Ventilation inhomogeneities in relation to standard lung function in patients with cystic fibrosis.

Abstract:

:Based on serial lung function measurements performed in 142 children (68 males; 74 females) with cystic fibrosis (CF), prospectively evaluated over an age range of 6 to 20 years, we attempted to determine whether the lung clearance index (LCI) as a measure of ventilation inhomogeneities could be a discriminating factor of disease progression. Annual follow-up lung function measurements featuring FRC determined by whole-body plethysmography and multibreath nitrogen washouts, effective specific airway resistance, flow-volume curves, LCI, and gas exchange characteristics were analyzed by linear mixed-model analysis and Kaplan-Meier statistics. The earliest occurring and strongest factor of progression was the LCI, followed by maximal expiratory flow (MEF(50)) and FRC determined by plethysmography (p < 0.0001). Associations between onset of chronic Pseudomonas aeruginosa infection and CF transmembrane conductance regulator (CFTR) genotype with FEV(1) (p = 0.027) and FVC (p = 0.007) were identified. The study shows that the LCI predicts earlier in life and represented much better functional progression than FEV(1). Moreover, there is no single functional predictor of progression in CF, but aside from risk factors, such as onset of chronic P. aeruginosa infection and genotype, pulmonary hyperinflation, airway obstruction, and ventilation inhomogeneities are important pathophysiologic processes that should be evaluated concomitantly as determinants of lung progression in CF.

authors

Kraemer R,Blum A,Schibler A,Ammann RA,Gallati S

doi

10.1164/rccm.200407-948OC

keywords:

subject

Has Abstract

pub_date

2005-02-15 00:00:00

pages

371-8

issue

4

eissn

1073-449X

issn

1535-4970

pii

200407-948OC

journal_volume

171

pub_type

杂志文章
  • Calcitonin elevation in small cell lung cancer without ectopic production.

    abstract::To determine the relative contribution of ectopic calcitonin (CT) production versus nonectopic secretion of CT in patients with small cell lung cancer (SCLC), serum and urine immunoreactive CT (iCT) levels of 86 different subjects were measured by radioimmunoassay (RIA) using two polyclonal antisera (Ab3b and Ab4). Th...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.149.1.8111580

    authors: Kelley MJ,Becker KL,Rushin JM,Venzon D,Phelps R,Ihde DC,Bliss DP Jr,Melby K,Snider RH,Johnson BE

    更新日期:1994-01-01 00:00:00

  • Environmental exposure to crocidolite and mesothelioma: exposure-response relationships.

    abstract::This study aimed to estimate exposure-response relationships for mesothelioma and environmental exposure to crocidolite. All 4,659 former residents of Wittenoom, Western Australia (WA) who lived there between 1943 and 1993 for at least 1 mo and were not directly employed in the crocidolite industry, were followed-up t...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.157.1.96-11086

    authors: Hansen J,de Klerk NH,Musk AW,Hobbs MS

    更新日期:1998-01-01 00:00:00

  • Nosocomial acquisition of Burkholderia gladioli in patients with cystic fibrosis.

    abstract::Burkholderia gladioli has been reported as colonizing the airways of patients with cystic fibrosis (CF) but has not previously been associated with adverse outcome. We describe six patients with CF in whom the same strain of B. gladioli, on the basis of ribotyping and biochemical characteristics, was grown in their sp...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.155.4.9105090

    authors: Wilsher ML,Kolbe J,Morris AJ,Welch DF

    更新日期:1997-04-01 00:00:00

  • High concentrations of keratinocyte growth factor in airways of premature infants predicted absence of bronchopulmonary dysplasia.

    abstract::Premature lungs are highly susceptible to lung injuries, leading to bronchopulmonary dysplasia (BPD). Keratinocyte growth factor (KGF) is produced by the developing lung and may reduce the risk of BPD by preventing injury to the lung epithelium and enhancing its repair. To determine whether KGF concentrations in the a...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/rccm.200112-134BC

    authors: Danan C,Franco ML,Jarreau PH,Dassieu G,Chailley-Heu B,Bourbon J,Delacourt C

    更新日期:2002-05-15 00:00:00

  • Consequences of vascular flow on lung injury induced by mechanical ventilation.

