Arteriohepatic Dysplasia. I. Pitfalls in Diagnosis and Management
โ Scribed by James Markowitz; Fredric Daum; Ellen I. Kahn; Keith M. Schneider; Henry B. So; R. Peter Altman; Harvey W. Aiges; Garth Alperstein; Mervin Silverberg
- Publisher
- John Wiley and Sons
- Year
- 2007
- Tongue
- English
- Weight
- 379 KB
- Volume
- 3
- Category
- Article
- ISSN
- 0270-9139
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โฆ Synopsis
Differentiating intrahepatic cholestasis from extrahepatic biliary tract obstruction may be difficult. Four patients with intraoperative cholangiographic evidence of extrahepatic ductal atresia who underwent hepatoportoenterostomy are described. All were ultimately shown to have arteriohepatic dysplasia with hypoplastic but patent extrahepatic ductal systems. The difficulty in establishing an accurate diagnosis, hazards associated with hepatoportoenterostomy, and suggestions for evaluation and management are discussed.
๐ SIMILAR VOLUMES
The diagnosis of arteriohepatic dysplasia may be difficult, particularly in very young patients with no family history, and the pathogenesis of the disorder remains obscure. It has been reported that the typical ultrastructural changes of cholestasis are scant in this condition and proposed that the
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