3,777 results match your criteria Restrictive Lung Disease


Comment on 'Fatal, incidental, idiopathic pulmonary fibrosis in a patient receiving long-term low-dose methotrexate for psoriasis': reply from author.

Clin Exp Dermatol 2019 Apr 24. Epub 2019 Apr 24.

Respiratory Medicine, Ninewells Hospital, Dundee, UK.

There appears to be some overlap in pathogenetic mechanisms between psoriasis and idiopathic pulmonary fibrosis e.g. raised IL-17; indeed, the possibility of a restrictive form of psoriatic lung disease has been raised although it appears to be rare. Read More

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http://dx.doi.org/10.1111/ced.13993DOI Listing

Dyspnoea and restrictive lung disease due to mediastinal and pleural lipomatosis in morbid obesity.

Respirol Case Rep 2019 Jul 9;7(5):e00421. Epub 2019 Apr 9.

Department of Respiratory Medicine Waikato Hospital Hamilton New Zealand.

Dyspnoea in obese patients can be multifactorial and complex. Mediastinal and pleural lipomatosis can be associated with obesity and is usually considered asymptomatic and benign. We report an obese 39-year-old man who presented with progressive dyspnoea, where in addition to obstructive sleep apnoea and obesity hypoventilation syndrome, was found to have massive mediastinal and pleural lipomatosis causing restrictive lung disease. Read More

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https://onlinelibrary.wiley.com/doi/abs/10.1002/rcr2.421
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http://dx.doi.org/10.1002/rcr2.421DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6454808PMC
July 2019
1 Read

Pleuroparenchymal fibroelastosis (PPFE) treated with lung transplantation and review of the literature.

BMJ Case Rep 2019 Apr 20;12(4). Epub 2019 Apr 20.

Department of Medicine, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.

A 26-year-old woman presented with a 15-year history of non-progressive dyspnoea. Chest imaging showed bilateral apical pleural and parenchymal scarring, pleural thickening and bronchiectasis. Pulmonary function tests showed a moderate restrictive defect. Read More

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http://dx.doi.org/10.1136/bcr-2019-229402DOI Listing

Clinico-radiologic features of pleuroparenchymal fibroelastosis in children.

Pediatr Radiol 2019 Apr 19. Epub 2019 Apr 19.

Department of Pediatric Radiology, Texas Children's Hospital, 6701 Fannin St., Houston, TX, 77030, USA.

Background: Pleuroparenchymal fibroelastosis (PPFE) may be underdiagnosed clinically and radiographically in children with a remote history of cancer, leading to a delay in care and unnecessary lung biopsies.

Objective: To describe the characteristic clinical and radiologic findings of PPFE in a cohort of children to facilitate recognition and noninvasive diagnosis.

Materials And Methods: Clinical presentation, history of chemotherapy or radiation therapy, lung or bone marrow transplantation, and lung function testing and outcome were retrospectively extracted from the electronic medical records of eight children treated at our institution's pulmonary medicine clinic with histopathology confirmation of PPFE from 2008 to 2018. Read More

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http://link.springer.com/10.1007/s00247-019-04405-5
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http://dx.doi.org/10.1007/s00247-019-04405-5DOI Listing
April 2019
2 Reads

Non-invasive techniques to assess restrictive lung disease in workers exposed to free crystalline silica.

Med Lav 2019 Apr 19;110(2):83-92. Epub 2019 Apr 19.

Department of Medicine and Surgery, University of Parma, Via Gramsci 14, 43126 Parma, Italy.

Objectives: To compare the reliability of spirometry and body plethysmography in detecting restrictive lung disease in clay excavation workers exposed to free crystalline silica (FCS). The exhaled breath condensate (EBC) biomarkers of oxidative stress were also assessed in order to evaluate early lung damage.

