Wilson's disease: Current status

The American Journal of Medicine

Volumn 92, Issue 6, 1992, Pages 643-654

  Yarze, Joseph C ^a   Martin, Paul ^a   Muñoz, Santiago J ^a   Friedman, Lawrence S ^a  

^a THOMAS JEFFERSON UNIVERSITY   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CERULOPLASMIN; COPPER; PENICILLAMINE; PREDNISONE; PYRIDOXINE DERIVATIVE; TRIENTINE; ZINC;

BONE MARROW TOXICITY; CHROMOSOME 13; DIET THERAPY; DRUG HYPERSENSITIVITY; EYE DISEASE; GASTROINTESTINAL TOXICITY; HEPATOBILIARY DISEASE; HUMAN; LIVER TRANSPLANTATION; LUPUS ERYTHEMATOSUS NEPHRITIS; MENTAL DISEASE; NEUROLOGIC DISEASE; ORAL DRUG ADMINISTRATION; PATHOGENESIS; PREGNANCY; PRIORITY JOURNAL; REVIEW; RHEUMATIC DISEASE; SKIN TOXICITY; WILSON DISEASE;

ADMINISTRATION, ORAL; CERULOPLASMIN; COPPER; COPPER RADIOISOTOPES; DRUG MONITORING; GENETIC SCREENING; HEPATOLENTICULAR DEGENERATION; HUMAN; LIVER; LIVER TRANSPLANTATION; OPHTHALMOSCOPY; PENICILLAMINE; SULFATES; TRIETHYLENETETRAMINE; ZINC; ZINC SULFATE;

EID: 0026631625     PISSN: 00029343     EISSN: None     Source Type: Journal    
DOI: 10.1016/0002-9343(92)90783-8     Document Type: Review

References (167)

