Wilson's Disease in Two Siblings-One with Fatal Outcome
| Autor: | Gunnar Westgaard, Inge Romslo, Ove Lange, Erling Sagen, Jonathan Bland |
|---|---|
| Rok vydání: | 2009 |
| Předmět: |
Male
medicine.medical_specialty Pathology Adolescent Urinary system Urine Disease Kidney Gastroenterology Excretion Hepatolenticular Degeneration Internal medicine Internal Medicine Humans Medicine Child business.industry Penicillamine Brain medicine.disease Wilson's disease medicine.anatomical_structure Liver Abnormal Liver Function Test Female Autopsy business Copper medicine.drug |
| Zdroj: | Acta Medica Scandinavica. 219:331-335 |
| ISSN: | 0001-6101 |
| DOI: | 10.1111/j.0954-6820.1986.tb03320.x |
| Popis: | The clinical and biochemical findings in two siblings with Wilson's disease are described. One of them, an 11-year-old girl, developed acute liver failure terminating in death within a few weeks. Prior to her terminal illness she had been in good health without symptoms suggestive of Wilson's disease. Copper contents of urine, liver, kidney and brain were 20–100 times above the upper normal limits. The liver showed extensive micronodular cirrhosis with nonbile pigment deposits. Her 15-year-old brother had abnormal liver function tests with urinary copper excretion 20 times above the upper normal limit. Treatment with penicillamine was started. Following a short period of deterioration his condition has steadily improved. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
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