Abstract

MACHADO, Maren K; BERNARDINI, Alejandra  and  GIACHETTO, Gustavo. Neonatal cholestasis and hypoglycemia like form of congenital hypopituitarism presentation. Arch. argent. pediatr. [online]. 2011, vol.109, n.3, pp.e59-e61. ISSN 0325-0075.

Congenital hypopituitarism is a rare disease, of variable clinic. The neonatal hypoglycemia is one of the habitual forms of presentation; the cholestasis is a rare symptom of this disease. This is the case of a 2-months-old infant hospitalized for cho-lestatic jaundice. He added repeated episodes of severe hypo-glycemia. We investigated metabolic and endocrine causes. The etiology was clarifed by obtaining a critical sample that demonstrated the counterregulatory hormone defciency. The diagnosis of congenital hypopituitarism was completed with confrmation of thyroid hormone and growth hormone def-ciencies. It was confrmed the neuro-anatomical defect of "syndrome of pituitary stalk section" determined by pituitary stalk agenesis, pituitary hipoplasia, and ectopic neurohypophysis. Hormone replacement therapy was started with good response and outcome.

Keywords : Cholestasis; Hypoglycemia; Hypotituitarism.

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