Persistent osteopenia in adult cystic fibrosis transmembrane conductance regulator-deficient mice.

Abstract:

RATIONALE:A loss of function mutation in the cystic fibrosis transmembrane conductance regulator gene is believed to be an independent risk factor for bone disease in patients with cystic fibrosis. OBJECTIVES:The objective of this work was to use congenic mice as a preclinical model to examine the bone phenotype of Cftr(-/-) mice and control littermates at 8, 12, and 28 weeks of age. METHODS:The bone phenotype of control and Cftr(-/-) mice was evaluated by quantitative imaging, histologic and histomorphometric analyses, and serum levels of bone biomarkers. MEASUREMENTS AND MAIN RESULTS:At 12 weeks of age, Cftr(-/-) mice were smaller, had lower bone mineral density, cortical bone thinning, and altered trabecular architecture compared with Cftr(+/+) or Cftr(+/-) control mice. In skeletally mature 28-week-old mice, there were persistent deficits in cortical and trabecular bone structure in Cftr(-/-) mice despite significant, quantifiable improvements. Cftr(-/-) mice also had lower serum insulin-like growth factor-I levels at 12 weeks of age than did control mice, whereas parathyroid hormone and 25-hydroxyvitamin D levels were not significantly different. CONCLUSIONS:Persistent osteopenia and structural abnormalities in adult Cftr(-/-) mice, in the absence of overt respiratory and gastrointestinal disease, suggest that loss of Cftr function has a direct impact on bone metabolism in Cftr(-/-) mice that is not sex specific or subject to haplotype insufficiency.

authors

Haston CK,Li W,Li A,Lafleur M,Henderson JE

doi

10.1164/rccm.200705-659OC

subject

Has Abstract

pub_date

2008-02-01 00:00:00

pages

309-15

issue

3

eissn

1073-449X

issn

1535-4970

pii

200705-659OC

journal_volume

177

pub_type

杂志文章
  • Ventilatory responses to passive leg motion in children with congenital central hypoventilation syndrome.

    abstract::During exercise, children with congenital central hypoventilation syndrome (CCHS) demonstrate coupling of VE to exercise load, despite the absence of a VE response to changes in FICO2. To assess the effect of movement on VE, we studied six CCHS patients and six matched controls during passive motion in a motor-driven ...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.153.2.8564130

    authors: Gozal D,Marcus CL,Ward SL,Keens TG

    更新日期:1996-02-01 00:00:00

  • Interleukin-8 messenger ribonucleic acid expression correlates with tumor progression, tumor angiogenesis, patient survival, and timing of relapse in non-small-cell lung cancer.

    abstract::Tumor-associated angiogenesis is important for tumor growth and metastasis. Interleukin (IL)-8 was recently reported to be an important angiogenic factor both in vitro and in vivo. In this study we evaluated, for the first time, IL-8 messenger RNA (mRNA) expression in non-small-cell lung cancer (NSCLC), using real-tim...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.162.5.2002108

    authors: Yuan A,Yang PC,Yu CJ,Chen WJ,Lin FY,Kuo SH,Luh KT

    更新日期:2000-11-01 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

  • Airway inflammation in nonasthmatic subjects with chronic cough.

    abstract::The physiopathology of chronic cough remains obscure. We evaluated the possibility that chronic cough in nonasthmatic subjects is associated with airway inflammation, and if this is so, what the relationship between this inflammation and the possible etiology of cough might be, as well as its response to inhaled stero...

