Results 11 to 20 of about 177,432 (192)
Hepatolenticular degeneration-induced hepatic dysfunction with extremely atypical clinical manifestations: a Case Report [PDF]
Hepatolenticular Degeneration (HLD) is a rare condition caused by a genetic copper metabolism disorder and a basal ganglia-dominated degenerative brain disease. Its characteristic clinical features include progressive extrapyramidal symptoms, psychiatric
Zhuang Tao, Meixia Wang
exaly +6 more sources
Hepatolenticular degeneration [PDF]
Hepatolenticular degeneration or Wilson's disease is the hereditary disease, caused by derangement of copper metabolism and its precipitation in brain and internal.
O. Yu. Koryagina +2 more
doaj +3 more sources
Hepatolenticular degeneration: diagnostic difficulties (practical experience) [PDF]
The article deals with severe hereditary disease - hepatolenticular degeneration (Wilson - Konovalov disease). Hepatolenticular degeneration is a monogenic autosomal recessive genetic disorder.
A. V. Ovchinnikov, V. V. Shprakh
doaj +3 more sources
Diagnosis and treatment of hepatolenticular degeneration
Hepatolenticular degeneration (Wilson-Konovalov disease or hepatolenticular disease) is a rare hereditary disease caused by impaired copper metabolism and copper accumulation in the liver and, subsequently, in other organs.
V. V. Skvortsov, O. A. Leshina
doaj +2 more sources
Clinical case of fulminant hepatitis in a child with hepatolenticular degeneration
The article presents a detailed analysis of the diagnostic search for Wilson’s disease in childhood. Polymorphism of clinical symptoms of the disease has led to various descriptions of this pathology and its manifestations. Despite more than a century of
I.M. Nesina +4 more
doaj +2 more sources
Hepatolenticular degeneration: report of three cases
Hepatolenticular degeneration (Wilson disease) is a rare inherited disease that usually affects the liver, but may present in different forms and have multiple systemic complications. Diagnosis requires a high index of suspicion, mainly in young patients,
Castaño, Orlando +3 more
doaj +2 more sources
Clinical and gene variation characteristic of 75 cases of hepatolenticular degeneration in children [PDF]
Objective To investigate the clinical characteristics of the hepatolenticular degeneration in children, and to clarify the significance of gene diagnosis in children with hepatolenticular degeneration.
ZHANG Simin, WANG Wei, MA Mingsheng, QIU Zhengqing
doaj +2 more sources
Clinical observation and risk assessment after splenectomy in hepatolenticular degeneration patients associated with hypersplenism [PDF]
BackgroundBoth hepatolenticular degeneration (HLD) and viral hepatitis B (HBV) can cause hypersplenism, but whether splenectomy is needed or can be performed in HLD patients associated with hypersplenism is still controversial.
Wanzong Zhang +9 more
doaj +2 more sources
The metabolic balances of sulfur in two cases of hepatolenticular degeneration were studied. A positive balance was found in both cases; in one patient submitted to mixed diet, the average was significantly higher than the normal mean. The administration
Horacio M. Canelas +2 more
doaj +2 more sources
Two cases of Wilson–Konovalov disease [PDF]
Wilson–Konovalov disease (hepatolenticular degeneration) is a monogenic autosomal recessive genetic disorder. For its pathogenesis, there is the underlying genetic disorder of copper metabolism in which copper accumulates in excessive amounts in target ...
Alla Anatolyevna Strutsenko +4 more
doaj +2 more sources

