G. Fo, Bilongo-Bouyou Asw, Gatsongui Bm, Ngolet Lo, O. N., Malanda F, Elira Dockekias A
{"title":"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","authors":"G. Fo, Bilongo-Bouyou Asw, Gatsongui Bm, Ngolet Lo, O. N., Malanda F, Elira Dockekias A","doi":"10.16966/2471-5026.131","DOIUrl":null,"url":null,"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.","PeriodicalId":138925,"journal":{"name":"Journal of Blood Disorders and Medicine","volume":"6 1","pages":"0"},"PeriodicalIF":0.0000,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"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\",\"authors\":\"G. Fo, Bilongo-Bouyou Asw, Gatsongui Bm, Ngolet Lo, O. N., Malanda F, Elira Dockekias A\",\"doi\":\"10.16966/2471-5026.131\",\"DOIUrl\":null,\"url\":null,\"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.\",\"PeriodicalId\":138925,\"journal\":{\"name\":\"Journal of Blood Disorders and Medicine\",\"volume\":\"6 1\",\"pages\":\"0\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"1900-01-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of Blood Disorders and Medicine\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.16966/2471-5026.131\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Blood Disorders and Medicine","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.16966/2471-5026.131","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
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
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.