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

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Published/Copyright: June 24, 2020
Journal of Basic and Clinical Physiology and Pharmacology
From the journal Journal of Basic and Clinical Physiology and Pharmacology Volume 31 Issue 6

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.

Keywords: autonomic dysfunction; HRV; triple A syndrome
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.

References

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

© 2020 Walter de Gruyter GmbH, Berlin/Boston

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https://doi.org/10.1515/jbcpp-2019-0293
Keywords for this article
autonomic dysfunction; HRV; triple A syndrome

Articles in the same Issue

  1. Reviews
  2. An evidence-based systematic review on emerging therapeutic and preventive strategies to treat novel coronavirus (SARS-CoV-2) during an outbreak scenario
  3. Testis and blood-testis barrier in Covid-19 infestation: role of angiotensin-converting enzyme 2 in male infertility
  4. An overview of high-mobility group box 1, a potent pro-inflammatory cytokine in asthma
  5. Original Articles
  6. The effect of novel simple saffron syrup on fatigue reduction in patients with multiple sclerosis
  7. Effects of atenolol injected into the nucleus accumbens septi in rats in the elevated plus-maze test
  8. Beneficial role of central anticholinergic agent in preventing the development of symptoms in mouse model of post-traumatic stress disorder
  9. Pulmonary function severity in relation to interleukin-23 levels in patients with psoriasis vulgaris
  10. Effect of dexamethasone on the expression of MMPs, adenosine A1 receptors and NFKB by human trabecular meshwork cells
  11. Beneficial effect of Bidens pilosa L. (Asteraceae) in a rat model of colitis
  12. Case Reports
  13. 4th A in a triple A syndrome – A rare case report
  14. Is COVID-19 spreading and curing silently: an observation of three family clusters in Bangladesh
  15. Letter to the Editor
  16. SARS-CoV-2 infection, oxidative stress and male reproductive hormones: can testicular-adrenal crosstalk be ruled-out?
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