patients with cirrhosis and refractory ascites. Renal dysfunction progresses slowly, and overall survival is longer than in type I disease.
The underlying pathophysiology involves profound systemic vasodilation due to liver disease, resulting in reduced effective arterial blood volume. This triggers activation of the sympathetic nervous system and the renin–angiotensin–aldosterone system, along with increased production of vasoactive substances. The net effect is dominant renal vasoconstriction, reduced renal blood flow, decreased glomerular filtration rate, and markedly low urinary sodium excretion.
Etiology And Precipitating Factors
Hepatorenal syndrome most commonly arises in chronic liver disease, particularly cirrhosis and severe alcoholic hepatitis, but may also occur in fulminant hepatic failure. Common precipitating factors include gastrointestinal bleeding, aggressive diuresis, large-volume paracentesis without albumin replacement, exposure to nephrotoxic medications such as NSAIDs or aminoglycosides, and systemic infections. Spontaneous bacterial peritonitis is a particularly important trigger and significantly increases the risk of renal failure.
Clinical Features
Patients typically exhibit signs of advanced liver disease, including ascites, portal hypertension, jaundice, hepatic encephalopathy, and coagulopathy. Progressive oliguria is common. Vital signs may reveal hypotension or tachycardia, particularly in the presence of bleeding or sepsis. Dyspnea and tachypnea may occur due to tense ascites or volume overload.
Diagnostic Evaluation
Laboratory findings usually demonstrate rising blood urea nitrogen and creatinine, though creatinine may underestimate renal dysfunction due to low muscle mass or interference from hyperbilirubinemia. Urinalysis typically shows no casts or protein, helping distinguish hepatorenal syndrome from acute tubular necrosis. Urine sodium is characteristically very low, with a fractional excretion of sodium below 1% and concentrated urine relative to plasma.
Additional studies include coagulation profiles, cultures to evaluate for infection, and renal ultrasound to exclude obstructive uropathy. Hepatorenal syndrome remains a diagnosis of exclusion, requiring elimination of other causes of renal failure such as hypovolemia, intrinsic renal disease, obstruction, and drug toxicity.
Differential Considerations
Important alternatives include prerenal azotemia due to volume depletion, acute tubular necrosis, glomerulonephritis related to viral hepatitis, obstructive uropathy, medication-induced nephrotoxicity, sepsis-related renal failure, and post–liver transplant renal dysfunction.
Initial Stabilization And Support
Management begins with standard airway, breathing, and circulation assessment. Hypotension and hypovolemia should be corrected promptly using isotonic fluids and albumin. Close monitoring of urine output and hemodynamic status is essential. Life-threatening complications of renal failure, including hyperkalemia, metabolic acidosis, hypoxemia, and uremic complications, must be addressed immediately.
Emergency Department Management
Treatment focuses on identifying and correcting reversible contributors while providing supportive care. All potentially nephrotoxic medications should be discontinued. Infections must be treated aggressively with early antibiotics. Electrolyte abnormalities, hypoglycemia, hepatic encephalopathy, and other complications of liver failure should be corrected. Large-volume paracentesis with albumin replacement may transiently improve renal perfusion in patients with tense ascites.
Advanced therapies such as transjugular intrahepatic portosystemic shunt placement may improve renal function in selected patients. Dialysis is reserved for management of complications and as a bridge to recovery or transplantation. Liver transplantation remains the only definitive treatment.
Pharmacologic Options
No medication is considered definitive therapy. Vasoconstrictor-based regimens may be used in select cases after excluding other causes of renal failure. Options include low-dose dopamine, combinations of midodrine and octreotide, or prostaglandin analogs. Vasopressin analogs such as terlipressin show benefit but are not widely available in all regions.
Disposition And Prognosis
All patients with suspected hepatorenal syndrome require hospital admission with gastroenterology and nephrology consultation. Intensive care admission is indicated for those with encephalopathy, severe electrolyte disturbances, or cardiopulmonary compromise. There are no circumstances in which discharge from the emergency department is appropriate.
Key Clinical Insights And Common Errors
Any degree of renal dysfunction in a patient with advanced liver disease must be treated as a medical emergency. Delayed recognition, failure to exclude reversible causes, or continued exposure to nephrotoxic agents significantly worsens outcomes. Early consultation and consideration of transplant eligibility are critical.
Overview And Definition
Hepatorenal syndrome is a form of renal failure that occurs in patients with advanced acute or chronic liver disease in the absence of intrinsic renal pathology. It reflects a severe reduction in renal perfusion caused by advanced hepatic dysfunction. The kidneys are structurally normal, and renal failure does not improve despite adequate volume resuscitation.
Disease Classification And Mechanism
Type I hepatorenal syndrome is an acute and rapidly progressive condition characterized by a sudden decline in renal function, defined by a doubling of serum creatinine or a 50% reduction in creatinine clearance within two weeks. It is commonly associated with acute liver failure or severe alcoholic hepatitis, often presents with oliguria or anuria, and carries a mortality rate approaching 90% within three months.
Type II hepatorenal syndrome has a more indolent course and typically occurs in patients with cirrhosis and refractory ascites. Renal dysfunction progresses slowly, and overall survival is longer than in type I disease.
The underlying pathophysiology involves profound systemic vasodilation due to liver disease, resulting in reduced effective arterial blood volume. This triggers activation of the sympathetic nervous system and the renin–angiotensin–aldosterone system, along with increased production of vasoactive substances. The net effect is dominant renal vasoconstriction, reduced renal blood flow, decreased glomerular filtration rate, and markedly low urinary sodium excretion.
