Korean J Pediatr Gastroenterol Nutr.
2000 Sep;3(2):188-194. 10.5223/kjpgn.2000.3.2.188.
A Case of Triple A Syndrome
- Affiliations
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- 1Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea.
- 2Department of Pediatrics, Pundang CHA Hospital, College of Medicine, Pochon CHA University, Sungnam, Korea.
- KMID: 2459712
- DOI: http://doi.org/10.5223/kjpgn.2000.3.2.188
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.
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