Risk Factors of avascular Osteonecrosis of the Femoral Head in Children at the National Reference Center for Sickle Cell Disease in Brazzaville, Congo

F. O. Galiba Atipo-Tsiba *

National Reference Center for Sickle Cell Disease, Brazzaville, Congo and Faculty of Health Sciences, Marien Ngouabi University, Brazzaville, Congo.

A. Bilongo-Bouyou

Faculty of Health Sciences, Marien Ngouabi University, Brazzaville, Congo and Department of Traumatology and Orthopedics, Teatching Hospital of Brazzaville, Congo.

J. C. Bango

National Reference Center for Sickle Cell Disease, Brazzaville, Congo.

J. A. Elira Samba

National Reference Center for Sickle Cell Disease, Brazzaville, Congo.

B. M. Gatsongui

National Reference Center for Sickle Cell Disease, Brazzaville, Congo.

F. Malanda

National Reference Center for Sickle Cell Disease, Brazzaville, Congo and Faculty of Health Sciences, Marien Ngouabi University, Brazzaville, Congo.

L. O. Ngolet

National Reference Center for Sickle Cell Disease, Brazzaville, Congo and Faculty of Health Sciences, Marien Ngouabi University, Brazzaville, Congo.

A. Elira Dokekias

National Reference Center for Sickle Cell Disease, Brazzaville, Congo and Faculty of Health Sciences, Marien Ngouabi University, Brazzaville, Congo.

*Author to whom correspondence should be addressed.


Abstract

Introduction: The avascular osteonecrosis of the femoral head (AOFH) is a common complication of sickle cell disease (SCD). It exposes to lameness and sometimes to a very debilitating handicap. It is a source of desocialization, particularly in developing countries where prosthetic surgery remains inaccessible to the majority. This survey aimed to identify the risk of occurence AOFH.

Materials and Methods: It was a case-control study carried between october 2017 and september 2021 at the National Reference Center for SCD « Antoinette Sassou Nguesso » in Brazzaville. It concerned 31 children with clinical and radiographic signs of AOFH. Clinical (age at diagnosis of SCD, frequency of vasoocclusive crises and hospitalization for vasoocclusive crises, number of blood transfusion) as well as hematological examination (blood count in the intercritical period) and hydroxyurea treatment were compared with those of 62 children with no clinical and radiographic signs of AOFH. The chi2 statistical test and the odds ratio were used for the comparison (P ˂ 0.05).

Results: The sex ratio was 1.38 versus 0.93 (p = 0.50). The mean age of diagnostic of SCD was 3.76 ± 2.56 years versus 3.94 ± 2.01 years (p = 0.81). Logistic regression showed that SCD children with AOFH had a significantly higher rate of annual frequency of VOC (4.16 ± 1.18 vs 2.91 ± 1.68; P = 0.015), annual frequency of hospitalization for VOC (3.74±1.65 vs 1.45 ± 1.28; P = 0.000) and number of blood transfusions (3.38 ± 2.69 vs 2.42 ± 2.32; P = 0.03).

Conclusion: Emphasis should be placed on the prevention and early management of acute complications of SCD. The role of hydroxyurea should be clarified by further work.

Keywords: AOFH, children, femoral head, osteonecrosis, risk factors, sickle cell disease


How to Cite

Atipo-Tsiba , F. O. G., Bilongo-Bouyou , A., Bango , J. C., Samba , J. A. E., Gatsongui , B. M., Malanda , F., Ngolet , L. O., & Dokekias , A. E. (2023). Risk Factors of avascular Osteonecrosis of the Femoral Head in Children at the National Reference Center for Sickle Cell Disease in Brazzaville, Congo. International Blood Research & Reviews, 14(4), 1–7. https://doi.org/10.9734/ibrr/2023/v14i4311

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References

Piel FB, Patil AP, Howes RE. Global distribution of the sickle cell gene and geographical confirmation of the malaria hypothesis. Nat Commun. 2010;1:104.

Odièvre MH, Quinet B. Drépanocytose chez l’enfant. J Pediatr Pueric. 2022;35 (2):73-92.

Piel FB, Patil AP, Howes RE, Nyangiri OA, Gething PW, Dewi M, et al. Global epidemiology of Sickle haemoglobin in neonates: A contemporary geostatistical model based map and population estimates. The Lancet. 2013;381(9861): 142-151.

Milner PF, Kraus AP, Sebes JI, Sleeper LA, Dukes KA, Embury SH, et al. Sickle cell disease as a cause of osteonecrosis of the femoral head. N Engl J Med. 1991;325:1476-81.

Naseer ZA, Bachabi M, Jones LC, Sterling RS, Khanuja HS. Osteonecrosis in sickle cell disease. South Med J. 2016; 109(9):525-30.

Mouba JF, Mimbila M, Lentombo LE, Thardin JF, Ondo A. Nécrose aseptique de la tête fémorale chez l'enfant drépanocytaire: Expérience de Libreville (Gabon). Santé. 2011;21(2):89-92.

Galiba Atipo-Tsiba FO, Bilongo-Bouyou ASW, Gatsongui BM, Ngolet LO, Osseke N, Malanda F, et al. Aseptic osteonecrosis of the femoral head in children living with sickle cell disease at the National Reference Center for sickle cell disease of Brazzaville, Congo. J Blood Disord Med 2023;5(1). DX.DOI. org/10.16966/2471-5026.131.

