Edit concept Create issue ticket

Secondary Biliary Cirrhosis

Obstructive Liver Cirrhosis


Presentation

  • This case report documents a patient who presented with retained common bile duct (CBD) stones, recurrent attacks of cholangitis, CBD stricture, and secondary biliary cirrhosis.[ncbi.nlm.nih.gov]
  • The clinical presentation was with fever, weight loss, cholestatic jaundice with hepatosplenomegaly and generalized lymphadenopathy.[ncbi.nlm.nih.gov]
  • A rationalisation of the 1350 boxes used throughout the book gives a simpler and clearer presentation of the various categories.[books.google.com]
  • We present the case of a 35-year-old patient with a very uncommon association of Budd-Chiari syndrome secondary to hepatocellular carcinoma (HCC) developed on a non-cirrhotic right liver lobe and secondary biliary cirrhosis of the left liver lobe, that[ncbi.nlm.nih.gov]
  • […] with a broad spectrum of presentations from mild alteration of liver function tests to deep jaundice with complete biliary obstruction, recurrent cholangitis, secondary biliary cirrhosis and liver failure.[freethesaurus.com]
Generalized Lymphadenopathy
  • The clinical presentation was with fever, weight loss, cholestatic jaundice with hepatosplenomegaly and generalized lymphadenopathy.[ncbi.nlm.nih.gov]
Easy Bruising
  • The most common initial symptoms are: Itchy skin Fatigue Jaundice symptoms like yellowing of the eyes and skin Other manifestations may include: Darkening of exposed areas of the skin Fever Bone pain Pale-colored, foul-smelling, bulky stools Easy bruising[digestion.ygoy.com]
  • Cirrhosis can lead to Easy bruising or bleeding, or nosebleeds Swelling of the abdomen or legs Extra sensitivity to medicines High blood pressure in the vein entering the liver Enlarged veins called varices in the esophagus and stomach.[icdlist.com]
  • Some of the signs and symptoms of liver disease include: jaundice (yellowing of the skin and eyes) pruritus (itching) dark, tea-colored urine weight loss muscle wasting ascites (swelling of the abdomen with fluid) fatigue easy bruising and bleeding (bleeding[surgery.uic.edu]
  • Easy bruising and bleeding. Periods may become heavier. Bleeding into the bowel and stomach may occur. Stomach ulcers are more common. There may be swelling of the stomach (ascites) due to fluid leak from the liver.[patient.info]
Weight Loss
  • The clinical presentation was with fever, weight loss, cholestatic jaundice with hepatosplenomegaly and generalized lymphadenopathy.[ncbi.nlm.nih.gov]
  • Characterized by progressive weight loss and fatigue, steatorrhea, splenomegaly and hepatomegaly. Accession portal hypertension and hepatic failure is observed in the late stage secondary biliary cirrhosis.[medicalformat.com]
  • loss, weakness, fatigue, and even osteoporosis as a result of vitamin D malabsorption and subsequent calcium deficiency.[aafp.org]
Loss of Appetite
  • Case presentation A 58-year-old female patient, who complained of icterus appearing in the last 6-7 months, along with the symptoms of fatigue and loss of appetite continued for 2-3 years, was referred to our clinic.[link.springer.com]
  • As the disease progresses, symptoms may include weakness, fatigue, loss of appetite, nausea, vomiting, weight loss, abdominal pain and bloating, itching, and spiderlike blood vessels on the skin.[surgery.ucsf.edu]
Jaundice
  • The clinical presentation was with fever, weight loss, cholestatic jaundice with hepatosplenomegaly and generalized lymphadenopathy.[ncbi.nlm.nih.gov]
  • Subsequent skin biopsy was consistent with cutaneous graft-versus-host disease, and biopsy-proven hepatic involvement manifested by severe, unremitting cholestatic jaundice soon followed.[ncbi.nlm.nih.gov]
  • The diagnosis of cirrhosis should never be established without thorough visualization of the entire biliary tract in patients with biochemical or clinical jaundice of unknown origin.[ncbi.nlm.nih.gov]
  • In such a case presenting as persisting and fluctuating obstructive jaundice in HCC, an intrabile duct tumor growth should be suspected.[jpatholtm.org]
  • SECONDARY BILIARY CI RR H OS I S Symptoms Clinical features · Jaundice · Pruritus · Biliary colics. SECONDARY BILIARY CI RR H OS I S Diagnosis Invest i gatio n · Cholangiography · Liver biopsy.[medicscientist.com]
Hepatomegaly
  • Characterized by progressive weight loss and fatigue, steatorrhea, splenomegaly and hepatomegaly. Accession portal hypertension and hepatic failure is observed in the late stage secondary biliary cirrhosis.[medicalformat.com]
  • P hy sica l e x am i natio n : May be normal (no finding) Jaundice Melanosis Xanthomas Hepatomegaly Splenomegaly Clubbing Bone tenderness Sicca syndrome (drying up of mucus membranes) CREST syndrome.[medicscientist.com]
  • In her physical examination, icteric appearance, moderate hepatomegaly and kyphosis was detected.[link.springer.com]
  • Her bowel sounds are normal and hepatomegaly is present. A right upper quadrant ultrasound shows no evidence of extrahepatic biliary dilation.[medbullets.com]
  • In later phases of the disease, patients may develop hepatomegaly, xanthomas/xanthalasmas, melanotic patches, or evidence of portal hypertension (splenomegaly, ascites, edema, encephalopathy).[clinicaladvisor.com]
Scleral Icterus
  • icterus Vascular spiders (spider telangiectasias, spider angiomata) Splenomegaly Testicular atrophy Most patients with cirrhosis severe enough to lead to ascites have additional stigmata of cirrhosis on physical examination.[aafp.org]
Fetor Hepaticus
  • hepaticus—a sweet, pungent breath odor Gynecomastia Hepatomegaly Jaundice Kayser-Fleischer ring—brown-green ring of copper deposit around the cornea, pathognomonic for Wilson’s disease Nail changes: Muehrcke’s nails—paired horizontal white bands separated[aafp.org]
Pruritus
  • SECONDARY BILIARY CI RR H OS I S Symptoms Clinical features · Jaundice · Pruritus · Biliary colics. SECONDARY BILIARY CI RR H OS I S Diagnosis Invest i gatio n · Cholangiography · Liver biopsy.[medicscientist.com]
  • The secondary biliary cirrhosis is characterized by jaundice, pruritus, pyrexia, pain in the right upper quadrant.[medicalformat.com]
  • Affecting chiefly middle-aged women, it is characterized by chronic cholestasis with pruritus, jaundice, and hypercholesterolemia with xanthomas, and malabsorption. secondary biliary cirrhosis cirrhosis resulting from chronic bile obstruction due to congenital[medical-dictionary.thefreedictionary.com]
  • The typical patient is a middle-aged woman presenting with symptoms of fatigue and pruritus and laboratory test evidence of cholestasis.[radiopaedia.org]
  • The pruritus has a diurnal variation and tends to be worst in the evenings.[clinicaladvisor.com]
Dark Urine
  • With an increase in the appearance of jaundice is observed dark urine and feces discolored. Often there is nausea, low-grade fever, episodic fever, pain in the right abdomen associated recurrent cholangitis or biliary colic.[medicalformat.com]
  • Clinical description Clinical signs of cholestasis (discolored stools, dark urine) appear within the first year of life in about one third of patients, or later with recurrent episodes of jaundice and mild pruritus.[orpha.net]
  • Jaundice, dark urine, and darkening of the skin exposed to the sun occur as the liver disease progresses. The slow progression results eventually in portal hypertension, the accumulation of fluids, and eventually liver failure and death.[healthcentral.com]

