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Pathology - Hypertensive Nephrosclerosis
Linked to chronic, uncontrolled hypertension for more than 15 years.
African Americans and individuals over the age of 45 have an increased likelihood of getting hypertensive nephrosclerosis.
Pathology
Gross: Kidneys that are reduced in size and have visible scars.
Light microscopy shows mesangial cell growth, decreased glomerular tuft, sclerosis and hyalinization of afferent arterioles, interstitial fibrosis, and eventual development of secondary focal segmental glomerulosclerosis.
Symptoms and signs
Nephrotic syndrome typically includes fluctuating levels of protein in the urine, along with hypertension, hypertensive retinopathy, and left ventricular hypertrophy.
Treating hypertension aggressively with ACE inhibitors; considering dialysis and/or transplant for individuals with end-stage renal disease.
Hypertensive nephrosclerosis is the primary cause of end-stage renal disease (ESRD) in African Americans.
Linked to chronic, uncontrolled hypertension for more than 15 years.
African Americans and individuals over the age of 45 have an increased likelihood of getting hypertensive nephrosclerosis.
Pathology
Gross: Kidneys that are reduced in size and have visible scars.
Light microscopy shows mesangial cell growth, decreased glomerular tuft, sclerosis and hyalinization of afferent arterioles, interstitial fibrosis, and eventual development of secondary focal segmental glomerulosclerosis.
Symptoms and signs
Nephrotic syndrome typically includes fluctuating levels of protein in the urine, along with hypertension, hypertensive retinopathy, and left ventricular hypertrophy.
Treating hypertension aggressively with ACE inhibitors; considering dialysis and/or transplant for individuals with end-stage renal disease.
Hypertensive nephrosclerosis is the primary cause of end-stage renal disease (ESRD) in African Americans.
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Pathology - Lupus Nephropathy
Renal involvement in systemic lupus erythematosus.
There are five different kidney histologic patterns. 1. Type I: normal; 2. Type II (mesangial form): focal and segmental glomerular involvement with increased mesangial matrix; 3. Type III (focal proliferative form): affects less than half of glomeruli, causing extensive damage to individual glomeruli; 4. Type IV (diffuse proliferative form): the most severe form involving all glomeruli with significant inflammation, mesangial proliferation, and scarring. Light microscopy shows wire-loop irregularity due to immune complex deposition and significant thickening of the glomerular basement membrane. Examining endothelial cell growth with electron microscopy. Immunofluorescence shows significant accumulation of immunological complexes in the subendothelial layer.
(5) Type V (membranous form) resembles membranous glomerulonephritis.
Clinical Symptoms
Type I: No observable clinical symptoms.
Types II and III present with mild to moderate levels of proteinuria and hematuria.
Type IV involves a combination of symptoms from both nephrotic and nephritic illnesses.
Nephrotic syndrome is classified as Type V.
Therapies
Types I and II: No intervention required.
Types III, IV, and V involve immunosuppressive treatment such as corticosteroids, cyclophosphamide, and/or azathioprine. Severe cases may require transplantation or dialysis.
The degree of renal lesions frequently dictates the overall outcome of patients with systemic lupus erythematosus (SLE).
Renal involvement in systemic lupus erythematosus.
There are five different kidney histologic patterns. 1. Type I: normal; 2. Type II (mesangial form): focal and segmental glomerular involvement with increased mesangial matrix; 3. Type III (focal proliferative form): affects less than half of glomeruli, causing extensive damage to individual glomeruli; 4. Type IV (diffuse proliferative form): the most severe form involving all glomeruli with significant inflammation, mesangial proliferation, and scarring. Light microscopy shows wire-loop irregularity due to immune complex deposition and significant thickening of the glomerular basement membrane. Examining endothelial cell growth with electron microscopy. Immunofluorescence shows significant accumulation of immunological complexes in the subendothelial layer.
(5) Type V (membranous form) resembles membranous glomerulonephritis.
Clinical Symptoms
Type I: No observable clinical symptoms.
Types II and III present with mild to moderate levels of proteinuria and hematuria.
Type IV involves a combination of symptoms from both nephrotic and nephritic illnesses.
Nephrotic syndrome is classified as Type V.
Therapies
Types I and II: No intervention required.
