Background: Congenital adiptical diabetes insipidus is rare condition in infancy. Immediate diagnosis and treatment is required to ensure normal development.
Case report: A boy, from the 2nd pregnancy, 1st term delivery was born with weight 4480 g. Bottle feeding from the 1st months. Weight at 1st months 4500 g, at 2nd 5000 g. Until the first 2 months mother complained on childs apathia, feeding problems, vomiting, weight stagnation. At 2 months of age all these symptoms increased. A child was consulted by surgeon and neurologist no problems were found. A boy was dehydrated, and sent to the intensive care department of University hospital (Minsk), than to the endocrinological department. Pathological laboratory findings: Na 151170 (norm 132145 mmol/l), K 3.45.1 (3.15.1 mmol/l), heamoglobin 87119, ph 7.347.44 (7.357.45), ABE (−3.5)−3.8 ((−2)−3) mmol/l), SBE (−1.9)−3.9 ((−1.5)−3 mmol/l), pO2 58.180.7 (6595 mmHg), pCO2 3444.2 (3545 mmHg) urine density 10011003. Hormonal assay: TSH 1.9 (norm: 0.34 IU/ml), free T4 17 (1123 nmol/ml), cortisole 4861367 (170720 nmol/ml), aldosterone 1344 (<1900 ng/ml). On the neurosonography little brain vessel cyst was found without any hemodynamic changes. The urine volume was increased (4 ml/kg per h). Congenital diabetes insipidus adiptical variant was diagnosed. The boy was giving desmopressine 0.3 mg/daily, feeding with low levels of Na, but his condition was not still stable, so, hypothyazide 25 mg/daily was added. At the age of 4 months mother came to the endocrinologist and complained on apathia, vomiting. Height 63 cm, weight 6630 g., blood pressure 120/60, urine density 10061007, Na 142 mmol/l, K 4.6 mmol/l. Brain MRI with contrast showed middle periventricular oedema. Enalapril 5 mg/daily and metoclopramide 10 mg/daily (continuing vomiting) were added to the treatment. Symptomatic arterial hypertension was added to the diagnosis.
Conclusion: Reported case of congenital adiptical diabetes insipidus in infancy is rare and such cases in Belarus are limited.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology