Cirrhosis of the liver biliary

Cirrhosis of the liver biliary

Primary biliary cirrhosis is a chronic cholestatic granulomatous destructive inflammatory disease of the interlobular and septal bile ducts, presumably due to autoimmune reactions and capable of progressing to cirrhosis or being associated with it. Women 35-60 years old are more often ill. Primary biliary cirrhosis is the outcome of chronic hepatitis and other chronic forms of intrahepatic cholestasis with segmental destruction, and then complete desolation of the septal bile ducts and impaired liver architectonics. Genetic aspects. Occurs as a result of deficiency of dihydrolipoamide acetyltransferase (109720, [EC, R]). Clinical picture

    • At an early stage of the disease, the manifestations are poor, the diagnosis is suggested when a significant increase in serum ALP is detected.
    • Most often, the primary symptomatology is represented by pruritus (may be extremely pronounced, especially at night)
    • In the later stages of the disease, jaundice, osteogenesis disorders, xanthomas, an enlarged hard, painless liver, an enlarged spleen are observed.
    • Late onset of portal hypertension
    • Often combined with Sjögren’s syndrome, the presence of antithyroid antibodies, rheumatoid arthritis, CREST syndrome.


    • Increase in serum cholesterol, alkaline phosphatase (4-6 times), direct bilirubin
    • The most reliable functional criterion is the appearance of antimitochondrial antibodies
    • Increase in IgG and IgM titers
    • Pronounced hyperbilirubinemia (>342 µmol/l), mainly due to associated
    • Extrahepatic nocturnal biliary obstruction is ruled out by retrograde cholangiopancreatography or, more informatively, by FEGDS with retrograde cholangiopancreatography
    • Liver biopsy. Treatment is conservative, supportive – see Primary sclerosing cholangitis. Primary biliary cirrhosis is one of the main indications for liver transplantation.

Diet. As food additives, triglycerides with an average chain length are used (in a small amount they are found in butter, coconut oils). Secondary biliary cirrhosis develops with a long-term violation of the outflow of bile at the level of large intrahepatic and extrahepatic bile ducts. Etiology

    • Benign diseases of the biliary tract and pancreas (cholelithiasis, fibrous-indurated pancreatitis)
    • Inflammatory and cicatricial narrowing of the biliary tract and pancreas
    • Primary and metastatic tumors of the hepatopancreatoduodenal zone
    • Congenital malformations of the extrahepatic biliary tract
    • Parasitic diseases of the liver and biliary tract (echinococcosis of the liver, ascariasis, opisthorchiasis). Pathomorphology. Expansion of the bile ducts. Pronounced cholestasis on the periphery of the hepatic lobules is pathognomonic. Diagnostic value are necrosis in the peripheral parts of the hepatic lobules with the formation of gall lakes. The clinical picture is similar to that in primary biliary cirrhosis, but may be masked by manifestations of the underlying disease. Perhaps the development of cholangitis with chills, fever, leukocytosis and jaundice. Antimitochondrial ATs are traditionally absent. Treatment is focused on the elimination of obstruction, including surgery with the imposition of external drainage and the installation of a permanent duct dilator.


    • Hypertrophic cirrhosis of the Andes
    • Cholangiolytic cirrhosis


    • K74.3 Primary biliary cirrhosis
    • K74.4 Secondary biliary cirrhosis


Cystinosis is a hereditary disease caused by a violation of the transport of cystine from lysosomes with the deposition of its crystals in the reticular cells of the bone marrow, liver, spleen and lymphatic system, as well as in the cells of the cornea and conjunctiva. Frequency – 1:100000 (in England and France – up to 1:25000). There are 3 clinical forms (all p, 219800 – a form that manifests itself in early childhood, 219900 – manifests itself in adolescence [late juvenile form], 219750 – the form manifests itself in adults, there is no damage to kidney function). Early nephropathic cystinosis (* 219800 , 17ql3, CTNS gene, p)

    • clinical picture. Deposition of cystine crystals in the cornea and conjunctiva, photophobia, corneal erosion, retinopathy, nephropathy, renal failure, hypothyroidism, ovarian hypofunction, endocrine and exocrine pancreatic insufficiency, growth retardation, memory disorders, myopathy, general muscle weakness, rickets-like changes, atrophy brain on CT; early onset, intermittent course.
    • Laboratory findings: Cystine crystals in leukocytes of blood products and bone marrow, cystine deposition in pancreatic islet cells, aorta, atrophic ovaries, and brain.

Late nephropathic cystinosis (#219900, Cystinosine Ralle, p) differs from the early variant in normal growth and onset in adolescence. Cystinosis benign in adults (#219750, Cystinosine Ralle, p) – onset in adulthood, absence of aminoaciduria and renal tubular dysfunction. Treatment:. The constant use of cysteamine slows down the damage to the kidneys and other organs by cystine. Kidney transplantation is relatively effective because does not eliminate the cause, degeneration of internal organs continues due to the accumulation of cystine.


    • Abdergalden-Fanconi syndrome
    • Lignac syndrome
    • Lignac-Fanconi syndrome
    • Abdergalden-Kaufmann-Lignac syndrome
    • Diabetes glycophosphamine


    • E72.0 Violations of transport of amino acids. cystinosis
    • Tubulo-interstitial kidney damage in cystinosis (E72.0+) MSH
    • 219800 Early nephropathic cystinosis
    • 219900 Late nephropathic cystinosis
    • 219750 Adult cystinosis, benign

Literature. Town M et al: A novel gene encoding an integral membrane protein is mutated in nephronopatic cystinosis, Nature Genetics 18(4), 319-324, 1998

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