Types III, IV, and V involve immunosuppressive treatment such as corticosteroids, cyclophosphamide, and/or azathioprine. Severe cases may require transplantation or dialysis.
The degree of renal lesions frequently dictates the overall outcome of patients with systemic lupus erythematosus (SLE).
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Pathology - Poststreptococcal Glomerulonephritis (Acute Proliferative Glomerulonephritis)
Commonly observed in youngsters after being infected with nephritogenic strains of group A beta-hemolytic streptococci.
Study of diseases
The gross pathology shows a severe inflammatory response affecting all glomeruli in both kidneys, leading to pinpoint hemorrhages on the surfaces of the kidneys.
Under light microscopy, the glomeruli seem enlarged, hypercellular, and swollen with increased mesangial and endothelial cell proliferation. The thickness of the glomerular basement membrane is within normal range.
Electron microscopy reveals electron-dense humps located on the epithelial side of the basement membrane (subepithelial localization).
Immunofluorescence shows a coarse granular pattern for lgG or C3 (lumpy-bumpy).
Clinical Symptoms
Nephritic syndrome presenting with hypertension and swelling around the eyes.
Laboratory results show presence of red blood cells and/or red cell casts in urine, low levels of serum C3, high ASO antibody titer indicating recent streptococcal infection, and azotemia.
Treatment typically results in spontaneous resolution.
Poststreptococcal glomerulonephritis is an immunological complex disease characterized by antigen-antibody complexes of streptococcal origin. It is considered the classic example of the nephritic syndrome.
Commonly observed in youngsters after being infected with nephritogenic strains of group A beta-hemolytic streptococci.
Study of diseases
The gross pathology shows a severe inflammatory response affecting all glomeruli in both kidneys, leading to pinpoint hemorrhages on the surfaces of the kidneys.
Under light microscopy, the glomeruli seem enlarged, hypercellular, and swollen with increased mesangial and endothelial cell proliferation. The thickness of the glomerular basement membrane is within normal range.
Electron microscopy reveals electron-dense humps located on the epithelial side of the basement membrane (subepithelial localization).
Immunofluorescence shows a coarse granular pattern for lgG or C3 (lumpy-bumpy).
Clinical Symptoms
Nephritic syndrome presenting with hypertension and swelling around the eyes.
Laboratory results show presence of red blood cells and/or red cell casts in urine, low levels of serum C3, high ASO antibody titer indicating recent streptococcal infection, and azotemia.
Treatment typically results in spontaneous resolution.
Poststreptococcal glomerulonephritis is an immunological complex disease characterized by antigen-antibody complexes of streptococcal origin. It is considered the classic example of the nephritic syndrome.
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Pathology - Hepatocellular Carcinoma
Etiology
Alcoholic cirrhosis, HBV, HCV, hemochromatosis, Wilson disease, alpha-1-antitrypsin deficiency, and hepatic carcinogens such as aflatoxin B1 are also considered risk factors.
Pathology
Gross: May present as a single mass, numerous masses, or infiltrative carcinoma; exhibiting a pale and green hue.
Macroscopic: Tumors vary in appearance from structures mimicking hepatic tissue to aggressive masses; can infiltrate portal veins or IVC.
Typically disseminated by the bloodstream.Frequently to the lungs. vertebrae. adrenal or cerebral
Symptoms include fever, jaundice, weight loss, worsening hepatomegaly, worsening or bloody ascites, and cirrhotic symptoms.
Laboratory results: High levels of AFP, increased white blood cell count, low red blood cell count, rapid rise in ALP
Therapy
Options include surgical resection, chemotherapy, radiation, and liver transplant.
Death frequently happens within 10 months of being diagnosed.
Hepatocellular carcinoma is the most prevalent primary cancerous tumor in the liver among adults. Cholangiocarcinoma is a malignant tumor that originates from the biliary ducts within the liver. It is linked to clonorchis sinensis infestation and primary sclerosing cholangitis.
Liver adenomas are associated with the usage of oral contraceptives and can lead to intraperitoneal hemorrhage.
Liver angiosarcomas are associated with exposure to polyvinyl chloride (Thorotrast).
Etiology
Alcoholic cirrhosis, HBV, HCV, hemochromatosis, Wilson disease, alpha-1-antitrypsin deficiency, and hepatic carcinogens such as aflatoxin B1 are also considered risk factors.
