Department of Hematology, Universite Cheikh Anta Diop, Dakar, Senegal; Department of Hematology, National Blood Transfusion Center, Dakar, Senegal.
Haemophilia. 2014 Jan;20(1):73-7. doi: 10.1111/hae.12249. Epub 2013 Aug 6.
Despite significant progres on haemophilia care in developed world, this disease remains unknown in many sub-Saharan African countries. The objectives of this article were to report Senegalese experience on the management of haemophilia care through 18 years of follow-up. This cohort study included 140 patients (127 haemophilia A, 13 haemophilia B), followed in Dakar's haemophilia treatment centre from 1995 to 2012. Our study reported a prevalence of 2.3/100,000 male births, accounting for 11.6% of what is expected in Senegal. From the period 1995-2003 to 2004-2012, significant progress was seen including 67.9% increase in new patient's identification, 11.3 years reduction in mean age at diagnosis (from 15.5 to 4.2 years), lower mortality rate (from 15.3% to 6.8%) and age at death evolved from 6.5 to 23.3 years. Of the 50 haemophilia A patients who were tested for inhibitor presence, 10 were positive (eight severe and two moderate) that is prevalence of 20%. All patients were low responders since inhibitor titre was between 1.5 and 3.8 BU. Disabilities were seen in 36.5% of patients above 20 years old who had musculoskeletal sequels and 39% had no scholar or professional activities in our setting. Implementing haemophilia care in sub-Saharan Africa is a great challenge as this disease is not yet counted in national health problems in many countries. Lessons learned from this study show a significant improvement in diagnosis and prognosis parameters. This emphasizes the needs to set up such follow-up initiatives and to enhance medical and lay cooperation for better results.
尽管在发达国家,血友病的治疗已经取得了显著进展,但在许多撒哈拉以南非洲国家,这种疾病仍然不为人知。本文的目的是报告塞内加尔在血友病治疗方面的经验,该经验是通过 18 年的随访得出的。这项队列研究包括 1995 年至 2012 年在达喀尔血友病治疗中心接受治疗的 140 名患者(127 名血友病 A,13 名血友病 B)。我们的研究报告称,这种疾病的患病率为每 10 万男性出生 2.3 例,占塞内加尔预期患病率的 11.6%。从 1995-2003 年到 2004-2012 年,取得了显著进展,包括新患者识别率增加了 67.9%,诊断时的平均年龄降低了 11.3 年(从 15.5 岁降至 4.2 岁),死亡率降低了(从 15.3%降至 6.8%),死亡年龄从 6.5 岁提高到 23.3 岁。在接受抑制剂检测的 50 名血友病 A 患者中,有 10 名呈阳性(8 名严重,2 名中度),阳性率为 20%。由于抑制剂滴度在 1.5 至 3.8 BU 之间,所有患者均为低应答者。在我们的环境中,36.5%年龄超过 20 岁的患者出现残疾,39%的患者没有学术或职业活动。在许多国家,这种疾病尚未被列入国家卫生问题,因此在撒哈拉以南非洲实施血友病治疗是一项巨大的挑战。从这项研究中吸取的经验表明,在诊断和预后参数方面有了显著改善。这强调了需要建立这种随访举措,并加强医学和非医学合作,以取得更好的结果。