Background: Neurogenic diabetes insipidus (NDI) is extremely rare in the neonatal period. In most cases, its secondary to CNS injury. The clinical presentation in this group (particularly in preterm) is unspecific and a high degree of suspicion for the diagnosis is necessary.
Objective and hypotheses: Diagnose NDI in a neonate with sodium and diuresis instability.
Method: We present a case report.
Results: Male, born at 25 weeks and 3 days of gestation. Eutocic birth delivery complicated by feet appearance, Apgar Index 1′/5′/10′: 1/3/7. Difficult intubation caused by facial anomalies consistent with PierreRobin sequence (microretrognatia and posterior midline cleft palate). Transfontanellar ultrasound at D10 of life reveals bilateral grade III intraventricular hemorrhage with hemorrhagic infarction associated with hydrocephalus. Initially natremic instability, after D11 he expresses a persistent hypernatremia (max 166 mEq/l) associated with increased diuresis (max 11.3 ml/kg per h), plasma osmolarity increased and urinary osmolarity decreased. The hypothesis of NDI was made, and the newborn started nasal desmopressin, with reduced sodium levels and urine output. In the beginning there was instability of both parameters, therefore the need of constant therapeutic setting. At 4 months of chronological age, he still needs desmopressin to maintain both parameters in a normal range.
Conclusion: The main risk factors for neonatal NDI are asphyxia, severe infections, CNS anomalies, and intraventricular hemorrhage. Hypernatremia may occur in neonates, especially in preterm with severe disease, yet if it persists despite increased fluids intake, it should be seen as an alarm signal. Treatment with desmopressin is effective but its dosage is variable and should be adapted individually. The aim is to keep sodium levels, diuresis and urine osmolality within the normal range, allowing a better metabolic balance and limiting aggression injured CNS.
20 - 22 Sep 2014
European Society for Paediatric Endocrinology