Pathology
Gross: May present as a single mass, numerous masses, or infiltrative carcinoma; exhibiting a pale and green hue.
Macroscopic: Tumors vary in appearance from structures mimicking hepatic tissue to aggressive masses; can infiltrate portal veins or IVC.
Typically disseminated by the bloodstream.Frequently to the lungs. vertebrae. adrenal or cerebral
Symptoms include fever, jaundice, weight loss, worsening hepatomegaly, worsening or bloody ascites, and cirrhotic symptoms.
Laboratory results: High levels of AFP, increased white blood cell count, low red blood cell count, rapid rise in ALP
Therapy
Options include surgical resection, chemotherapy, radiation, and liver transplant.
Death frequently happens within 10 months of being diagnosed.
Hepatocellular carcinoma is the most prevalent primary cancerous tumor in the liver among adults. Cholangiocarcinoma is a malignant tumor that originates from the biliary ducts within the liver. It is linked to clonorchis sinensis infestation and primary sclerosing cholangitis.
Liver adenomas are associated with the usage of oral contraceptives and can lead to intraperitoneal hemorrhage.
Liver angiosarcomas are associated with exposure to polyvinyl chloride (Thorotrast).
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Pathology - Acute and Chronic Cholecystitis
Acute cholecystitis can be caused by gallstones obstructing the cystic duct (calculous) or by obstruction from bile sludge (acalculous). Acalculous cholecystitis can result from severe injuries or burns, infections such as CMV, Cryptococcus, or Salmonella, postoperative conditions, or prolonged parenteral feeding.
Chronic cholecystitis is a common consequence of gallstones and is characterized by recurring episodes of acute cholecystitis.
Cholecystitis predominantly affects women over the age of 40.
Acute gallbladder condition presents with an enlarged and discolored gallbladder, possibly showing an obstructing stone at the neck and cloudy or purulent bile in the lumen.
Microscopic findings include a thickened and swollen wall with an inflammatory infiltration and vascular congestion.
Chronic condition characterized by the thickening of the gallbladder wall due to significant fibrosis. Microscopic subepithelial fibrosis with mononuclear infiltrate; protrusion of mucosal epithelium through wall (Rokitansky-Aschoff sinuses).
Symptoms include nausea, vomiting, fever, tenderness in the right upper quadrant upon palpation, and the presence of Murphy sign (inspiratory arrest with probing of the right upper quadrant).
Laboratory results show elevated ALP levels, leukocytosis, and slightly increased bilirubin levels.
Chronic symptoms include nausea, vomiting, repeated episodes of colicky discomfort in the right upper quadrant, and intolerance to fatty foods.
Possible complications involve bacterial superinfection, sepsis, and gallbladder perforation or blockage.
Treatment includes antibiotics, cholecystectomy, and ERCP to remove gallstones.
Acute cholecystitis can be caused by gallstones obstructing the cystic duct (calculous) or by obstruction from bile sludge (acalculous). Acalculous cholecystitis can result from severe injuries or burns, infections such as CMV, Cryptococcus, or Salmonella, postoperative conditions, or prolonged parenteral feeding.
Chronic cholecystitis is a common consequence of gallstones and is characterized by recurring episodes of acute cholecystitis.
Cholecystitis predominantly affects women over the age of 40.
Acute gallbladder condition presents with an enlarged and discolored gallbladder, possibly showing an obstructing stone at the neck and cloudy or purulent bile in the lumen.
Microscopic findings include a thickened and swollen wall with an inflammatory infiltration and vascular congestion.
Chronic condition characterized by the thickening of the gallbladder wall due to significant fibrosis. Microscopic subepithelial fibrosis with mononuclear infiltrate; protrusion of mucosal epithelium through wall (Rokitansky-Aschoff sinuses).
Symptoms include nausea, vomiting, fever, tenderness in the right upper quadrant upon palpation, and the presence of Murphy sign (inspiratory arrest with probing of the right upper quadrant).
Laboratory results show elevated ALP levels, leukocytosis, and slightly increased bilirubin levels.
Chronic symptoms include nausea, vomiting, repeated episodes of colicky discomfort in the right upper quadrant, and intolerance to fatty foods.
