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Publikováno v:
The Turkish journal of pediatrics. 43(3)
Here we report two unusual patients with Gaucher disease type I. Both girls admitted with hepatosplenomegaly, growth retardation, and anemia at four and 2.5 years of age, and Gaucher cells were seen on bone marrow aspirates. Thalassemic face was firs
Publikováno v:
Indian pediatrics. 38(9)
Publikováno v:
The Turkish journal of pediatrics. 42(3)
Wilson disease is an autosomal recessively inherited disease of copper metabolism and is characterized by liver and central nervous system dysfunction. The heterozygote carrier state rate is about one in 90 persons and the incidence of the disease is
Publikováno v:
The Turkish journal of pediatrics. 41(1)
Wilson's disease, an inborn defect of copper metabolism, is a fatal disease unless specific treatment is given. Hepatic presentation mimics almost all kinds of liver disease and the diagnosis is sometimes problematic. The diagnosis is based on clinic
Publikováno v:
The Turkish journal of pediatrics. 41(1)
The beneficial effect of ursodeoxycholic add have been documented in adults but experience with this agent is limited in the pediatric population. The objective of this study was to evaluate ursodeoxycholic acid treatment in children with cholestatic
Publikováno v:
Indian pediatrics. 37(1)
To evaluate clinical, laboratory findings, treatment and long-term follow-up of children with Wilson's disease with hepatic presentation.Retrospective study with a median follow-up period of 9 years.University medical center.Thirty-four children with
Publikováno v:
The Turkish journal of pediatrics. 42(1)
Interferon-alpha therapy has been proven efficient in chronic hepatitis C infection. Although it has been used as a standard therapy in adults, there are limited data on benefits of interferon treatment in children. We conducted a study of recombinan
Publikováno v:
The Turkish journal of pediatrics. 42(1)
Variceal bleeding due to portal hypertension is a frequent and severe complication of cirrhosis in children as in adults. The prophylactic approach is important for these high mortality bleedings, both for the first and for recurrent attacks. Varicea
A cause of fatty liver: neutral lipid storage disease with ichthyosis--electron microscopic findings
Publikováno v:
Digestive diseases and sciences. 44(11)