Wilson Hastalığı Ve Klinik Spektrumu

Year 1987, Volume: 40 Issue: 4 , 329 - 338 , 31.12.1987

Cihan Yurdaydın

https://izlik.org/JA98GR65YX

Abstract

Hastanemiz Gastroenteroloji kliniğinde son 15 yılda ve Nöroloji kliniğinde son on yılda Wilson hastalığı tanısı almış toplam on olgu retrospektif olarak incelendi. On olgunun yarsının hepatik, yarısının nörolojik semptomatolojiyle başladığı, sonuçta ise beş olguda kombine karaciğer ve sinir Sistemi hastalığı, üç hastada yalnız sinir sistemi, iki hastada ise karaciğer hastalığı geliştiği gözlenmiştir. Muntazam kontrollere gelen yedi olgunun incelenmesinde, üç hastanın penisillamine tedavisine iyi cevap verdiği gözlendi ve bunlardan ikinsinde sadece sinir sistemi tutulumu vardı. Özellikle karaciğer tutulumu olan olgularda tanı ileri strüktürel hasar geliştikten sonra
konduğu için, tedaviden iyi sonuç alınamamıştır. Wilson hastalığında ideal olan, fonksiyonel bozukluk gelişmeden tedaviye başlamaktır. Bu yönden Wilson hastalarının asemptomatik homozigot kradeşlerinin ortaya çıkartılması gereği vurgulanmıştır. Yine 30 yaşın altındaki HBsAg (—) kronik hepatit olgularının etyolojik tanısında Wilson hastalığı göz önünde tutulmalıdır.

Keywords

gastroenteroloji , wilson hastalığı , kronik hepatit

Wilson's Disease and Its Clinical Spectrum

https://izlik.org/JA98GR65YX

Abstract

This reporl retrospeclively describes 10 palients for whom the diagnosis of Wilson's disease were made in the last ten years in the departments of gastroenterology and neurology of our hospital. Half of the cases presented with hepatic and the other half with neurologic symptomatolgy. Eventually five cases developed mixed hepatic and brain disease, three cases developed brain and two cases hepatic disease, Of the seven patients with regular controls three patlents had a good response to penicillamine treatment and two of them had brain disease. Especially in cases of hepatic involvement the di- agnosis was made after severe hepatic damage and this cases yield no good responese İrom treatment. Al the clinical manifestations of Wilson's disease can be prevented if the disease is diagnosed before functional impairmenti OCCUrS. For this reason we believe that it is very important to select the asyptomatic homozygote sibling of index cases. Alsa in the evaluation of HBsAg (—) chronic hepatitis cases under 30 years old the possibility of Wilson's disease should always be checked.

Keywords

gasteronogy , neurology , diagnosis

References

• 1-Cartwright GE : Diagnosis of treatable Wilson's disease, N Eng J Med 298: 1347, 1978.
• 2-Dobyns WB, Goldstein NP, Gordon H : Clinical spectrum of Wilson's disease, Mayo Clin Proc 54 : 35, 1979.
• 3-Fleming CR ve ark. : Pigmented corneal rings in non-Wilsonian liver disease, Ann Intern Med 86 : 285, 1977.
• 4-Gibbs K ve Walshe JM: A study of the caeruloplasmin concentrations found in 75 patients with Wilson's disease, their kinships and various control groups, © J Med 48 : 447, 1979,
• 5-Hansotia P, Harris R, Kennedy J : EEG changes in Wilson's disease Electroenceph Clin Neurophysiol 27 : 523, 1969.
• 6-Hoogenraad TV, Van den Hamer CJA, Van Hatum J : Effective treatment of Wilson's disease with oral zinc sulphate : two case reports, Br Med J 289 : 273, 1984.
• 7-McCullough AJ ve ark. : Diagnosis of Wilson's disease presenting as fulminant hepatic failure, Gastroenterology 84 : 161, 1983.
• 8-Nazer H ve ark. : Wilson's disease : clinical presentation and use of prognostic index, Gut 27 : 1377, 1986.
• 9-Rakela J ve ark : Fulminant Wilson's disease treated with post-dilution hemofiltration and orthotopic liver transplantation, Gastroenterology 90 : 2004, 1986.
• 10-Roche-Sicot J ve Bönhamon JP : Acute intravascular hemolysis and acute liver İailure associated as a first manifestation of Wilson's disease, Ann Intern Med 86 : 301, 1977.
• 11-Shrlock S ; Disease of the liver and biliary system, Blackweli Scientific Publications, seventh edition, 1985, s 373.
• 12-Sternlieb I ve Scheinberg IH : Chronic hepatitis as a first manifestation Of Wilson's disease, Ann Intern Med 76 : 59, 1972.
• 13-Van Caillie-Bertrand M ve ark : Oral zinc sulphate for Wilson's disease, Arch Dis Child 60 : 656, 1985.
• 14-Van Caillie-Bertrand M ve ark : Wilson's disease : assessment of D-penicillemine treatment, Arch Dis Child 60 : 652, 1985.
• 15-Welshe JM : Treatment of Wilson's disease with trientine dihydrochloride, Lan-I cet i : 643, 1982.
• 16-Walshe JM : Treatment of Wilson's disease with zinc ,sulphate, Br Med J 289 : 558, 1984.
• 17-Walshe JM ve Dixon AK : Dangers of non-compliance in Wilson's disease, Lanceti: 845, 1986. ,
• 18-Wilkinson ML, Portmann B, Williams R : Wilson's disease and hepatocellular carcinoma : possible protective role of copper, Gut 24 : 767, 1983