Akinyoola A L, Adediran I A, Asaleye C M
Department of Orthopaedic Surgery and Traumatology, Obafemi Awolowo University, Ile-Ife, Nigeria.
Niger Postgrad Med J. 2007 Sep;14(3):217-20.
Avascular necrosis (AVN) especially of the femoral head, has long been recognised as a manifestation of sickle cell disease (SCD). Despite this knowledge the actual prevalence of this complication among sickle cell patients in Nigeria is not known.
To determine the prevalence and patterns of presentation of avascular necrosis of the femoral head in sickle cell disease.
A retrospective study carried out at the Orthopaedic and Haematology clinics of Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Nigeria. Four hundred and sixteen patients with sickle cell disease seen over a 15-year period.
Of the 416 patients with sickle cell disease 340 (81.7%) had haemoglobin SS genotype while 76(18.3%) had SC genotype. Sixty-six (15.9%) patients 35 males (53%) and 31 females (47%) had clinical and radiologic features of avascular necrosis (AVN) of the femoral head. Fifty-three of them (80.3%) had Hb SS while 13 had Hb SC. The peak age incidence in both Hb SS and Hb SC was 21-30 years. Forty patients (60.6%) presented with stage IV disease (Ficat and Arlet Staging) while 21.2% had stage III and 18.2% had stage II. No patient was diagnosed in stage 1 or 0.
The prevalence of AVN of femoral head in Hb SC and HbSS is similar i.e. the difference is not statistically significant (P=0.48). Most of our patients present late with advanced diseases. Considering the paucity of facilities available for total hip replacement in Nigeria and the young age group affected we recommend regular screening of patients with sickle cell disorder for AVN and regular community educational programmes for early diagnosis.
长期以来,缺血性坏死(AVN)尤其是股骨头缺血性坏死,一直被认为是镰状细胞病(SCD)的一种表现。尽管有此认识,但尼日利亚镰状细胞病患者中这种并发症的实际患病率尚不清楚。
确定镰状细胞病患者股骨头缺血性坏死的患病率及表现模式。
在尼日利亚伊费奥巴费米·阿沃洛沃大学教学医院综合院区的骨科和血液科诊所进行了一项回顾性研究。对15年间诊治的416例镰状细胞病患者进行研究。
416例镰状细胞病患者中,340例(81.7%)为血红蛋白SS基因型,76例(18.3%)为SC基因型。66例(15.9%)患者(35例男性,占53%;31例女性,占47%)具有股骨头缺血性坏死(AVN)的临床和放射学特征。其中53例(80.3%)为Hb SS型,13例为Hb SC型。Hb SS型和Hb SC型的发病高峰年龄均为21 - 30岁。40例患者(60.6%)表现为IV期疾病(菲卡特和阿莱特分期),21.2%为III期,18.2%为II期。无患者被诊断为I期或0期。
Hb SC型和Hb SS型患者股骨头缺血性坏死的患病率相似,即差异无统计学意义(P = 0.48)。我们的大多数患者就诊时病情已进展至晚期。鉴于尼日利亚全髋关节置换可用设施匮乏且受累患者为年轻群体,我们建议对镰状细胞病患者定期筛查缺血性坏死,并开展定期社区教育项目以实现早期诊断。