Etiology And Precipitating Factors
Hepatorenal syndrome most commonly arises in chronic liver disease, particularly cirrhosis and severe alcoholic hepatitis, but may also occur in fulminant hepatic failure. Common precipitating factors include gastrointestinal bleeding, aggressive diuresis, large-volume paracentesis without albumin replacement, exposure to nephrotoxic medications such as NSAIDs or aminoglycosides, and systemic infections. Spontaneous bacterial peritonitis is a particularly important trigger and significantly increases the risk of renal failure.
Clinical Features
Patients typically exhibit signs of advanced liver disease, including ascites, portal hypertension, jaundice, hepatic encephalopathy, and coagulopathy. Progressive oliguria is common. Vital signs may reveal hypotension or tachycardia, particularly in the presence of bleeding or sepsis. Dyspnea and tachypnea may occur due to tense ascites or volume overload.
Diagnostic Evaluation
Laboratory findings usually demonstrate rising blood urea nitrogen and creatinine, though creatinine may underestimate renal dysfunction due to low muscle mass or interference from hyperbilirubinemia. Urinalysis typically shows no casts or protein, helping distinguish hepatorenal syndrome from acute tubular necrosis. Urine sodium is characteristically very low, with a fractional excretion of sodium below 1% and concentrated urine relative to plasma.
Additional studies include coagulation profiles, cultures to evaluate for infection, and renal ultrasound to exclude obstructive uropathy. Hepatorenal syndrome remains a diagnosis of exclusion, requiring elimination of other causes of renal failure such as hypovolemia, intrinsic renal disease, obstruction, and drug toxicity.
Differential Considerations
Important alternatives include prerenal azotemia due to volume depletion, acute tubular necrosis, glomerulonephritis related to viral hepatitis, obstructive uropathy, medication-induced nephrotoxicity, sepsis-related renal failure, and post–liver transplant renal dysfunction.
Initial Stabilization And Support
Management begins with standard airway, breathing, and circulation assessment. Hypotension and hypovolemia should be corrected promptly using isotonic fluids and albumin. Close monitoring of urine output and hemodynamic status is essential. Life-threatening complications of renal failure, including hyperkalemia, metabolic acidosis, hypoxemia, and uremic complications, must be addressed immediately.
Emergency Department Management
Treatment focuses on identifying and correcting reversible contributors while providing supportive care. All potentially nephrotoxic medications should be discontinued. Infections must be treated aggressively with early antibiotics. Electrolyte abnormalities, hypoglycemia, hepatic encephalopathy, and other complications of liver failure should be corrected. Large-volume paracentesis with albumin replacement may transiently improve renal perfusion in patients with tense ascites.
Advanced therapies such as transjugular intrahepatic portosystemic shunt placement may improve renal function in selected patients. Dialysis is reserved for management of complications and as a bridge to recovery or transplantation. Liver transplantation remains the only definitive treatment.
Pharmacologic Options
No medication is considered definitive therapy. Vasoconstrictor-based regimens may be used in select cases after excluding other causes of renal failure. Options include low-dose dopamine, combinations of midodrine and octreotide, or prostaglandin analogs. Vasopressin analogs such as terlipressin show benefit but are not widely available in all regions.
Disposition And Prognosis
All patients with suspected hepatorenal syndrome require hospital admission with gastroenterology and nephrology consultation. Intensive care admission is indicated for those with encephalopathy, severe electrolyte disturbances, or cardiopulmonary compromise. There are no circumstances in which discharge from the emergency department is appropriate.
Key Clinical Insights And Common Errors
Any degree of renal dysfunction in a patient with advanced liver disease must be treated as a medical emergency. Delayed recognition, failure to exclude reversible causes, or continued exposure to nephrotoxic agents significantly worsens outcomes. Early consultation and consideration of transplant eligibility are critical.
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Emergency And Acute Medicine – Hepatic Injury
Basics And Description
The liver’s size and anatomic location make it particularly vulnerable to injury. It is the most frequently injured solid organ in penetrating trauma and the second most commonly injured organ in blunt abdominal trauma, after the spleen. The liver is highly susceptible to blunt forces from direct impact or deceleration. Understanding the mechanism of injury is essential in evaluating for hepatic trauma. In blunt injury, the direction and magnitude of deceleration or compressive forces should be assessed. In penetrating trauma, the type, caliber, and distance of the weapon, as well as the length and characteristics of knives or impaling objects, are important. Hepatic injuries range from subcapsular hematomas and minor lacerations to severe fragmentation. Associated injuries commonly involve the ribs, spleen, diaphragm, kidneys, lungs, gallbladder, pancreas, and major blood vessels. Overall mortality is approximately 8–10%. Nonoperative management is increasingly common in isolated blunt hepatic trauma. In children, poorly developed abdominal musculature and a smaller anteroposterior diameter increase susceptibility to compressive forces.
Etiology
Hepatic injury most commonly results from trauma. Blunt mechanisms include deceleration, acceleration, and compression forces. Penetrating mechanisms include stab wounds, gunshot wounds, and impalement by foreign objects.
Diagnosis – Signs And Symptoms
Clinical presentation is variable, and neither history nor physical examination alone is sensitive or specific for hepatic injury. A history of trauma is usually available from the patient or prehospital providers. Systemic signs may reflect acute blood loss, including dizziness, weakness, tachycardia, and hypotension. Local findings may include right upper quadrant tenderness, guarding, abdominal distention, rigidity, rebound tenderness, contusions or abrasions, and penetrating wounds to the right chest, flank, or abdomen.
