Achalasia-alacrima-ACTH insensitivity syndrome (Triple-A-syndrome).
Achalasia is a rare pathology in infancy. It is still more infrequent when associated with adrenal insufficiency and alacrima, a disorder known as Allgrove's syndrome, the etiology of which remains unclear. We describe a 9-year-old girl who presents with glucocorticoid insufficiency, partial mineralocorticoid deficiency, achalasia, and alacrima.
Dugardeyn
C
C
Department of Radiology, Hôpital Universitaire des Enfants Reine Fabiola, Brussels, Belgium.
Anooshiravani
M
M
Christophe
C
C
Goyens
P
P
Perlmutter
N
N
D000324:Adrenocorticotropic Hormone / Q000097:blood*; D002648:Child; D003241:Consanguinity; D004931:Esophageal Achalasia / Q000000981:diagnostic imaging* / Q000235:genetics; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000172:deficiency*; D007765:Lacrimal Apparatus / Q000002:abnormalities*; D011859:Radiography; D013577:Syndrome
D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone