Wilson's disease (hepatolenticular degeneration) and pregnancy

JAMA ◽  
1966 ◽  
Vol 195 (11) ◽  
pp. 960-962 ◽  
Author(s):  
F. E. Dreifuss
Keyword(s):  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Hepatolenticular Degeneration

Wilson's Disease

1984 ◽  
Vol 5 (7) ◽  
pp. 217-222
Author(s):  
Caroline A. Riely
Keyword(s):  
Wilson’S Disease ◽  
Acute Phase Reactant ◽  
Wilson's Disease ◽  
Clinical Findings ◽  
The Body ◽  
Hepatolenticular Degeneration ◽  
Ferric Ions ◽  
Pediatric Age Group ◽  
Life Threatening ◽  
Response To Stress

Wilson's disease (hepatolenticular degeneration) is a relatively rare cause of illness in the pediatric age group. But, as a chronic life-threatening disease that is treatable, even "curable," its investigation should be thoroughly pursued by the pediatrician. The recognition of Wilson's disease provides the opportunity to prevent this illness in presymptomatic family members. Therefore, Wilson's disease should be included in the differential diagnosis of all forms of liver disease in pediatrics and appropriately excluded. The clinical findings that are compatible with a diagnosis of Wilson's disease are protean, and confirming or denying this diagnosis is often difficult. In order to appreciate these problems, it is necessary first to understand the normal physiology of copper in the body and the derangements in this homeostasis that characterize Wilson's disease (Fig 1). COPPER HOMEOSTASIS Normal Copper is absorbed from the diet in excess of the body's requirement for it. Many foods contain copper, but chocolate, nuts, mushrooms, liver, and shellfish are particularly rich sources. Once absorbed, copper is transported free in the blood to the liver. Here, a certain fraction is incorporated in an irreversible fashion into ceruloplasmin. This copper-containing protein separates in the α2-globulins, can be an acute-phase reactant, increasing in concentration in response to stress. Alternatively, in the presence of hepatic failure with decrerased protein synthesis, its concentration in blood decreases. Ceruloplasmin is an oxidative enzyme used in a variety of pathways, including the oxidation of ferrous to ferric ions.

Uric Acid in Two Patients with Wilson's Disease (Hepatolenticular Degeneration).

1954 ◽  
Vol 86 (3) ◽  
pp. 440-441 ◽  
Author(s):  
C. Bishop ◽  
W. T. Zimdahl ◽  
J. H. Talbott
Keyword(s):  
Uric Acid ◽  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Hepatolenticular Degeneration

Wilson's Disease: Liver Cirrhosis and Virus-like Particles

1968 ◽  
Vol 26 ◽  
pp. 18-19
Author(s):  
M. A. Hairstone ◽  
A. Modjtabai ◽  
J. Azerbeygui ◽  
T. Shamsa
Keyword(s):  
Electron Microscopy ◽  
Liver Cirrhosis ◽  
Wilson’S Disease ◽  
Light And Electron Microscopy ◽  
Copper Metabolism ◽  
Wilson's Disease ◽  
Hepatolenticular Degeneration ◽  
Thin Sections ◽  
Body Tissues ◽  
Lenticular Nucleus

Hepatolenticular degeneration (Wilson's disease) manifests itself in many ways the most obvious of which is a disorder of copper metabolism. Normally copper beyond a certain threshhold is transferred to ceruplasmin which may circulate in the plasma. In patients with Wilson's disease this transfer apparently does not take place. There follows an accumulation of the metal in body tissues most notably the lenticular nucleus and the liver.Needle biopsies taken of the liver of an 11 year old male with Wilson's disease was prepared for light and electron microscopy by conventional methods. Thin sections were examined with Elmiskop 1A.Histologically the liver alteration was that of cirrhosis in which the cells showed fatty and glycogenic degeneration, cytoplasmic coagulation, fibrosis, and pigment deposition. Electron microscopy confirmed and extended light microscopy.

Changes of Some Clotting Factors in Hepatolenticular Degeneration (Wilson’s Disease)

Digestion ◽  
1965 ◽  
Vol 104 (1-6) ◽  
pp. 325-334
Author(s):  
J. Procházka ◽  
Dagmar Hauftová
Keyword(s):  
Wilson’S Disease ◽  
Wilson's Disease ◽  
Hepatolenticular Degeneration ◽  
Clotting Factors
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