    abstract::To investigate whether the magnitude of blood flow contributes to ventilator-induced lung injury, 14 sets of isolated rabbit lungs were randomized for perfusion at either 300 (Group A: n = 7) or 900 ml/ min (Group B: n = 7) while ventilated with 30 cm H2O peak static pressure. Control lungs (Group C: n = 7) were venti...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.157.6.9612006

    authors: Broccard AF,Hotchkiss JR,Kuwayama N,Olson DA,Jamal S,Wangensteen DO,Marini JJ

    更新日期:1998-06-01 00:00:00

  • Use of the child health questionnaire in a sample of moderate and low-income inner-city children with asthma.

    abstract::The Child Health Questionnaire (CHQ-PF50) is one of several recent efforts to gauge pediatric, health-related quality of life from the patient's (or parent's) perspective. Although tested extensively with healthy children, more information is needed about CHQ performance among children with chronic conditions such as ...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.162.4.2001067

    authors: Asmussen L,Olson LM,Grant EN,Landgraf JM,Fagan J,Weiss KB

    更新日期:2000-10-01 00:00:00

  • Alterations in lung mast cell populations in patients with chronic obstructive pulmonary disease.

    abstract:RATIONALE:Mast cells have important roles in innate immunity and tissue remodeling but have remained poorly studied in inflammatory airway diseases like chronic obstructive pulmonary disease (COPD). OBJECTIVES:To perform a detailed histological characterization of human lung mast cell populations at different severiti...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/rccm.200906-0932OC

    authors: Andersson CK,Mori M,Bjermer L,Löfdahl CG,Erjefält JS

    更新日期:2010-02-01 00:00:00

  • Fibrinogen levels and obstructive sleep apnea in ischemic stroke.

    abstract::The plasma level of fibrinogen is felt to be an independent risk factor for vascular events. Obstructive sleep apnea (OSA) has a high prevalence in patients with stroke and may also be an independent risk factor. The aim of our study was to determine the association between OSA and plasma levels of fibrinogen in patie...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.162.6.2001048

    authors: Wessendorf TE,Thilmann AF,Wang YM,Schreiber A,Konietzko N,Teschler H

    更新日期:2000-12-01 00:00:00

  • Interferon-γ production by neutrophils during bacterial pneumonia in mice.

    abstract:RATIONALE:Neutrophils are usually the first circulating leukocytes to respond during bacterial pneumonia. Their expression of oxidants, proteases, and other mediators present in granules is well documented, but their ability to produce mediators through transcription and translation after migration to an inflammatory s...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/rccm.201004-0592OC

    authors: Yamada M,Gomez JC,Chugh PE,Lowell CA,Dinauer MC,Dittmer DP,Doerschuk CM

    更新日期:2011-05-15 00:00:00

  • Bronchoalveolar lavage enzyme-linked immunospot for a rapid diagnosis of tuberculosis: a Tuberculosis Network European Trialsgroup study.

    abstract:RATIONALE:The rapid diagnosis of pulmonary tuberculosis (TB) is difficult when acid fast bacilli (AFB) cannot be detected in sputum smears. OBJECTIVES:Following a proof of principle study, we examined in routine clinical practice whether individuals with sputum AFB smear-negative TB can be discriminated from those wit...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 临床试验,杂志文章,多中心研究

    doi:10.1164/rccm.200904-0557OC

    authors: Jafari C,Thijsen S,Sotgiu G,Goletti D,Domínguez Benítez JA,Losi M,Eberhardt R,Kirsten D,Kalsdorf B,Bossink A,Latorre I,Migliori GB,Strassburg A,Winteroll S,Greinert U,Richeldi L,Ernst M,Lange C,Tuberculosis Network Eu

    更新日期:2009-10-01 00:00:00

  • Long-Term Outcomes after Protocolized Sedation versus Usual Care in Ventilated Pediatric Patients.

    abstract:RATIONALE:Whether a nurse-implemented goal-directed sedation protocol resulting in more awake yet calm intubated children affects postdischarge functional status, health-related quality of life, or risk for post-traumatic stress disorder is unknown. OBJECTIVES:To compare postdischarge outcomes in children with acute r...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章,随机对照试验

    doi:10.1164/rccm.201708-1768OC

    authors: Watson RS,Asaro LA,Hertzog JH,Sorce LR,Kachmar AG,Dervan LA,Angus DC,Wypij D,Curley MAQ,RESTORE Study Investigators and the Pediatric Acute Lung Injury and Sepsis Investigators (PALISI) Network.