Possible complications involve bacterial superinfection, sepsis, and gallbladder perforation or blockage.
Treatment includes antibiotics, cholecystectomy, and ERCP to remove gallstones.
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Pathology - Cholelithiasis (Gallstones)
Cholelithiasis refers to the presence of gallstones.
Cholesterol stones are linked to obesity, advanced age, Crohn's illness, cystic fibrosis, many pregnancies, sudden weight reduction, clofibrate and estrogen usage, and Native American ancestry.
Pigment stones are linked to alcoholic cirrhosis, hemolytic anemia, biliary tract infection, and advanced age.
Cholelithiasis is predominantly observed in obese women who have given birth multiple times and are over the age of 40.
Pathophysiology: Stones develop in the gallbladder when cholesterol and bilirubin exceed the capacity of bile acids and lecithin to keep them dissolved.
Cholesterol stones are typically radiolucent, however 10%-20% may seem opaque due to calcification.
Pigment stones are radio-opaque and made up of bile pigments.
Assorted gemstones: Consisting of both cholesterol and bile pigments, this kind is the most prevalent and radiolucent.
Typically without symptoms, but can manifest as biliary colic characterized by colicky epigastric and Right Upper Quadrant pain and nausea, particularly after consuming a fatty meal.
Possible complications involve biliary colic, common bile duct obstruction, cholecystitis, acute pancreatitis, gallstone ileus, ascending cholangitis, and potential cancer of the gallbladder.
If experiencing symptoms, have cholecystectomy; if not, no treatment is necessary.
Cholelithiasis refers to the presence of gallstones.
Cholesterol stones are linked to obesity, advanced age, Crohn's illness, cystic fibrosis, many pregnancies, sudden weight reduction, clofibrate and estrogen usage, and Native American ancestry.
Pigment stones are linked to alcoholic cirrhosis, hemolytic anemia, biliary tract infection, and advanced age.
Cholelithiasis is predominantly observed in obese women who have given birth multiple times and are over the age of 40.
Pathophysiology: Stones develop in the gallbladder when cholesterol and bilirubin exceed the capacity of bile acids and lecithin to keep them dissolved.
Cholesterol stones are typically radiolucent, however 10%-20% may seem opaque due to calcification.
Pigment stones are radio-opaque and made up of bile pigments.
Assorted gemstones: Consisting of both cholesterol and bile pigments, this kind is the most prevalent and radiolucent.
Typically without symptoms, but can manifest as biliary colic characterized by colicky epigastric and Right Upper Quadrant pain and nausea, particularly after consuming a fatty meal.
Possible complications involve biliary colic, common bile duct obstruction, cholecystitis, acute pancreatitis, gallstone ileus, ascending cholangitis, and potential cancer of the gallbladder.
If experiencing symptoms, have cholecystectomy; if not, no treatment is necessary.
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Pathology - Primary Sclerosing Cholangitis
Believed to be autoimmune in nature; 60%-80% of cases are linked to inflammatory bowel disease, typically ulcerative colitis.
Prevalent among males aged 20 to 40.
Pathology
Gross: Liver shows a progressive green tint.
Microscopic findings include fibrosis surrounding bile ducts known as "onion skin fibrosis" along with a lymphocytic infiltration. Fibrosis leads to narrowing or blockage of the bile ducts, causing cholestasis and eventually biliary cirrhosis.
Clinical Symptoms
Symptoms of obstructive jaundice include itching, fatigue, unintentional weight loss, fatty stools due to poor absorption, and enlargement of the liver and spleen.
Complications may arise such as the formation of SBC leading to portal hypertension and a higher occurrence of cholangiocarcinoma.
Laboratory results: Elevated ALP and bilirubin levels; p-ANCA antibodies present in 80% of patients.
Treatment includes Ursodeoxycholic acid, vitamin supplements, symptomatic treatment of biliary obstruction by endoscopic dilatation, and liver transplant for severe illness.
Notes
Believed to be autoimmune in nature; 60%-80% of cases are linked to inflammatory bowel disease, typically ulcerative colitis.
Prevalent among males aged 20 to 40.
Pathology
Gross: Liver shows a progressive green tint.
Microscopic findings include fibrosis surrounding bile ducts known as "onion skin fibrosis" along with a lymphocytic infiltration. Fibrosis leads to narrowing or blockage of the bile ducts, causing cholestasis and eventually biliary cirrhosis.