Coulibaly Y, Coulibaly T, Maiga AK, Konate M, Keita S, Traore SY. Osteonécrose aseptique de la tête fémorale chez les drépanocytaires aspects épidémiocliniques et thérapeutiques dans le service de chirurgie orthopédique et traumatologique. Mali Médical. 2009;4: 43-46.

Daltro G, Do Socorro Salim Ramos N, SarmentoTrindade SM, Cortez de Araujo PI, Borges SM. Pediatric sickle cell disease osteonecrosis of the femoral head: A treatment proposal. Clin Pediatri. 2020;3:1022.

Malheiros CD, Lisle L, Castelar M, Sá KN, Matos MA. Hip dysfunction and quality of life in patients with sickle cell disease. Clin Pediatri (Phila). 2015;54(14) :1354-58.

Matos MA, Silva LLS, Alves GB, Alcântara Júnior WS, Veiga D. Necrosis of the Femoral Head and Health-Related Quality of Life of Children and Adolescents. Acta Ortop Bras. 2018;26:227-30.

Tezol O, Karahan F, Unal S. Sickle cell disease and psychosocial well-being : comparison of patients with preclinical and clinical avascular necrosis of the femoral head. Turk Arch Pediatr. 2021;56(4): 308-15.

Ngolet LO, Okouango Nguelongo Ova JD, Ntsiba H, Elira DA. Complications chroniques du sujet drépanocytaire adulte à Brazzaville. Heal Sci Dis. 2017;18: 56-59.

Mahadeo KM, Oyeku S, Taragin B, Rajpathak SN, Moody K, Santizo R et al. Increased prevalence of osteonecrosis of the femoral head in children and adolescents with sickle cell disease. Am J Hematol. 2011;86(9):806-8.

Leandro MP, De Sá CKC, Filho DPS, De Souza LAA, Salles C, Tenório MCC, et al. Association and risk factors of osteonecrosis of femoral head in sickle cell disease: A systemati creview. Indian J Orthop. 2021;56(2):216-25.

Akinyoola AL, Adediran IA, Asaleye CM, Bolarinwa AR. Risk factors for osteonecrosis of the femoral head in patients with sickle cell disease. Int Orthop. 2009;33:923-6.

Almeida-Matos M, Carrasco J, Lisle L, Castelar M. Avascular necrosis of the femoral head in sickle cell disease in pediatric patients suffering from hip dysfunction. Rev Salud Publica (Bogota). 2016;18(6):986-95.

Adesina O, Brunson A, Keegan THM, Wun T. Osteonecrosis of the femoral head in sickle cell disease: Prevalence, comorbidities, and surgical outcomes in California. Blood Adv. 2017;1(16):1287-95.

Adekile A. The genetic and clinical significance of fetal hemoglobin expression in sickle cell disease. Med Princ Pract. 2021;30(3):201-211.

Gupta R, Adekile AD. MRI follow-up and natural history of avascular necrosis of the femoral head in Kuwaiti children with sickle cell disease. J Pediatr Hematol Oncol. 2004;26(6):351-353.

Worrall D, Smith-Whitley K, Wells L. Hemoglobin to hematocrit ratio : the strongest predictor of femoral head osteonecrosis in children with sickle cell disease. J Pediatr Orthop. 2016;36(2): 139-44.

Naik RP, Streiff MB, Lanzkron S. Sickle cell disease and venous thromboembolism : What the anticoagulation expert needs to know. J Thromb Thrombolysis. 2013; 35:352-358.

Adekile AD, Gupta R, Al-Khayat A, Mohammed A, Atyani S, Thomas D. Risk of avascular necrosis of the femoral head in children with sickle cell disease on hydroxyurea: MRI evaluation. Pediatr Blood Cancer. 2019;66(2):e27503.

Mukisi-Mukasa M, Samuel-Leborgne Y, Kéclard L, Le Turdu-Chicot C, Christophe-Duchange E. Prevalence, clinical features and risk factors of osteonecrosis of femoral head among adult patients with sickle cell disease. Orthopedics. 2000;23:357-63.

Galkin O, Pan W, Filobelo L, Hirsch RE, Nagel RL, Vekilov PG. Two-step mechanism of homogeneous nucleation of sickle cell hemoglobin polymers. Biophys J 2007;93:902-13.

Uzunova VV, Weichun P, Galkin O, Vekilov PG. Free heme and the polymerization of sickle cell hemoglobin. Biophys J. 2010;99:1976-85.

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(1):37-47.

Adesina OO, Neumayr LD. Osteonecrosis in sickle cell disease: An update on risk factors, diagnosis, and management. Hematology Am Soc Hematol Educ Program. 2019;2019(1):351-58.

Voskaridou E, Christoulas D, Bilalis A, Plata E, Varvagiannis K, Stamatopoulos G, et al. The effect of prolonged administration of hydroxyurea on morbidity and mortality in adult patients with sickle cell syndromes: results of a 17-year, single-center trial (LaSHS). Blood. 2010; 115(12): 2354-63.

Adekile A, Menzel S, Gupta R, Al-Sharida S, Farag A, Haider M, et al. Response to hydroxyurea among Kuwaiti patients with sickle cell disease and elevated baseline HbF levels. Am J Hematol. 2015;90 (7):138-39.