Portal hypertension in a neonate with congenital hepatic fibrosis

Fayez K Ghishan, S. Nau, M. K. Younoszai

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

The term 'congenital hepatic fibrosis' (CHF) was initially introduced to describe fibrosis of the liver, an entity distinct from cirrhosis. Since then about 100 cases have been reported. CHF may be accompanied by cystic renal disease (in approximately 50%) and by pulmonary emphysema (one case). The hepatic lesion in CHF is asymptomatic until the age of 5 yr or older, when portal hypertension leads to gastrointestinal hemorrhage. In the newborn period CHF is usually found at autopsy in patients who die with uremia secondary to infantile polycystic disease. We report the first newborn infant with CHF to present with multiple congenital anomalies and refractory ascites secondary to portal hypertension. Contrary to what has been reported in the literature of the appearance of the hepatic complications in older children, CHF may present in newborns, with hepatic complications. This is the first report of fatal CHF secondary to hepatic complications in the newborn period.

Original languageEnglish (US)
Pages (from-to)243-244
Number of pages2
JournalSouthern Medical Journal
Volume74
Issue number2
StatePublished - 1981
Externally publishedYes

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Portal Hypertension
Newborn Infant
Liver
Cystic Kidney Diseases
Pulmonary Emphysema
Gastrointestinal Hemorrhage
Uremia
Congenital Hepatic Fibrosis
Ascites
Liver Cirrhosis
Autopsy
Fibrosis

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Portal hypertension in a neonate with congenital hepatic fibrosis. / Ghishan, Fayez K; Nau, S.; Younoszai, M. K.

In: Southern Medical Journal, Vol. 74, No. 2, 1981, p. 243-244.

Research output: Contribution to journalArticle

Ghishan, Fayez K ; Nau, S. ; Younoszai, M. K. / Portal hypertension in a neonate with congenital hepatic fibrosis. In: Southern Medical Journal. 1981 ; Vol. 74, No. 2. pp. 243-244.
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