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Archivos de Pediatría del Uruguay

Print version ISSN 0004-0584On-line version ISSN 1688-1249

Abstract

OSPITALECHE, Mariangel; GHIONE, Andrea; RAMIREZ, María José  and  SILVERA, Fernando. Neonatal shock as the initial presentation of panhypopituitarism. A clinical case. Arch. Pediatr. Urug. [online]. 2017, vol.88, n.3, pp.161-167. ISSN 0004-0584.

Congenital panhypopituitarism is a rare disease which may have variable clinical presentations, depending on the hormonal deficit. Disruption of the hypophyseal stem, one of the etiologies, is diagnosed in only 23% of the cases in the neonatal period, causing hypogenesis of the anterior pituitary and agenesis or ectopia of the posterior pituitary. Early diagnosis and timely treatment have an impact on morbidity and mortality. We present the case of a term newborn, female, small for gestational age, product of pregnancy and childbirth without complications. No dysmorphism. She was admitted into the neonatal unit at 14 hours of life, due to mild hypothermia and hypoglycemia with high parenteral glucose requirements. In the evolution, repeated episodes of shock of unclear etiology occurred, which responded to the treatment applied. Infectious, cardiovascular and metabolic causes were ruled out. The patient added abnormal movements, hemodynamic instability and anisocoria. Thus, MRI of the skull was requested, and it revealed ectopic neurohypophysis associated with hypophyseal stem and hypoplastic adenohypophysis.

Keywords : Congenital hypopituitarism; Shock; Infant newborn.

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