A Case of Triple A Syndrome / 대한소아소화기영양학회지
Korean Journal of Pediatric Gastroenterology and Nutrition
; : 188-194, 2000.
Article
em Ko
| WPRIM
| ID: wpr-741343
Biblioteca responsável:
WPRO
ABSTRACT
Achalasia is very uncommon in children, and cases accompanied with alacrima and adrenal insufficiency is even more uncommon. When these three disorders are seen altogether, it is called triple A syndrome. It is inherited in an autosomal recessive manner and has potentially life-threatening sequelae. So, pediatricians should always consider the possibility of triple A syndrome when seeing children with achalasia. Neurological abnormalities such as autonomic neuropathy, peripheral neuropathy, sensory impairment and mental retardation occasionally accompany. We report a 2-year-old girl who presented with repeated vomiting, short stature and alacrima. Diagnosis of achalasia was made after perfoming esophagogram and endoscopy and was confirmed with esophageal manometry. After pneumatic dilatation, she became asymptomatic.
Palavras-chave
Texto completo:
1
Base de dados:
WPRIM
Assunto principal:
Vômito
/
Acalasia Esofágica
/
Insuficiência Adrenal
/
Doenças do Sistema Nervoso Periférico
/
Diagnóstico
/
Dilatação
/
Endoscopia
/
Manometria
/
Deficiência Intelectual
Tipo de estudo:
Diagnostic_studies
Limite:
Child
/
Child, preschool
/
Female
/
Humans
Idioma:
Ko
Revista:
Korean Journal of Pediatric Gastroenterology and Nutrition
Ano de publicação:
2000
Tipo de documento:
Article