Journal of Child

SUBMITINFORMATION
EnglishTürkçe

Case Study


DOI :10.5222/j.child.2019.59913   IUP :10.5222/j.child.2019.59913    Full Text (PDF)

Acute Chest Syndrome in Children with Sickle Cell Disease: Case Report and Review of the Literature

Nergis Akay, Deniz Tuğcu, Rumeysa Tuna, Süheyla Ocak, Serap Karaman, Ayşegül Ünüvar, Sevgi Beşışık Kalayoğlu, Zeynep Karakaş

Sickle cell anemia (SCA) is the most common hemoglobinopathy all over the world. While the overall incidence of sickle cell disease trait in Turkey is %0.3-0.6, especially some places in Çukurova area it reaches the level of 3-44%. Painful vaso- occlusive crisis which is the most important complication of this disease, is predicted to increase in frequency and HbS polymerization is responsible in its pathophysiology. As an acute complication of SCA, acute chest syndrome is the second most common cause of hospitalization and mortality. İnitial approach to patients with an acute chest syndrome includes supportive treatments. Whereas, solely transfusion is not enough for treatment if there is worsening in respiratory signs and chest radiography; deepening of hypoxemia which requires an oxygen support. Therapeutic red cell apheresis can be used with the aim to treat acute complication of sickle cell disease. In this article we present successful therapeutic red cell apheresis and supportive treatments that we practise in our case who developed acute chest syndrome, and followed up by us with the diagnosis of sickle cell disease.

Keywords: Sickle cell anemia, acute chest syndrome, treatment
DOI :10.5222/j.child.2019.59913   IUP :10.5222/j.child.2019.59913    Full Text (PDF)

Orak Hücreli Anemili Hastada Akut Göğüs Sendromu: Vaka Sunumu ve Literatürün Gözden Geçirilmesi

Nergis Akay, Deniz Tuğcu, Rumeysa Tuna, Süheyla Ocak, Serap Karaman, Ayşegül Ünüvar, Sevgi Beşışık Kalayoğlu, Zeynep Karakaş

Orak hücre anemisi (OHA) dünyada en sık görülen hemoglobinopatilerdendir. OHA taşıyıcılığı sıklığı Türkiye genelinde %0.3-0.6 arasındayken, özellikle Çukurova bölgesinde bazı yörelerde bu sıklık %3-44’e ulaşmaktadır. Sıklığının artması öngörülen bu hastalığın en önemli komplikasyonu vazookluzif ağrılı krizler olup patofizyolojisinde HbS polimerizasyonu sorumludur. Akut komplikasyonlar arasında gösterilen akut göğüs sendromu; ikinci en sık hastanede yatış ve mortalite nedeni olarak gösterilmektedir. Akut göğüs sendromu gelişen hastalara ilk yaklaşım destekleyici tedaviler iken, solunum bulgularında ve akciğer grafisinde kötüleşme, oksijen desteği gerektirecek kadar hipokseminin derinleşmesi durumunda; basit transfüzyon tedavi için yeterli olamamaktadır. Terapötik eritrosit aferezi; orak hücre hastalığı komplikasyonlarını tedavi etmek amacı ile yapılabilmektedir. Bu makalede orak hücre anemili tanısı ile izlenen ve akut göğüs sendromu gelişen vakamızın, destek tedavileri ve terapötik eritrosit aferezi ile başarılı tedavisi sunulmuştur. 

Keywords: Orak hücreli anemi, akut göğüs sendromu, tedavi

PDF View

References

  • 1. Kato GJ, Piel FB, Reid CD, Gaston MH, Frempong KO, Krishnamurti L et al. Sickle cell disease Nature Reviews Disease Primers 2018 Mar; (4):18010. google scholar
  • 2. Quinn CT. Sickle cell disease in childhood: From Newborn screening through transition to adult medical care. Pediatr Clin North Am. 2013 Dec; 60(6):1363- 81. google scholar
  • 3. Frédéric B. Piel, Martin H. Steinberg and David C. Rees. Sickle cell disease. N Engl J Med. 2017;376:1561-73. https://doi.org/10.1056/NEJMra1510865 google scholar
  • 4. Abbas HA, Kahale M, Hosn MA, Inati A. A review of acute chest syndrome in pediatric sickle cell disease. Pediatric Annals 2013 Mar; 42(3):115-20. google scholar
  • 5. Young RC, Castro O, Baxter RP, Dunn R, Armstrong EM, Cook FJ, et al. The lung in sickle cell disease: A clinical overview of common vasculer, infectious, and other problems. J Natl Med Assoc. 1981 Jan; 73(1):19-26. google scholar
  • 6. Jain S, Bakshi N and Krishnamurti L. Acute chest syndrome in children with sickle cell disease pediatric. Allergy Immunology and Pulmonology. 2017 Dec 1; 30(4):191-201. https://doi.org/10.1089/ped.2017.0814 google scholar
  • 7. Paul RN, Castro OL, Aggarwal A, Oneal PA. Acute chest syndrome: sickle cell disease. European Journal of Heamatology September 2011;87(3):191-207. google scholar
  • 8. Howard J, Hart N, MRoberts‐Harewood M, Cummins M, Awogbade M. Bernard Davis on behalf of the BCSH Committee Guideline on the management of acute chest syndrome in sickle cell disease. British Journal of Haemotology 2015;169(4):492-505. https://doi.org/10.1111/bjh.13348 google scholar
  • 9. Miller ST. How I treat acute chest syndrome in children with sickle cell disease Blood 2011;117(20):5297-305. https://doi.org/10.1182/blood-2010-11-261834 google scholar
  • 10. Kim HC. Red cell exchange: special focus on sickle cell disease ASH Education Book December 2014;1: 450-6. google scholar

