A case report: Alagille syndrome

A case report: Alagille syndrome

Alagille syndrome consists of 5 major features comprising paucity of interlobular bile ducts, characteristic facies, posterior embryotoxon, vertebral defects and peripheral pulmonic stenosis. The female patient in this report met 4 of the 5 major features except ocular abnormality. The first clinica...

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Veröffentlicht in:Journal of the Medical Association of Thailand 2000-04, Vol.83 (4), p.451-454
Hauptverfasser: Ukarapol, N, Wongsawasdi, L, Sittiwangkul, R
Format: Artikel
Sprache:eng
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Alagille Syndrome - diagnosis
Alagille Syndrome - therapy
Female
Humans
Infant
Prognosis
Risk Assessment
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container_title Journal of the Medical Association of Thailand
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creator Ukarapol, N
Wongsawasdi, L
Sittiwangkul, R
description Alagille syndrome consists of 5 major features comprising paucity of interlobular bile ducts, characteristic facies, posterior embryotoxon, vertebral defects and peripheral pulmonic stenosis. The female patient in this report met 4 of the 5 major features except ocular abnormality. The first clinical presentations were prolonged jaundice and generalized ecchymoses. She was treated by plasma replacement and vitamin supplement, particularly vitamin K1, which produced clinical improvement. This report also reviews the literature of Alagille syndrome.
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subjects Alagille Syndrome - diagnosis
Alagille Syndrome - therapy
Female
Humans
Infant
Prognosis
Risk Assessment
title A case report: Alagille syndrome
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