Triple A Syndrome: A Case Report

ABSTRACT

Triple A syndrome is a rarely seen autosomal recessive disease characterized by achalasia, adrenal failure and alacrima. The syndrome is frequently seen in childhood. The appearance of its components are usually ordered as alacrima, achalasia and adrenal failure. The majority of the patients diagnosed in the later stages predominantly present with neurological symptoms. In this study, a 21-year-old male who was referred to our clinic with clinical findings of chronic adrenal failure and was diagnosed WİTH triple A syndrome is presented. This patient had been operated three years ago due to achalasia. The diagnosis and treatment of adrenal failure in this syndrome is the most important determinant and indicator in the prognosis of the disease.