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

G. Fo, Bilongo-Bouyou Asw, Gatsongui Bm, Ngolet Lo, O. N., Malanda F, Elira Dockekias A
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Abstract

Introduction: Aseptic Osteonecrosis of the Femoral Head (AOFH) is a degenerative complication of sickle cell disease. In the absence of care, it is a source of disability through painful lameness and functional impotence that it causes over time. Its data in african children are scarce. This work aimed at determining its prevalence and describing its characteristics. Methodology: It was about a descriptive study conducted over a4 years- period at the CNRDr on children suffering from homozygous sickle cell disease and presenting with ONFH. The studied variables were epidemiological, clinical and paraclinical, collected from medical records. The Postel Merle-d’Aubigné functional score was used to assess the functional status of the hips and the Arlet and Ficat classification for radiological staging. Results: The study involved 31 children (18 boys and 13 girls). The prevalence of AOFH was 2.15%. The average age was 11.51 ± 2.17 years old. Almost half were under 11 (45.16%). At diagnosis, the damage was bilateral in 10/31 cases, bringing the number of pathological hips to 41/62. Hip pain was constant and associated with lameness in 83.87% of cases. Hip function was good, fair, and poor respectively in 41.94%, 54.84% and 3.22% of cases. One child presented with two stiff hips. Radiologically, stages I and II on one hand and III and IV on the other hand of Arlet and Ficat were respectively found in 45.16% and 54.84% of cases. Conclusion: We report one of the most important series of black Africa. AOFH affects young children. The diagnosis is frequently late, made at the stage of functional impotence with joint destruction. The search for predictive factors is important to improve the prognosis of these young patients.
刚果布拉柴维尔镰状细胞病国家参考中心镰状细胞病患儿股骨头无菌性骨坏死
无菌性股骨头坏死(AOFH)是镰状细胞病的一种退行性并发症。在缺乏护理的情况下,随着时间的推移,它会导致痛苦的跛行和功能性阳痿,从而成为残疾的根源。非洲儿童的数据很少。这项工作旨在确定其流行程度并描述其特征。方法:这是一项在CNRDr对患有纯合子镰状细胞病并表现为ONFH的儿童进行的为期a4年的描述性研究。研究变量为流行病学、临床和临床辅助,收集自医疗记录。Postel - Merle-d ' aubign功能评分用于评估髋关节的功能状态,Arlet和Ficat分级用于放射分期。结果:本研究涉及31名儿童(18名男孩,13名女孩)。AOFH患病率为2.15%。平均年龄11.51±2.17岁。近一半(45.16%)在11岁以下。诊断时10/31例为双侧损伤,病理髋数为41/62。83.87%的患者髋部持续疼痛并伴有跛行。髋关节功能良好、一般和较差的分别占41.94%、54.84%和3.22%。一名儿童表现为两髋僵硬。放射学上,Arlet和Ficat分别为I、II期和III、IV期,分别占45.16%和54.84%。结论:我们报告了一个最重要的黑非洲系列。AOFH影响幼儿。诊断往往很晚,在功能阳痿与关节破坏的阶段。寻找预测因素对改善这些年轻患者的预后非常重要。
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