Essential Workup
Because physical examination is unreliable, objective evaluation is required. This includes imaging studies of the abdomen or surgical exploration when indicated.
Diagnostic Tests And Interpretation
No laboratory study is specific for liver injury. A baseline hemoglobin level should be obtained, and type and crossmatch considered if hemorrhage is suspected. Liver function tests are not useful in the acute setting. Plain abdominal radiographs have little diagnostic value. Bedside ultrasonography is an important screening tool in both blunt and penetrating trauma and is the procedure of choice in unstable patients. It may identify free intraperitoneal fluid in the hepatorenal (Morison) pouch or parenchymal injury, suggesting intra-abdominal hemorrhage. Contrast-enhanced CT scan is the best modality for defining the extent of hepatic injury and associated organ damage but requires hemodynamic stability. Active contrast extravasation during the arterial phase suggests vascular injury or high-grade liver trauma requiring intervention. Diagnostic peritoneal lavage is rarely used but is sensitive for hemoperitoneum, though nonspecific for bleeding source. Operative exploration is required if results are positive.
Differential Diagnosis
Consider other causes of intraperitoneal injury, retroperitoneal injury, thoracic or diaphragmatic injury, splenic injury, and vascular trauma.
Prehospital Management
Details of the injury mechanism should be obtained early. Large-bore intravenous access is essential due to the risk of rapid hemorrhage. Penetrating wounds or evisceration should be covered with moist saline dressings, and direct pressure applied to control bleeding. Full spinal immobilization is recommended except in isolated penetrating trauma.
Initial Stabilization And Therapy
Airway, breathing, and circulation take priority. Airway control may be required, particularly if associated head injury is present. Supplemental oxygen, cardiac monitoring, and pulse oximetry should be initiated. Adequate intravenous access is critical and may include peripheral large-bore lines, central venous access, intraosseous access, or venous cutdown. Fluid resuscitation typically begins with crystalloid solutions, followed by packed red blood cells and fresh frozen plasma as needed.
Emergency Department Treatment And Procedures
Immediate laparotomy is indicated in patients with hemodynamic instability, gunshot wounds to the anterior abdomen, clear signs of intraperitoneal hemorrhage, positive diagnostic studies such as DPL, or failure of nonoperative management. Stab wounds may be managed with local wound exploration and adjunct imaging when intraperitoneal penetration is uncertain. Nonoperative management may be appropriate in hemodynamically stable patients with normal mental status, no evidence of other intra-abdominal injury, isolated low-grade hepatic injuries confirmed by imaging, and minimal transfusion requirements. High-grade injuries have lower success rates with nonoperative strategies. Patients should remain NPO with strict bedrest. Selective angiography with embolization may control persistent bleeding and reduce the need for surgery. Adjunctive therapies such as recombinant factor VIIa or prothrombin complex concentrate have been used to aid hemostasis in select cases.
Medications
Management includes intravenous crystalloid fluids, packed red blood cells, fresh frozen plasma, and in select cases recombinant factor VIIa or prothrombin complex concentrate.
Follow-Up And Disposition
All patients with hepatic injury require hospital admission for operative management or close observation with serial examinations, hematocrit measurements, and repeat imaging as indicated. ICU admission is often required during the first 48 hours. Patients with suspected or confirmed hepatic injury should not be discharged from the emergency department.
Follow-Up Recommendations
Serial imaging, physical examinations, and hematocrit monitoring are essential to detect delayed deterioration. Penetrating injuries should be reported to appropriate authorities.
Clinical Insights And Common Errors
Early surgical consultation is critical in unstable patients. Failure to obtain appropriate imaging or relying on a negative ultrasound alone are common pitfalls. Inadequate resuscitation with fluids and blood products can worsen outcomes. Once hepatic injury is identified, associated injuries to surrounding organs must be actively excluded, and pregnancy testing should be performed in women of childbearing age.
Basics And Description
The liver’s size and anatomic location make it particularly vulnerable to injury. It is the most frequently injured solid organ in penetrating trauma and the second most commonly injured organ in blunt abdominal trauma, after the spleen. The liver is highly susceptible to blunt forces from direct impact or deceleration. Understanding the mechanism of injury is essential in evaluating for hepatic trauma. In blunt injury, the direction and magnitude of deceleration or compressive forces should be assessed. In penetrating trauma, the type, caliber, and distance of the weapon, as well as the length and characteristics of knives or impaling objects, are important. Hepatic injuries range from subcapsular hematomas and minor lacerations to severe fragmentation. Associated injuries commonly involve the ribs, spleen, diaphragm, kidneys, lungs, gallbladder, pancreas, and major blood vessels. Overall mortality is approximately 8–10%. Nonoperative management is increasingly common in isolated blunt hepatic trauma. In children, poorly developed abdominal musculature and a smaller anteroposterior diameter increase susceptibility to compressive forces.
Etiology
Hepatic injury most commonly results from trauma. Blunt mechanisms include deceleration, acceleration, and compression forces. Penetrating mechanisms include stab wounds, gunshot wounds, and impalement by foreign objects.
Diagnosis – Signs And Symptoms
Clinical presentation is variable, and neither history nor physical examination alone is sensitive or specific for hepatic injury. A history of trauma is usually available from the patient or prehospital providers. Systemic signs may reflect acute blood loss, including dizziness, weakness, tachycardia, and hypotension. Local findings may include right upper quadrant tenderness, guarding, abdominal distention, rigidity, rebound tenderness, contusions or abrasions, and penetrating wounds to the right chest, flank, or abdomen.
