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Turkiye Klinikleri Pediatri, vol.25, no.2, pp.119-123, 2016 (Scopus)
© Copyright 2016 by Türkiye Klinikleri.Triple A syndrome is a rare autosomal recessive disorder characterized by alacrima, adrenocorticotropic hormone resistant adrenal insufficiency and Achalasia. The clinical findings can be heterogeneous. Neurological findings such as autonomic dysfunction may accompany this syndrome, and therefore, it is sometimes called as 4 A syndrome. This report describes a 7-year-old girl presented with a hypoglycemic seizure to emergency. She had tonsillitis, dark cutaneous pigmentation and unnoticed alacrima. It was learned that she was operated for Achalasia at the age of 5 years. The clinical and laboratory findings were consistent with adrenal insufficiency and she was diagnosed as Triple A syndrome. As the components of Triple A syndrome may develop at different times, careful follow-up should be done in patients with Achalasia and alacrima in respect of adrenal insufficiency.