    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.149.2.8306050

    authors: Boulet LP,Milot J,Boutet M,St Georges F,Laviolette M

    更新日期:1994-02-01 00:00:00

  • Granulocyte-macrophage colony-stimulating factor amplifies lipopolysaccharide-induced bronchoconstriction by a neutrophil- and cyclooxygenase 2-dependent mechanism.

    abstract::Granulocyte-macrophage colony-stimulating factor (GM-CSF) is used to ameliorate neutropenia in patients after antineoplastic treatment. It has also been suggested as an adjunct treatment in septic patients; however, the recruitment and priming of leukocytes by GM-CSF bears the hazard of a hyperinflammatory response. I...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.163.2.2004031

    authors: Wollin L,Uhlig S,Nüsing R,Wendel A

    更新日期:2001-02-01 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

  • Analysis of plasma-protein leakage and local secretion in sputum from patients with asthma and chronic obstructive pulmonary disease.

    abstract::In order to assess the usefulness of sputum analysis in studying plasma-protein exudation and local secretion of proteins in the airways, we measured specific proteins in the sputum sol phase (SSP) and sputum gel phase (SGP) from patients with stable asthma or chronic obstructive pulmonary disease (COPD). Protein leve...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.150.6.7952610

    authors: Schoonbrood DF,Lutter R,Habets FJ,Roos CM,Jansen HM,Out TA

    更新日期:1994-12-01 00:00:00

  • Human respiratory syncytial virus produces prolonged alterations of neural control in airways of developing ferrets.

    abstract::A dysfunction of pathways that normally cause contraction or relaxation of airways has been proposed to explain heightened levels of responsiveness produced by various insults to the airway. For example, we previously reported (4) that infection of cotton rats with the human respiratory syncytial virus (HRSV) leads to...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.157.5.9705026

    authors: Colasurdo GN,Hemming VG,Prince GA,Gelfand AS,Loader JE,Larsen GL

    更新日期:1998-05-01 00:00:00

  • Inhomogeneities of ventilation and the diffusing capacity to perfusion in various chronic lung diseases.

    abstract::Although impairment of gas exchange caused by ventilation-perfusion (VA/Q) mismatch has been extensively analyzed, there have been no systematic studies focused on determining the distributions of diffusion properties in dose connection with those of VA/Q. We attempted to clarify the simultaneous distributions of VA/Q...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.156.1.9607090

    authors: Yamaguchi K,Mori M,Kawai A,Takasugi T,Oyamada Y,Koda E

    更新日期:1997-07-01 00:00:00

  • Inhaled tryptase causes bronchoconstriction in sheep via histamine release.

    abstract::Allergen-induced bronchoconstriction involves mast cell activation. Tryptase is a mast cell serine protease that is released during this process, but little is known about the action of tryptase in the airway. The purpose of this study was to determine: (1) if aerosolized tryptase causes bronchoconstriction, and (2) t...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.154.3.8810600

    authors: Molinari JF,Scuri M,Moore WR,Clark J,Tanaka R,Abraham WM

    更新日期:1996-09-01 00:00:00

  • Hypoxic Pulmonary Vasoconstriction Does Not Explain All Regional Perfusion Redistribution in Asthma.

    abstract:RATIONALE:Regional hypoventilation in bronchoconstricted patients with asthma is spatially associated with reduced perfusion, which is proposed to result from hypoxic pulmonary vasoconstriction (HPV). OBJECTIVES:To determine the role of HPV in the regional perfusion redistribution in bronchoconstricted patients with a...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/rccm.201612-2438OC

    authors: Kelly VJ,Hibbert KA,Kohli P,Kone M,Greenblatt EE,Venegas JG,Winkler T,Harris RS

    更新日期:2017-10-01 00:00:00

  • Role of nasal interleukin-8 in neutrophil recruitment and activation in children with virus-induced asthma.

    abstract::Neutrophil infiltration is a major feature in the pathogenesis of the common cold, and respiratory viral infection is the major cause of asthma exacerbations. The factors regulating the neutrophil influx are unknown. Interleukin-8 (IL-8) is a potent neutrophil chemoattractant, which has been implicated in several infl...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.155.4.9105080

    authors: Teran LM,Johnston SL,Schröder JM,Church MK,Holgate ST

    更新日期:1997-04-01 00:00:00

  • One year treatment with salmeterol compared with beclomethasone in children with asthma. The Dutch Paediatric Asthma Study Group.

    abstract::The aim of this study was to compare the effects of salmeterol and beclomethasone on lung function and symptoms in children with mild to moderate asthma. Sixty-seven children not treated with inhaled corticosteroids were randomized in a double-blind parallel study either to salmeterol 50 micrograms b.i.d. or beclometh...