Methods: The study involved 62 workers (58 males and 4 females) at a company that extracts and processes clay. Read More

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http://dx.doi.org/10.23749/mdl.v110i2.7471DOI Listing
April 2019
2 Reads

Heart failure in cardiomyopathies: a position paper from the Heart Failure Association of the European Society of Cardiology.

Eur J Heart Fail 2019 Apr 16. Epub 2019 Apr 16.

Berlin-Brandenburg Center for Regenerative Therapies, Deutsches Zentrum für Herz-Kreislauf-Forschung (DZHK) Berlin, Department of Cardiology, Campus Virchow Klinikum, Charite - Universitaetsmedizin Berlin, Berlin, Germany.

Cardiomyopathies are a heterogeneous group of heart muscle diseases and an important cause of heart failure (HF). Current knowledge on incidence, pathophysiology and natural history of HF in cardiomyopathies is limited, and distinct features of their therapeutic responses have not been systematically addressed. Therefore, this position paper focuses on epidemiology, pathophysiology, natural history and latest developments in treatment of HF in patients with dilated (DCM), hypertrophic (HCM) and restrictive (RCM) cardiomyopathies. Read More

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http://dx.doi.org/10.1002/ejhf.1461DOI Listing
April 2019
2 Reads
6.526 Impact Factor

Lung function measurements in the prediabetes stage: data from the ILERVAS Project.

Acta Diabetol 2019 Apr 15. Epub 2019 Apr 15.

Endocrinology and Nutrition Department, Hospital Universitari Vall d'Hebron, Diabetes and Metabolism Research Unit, Vall d'Hebron Institut de Recerca (VHIR), Universitat Autònoma de Barcelona, Barcelona, Catalonia, Spain.

Aims: Patients with type 2 diabetes have been considered a susceptible group for pulmonary dysfunction. Our aim was to assess pulmonary function on the prediabetes stage.

Methods: Pulmonary function was assessed in 4,459 non-diabetic subjects, aged between 45 and 70 years, without cardiovascular disease or chronic pulmonary obstructive disease from the ongoing study ILERVAS. Read More

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http://link.springer.com/10.1007/s00592-019-01333-6
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http://dx.doi.org/10.1007/s00592-019-01333-6DOI Listing
April 2019
3 Reads

CP*Trends: An Online Tool to Compare Cohort and Period Trends Across Cancer Sites.

Am J Epidemiol 2019 Apr 16. Epub 2019 Apr 16.

Division of Cancer Control and Populations sciences, National Cancer Institute, Bethesda, Maryland.

Cohort or period components of trend can provide a rationale for new research or point to clues of effectiveness for control strategies. Graphical display of trends guides models that quantify the experience of a population. A method for smoothing rates by single years of age and year are developed and displayed to show the contribution of period and cohort on trend. Read More

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https://academic.oup.com/aje/advance-article/doi/10.1093/aje
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http://dx.doi.org/10.1093/aje/kwz089DOI Listing
April 2019
2 Reads

A 1-Month Physical Therapy-Based Outpatient Program for Adults Awaiting Lung Transplantation: A Retrospective Analysis of Exercise Capacity, Symptoms, and Quality of Life.

Cardiopulm Phys Ther J 2019 Apr;30(2):61-69

Duke University School of Medicine, Departments of Orthopaedic Surgery, Medicine, Cell Biology.

Purpose: Rehabilitation can improve health outcomes in candidates for lung transplantation. The purpose of this study was to retrospectively evaluate the effect of a one-month physical therapy (PT)-based outpatient program on exercise capacity, symptoms, quality of life and examine predictors of functional outcome changes in adults awaiting lung transplantation.

Methods: Participants (n=141) completed a 23-session exercise and educational program over one month. Read More

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http://dx.doi.org/10.1097/CPT.0000000000000087DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6456901PMC
April 2019
1 Read

Pulmonary Langerhans cell histiocytosis in cats and a literature review of feline histiocytic diseases.

J Feline Med Surg 2019 Apr 12:1098612X19842384. Epub 2019 Apr 12.

