title: Three cases of triple A syndrome (Allgrove syndrome) in pediatric surgeons' view.
abstract: 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.
authors.affiliation: a Department of Paediatric Surgery, Istanbul Medical Faculty , Istanbul University , Istanbul , Turkey.
authors.forename: Başak
authors.initials: B
authors.lastname: Erginel
authors.affiliation: a Department of Paediatric Surgery, Istanbul Medical Faculty , Istanbul University , Istanbul , Turkey.
authors.forename: Feryal
authors.initials: F
authors.lastname: Gün
authors.affiliation: a Department of Paediatric Surgery, Istanbul Medical Faculty , Istanbul University , Istanbul , Turkey.
authors.forename: Hakan
authors.initials: H
authors.lastname: Kocaman
authors.affiliation: a Department of Paediatric Surgery, Istanbul Medical Faculty , Istanbul University , Istanbul , Turkey.
authors.forename: Alaadin
authors.initials: A
authors.lastname: Çelik
authors.affiliation: a Department of Paediatric Surgery, Istanbul Medical Faculty , Istanbul University , Istanbul , Turkey.
authors.forename: Tansu
authors.initials: T
authors.lastname: Salman
mesh_terms: D000293:Adolescent; D000309:Adrenal Insufficiency / Q000175:diagnosis* / Q000601:surgery*; D002675:Child, Preschool; D004931:Esophageal Achalasia / Q000175:diagnosis* / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D010372:Pediatrics / Q000379:methods; D035583:Rare Diseases; D018570:Risk Assessment; D012494:Sampling Studies; D012720:Severity of Illness Index; D016896:Treatment Outcome
keywords: Achalasia; Allgrove syndrome; Triple A