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

Year 2019, , 37 - 42, 01.04.2019

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

https://doi.org/10.5222/j.child.2019.59913

Abstract

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

References

  • 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.
  • Quinn CT. Sickle cell disease in childhood: From Newborn screening through transition to adult medi- cal care. Pediatr Clin North Am. 2013 Dec; 60(6):1363- 81.
  • 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
  • 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.
  • 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.
  • 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
  • Paul RN, Castro OL, Aggarwal A, Oneal PA. Acute chest syndrome: sickle cell disease. European Journal of Heamatology September 2011;87(3):191-207.
  • 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
  • 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
  • Kim HC. Red cell exchange: special focus on sickle cell disease ASH Education Book December 2014;1: 450-6.

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

Year 2019, , 37 - 42, 01.04.2019

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

https://doi.org/10.5222/j.child.2019.59913

Abstract

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

References

  • 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.
  • Quinn CT. Sickle cell disease in childhood: From Newborn screening through transition to adult medi- cal care. Pediatr Clin North Am. 2013 Dec; 60(6):1363- 81.
  • 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
  • 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.
  • 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.
  • 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
  • Paul RN, Castro OL, Aggarwal A, Oneal PA. Acute chest syndrome: sickle cell disease. European Journal of Heamatology September 2011;87(3):191-207.
  • 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
  • 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
  • Kim HC. Red cell exchange: special focus on sickle cell disease ASH Education Book December 2014;1: 450-6.
There are 10 citations in total.

Details

Primary Language Turkish
Journal Section Research Articles
Authors

Deniz Tuğcu This is me

Rumeysa Tuna This is me

Süheyla Ocak This is me

Serap Karaman This is me

Ayşegül Ünüvar This is me

Sevgi Kalayoğlu Beşışık This is me

Zeynep Karakaş This is me

Nergis Akay This is me

Publication Date April 1, 2019
Published in Issue Year 2019

Cite

APA Tuğcu, D., Tuna, R., Ocak, S., Karaman, S., et al. (2019). Orak Hücreli Anemili Hastada Akut Göğüs Sendromu: Vaka Sunumu ve Literatürün Gözden Geçirilmesi. Çocuk Dergisi, 19(1), 37-42. https://doi.org/10.5222/j.child.2019.59913
AMA Tuğcu D, Tuna R, Ocak S, Karaman S, Ünüvar A, Kalayoğlu Beşışık S, Karakaş Z, Akay N. Orak Hücreli Anemili Hastada Akut Göğüs Sendromu: Vaka Sunumu ve Literatürün Gözden Geçirilmesi. Çocuk Dergisi. April 2019;19(1):37-42. doi:10.5222/j.child.2019.59913
Chicago Tuğcu, Deniz, Rumeysa Tuna, Süheyla Ocak, Serap Karaman, Ayşegül Ünüvar, Sevgi Kalayoğlu Beşışık, Zeynep Karakaş, and Nergis Akay. “Orak Hücreli Anemili Hastada Akut Göğüs Sendromu: Vaka Sunumu Ve Literatürün Gözden Geçirilmesi”. Çocuk Dergisi 19, no. 1 (April 2019): 37-42. https://doi.org/10.5222/j.child.2019.59913.
EndNote Tuğcu D, Tuna R, Ocak S, Karaman S, Ünüvar A, Kalayoğlu Beşışık S, Karakaş Z, Akay N (April 1, 2019) Orak Hücreli Anemili Hastada Akut Göğüs Sendromu: Vaka Sunumu ve Literatürün Gözden Geçirilmesi. Çocuk Dergisi 19 1 37–42.
IEEE D. Tuğcu, R. Tuna, S. Ocak, S. Karaman, A. Ünüvar, S. Kalayoğlu Beşışık, Z. Karakaş, and N. Akay, “Orak Hücreli Anemili Hastada Akut Göğüs Sendromu: Vaka Sunumu ve Literatürün Gözden Geçirilmesi”, Çocuk Dergisi, vol. 19, no. 1, pp. 37–42, 2019, doi: 10.5222/j.child.2019.59913.
ISNAD Tuğcu, Deniz et al. “Orak Hücreli Anemili Hastada Akut Göğüs Sendromu: Vaka Sunumu Ve Literatürün Gözden Geçirilmesi”. Çocuk Dergisi 19/1 (April 2019), 37-42. https://doi.org/10.5222/j.child.2019.59913.
JAMA Tuğcu D, Tuna R, Ocak S, Karaman S, Ünüvar A, Kalayoğlu Beşışık S, Karakaş Z, Akay N. Orak Hücreli Anemili Hastada Akut Göğüs Sendromu: Vaka Sunumu ve Literatürün Gözden Geçirilmesi. Çocuk Dergisi. 2019;19:37–42.
MLA Tuğcu, Deniz et al. “Orak Hücreli Anemili Hastada Akut Göğüs Sendromu: Vaka Sunumu Ve Literatürün Gözden Geçirilmesi”. Çocuk Dergisi, vol. 19, no. 1, 2019, pp. 37-42, doi:10.5222/j.child.2019.59913.
Vancouver Tuğcu D, Tuna R, Ocak S, Karaman S, Ünüvar A, Kalayoğlu Beşışık S, Karakaş Z, Akay N. Orak Hücreli Anemili Hastada Akut Göğüs Sendromu: Vaka Sunumu ve Literatürün Gözden Geçirilmesi. Çocuk Dergisi. 2019;19(1):37-42.