Erginel Başak, Gün Feryal, Kocaman Hakan, Çelik Alaadin, Salman Tansu
a Department of Paediatric Surgery, Istanbul Medical Faculty , Istanbul University , Istanbul , Turkey.
Acta Chir Belg. 2016 Apr;116(2):119-21. doi: 10.1080/00015458.2016.1139835. Epub 2016 Apr 21.
Triple A syndrome, also known as Allgrove syndrome, is a rare disease, and presents mainly in children. Its cardinal symptoms are achalasia, alacrima, and adrenocorticotropic hormone (ACTH) insensitivity. We report three cases of Triple A syndrome. Our aim is to inform pediatric surgeons about the existence of this rare syndrome and to highlight the need for suspicion of alacrima and ACTH insensitivity in cases of pediatric achalasia. Triple A syndrome should be considered in patients presenting with achalasia. Alacrima should be investigated by a Schirmer test, and adrenal dysfunction should be tested in cases of suspected triple A.
三 A 综合征,也称为奥尔格罗夫综合征,是一种罕见疾病,主要发生于儿童。其主要症状为贲门失弛缓症、无泪症和促肾上腺皮质激素(ACTH)不敏感。我们报告三例三 A 综合征病例。我们的目的是告知小儿外科医生这种罕见综合征的存在,并强调在小儿贲门失弛缓症病例中怀疑有无泪症和 ACTH 不敏感的必要性。出现贲门失弛缓症的患者应考虑三 A 综合征。应通过泪液分泌试验检查有无泪症,疑似三 A 综合征的病例应检测肾上腺功能障碍。