Essential Workup
Because physical examination is unreliable, objective evaluation is required. This includes imaging studies of the abdomen or surgical exploration when indicated.
Diagnostic Tests And Interpretation
No laboratory study is specific for liver injury. A baseline hemoglobin level should be obtained, and type and crossmatch considered if hemorrhage is suspected. Liver function tests are not useful in the acute setting. Plain abdominal radiographs have little diagnostic value. Bedside ultrasonography is an important screening tool in both blunt and penetrating trauma and is the procedure of choice in unstable patients. It may identify free intraperitoneal fluid in the hepatorenal (Morison) pouch or parenchymal injury, suggesting intra-abdominal hemorrhage. Contrast-enhanced CT scan is the best modality for defining the extent of hepatic injury and associated organ damage but requires hemodynamic stability. Active contrast extravasation during the arterial phase suggests vascular injury or high-grade liver trauma requiring intervention. Diagnostic peritoneal lavage is rarely used but is sensitive for hemoperitoneum, though nonspecific for bleeding source. Operative exploration is required if results are positive.
Differential Diagnosis
Consider other causes of intraperitoneal injury, retroperitoneal injury, thoracic or diaphragmatic injury, splenic injury, and vascular trauma.
Prehospital Management
Details of the injury mechanism should be obtained early. Large-bore intravenous access is essential due to the risk of rapid hemorrhage. Penetrating wounds or evisceration should be covered with moist saline dressings, and direct pressure applied to control bleeding. Full spinal immobilization is recommended except in isolated penetrating trauma.
Initial Stabilization And Therapy
Airway, breathing, and circulation take priority. Airway control may be required, particularly if associated head injury is present. Supplemental oxygen, cardiac monitoring, and pulse oximetry should be initiated. Adequate intravenous access is critical and may include peripheral large-bore lines, central venous access, intraosseous access, or venous cutdown. Fluid resuscitation typically begins with crystalloid solutions, followed by packed red blood cells and fresh frozen plasma as needed.
Emergency Department Treatment And Procedures
Immediate laparotomy is indicated in patients with hemodynamic instability, gunshot wounds to the anterior abdomen, clear signs of intraperitoneal hemorrhage, positive diagnostic studies such as DPL, or failure of nonoperative management. Stab wounds may be managed with local wound exploration and adjunct imaging when intraperitoneal penetration is uncertain. Nonoperative management may be appropriate in hemodynamically stable patients with normal mental status, no evidence of other intra-abdominal injury, isolated low-grade hepatic injuries confirmed by imaging, and minimal transfusion requirements. High-grade injuries have lower success rates with nonoperative strategies. Patients should remain NPO with strict bedrest. Selective angiography with embolization may control persistent bleeding and reduce the need for surgery. Adjunctive therapies such as recombinant factor VIIa or prothrombin complex concentrate have been used to aid hemostasis in select cases.
Medications
Management includes intravenous crystalloid fluids, packed red blood cells, fresh frozen plasma, and in select cases recombinant factor VIIa or prothrombin complex concentrate.
Follow-Up And Disposition
All patients with hepatic injury require hospital admission for operative management or close observation with serial examinations, hematocrit measurements, and repeat imaging as indicated. ICU admission is often required during the first 48 hours. Patients with suspected or confirmed hepatic injury should not be discharged from the emergency department.
Follow-Up Recommendations
Serial imaging, physical examinations, and hematocrit monitoring are essential to detect delayed deterioration. Penetrating injuries should be reported to appropriate authorities.
Clinical Insights And Common Errors
Early surgical consultation is critical in unstable patients. Failure to obtain appropriate imaging or relying on a negative ultrasound alone are common pitfalls. Inadequate resuscitation with fluids and blood products can worsen outcomes. Once hepatic injury is identified, associated injuries to surrounding organs must be actively excluded, and pregnancy testing should be performed in women of childbearing age.
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Emergency And Acute Medicine – Hepatitis
Basics And Description
Hepatitis refers to inflammation of the liver caused by infectious, toxic, metabolic, or autoimmune processes. Disease progression may range from transient hepatocellular injury to fibrosis, cirrhosis, and liver failure. Infectious causes, particularly viral hepatitis, account for the majority of cases encountered in emergency and acute care settings.
Etiology
No specific cause is identified in approximately 5–10% of acute and chronic hepatitis cases and up to 50% of fulminant hepatic failure.
Hepatitis A is transmitted via the fecal–oral route with an incubation period of 2–7 weeks. It does not cause chronic disease, though up to 10% of patients experience a relapsing course. Fulminant hepatic failure occurs in about 0.1%.
Hepatitis B is transmitted through percutaneous or mucosal exposure to body fluids and perinatally, with an incubation period of 8–22 weeks. Most infections are subclinical. Risk of chronic infection depends on age and immune status and carries long-term risks of cirrhosis and hepatocellular carcinoma.
Hepatitis C is primarily bloodborne, with sexual and perinatal transmission occurring less commonly. Incubation is 6–10 weeks. Fulminant hepatic failure is rare, but approximately 80% progress to chronic disease with risk of cirrhosis and hepatocellular carcinoma.
Hepatitis D occurs only in the presence of hepatitis B, either as coinfection or superinfection, and is associated with a higher risk of cirrhosis.
