A case of severe transient hyperammonemia in a newborn / 소아과
Korean Journal of Pediatrics
;
: 598-602, 2010.
Artículo
en Inglés
| WPRIM
| ID: wpr-48185
ABSTRACT
Transient hyperammonemia in a newborn is an overwhelming disease manifested by hyperammonemic coma. The majority of affected newborns are premature and have mild respiratory syndrome. The diagnosis may be difficult to determine. This metabolic disorder is primarily characterized by severe hyperammonemia in the postnatal period, coma, absence of abnormal organic aciduria and normal activity of the enzymes of the urea cycle. Hyperammonemic coma may develop within 2-3 days of life, although its etiology is unknown. Laboratory studies reveal marked hyperammonemia (>4,000 micromol/L). The degree of neurologic impairment and developmental delay in this disorder depends on the duration of hyperammonemic coma. Moreover, the infant may succumb to the disease if treatment is not started immediately and continued vigorously. Hyperammonemic coma as a medical emergency requires dialysis therapy. Here, we report a case of severe transient hyperammonemia in a preterm infant (35 week of gestation) presented with respiratory distress, seizure, and deep coma within 48 hours and required ventilatory assistance and marked elevated plasma ammonia levels. He survived with aggressive therapy including peritoneal dialysis, and was followed 2 years later without sequelae.
Texto completo:
Disponible
Índice:
WPRIM (Pacífico Occidental)
Asunto principal:
Plasma
/
Convulsiones
/
Urea
/
Recien Nacido Prematuro
/
Diálisis Peritoneal
/
Coma
/
Hiperamonemia
/
Diálisis
/
Urgencias Médicas
/
Amoníaco
Límite:
Humanos
/
Lactante
/
Recién Nacido
Idioma:
Inglés
Revista:
Korean Journal of Pediatrics
Año:
2010
Tipo del documento:
Artículo
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