Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “HEPATOLENTICULAR DEGENERATION”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 73 records · Page 4Linked to original sources
PubMedDate not supplied

[Relation between serum immunoglobulin levels and anticerebral antibodies, detected by the Coon method, in hepatolenticular degeneration].

Antibodies to brain tissue were determined by indirect immunofluorescence of Coons in 23 patients with hepatolenticular degeneration. In 14 patients the levels of immunoglobulins A, G and M were determined as well. Control investigations were done in 37 donors. Positive results of Coons reaction with bright fluorescence of nervous tissue antigens were obtained in patients with increased serum immunoglobulin levels. Qualitative differences were obtained also in the reaction of Coons with sera from various patients. These data evidence that immunoglobulin F (ab)2 fragments as well as Fc fragments participate in the immunofluorescent reaction which calls for caution in the interpretation of the results of this method.

Adolescent↗
PubMed1978

Heart involvement in hepatolenticular degeneration.

The case of a 10-year-old boy with a mild abdominal form of hepatolenticular degeneration, showing clinical, laboratory and histological manifestations of heart involvement is reported. The microscopical examination of myocardial fragments collected by intracavitary puncture biopsy, using hematoxylin-eosine and the histochemical method with rubeanic acid, showed a medium degree of myocardial damage and the presence of copper deposition in the tissue. Copper concentration on a myocardial biopsy fragment was almost 10 times the normal heart content. Comments are made on the relationship of the clinical, laboratory and pathological findings with the degree of copper storage on the myocardium.

Cardiomyopathies↗
PubMed1987

[Hepatolenticular degeneration. Apropos of 102 cases].

The author reports the clinical and laboratory findings in 102 patients of hepatolenticular degeneration (HLD) followed up in the Department of Neurology, University of São Paulo Medical School, since 1946. The problem of the early diagnosis of the relatives is analysed, the pathology of Wilson's disease is reviewed, and the relationship of HLD with other hepatocerebral diseases is examined. Etiopathogenesis is discussed according to current researches, the role of the decreased biliary copper excretion being emphasized. The results of treatment with D-penicillamine in 84 cases are commented.

Female↗
PubMed1983

Hepatolenticular degeneration: histological study of conjunctiva and observation of chalazii during D-penicillamine treatment.

Three patients with hepatolenticular degeneration (Wilson's disease) are described. Bilateral blepharoconjunctivitis and Kayser-Fleischer ring was observed in all 3 patients. In 2 patients, long-lasting, bilateral chalazii appeared during chronic treatment with D-penicillamine. Histochemical and ultrastructural studies of pre-treatment conjunctival biopsies showed no copper storage in the tissue thus supporting the hypothesis that copper storage in Descemet's membrane derives from the anterior chamber.

Adolescent↗
PubMed1997

[Clinical study of integrated traditional and Western medicine therapy on hepatolenticular degeneration].

OBJECTIVE: To investigate the curative effect of integrated traditional and western medicine (TCM-WM) therapy on hepatolenticular degeneration (HLD). METHODS: Eighty patients with HLD were divided randomly into two groups (TCM-WM group and WM group), TCM-WM group (40 cases) were given orally dimercaptosuccinic acid (DMSA) and Gandou ([symbol: see text]) decoction for 1 month. The efficacy was compared with that of 40 cases treated with DMSA as the control. The changes of urinary trace and macro-elements before and after treatment were observed. RESULTS: The total effective rate of TCM-WM group was significantly higher than that of WM group (P < 0.05), especially more patients with hepatic type responded well to treatment with TCM-WM than with only chelating agent DMSA. Urinary trace and macro-elements were all obviously elevated in patients of two groups after treatment (P < 0.01). CONCLUSION: TCM-WM therapy appears extremely promising as new cupruretic method for treatment of HLD. It is particularly suitable for treating the patient with hepatic type.

Adolescent↗
PubMed2002

[Effect of gandou tablet I on neurological electrophysiology in patients of hepatolenticular degeneration].

OBJECTIVE: To observe the influence of Gandou Tablet I (GD I) on neurologic electrophysiology in patients of hepatolenticular degeneration (HLD). METHODS: Vigilance-controlled electroencephalogram (VEEG), electromyogram (EMG) and brain-stem auditory evoked potential (BAEP) in 32 cases of HLD were examined before GD I treatment, and reexamined after 4 weeks of treatment in those with abnormal neurologic electrophysiology. RESULTS: At the same time of clinical symptom improvement after treatment, the abnormal VEEG, EMG and BAEP also improved with the effective rate of 71.43%, 70% and 66.67% respectively, their constitutional ratio were different insignificantly, P > 0.05. The peak latency (PL) of III, V waves and interpeak latency (IPL) of I-III, III-V and I-V waves in patients before treatment were all greatly prolonged, compared with those in the healthy control group (P < 0.05 or 0.01), the IPL of III-V and I-V waves were markedly improved after treatment. CONCLUSION: Neurologic electrophysiologic examination was valuable for complementary diagnosis and therapeutic effect evaluation of HLD. GD I could improve the abnormal neurologic electrophysiologic parameters.

Adolescent↗
PubMed1998

[Study on therapeutical effect of treating hepatolenticular degeneration by the HLD-Tablet I].

OBJECTIVE: To investigate the efficacy of HLD-Tablet I in treating hepatolenticular degeneration (HLD) and its effect on urinary trace element in 24 hours. METHODS: After 4 weeks of treatment on 34 cases of HLD by oral taken HLD-Tablet I, the clinical practice was closely observed on the patients' change of symptoms, signs and daily life pattern, and measured the level of urinary trace element in 24 hours before the therapy and after that every week. RESULTS: The total effective rate reached 70.59%, marked effective rate was 8.82%. The effect of treatment was better in the infantile and mild patient. After treatment, the daily output of urinary copper was obviously increased every week than that before (P < 0.01). It was highly negative linear correlated with the duration of treatment (r = -0.96, P < 0.05). But it was insignificantly different between any two weekly excretion of urinary copper per 24 hours after treatment by the HLD-Tablet I (P > 0.05), so its effect on urinary copper excretion was not decreased. CONCLUSION: The HLD-Tablet I could increase the output of urinary copper, hence it was effective in treating HLD without apparent toxic or side reaction.

Adolescent↗
PubMed1991

[Interaction of molecular forms of ceruloplasmin with specific membrane receptors from healthy persons and patients with hepatolenticular degeneration].

The ceruloplasmin (CP) interaction with the specific receptor localized on the erythrocyte membrane of healthy donors and of patients with hepatolenticular degeneration (HLD) was studied by using Scatchard analysis. It was found that in patients with the Wilson's disease the CP receptor is unaffected by the pathological process. The molecular mass of the erythrocyte receptor of CP is about 130 kDa. The CP binding to the specific receptor is followed by limited proteolysis of the latter. No CP binding to the receptor occurs in the presence of serine protease inhibitors. The CP-like protein previously detected in the sera of Wilson gene carriers can specifically interact with the CP receptor.

Ceruloplasmin↗