    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.156.3.9611067

    authors: Verberne AA,Frost C,Roorda RJ,van der Laag H,Kerrebijn KF

    更新日期:1997-09-01 00:00:00

  • Calcium channel blocker reduces airway remodeling in severe asthma. A proof-of-concept study.

    abstract:RATIONALE:Severe asthma is a major public health issue throughout the world. Increased bronchial smooth muscle (BSM) mass, a characteristic feature of airway remodeling in severe asthma, is associated with resistance to high-intensity treatment and poor prognosis. In vitro, the Ca(2+)-channel blocker gallopamil decreas...

    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.201410-1874OC

    authors: Girodet PO,Dournes G,Thumerel M,Begueret H,Dos Santos P,Ozier A,Dupin I,Trian T,Montaudon M,Laurent F,Marthan R,Berger P

    更新日期:2015-04-15 00:00:00

  • Smooth reference equations for slow vital capacity and flow-volume curve indexes.

    abstract::We derived reference values for slow vital capacity (VC) and flow-volume curve indexes (FVC, FEV(1), and flows) from the 1,185 tracings provided by 1,039 "normal" subjects who participated in one or both cross-sectional surveys of the Po River Delta study in 1980-1982 and in 1988-1991. Definition of "normal" was based...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.161.3.9906006

    authors: Pistelli F,Bottai M,Viegi G,Di Pede F,Carrozzi L,Baldacci S,Pedreschi M,Giuntini C

    更新日期:2000-03-01 00:00:00

  • DNA fingerprinting with two probes decreases clustering of Mycobacterium tuberculosis.

    abstract::DNA fingerprinting of Mycobacterium tuberculosis is used to study the epidemiology of tuberculosis, but the specificity of the widely used IS6110 technique has not been validated. Isolates from Denver, Colorado from December 1988 through June 1994 were fingerprinted with the IS6110 technique. Available records were re...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.155.3.9117000

    authors: Burman WJ,Reves RR,Hawkes AP,Rietmeijer CA,Yang Z,el-Hajj H,Bates JH,Cave MD

    更新日期:1997-03-01 00:00:00

  • A Disintegrin and A Metalloproteinase-9 (ADAM9): A Novel Proteinase Culprit with Multifarious Contributions to COPD.

    abstract:INTRODUCTION:Proteinases with a disintegrin and a metalloproteinase domain (ADAMs) have not been well studied in COPD. We investigated whether ADAM9 is linked to COPD in humans and mice. METHODS:ADAM9 blood and lung levels were measured in COPD patients versus controls, and air- versus cigarette smoke (CS)-exposed wil...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/rccm.201711-2300OC

    authors: Wang X,Polverino F,Rojas-Quintero J,Zhang D,Sánchez J,Yambayev I,Lindqvist E,Virtala R,Djukanovic R,Davies DE,Wilson S,O'Donnell R,Cunoosamy D,Hazon P,Higham A,Singh D,Olsson H,Owen CA

    更新日期:2018-06-04 00:00:00

  • Association study of the chromosomal region containing the FCER2 gene suggests it has a regulatory role in atopic disorders.

    abstract::On the basis of studies with animal models, the gene for the low-affinity receptor for immunoglobulin E (IgE) (FCER2, CD23) has been implicated as a candidate for IgE-mediated allergic diseases and bronchial hyperreactivity, or related traits. Given evidence for genetic complexity in atopic disorders, we sought to stu...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.161.3.9810056

    authors: Laitinen T,Ollikainen V,Lázaro C,Kauppi P,de Cid R,Antó JM,Estivill X,Lokki H,Mannila H,Laitinen LA,Kere J