Citations

Copy and paste a formatted citation or use one of the options to export in your chosen format


EXPORT



APA

Akay, N., Tuğcu, D., Tuna, R., Ocak, S., Karaman, S., Ünüvar, A., Beşışık Kalayoğlu, S., & Karakaş, Z. (2019). Acute Chest Syndrome in Children with Sickle Cell Disease: Case Report and Review of the Literature. Journal of Child, 19(1), 37-42. https://doi.org/10.5222/j.child.2019.59913


AMA

Akay N, Tuğcu D, Tuna R, Ocak S, Karaman S, Ünüvar A, Beşışık Kalayoğlu S, Karakaş Z. Acute Chest Syndrome in Children with Sickle Cell Disease: Case Report and Review of the Literature. Journal of Child. 2019;19(1):37-42. https://doi.org/10.5222/j.child.2019.59913


ABNT

Akay, N.; Tuğcu, D.; Tuna, R.; Ocak, S.; Karaman, S.; Ünüvar, A.; Beşışık Kalayoğlu, S.; Karakaş, Z. Acute Chest Syndrome in Children with Sickle Cell Disease: Case Report and Review of the Literature. Journal of Child, [Publisher Location], v. 19, n. 1, p. 37-42, 2019.


Chicago: Author-Date Style

Akay, Nergis, and Deniz Tuğcu and Rumeysa Tuna and Süheyla Ocak and Serap Karaman and Ayşegül Ünüvar and Sevgi Beşışık Kalayoğlu and Zeynep Karakaş. 2019. “Acute Chest Syndrome in Children with Sickle Cell Disease: Case Report and Review of the Literature.” Journal of Child 19, no. 1: 37-42. https://doi.org/10.5222/j.child.2019.59913


Chicago: Humanities Style

Akay, Nergis, and Deniz Tuğcu and Rumeysa Tuna and Süheyla Ocak and Serap Karaman and Ayşegül Ünüvar and Sevgi Beşışık Kalayoğlu and Zeynep Karakaş. “Acute Chest Syndrome in Children with Sickle Cell Disease: Case Report and Review of the Literature.” Journal of Child 19, no. 1 (Apr. 2024): 37-42. https://doi.org/10.5222/j.child.2019.59913


Harvard: Australian Style

Akay, N & Tuğcu, D & Tuna, R & Ocak, S & Karaman, S & Ünüvar, A & Beşışık Kalayoğlu, S & Karakaş, Z 2019, 'Acute Chest Syndrome in Children with Sickle Cell Disease: Case Report and Review of the Literature', Journal of Child, vol. 19, no. 1, pp. 37-42, viewed 26 Apr. 2024, https://doi.org/10.5222/j.child.2019.59913


Harvard: Author-Date Style

Akay, N. and Tuğcu, D. and Tuna, R. and Ocak, S. and Karaman, S. and Ünüvar, A. and Beşışık Kalayoğlu, S. and Karakaş, Z. (2019) ‘Acute Chest Syndrome in Children with Sickle Cell Disease: Case Report and Review of the Literature’, Journal of Child, 19(1), pp. 37-42. https://doi.org/10.5222/j.child.2019.59913 (26 Apr. 2024).


MLA

Akay, Nergis, and Deniz Tuğcu and Rumeysa Tuna and Süheyla Ocak and Serap Karaman and Ayşegül Ünüvar and Sevgi Beşışık Kalayoğlu and Zeynep Karakaş. “Acute Chest Syndrome in Children with Sickle Cell Disease: Case Report and Review of the Literature.” Journal of Child, vol. 19, no. 1, 2019, pp. 37-42. [Database Container], https://doi.org/10.5222/j.child.2019.59913


Vancouver

Akay N, Tuğcu D, Tuna R, Ocak S, Karaman S, Ünüvar A, Beşışık Kalayoğlu S, Karakaş Z. Acute Chest Syndrome in Children with Sickle Cell Disease: Case Report and Review of the Literature. Journal of Child [Internet]. 26 Apr. 2024 [cited 26 Apr. 2024];19(1):37-42. Available from: https://doi.org/10.5222/j.child.2019.59913 doi: 10.5222/j.child.2019.59913


ISNAD

Akay, Nergis - Tuğcu, Deniz - Tuna, Rumeysa - Ocak, Süheyla - Karaman, Serap - Ünüvar, Ayşegül - Beşışık Kalayoğlu, Sevgi - Karakaş, Zeynep. “Acute Chest Syndrome in Children with Sickle Cell Disease: Case Report and Review of the Literature”. Journal of Child 19/1 (Apr. 2024): 37-42. https://doi.org/10.5222/j.child.2019.59913



TIMELINE


Published Online30.04.2019

LICENCE


Attribution-NonCommercial (CC BY-NC)

This license lets others remix, tweak, and build upon your work non-commercially, and although their new works must also acknowledge you and be non-commercial, they don’t have to license their derivative works on the same terms.


SHARE




Istanbul University Press aims to contribute to the dissemination of ever growing scientific knowledge through publication of high quality scientific journals and books in accordance with the international publishing standards and ethics. Istanbul University Press follows an open access, non-commercial, scholarly publishing.