Cystic fibrosis clinical score: A new scoring system to evaluate acute pulmonary exacerbation

Cystic fibrosis clinical score: A new scoring system to evaluate acute pulmonary exacerbation
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DOI:
10.1016/s0149-2918(99)80035-6
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发表时间:
1999-08-01
影响因子:
3.2
通讯作者:
Church, D
Church, D
中科院分区:
医学3区
文献类型:
--
作者:
Kanga, J;Kuhn, R;Church, D

文献摘要

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虽然肺功能测试被用来评估囊性纤维化(CF)患者的阻塞性呼吸道疾病的急性变化,但这些测试在幼儿或重症患者中相对困难,而且可能很昂贵。其他标准测试(如Shwachman-Kutczycki和美国国立卫生研究院[NIH]评分系统)评估疾病严重程度和预测预后,但不衡量临床状态的日常变化。因此,它们为评估急性肺加重的开始提供的信息很少。需要替代的评分系统来更好地识别肺恶化的开始,预测干预后呼吸功能的恶化或改善,并将评分与疾病严重程度评分区分开来。这项研究旨在比较一种新的10分、最高50分的急性临床评分系统与1秒用力呼气量(FEV)和用力肺活量(FVC)变量在开始抗菌治疗之前和治疗结束前经历急性肺恶化的CF儿童。130名5岁至17岁(中位年龄11岁)的儿童入院时NIH评分中位数与(39至85岁)相近。入院时的囊性纤维化临床评分(CFCS)与研究开始时改良的美国国立卫生研究院评分高度相关(r=-.68,P=0.0001)。入院时CFCs总量与FEV1(r=-0.57,P=0.0001)和FVC(r=-0.55,P=0.0001)呈负相关;裂纹、呼吸困难、痰和呼吸频率是与这两个肺功能变量中的任何一个最相关的4个成分。抗菌治疗前后CFCs的变化与FEV1的变化(r=-.31,P=0.0016)和FVC的变化(r=-.47,P=0.0001)也呈正相关。所有患者在治疗结束时都观察到了临床改善,只有1例患者的CFCs总量增加。临床复发的患者从治疗结束到2-4周的随访期间CFCs平均增加8.5分,表明急性加重的体征和症状正在恶化。这些数据表明,CFCs是评估CF患者健康状况的一个可预测的和可选的肺功能替代指标。在进一步验证后,该评分系统可以用于评估门诊环境中的健康状况、住院需求以及急性肺加重期间随后的改善情况,以及比较治疗方案的效果。
Although pulmonary function tests are used to evaluate acute changes in obstructive airway disease in patients with cystic fibrosis (CF), these tests are relatively difficult to perform in young children or severely ill patients and may be costly. Other standard tests (eg, the Shwachman-Kutczycki and National Institutes of Health [NIH] scoring systems) evaluate disease severity and predict prognosis but do not measure day-to-day changes in clinical status. They thus provide little information for assessing the start of acute pulmonary exacerbation. Alternative scoring systems are needed to better identify the start of pulmonary exacerbation, to predict worsening or improvement of respiratory function after intervention, and to distinguish the scores from illness severity scores. This study was undertaken to compare a new 10- component, 50-point-maximum, acute clinical scoring system with forced expiratory volume in 1 second (FEV,) and forced vital capacity (FVC) variables in children with CF who were experiencing an acute pulmonary exacerbation before antimicrobial therapy was initiated and until the end of therapy. One hundred thirty children aged 5 to 17 years (median age, 11 years) had a median NIH score of similar to 64 (range, 39 to 85)at admission. The cystic fibrosis clinical score (CFCS) at admission was found to correlate highly with the modified NIH score at study entry (r = -.68, P = 0.0001). The total CFCS at entry was correlated inversely with both FEV1 (r = -.57, P = 0.0001) and FVC (r = -.55, P = 0.0001) measurements; crackles, dyspnea, sputum production, and respiratory rate were the 4 components most highly associated with either pulmonary function variable. The change in total CFCS from start to end of antimicrobial therapy also correlated with changes in FEV1 (r = -.31, P = 0.0016) and change in FVC (r = -.47, P = 0.0001). Clinical improvement was observed in all patients at the end of therapy, and only 1 patient had an increase in total CFCS. Patients who experienced clinical relapse had a mean increase of 8.5 points in the CFCS from end of therapy to 2- to 4-week follow-up, indicating worsening signs and symptoms of acute exacerbation. These data suggest that the CFCS is a predictive and optional surrogate of pulmonary function in assessing the health of patients with CF. Following further validation, this scoring system could be used to evaluate health status in the outpatient setting, the need for hospitalization, and subsequent improvement during an acute pulmonary exacerbation, as well as to compare the efficacy of therapeutic regimens.