Treatment patterns in people with cystic fibrosis: have they changed since the introduction of ivacaftor?
Treatment patterns in people with cystic fibrosis: have they changed since the introduction of ivacaftor?
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DOI：
10.1016/j.jcf.2021.08.014
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发表时间：
2022-03
影响因子：
5.2
通讯作者：
Keogh, Ruth H.
中科院分区：
文献类型：
--
作者：
Granger, Emily;Davies, Gwyneth;Keogh, Ruth H.
在英国接受ivacaftor治疗的囊性纤维化人群中，纵向治疗模式与当代未接受治疗的个体队列中观察到的不同，这是由于他们的基因型，尽管在引入ivacaftor之前基因型组之间的差异很小。与未接受ivacaftor治疗的人相比，接受ivacaftor治疗的人不太可能继续其他治疗，如吸入抗生素，dornase alfa，高渗盐水，慢性口服抗生素和补充喂养。依伐卡托治疗和非依伐卡托治疗人群之间使用阿法链道酶和高渗盐水的差异对于肺功能较高的人群更大。2012年底，ivacaftor在英国开始用于6岁及以上患有G551 D突变的囊性纤维化（CF）患者。治疗模式的长期变化以前没有报道过。我们研究了服用依伐卡托的G551 D突变CF患者的长期治疗模式，并使用英国囊性纤维化登记处将其与非依伐卡托治疗的队列进行了比较。使用2007-2018年的数据，我们比较了四个队列之间的治疗模式：1：依伐卡托治疗; 2：依伐卡托时代（2013-2018年），不合格的基因型（无G551 D突变）; 3：前依伐卡托时代（2007-2012年），合格的基因型（G551 D突变）; 4：前依伐卡托时代，不合格的基因型。治疗包括：吸入抗生素、阿法链核酸酶、高渗盐水、长期口服抗生素和补充喂养。截至2012年，接受每种治疗的人的百分比在按基因型定义的两个队列之间相似，并且倾向于以相似的斜率逐年增加。一旦引入依伐卡托，依伐卡托治疗队列中其他治疗的使用倾向于逐年减少或保持稳定，而非依伐卡托治疗队列中则保持稳定或增加。这导致了ivacaftor时代两个队列之间治疗使用的差异，随着时间的推移变得更加明显。自从在CF中广泛使用的一些关键治疗中引入ivacaftor以来，我们已经显示出治疗模式的明显分歧。需要进一步的研究来调查治疗模式的差异是否与健康结果的变化有关。
Longitudinal treatment patterns among the ivacaftor-treated cystic fibrosis population in the UK differ from those seen in a contemporary cohort of individuals untreated due to their genotype, despite minimal differences between the genotype groups prior to the introduction of ivacaftor. People who are treated with ivacaftor were less likely to continue other treatments such as inhaled antibiotics, dornase alfa, hypertonic saline, chronic oral antibiotics and supplementary feeding, compared to people who are not treated with ivacaftor. The differences in use of dornase alfa and hypertonic saline solution between ivacaftor-treated and non-ivacaftor-treated people, are larger for people with higher lung function. In late 2012, ivacaftor became available in the UK for people with cystic fibrosis (CF) aged 6 years and over with a G551D mutation. Long-term changes in treatment patterns have not previously been reported. We investigated long-term treatment patterns in people with CF with a G551D mutation who took ivacaftor and compared these with non-ivacaftor-treated cohorts using the UK Cystic Fibrosis Registry. Using 2007-2018 data we compared treatment patterns between four cohorts: 1: ivacaftor-treated; 2: ivacaftor era (2013-2018), ineligible genotype (no G551D mutation); 3: pre-ivacaftor era (2007-2012), eligible genotype (G551D mutation); 4: pre-ivacaftor era, ineligible genotype. Treatments included: inhaled antibiotics, dornase alfa, hypertonic saline, chronic oral antibiotics and supplementary feeding. Up to 2012 the percentages of people taking each treatment were similar between the two cohorts defined by genotype and tended to increase by year with a similar slope. Once ivacaftor was introduced, the use of other treatments tended to decrease or remain stable by year for the ivacaftor-treated cohort, whereas it remained stable or increased in the non-ivacaftor-treated cohort. This led to differences in treatment use between the two cohorts in the ivacaftor-era, which became more marked over time. We have shown a clear divergence in treatment patterns since the introduction of ivacaftor in a number of key treatments widely used in CF. Further research is needed to investigate whether the differences in treatment patterns are associated with changes in health outcomes.
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3-95 岁年龄范围的多种族肺活量测定参考值：2012 年全球肺功能方程。
DOI：
10.1183/09031936.00080312
发表时间：
2012-12
期刊：
The European respiratory journal
影响因子：
--
作者：
Quanjer PH;Stanojevic S;Cole TJ;Baur X;Hall GL;Culver BH;Enright PL;Hankinson JL;Ip MS;Zheng J;Stocks J;ERS Global Lung Function Initiative
通讯作者：
ERS Global Lung Function Initiative
影响因子：
3.6
作者：
Quittner A;Suthoff E;Rendas-Baum R;Bayliss MS;Sermet-Gaudelus I;Castiglione B;Vera-Llonch M
通讯作者：
Vera-Llonch M
影响因子：
5.2
作者：
Davies, Gwyneth;Rowbotham, Nicola J.;Smyth, Alan R.
通讯作者：
Smyth, Alan R.
影响因子：
7.7
作者：
Taylor-Robinson D;Archangelidi O;Carr SB;Cosgriff R;Gunn E;Keogh RH;MacDougall A;Newsome S;Schlüter DK;Stanojevic S;Bilton D;CF-EpinNet collaboration
通讯作者：
CF-EpinNet collaboration
影响因子：
5.2
作者：
Volkova, Nataliya;Moy, Kristin;Bilton, Diana
通讯作者：
Bilton, Diana