1 Department of Veterinary Pathology, College of Veterinary Medicine, Federal University of Rio Grande do Sul, Porto Alegre, Brazil.

Objectives: The aim of this study was to report the clinical, radiographic and pathological features of pulmonary Langerhans cell histiocytosis in four cats, and carry out a literature review of feline histiocytic diseases.

Methods: Necropsy reports archived at the Department of Veterinary Pathology were reviewed. The clinical information was then obtained from the clinical records at the Veterinary Hospital. Read More

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http://dx.doi.org/10.1177/1098612X19842384DOI Listing
April 2019
1 Read

Forced Oscillation Technique and Small Airway Involvement in Chronic Hypersensitivity Pneumonitis.

Arch Bronconeumol 2019 Apr 8. Epub 2019 Apr 8.

Divisao de Pneumologia, Instituto do Coracao (InCor), Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Sao Paulo, Brazil.

Objective: Hypersensitivity pneumonitis (HP) is an interstitial lung disease caused by the inhalation of specific organic antigens or low-molecular weight substances in genetically susceptible individuals. Although small airway involvement is prominent in patients with chronic HP, conventional pulmonary function tests (PFTs) are relatively insensitive to identify it. Thus, the authors aimed to evaluate resistance (R5) and reactance (X5) values at 5Hz on inspiration, expiration, and whole breath, as well as small airway resistance (R) values using a forced oscillation technique (FOT) in patients with chronic HP, and their responses after bronchodilator. Read More

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http://dx.doi.org/10.1016/j.arbres.2019.01.022DOI Listing
April 2019
1 Read

Oxygen Availability in Respiratory Muscles During Exercise in Children Following Fontan Operation.

Front Pediatr 2019 26;7:96. Epub 2019 Mar 26.

Department of Pediatric Cardiology and Congenital Heart Defects, German Heart Centre, Munich, Germany.

As survival of previously considered as lethal congenital heart disease forms is the case in our days, issues regarding quality of life including sport and daily activities emerge. In patients with Fontan circulation, there is no pump to propel blood into the pulmonary arteries since the systemic veins are directly connected to the pulmonary arteries. The complex hemodynamics of Fontan circulation include atrial function, peripheral muscle pump, integrity of the atrioventricular valve, absence of restrictive, or obstructive pulmonary lung function. Read More

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http://dx.doi.org/10.3389/fped.2019.00096DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6443956PMC
March 2019
1 Read

A Nonsmoker Man in His 40s With a Diagnosis of Genetic-Related Idiopathic Pulmonary Fibrosis (Surfactant-Protein C Gene Mutation).

Chest 2019 Apr;155(4):e91-e96

Department of Cardiac-Thoracic-Vascular Sciences and Public Health, University of Padova, Padova, Italy. Electronic address:

A nonsmoker man in his 40s underwent bilateral lung transplantation with a referral diagnosis of genetic-related idiopathic pulmonary fibrosis (IPF). The patient had no medical history in childhood and early adulthood, nor was there a family history of IPF. His nonsmoker father presented with lung cancer at 59 years of age. Read More

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http://dx.doi.org/10.1016/j.chest.2018.12.015DOI Listing
April 2019
2 Reads

Chronic lung allograft dysfunction: light at the end of the tunnel?

Curr Opin Organ Transplant 2019 Apr 1. Epub 2019 Apr 1.

Department of Chronic Diseases, Metabolism and Aging, KU Leuven.

Purpose Of Review: Chronic lung allograft dysfunction (CLAD) has been recently introduced as an umbrella-term encompassing all forms of chronic pulmonary function decline posttransplant with bronchiolitis obliterans syndrome and restrictive allograft syndrome as the most important subtypes. Differential diagnosis and management, however, remains complicated.