Clinical Symptoms
Symptoms of obstructive jaundice include itching, fatigue, unintentional weight loss, fatty stools due to poor absorption, and enlargement of the liver and spleen.
Complications may arise such as the formation of SBC leading to portal hypertension and a higher occurrence of cholangiocarcinoma.
Laboratory results: Elevated ALP and bilirubin levels; p-ANCA antibodies present in 80% of patients.
Treatment includes Ursodeoxycholic acid, vitamin supplements, symptomatic treatment of biliary obstruction by endoscopic dilatation, and liver transplant for severe illness.
Notes
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Pathology - Hemochromatosis
Primary hemochromatosis is an autosomal recessive disorder caused by a mutation on chromosome 6 that leads to increased iron absorption in the intestinal mucosa. It is linked to HLA-A3.
Prevalent among individuals of northern European descent, typically manifesting after the age of 30.
Secondary hemochromatosis is an acquired condition resulting from prolonged transfusion therapy together with inefficient erythropoiesis. It is observed in cases of thalassemia major and iron overload disorders.
Iron accumulation as hemosiderin in liver, pancreas, heart, adrenals, testes, pituitary, kidneys, and skin.
Pathology
Hepatic: Gross: sizable; dark brown; micronodular.
Microscopic: buildup of hemosiderin in liver cells starting in periportal hepatocytes, leading to cirrhosis over time.
The classic trio consists of cirrhosis, diabetes, and skin darkening known as bronze diabetes. Other associated symptoms include arthropathy, hepatomegaly, and restrictive cardiomyopathy.
Complications involve a higher likelihood of infection with Vibrio vulnificus, Listeria, and other iron-dependent pathogens, as well as an increased risk of hepatocellular cancer.
Laboratory results show slightly raised liver function tests, higher blood iron levels, lower total iron-binding capacity, and transferrin saturation exceeding 80%. The serum ferritin level is greater than 1000 ug/L.
Administer weekly phlebotomy and deferoxamine treatment while following a low-iron diet.
Screening first-degree relatives of patients with primary hemochromatosis
Primary hemochromatosis is an autosomal recessive disorder caused by a mutation on chromosome 6 that leads to increased iron absorption in the intestinal mucosa. It is linked to HLA-A3.
Prevalent among individuals of northern European descent, typically manifesting after the age of 30.
Secondary hemochromatosis is an acquired condition resulting from prolonged transfusion therapy together with inefficient erythropoiesis. It is observed in cases of thalassemia major and iron overload disorders.
Iron accumulation as hemosiderin in liver, pancreas, heart, adrenals, testes, pituitary, kidneys, and skin.
Pathology
Hepatic: Gross: sizable; dark brown; micronodular.
Microscopic: buildup of hemosiderin in liver cells starting in periportal hepatocytes, leading to cirrhosis over time.
The classic trio consists of cirrhosis, diabetes, and skin darkening known as bronze diabetes. Other associated symptoms include arthropathy, hepatomegaly, and restrictive cardiomyopathy.
Complications involve a higher likelihood of infection with Vibrio vulnificus, Listeria, and other iron-dependent pathogens, as well as an increased risk of hepatocellular cancer.
Laboratory results show slightly raised liver function tests, higher blood iron levels, lower total iron-binding capacity, and transferrin saturation exceeding 80%. The serum ferritin level is greater than 1000 ug/L.
Administer weekly phlebotomy and deferoxamine treatment while following a low-iron diet.
Screening first-degree relatives of patients with primary hemochromatosis
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Pathology - Wilson Disease
This is a rare autosomal recessive condition caused by a mutation in the copper-transporting protein (P-type ATPase), resulting in impaired copper conjugation to ceruloplasmin.
The condition typically begins between the ages of 10 and 30.
Pathophysiology: Enhanced copper uptake in the intestines and reduced elimination through bile results in copper buildup in several tissues, particularly in the parenchymal cells of the liver, kidney, brain, and cornea.
The liver can exhibit a range of conditions from acute and chronic hepatitis to micronodular or macronodular cirrhosis, with Mallory bodies being seen on biopsy.