Workup

  • […] with seemingly intact, newly formed limiting plates Stage 3 (septal stage): Septal fibrosis with active inflammatory, passive paucicellular septa, or both are present Stage 4 (cirrhosis): Nodules with various degrees of inflammation are present See Workup[emedicine.medscape.com]
ST Elevation
  • A cardiology evaluation was requested and the patient was found to have a non-ST elevation myocardial infarction, which was treated medically.[acgcasereports.gi.org]
Gram-Positive Rods
  • Histopathologic examination of the gallbladder revealed gangrenous cholecystits with gram-positive rods invading the gallbladder wall. Cultures from the gallbladder and blood grew Clostridium perfringens .[acgcasereports.gi.org]
Foam Cell
  • Bile from damaged intrahepatic ducts accumulate in the areas of periportal necrosis, forming a so-called bile "lake", surrounded by foam cells rich in cholesterol.[medicalformat.com]

Treatment

  • However, even after cirrhosis occurs, aggressive treatment does not increase the mortality of treatment or the treatment failure rate.[ncbi.nlm.nih.gov]
  • Endoscopic or surgical treatment of choledocholithiasis must be considered early in treatment.[ncbi.nlm.nih.gov]
  • Biliary Cirrhosis (Primary and Secondary) Symptoms Diagnosis and Treatment BILI ARY CIRRHOSIS Biliary cirrhosis occurs due to injury or prolonged obstruction of intrahepatic or extrahepatic biliary system.[medicscientist.com]

