[Arhinencephaly detected by a pitressin-sensitive diabetes insipidus].
A 4-month old child presented with facial malformations and severe hypernatremia. Hypernatremia was secondary to diabetes insipidus due to a disorder of ADH secretion, associated with cerebral malformations. Clofibrate treatment was ineffective. However, after the patient was treated by a low osmotic residue diet, an increased water-intake and hydrochlorothiazide, natremia became normal and growth resumed.
D000015:Abnormalities, Multiple / Q000175:diagnosis*; D002994:Clofibrate / Q000627:therapeutic use; D003919:Diabetes Insipidus / Q000150:complications* / Q000209:etiology; D005145:Face / Q000002:abnormalities; D006801:Humans; D006852:Hydrochlorothiazide / Q000627:therapeutic use; D006955:Hypernatremia / Q000188:drug therapy / Q000209:etiology; D007223:Infant; D008032:Limbic System / Q000002:abnormalities; D008297:Male; D014667:Vasopressins / Q000378:metabolism
D006852:Hydrochlorothiazide; D014667:Vasopressins; D002994:Clofibrate
Arhinencephalie revelee par un diabete insipide pitresso-sensible.