Isolated Polycystic Liver Disease (PCLD)

Isolated Polycystic Liver Disease(来自ICD-11)
别称:
Autosomal Dominant Polycystic Liver Disease
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Isolated Polycystic Liver Disease, also known as autosomal dominant polycystic liver disease, is related to polycystic liver disease 1 with or without kidney cysts and polycystic kidney disease 4, and has symptoms including hepatosplenomegaly An important gene associated with Isolated Polycystic Liver Disease is PRKCSH (Protein Kinase C Substrate 80K-H), and among its related pathways/superpathways are Metabolism of proteins and WNT Signaling. The drugs Pasireotide and Somatostatin have been mentioned in the context of this disorder. Affiliated tissues include liver and kidney, and related phenotypes are hepatomegaly and abdominal distention
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参考文献
MALACARDS
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Unknown
1-9/100000
17
209
1

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