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General Anesthesia Management in a Patient with Triple A Syndrome

Year 2021, Volume: 8 Issue: 1, 186 - 188, 31.12.2020
https://doi.org/10.34087/cbusbed.784118

Abstract

Triple A (Allgrove, AAA) syndrome is a rare autosomal recessive disorder that coexists with achalasia, alacrimia and adrenocorticotropic hormone–resistant adrenal insufficiency. Neurological disorders and autonomic neuropathy are often conditions with this syndrome. Adrenal insufficiency; may cause an adrenal crisis in conditions such as surgery, infection, or trauma. This can put anesthetists in difficult situations during anesthesia for various surgeries. With this case, we aimed to share our experiences regarding the points to be considered in an anesthesia method with triple A syndrome.

References

  • 1. Allgrove J, Clayden GS, Grant DB, Macaulay JC. Familial glucocorticoid deficiency with achalasia of the cardia and deficient tear production. Lancet 1978;1(8077):1284–1286. doi:10.1016/s0140-6736(78)91268-0
  • 2. Roucher-Boulez F, Brac de la Perriere A, Jacquez A, et al. Triple-A syndrome: a wide spectrum of adrenal dysfunction. Eur J Endocrinol 2018;178(3):199–207. doi:10.1530/EJE-17-0642
  • 3. Huebner A, Yoon SJ, Ozkinay F, et al. Triple A syndrome–clinical aspects and molecular genetics. Endocr Res 2000;26(4):751–759.
  • 4. Gazarian M, Cowell CT, Bonney M, Grigor WG. The “4A” syndrome: adrenocortical insufficiency associated with achalasia, alacrima, autonomic and other neurological abnormalities. Eur J Pediatr 1995;154(1):18–23.
  • 5. Brooks BP, Kleta R, Stuart C, et al. Genotypic heterogeneity and clinical phenotype in triple A syndrome: a review of the NIH experience 2000–2005. Clin Genet 2005;68(3):215–221. doi:10.1111/j.1399-0004.2005.00482.x
  • 6. Huebner A, Elias LL, Clark AJ. ACTH resistance syndromes. J Pediatr Endocrinol Metab 1999;12(Suppl 1):277–293.
  • 7. Brown B, Agdere L, Muntean C, David K. Alacrima as a harbinger of adrenal insufficiency in a child with Allgrove (AAA) syndrome. Am J Case Rep 2016;17:703–706. doi:10.12659/AJCR.899546
  • 8. Dumic M, Barisic N, Kusec V, et al. Long-term clinical follow-up and molecular genetic findings in eight patients with triple A syndrome. Eur J Pediatr 2012;171(10):1453–1459. doi:10.1007/s00431-012-1745-1
  • 9. Prpic I, Huebner A, Persic M, Handschug K, Pavletic M. Triple A syndrome: genotype-phenotype assessment. Clin Genet 2003;63(5):415–417.
  • 10. Handschug K, Sperling S, Yoon SJ, Hennig S, Clark AJ, Huebner A. Triple A syndrome is caused by mutations in AAAS, a new WD-repeat protein gene. Hum Mol Genet 2001;10(3):283–290. doi:10.1093/hmg/10.3.283
  • 11.Prasad R, Metherell LA, Clark AJ, Storr HL. Deficiency of ALADIN impairs redox homeostasis in human adrenal cells and inhibits steroidogenesis. Endocrinology 2013;154(9):3209–3218. doi:10.1210/en.2013-1241
  • 12. Gilio F, Di Rezze S, Conte A, et al. Case report of adult-onset Allgrove syndrome. Neurol Sci 2007;28(6):331–335. doi:10.1007/s10072-007-0848-3
  • 13. Hadj-Rabia S, Salomon R, Pelet A, et al. Linkage disequilibrium in inbred North African families allows fine genetic and physical mapping of triple A syndrome. Eur J Hum Genet 2000;8(8):613–620. doi:10.1038/sj.ejhg.5200508
  • 14.Hines RL, Marschall KE. Adrenal insufficiency. In: Hines RL, editor. Stoelting's Anesthesia and Co-Existing Disease. 5th ed. Philadelphia: Churchill Livingstone, Elsevier; 2008. pp. 436–7. [Google Scholar]
  • 15Kasar PA, Khadilkar VV, Tibrewala VN. Allgrove syndrome. Indian J Pediatr. 2007;74:959–61.[PubMed] [Google Scholar]
  • 16Anaesthetic management of a patient with Allgrove syndromeBG Arun, BS Deepak, and Murali R Chakravarthy
  • 17Sarathi V, Shah NS. Triple-A syndrome. Adv Exp Med Biol. 2010;685:1–8. doi:10.1007/978-1-4419-6448-9_1
  • 18 Babu K, Murthy KR, Babu N, Ramesh S. Triple A syndrome with ophthalmic manifestations in two siblings. Indian J Ophthalmol. 2007;55:304–6. [PubMed] [Google Scholar]