    更新日期:2000-03-01 00:00:00

  • Lymphocyte subsets in bronchoalveolar lavage fluid of children without bronchopulmonary disease.

    abstract::Bronchoalveolar lavage (BAL) is increasingly used in the pediatric age group. However, normal values for BAL fluid (BALF) constituents are lacking. As part of an investigation to define reference values in children, we studied lymphocyte surface markers of BALF in 28 children 3 to 16 yr of age without bronchopulmonary...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.152.1.7599820

    authors: Ratjen F,Bredendiek M,Zheng L,Brendel M,Costabel U

    更新日期:1995-07-01 00:00:00

  • Epithelial Na(+) channel (ENaC) expression in the developing normal and abnormal human perinatal lung.

    abstract::Impaired lung epithelial Na(+) channel (ENaC) activity at the time of birth results in respiratory distress. To investigate potential mechanisms, the ontogeny and cellular distribution of the alphaENaC subunit mRNA expression was studied in normal, immature, and abnormal (hypoplastic) human fetal lungs using nonradioi...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.161.4.9905064

    authors: Smith DE,Otulakowski G,Yeger H,Post M,Cutz E,O'Brodovich HM

    更新日期:2000-04-01 00:00:00

  • Sputum metalloproteinase-9/tissue inhibitor of metalloproteinase-1 ratio correlates with airflow obstruction in asthma and chronic bronchitis.

    abstract::Asthma and chronic bronchitis are inflammatory diseases with extracellular matrix (ECM) remodeling and collagen deposition. Collagen homeostasis is controlled by metalloproteinases (MMPs) and tissue inhibitors of metalloproteinases (TIMPs). We evaluated MMP and TIMP balance in induced sputum of 10 control, 31 untreate...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.158.6.9803014

    authors: Vignola AM,Riccobono L,Mirabella A,Profita M,Chanez P,Bellia V,Mautino G,D'accardi P,Bousquet J,Bonsignore G

    更新日期:1998-12-01 00:00:00

  • Use of flow-volume curves in detecting secretions in ventilator-dependent patients.

    abstract::A noninvasive means of detecting airway secretions in ventilator-dependent patients is desirable because endotracheal suctioning can result in life-threatening complications. In a patient who had copious secretions, we observed a sawtooth pattern on his flow-volume curve that disappeared after suctioning. Accordingly,...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.150.3.8087350

    authors: Jubran A,Tobin MJ

    更新日期:1994-09-01 00:00:00

  • Gastric mucosal pH and blood flow during weaning from mechanical ventilation in patients with chronic obstructive pulmonary disease.

    abstract::To determine if gastric intramucosal pH changes during weaning from mechanical ventilation are related to gastric mucosal blood flow modifications, we studied 16 ventilator-supported patients with chronic obstructive pulmonary disease (COPD) who tolerated a 2-h trial of spontaneous breathing with pressure support vent...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.160.5.9901018

    authors: Bocquillon N,Mathieu D,Neviere R,Lefebvre N,Marechal X,Wattel F

    更新日期:1999-11-01 00:00:00

  • Mesenchymal stem cells reduce inflammation while enhancing bacterial clearance and improving survival in sepsis.

    abstract:RATIONALE:Sepsis refers to the clinical syndrome of severe systemic inflammation precipitated by infection. Despite appropriate antimicrobial therapy, sepsis-related morbidity and mortality remain intractable problems in critically ill patients. Moreover, there is no specific treatment strategy for the syndrome of seps...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/rccm.201001-0010OC

    authors: Mei SH,Haitsma JJ,Dos Santos CC,Deng Y,Lai PF,Slutsky AS,Liles WC,Stewart DJ

    更新日期:2010-10-15 00:00:00

  • Biomarkers of lung inflammation in recreational joggers exposed to ozone.

