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  • Title: [Neonatal cholestasis and hypoglycemia like form of congenital hypopituitarism presentation].
    Author: Machado MK, Bernardini A, Giachetto G.
    Journal: Arch Argent Pediatr; 2011 Jun; 109(3):e59-61. PubMed ID: 21660379.
    Abstract:
    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 cholestatic jaundice. He added repeated episodes of severe hypoglycemia. We investigated metabolic and endocrine causes. The etiology was clarified by obtaining a critical sample that demonstrated the counterregulatory hormone deficiency. The diagnosis of congenital hypopituitarism was completed with confirmation of thyroid hormone and growth hormone deficiencies. It was confirmed 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.
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