Triple A Sendromlu Hastada Genel Anestezi Yönetimi – Olgu Sunumu

Year 2021, Volume: 8 Issue: 1, 186 - 188, 31.12.2020
https://doi.org/10.34087/cbusbed.784118

Abstract

Triple A (Allgrove, AAA) sendromu akalazya, alakrima ve adrenal yetmezliğin birlikte görüldüğü nadir otozomal resesif bir bozukluktur. Nörolojik bozukluklar ve otonom nöropati de bu sendromla birlikte sık görülen durumlardır. Adrenal yetmezlik; ameliyat, enfeksiyon veya travma gibi durumlarda adrenal krize sebep olabilir. Bu da çeşitli ameliyatlar için anestezi sırasında anestezistleri zor durumlarla karşı karşıya getirebilir. Bu olgu ile Triple A sendromlu bir çocuğun anestezi yönteminde dikkat edilmesi gereken noktalarla ilgili deneyimlerimizi paylaşmayı amaçladık.

References

  • 1. Allgrove J, Clayden GS, Grant DB, Macaulay JC. Familial glucocorticoid deficiency with achalasia of the cardia and deficient tear production. Lancet 1978;1(8077):1284–1286. doi:10.1016/s0140-6736(78)91268-0
  • 2. Roucher-Boulez F, Brac de la Perriere A, Jacquez A, et al. Triple-A syndrome: a wide spectrum of adrenal dysfunction. Eur J Endocrinol 2018;178(3):199–207. doi:10.1530/EJE-17-0642
  • 3. Huebner A, Yoon SJ, Ozkinay F, et al. Triple A syndrome–clinical aspects and molecular genetics. Endocr Res 2000;26(4):751–759.
  • 4. Gazarian M, Cowell CT, Bonney M, Grigor WG. The “4A” syndrome: adrenocortical insufficiency associated with achalasia, alacrima, autonomic and other neurological abnormalities. Eur J Pediatr 1995;154(1):18–23.
  • 5. Brooks BP, Kleta R, Stuart C, et al. Genotypic heterogeneity and clinical phenotype in triple A syndrome: a review of the NIH experience 2000–2005. Clin Genet 2005;68(3):215–221. doi:10.1111/j.1399-0004.2005.00482.x
  • 6. Huebner A, Elias LL, Clark AJ. ACTH resistance syndromes. J Pediatr Endocrinol Metab 1999;12(Suppl 1):277–293.
  • 7. Brown B, Agdere L, Muntean C, David K. Alacrima as a harbinger of adrenal insufficiency in a child with Allgrove (AAA) syndrome. Am J Case Rep 2016;17:703–706. doi:10.12659/AJCR.899546
  • 8. Dumic M, Barisic N, Kusec V, et al. Long-term clinical follow-up and molecular genetic findings in eight patients with triple A syndrome. Eur J Pediatr 2012;171(10):1453–1459. doi:10.1007/s00431-012-1745-1
  • 9. Prpic I, Huebner A, Persic M, Handschug K, Pavletic M. Triple A syndrome: genotype-phenotype assessment. Clin Genet 2003;63(5):415–417.
  • 10. Handschug K, Sperling S, Yoon SJ, Hennig S, Clark AJ, Huebner A. Triple A syndrome is caused by mutations in AAAS, a new WD-repeat protein gene. Hum Mol Genet 2001;10(3):283–290. doi:10.1093/hmg/10.3.283
  • 11.Prasad R, Metherell LA, Clark AJ, Storr HL. Deficiency of ALADIN impairs redox homeostasis in human adrenal cells and inhibits steroidogenesis. Endocrinology 2013;154(9):3209–3218. doi:10.1210/en.2013-1241
  • 12. Gilio F, Di Rezze S, Conte A, et al. Case report of adult-onset Allgrove syndrome. Neurol Sci 2007;28(6):331–335. doi:10.1007/s10072-007-0848-3
  • 13. Hadj-Rabia S, Salomon R, Pelet A, et al. Linkage disequilibrium in inbred North African families allows fine genetic and physical mapping of triple A syndrome. Eur J Hum Genet 2000;8(8):613–620. doi:10.1038/sj.ejhg.5200508
  • 14.Hines RL, Marschall KE. Adrenal insufficiency. In: Hines RL, editor. Stoelting's Anesthesia and Co-Existing Disease. 5th ed. Philadelphia: Churchill Livingstone, Elsevier; 2008. pp. 436–7. [Google Scholar]
  • 15Kasar PA, Khadilkar VV, Tibrewala VN. Allgrove syndrome. Indian J Pediatr. 2007;74:959–61.[PubMed] [Google Scholar]
  • 16Anaesthetic management of a patient with Allgrove syndromeBG Arun, BS Deepak, and Murali R Chakravarthy
  • 17Sarathi V, Shah NS. Triple-A syndrome. Adv Exp Med Biol. 2010;685:1–8. doi:10.1007/978-1-4419-6448-9_1
  • 18 Babu K, Murthy KR, Babu N, Ramesh S. Triple A syndrome with ophthalmic manifestations in two siblings. Indian J Ophthalmol. 2007;55:304–6. [PubMed] [Google Scholar]
There are 18 citations in total.

