Main Article Content
Sickle cell anemia, children, spirometry, transplant, asthma.
Children with sickle cell anemia (SCA) are prone to invasive infections caused by S. pneumonia, H. influenzae, and Plasmodium falciparum. The presence of asthma increases the risk of mortality, and it has been shown an association between the presence of bronchial hyperactivity and the onset of acute chest syndrome. Like thalassemia, allogeneic hematopoietic stem cell transplantation (HSCT) is curative in most individuals with SCA. We report pulmonary function analyzed by spirometry, before and after transplantation, in a group of Nigerian SCA children. Our results indicate that restrictive pulmonary pattern (RVP) is a common finding in these patients. Although we do not observe significant improvements in lung function after 3-6 months of transplantation, it is useful to perform a spirometric evaluation of these patients at the time of transplant.
2. Caboot JB, Allen JL. Pulmonary complications of sickle cell disease in children. Curr Opin Pediatr. 2008;20:279-87.
3. Boyd JH, Macklin EA, Strunk RC, DeBaun MR. Asthma is associated with increased mortality in individuals with sickle cell anemia. Haematologica. 2007;92:1115-8.
4. Sylvester KP, Patey RA, Rafferty GF, Rees D, Thein SL, Greenough A. Airway hyperresponsiveness and acute chest syndrome in children with sickle cell anemia. Pediatr Pulmonol. 2007;42:272-6.
5. Sylvester KP, Patey RA, Broughton S, Rafferty GF, Rees D, Thein SL, Greenough A. Temporal relationship of asthma to acute chest syndrome in sickle cell disease. Pediatr Pulmonol. 2007;42:103-6.
6. Boyd JH, Macklin EA, Strunk RC, DeBaun MR. Asthma is associated with acute chest syndrome and pain in children with sickle cell anemia. Blood. 2006;108:2923-7.
7. Bryant R. Asthma in the pediatric sickle cell patient with acute chest syndrome. J Pediatr Health Care. 2005;19:157-62.
8. Knight-Madden JM, Forrester TS, Lewis NA, Greenough A. Asthma in children with sickle cell disease and its association with acute chest syndrome. Thorax. 2005;60:206-10.
9. Nordness ME, Lynn J, Zacharisen MC, Scott PJ, Kelly KJ. Asthma is a risk factor for acute chest syndrome and cerebral vascular accidents in children with sickle cell disease. Clin Mol Allergy. 2005;3:2 .
10. Boyd JH, Moinuddin A, Strunk RC, DeBaun MR. Asthma and acute chest in sickle-cell disease. Pediatr Pulmonol. 2004;38:229-32.
11. Bernaudin F, Strunk RC, Kamdem A, Arnaud C, An P, Torres M, Delacourt C, DeBaun MR. Asthma is associated with acute chest syndrome, but not with an increased rate of hospitalization for pain among children in France with sickle cell anemia: a retrospective cohort study. Haematologica. 2008;93:1917-8 .
12. Frei-Jones MJ, Field JJ, DeBaun MR.Risk factors for hospital readmission within 30 days: a new quality measure for children with sickle cell disease. Pediatr Blood Cancer. 2009;52:481-5.
13. Isgrò A, Paciaroni K, Gaziev J, Sodani P, Gallucci C, Marziali M, Angelis GD, Alfieri C, Ribersani M, Roveda A, Akinyanju OO, Wakama TT, Olowoselu FO, Adediran A, Lucarelli G. Haematopoietic stem cell transplantation in Nigerian sickle cell anaemia children. Niger Med J. 2015;56(3), 175-9.
14. Miller MR, Hankinson J, Brusasco V, Burgos F, Casaburi R, Coates A, Crapo R, Enright P, Van der Grinten CPM, Gustafsson P, Jensen R, Johnson DC, MacIntyre N, McKay R, Navajas D, Pedersen OF, Pellegrino R, Viegi G and Wanger J. Standardization of spirometry. Eur Respir J. 2005; 26: 319–338
15. Klings ES, Wyszynski DF, Nolan VG, Steinberg MH. Abnormal Pulmonary Function in Adults with Sickle Cell Anemia. Am J Respir Crit Care Med. 2006;173(11):1264–1269.