Recent Progress in Hepatic Involvement in Shwachman-Diamond Syndrome / 罕见病研究
JOURNAL OF RARE DISEASES
;
(4): 347-351, 2022.
Artigo
em Inglês
| WPRIM
| ID: wpr-1005026
ABSTRACT
Shwachman-Diamond syndrome is a multisystem autosomal recessive disorder characterized by pancreatic and bone marrow abnormalities with frequent liver involvement.Patients with SDS display aminotransaminase elevation and hepatomegaly in their early childhood. For most of the patients, the syptoms tend to improve as they grow. However, a number of the children with progress into cirrhosis even liver failure, and the prognosis is poor.This paper summarizes advances in the epidemiology, pathogenesis, clinical manifestations, and diagnosis and treatment of hepatopathy in Shwachman-Diamond syndrome.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Idioma:
Inglês
Revista:
JOURNAL OF RARE DISEASES
Ano de publicação:
2022
Tipo de documento:
Artigo
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