Sickle cell avascular necrosis: Prevalence and clinical profiles in a tertiary hospital northwestern Nigeria
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
Acute painful crises are the hallmark of Sickle Cell Anaemia (SCA). However, chronic daily pain also occurs in SCA patients with a high frequency. Avascular Necrosis (AVN) of the femur is an important cause of chronic pain and adversely affects their quality of life. The aim is to determine the prevalence of AVN among SCA patients in Zaria and describe some of its clinical and laboratory features. A cross-sectional study of 58 SCA patients in steady state. Data on gender, age, presence of radiologically diagnosed AVN, number of pain crises, blood transfusions in the previous 12 months and laboratory parameters were collated. Data were analyzed using JASP version 0.11.1.0. A critical level of α of 0.05 was set. Females constituted 40 out of 58 (69.0%) of the study participants. The median age was 23 (19.8, 28.0) years. AVN was present in 6 out of 58 (10.3%). There was no relationship between gender and AVN (FET, p=1.00, OR=0.889 95% CI 0.147, 5.359). There was no age difference between patients with AVN and those without AVN (29.92 vs 29.45, MWU=153.500, p=0.956). Patients with AVN had lower mean rank HCT levels (24.08 vs 30.13, MWU=123.500, p=0.414), higher pain episodes (31.67 vs 29.25, MWU=169.000,p=0.747), number of blood transfusions (33.42 vs 29.05, MWU=179.500, P=0.549) and platelets (34.00 vs 28.98, MWU=183.000, p=0.499) compared to those without AVN. Avascular necrosis is common among SCA patients in Zaria. These patients have more blood transfusions and bone pain episodes compared to those without AVN.
Brandow AM, Zappia KJ, Stucky CL. Sickle cell disease: a natural model of acute and chronic pain. Pain. 2017;158 Suppl 1: S79–S84. doi: 10.1097/j.pain.0000000000000824 DOI: https://doi.org/10.1097/j.pain.0000000000000824
Shah KN, Racine J, Jones LC, Aaron RK. Pathophysiology and risk factors for osteonecrosis. Curr Rev Musculoskelet Med. 2015;8:201–209. doi:10.1007/s12178-015-9277-8 DOI: https://doi.org/10.1007/s12178-015-9277-8
Matos MA, Silva LLDS, Alves GB, et al. Necrosis of the femoral head and health-related quality of life of children and adolescents. Acta Ortop Bras. 2018;26:227-230. doi: 10.1590/1413-785220182604174253. PMID: 30210249; PMCID: PMC6131276. DOI: https://doi.org/10.1590/1413-785220182604174253
Mosaku SK, Oyekunle AA, Aneke JC, et al Avascular necrosis significantly impairs quality of life in sickle cell disease. J Clin Sci [serial online] 2015 [cited 2019 Oct 15]; 12:41-7. Available from: http://www.jcsjournal.org/text.asp?2015/12/1/41/160768 DOI: https://doi.org/10.4103/1595-9587.160768
Martí‐Carvajal AJ, Solà I, Agreda‐Pérez LH. Treatment for avascular necrosis of bone in people with sickle cell disease. Cochrane Database of Systematic Reviews 2016, Issue 8. Art. No.: CD004344. DOI: 10.1002/14651858.CD004344.pub6. DOI: https://doi.org/10.1002/14651858.CD004344.pub6
Akinyoola AL, Adediran IA, Asaleye CM. Avascular necrosis of the femoral head in sickle cell disease in Nigeria: a retrospective study. Niger Postgrad Med J. 2007 Sep;14:217-20.
JASP Team (2019). JASP (Version 0.11.1)[Computer software].
Akpan IS, Uboh EE. The pattern of morbidity in adult Nigerians with sickle cell anaemia: A tertiary healthcare setting perspective. Int J Health Sci Res. 2018; 8:18-27.
Alhumaid AM, Aleidi AS, Alfakhri AS, et al Clinical features and outcome of sickle cell anemia in a tertiary center: A retrospective cohort study. J Appl Hematol 2018; 9:22-8 DOI: https://doi.org/10.4103/joah.joah_48_17
Madu A J, Madu A K, Umar G K, et al Avascular necrosis in sickle cell (homozygous S) patients: Predictive clinical and laboratory indices. Niger J Clin Pract 2014; 17:86-9 DOI: https://doi.org/10.4103/1119-3077.122852
Milner PF, Kraus AP, Sebes JI, et al. Sickle cell disease as a cause of osteonecrosis of the femoral head. N Engl J Med. 1991; 325:1476–1481 DOI: https://doi.org/10.1056/NEJM199111213252104
Ya-Zhou Zhang, Xu-Yang Cao, Xi-Cheng Li, et al. Accuracy of MRI diagnosis of early osteonecrosis of the femoral head: a meta-analysis and systematic review. Journal of Orthopaedic Surgery and Research (2018) 13:167 https://doi.org/10.1186/s13018-018-0836-8 DOI: https://doi.org/10.1186/s13018-018-0836-8
Azzali E, Milanese G, Martella I, C et al. Imaging of osteonecrosis of the femoral head. Acta Biomed 2016; Vol. 87, Supplement 3: 6-12
Hsu H, Nallamothu SV. Hip Osteonecrosis. [Updated 2019 Jun 17]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2019 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK499954/
Wasserstein RL, Schirm AL, Lazar NA. Moving to a World Beyond “p < 0.05”, The American Statistician 2019;73:sup1, 1-19, DOI:10.1080/00031305.2019.1583913 DOI: https://doi.org/10.1080/00031305.2019.1583913
Amrhein V, Greenland S, McShane B. Scientists rise up against statistical significance. Nature 2019; 567: 305-307 DOI: https://doi.org/10.1038/d41586-019-00857-9
Gladwin MT, Schechter AN, Ognibene FP, et al. Divergent Nitric Oxide Bioavailability in Men and Women with Sickle Cell Disease. Circulation. 2003; 107:271-278 DOI: https://doi.org/10.1161/01.CIR.0000044943.12533.A8
Xiaodong Zhao, Fuqiang Yang, Luwei Sun, Ali Zhang. Association between NOS3 polymorphisms and osteonecrosis of the femoral head. Artificial Cells, Nanomedicine, and Biotechnology 2019; 47:1423-1427, DOI: 10.1080/21691401.2019.1593995 DOI: https://doi.org/10.1080/21691401.2019.1593995
Zhang GP, Sun JN, Wang J, et al. Correlation between polymorphism of endothelial nitric oxide synthase and avascular necrosis of femoral head. Int J Clin Exp Med. 2015;8(10):18849-54. PMID: 26770506; PMCID: PMC4694406.
Zheng L, Wang W, Ni J, et al. The association of eNOS gene polymorphism with avascular necrosis of femoral head. PLoS One. 2014 Feb 3;9: e87583. doi: 10.1371/journal.pone.0087583. PMID: 24498338; PMCID: PMC3911980. DOI: https://doi.org/10.1371/journal.pone.0087583
Kato GJ, Gladwin MT, Steinberg MH. Deconstructing sickle cell disease: reappraisal of the role of hemolysis in the development of clinical subphenotypes. Blood Rev 2007; 21:37–47. DOI: https://doi.org/10.1016/j.blre.2006.07.001
PAGEPress has chosen to apply the Creative Commons Attribution NonCommercial 4.0 International License (CC BY-NC 4.0) to all manuscripts to be published.