Siegert T, Schmidt H
Kinderklinik und Kinderpoliklinik, Dr. von Haunerschen Kinderspital, LMU München, München, Germany.
Eur J Pediatr Surg. 2001 Dec;11(6):419-21. doi: 10.1055/s-2001-19729.
Triple-A syndrome is a very rare pathology in childhood, consisting of achalasia, alacrima and adrenal insufficiency. The order of manifestation of these three main symptoms is variable. Here we present a case of Triple-A syndrome with recurrent vomiting caused by achalasia as first symptom. At the same time, alacrima was diagnosed. Several years later adrenal insufficiency occurred and substitution therapy with hydrocortisone was started. The follow-up of patients with achalasia should pay attention to symptoms of adrenal insufficiency.