Hypodipsic hypernatremia and hypertriglyceridemia associated with cleft lip and cleft palate : A new hypothalamic dysfunction syndrome ?

We report on a 4-month-old girl with congenital hypodipsic hypernatremia resulting from decreased sensitivity of the hypothalamic osmoreceptors with increased tonicity in association with hyperlipemia and cleft lip and cleft palate. We postulate that the link among these various derangements is hypothalamic dysfunction.