Hepatitis E is the most common cause of acute hepatitis worldwide, transmitted fecal–orally, often via contaminated water. Fulminant hepatic failure occurs in up to 10% and is particularly severe in pregnancy.
Alcoholic hepatitis results from chronic heavy alcohol use and may progress to cirrhosis. Severity can be estimated using the Maddrey discriminant function.
Other causes include abscess-related hepatitis, secondary viral infections such as CMV or EBV, medication- or toxin-induced injury, and autoimmune hepatitis.
In children, hepatitis A predominates, while perinatal hepatitis B commonly progresses to chronic disease. In pregnancy, hepatitis E carries high mortality, and pregnancy-specific liver diseases such as acute fatty liver of pregnancy and HELLP syndrome must be considered.
Diagnosis – Signs And Symptoms
Many patients are asymptomatic. The preicteric phase may mimic viral gastroenteritis with fever, malaise, nausea, vomiting, anorexia, and arthralgia. The icteric phase includes jaundice, dark urine, light stools, pruritus, rash, and right upper quadrant pain. Fulminant hepatic failure presents with coagulopathy, encephalopathy, cerebral edema, bleeding, and altered mental status.
Physical Examination
Findings vary by disease phase. Early disease may show fever, dehydration, or arthritis. Icteric findings include jaundice of skin and sclerae, tender hepatomegaly, rash, and dehydration. Severe disease may demonstrate bruising, asterixis, and disorientation.
Essential Workup
A detailed history focusing on travel, exposures, alcohol use, medications, and risk factors is critical. Viral serologies are central to diagnosis of infectious causes.
Diagnostic Tests And Interpretation
Laboratory evaluation includes CBC, metabolic panel, liver function tests, coagulation studies, albumin, and glucose. Transaminase elevation reflects hepatocellular injury but does not correlate reliably with severity. Markedly elevated alkaline phosphatase suggests cholestatic disease. Prolonged INR and low albumin indicate impaired synthetic function. Ammonia levels are useful in altered mental status.
Viral serologies define etiology, including HAV IgM, HBV surface and core antibodies, HCV antibody and RNA, HDV testing when indicated, and HEV IgM. Additional tests include α-fetoprotein in chronic viral hepatitis, Monospot testing for EBV, and urinalysis for bilirubin.
Imaging includes right upper quadrant ultrasound to assess for biliary obstruction and head CT when hepatic encephalopathy is suspected.
Differential Diagnosis
Consider nonalcoholic fatty liver disease, biliary disease, ischemic hepatitis, congestive heart failure, metabolic and genetic disorders, liver abscess, heat stroke, Wilson disease, Budd–Chiari syndrome, and pregnancy-related liver disease.
Initial Stabilization And Therapy
Airway, breathing, and circulation are priorities in fulminant hepatic failure or severe encephalopathy. Judicious intravenous fluid resuscitation is required.
Emergency Department Treatment And Procedures
Management is largely supportive. Correct hypovolemia and electrolyte disturbances, treat nausea and vomiting, and avoid hepatotoxic medications and alcohol. Use medications with minimal hepatic metabolism when sedation or analgesia is required. Correct coagulopathy only in the presence of active bleeding. Administer N-acetylcysteine for acetaminophen toxicity and consider its use in fulminant hepatic failure. Steroids may benefit severe alcoholic hepatitis. Manage pruritus with bile acid sequestrants. Perform paracentesis for tense ascites causing respiratory compromise. Provide postexposure prophylaxis for hepatitis A and B when indicated.
Medications
Commonly used agents include ondansetron or metoclopramide for nausea, cholestyramine or ursodeoxycholic acid for pruritus, N-acetylcysteine, prednisone for select alcoholic hepatitis cases, vitamin K for coagulopathy, and thiamine in malnourished patients.
Follow-Up And Disposition
Admit patients with intractable vomiting, dehydration, electrolyte abnormalities, pregnancy, immunocompromise, suspected toxic hepatitis, advanced age, or evidence of significant liver dysfunction. Fulminant hepatic failure requires ICU care and early transfer to a transplant center. Patients with mild disease and good oral tolerance may be discharged with close follow-up.
Follow-Up Recommendations
Arrange hepatology or gastroenterology follow-up for definitive diagnosis and management. Counsel on strict hygiene, avoidance of alcohol and acetaminophen, vaccination when appropriate, and limitation of strenuous activity.
Clinical Insights And Common Errors
Acute hepatitis is frequently misdiagnosed as a nonspecific viral illness. Emergency management is primarily supportive. Early recognition of fulminant hepatic failure and prompt transfer to a transplant-capable center are critical. Maintain high suspicion for pregnancy-related liver disease in pregnant patients with compatible symptoms.
Basics And Description
Hepatitis refers to inflammation of the liver caused by infectious, toxic, metabolic, or autoimmune processes. Disease progression may range from transient hepatocellular injury to fibrosis, cirrhosis, and liver failure. Infectious causes, particularly viral hepatitis, account for the majority of cases encountered in emergency and acute care settings.
Etiology
No specific cause is identified in approximately 5–10% of acute and chronic hepatitis cases and up to 50% of fulminant hepatic failure.
Hepatitis A is transmitted via the fecal–oral route with an incubation period of 2–7 weeks. It does not cause chronic disease, though up to 10% of patients experience a relapsing course. Fulminant hepatic failure occurs in about 0.1%.
