Improved survival of children and adolescents with sickle cell disease
Improved survival of children and adolescents with sickle cell disease
复制标题
DOI：
10.1182/blood-2009-07-233700
复制
发表时间：
2010-04-29
期刊：
影响因子：
20.3
通讯作者：
Buchanan, George R.
中科院分区：
文献类型：
--
作者：
Quinn, Charles T.;Rogers, Zora R.;Buchanan, George R.
患有镰状细胞病 (SCD) 的幼儿的生存率有所改善，但对年龄较大的儿童和青少年的了解较少。我们研究了达拉斯新生儿队列 (DNC)，以估计患有 SCD 的新生儿当代 18 年生存率，并记录死亡原因和年龄随时间的变化。我们还探讨了医疗保健质量的改善是否与生存暂时相关。 DNC 目前包括 940 名受试者，并进行了 8857 患者年的随访。大多数患有镰状细胞性贫血的儿童 (93.9%) 和几乎所有患有轻度 SCD 的儿童 (98.4%) 现在都能活到成年。死亡发生率和死亡率模式随着队列的持续时间而变化。败血症不再是死亡的主要原因。该队列中最近发生的所有死亡均发生在 18 岁或以上的患者中，大多数是在过渡到成人护理后不久。随着时间的推移，民主党全国委员会的护理质量不断提高，对幼儿的初次就诊和预防性干预措施明显更加及时。总之，大多数患有 SCD 的儿童现在都能度过童年，但过渡到成人医疗护理的年轻人早逝的风险很高。 （血。2010；115（17）：3447-3452）
The survival of young children with sickle cell disease (SCD) has improved, but less is known about older children and adolescents. We studied the Dallas Newborn Cohort (DNC) to estimate contemporary 18-year survival for newborns with SCD and document changes in the causes and ages of death over time. We also explored whether improvements in the quality of medical care were temporally associated with survival. The DNC now includes 940 subjects with 8857 patient-years of follow-up. Most children with sickle cell anemia (93.9%) and nearly all children with milder forms of SCD (98.4%) now live to become adults. The incidence of death and the pattern of mortality changed over the duration of the cohort. Sepsis is no longer the leading cause of death. All the recent deaths in the cohort occurred in patients 18 years or older, most shortly after the transition to adult care. Quality of care in the DNC has improved over time, with significantly more timely initial visits and preventive interventions for young children. In summary, most children with SCD now survive the childhood years, but young adults who transition to adult medical care are at high risk for early death. (Blood. 2010; 115(17): 3447-3452)