Belgian Journal of Paediatrics
Triple A syndrome, a challenging race for the diagnosis in a potentially lethal pathology: a case report
Vol. 24 No. 4 (2022), Case reports
Vol. 24 No. 4 (2022)

Triple A syndrome, a challenging race for the diagnosis in a potentially lethal pathology: a case report

Case reports
Published 2023-01-20
Tiffany Brose
Faculty of Medicine, University of Liège, Belgium
Alexandra Bobarnac
Division of Pediatric Gastro-Enterology, Department of Pediatrics, CHC-Montlégia, Liège, Belgium
Sophie Lambert
Division of Pediatric Endocrinology, Department of Pediatrics, CHC-Montlégia, Liège, Belgium
Olivia Bauraind
Division of Pediatric Gastro-Enterology, Department of Pediatrics, CHC-Montlégia, Liège, Belgium
Stéphanie Colinet
Division of Pediatric Gastro-Enterology, Department of Pediatrics, CHC-Montlégia, Liège, Belgium
Isabelle Paquot
Division of Pediatric Gastro-Enterology, Department of Pediatrics, CHC-Montlégia, Liège, Belgium
Marc Dirix
Division of Pediatric Surgery, Department of Surgery, CHC-Montlégia, Liège, Belgium
Jamil Khamis
Division of Pediatric Radiology, Department of Radiology, CHC-Montlégia, Liège, Belgium
Florence Roucher
Yves Morel Laboratory, CHU Lyon, France
Pierre Philippet
Division of Pediatric Hemato-oncology and Immunology, Department of Pediatrics, CHC-Montlégia, Liège, Belgium
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Keywords

Triple A syndrome
Allgrove syndrome
Esophageal achalasia
Alacrima
Adrenal insufficiency

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How to Cite

Brose, T., Bobarnac, A., Lambert, S., Bauraind, O., Colinet, S., Paquot, I., Dirix, M., Khamis, J., Roucher, F., & Philippet, P. (2023). Triple A syndrome, a challenging race for the diagnosis in a potentially lethal pathology: a case report. Belgian Journal of Paediatrics, 24(4), 281–283. Retrieved from http://belgjpaediatrics.com/index.php/bjp/article/view/53
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Abstract

Triple A syndrome is a rare disease that associates achalasia, alacrima and adrenal insufficiency. Here we report a case of a 15 year-old girl presenting this typical triad. The symptoms being aspecific, diagnosis was delayed, with a major impact on her growth and development. Despite the rarity of this syndrome, diagnosis must be made as early as possible to avoid lethal consequences (acute adrenal insufficiency, denutrition). We confirmed the diagnosis with imaging and genetic analysis. The patient underwent surgical and medical treatments and was followed to prevent potential complications.

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