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以色列囊性纤维化患者的咯血——患病率、治疗及临床特征

Hemoptysis in Israeli CF patients--prevalence, treatment, and clinical characteristics.

作者信息

Efrati Ori, Harash Oshrit, Rivlin Joseph, Bibi Haim, Meir Mei-Zahav, Blau Hannah, Mussaffi Huda, Barak Asher, Levy Isaac, Vilozni Daphna, Kerem Eitan, Modan-Moses Dalit

机构信息

Pediatric Pulmonology Unit, Safra Children's hospital, Tel Hashomer. Israel.

Pediatric Pulmonology Unit, Safra Children's hospital, Tel Hashomer. Israel.

出版信息

J Cyst Fibros. 2008 Jul;7(4):301-306. doi: 10.1016/j.jcf.2007.11.007. Epub 2008 Jan 9.

Abstract

OBJECTIVE

To identify the characteristics of CF patients with hemoptysis in Israel and to compare clinical features and risk factors to a control group of CF patients without hemoptysis.

DESIGN

Retrospective chart review.

PATIENTS

All CF patients in Israel who experienced hemoptysis between 2001 and 2005 and a control group of sex- and age-matched patients with no history of hemoptysis.

RESULTS

40/440 CF patients (9.1%) experienced hemoptysis during the study period, 50% were male. Ten patients (25%) were under 13 years old at the first hemoptysis episode. Pulmonary exacerbation was the precipitating factor in 90%. Twenty three patients showed moderate or major hemoptysis. 35/40 patients responded well to conservative therapy. Bronchial artery embolization (BAE) was performed in 5 patients with no recurrence of bleed within 24 h. However all of these patients experienced recurrent hemoptysis. One patient died during the follow-up period because of end stage lung disease. Pulmonary function tests, body-mass index, coagulation tests, pancreatic status, presence of bronchiectasis, sputum cultures and genetic mutations were similar in the two groups. A high incidence (57.5%) of associated diseases including cystic fibrosis related diabetes, cirrhosis and portal hypertension, and distal intestinal obstruction syndrome was found among hemoptysis patients, compared to only 5.2% in the control group (p<0.001).

CONCLUSIONS

Hemoptysis, even major, did not seem to be a risk factor for mortality in our patients. A higher incidence of hemoptysis was found in our pediatric patients compared to other series. BAE shows a high immediate rate of success in controlling hemoptysis, but does not prevent future disease.

摘要

目的

确定以色列咯血的囊性纤维化(CF)患者的特征,并将其临床特征和危险因素与无咯血的CF患者对照组进行比较。

设计

回顾性病历审查。

患者

2001年至2005年间在以色列经历咯血的所有CF患者,以及年龄和性别匹配且无咯血病史的患者对照组。

结果

在研究期间,440例CF患者中有40例(9.1%)出现咯血,其中50%为男性。10例患者(25%)在首次咯血发作时年龄小于13岁。90%的咯血由肺部加重引发。23例患者出现中度或大量咯血。40例患者中有35例对保守治疗反应良好。5例患者接受了支气管动脉栓塞术(BAE),术后24小时内无再次出血,但所有这些患者均出现复发性咯血。1例患者在随访期间因终末期肺病死亡。两组患者的肺功能测试、体重指数、凝血测试、胰腺状况、支气管扩张的存在、痰培养和基因突变情况相似。咯血患者中发现包括囊性纤维化相关糖尿病、肝硬化和门静脉高压以及远端肠梗阻综合征在内的相关疾病发生率较高(57.5%),而对照组仅为5.2%(p<0.001)。

结论

咯血,即使是大量咯血,在我们的患者中似乎并非死亡危险因素。与其他系列研究相比,我们的儿科患者咯血发生率更高。BAE在控制咯血方面显示出较高的即时成功率,但不能预防未来疾病。

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