α1-Antitrypsin deficiency presenting with ascites and cirrhosis in the neonatal period

Fayez K Ghishan, George F. Gray, Harry L. Greene

Research output: Contribution to journalArticle

26 Citations (Scopus)

Abstract

We report a 2-wk-old baby with α1-antitrypsin deficiency of Pizz phenotype, who presented with massive ascites and hepatomegaly. Liver biopsy disclosed active established cirrhosis. Unlike all other reported newborns with Pizz type, the patient had no evidence of neonatal cholestatic jaundice. The presentation of Pizz with established liver cirrhosis at such an early age not only adds another cause of cryptogenic cirrhosis in the neonatal period, but also points to a liver insult in some patients with Pizz, which is already determined in utero.

Original languageEnglish (US)
Pages (from-to)435-438
Number of pages4
JournalGastroenterology
Volume85
Issue number2
StatePublished - 1983
Externally publishedYes

Fingerprint

Ascites
Fibrosis
Neonatal Jaundice
Hepatomegaly
Obstructive Jaundice
Liver
Liver Cirrhosis
Newborn Infant
Phenotype
Biopsy
Cryptogenic Cirrhosis

ASJC Scopus subject areas

  • Gastroenterology

Cite this

α1-Antitrypsin deficiency presenting with ascites and cirrhosis in the neonatal period. / Ghishan, Fayez K; Gray, George F.; Greene, Harry L.

In: Gastroenterology, Vol. 85, No. 2, 1983, p. 435-438.

Research output: Contribution to journalArticle

Ghishan, Fayez K ; Gray, George F. ; Greene, Harry L. / α1-Antitrypsin deficiency presenting with ascites and cirrhosis in the neonatal period. In: Gastroenterology. 1983 ; Vol. 85, No. 2. pp. 435-438.
@article{1e09891fb19e41c089279c98de520558,
title = "α1-Antitrypsin deficiency presenting with ascites and cirrhosis in the neonatal period",
abstract = "We report a 2-wk-old baby with α1-antitrypsin deficiency of Pizz phenotype, who presented with massive ascites and hepatomegaly. Liver biopsy disclosed active established cirrhosis. Unlike all other reported newborns with Pizz type, the patient had no evidence of neonatal cholestatic jaundice. The presentation of Pizz with established liver cirrhosis at such an early age not only adds another cause of cryptogenic cirrhosis in the neonatal period, but also points to a liver insult in some patients with Pizz, which is already determined in utero.",
author = "Ghishan, {Fayez K} and Gray, {George F.} and Greene, {Harry L.}",
year = "1983",
language = "English (US)",
volume = "85",
pages = "435--438",
journal = "Gastroenterology",
issn = "0016-5085",
publisher = "W.B. Saunders Ltd",
number = "2",

}

TY - JOUR

T1 - α1-Antitrypsin deficiency presenting with ascites and cirrhosis in the neonatal period

AU - Ghishan, Fayez K

AU - Gray, George F.

AU - Greene, Harry L.

PY - 1983

Y1 - 1983

N2 - We report a 2-wk-old baby with α1-antitrypsin deficiency of Pizz phenotype, who presented with massive ascites and hepatomegaly. Liver biopsy disclosed active established cirrhosis. Unlike all other reported newborns with Pizz type, the patient had no evidence of neonatal cholestatic jaundice. The presentation of Pizz with established liver cirrhosis at such an early age not only adds another cause of cryptogenic cirrhosis in the neonatal period, but also points to a liver insult in some patients with Pizz, which is already determined in utero.

AB - We report a 2-wk-old baby with α1-antitrypsin deficiency of Pizz phenotype, who presented with massive ascites and hepatomegaly. Liver biopsy disclosed active established cirrhosis. Unlike all other reported newborns with Pizz type, the patient had no evidence of neonatal cholestatic jaundice. The presentation of Pizz with established liver cirrhosis at such an early age not only adds another cause of cryptogenic cirrhosis in the neonatal period, but also points to a liver insult in some patients with Pizz, which is already determined in utero.

UR - http://www.scopus.com/inward/record.url?scp=0020580288&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0020580288&partnerID=8YFLogxK

M3 - Article

C2 - 6602727

AN - SCOPUS:0020580288

VL - 85

SP - 435

EP - 438

JO - Gastroenterology

JF - Gastroenterology

SN - 0016-5085

IS - 2

ER -