hepatic metabolic diseases
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Classification
disorders of carbohydrate metabolism
disorders of glycoprotein
disorders of glycolipids
endoplasmic reticulum storage diseases
disorders of amino acids metabolism
peroxsomal disorders
mitochondrial cytopathies
disorders of porphyrin metabolism
disorders of metals metabolism
disorders of bile acid metabolism
disorders of bilirubin metabolism
miscellaneous metabolic disorders of the liver
Lesional patterns
hepatitic pattern
cholestatic pattern
- alpha-1-antitrypsin deficiency
- Niemann-Pick diseases type C
ductopenic pattern
- alpha1-antitrypsin deficiency
- inborn errors of bile acid metabolism
- peroxisomal diseases (cerebrohepatorenal syndrome, Zellweger syndrome)
- coprostanic acidemia
- Byler disease
- cystic fibrosis
steatotic pattern
- inherited metabolic diseases
- Wolman disease
- cholesterol ester storage disease
- mitochondrial respiratory chain diseases
- fatty acid oxydation diseases
- pyruvate carboxylase deficiency
- pyruvate dehydrogenasedeficiency
- neonatal adrenoleukodystrophy
- infantile Refsum disease
- herediatry tyrosinemia
- Wilson disease
- organic acidurias
- urea cycle diseases
hepatic storage diseases
cirrhotic pattern (metabolic cirrhosis)
- hereidtary tyrosinemia
- galactosemia
- Wolman disease
- cerebrohepatorenal syndrome (Zellweger syndrome)
- glycogen storage disease type IV (GSD4)
- indian childhood cirhosis
- alpha-antitrypsin deficiency
- Wilson disease
- mitochondrial respiratory chain diseases
- neonatal adrenoleukodystrophy
- infantile Refsum disease
- familial cholestatic syndromes
- mucopolysaccharidoses
- sialidosis
- Gaucher disease
- Niemann-Pick diseases
- hemochromatosis
tumoral pattern
-
focal nodular hyperplasia
- glycogen storage type I (GSD1)
- adenoma
- glycogen storage type I (GSD1)
- hepatoblastoma
- glycogen storage type Ia (GSD1a)
- regenerative nodule and hepatocellular carcinoma
- tyrosinemia
- glycogen storage type I (GSD1)
- glycogen storage type Ia (GSD1a)
- glycogen storage type IV (GSD4)
- alpha-1-antitrypsin deficiency
- hemochromatosis
- galactosemia
- familial cholestatic syndromes
- Wilson disease
- porphyria
References
Jevon GP, Dimmick JE. Histopathologic approach to metabolic liver disease: Part 1. Pediatr Dev Pathol. 1998 May-Jun;1(3):179-99. PMID: 10463278
Jevon GP, Dimmick JE. Histopathologic approach to metabolic liver disease: Part 2. Pediatr Dev Pathol. 1998 Jul-Aug;1(4):261-9. PMID: 10463287