Department of Medicine, Division of Hematology, Washington University School of Medicine, St. Louis, MO 63110, USA.
Hematology Am Soc Hematol Educ Program. 2009:45-53. doi: 10.1182/asheducation-2009.1.45.
A physician diagnosis of asthma in children and adults with sickle cell disease (SCD) has been associated with increased rates of pain and acute chest syndrome (ACS) episodes and premature death. Despite the clinical significance of a doctor's diagnosis of asthma in individuals with SCD, the criteria for a physician diagnosis of asthma are not well defined. Many features of asthma are common in individuals with SCD, including symptoms of wheezing, obstructive lung disease and airway hyper-responsiveness. However, it is not clear if these signs and symptoms of asthma reflect a physician diagnosis of asthma, or if these asthma features are related to SCD. Further complicating the diagnosis of asthma in children with SCD is the significant overlap in clinical manifestations between an asthma exacerbation and an ACS episode. Evidence supporting the concept that asthma and SCD are separate co-morbid conditions includes a similar prevalence of asthma between children with SCD and those in the general population and the observation that asthma is inherited in a familial pattern in the families of children with SCD. In contrast, there is significant evidence that asthma-like features may be associated with SCD without a diagnosis of asthma, including a higher than expected prevalence of airway hyper-responsiveness and obstructive lung disease. Regardless of whether SCD and asthma are distinct or overlapping co-morbid conditions, we recommend a systematic and complete evaluation of asthma when the diagnosis is suspected or when patients have multiple episodes of pain or ACS.
医生对患有镰状细胞病 (SCD) 的儿童和成人的哮喘诊断与疼痛和急性胸部综合征 (ACS) 发作以及过早死亡的发生率增加有关。尽管医生对 SCD 个体的哮喘诊断具有临床意义，但哮喘的医生诊断标准尚未明确界定。哮喘的许多特征在 SCD 个体中很常见，包括喘息、阻塞性肺疾病和气道高反应性的症状。然而，尚不清楚这些哮喘症状是否反映了医生对哮喘的诊断，或者这些哮喘特征是否与 SCD 有关。进一步使 SCD 儿童哮喘的诊断复杂化的是哮喘加重和 ACS 发作之间临床表现的显著重叠。支持哮喘和 SCD 是两种不同的合并症这一概念的证据包括 SCD 儿童与普通人群中哮喘的患病率相似，以及观察到哮喘在 SCD 儿童的家族中呈家族模式遗传。相比之下，有大量证据表明，即使没有哮喘的诊断，也可能与 SCD 相关的哮喘样特征，包括高于预期的气道高反应性和阻塞性肺疾病的患病率。无论 SCD 和哮喘是独立的还是重叠的合并症，我们建议在怀疑诊断或患者出现多次疼痛或 ACS 时，对哮喘进行系统和全面的评估。
