Abstract:
:Deficiency of mannose-binding lectin has been shown to be a risk factor for cystic fibrosis (CF) patients. We, therefore, decided to treat a patient with CF, mannose-binding lectin deficiency, severe bronchopulmonary Pseudomonas aeruginosa infection, and rapid deterioration of lung function with purified mannose-binding lectin in an attempt to ameliorate the course of the lung disease. The mannose-binding lectin used originated from pooled human donor plasma and was given as an intravenous infusion twice a week for a period of 3 months. The patients's clinical condition was stabilized during the treatment period, but was not improved. No adverse events were observed. However, the lung function assessed as percent forced expiratory volume in 1 sec (FEV1%) and percent forced vital capacirt (FVC%) correlated significantly with the mannose-binding serum lectin levels (rho=+0.68, P=0.008, and rho=+0.73, P=0.004). Additionally, an inverse correlation with the acute phase-reactant C-reactive protein and the proinflammatory cytokine IL-6 was observed (rho=-0.49, P=0.007 and rho=-0.41, P=0.04, respectively). The results emphasize the importance of mannose-binding lectin as a secondary disease modifier in CF. Moreover, purified mannose-binding lectin can safely be administered to chronically ill patients, and may be a potential treatment in CF and other diseases in which mannose-binding lectin deficiency plays a pathophysiological role.
journal_name
Pediatr Pulmonoljournal_title
Pediatric pulmonologyauthors
Garred P,Pressler T,Lanng S,Madsen HO,Moser C,Laursen I,Balstrup F,Koch C,Koch Cdoi
10.1002/ppul.10064subject
Has Abstractpub_date
2002-03-01 00:00:00pages
201-7issue
3eissn
8755-6863issn
1099-0496pii
10.1002/ppul.10064journal_volume
33pub_type
杂志文章abstract::The tidal flow volume (TFV) loop ratios of (1) time to peak flow (tPTEF) to total expiratory time (tE) [tPTEF/tE] and (2) volume to peak flow (VPTEF) to expired volume (VE) [VPTEF/VE] are reported to decrease with age in early life, and to decrease in subjects with obstructive airways disease (OAD). However, the mecha...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/(sici)1099-0496(199712)24:6<391::aid-ppul3
更新日期:1997-12-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21187
更新日期:2010-05-01 00:00:00
abstract::Our objective was to study meconium-induced lung injury in isolated perfused rat lungs exposed to anoxia. Our working hypothesis was that meconium-induced lung injury is independent of preexisting hypoxia, and that hypoxia will increase severity of lung injury observed after meconium aspiration. We compared five diffe...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.20123
更新日期:2005-04-01 00:00:00
abstract::The collection of sputum for microbiological examination in young cystic fibrosis patients can be very difficult. However, a knowledge of bacterial flora colonizing the patient's airways is of paramount importance for proper antimicrobial therapy. It is also known that cystic fibrosis patients colonized by Pseudomonas...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.10352
更新日期:2003-10-01 00:00:00
abstract:OBJECTIVE:Telomere length is associated with poorer lung health in older adults, possibly from cumulative risk factor exposure, but data are lacking in pediatric and population-based cohorts. We examined associations of telomere length with lung function in children and mid-life adults. METHODS:Data were drawn from a ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24489
更新日期:2019-12-01 00:00:00
abstract::In this article, we describe existing CF registries with a focus on US registry data collected through the CF Foundation Patient Registry (CFFPR) and the Epidemiologic Study of CF (ESCF); highlight what registries have taught us regarding epidemiology of CF; showcase the impact of registries on research and clinical c...
journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
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更新日期:2017-11-01 00:00:00
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journal_title:Pediatric pulmonology
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更新日期:2010-02-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
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更新日期:2013-10-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24250
更新日期:2019-04-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
doi:10.1002/ppul.24724
更新日期:2020-05-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.1950080308
更新日期:1990-01-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/ppul.24626
更新日期:2020-06-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24164
更新日期:2018-11-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.24709
更新日期:2020-05-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21454
更新日期:2011-09-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21609
更新日期:2012-07-01 00:00:00
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pub_type: 杂志文章
doi:10.1002/ppul.24974
更新日期:2020-10-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章,评审
doi:10.1002/(sici)1099-0496(200005)29:5<382::aid-ppul7
更新日期:2000-05-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.22532
更新日期:2013-01-01 00:00:00
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pub_type: 杂志文章
doi:10.1002/ppul.22766
更新日期:2013-07-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章
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更新日期:1994-11-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1002/ppul.1040
更新日期:2001-04-01 00:00:00
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pub_type: 杂志文章
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更新日期:2019-06-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23369
更新日期:2016-08-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章,随机对照试验
doi:10.1002/ppul.24144
更新日期:2018-10-01 00:00:00
abstract:UNLABELLED:SUMMARY RATIONALE: Asthma therapy should be stepped up or stepped down in response to changes in asthma control. However, there is little evidence available on the optimal timing, sequence, and degree of medication reductions. In this study we analyzed clinically stable asthmatic children who underwent a med...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.21278
更新日期:2010-09-01 00:00:00
abstract:OBJECTIVE:Medication adherence among adolescents with cystic fibrosis (CF) is often suboptimal and this has significant impact on their health and quality of life. The purpose of the study was to evaluate the impact of frequent home pulmonary function (PFT) monitoring on medication adherence among adolescents with CF. ...
journal_title:Pediatric pulmonology
pub_type: 杂志文章
doi:10.1002/ppul.23950
更新日期:2018-04-01 00:00:00
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journal_title:Pediatric pulmonology
pub_type: 杂志文章,多中心研究
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更新日期:2019-10-01 00:00:00