Related Experiment Video
Updated: Dec 17, 2025

Cox-Maze IV Procedure Concomitant with Valvular Surgery In Situs Inversus Dextrocardia: A Single-Center Experience in China
Published on: February 11, 2022
4th A in a triple A syndrome - A rare case report
K MaheshKumar1, Shriraam Mahadevan2, Padmavathi Ramaswamy3
1Department of Biochemistry and Physiology, Government Yoga and Naturopathy Medical College and Hospital, Chennai, Tamil Nadu, India.
Abstract:
Objectives AAA (Allgrove) syndrome is a rare genetic disorder characterized by cardinal features of adrenal insufficiency, achalasia, and alacrimia. Case presentation A 21 year girl of known case of Triple A syndrome was referred for the evaluation of autonomic function. She was born full term with developmental delay and abnormal gait. Esophageal manometry study by pneumatic balloon dilatation revealed the presence of achalasia cardia. She had signs of peripheral neuropathy and had episodes of fainting and suspected orthostatic hypotension. Cardiovascular autonomic function and heart rate variability tests were conducted as per Ewing protocol, revealed that the patient had sympathovagal imbalance and sympathetic dominance. Conclusions The presence of autonomic dysfunction adds the 4th A to the Triple A syndrome (Adrenal insufficiency, Achalasia, Alacrimia and Autonomic dysfunction). Noninvasive autonomic function tests are recommended for Triple A syndrome patients to reduce the morbidity associated with autonomic dysfunction.
Related Concept Videos
Aneurysm II: Clinical Manifestations and Diagnostic Studies
Aortic Regurgitation III: Medical Management
Aneurysm III: Interprofessional Care
Type IV Collagen of Basal Lamina
A type IV collagen molecule has six alpha chains which can...
Acute Coronary Syndrome III: Diagnostic Studies
Aortic Regurgitation II: Clinical Features and Diagnostic Tests

