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Cholestasis Emil, Hizbullah and Martin
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Definition Cholestasis is a condition where bile cannot flow from the liver to the duodenum. Obstructive type of cholestasis where there is a mechanical blockage in the duct system that can occur from a gallstone or malignancy Metabolic types of cholestasis which are disturbances in bile formation that can occur because of genetic defects or acquired as a side effect of many medications.
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Causes Pregnancy – Increased estrogen increases cholesterol Cancer – Hypercoaguable state Biliary atresia – because of blindloop Gallstones Primary biliary cirrhosis – posthepatic obstructive jaundice Drugs – Erythromycin, anabolic steroids
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Signs and symptoms Pruritus is the primary symptom of cholestasis. Due to bile accumulation in skin. We can use naltrexone to relieve the pruritic symptoms. Jaundice because of increased conjugated bilinogen pale stool – post hepatic obstruction – no bile to duodenum dark urine – because very much urobilinogen in urine Xanthoma, Xanthelasma – due to hyperlipidemia
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Primary biliary cirrhosis Primary biliary cirrhosis, (PBC), is an autoimmune disease of the liver marked by slow progressive destruction of the small bile ducts of the liver, with the intralobular ducts and the Canals of Hering affected early in the disease. When these ducts are damaged, bile and other toxins build up in the liver (cholestasis) and over time damages the liver tissue in combination with immune related damage. This can lead to scarring, fibrosis and cirrhosis
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Primary biliary cirrhosis Diagnosis: Biopsy Labresults: elevated GGT and ALP (possibly ALT/AST later on) antimitochondrial antibody (AMA), antinuclear antibody (ANA) Treatment Ursodeoxycholic acid (Ursodiol). helps reduce the cholestasis Fat soluble vit supplementation Naltrexone to remove bile acids in circulation and remove pruritus Screen for esophageal varices Pic: bile duct inflammation and periductal granulomas from biopsy
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Primary sclerosing cholangitis Disease of the bile ducts that causes inflammation and obliterative fibrosis of bile ducts inside and/or outside of the liver. This pathological process impedes the flow of bile to the intestines and can ultimately lead to cirrhosis of the liver, liver failure, and other complications, including but not limited to bile duct and liver cancer The underlying cause of the inflammation remains unknown, but elements of autoimmunity and microbial dysbiosis have been described and are suggested by the fact that approximately 75% of those with PSC also have inflammatory bowel disease (IBD), most often ulcerative colitis The most definitive treatment for PSC is liver transplantation
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Primary sclerosing cholangitis diagnosed on the basis of having at least two of three clinical: serum alkaline phosphatase (ALP) > 1.5x the upper limit of normal, cholangiography demonstrating biliary strictures or irregularity consistent with PSC, and Liver histology from biopsy (ERCP), which typically reveals "beading" (alternating strictures and dilation) of the bile ducts inside and/or outside the liver. Management: There is no FDA-approved pharmacologic treatment for PSC. Some experts recommend a trial of ursodeoxycholic acid (UDCA) Antipruritics like cholestyramine Liver transplantation is the only proven long-term treatment of PSC. Indications for transplantation include recurrent bacterial cholangitis, decompensated cirrhosis, hepatocellular carcinoma Pic: cholangiogram
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Gallstones formed within the gallbladder as a concretion of bile components. Presence of gallstones in the gallbladder may lead to acute cholecystitis an inflammatory condition characterized by retention of bile in the gallbladder and often secondary infection by intestinal microorganisms, predominantly Escherichia coli, Klebsiella,Enterobacter and Bacteroides species. Complications: ascending cholangitis or pancreatitis; due to presence of gallstone in other parts of biliary tract such as common bile duct or pancreatic duct Gallstone ileus
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Gallstones Diagnosis using USG Treatment: Cholecystectomy ursodeoxycholic acid (Ursodiol) "lithotripsy" which is a method of concentrating ultrasonic shock waves onto the stones to break them into tiny pieces. They are then passed safely in the feces.
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Cholestasis in pregnancy Cholestasis of pregnancy occurs idiopathically in late pregnancy and triggers intense pruritus, usually on the hands and feet Cholestasis of pregnancy can make you intensely uncomfortable but poses no long-term risk to an expectant mother. For a developing baby, however, cholestasis of pregnancy can be dangerous, early birth is suggested because the doctors think that it is safer. (the meconium ileus might be passed before the child is born which may cause breathing problems)
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Cholestasis in pregnancy Treatment and management: is to deliver the baby During pregnancy you will be offered more appointments for antenatal screening of the fetus to make sure normal growth of the child. Vitamin K supplement – because obstetric cholestasis can affect your absorption of it. Calamine lotion – safe antipruritic cream for pregnant women.
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Source Harrisons principles of internal medicine ed19 Mayoclinic.org https://en.wikipedia.org/wiki/Cholestasis Robbins and Cotran Pathologic basis of disease
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