Hepatitis B is transmitted through percutaneous or mucosal exposure to body fluids and perinatally, with an incubation period of 8–22 weeks. Most infections are subclinical. Risk of chronic infection depends on age and immune status and carries long-term risks of cirrhosis and hepatocellular carcinoma.
Hepatitis C is primarily bloodborne, with sexual and perinatal transmission occurring less commonly. Incubation is 6–10 weeks. Fulminant hepatic failure is rare, but approximately 80% progress to chronic disease with risk of cirrhosis and hepatocellular carcinoma.
Hepatitis D occurs only in the presence of hepatitis B, either as coinfection or superinfection, and is associated with a higher risk of cirrhosis.
Hepatitis E is the most common cause of acute hepatitis worldwide, transmitted fecal–orally, often via contaminated water. Fulminant hepatic failure occurs in up to 10% and is particularly severe in pregnancy.
Alcoholic hepatitis results from chronic heavy alcohol use and may progress to cirrhosis. Severity can be estimated using the Maddrey discriminant function.
Other causes include abscess-related hepatitis, secondary viral infections such as CMV or EBV, medication- or toxin-induced injury, and autoimmune hepatitis.
In children, hepatitis A predominates, while perinatal hepatitis B commonly progresses to chronic disease. In pregnancy, hepatitis E carries high mortality, and pregnancy-specific liver diseases such as acute fatty liver of pregnancy and HELLP syndrome must be considered.
Diagnosis – Signs And Symptoms
Many patients are asymptomatic. The preicteric phase may mimic viral gastroenteritis with fever, malaise, nausea, vomiting, anorexia, and arthralgia. The icteric phase includes jaundice, dark urine, light stools, pruritus, rash, and right upper quadrant pain. Fulminant hepatic failure presents with coagulopathy, encephalopathy, cerebral edema, bleeding, and altered mental status.
Physical Examination
Findings vary by disease phase. Early disease may show fever, dehydration, or arthritis. Icteric findings include jaundice of skin and sclerae, tender hepatomegaly, rash, and dehydration. Severe disease may demonstrate bruising, asterixis, and disorientation.
Essential Workup
A detailed history focusing on travel, exposures, alcohol use, medications, and risk factors is critical. Viral serologies are central to diagnosis of infectious causes.
Diagnostic Tests And Interpretation
Laboratory evaluation includes CBC, metabolic panel, liver function tests, coagulation studies, albumin, and glucose. Transaminase elevation reflects hepatocellular injury but does not correlate reliably with severity. Markedly elevated alkaline phosphatase suggests cholestatic disease. Prolonged INR and low albumin indicate impaired synthetic function. Ammonia levels are useful in altered mental status.
Viral serologies define etiology, including HAV IgM, HBV surface and core antibodies, HCV antibody and RNA, HDV testing when indicated, and HEV IgM. Additional tests include α-fetoprotein in chronic viral hepatitis, Monospot testing for EBV, and urinalysis for bilirubin.
Imaging includes right upper quadrant ultrasound to assess for biliary obstruction and head CT when hepatic encephalopathy is suspected.
Differential Diagnosis
Consider nonalcoholic fatty liver disease, biliary disease, ischemic hepatitis, congestive heart failure, metabolic and genetic disorders, liver abscess, heat stroke, Wilson disease, Budd–Chiari syndrome, and pregnancy-related liver disease.
Initial Stabilization And Therapy
Airway, breathing, and circulation are priorities in fulminant hepatic failure or severe encephalopathy. Judicious intravenous fluid resuscitation is required.
Emergency Department Treatment And Procedures
Management is largely supportive. Correct hypovolemia and electrolyte disturbances, treat nausea and vomiting, and avoid hepatotoxic medications and alcohol. Use medications with minimal hepatic metabolism when sedation or analgesia is required. Correct coagulopathy only in the presence of active bleeding. Administer N-acetylcysteine for acetaminophen toxicity and consider its use in fulminant hepatic failure. Steroids may benefit severe alcoholic hepatitis. Manage pruritus with bile acid sequestrants. Perform paracentesis for tense ascites causing respiratory compromise. Provide postexposure prophylaxis for hepatitis A and B when indicated.
Medications
Commonly used agents include ondansetron or metoclopramide for nausea, cholestyramine or ursodeoxycholic acid for pruritus, N-acetylcysteine, prednisone for select alcoholic hepatitis cases, vitamin K for coagulopathy, and thiamine in malnourished patients.
Follow-Up And Disposition
Admit patients with intractable vomiting, dehydration, electrolyte abnormalities, pregnancy, immunocompromise, suspected toxic hepatitis, advanced age, or evidence of significant liver dysfunction. Fulminant hepatic failure requires ICU care and early transfer to a transplant center. Patients with mild disease and good oral tolerance may be discharged with close follow-up.
Follow-Up Recommendations
Arrange hepatology or gastroenterology follow-up for definitive diagnosis and management. Counsel on strict hygiene, avoidance of alcohol and acetaminophen, vaccination when appropriate, and limitation of strenuous activity.
Clinical Insights And Common Errors
Acute hepatitis is frequently misdiagnosed as a nonspecific viral illness. Emergency management is primarily supportive. Early recognition of fulminant hepatic failure and prompt transfer to a transplant-capable center are critical. Maintain high suspicion for pregnancy-related liver disease in pregnant patients with compatible symptoms.
- Published on
Emergency And Acute Medicine – Genital Herpes
Overview And Definition
Genital herpes is a lifelong, recurrent viral infection caused by herpes simplex virus (HSV). Approximately one in four Americans older than 30 years are seropositive for HSV-2, though most are asymptomatic. The infection is characterized by periods of latency and reactivation, with viral shedding that can occur even in the absence of visible lesions, contributing to ongoing transmission.
