The effects of a somatostatin analogue on the metabolism of an infant with Beckwith-Wiedemann syndrome and hyperinsulinaemic hypoglycaemia.
Eur J Pediatr
; 150(9): 634-7, 1991 Jul.
Article
en En
| MEDLINE
| ID: mdl-1915515
ABSTRACT
Hypoglycaemia is a frequent finding during the neonatal period and may be due to insulin overproduction. Patients with Beckwith-Wiedemann syndrome have reduced numbers of somatostatin-producing cells and decreased extractable somatostatin. In this study the effect of long-acting somatostatin (SMS201-995) on the glucose and insulin levels in an infant with Beckwith-Wiedemann syndrome and hyperinsulinaemic non-ketotic hypoglycaemia is described. SMS201-995 lowered basal insulin levels while maintaining normal glucose and insulin homeostasis. During fasting however, both glucose levels declined rapidly whereas insulin levels did not. The absence of both ketosis and elevated levels of free fatty acids and lactate during hypoglycaemia, as observed in our patient, are important diagnostic clues since the insulin levels themselves may sometimes be only slightly elevated.
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Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Síndrome de Beckwith-Wiedemann
/
Octreótido
/
Hiperinsulinismo
/
Hipoglucemia
Tipo de estudio:
Etiology_studies
Límite:
Humans
/
Male
/
Newborn
Idioma:
En
Revista:
Eur J Pediatr
Año:
1991
Tipo del documento:
Article
País de afiliación:
Países Bajos