Phosphorylase b kinase deficiency glycogenosis with cirrhosis of the liver

Amir F. Kagalwalla; Yasmeen A. Kagalwalla; Sulaiman Al Ajaji; Waldemer Gorka; M. Ashraf Ali

doi:10.1016/S0022-3476(95)70123-0

Phosphorylase b kinase deficiency glycogenosis with cirrhosis of the liver

Amir F. Kagalwalla, Yasmeen A. Kagalwalla, Sulaiman Al Ajaji, Waldemer Gorka, M. Ashraf Ali

Pediatrics

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19 Scopus citations

Abstract

We describe an Arab girl with complete absence of phosphorylase b kinase activity in the liver, symptomatic hypoglycemia, and persistently elevated serum aminotransferase values whose symptoms did not lessen with age; sequential liver biopsies showed progression to cirrhosis. Cirrhosis could not be ascribed to any other known cause. We conclude that type IX glycogenosis is not always associated with a benign outcome. (J PEDIATR 1995;127:602-5).

Original language	English (US)
Pages (from-to)	602-605
Number of pages	4
Journal	The Journal of pediatrics
Volume	127
Issue number	4
DOIs	https://doi.org/10.1016/S0022-3476(95)70123-0
State	Published - Oct 1995

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health

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title = "Phosphorylase b kinase deficiency glycogenosis with cirrhosis of the liver",

abstract = "We describe an Arab girl with complete absence of phosphorylase b kinase activity in the liver, symptomatic hypoglycemia, and persistently elevated serum aminotransferase values whose symptoms did not lessen with age; sequential liver biopsies showed progression to cirrhosis. Cirrhosis could not be ascribed to any other known cause. We conclude that type IX glycogenosis is not always associated with a benign outcome. (J PEDIATR 1995;127:602-5).",

author = "Kagalwalla, {Amir F.} and Kagalwalla, {Yasmeen A.} and {Al Ajaji}, Sulaiman and Waldemer Gorka and Ali, {M. Ashraf}",

year = "1995",

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T1 - Phosphorylase b kinase deficiency glycogenosis with cirrhosis of the liver

AU - Kagalwalla, Amir F.

AU - Kagalwalla, Yasmeen A.

AU - Al Ajaji, Sulaiman

AU - Gorka, Waldemer

AU - Ali, M. Ashraf

PY - 1995/10

Y1 - 1995/10

N2 - We describe an Arab girl with complete absence of phosphorylase b kinase activity in the liver, symptomatic hypoglycemia, and persistently elevated serum aminotransferase values whose symptoms did not lessen with age; sequential liver biopsies showed progression to cirrhosis. Cirrhosis could not be ascribed to any other known cause. We conclude that type IX glycogenosis is not always associated with a benign outcome. (J PEDIATR 1995;127:602-5).

AB - We describe an Arab girl with complete absence of phosphorylase b kinase activity in the liver, symptomatic hypoglycemia, and persistently elevated serum aminotransferase values whose symptoms did not lessen with age; sequential liver biopsies showed progression to cirrhosis. Cirrhosis could not be ascribed to any other known cause. We conclude that type IX glycogenosis is not always associated with a benign outcome. (J PEDIATR 1995;127:602-5).

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Phosphorylase b kinase deficiency glycogenosis with cirrhosis of the liver

Abstract

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