Disease Course And Natural History
Primary infection has an incubation period of approximately 2–12 days, with symptoms typically peaking 8–10 days after onset and lesions healing within about three weeks. Primary disease may present with a more severe clinical syndrome and complications such as meningitis, encephalitis, or hepatitis. Importantly, more than half of first recognized episodes are not true primary infections, as earlier asymptomatic exposure is common.
Recurrent infection results from viral reactivation in the dorsal root ganglia. The average patient experiences about four recurrences per year. Recurrences are generally milder, involve fewer lesions, and resolve within 10 days. HSV-1 genital infections recur significantly less often than HSV-2.
Asymptomatic infection is common, with intermittent viral shedding that frequently leads to transmission by individuals without symptoms or lesions.
Etiology And Risk Factors
Most cases (70–90%) of genital herpes are caused by HSV-2, with the remainder caused by HSV-1. Genital HSV-1 infections are increasing in prevalence, likely due to higher rates of oral–genital contact and reduced childhood exposure to HSV-1, leaving more adolescents and adults susceptible. Acquisition of HSV-2 in individuals with prior HSV-1 infection tends to be less symptomatic, whereas acquisition of HSV-1 in those with HSV-2 is uncommon.
Genital herpes is strongly associated with HIV and other sexually transmitted infections. HSV infection increases both susceptibility to HIV and HIV viral shedding during HSV reactivation. Despite extensive research, no effective HSV vaccine is currently available.
Clinical Manifestations
Patients commonly report local pain, itching, or burning. A prodrome of tingling, itching, or pain may occur 1–2 days before lesion eruption and can sometimes mimic radicular pain such as sciatica. Lesions typically begin as macules and papules, progress to vesicles and pustules, and then ulcerate by approximately day five. Skin lesions crust over, whereas mucosal lesions heal without crusting.
Associated manifestations may include herpetic cervicitis, vaginitis, or urethritis, presenting with dysuria, urinary hesitancy or retention, vaginal discharge, or pelvic pain. Systemic symptoms such as fever, malaise, headache, myalgias, photophobia, anorexia, and lymphadenopathy are more common during primary infection.
Physical Examination Findings
Examination may reveal grouped vesicles on an erythematous base involving the vulva, vagina, cervix, perineum, buttocks, penile shaft, or glans. On moist mucosal surfaces, ulcers may predominate. Atypical findings include localized edema, erythema, fissures, or crusts.
Special Populations
Pediatric patients with genital herpes require careful evaluation, as neonatal infection is often disseminated or involves the central nervous system and carries high morbidity and mortality. Congenital HSV infection without vesicles may resemble other congenital infections. Sexual abuse must be considered in children with genital HSV, and evaluation for other sexually transmitted infections is recommended.
Pregnancy is a high-risk context, particularly with primary infection, which is associated with significant neonatal morbidity. Suppressive antiviral therapy after 36 weeks’ gestation reduces lesion recurrence at delivery and lowers cesarean section rates.
Diagnostic Approach
Diagnosis is usually clinical, based on history and examination. Laboratory testing is useful when confirmation is needed or in atypical or severe cases. Viral culture from vesicle fluid or ulcer base is positive in most early lesions but loses sensitivity as lesions heal. PCR testing is more sensitive and is the diagnostic test of choice, especially for central nervous system involvement. Serologic testing detects prior exposure but is not useful for diagnosing acute disease, as it cannot distinguish recent from chronic infection.
Differential Diagnosis
Important considerations include syphilis, chancroid, lymphogranuloma venereum, granuloma inguinale, candidiasis, and Behçet syndrome.
Emergency Management
Treatment reduces symptom severity and duration but does not eradicate latent virus or prevent future recurrences once therapy is stopped.
Episodic therapy for recurrences shortens lesion duration if started during the prodrome or within one day of lesion onset.
Suppressive therapy, recommended for patients with frequent recurrences (six or more per year), reduces recurrence frequency by approximately 75% and decreases viral shedding.
Severe disease, systemic involvement, or infection in immunocompromised patients requires intravenous antiviral therapy. Resistance to acyclovir occurs in a minority of immunocompromised patients, in which case foscarnet may be effective. Women with urinary retention may require bladder catheterization due to sacral nerve involvement.
Pharmacologic Treatment
First-episode, recurrent, suppressive, and HIV-associated genital herpes are treated with oral or intravenous acyclovir, valacyclovir, or famciclovir, with regimen selection based on severity, immune status, and recurrence pattern. Dosing adjustments may be necessary in renal impairment.
Disposition And Counseling
Hospital admission is indicated for patients with central nervous system involvement, disseminated disease, severe pain or urinary retention, or significant immunosuppression. Immunocompetent patients without systemic involvement can be managed as outpatients.
Discharge counseling is essential and should emphasize avoidance of sexual contact during prodrome and active lesions, consistent use of barrier protection even when asymptomatic, and the lifelong nature of infection. Patients should be informed about the likelihood of recurrence and the option of suppressive therapy.
Key Clinical Pearls
Primary genital herpes may be severe and should be treated promptly. Always consider sexual abuse in children presenting with genital HSV. Genital herpes is lifelong, with intermittent asymptomatic shedding that plays a major role in transmission.
