Aliyu Zakari Y, Gordeuk Victor, Sachdev Vandana, Babadoko Aliyu, Mamman Aisha I, Akpanpe Peter, Attah Ester, Suleiman Yusuf, Aliyu Nurudeen, Yusuf Jamilu, Mendelsohn Laurel, Kato Gregory J, Gladwin Mark T
Department of Medicine and Center for Sickle Cell Disease, Howard University, Washington, District of Columbia 20060, USA.
Am J Hematol. 2008 Jun;83(6):485-90. doi: 10.1002/ajh.21162.
Pulmonary artery systolic hypertension is common and associated with increased mortality among adult sickle cell disease (SCD) patients in the United States. Although the prevalence of SCD is highest in sub-Saharan Africa, the frequency of pulmonary artery systolic hypertension and the risk factors for the development of pulmonary hypertension have not been reported from Africa. We studied 208 hydroxyurea naïve Nigerian SCD patients at steady state and 94 healthy controls. Pulmonary artery systolic hypertension was defined prospectively as tricuspid regurgitant jet velocity > or =2.5 m/sec. Results were compared with a previously published US prospective SCD cohort. Only 7% of Nigerians compared with 46% of US adults with SCD were >35 years. Tricuspid regurgitant jet velocity was > or =2.5 m/sec in 25% of Nigerian SCD patients. Higher jet velocity was associated with greater serum globulin (P = 0.002), blood urea nitrogen (P = 0.019) and lactate dehydrogenase concentrations (P = 0.026) and with inability to walk >300 m in 6 min (P = 0.042). Compared with the US cohort, Nigerian patients had more hemolysis as indicated by lower hemoglobin and higher lactate dehydrogenase concentrations (P < or = 0.003). Pulmonary hypertension is common among Nigerian SCD patients. The public health implication of this finding is significant considering the potential number of individuals at risk for this complication. Better understanding of the long term outcome of pulmonary hypertension and causes of death in SCD and the institution of preventive measures are major public health challenges for Africa. The inclusion of African sites in sickle cell pulmonary hypertension clinical trials should be encouraged.
肺动脉收缩期高血压在美国成年镰状细胞病（SCD）患者中很常见，且与死亡率增加相关。尽管撒哈拉以南非洲地区SCD的患病率最高，但非洲地区肺动脉收缩期高血压的发生率以及肺动脉高压发生的危险因素尚未见报道。我们研究了208例处于稳定期、未服用羟基脲的尼日利亚SCD患者以及94名健康对照者。肺动脉收缩期高血压被前瞻性地定义为三尖瓣反流射流速度≥2.5米/秒。将结果与之前发表的美国前瞻性SCD队列进行比较。与46%的美国成年SCD患者相比，只有7%的尼日利亚患者年龄超过35岁。25%的尼日利亚SCD患者三尖瓣反流射流速度≥2.5米/秒。较高的射流速度与更高的血清球蛋白（P = 0.002）、血尿素氮（P = 0.019）和乳酸脱氢酶浓度（P = 0.026）相关，也与6分钟内行走距离超过300米的能力缺失相关（P = 0.042）。与美国队列相比，尼日利亚患者有更多溶血表现，即血红蛋白水平较低和乳酸脱氢酶浓度较高（P≤0.003）。肺动脉高压在尼日利亚SCD患者中很常见。考虑到有发生这种并发症风险的潜在个体数量，这一发现对公共卫生具有重要意义。更好地了解肺动脉高压的长期结局、SCD患者的死亡原因以及采取预防措施是非洲面临的主要公共卫生挑战。应鼓励将非洲地区纳入镰状细胞性肺动脉高压的临床试验。