    更新日期:2018-06-01 00:00:00

  • Gelsolin secretion in interleukin-4-treated bronchial epithelia and in asthmatic airways.

    abstract:RATIONALE:The airway surface liquid, the thin layer of liquid covering the airways, is essential for mucociliary clearance and as a barrier against microbial and other noxious agents. Proteins secreted into the airway surface liquid by epithelial and nonepithelial cells may be important in innate immunity and to improv...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/rccm.200409-1185OC

    authors: Candiano G,Bruschi M,Pedemonte N,Caci E,Liberatori S,Bini L,Pellegrini C,Viganò M,O'Connor BJ,Lee TH,Galietta LJ,Zegarra-Moran O

    更新日期:2005-11-01 00:00:00

  • Effect of Nintedanib in Subgroups of Idiopathic Pulmonary Fibrosis by Diagnostic Criteria.

    abstract:RATIONALE:In the absence of a surgical lung biopsy, patients diagnosed with idiopathic pulmonary fibrosis (IPF) in clinical practice could participate in the INPULSIS trials of nintedanib if they had honeycombing and/or traction bronchiectasis plus reticulation, without atypical features of usual interstitial pneumonia...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/rccm.201602-0402OC

    authors: Raghu G,Wells AU,Nicholson AG,Richeldi L,Flaherty KR,Le Maulf F,Stowasser S,Schlenker-Herceg R,Hansell DM

    更新日期:2017-01-01 00:00:00

  • Single-Cell Transcriptomic Analysis of Human Lung Provides Insights into the Pathobiology of Pulmonary Fibrosis.

    abstract::Rationale: The contributions of diverse cell populations in the human lung to pulmonary fibrosis pathogenesis are poorly understood. Single-cell RNA sequencing can reveal changes within individual cell populations during pulmonary fibrosis that are important for disease pathogenesis. Objectives: To determine whether s...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/rccm.201712-2410OC

    authors: Reyfman PA,Walter JM,Joshi N,Anekalla KR,McQuattie-Pimentel AC,Chiu S,Fernandez R,Akbarpour M,Chen CI,Ren Z,Verma R,Abdala-Valencia H,Nam K,Chi M,Han S,Gonzalez-Gonzalez FJ,Soberanes S,Watanabe S,Williams KJN,Flozak

    更新日期:2019-06-15 00:00:00

  • Tenascin mRNA expression at the foci of recent injury in usual interstitial pneumonia.

    abstract::To elucidate which cells are synthesizing tenascin in usual interstitial pneumonia (UIP) we have analyzed thoracoscopic or open lung biopsies from 30 patients with UIP by mRNA in situ hybridization, using (35)S-labeled tenascin RNA probes. The phenotype of the cells expressing tenascin mRNA was confirmed by immunohist...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.161.3.9809115

    authors: Pääkkö P,Kaarteenaho-Wiik R,Pöllänen R,Soini Y

    更新日期:2000-03-01 00:00:00

  • Airway epithelial transcription factor NK2 homeobox 1 inhibits mucous cell metaplasia and Th2 inflammation.

    abstract:RATIONALE:Airway mucous cell metaplasia and chronic inflammation are pathophysiological features that influence morbidity and mortality associated with asthma and other chronic pulmonary disorders. Elucidation of the molecular mechanisms regulating mucous metaplasia and hypersecretion provides the scientific basis for ...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/rccm.201101-0106OC

    authors: Maeda Y,Chen G,Xu Y,Haitchi HM,Du L,Keiser AR,Howarth PH,Davies DE,Holgate ST,Whitsett JA

    更新日期:2011-08-15 00:00:00

  • Relation between shunt, aeration, and perfusion in experimental acute lung injury.

    abstract:RATIONALE:In a pulmonary process characterized by spatially heterogeneous loss of aeration, the impairment of gas exchange is expected to depend on the regional distribution of perfusion relative to that of aeration. OBJECTIVES:To investigate how regional aeration, shunt, and perfusion are interrelated at different le...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/rccm.200703-484OC

    authors: Musch G,Bellani G,Vidal Melo MF,Harris RS,Winkler T,Schroeder T,Venegas JG

