Peroral Endoscopic Myotomy in Pediatric Triple A Syndrome: Report of Three Cases

Triple A syndrome (Allgrove syndrome) is a rare inherited disorder characterized by adrenal insufficiency, alacrima, and achalasia. Despite POEM's established role in achalasia management, its effectiveness in Triple A syndrome remains largely undefined, as fewer than 10 cases have been reported. We present three genetically confirmed pediatric Triple A patients (two males, one female; 11-16 years) with long-standing achalasia, all with Eckardt scores of 12 on admission and successfully treated with POEM after failing conventional therapies.

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Publication Details

Journal
Revista Española de Enfermedades Digestivas
Published
2025-01-01
DOI
https://doi.org/10.17235/reed.2025.11589/2025
Primary Topic
Gastroesophageal reflux and treatments
Type
article
Field-Weighted Citation Impact
0.00
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article

Peroral Endoscopic Myotomy in Pediatric Triple A Syndrome: Report of Three Cases

Ali Çağatay Bozkına, Abdullah Murat Buyruk
Revista Española de Enfermedades Digestivas
Gastroesophageal reflux and treatments
article

Peroral Endoscopic Myotomy in Pediatric Triple A Syndrome: Report of Three Cases

Ali Çağatay Bozkına, Abdullah Murat Buyruk
article en

Abstract

Triple A syndrome (Allgrove syndrome) is a rare inherited disorder characterized by adrenal insufficiency, alacrima, and achalasia. Despite POEM's established role in achalasia management, its effectiveness in Triple A syndrome remains largely undefined, as fewer than 10 cases have been reported. We present three genetically confirmed pediatric Triple A patients (two males, one female; 11-16 years) with long-standing achalasia, all with Eckardt scores of 12 on admission and successfully treated with POEM after failing conventional therapies.

Revista Española de Enfermedades DigestivasVol. 117(12)
Ege University (TR)
Openalex Percentile: Top 63%
Gastroesophageal reflux and treatments
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