ESPN 51th Annual Meeting

ESPN 2018


 
SILDENAFIL USE IN THE NEPHROGENIC DIABETES INSIPIDUS
AYSE SEDA PINARBASI 1 NUMAN BAYDILLI 2 NESLIHAN GUNAY 1 ISMAIL DURSUN 1 MUAMMER HAKAN POYRAZOGLU 1 ZUBEYDE GUNDUZ 1 RUHAN DUSUNSEL 1

1- ERCIYES UNIVERSITY MEDICAL FACULTY PEDIATRIC NEPHROLOGY
2- ERCIYES UNIVERSITY MEDICAL FACULTY PEDIATRIC UROLOGY
 
Introduction:

Congenital nephrogenic diabetes insipidus (NDI) is a rare, hereditary disorder characterized by massive polyuria and polydipsia, not responding to arginine vasopressin and renal dysfunction. NDI is caused by mutations in the vasopressin type-2 receptor (V2P) or aquaporin-2 (AQP2) channels. AQP2 expression is increased by the activation of the cAMP signal cascade by binding of the arginine vasopressin to V2R. In addition, the activation of the nitric oxide / cGMP signaling pathway in animal experiments results in the AQP2 increasing in the apical part of the cortical collecting tubules of the mice. It has been reported that increasing the concentration of intact cGMP using selective phosphodiesterase type 5 inhibitor and thus results in increasing AQP2 expression. From this point, we present a case with NDI who did not response well classical treatment modality and gave good response to Sildenafil.

Material and methods:

13-year-old male patient was followed with nephrogenic diabetes insipidus since 8 month-old age. He was on low sodium and protein diet, amiloride and hydrochlorothiazide and indomethacin. He had poor quality of life due to polyuria and polydipsia, and his creatinine was going up. KUB ultrasound showed bilateral hydroureteronephrosis. Voiding cystourethrography revealed neurogenic bladder without VUR. The lumbosacral MR was normal. Clean intermittent catheterization was started using Mitrofanoff operation because of family decision. The sildenafil was started since his urine output was between 7 and 8 liters. The urine output was reduced to 4 liters/day after sildenafil treatment. We did not detect any side effect during the treatment.

Results:

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Conclusions:

Sildenafil may be a new option to improve quality of life of patients by reducing polyuria and polydipsia in patients with nephrogenic diabetes insipidus.