    更新日期:2008-02-01 00:00:00

  • Effects of glutathione S-transferase M1, maternal smoking during pregnancy, and environmental tobacco smoke on asthma and wheezing in children.

    abstract::The rise in childhood asthma prevalence suggests a role for environmental factors in the etiology of this evolving epidemic; however, genetics also influence the occurrence of asthma. Glutathione S-transferase (GST) M1 may play a role in asthma and wheezing occurrence among those exposed to tobacco smoke, as it functi...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/rccm.2112064

    authors: Gilliland FD,Li YF,Dubeau L,Berhane K,Avol E,McConnell R,Gauderman WJ,Peters JM

    更新日期:2002-08-15 00:00:00

  • Cell infiltration, ICAM-1 expression, and eosinophil chemotactic activity in asthmatic sputum.

    abstract::We have applied the technique of sputum induction by hypertonic saline in asthmatics and nonatopic control subjects to study an array of indices of airway inflammation believed to be relevant to asthma pathogenesis. Compatible with a central role for eosinophils and mast cells in asthma, sputum of asthmatic subjects c...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 临床试验,杂志文章

    doi:10.1164/ajrccm.155.2.9032180

    authors: Louis R,Shute J,Biagi S,Stanciu L,Marrelli F,Tenor H,Hidi R,Djukanović R

    更新日期:1997-02-01 00:00:00

  • Developmental changes in crossbridge properties and myosin isoforms in hamster diaphragm.

    abstract::The aim of this study was to determine the effects of maturation on crossbridge properties and myosin isoform composition in hamster diaphragm muscle. Diaphragm strips were obtained at postnatal Days 1 and 8 and in adults (10 to 12 wk). Peak isometric tension and maximum unloaded shortening velocity (Vmax) increased w...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.156.3.9701051

    authors: Coirault C,Lambert F,Joseph T,Blanc FX,Chemla D,Lecarpentier Y

    更新日期:1997-09-01 00:00:00

  • Derivation and validation of a composite index of severity in chronic obstructive pulmonary disease: the DOSE Index.

    abstract:RATIONALE:Chronic obstructive pulmonary disease (COPD) is increasingly recognized as a multicomponent disease with systemic consequences and effects on quality of life. Single measures such as lung function provide a limited reflection of how the disease affects patients. Composite measures have the potential to accoun...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/rccm.200902-0271OC

    authors: Jones RC,Donaldson GC,Chavannes NH,Kida K,Dickson-Spillmann M,Harding S,Wedzicha JA,Price D,Hyland ME

    更新日期:2009-12-15 00:00:00

  • Physiologic Responsiveness Should Guide Entry into Randomized Controlled Trials.

    abstract::Most randomized trials in critical care report no mortality benefit; this may reflect competing pathogenic mechanisms, patient heterogeneity, or true ineffectiveness of interventions. We hypothesize that in acute respiratory distress syndrome (ARDS), randomizing only those patients who show a favorable physiological r...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章,评审

    doi:10.1164/rccm.201410-1832CP

    authors: Goligher EC,Kavanagh BP,Rubenfeld GD,Ferguson ND

    更新日期:2015-12-15 00:00:00

  • Reactivity to IgE-dependent histamine-releasing activity in asthma or rhinitis.

    abstract::We investigated the relation between IgE reactive with histamine-releasing factor (HRF) and clinical status in patients with asthma or rhinitis. Sera were used to passively sensitize purified, lactic-acid treated basophils. IgE-independent HRF due to chemokines was removed from mononuclear cell supernatants with hepar...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.154.2.8756800

    authors: Pasmans SG,Aalbers M,van der Veen MJ,Knol EF,van der Zee JS,Jansen HM,Aalberse RC

    更新日期:1996-08-01 00:00:00

  • Inhaled PGE2 prevents aspirin-induced bronchoconstriction and urinary LTE4 excretion in aspirin-sensitive asthma.

    abstract::Bronchial overproduction of leukotrienes and inhibition of prostaglandin synthesis are involved in the pathogenesis of aspirin-induced asthma. We investigated whether inhaled prostaglandin E2 (PGE2) attenuates the response to bronchial challenge with lysine acetylsalicylate (LASA) and the associated increase in urinar...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1164/ajrccm.153.2.8564100

    authors: Sestini P,Armetti L,Gambaro G,Pieroni MG,Refini RM,Sala A,Vaghi A,Folco GC,Bianco S,Robuschi M