Details

Primary Language Turkish
Subjects Anaesthesiology
Journal Section Olgu Sunumu
Authors

İsmet TOPÇU 0000-0002-2783-2865

İlknur BAHAR BAŞKAVAS 0000-0002-6981-3857

Publication Date December 31, 2020
Published in Issue Year 2021 Volume: 8 Issue: 1

Cite

APA TOPÇU, İ., & BAHAR BAŞKAVAS, İ. (2020). Triple A Sendromlu Hastada Genel Anestezi Yönetimi – Olgu Sunumu. Celal Bayar Üniversitesi Sağlık Bilimleri Enstitüsü Dergisi, 8(1), 186-188. https://doi.org/10.34087/cbusbed.784118
AMA TOPÇU İ, BAHAR BAŞKAVAS İ. Triple A Sendromlu Hastada Genel Anestezi Yönetimi – Olgu Sunumu. CBU-SBED: Celal Bayar University-Health Sciences Institute Journal. December 2020;8(1):186-188. doi:10.34087/cbusbed.784118
Chicago TOPÇU, İsmet, and İlknur BAHAR BAŞKAVAS. “Triple A Sendromlu Hastada Genel Anestezi Yönetimi – Olgu Sunumu”. Celal Bayar Üniversitesi Sağlık Bilimleri Enstitüsü Dergisi 8, no. 1 (December 2020): 186-88. https://doi.org/10.34087/cbusbed.784118.
EndNote TOPÇU İ, BAHAR BAŞKAVAS İ (December 1, 2020) Triple A Sendromlu Hastada Genel Anestezi Yönetimi – Olgu Sunumu. Celal Bayar Üniversitesi Sağlık Bilimleri Enstitüsü Dergisi 8 1 186–188.
IEEE İ. TOPÇU and İ. BAHAR BAŞKAVAS, “Triple A Sendromlu Hastada Genel Anestezi Yönetimi – Olgu Sunumu”, CBU-SBED: Celal Bayar University-Health Sciences Institute Journal, vol. 8, no. 1, pp. 186–188, 2020, doi: 10.34087/cbusbed.784118.
ISNAD TOPÇU, İsmet - BAHAR BAŞKAVAS, İlknur. “Triple A Sendromlu Hastada Genel Anestezi Yönetimi – Olgu Sunumu”. Celal Bayar Üniversitesi Sağlık Bilimleri Enstitüsü Dergisi 8/1 (December 2020), 186-188. https://doi.org/10.34087/cbusbed.784118.
JAMA TOPÇU İ, BAHAR BAŞKAVAS İ. Triple A Sendromlu Hastada Genel Anestezi Yönetimi – Olgu Sunumu. CBU-SBED: Celal Bayar University-Health Sciences Institute Journal. 2020;8:186–188.
MLA TOPÇU, İsmet and İlknur BAHAR BAŞKAVAS. “Triple A Sendromlu Hastada Genel Anestezi Yönetimi – Olgu Sunumu”. Celal Bayar Üniversitesi Sağlık Bilimleri Enstitüsü Dergisi, vol. 8, no. 1, 2020, pp. 186-8, doi:10.34087/cbusbed.784118.
Vancouver TOPÇU İ, BAHAR BAŞKAVAS İ. Triple A Sendromlu Hastada Genel Anestezi Yönetimi – Olgu Sunumu. CBU-SBED: Celal Bayar University-Health Sciences Institute Journal. 2020;8(1):186-8.