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Accessible Unlicensed Requires Authentication Published by De Gruyter June 24, 2020

4th A in a triple A syndrome – A rare case report

K. MaheshKumar, Shriraam Mahadevan and Padmavathi Ramaswamy



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.


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.

Corresponding author: Dr. Padmavathi Ramaswamy, MD, Ph.D., Associate Dean - PG studies & Professor, Department of Physiology, Sri Ramachandra Medical College and Research Institute, Sri Ramachandra Institute of Higher Education and Research(SRIHER), Chennai, Tamil Nadu, India, E-mail: .

  1. Research funding: No funding has been involved.

  2. Author contributions: All the authors have made substantial contributions to the conception or design of the work; or the acquisition, analysis, or interpretation of data for the work; participated in drafting the work or revising it critically for important intellectual content; gave final approval of the version to be published and agreed to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

  3. Competing interests: No conflict of interests exists.

  4. Informed consent: Informed consent was obtained from the patient included in the study.

  5. Ethical approval: Ethical approval for the present was obtained from the host institution ethics committee.


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Received: 2019-10-19
Accepted: 2020-03-03
Published Online: 2020-06-24

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