Adipsic diabetes insipidus in a child with dysgenesis of corpus callosum
Authors:
L. Kovács; V. Jankó; G. Nagyová; T. Dallos
Authors‘ workplace:
2. detská klinika, Lekárska fakulta Univerzity Komenského a Detskej fakultnej nemocnice s poliklinikou, Bratislava, Slovensko
prednosta prof. MUDr. L. Kovács, DrSc., MPH
Published in:
Čes-slov Pediat 2014; 69 (1): 12-20.
Category:
Case Report
Overview
Rare combined defect of disturbed thirst and vasopressin secretion is associated with increased risk of life threatening episodes of plasma hyperosmolality and hypernatremia. We report a case of adipsic diabetes insipidus associated with dysgenesis of corpus callosum. A 13-year old boy was admitted in a hot summer day complaining for dizziness, muscle cramps and overall weakness. Admission laboratory evaluation revealed serum sodium of 186 mmol/l, plasma osmolality 374 mOsm/kg and urine osmolality of 616 mOsm/kg. After rehydration treatment, he never complained of thirst. Desmopressin treatment was initiated with fixed oral water intake, and on this regimen his serum sodium concentrations stabilized at levels of 148 a 152 mmol/l. Regulation of water homeostasis was evaluated by measuring plasma and urine osmolality. Short-time water restriction and head-up tilt tests were performed to assess osmo- and baroregulated AVP secretion by means of plasma copeptin concentrations. Our findings were consistent with loss of osmoregulatory function of thirst and vasopressin secretion with intact AVP response to baroregulatory stimuli. This may be responsible for production of concentrated urine in the presence of significant volume contraction.
Key words:
adipsic diabetes insipidus, dysgenesis of corpus callosum, hypernatremia, thirst, arginine, vasopressin, copeptin
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Labels
Neonatology Paediatrics General practitioner for children and adolescentsArticle was published in
Czech-Slovak Pediatrics
2014 Issue 1
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