Cystic fibrosis: relationship between clinical status and F508 deletion.
Cystic fibrosis: relationship between clinical status and F508 deletion.
复制标题
囊性纤维化：临床状态与 F508 缺失之间的关系。
DOI：
10.1016/s0022-3476(05)80490-1
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发表时间：
1991
期刊：
影响因子：
--
通讯作者：
Hazinski,TA
中科院分区：
文献类型：
--
作者：
Campbell3rd,PW;Phillips3rd,JA;Krishnamani,MR;Maness,KJ;Hazinski,TA
方法连续60例确诊为CF的患者（两次汗液氯化物试验阳性结果和相关的肺或胰腺受累）在范德比尔特囊性纤维化中心的常规门诊访视期间进行检查，该中心目前照顾98名患者，该中心于1990年5月结束;受试者包括31名男性和29名女性患者，年龄从12个月到25岁不等。从进入研究的每例患者或适当的亲属处获得知情同意书。在缺失分析之前，使用Doershuk等人修改的Shwachman和Kulczycki临床评分系统为每名受试者分配临床评分（通过PWC）。8该评分基于四项临床和放射学测量，范围在20（最差）和100（最佳）之间。如果患者不需要胰酶，没有脂肪代谢，并且3天粪便脂肪收集正常，则将其归类为胰腺充足。动态bp碱基对CF囊性纤维化FEF 25 -75最大流速介于肺活量的25%和75%之间
METHODS Sixty consecutive patients with confirmed CF (positive results on two sweat chloride tests and associated pulmonary or pancreatic involvement) were examined during routine clinic visits during a 5-month period ending May 1990 at the Vanderbilt Cystic Fibrosis Center, which currently cares for 98 patients; The subjects consisted of 31 male and 29 female patients who ranged in age from 12 months to 25 years. Informed consent was obtained from each patient who entered the study or from appropriate relatives. Before deletion analysis, each subject was assigned a clinical score (by PWC) with the use Of the Shwachman and Kulczycki clinical scoring system as modified by Doershuk et al. 8 This score is based on four clinical and radiographic measures and has a range between 20 (worst) and 100 (best). Patients were classified as pancreatic sufficient if they did not require pancreatic enzymes, had no steatorrhea, and had normal 3-day stool fat collections. Dynamic bp Base pair CF Cystic fibrosis FEF25-75 Maximum flow rate between 25% and 75% of vital capacity