1. Progressive lenticular degeneration a familial nervous disease associated with cirrhosis of the liver
2. La Dégénérescence Hépato-lenticulaire
3. Genetic analysis of 30 families with Wilson's disease (hepatolenticular degeneration)
4. Wilson's disease
5. Wilson's disease in Scotland
6. Assignment of the gene for Wilson's disease to chromosome 13: linkage to the esterase D locus
7. Expression of the gene of ceruloplasmin in Konovalov-Wilson's disease
8. Molecular studies of ceruloplasmin deficiency in Wilson's disease
9. Wilson's disease presenting as chronic active hepatitis
10. A study of the caeruloplasmin concentrations found in 75 patients with Wilson's disease, their kinships and various control groups
11. A Clinical and biochemical study of hepatolenticular degeneration (Wilson's disease)
12. Mapping ceruloplasmin cDNA to human chromosome 3
13. Biliary secretion of copper in healthy man
14. 64Cu
15. Studies on the nature and excretion of biliary copper in man
16. Abnormalities of the physiology of copper in Wilson's disease. III. The excretion of copper
17. Defective biliary excretion of copper in Wilson's disease
18. Wilson's disease: identification of an abnormal copper-binding protein
19. Studies of cholecystokinin-stimulated biliary secretions reveal a high molecular weight copper-binding substance in normal subjects that is absent in patients with Wilson's disease
20. The nature of the Copper complexes in bile and their relationship to the absorption and excretion of copper in normal subjects and in Wilson's disease
21. Is Wilson's disease caused by a controller gene mutation resulting in perpetuation of the fetal mode of copper metabolism into childhood?
22. Lysosomal defect of hepatic copper excretion in Wilson's disease (hepatolenticular degeneration)
23. Long-term therapy of Wilson's disease
24. Clinical spectrum of Wilson's disease (hepatolenticular degeneration)
25. Changes in the distribution of hepatic copper in relation to progression of Wilson's disease (hepatolenticular degeneration)
26. Diagnosis of Wilson's disease presenting as fulminant hepatitis
27. Wilson's disease (hepatolenticular degeneration) of late adult onset
28. Wilson's disease
29. Wilson's disease: clinical and laboratory manifestations in 40 patients
30. Spontaneous bacterial peritonitis in Wilson's disease
31. Chronic hepatitis as a first manifestation of Wilson's disease
32. Wilson's disease presenting as chronic active hepatitis
33. Fulminant hepatitis: a presentation of Wilson's disease
34. Dangers of non-compliance in Wilson's disease
35. Acute intravascular hemolysis and acute liver failure associated as a first manifestation of Wilson's disease
36. Wilson's disease
37. Unmeasurable serum alkaline phosphatase activity in Wilson's disease associated with fulminant hepatic failure and hemolysis
38. Fulminant Wilsonian hepatitis
39. Hepatocellular carcinoma in Wilson's disease
40. 3H-thymidine into the liver DNA of rats stimulated by dimethylnitrosamine and diethylnitrosamine
41. Cholelithiasis and Wilson's disease
42. The pallial component in hepatolenticular degeneration
43. Wilson's disease: an analysis of the cranial computerized tomographic appearances found in 60 patients and the changes in response to treatment with chelating agents
44. Clinical assessment of 31 patients with Wilson's disease: correlations with structural changes on magnetic resonance imaging
45. The ophthalmologic manifestations of Wilson's disease
46. Wilson's disease: electron microscopic, x-ray energy spectroscopic and atomic absorption spectroscopic studies of corneal copper deposition and distribution
47. Kayser-Fleischer-like rings in patients without Wilson's disease
48. “Sunflower cataract” in Wilson's disease
49. Haemolytic anaemia in Wilson's disease
50. The mechanisms of hemolysis in Wilson's disease: study of a case and review of the literature
51. Hemolytic anemia in Wilson's disease: clinical findings and biochemical mechanisms
52. Wilson's disease and the Fanconi syndrome
53. Bicarbonate excretion in Wilson's disease (hepatolenticular degeneration)
54. Renal stones in Wilson's disease
55. Skeletal changes in Wilson's disease: a radiologic study
56. Osteoarticular changes and synovial biopy in Wilson's disease
57. Arthropathy of Wilson's disease: study of clinical and radiological features in 32 patients
58. Tissue copper levels in Chinese patients with Wilson's disease
59. The arthropathy of Wilson's disease: clinical and pathologic features
60. Degradation of articular cartilage by copper and hydrogen peroxide
61. Copper salt-dependent hydroxyl radical formation damage to proteins acting as antioxidants
62. Skin pigmentation in Wilson's disease
63. Azure lunulae
64. Hepatolenticular degeneration associated with acanthosis nigricans
65. The cardiomyopathy of Wilson's disease: myocardial alterations in nine cases
66. Cardiac Wilson's disease
67. Wilson's disease: 35 years experience
68. Undiagnosed Wilson's disease as a cause of unexplained miscarriage
69. Endocrine studies of the ovulatory disturbances in Wilson's disease (hepatolenticular degeneration)
70. Glucose intolerence in Wilson's disease: normalization after treatment with penicillamine
71. Hypoparathyroidism in Wilson's disease
72. The liver in Wilson's disease
73. Changes in hepatic structure in Wilson's disease
74. Hepatocellular changes in Wilson's disease: histochemical and electron microscopic studies
75. Mitochondrial and fatty changes in hepatocytes of patients with Wilson's disease
76. The peroxisomes of human hepatocytes
77. Orcein positive hepatocellular material in long-standing biliary diseases. I. Histochemical characteristics
78. Canine hepatic lysosomal copper protein: identification as metallothionein
79. The significance of variations of copper in liver disease
80. Histology of the liver in Wilson's disease
81. Ceruloplasmin in health and disease
