Triple A syndrome 3A (Allgrove syndrome) is a rare autosomal recessive multiorgans dysfunction characterized by alacrima, achalasia which is the absence of esophageal muscle peristalsis and lower sphincter failure to relax and adrenal insufficiency. About third of patient additional features like neurological and autonomic manifestations reported (making the syndrome 4A), the spectrum of neurological symptoms varies including gait disturbances, parkinsonism, muscle wakeness, mental retardation, peripheral sensory and motor neuropathy. Here we reported A 18 years old male, who had postnatal recurrent conjunctivitis so alacrima was diagnosed, in the sventh years he developed achalasia signs; dysphagia and regurgitation and laparscopic surgical myotomy and fundoplication were done, when he became 16 he presented to our clinic for poor appetite, weight loss,and failure to thrive. Assessment of ACTH, cortisol, ACTH stimulation test confirmed he had adrenal insufficiency and physical examination showed he had foots deformity due to muscular atrophy caused by neuropathy.treatment performed by managing symptoms of the condition(replacement of glucocorticoids, surgical correction of achalasia, artificial tears).The follow-up was over a period of 6 months and we noted a great improvement of patient's condition.
Keywords: Achalasia cardia; Adrenal insufficiency; Alacrimia; Allgrove's syndrome; Triple a syndrome; case report.
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