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Alacrimia Revealing Triple A Syndrome: A Case Report

  •   M. Achargui
  •   R. Azhrai
  •   Y. Harrar
  •   F. Mabrouki
  •   S. Chariba
  •   A. Maadane
  •   R. Sekhoukh

Asian Journal of Research and Reports in Ophthalmology, Volume 6, Issue 1, Page 24-28

Published: 25 February 2023

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Abstract


Introduction: Allgrove's syndrome is a rare hereditary disease associating alacrimia, achalasia and Addison's disease. Neurological signs are observed in a third of the cases. We report the observation of a 10-year-old child with congenital alacrimia in the context of Allgrove syndrome.

Patients and Methods: We report the case of a 10-year-old patient with triple A syndrome.

Results: The ophthalmological examination revealed a superficial punctate keratitis with an altered Schirmer's test. The somatic examination found a perioral melanoderma, with amyotrophy of the leg and hollow foot. The explorations performed showed an Addison's syndrome in both children associated with achalasia.

Discussion: Congenital alacrimia is the most frequently reported sign, it concerns the basic lacrimal secretion and often also the reflex secretion. Adrenocortical insufficiency is manifested during the first decade of life by severe hypoglycemic crises. Achalasia is revealed between 6 months and 15 years of age by vomiting and dysphagia.

Conclusion: In front of any alacrimia of the child or the young adult must evoke a syndrome of Allgrove. A careful search for adrenal insufficiency, achalasia and associated neurological signs is necessary and allows to watch out for complications that could put at risk the vital prognosis.

Keywords:
  • Alacrimia
  • triple A syndrome
  • pediatric population
  • Full Article – PDF
  • Review History

How to Cite

Achargui , M., Azhrai , R., Harrar , Y., Mabrouki , F., Chariba , S., Maadane , A., & Sekhoukh , R. (2023). Alacrimia Revealing Triple A Syndrome: A Case Report. Asian Journal of Research and Reports in Ophthalmology, 6(1), 24–28. Retrieved from https://journalajrrop.com/index.php/AJRROP/article/view/73
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References

Yang H, Zhang H, Lu L, Wang O, Xing X, Zhang M, Lu Z. Clinical and genetic characterization of a Chinese patient with triple A syndrome and novel compound heterozygous mutations in the AAAS gene. J Pediatr Endocrinol Metab. 2013;26(3-4): 389-91.

Moore PS, Couch RM, Perry YS, Shuckett EP, Winter JS. Syndrome d'Allgrove: Un syndrome autosomique récessif d'insensibilité à l'ACTH, d'achalasie et d'alacrime Clin Endocrinol (Oxf). 1991;34: 107–14

Pedreira CC, Zacharin MR. Syndrome d'Allgrove: Lorsqu'un trouble pédiatrique reconnaissable survient à l'âge adulte MJA. 2004;180:74–5.

Djemai M, Abada-Bendib M, Masmoudi AN, Azzôug, Chentli M. Syndrome d'Allgrove: Revue de 3 familles. Revue Neurologique. 2007;163(4):112.

Mullaney P, et al. Kératoconjonctivite sèche associée à une achalasie du cardia, une insuffisance corticosurrénalienne et une dégénérescence des glandes lacrymales: la kératoconjonctivite sèche secondaire à une dégénérescence des glandes lacrymales peut être parallèle à des modifications dégénératives de la fonction œsophagienne et corticosurrénalienne. Ophtalmologie. 1998;105(4):643–50.

Kimber J, McLean BN, Prevett M, Hammans SR. Syndrome d'Allgrove ou 4 « A »: Un syndrome autosomique récessif provoquant une maladie neurologique multisystémique. J Neurol Neurosurg Psychiatrie. 2003;74(5):654–7.

Sinan H, Tatum RP, Soares VR, et al. Prevalence of respiratory symptoms in patients with achalasia. Dis Esophagus. 2011;24:224–8.

Mehdi NF, Weinberger M, Abu-Hasan MN. Achalasia: Unusual cause of chronic cough in children. Cough. 2008;4:6.

Azizkhan RG, Tapper D, Eraklis A. Achalasia in childhood: A 20-year experience. J Pediatr Surg. 1980;15: 452–6.

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