Endocrine Abstracts

Introduction: Central diabetes insipidus (CDI) is a rare but potentially dangerous dysregulation of the water balance secondary to an arginine vasopressin (AVP) deficiency. Diabetes insipidus may have different manifestations in children compared to adults. CDI is more common in children than nephrogenic diabetes insipidus.

Methods: A retrospective study of eight patients collected in the pediatric department of Mahdia.

Results: The average age of patients was 2.8 years. The reason for hospitalization was a prolonged fever in three patients, weight stagnation in three patients, severe dehydration in one patient and headache and decreased visual acuity in one patient. A history of polyuropolydypsic syndrome was found in five patients only. Diuresis was quantified at 9 ml/kg per hour on average. In biology, the plasma osmolarity was 296.8 mosmol/Kg and the urinary osmolarity was 95.8 mosmol/kg with a urinary density at 1001 on average.Water deprivation test was carried out in four patients only confirming the diagnosis of CDI while the four remaining patients had underwent directly a Desmopressin stimulation test. The etiological assessment revealed a cerebral malformation in 4 patients, a Langerhans cell histiocytosis in three patients and a craniopharyngioma in one patient. Hormonal assessment revealed adrenal insufficiency in one patient and thyrotropic insufficiency in two patients. Treatment with intranasal Desmopressin was started at the mean dose of 0.125 ml/day in combination with the etiological treatment. The evolution was favorable in most of our patients.

Conclusion: The discovery of a CDI in children leads to an etiological investigation with an urgent need to identify tumor pathologies (craniopharyngioma, dysgerminoma). Infiltrative pathologies may be already known or reveal the disease (histiocytosis, and more rarely sarcoidosis). The objective of the management is to ensure a normal water balance by normalizing the diuresis by the administration of Desmopressin, to prevent any water retention and therefore any hyponatremia. It is associated with the treatment of the etiology of the CDI and the hormone replacement therapy of the associated anterior pituitary insufficiency.