Wilson Hastalıklı Çocuklarda Böbrek Fonksiyonlarının Değerlendirilmesi

Yıl 2017, Cilt: 15 Sayı: 1, 6 - 11, 01.04.2017

Yılmaz Tabel , Mukadder Ayşe Selimoğlu Fatma İlknur Varol Ahmet Taner Elmas Şükrü Güngör Hamza Karabiber

https://doi.org/10.4274/jcp.43760

Öz

Giriş: Bu çalışmada Wilson hastalığında WH böbrek fonksiyonlarının üriner N-Asetil-β-D-glukozaminidaz NAG ve NAG/kreatinin aktivite indeksi kullanılarak değerlendirilmesi amaçlanmıştır.Gereç ve Yöntem: WH tanısı alan 20 hasta çalışma grubu, benzer yaş ve cinsiyette olan 37 sağlıklı çocuk kontrol grubu olarak alındı. NAG düzeyleri mlU/L olarak hesaplanırken, NAG aktivite indeksi mlU/mg kreatinin olarak belirlendi.Bulgular: Toplam dört hasta %20 tanı anında asemptomatik iken; sırasıyla kronik hepatit, nörolojik WH, fulminan hepatit ve akut hepatit ise altı %30 , beş %25 , üç %15 ve iki %10 hastada saptandı. Çalışma anında toplam 13 hasta %65 trientin ve çinko tedavisi alırken, yedi %35 hasta ise D-penisilamin ve çinko tedavileri almaktaydı. Olguların 10’unda %50 proteinüri, birinde %5 glikozüri ve bir hastada %5 mikroskopik hematüri saptandı. Hasta grubunun idrar sodyumu ve kreatinini kontrol grubundan anlamlı olarak düşük iken p=0,048 ve p=0,001 , NAG ve NAG indeks anlamlı olarak daha yüksek saptandı p=0,049 ve p=0,03 . Klinik başvuru şeklinin bu parametreler üzerinde etkili olmadığı gözlendi p> 0,05 . İdrar kreatinini trientin alanlarda anlamlı olarak yüksek bulundu p=0,004 . Child-Pugh skoru ile bu parametreler arasında herhangi bir ilişki saptanmadı p> 0,05 . Sonuç: Bu çalışmada; WH’li çocuklarda WH’nin kendisinin ve/veya kullanılan ilaçların böbrek fonksiyonları üzerine olumsuz etkisinin olduğu, karaciğer hasarının ciddiyeti ve klinik özelliklerin etkisinin olmadığı görülmüştür

Anahtar Kelimeler

Çocuk, böbrek fonksiyonları, N-asetil-β-Dglikozaminidaz, Wilson hastalığı

Evaluation of Renal Function in Children with Wilson’s Disease

Öz

Introduction: In this study it was aimed to evaluate the renal functions in Wilson’s disease WD using urinary N-acetyl-β-D-glucosaminidase NAG and NAG/creatinine activity index.Materials and Methods: Twenty children of similar age and gender with WD were determined to be the patient group and 37 healthy children were determined to be the control group for the study. NAG levels were calculated as mlU/L and NAG activity index was determined as mlU/mg.Results: While four 20% patients were asymptomatic at diagnosis, chronic hepatitis, neurologic WD, fulminant hepatitis, and acute hepatitis were observed in six 30% , five 25% , three 15% , and two 10% patients, respectively. Of children, 13 65% were on trientine and zinc treatment and seven 35% were on d-penicillamine and zinc. Ten 50% children had proteinuria, one 5% had glycosuria, and one 5% had microscopic hematuria. While mean urine sodium and creatinine levels were significantly lower compared to controls p=0.048 and p=0.001, respectively , NAG and NAG index were significantly higher p=0.049 and p=0.03 . Clinical presentation was observed to be not effective on those parameters p> 0.05 . No relationship was found between Child-Pugh score and these parameters p> 0.05 . Conclusions: It was concluded that while WD itself and/or the drugs that are used for the treatment have negative effects on renal functions for children with WD, however it does not have any effect on the liver damage severity and clinical presentation of the disease.

Anahtar Kelimeler

Children, renal function, N-acetyl-β-Dglucosaminidase, Wilson’s disease

Kaynakça

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