    abstract::Humans exhibit an acute inflammatory response in the lungs after controlled laboratory exposure to ozone. The present study was designed to test whether biomarkers of inflammation are detectable in humans exposed to ozone and associated copollutants under natural conditions outdoors. Bronchoscopy with bronchoalveolar ...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.154.5.8912760

    authors: Kinney PL,Nilsen DM,Lippmann M,Brescia M,Gordon T,McGovern T,El-Fawal H,Devlin RB,Rom WN

    更新日期:1996-11-01 00:00:00

  • Increased nitrotyrosine in exhaled breath condensate of patients with asthma.

    abstract::The reaction of nitric oxide (NO) and superoxide anions (O(2)(-)) in the airway results in the formation of peroxynitrite, a highly reactive oxidant species. Peroxynitrite reacts with tyrosine residues in proteins to form the stable product nitrotyrosine. We investigated whether nitrotyrosine in exhaled breath condens...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.162.4.9912064

    authors: Hanazawa T,Kharitonov SA,Barnes PJ

    更新日期:2000-10-01 00:00:00

  • Transfusion-related acute lung injury in the critically ill: prospective nested case-control study.

    abstract:RATIONALE:Acute lung injury (ALI) that develops 6 hours after transfusion (TRALI) is the leading cause of transfusion-related mortality. Several transfusion characteristics have been postulated as risk factors for TRALI, but the evidence is limited to retrospective studies. OBJECTIVES:To compare patient and transfusio...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/rccm.200702-271OC

    authors: Gajic O,Rana R,Winters JL,Yilmaz M,Mendez JL,Rickman OB,O'Byrne MM,Evenson LK,Malinchoc M,DeGoey SR,Afessa B,Hubmayr RD,Moore SB

    更新日期:2007-11-01 00:00:00

  • Serum Matrix Metalloproteinase-7, Respiratory Symptoms, and Mortality in Community-Dwelling Adults. MESA (Multi-Ethnic Study of Atherosclerosis).

    abstract:RATIONALE:Matrix metalloproteinase-7 (MMP-7) has been implicated in interstitial lung disease pathobiology and proposed as a diagnostic and prognostic biomarker of idiopathic pulmonary fibrosis. OBJECTIVES:To test associations between serum MMP-7 and lung function, respiratory symptoms, interstitial lung abnormalities...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/rccm.201701-0254OC

    authors: Armstrong HF,Podolanczuk AJ,Barr RG,Oelsner EC,Kawut SM,Hoffman EA,Tracy R,Kaminski N,McClelland RL,Lederer DJ

    更新日期:2017-11-15 00:00:00

  • Relationship between extent of pulmonary emphysema by high-resolution computed tomography and lung elastic recoil in patients with chronic obstructive pulmonary disease.

    abstract::We investigated the relationship between the extent of pulmonary emphysema, assessed by quantitative high-resolution computed tomography (HRCT), and lung mechanics in 24 patients with chronic obstructive pulmonary disease (COPD). The extent of emphysema was quantified as the relative lung area with CT numbers < -950 H...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/ajrccm.164.4.2010066

    authors: Baldi S,Miniati M,Bellina CR,Battolla L,Catapano G,Begliomini E,Giustini D,Giuntini C

    更新日期:2001-08-15 00:00:00

  • Respiratory symptoms in the first 7 years of life and birth weight at term: the PIAMA Birth Cohort.

    abstract:RATIONALE:The relation between birth weight and respiratory symptoms and asthma in children remains unclear. Previous studies focused on a relation at separate ages. A longitudinal analysis may lead to a better understanding. OBJECTIVES:To estimate the effect of birth weight on the development and course of respirator...

    journal_title:American journal of respiratory and critical care medicine

    pub_type: 杂志文章

    doi:10.1164/rccm.200610-1441OC

    authors: Caudri D,Wijga A,Gehring U,Smit HA,Brunekreef B,Kerkhof M,Hoekstra M,Gerritsen J,de Jongste JC

    更新日期:2007-05-15 00:00:00