Brain: Degeneration and formation of empty spaces in basal ganglia
Hepatitis, hypersplenism, hemolytic anemia, portal hypertension, psychosis, or dementia: Kayser-Fleischer rings are thin brown rings observed around the corneas during eye examinations. Choreiform movements refer to extrapyramidal motor symptoms that resemble those seen in Parkinson's disease.
Complications involve a higher likelihood of developing hepatocellular carcinoma.
Laboratory results include reduced serum ceruloplasmin, increased copper in urine, amino acids in urine, glucose in urine, and potentially very low alkaline phosphatase levels.
Penicillamine is used for copper chelation in treatment. Liver transplant for severe instances. diet low in copper
This is a rare autosomal recessive condition caused by a mutation in the copper-transporting protein (P-type ATPase), resulting in impaired copper conjugation to ceruloplasmin.
The condition typically begins between the ages of 10 and 30.
Pathophysiology: Enhanced copper uptake in the intestines and reduced elimination through bile results in copper buildup in several tissues, particularly in the parenchymal cells of the liver, kidney, brain, and cornea.
The liver can exhibit a range of conditions from acute and chronic hepatitis to micronodular or macronodular cirrhosis, with Mallory bodies being seen on biopsy.
Brain: Degeneration and formation of empty spaces in basal ganglia
Hepatitis, hypersplenism, hemolytic anemia, portal hypertension, psychosis, or dementia: Kayser-Fleischer rings are thin brown rings observed around the corneas during eye examinations. Choreiform movements refer to extrapyramidal motor symptoms that resemble those seen in Parkinson's disease.
Complications involve a higher likelihood of developing hepatocellular carcinoma.
Laboratory results include reduced serum ceruloplasmin, increased copper in urine, amino acids in urine, glucose in urine, and potentially very low alkaline phosphatase levels.
Penicillamine is used for copper chelation in treatment. Liver transplant for severe instances. diet low in copper
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Pathology - Portal Hypertension
Prehepatic portal venous blockage is caused by either portal vein thrombosis or splenic intrahepatic portal venous obstruction. Resulting from cirrhosis, metastatic tumor, or schistosomiasis
Posthepatic portal venous blockage can be caused by constrictive pericarditis, tricuspid insufficiency, congestive heart failure, or Budd-Chiari syndrome.
Portal venous blockage causes an increased utilization of venous collaterals in the portal systemic anastomoses, resulting in varices in the submucosal veins of the esophagus, rectal veins, and paraumbilical-inferior epigastric veins. Moreover. Elevated portal vein pressure causes reduced capillary fluid reabsorption, resulting in the accumulation of fluid in the peritoneal cavity known as ascites.
Clinical Symptoms
Enlarged spleen; swollen veins in the esophagus; swollen veins in the rectum; swollen veins around the belly button; vomiting blood; little dilated blood vessels on the skin. Hypotension and pallor are symptoms of ascites.
Therapy
Splenectomy cures portal hypertension resulting from splenic vein thrombosis.
Treatment options for variceal hemorrhage include band ligation or sclerotherapy. Anticoagulation for portal vein thrombosis Cirrhosis treatment notes
Prehepatic portal venous blockage is caused by either portal vein thrombosis or splenic intrahepatic portal venous obstruction. Resulting from cirrhosis, metastatic tumor, or schistosomiasis
Posthepatic portal venous blockage can be caused by constrictive pericarditis, tricuspid insufficiency, congestive heart failure, or Budd-Chiari syndrome.
Portal venous blockage causes an increased utilization of venous collaterals in the portal systemic anastomoses, resulting in varices in the submucosal veins of the esophagus, rectal veins, and paraumbilical-inferior epigastric veins. Moreover. Elevated portal vein pressure causes reduced capillary fluid reabsorption, resulting in the accumulation of fluid in the peritoneal cavity known as ascites.
Clinical Symptoms
Enlarged spleen; swollen veins in the esophagus; swollen veins in the rectum; swollen veins around the belly button; vomiting blood; little dilated blood vessels on the skin. Hypotension and pallor are symptoms of ascites.
Therapy
Splenectomy cures portal hypertension resulting from splenic vein thrombosis.
Treatment options for variceal hemorrhage include band ligation or sclerotherapy. Anticoagulation for portal vein thrombosis Cirrhosis treatment notes
