Three cases of triple A syndrome (Allgrove syndrome) in pediatric surgeons' view

被引:2
|
作者
Erginel, Basak [1 ]
Gun, Feryal [1 ]
Kocaman, Hakan [1 ]
Celik, Alaadin [1 ]
Salman, Tansu [1 ]
机构
[1] Istanbul Univ, Istanbul Fac Med, Dept Paediat Surg, Istanbul, Turkey
关键词
Achalasia; Allgrove syndrome; Triple A; A-SYNDROME; PHENOTYPE; ACHALASIA; MUTATION; GENE;
D O I
10.1080/00015458.2016.1139835
中图分类号
R61 [外科手术学];
学科分类号
摘要
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.
引用
收藏
页码:119 / 121
页数:3
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