Introduction: Hereditary nephrogenic diabetes insipidus (NDI) is characterized by inability to concentrate the urine, which results in polyuria and polydipsia. Affected untreated infants usually have poor feeding and failure to thrive, and rapid onset of severe dehydration with illness, hot environment, or the withholding of coverwater. Here we report the case of an early onset of NDI and we underline the difficulty of treatment.
Case report: We report a case of 1 month’s boy with no medical history who presented fever with a loss of wait. On the physical examination he wasdehydrated and sluggish. Laboratory data showed hypernatremia, renal failure and increased serum osmolarity (up to 355,5 mmol/L). It was associated with polyuria between 10 and 24 mL/ kg/h,and low urine osmolarity (102 mmol/L). As the Diabetes insipidus diagnosis was suspected, we completed by the test with oral vasopressin analog. The negative result of the test, confirmed the diagnostic of nephrogenic diabetes insipidus.
Electrolyte disorders were normalized after rehydration and treatment based on anti-inflammatory drugs, which allowed normal weight and height growth with standard fluid supply. Biological and radiological tests were all normal.
Conclusion: Nephrogenic diabetes insipidus (CDI) is a rare disease during early childhood, making it diagnosis difficult and delaying medical treatment.