Childhood nephrogenic diabetes insipidus revealed by urinary megabladder in two adult male brothers
Carmen Barbu1,2, Dariana Ionita2, Liudmila Minea2 & Simona Fica1,2
We present the history of two brothers, 19 and respectively 21 years old who were admitted in our department for reevaluation in the context of renal complications. From their medical history we keep in mind that central DI was diagnosed 15 years ago and desmopresin treatment was indicated. Meanwhile, patients received desmopresin unsteady for very short periods of time (less than 2 years) due to poor adherence and obvious inefficiency of the treatment. They claim medical care when clinical signs of megabladder appered raising the suspicion of obstructive renal disease.
At the admission, biological parameters were within normal limits. Abdominal ultrasound revealed hydronephrosis grade I respective III and megabladder in both brothers. CT scans confirmed these issues without no evidence of an obstructive cause. The thirst and desmopresin treatment test revealed the diagnosis of nephrogenic DI with minimal response to desmopresin in the eldest brother.
Treatment was started and urine volume was reduced to 4 l/day with 3 tablets hydrochlorothiazide and 2 tablets indometacinum associated to desmopresin in the eldest brother.
The important aspects of the cases could be pointed as: in the childhood, inefficient treatment was interpreted as psychogenic polydipsia, lowering the adherence to treatment or further investigations. Consequently they drop off the treatment until complications appeared. Moreover, lack of medical records in this period of time raised the question whether renal disease is cause or the complication of the DI.
This case showed us that nephrogenic DI could have a significant impact in terms of biological complications and psycho-social field due to both diagnosis and treatment difficulties.
In this case severe hydronephrosis and megabladder appeared to be an adaptative response of the body in the attempt of young patients to manage the polyuria from social point of view.