Chapter 14: Liver the porphyrias Flashcards
A patient presents with colicky abdominal pain and neuropsychiatric symptoms. Porphobilinogen is elevated in the serum.
Acute intermittent porphyria.
Autosomal dominant mutation in porphobilinogen deaminase, a step in heme synthesis.
A middle aged patient presents with cutaneous photosensitivity and liver disease with hepatic iron overload.
Porphyria cutanea tarda - autosomal dominant.
Uroporphyrinogen decarboxylase activity in the liver is deficient.
A patient dies of heart failure. At autopsy, the liver is shrunken and has an accentuated lobular pattern with alternating light and dark areas.
Fibrous strands envelop terminal venules, septa radiate from centrilobular zones.
Chronic passive congestion.
Nutmeg liver.
Acute occlusion of the intrahepatic branches of the portal vein produces what finding?
Zahn infarct - dark red triangular area with its base on the surface of the liver. Actually not an infarct, just congestion.
What bacteria cause pyogenic liver abscesses?
Staphylococci, streptococci, and gram-negative enterobacteria.
What causes pylephlebitic abscesses?
Intraabdominal suppuration - peritonitis, diverticulitis - with organisms transmitted to the liver in portal blood.
What causes cholangitic abscesses?
Biliary obstruction from any cause - ascending cholangitis. Biliary dissemination of organisms.
A homosexual man presents with fever, RUQ, weight loss, and RUQ pain. Liver abscesses are seen on imaging. The following biopsy is taken.
Amebiasis - protozoan.
Other protozoa: Malaria (Kupffer cell hypertrophy and hyperplasia)
Visceral leishmaniasis: Kala-azar. Hyperplasia of mononuclear phagocytes in the liver. Donovan bodies in Kupffer cells.
A patient has Weil syndrome. What is the infectious agent?
Leptospira spirochetes - zoonosis. Focal necrosis, enlarged Kupffer cells, centrilobular cholestasis.
What are some Helminths that infect the liver?
Schistosomiasis
Ascariasis: Duodenum -> biliary tree -> biliary colic.
Liver flukes: Clonorchis sinensis and Fasciola hepatica. Associated with cholangiocarcinoma
Echinococcosis: Cystic hydatid disease: Zoonosis. Expands slowly.
A neonate presents with cholestasis and jaundice. A biopsy of the liver is taken and shows multinucleated hepatocytes.
Neonatal hepatitis.
Characteristic lesion - giant cell transformation of hepatocytes. Appear detached from liver plate, pale, may contain up to 40 nuclie.
A neonate is found with inflammatory obliteration of the lumen of all or part of the biliary tree outside the liver.
No calculi, tumor, or rupture.
Extrahepatic biliary atresia.
Other organ abnormalities in 20% of cases.
Liver transplant.
A neonate is found to have few bile ducts within the liver.
Intrahepatic biliary atresia.
Alagille syndrome: Syndromic bile duct paucity. Autosomal dominant - defects of the heart, eye, skeleton, kidneys, CNS. Notch.
