Wilson's Disease.
نویسندگان
چکیده
Early diagnosis permits preventive therapy to preempt development of organ damage. In all diagnosed patients, both symptomatic and asymptomatic, pharmacologic therapy is lifelong, and maintenance treatment to prevent copper toxicity is mandatory. Patients with either fulminant hepatic failure or hepatic insufficiency unresponsive to medical therapy should be considered for orthotopic liver transplantation, which effectively cures Wilson's disease. Prognosis is excellent for compliant patients receiving pharmacologic therapy.
منابع مشابه
Hypoparathyroidism as a rare manifestation of Wilson,s disease: a case report
A 13 years old girl was admitted to the pediatric ward of hospital No 1 of Kerman university of medical sciences because of crampy pains, pins and needles sensation of the extremities and history of an episode of seizure. Her problem started one week prior to admission with pain in the leg muscles, flexion of the fingers followed by seizure. On physical examination, the prominent finding inclu...
متن کاملراشیتیسم مقاوم به درمان به عنوان اولین تظاهر بیماری ویلسون (یک مورد نادر)
Rickets by definition is a condition in which the bone mineralization is defective. Among the large cause of Rickets (Vitamin D deficiency, gastrointestinal disorders, acidosis, renal tubular abnormalities...), Wilson's disease is a relatively rare cause. There are few cases of Wilson's disease presenting first as rickets in the literature. Here we present an Afghan girl with Wilson's disea...
متن کاملWilson's Disease : A Case Report
Wilson's disease is a rare metabolic disorder involving copper metabolism may present with hepatic, neurological and psychiatric manifestations. We present a case of Wilson's disease with behavioral symptoms, which responded to risperidone.
متن کاملDiagnosis of Wilson's disease: an experience over three decades.
BACKGROUND Wilson's disease is a rare but treatable condition that often presents diagnostic dilemmas. These dilemmas have for the most part not been resolved by the identification and cloning of the Wilson's disease gene. AIMS To report our experience over three decades with patients with Wilson's disease in order to illustrate the diverse patterns of presentation and thereby broaden the app...
متن کاملHypothyroidism and dyshormonogenesis induced by D-penicillamine in children with Wilson's disease and healthy infants born to a mother with Wilson's disease.
Two siblings born to a mother with Wilson's disease, who was taking D-penicillamine, developed transient goitrous hypothyroidism. A prospective evaluation of 5 patients with Wilson's disease taking and not taking D-penicillamine for as long as 9.5 years showed subclinical hypothyroidism. D-penicillamine probably inhibited thyroperoxidase activity in utero in healthy infants and during childhood...
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ورودعنوان ژورنال:
- Current treatment options in gastroenterology
دوره 2 1 شماره
صفحات -
تاریخ انتشار 1999