Tek taraflı renal agenezi ve hipoplazili çocukların klinik özellikleri ve izlem sonuçları

Amaç: Az nefron sayısına sahip çocuklarda, yaşamlarının ilerleyen dönemlerinde hipertansiyon, proteinüri ve kronik böbrek yetmezliği gelişme riski yüksektir. Bu renal hasar, glomerüler hiperfiltrasyondam kaynaklanmaktadır. Bu çalışmanın amacı, tek taraflı agenezik ve hipoplazik böbreği olan çocukların klinik özelliklerinin ve izlem sonuçlarının değerlendirilmesidir.Yöntem: Celal Bayar Üniversitesi Tıp Fakültesi ve Dr. Behçet Uz Çocuk Hastanesinde takip edilen, tek taraflı renal agenezisi ve hipoplazisi olan hastaların klinik, radyolojik ve nükleer tıp inceleme bulguları değerlendirildi.Bulgular: Bu çalışma ortalama yaşı 8,9±4 yıl olan 30 (%60) erkek ve 20 (%40) kız hastadan oluşmaktaydı. Yirmi beş hasta renal agenezi, 25 hasta renal hipoplazi tanısı aldı. Laboratuvar incelemesinde ortalama serum kreatinin düzeyi 0,45±0,22 mg/dl ve tahmin edilen glomerüler filtrasyon hızı (eGFR) 144±30,3 ml/dk. idi. Yirmi dokuz (%58) hastada hiperfiltrasyon saptandı. Proteinüri ve hipertansiyonu olan 1 (%4) hasta vardı. On beş (%30) hastada ürolojik anamoli mevcuttu.Sonuç: Bu çalışmada, tek taraflı renal agenezisi ve hipoplazisi olan hastaların serum kreatinin düzeyi normal bulundu. Ancak bu hastaların 29 (%58) hiperfiltrasyon vardı. Hiperfiltasyonu olan çocukların çoğunda yaşamlarının ilerleyen döneminde renal hasar geliştiği için bu çocukların doğumdan itibararen klinik takiplerinin yapılması gerekmektedir.

Clinical characteristics and follow-up results of the children with unilateral renal agenesis and hypoplasia

Objective: Children with an inadequate number of nephrons have an increased risk of developing hypertension, proteinuria and chronic kidney disease in later life. This renal injury is caused by glomerular hyperfiltration. The aim of this study is to evaluate clinical characteristics and follow-up results of the children with unilateral renal agenesis and hypoplasia.Methods: We evaluated the clinical, radiological, and nuclear medicine findings of the children with unilateral agenetic or hypoplastic kidneys who were followed at Celal Bayar University School of Medicine and Dr. Behcet Uz Children's Hospital between 2005 and 2012. Results: The study consisted of 30 (60%) males and 20 (40%) females with a mean age of 8.9±4 years. A total of 25 patients were with renal agenesis and 25 with renal hypoplasia were diagnosed. On laboratory evaluation, mean serum creatinine level was 0.45±0.22 mg/dl and the mean estimated glomerular filtration rate (eGFR) was 144±30.3 ml/min. 29 patient (58%) had hyperfiltration according to an eGFR. Proteinuria and hypertension were noted in 1 (4%) patient. Urological anomalies were found in 15 patients (30%).Conclusion: Our study has determined that children with unilateral renal agenesis and hypoplasia had normal serum creatinine values. Howewer 29 patients (58%) had hyperfiltration according to eGFR. Because many children with hiperfiltration develop renal injury in later life, we emphasize the need for clinical follow- up in these patients starting a birth.

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