van Beers Eduard J, van Tuijn Charlotte F J, Mac Gillavry Melvin R, van der Giessen Anna, Schnog John-John B, Biemond Bart J
Department of Haematology, F4-224, Academic Medical Center, PO box 22660, 1100 DD Amsterdam, The Netherlands.
Haematologica. 2008 May;93(5):757-60. doi: 10.3324/haematol.12152. Epub 2008 Mar 26.
In daily clinical practice, the frequency of painful crises (pain rate) is an important parameter of sickle cell disease severity. We assessed the prevalence of sickle cell disease-related organ damage and complications and their relation to pain rate. Organ damage and history of vaso-occlusive complications were obtained via systematic screening of consecutive patients and by chart review. In 104 adult sickle cell patients pain rate was related to a history of acute chest syndromes, avascular osteonecrosis, iron overload, priapism and cholelithiasis. However, major disease-related complications, such as microalbuminuria and pulmonary hypertension, were detected in 23% and 24% respectively of patients without painful crises in the study period underlining the importance of systematic screening for developing organ damage in sickle cell patients irrespective of pain rate.
在日常临床实践中，疼痛危象的发生频率（疼痛率）是镰状细胞病严重程度的一个重要参数。我们评估了镰状细胞病相关器官损害和并发症的患病率及其与疼痛率的关系。通过对连续患者进行系统筛查和查阅病历，获取器官损害和血管闭塞性并发症的病史。在104例成年镰状细胞病患者中，疼痛率与急性胸部综合征、无菌性骨坏死、铁过载、阴茎异常勃起和胆结石病史有关。然而，在研究期间，分别有23%和24%无疼痛危象的患者检测出主要的疾病相关并发症，如微量白蛋白尿和肺动脉高压，这突出了对镰状细胞病患者进行系统筛查以发现器官损害的重要性，而不论其疼痛率如何。
