Improvement of medical care in a cohort of newborns with sickle-cell disease in North Paris: impact of national guidelines
Improvement of medical care in a cohort of newborns with sickle-cell disease in North Paris: impact of national guidelines
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DOI：
10.1111/bjh.14015
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发表时间：
2016-06-01
影响因子：
6.5
通讯作者：
Benkerrou, Malika
中科院分区：
文献类型：
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作者：
Couque, Nathalie;Girard, Delphine;Benkerrou, Malika
我们对1995-2009年出生的镰状细胞病（SCD）新生儿进行了回顾性研究，随后在多中心医院网络中进行。我们评估了患者的结果，医疗护理和遵守2005年12月公布的国家指南。分析了1033例患者（742例SS/S度-地中海贫血）的数据，随访6776患者年（平均年龄71 ± 39岁）。SCD相关死亡（n=13）仅发生在中位年龄为231个月的SS基因型患者中，主要是由于急性贫血（n=5，包括2例急性脾隔离）和感染（n=3）。治疗不依从性与SCD相关死亡风险增加10倍相关（P= 0.01）。所有卒中患者（n=12）、几乎所有经颅多普勒（TCD）异常患者（n=76）或急性胸部综合征> 1例/终生（n=64）和/或3例严重血管闭塞性危象/年（n=100）的患者均接受了强化治疗。只有2/3的患者基线血红蛋白
We conducted a retrospective study on newborns with sickle-cell disease (SCD), born 1995-2009, followed in a multicentre hospital-based network. We assessed patient outcomes, medical care and compliance with the national guidelines published in December 2005. Data from 1033 patients (742 SS/S degrees-thalassaemia) with 6776 patient-years of follow-up were analysed (mean age 71 +/- 39years). SCD-related deaths (n=13) occurred only in SS-genotype patients at a median age of 231months, mainly due to acute anaemia (n=5, including 2 acute splenic sequestrations) and infection (n=3). Treatment non-compliance was associated with a 10-fold higher risk of SCD-related death (P=001). Therapeutic intensification was provided for all stroke patients (n=12), almost all patients with abnormal transcranial Doppler (TCD) (n=76) or with >1acute chest syndrome/lifetime (n=64) and/or 3 severe vaso-occlusive crises/year (n=100). Only 2/3 of patients with baseline haemoglobin