Prognosis

  • Presents as asymptomatic elevation in alkaline phosphatase (better prognosis) or with pruritus, S e con d a ry b i li a ry c irrho sis i s due to l ong-stan d ing obst ru c ti o n , of l a r ge r extrahepatic ducts .[medicscientist.com]
  • […] hyperintensity (cuffing) segmental hypertrophy, notably of the caudate lobe hepatic surface irregularity due to regenerative nodules regional lymphadenopathy: tends to dominate in the gastrohepatic ligament and porta hepatis 4 splenomegaly Treatment and prognosis[radiopaedia.org]
  • Prognosis The most reliable determinants of patient prognosis in primary biliary cholangitis are the height of the serum bilirubin level and the Mayo risk score.[emedicine.medscape.com]
  • People with primary biliary cholangitis and cirrhosis have a poor prognosis and higher risk of other complications. Increased pressure in the portal vein (portal hypertension).[mayoclinic.org]

Etiology

  • […] liver Prediction and prevention of secondary biliary cirrhosis of the liver Secondary biliary cirrhosis - Treatment Secondary biliary cirrhosis Gastroenterology primary and secondary biliary cirrhosis account for 5-10% of liver cirrhosis of various etiologies[medicalformat.com]
  • […] macronodular) resulting as a sequel to acute hepatitis. postnecrotic cirrhosis cirrhosis following submassive necrosis of the liver (subacute yellow atrophy) due to toxic or viral hepatitis. primary biliary cirrhosis a rare form of biliary cirrhosis of unknown etiology[medical-dictionary.thefreedictionary.com]
  • Physical examination of patients with cirrhosis may reveal a variety of findings that necessitate a hepatic- or gastrointestinal-based work-up to determine the etiology.[aafp.org]
  • Emphysematous cholecystitis is more common in males, and more than one-third of patients with this condition have diabetes mellitus. 2,6 The exact etiology of emphysematous cholecystitis is unknown, but an initial ischemic insult with secondary infection[acgcasereports.gi.org]
  • Etiology PFIC3 is due to mutations in the ABCB4 gene (7q21), encoding the multi-drug resistant 3 protein (MDR3), resulting in impaired biliary phospholipid (PL) secretion.[orpha.net]

Epidemiology

  • Summary Epidemiology Estimated prevalence at birth of PFIC types 1-3 varies between 1/50,000 and 1/100,000. PFIC3 represents one third of PFIC cases.[orpha.net]
  • Epidemiology United States statistics Although the epidemiology of primary biliary cholangitis has not been studied systematically, the published prevalence is 65.4 cases for women and 12.1 cases for men (40.2 cases overall) per 100,000 population.[emedicine.medscape.com]
  • "USA PBC Epidemiology Group. Risk factors and comorbidities in primary biliary cirrhosis: a controlled interview-based study of 1032 patients". Hepatology. vol. 42. 2005. pp. 1194-202.[clinicaladvisor.com]
  • Relation of hemochromatosis with hepatocellular carcinoma: epidemiology, natural history, pathophysiology, screening, treatment, and prevention. Med Clin North Am . 2005;89:391–409. 19. Neimark E, Schilsky ML, Shneider BL.[aafp.org]
  • Epidemiology of liver disease in cystic fibrosis: a longitudinal study. J Hepatol 2004; 41: 920-925. [PubMed] Lewindon PJ, Pereira TN, Hoskins AC, et al . The role of hepatic stellate cells and transforming growth factor-beta (1) in cystic fibrosis.[cfmedicine.com]
Sex distribution
Age distribution

Pathophysiology

  • Approximately 80% of patients with PSC have inflammatory bowel disease, more commonly ulcerative colitis than Crohn’s disease. 21 Pathophysiology and Natural History The pathophysiology of PSC is unclear, but there is evidence suggesting an autoimmune[clevelandclinicmeded.com]
  • Although numerous pathophysiologic mechanisms of injury exist, the final common pathway is persistent wound healing resulting in hepatic parenchymal fibrosis.[aafp.org]
  • Pathophysiology The exact mechanism of the liver damage is unknown, although evidence indicates that it can be of autoimmune origin.[emedicine.medscape.com]

Prevention

  • When a fatal obstruction of the biliary tract in order to prevent infection or recurrence of cholangitis conducted antibiotic therapy.[medicalformat.com]
  • Decompression of biliary obstruction may be achieved surgically, endoscopically and via the percutaneous transhepatic route, bacterial infections can be controlled by powerful antibiotics, thus preventing the progress of cholangitis to secondary biliary[ncbi.nlm.nih.gov]
  • This volume offers authoritative, evidence-based recommendations for preventing and managing complications in all current general surgery procedures.[books.google.com]
  • […] function, (4) to prevent or resolve gastrointestinal bleeding, and (5) to provide adequate nutritional intake and a positive nitrogen balance.[medical-dictionary.thefreedictionary.com]
  • Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets[books.google.com]

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!