Department of Pediatrics, School of Medical Sciences, University of Campinas, P.O. Box: 6111, Campinas, SP, 13081-970, Brazil.
Department of Medical Genetics, School of Medical Sciences, University of Campinas, P.O. Box: 6111, Campinas, SP, 13081-970, Brazil.
Med Microbiol Immunol. 2017 Dec;206(6):447-461. doi: 10.1007/s00430-017-0521-2. Epub 2017 Sep 30.
The Burkholderia cepacia complex (BCC) can cause a severe decline in lung function in cystic fibrosis (CF). Our objective was to determine the BCC prevalence and to evaluate its clinical impact on CF. Clinical and laboratory variables were determined for CF patients with BCC (Group-A = 50 patients) and without BCC (Group-B = 134 patients). The microorganisms were identified by biochemical tests, the Vitek2Compact test, recA-PCR and recA-nested-PCR with species-specific primers and DNA sequencing. The patients were evaluated by the Shwachman-Kulczycki score (SKCS), Bhalla score (BS), spirometry and body mass index (BMI). The BCC prevalence was 22.5%. The most common species were Burkholderia multivorans (30%), Burkholderia cepacia (24%), Burkholderia cenocepacia IIIA (10%), B. cenocepacia IIIB (2%) and Burkholderia vietnamiensis (2%). There was difference between the groups in nutritional status (p = 0.02) and general activity (p = 0.026). There was difference in total BS points (p = 0.04) and the following parameters: bronchiectasis severity (p = 0.007), peribronchial thickening (p = 0.013), bronchiectasis extent (p = 0.01) and general aspects of the affected bronchial zone (p = 0.02). The respiratory disorder classifications were as follows: obstructive-4.8% (Group-A) and 23.8% (Group-B); restrictive-9.5% (Group-A and Group-B); obstructive + restrictive-19% (Group-A) and 1.6% (Group-B); and obstructive + restrictive with a decreased forced expiratory flow-47.6% (Group-A) and 30.2% (Group-B) (p = 0.02). Nutritional status was a minor contributing factor to weight, height and BMI in the Group-A (p = 0.02). The BCC prevalence, particularly the prevalence of B. multivorans, was higher in this study. The SKCS, BS, spirometry and nutritional status results showed that BCC has a negative impact on clinical status. Phenotypic methods are useful for the identification of presumptive BCC. The Vitek2Compact test showed accuracy in BCC identification. PCR, nested-PCR, and recA sequencing showed specificity in BCC species identification.
洋葱伯克霍尔德菌复合群（BCC）可导致囊性纤维化（CF）患者的肺功能严重下降。本研究旨在确定 BCC 的流行率，并评估其对 CF 的临床影响。对患有 BCC 的 CF 患者（A 组=50 例）和无 BCC 的 CF 患者（B 组=134 例）进行临床和实验室变量测定。采用生化试验、Vitek2Compact 试验、recA-PCR 和 recA 巢式-PCR 联合种特异性引物和 DNA 测序对微生物进行鉴定。通过 Shwachman-Kulczycki 评分（SKCS）、Bhalla 评分（BS）、肺量测定和体重指数（BMI）对患者进行评估。BCC 的流行率为 22.5%。最常见的物种是伯克霍尔德菌多噬亚种（30%）、洋葱伯克霍尔德菌（24%）、洋葱伯克霍尔德菌 Cenocepacia IIIA（10%）、Cenocepacia IIIB（2%）和越南伯克霍尔德菌（2%）。两组间在营养状况（p=0.02）和一般活动能力（p=0.026）方面存在差异。总 BS 评分（p=0.04）和以下参数存在差异：支气管扩张严重程度（p=0.007）、支气管周围增厚（p=0.013）、支气管扩张程度（p=0.01）和受累支气管区域的一般方面（p=0.02）。呼吸障碍分类如下：阻塞性-4.8%（A 组）和 23.8%（B 组）；限制性-9.5%（A 组和 B 组）；阻塞性+限制性-19%（A 组）和 1.6%（B 组）；阻塞性+限制性伴呼气流量降低-47.6%（A 组）和 30.2%（B 组）（p=0.02）。营养状况是 A 组体重、身高和 BMI 的次要影响因素（p=0.02）。BCC 的流行率，特别是伯克霍尔德菌多噬亚种的流行率，在本研究中更高。SKCS、BS、肺量测定和营养状况结果表明，BCC 对临床状况有负面影响。表型方法有助于鉴定推定的 BCC。Vitek2Compact 试验在 BCC 鉴定方面具有准确性。PCR、巢式 PCR 和 recA 测序在 BCC 种鉴定方面具有特异性。
