Absence of rectoanal inhibitory reflex in a child with multiple endocrine neoplasia type 2B.
J Pediatr Surg
; 33(8): 1268-71, 1998 Aug.
Article
em En
| MEDLINE
| ID: mdl-9722002
ABSTRACT
BACKGROUND:
Patients with multiple endocrine neoplasia (MEN) type 2B inherently present with gastrointestinal motility disorders as well as medullary thyroid carcinoma, adrenal pheochromocytoma, and Marfanoid habitus.METHODS:
The authors examined gut motility function in a 7-year-old girl with MEN type 2B who had suffered from chronic constipation and recurrent acalculous cholecystitis since infancy.RESULTS:
Results of total gastrointestinal barium meal and enema studies showed marked hypoperistalsis of the gut and entire colonic dilatation. Histopathologic study results of the gut wall from the stomach, duodenum, and rectum showed hyperplasia of the submucosal and intramuscular neural plexuses in all specimens. Anorectal manometry demonstrated disarrangement of the internal sphincter rhythmic wave and a complete absence of the rectoanal inhibitory reflex.CONCLUSION:
These data suggest that gut motility disorders in MEN type 2B are caused by inadequately organized autonomic nervous system activity that originates from hyperplasia of the enteric nervous system.
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Base de dados:
MEDLINE
Assunto principal:
Canal Anal
/
Neoplasias da Glândula Tireoide
/
Reflexo Anormal
/
Carcinoma Medular
/
Neoplasia Endócrina Múltipla Tipo 2b
Tipo de estudo:
Diagnostic_studies
Idioma:
En
Revista:
J Pediatr Surg
Ano de publicação:
1998
Tipo de documento:
Article
País de afiliação:
Japão