Idiopathic neonatal hepatitis

Wilson s disease, haemochromatosis, galactosaemia, glycogenosis type IV, ai-antitrypsin deficiency, tyrosin-aemia, idiopathic neonatal hepatitis, Niemann-Pick disease, Gaucher s disease, fructose intolerance, defective urea cycle, etc. 

Tajiri, H., Tanaka, T., Sawada, A., Etani, Y., Kozaiwa, K., Mushiake, S., Mishiro, S. Three cases TT virus infection and idiopathic neonatal hepatitis. Intervirology 2001 44 364-369... 

A drastic reduction in numbers of interlobular bile ducts is termed ductopenia (or paucity of bile ducts). Ductopenia is considered to be present if the ratio of interlobular ducts to the number of portal tracts is <0.5 (normal value 0.9-1.8), i.e. in a sufficiently large biopsy specimen, no more than half of the portal fields should be without a bile duct. Ductopenia can appear as an isolated defect (non-syndromic) or in combination with other extrahepatic (syndromic) anomalies. Non-syndromic paucity constantly exhibits bile-duct dilatation with blunting of microvilli. Idiopathic neonatal hepatitis sometimes overlaps with non-syndromatic ductopenia. Children with ductopenia may survive into adulthood. (504) (s. fig. 32.16)... 

Defective Bile Acid Synthesis. Specific defects in bile acid synthesis have long been postulated. Two inborn errors of bile acid synthesis, both associated with idiopathic neonatal hepatitis, A -3-oxosteroid 5-P-reductase deficiency and 3-p-hydroxy-dehydrogenase isomerase deficiency, have been described. A third disorder associated with defective bile... 

Neonatal Jaundice 134 Biliary Atresia 135 Idiopathic Neonatal Hepatitis 136 Cystic Changes in the Liver and Biliary Tree 137... 

Idiopathic neonatal hepatitis is an inflammation of the liver parenchyma responsible for most cases of intrahepatic cholestasis, and accounts for at least 25%-40% of patients with neonatal cholestasis. A multitude of etiologies can be the cause (Gubernick et al. 2000). 

The clinical presentation of a patient with idiopathic neonatal hepatitis can be similar to BA however, these patients are more likely premature or between 1-4 weeks of age than those with BA. 

Histologically there is a transformation of hepa-tocytes into giant cells, with lobular disarrangement. Although the bile ducts appear normal, the differentiation among idiopathic neonatal hepatitis and BA may be difficult. 

Scintigraphy will show a decreased uptake of 99m Tc-IDA with excretion into the duodenum. Nevertheless, patients with BA for more than 3 months may depict a scintigraphic pattern similar to those with idiopathic neonatal hepatitis. 

Seizures occur more infrequently in horses than in dogs and cats. Seizures are seen in adult horses from brain trauma, bacterial meningitis, viral encephalitis and, rarely, hepatic encephalopathy or vascular accidents. Convulsions are seen in young neonatal foals with NMS as a result of brain hypoxia and in Arabian foals aged 3-9 months (idiopathic Arabian epilepsy). Anticonvulsant therapy is used to prevent the spread of the seizure focus, increase (raise) the seizure threshold and decrease the electrical excitement of abnormal... 

About 75% of cases of hepatitis in the neonate are idiopathic giant cell hepatitis, a disorder of unknown etiology characterized by cholestatic jaundice. There is a familial trend that may reflect an autosomal recessive inheritance. Jaundice appears withm the first 2 weeks. The child initially appears well and gains weight. The liver and spleen then become enlarged and stools become pale. Serum aminotransferases are usually >400 U/L the prothrombin time is prolonged. Liver biopsy reveals characteristic giant cells with hepatocyte... 

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