    更新日期:1996-02-01 00:00:00

  • Elevation of exhaled ethane concentration in asthma.

    abstract::Ethane is a product of lipid peroxidation as a result of oxidative stress and can be detected in the exhaled air. Oxidative stress plays a role in the pathogenesis of asthma. We measured exhaled ethane in 26 asthmatic subjects (mean age +/- SEM, 38 +/- 8 yr; 15 male, FEV(1) 60 +/- 4%) and compared it with exhaled nitr...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.162.4.2003064

    authors: Paredi P,Kharitonov SA,Barnes PJ

    更新日期:2000-10-01 00:00:00

  • Pneumonia in HIV-infected persons: increased risk with cigarette smoking and treatment interruption.

    abstract:RATIONALE:Bacterial pneumonia is a major cause of morbidity for HIV-infected persons and contributes to excess mortality in this population. OBJECTIVES:To evaluate the frequency and risk factors for occurrence of bacterial pneumonia in the present era of potent antiretroviral therapy. METHODS:We evaluated data from a...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章,随机对照试验

    doi:10.1164/rccm.200804-617OC

    authors: Gordin FM,Roediger MP,Girard PM,Lundgren JD,Miro JM,Palfreeman A,Rodriguez-Barradas MC,Wolff MJ,Easterbrook PJ,Clezy K,Slater LN

    更新日期:2008-09-15 00:00:00

  • The upper respiratory tract as a microbial source for pulmonary infections in cystic fibrosis. Parallels from island biogeography.

    abstract::A continuously mixed series of microbial communities inhabits various points of the respiratory tract, with community composition determined by distance from colonization sources, colonization rates, and extinction rates. Ecology and evolution theory developed in the context of biogeography is relevant to clinical mic...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/rccm.201312-2129PP

    authors: Whiteson KL,Bailey B,Bergkessel M,Conrad D,Delhaes L,Felts B,Harris JK,Hunter R,Lim YW,Maughan H,Quinn R,Salamon P,Sullivan J,Wagner BD,Rainey PB

    更新日期:2014-06-01 00:00:00

  • Acute Respiratory Distress Syndrome Neutrophils Have a Distinct Phenotype and Are Resistant to Phosphoinositide 3-Kinase Inhibition.

    abstract:RATIONALE:Acute respiratory distress syndrome is refractory to pharmacological intervention. Inappropriate activation of alveolar neutrophils is believed to underpin this disease's complex pathophysiology, yet these cells have been little studied. OBJECTIVES:To examine the functional and transcriptional profiles of pa...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/rccm.201509-1818OC

    authors: Juss JK,House D,Amour A,Begg M,Herre J,Storisteanu DM,Hoenderdos K,Bradley G,Lennon M,Summers C,Hessel EM,Condliffe A,Chilvers ER

    更新日期:2016-10-15 00:00:00

  • Specific HLA in pulmonary MAC infection in a Japanese population.

    abstract::Patients with pulmonary Mycobacterium avium complex (MAC) infection occasionally have neither past histories of pulmonary diseases nor underlying immunodeficiency conditions. Therefore, we hypothesized that MAC may be linked with a disease-susceptibility gene and determined human leukocyte-associated antigens (HLA) in...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.162.1.9908071

    authors: Takahashi M,Ishizaka A,Nakamura H,Kobayashi K,Nakamura M,Namiki M,Sekita T,Okajima S

    更新日期:2000-07-01 00:00:00

  • Correlation of structure and function in idiopathic pulmonary fibrosis.

    abstract::The early stage of idiopathic pulmonary fibrosis (IPF) is thought to involve a smaller number of alveoli and to be characterized predominantly by cellularity and minimal fibrosis, whereas advanced disease involves a large number of alveoli and is characterized predominantly by fibrosis with minimal cellularity. In add...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm/151.4.1180

    authors: Cherniack RM,Colby TV,Flint A,Thurlbeck WM,Waldron JA Jr,Ackerson L,Schwarz MI,King TE Jr

    更新日期:1995-04-01 00:00:00