Overview And Definition
Genital herpes is a lifelong, recurrent viral infection caused by herpes simplex virus (HSV). Approximately one in four Americans older than 30 years are seropositive for HSV-2, though most are asymptomatic. The infection is characterized by periods of latency and reactivation, with viral shedding that can occur even in the absence of visible lesions, contributing to ongoing transmission.
Disease Course And Natural History
Primary infection has an incubation period of approximately 2–12 days, with symptoms typically peaking 8–10 days after onset and lesions healing within about three weeks. Primary disease may present with a more severe clinical syndrome and complications such as meningitis, encephalitis, or hepatitis. Importantly, more than half of first recognized episodes are not true primary infections, as earlier asymptomatic exposure is common.
Recurrent infection results from viral reactivation in the dorsal root ganglia. The average patient experiences about four recurrences per year. Recurrences are generally milder, involve fewer lesions, and resolve within 10 days. HSV-1 genital infections recur significantly less often than HSV-2.
Asymptomatic infection is common, with intermittent viral shedding that frequently leads to transmission by individuals without symptoms or lesions.
Etiology And Risk Factors
Most cases (70–90%) of genital herpes are caused by HSV-2, with the remainder caused by HSV-1. Genital HSV-1 infections are increasing in prevalence, likely due to higher rates of oral–genital contact and reduced childhood exposure to HSV-1, leaving more adolescents and adults susceptible. Acquisition of HSV-2 in individuals with prior HSV-1 infection tends to be less symptomatic, whereas acquisition of HSV-1 in those with HSV-2 is uncommon.
Genital herpes is strongly associated with HIV and other sexually transmitted infections. HSV infection increases both susceptibility to HIV and HIV viral shedding during HSV reactivation. Despite extensive research, no effective HSV vaccine is currently available.
Clinical Manifestations
Patients commonly report local pain, itching, or burning. A prodrome of tingling, itching, or pain may occur 1–2 days before lesion eruption and can sometimes mimic radicular pain such as sciatica. Lesions typically begin as macules and papules, progress to vesicles and pustules, and then ulcerate by approximately day five. Skin lesions crust over, whereas mucosal lesions heal without crusting.
Associated manifestations may include herpetic cervicitis, vaginitis, or urethritis, presenting with dysuria, urinary hesitancy or retention, vaginal discharge, or pelvic pain. Systemic symptoms such as fever, malaise, headache, myalgias, photophobia, anorexia, and lymphadenopathy are more common during primary infection.
Physical Examination Findings
Examination may reveal grouped vesicles on an erythematous base involving the vulva, vagina, cervix, perineum, buttocks, penile shaft, or glans. On moist mucosal surfaces, ulcers may predominate. Atypical findings include localized edema, erythema, fissures, or crusts.
Special Populations
Pediatric patients with genital herpes require careful evaluation, as neonatal infection is often disseminated or involves the central nervous system and carries high morbidity and mortality. Congenital HSV infection without vesicles may resemble other congenital infections. Sexual abuse must be considered in children with genital HSV, and evaluation for other sexually transmitted infections is recommended.
Pregnancy is a high-risk context, particularly with primary infection, which is associated with significant neonatal morbidity. Suppressive antiviral therapy after 36 weeks’ gestation reduces lesion recurrence at delivery and lowers cesarean section rates.
Diagnostic Approach
Diagnosis is usually clinical, based on history and examination. Laboratory testing is useful when confirmation is needed or in atypical or severe cases. Viral culture from vesicle fluid or ulcer base is positive in most early lesions but loses sensitivity as lesions heal. PCR testing is more sensitive and is the diagnostic test of choice, especially for central nervous system involvement. Serologic testing detects prior exposure but is not useful for diagnosing acute disease, as it cannot distinguish recent from chronic infection.
Differential Diagnosis
Important considerations include syphilis, chancroid, lymphogranuloma venereum, granuloma inguinale, candidiasis, and Behçet syndrome.
Emergency Management
Treatment reduces symptom severity and duration but does not eradicate latent virus or prevent future recurrences once therapy is stopped.
Episodic therapy for recurrences shortens lesion duration if started during the prodrome or within one day of lesion onset.
Suppressive therapy, recommended for patients with frequent recurrences (six or more per year), reduces recurrence frequency by approximately 75% and decreases viral shedding.
Severe disease, systemic involvement, or infection in immunocompromised patients requires intravenous antiviral therapy. Resistance to acyclovir occurs in a minority of immunocompromised patients, in which case foscarnet may be effective. Women with urinary retention may require bladder catheterization due to sacral nerve involvement.
Pharmacologic Treatment
First-episode, recurrent, suppressive, and HIV-associated genital herpes are treated with oral or intravenous acyclovir, valacyclovir, or famciclovir, with regimen selection based on severity, immune status, and recurrence pattern. Dosing adjustments may be necessary in renal impairment.
Disposition And Counseling
Hospital admission is indicated for patients with central nervous system involvement, disseminated disease, severe pain or urinary retention, or significant immunosuppression. Immunocompetent patients without systemic involvement can be managed as outpatients.
Discharge counseling is essential and should emphasize avoidance of sexual contact during prodrome and active lesions, consistent use of barrier protection even when asymptomatic, and the lifelong nature of infection. Patients should be informed about the likelihood of recurrence and the option of suppressive therapy.
Key Clinical Pearls
Primary genital herpes may be severe and should be treated promptly. Always consider sexual abuse in children presenting with genital HSV. Genital herpes is lifelong, with intermittent asymptomatic shedding that plays a major role in transmission.
