Dosunmu Adedoyin O, Balogun Taiwo M, Adeyeye Olufunke O, Daniel Folashade A, Akinola Rachael A, Onakoya Josephine A, Akinbami Akinsegun A, Sagoe Aba O, Onadeko Babatunde O
Department of Haematology and Blood Transfusion, Lagos State University College of Medicine, Ikeja, Lagos, Nigeria.
Department of Medicine, Lagos State University College of Medicine, Ikeja, Lagos, Nigeria.
Niger Med J. 2014 Mar;55(2):161-5. doi: 10.4103/0300-1652.129661.
Sickle cell disease (SCD) is an inherited haemoglobinopathy characterised by recurrent organ hypoxia-reperfusion cycles which may result in repeated organ damage including the lungs and heart. In SCD, pulmonary hypertension is a known complication that may precede or complicate acute chest syndrome which is often fatal. This study seeks to know the prevalence of pulmonary hypertension and its relationship with clinical and laboratory parameters in sickle cell disease patients attending a tertiary hospital in Lagos.
This was a case - control study involving patients with sickle cell disease recruited from adult sickle cell clinic of Lagos State University Teaching Hospital, Ikeja and HbAA controls matched for age and sex from a tertiary educational institution in Lagos. Both the patients and controls were subjected to echocardiography and pulmonary hypertension was deduced from their cardiac tricuspid regurgitant jet velocity. Other parameters measured were age, body mass index, full blood count, red cell indices, foetal haemoglobin, chest X-ray, liver function tests, lactate dehydrogenase and pulmonary function tests. Consenting patients were 56 HbSS in steady state and 28 HbAA controls matched for age and sex. Data was analysed using SPSS version 16.0.
The mean age of patients was 22 ± 6 years. In two 2 of 56 (3.6%) of the participants with sickle cell disease, the pulmonary artery pressure was > 25mmHg and there was significant difference in the mean of the pulmonary artery pressure of the control and that of the patients (P-value 0.013). Also, using the appropriate correlation tests, there was significant relationship between the pulmonary artery pressure and lactate dehydrogenase, aspartate transferase and haematocrit in patients with sickle cell disease.
Sickle cell disease is an independent cause of pulmonary artery hypertension. Variation in cardiovascular reactions to recurrent hyperhaemolysis and hyperdynamic state in sickle cell disease may explain differences in the development of cardiac complications. Exploration of these reactions may reveal other therapeutic measures to prevent complications in sickle cell disease. Clinical assessment of adult patients with sickle cell disease should include echocardiography.
镰状细胞病（SCD）是一种遗传性血红蛋白病，其特征为反复出现器官缺氧 - 再灌注循环，这可能导致包括肺和心脏在内的反复器官损伤。在镰状细胞病中，肺动脉高压是一种已知的并发症，可能先于急性胸部综合征出现或使其复杂化，而急性胸部综合征往往是致命的。本研究旨在了解拉各斯一家三级医院中镰状细胞病患者肺动脉高压的患病率及其与临床和实验室参数的关系。
这是一项病例对照研究，研究对象包括从伊凯贾拉各斯州立大学教学医院成人镰状细胞诊所招募的镰状细胞病患者，以及从拉各斯一所高等教育机构选取的年龄和性别匹配的HbAA对照者。患者和对照者均接受超声心动图检查，并根据其心脏三尖瓣反流喷射速度推断是否患有肺动脉高压。测量的其他参数包括年龄、体重指数、全血细胞计数、红细胞指数、胎儿血红蛋白、胸部X线、肝功能检查、乳酸脱氢酶和肺功能检查。同意参与研究的患者有56例处于稳定状态的HbSS患者以及28例年龄和性别匹配的HbAA对照者。使用SPSS 16.0版软件对数据进行分析。
患者的平均年龄为22±6岁。在56例（3.6%）镰状细胞病参与者中，有2例肺动脉压>25mmHg，对照者和患者的肺动脉压平均值存在显著差异（P值为0.013）。此外，通过适当的相关性检验，镰状细胞病患者的肺动脉压与乳酸脱氢酶、天冬氨酸转氨酶和血细胞比容之间存在显著关系。
镰状细胞病是肺动脉高压的独立病因。镰状细胞病中对反复高溶血和高动力状态的心血管反应差异可能解释了心脏并发症发生发展的差异。对这些反应的探索可能揭示预防镰状细胞病并发症的其他治疗措施。成年镰状细胞病患者的临床评估应包括超声心动图检查。