82. Effect of estrogen on copper metabolism in Wilson's disease
83. Laboratory measures of copper metabolism in the differentiation of chronic active hepatitis and Wilson's disease in children
84. The concentration of copper and ceruloplasmin in maternal and infant plasma at delivery
85. Value for copper, iron and iron-binding capacity in the serum in kwashiorkor
86. Hereditary hypoceruloplasminemia
87. Wilson disease: clinical presentation, treatment, and survival
88. Direct measurement of serum non-ceruloplasmin copper in liver disease
89. Studies on copper metabolism. XIII. Hepatolenticular degeneration
90. Urinary copper excretion and hepatic copper concentrations in liver disease
91. Penicillamine-induced cupriuria in normal subjects and in patients with active liver disease
92. Value of urinary copper excretion after penicillamine challenge in the diagnosis of Wilson's disease
93. Liver-copper levels in liver disease: studies using neutron activation analysis
94. Copper and primary biliary cirrhosis
95. Hepatic copper accumulation in primary biliary cirrhosis
96. Liver copper levels in intrahepatic cholestasis of childhood
97. Increased hepatic copper content in Indian childhood cirrhosis
98. Hepatic copper overload and features of Indian childhood cirrhosis
99. Abnormalities of chemical tests for copper metabolism in chronic active liver disease: differentiation from Wilson's disease
100. Radiocopper studies in patients with Wilson's disease and their relatives
101. Radiocopper in diagnosing liver disease
102. The role of radiocopper in the diagnosis of Wilson disease
103. Studies on copper metabolism in demylelinating diseases of the central nervous system
104. The effects of BAL in hepatolenticular degeneration
105. The effect of BAL (2,3-dimercaptopropanol) on hepatolenticular degeneration (Wilson's disease)
106. Penicillamine: a new oral therapy for Wilson's disease
107. Prevention of Wilson's disease in asymptomatic patients
108. Treatment of hepatolenticular degeneration (Wilson's disease) in the asymptomatic stage
109. Presymptomatic Wilson's disease
110. Prevention of Wilson's disease in asymptomatic patients
111. Prevention of Wilson's disease: a long-term follow-up
112. Diagnosis and treatment of presymptomatic Wilson's disease
113. Penicillamine therapy in hepatolenticular degeneration
114. Wilson's disease a treatable liver disease in children
115. Liver copper concentration in Wilson's disease: effect of treatment with ‘anti-copper’ agents
116. Penicillamine may detoxify copper in Wilson's disease
117. Can Wilson's disease patients be decoppered
118. Studies on the sulfur-containing chelating agents
119. D-penicillamine induces rat hepatic metallothionein
120. The liver in Wilson's disease (hepatolenticular degeneration)
121. Copper metabolism, Wilson's disease and hepatic copper toxicosis
122. Wilson's disease
123. Disappearance of Kayser-Fleischer rings
124. Mechanism of inhibition of collagen cross-linking by penicillamine
125. Effect of penicillamine upon wound healing
126. Penicillamine and wound-healing in young guinea pigs
127. The management of penicillamine nephropathy in Wilson's disease: a new chelating agent
128. Triethylene tetramine dihydrochloride in Wilson's disease
129. Copper chelation in patients with Wilson's disease
130. Preparation of and clinical experiences with trien for treatment of Wilson's disease in absolute intolerance of D-penicillamine
131. Treatment of Wilson' disease with triethylene tetramine dihydrochloride
132. Treatment of Wilson's disease with trientine (triethylene tetramine) dihydrochloride
133. Assessment of the treatment of Wilson's disease with triethylene tetramine 2HCI (Trien 2HCI)
134. Treatment of Wilson's disease
135. The effect of dietary zinc on intestinal copper absorption
136. Regulation of intestinal metallothionein biosynthesis in rats by dietary zinc
137. In vivo and ex vivo effects of copper on rat liver metallothionein
138. Oral zinc sulphate as long-term treatment in Wilson's disease (hepatolenticular degeneration)
139. Three years of continuous oral zinc therapy in four patients with Wilson's disease
140. Oral zinc therapy for Wilson's disease
141. Oral zinc sulphate for Wilson's disease
142. Treatment of Wilson's disease with zinc. I. Oral zinc therapy regimens
143. Zinc therapy in Wilson's disease: observations in five patients
144. Zinc therapy of Wilson's disease two views
145. Excessive intake of zinc impairs immune responses
146. Zinc toxicity
147. Zinc-induced hypocalcemia and bone resorption in rats
148. A case of chronic zinc poisoning in calves fed zinc-contaminated roughage
149. Current concepts: the diagnosis of treatable Wilson's disease
150. Presymptomatic Wilson's disease: further questions and comments
151. Pregnancy in penicillamine-treated patients with Wilson's disease
152. The copper intrauterine device and its mode of action
153. Pregnancy in Wilson's disease
154. Portal hypertension in pregnancy
155. Bleeding esophageal varices in pregnancy
156. D-penicillamine treatment in pregnancy
157. Pregnancy in penicillamine-treated patients with Wilson's disease
158. Pregnancy and delivery in penicillamine treated patients with Wilson's disease
159. Neonatal abnormalities associated with D-penicillamine treatment during pregnancy
160. Congenital connectivetissue defect probably due to D-penicillamine treatment in pregnancy
161. Reversible cutis laxa due to maternal penicillamine treatment
162. Liver transplantation for Wilson's disease
163. Wilson's disease: clinical presentation and use of prognostic index
164. Perspectives on Wilson's disease
165. Fulminant Wilson's disease treated with postdilution hemofiltration and orthotopic liver transplantation
166. Hemofiltration with a high permeability membrane in the treatment of fulminant or end-stage Wilson's disease
167. Heterotopic liver transplantation for fulminant Wilson's disease