Recent Findings: Herein, we provide an overview of the different diagnostic criteria (pulmonary function, body plethysmography and radiology) used to differentiate bronchiolitis obliterans syndrome and restrictive allograft syndrome, their advantages and disadvantages as well as potential problems in making an accurate differential diagnosis. Read More

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http://dx.doi.org/10.1097/MOT.0000000000000632DOI Listing
April 2019
7 Reads

Isolated pulmonary interstitial glycogenosis associated with alveolar growth abnormalities: A long-term follow-up study.

Pediatr Pulmonol 2019 Mar 25. Epub 2019 Mar 25.

Pediatric Allergy, Pulmonary and Cystic Fibrosis Section, Hospital Universitari Vall d'Hebron, Barcelona, Spain.

Introduction: Pulmonary interstitial glycogenosis (PIG) is a rare infant interstitial lung disease characterized by an increase in the number of interstitial mesenchymal cells, presenting as enhanced cytoplasmic glycogen, and is considered to represent the expression of an underlying lung development disorder.

Methods: This study describes the clinical, radiological, and functional characteristics and long-term outcomes (median 12 years) of nine infants diagnosed with isolated PIG associated with alveolar simplification in the absence of other diseases.

Results: All patients presented with tachypnea. Read More

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http://dx.doi.org/10.1002/ppul.24324DOI Listing
March 2019
1 Read

Pleuroparenchymal fibroelastosis secondary to autologous hematopoietic stem cell transplantation: A case report.

Exp Ther Med 2019 Apr 13;17(4):2557-2560. Epub 2019 Feb 13.

Department of Pulmonary and Critical Care Medicine, Center of Respiratory Medicine, China-Japan Friendship Hospital, Beijing 100029, P.R. China.

Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease. Although an increased number of PPFE cases have been reported recently, the characteristics of this condition have not been well described. The present study reports on the case of a 34-year-old male patient who presented with unilateral lung abnormalities. Read More

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http://www.spandidos-publications.com/10.3892/etm.2019.7275
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http://dx.doi.org/10.3892/etm.2019.7275DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6425129PMC
April 2019
8 Reads

Respiratory dysfunction following initiation of mirabegron: A case report.

Respir Med Case Rep 2019 16;26:304-306. Epub 2019 Feb 16.

Northwestern University Feinberg School of Medicine, Division of Pulmonary and Critical Care Medicine, 240 East Huron Street, McGaw Pavilion, Suite M-349 Chicago, Illinois 60611, USA.

Background: Mirabegron, a β3 adrenergic receptor agonist, is FDA approved for treatment of overactive bladder. Approved in 2012 in the US, there have been no reports of any effects of mirabegron on pulmonary function.

Case Presentation: We report the case of a 65 year old male with a history of Parkinson's disease, OSA, and aspiration pneumonia presenting with subacute worsening dyspnea and found to have worsening restrictive ventilatory defect with a pattern consistent with neuromuscular weakness. Read More

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http://dx.doi.org/10.1016/j.rmcr.2019.02.012DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6402286PMC
February 2019
1 Read

Case report: new development of fibrosing interstitial lung disease triggered by HIV-related pneumocystis pneumonia.

BMC Pulm Med 2019 Mar 18;19(1):65. Epub 2019 Mar 18.

AIDS Clinical Center, National Center of Global Health and Medicine, 1-21-1 Toyama, Shinjuku-ku, Tokyo, 162-8655, Japan.

Background: Fibrosing interstitial lung disease is the poor prognostic non-infectious lung disease by unknown etiology. Here, we present one case developing interstitial pneumonia with fibrosis after treatment of pneumocystis pneumonia (PCP) in newly diagnosed HIV-1 infected case.

Case Presentation: A previously healthy 63-year old male was referred to our institute because of protracted dyspnea on effort in 2 weeks after pneumocystis pneumonia treatment. Read More

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http://dx.doi.org/10.1186/s12890-019-0831-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6423812PMC
March 2019
3 Reads

Predictive factors of obstructive sleep apnoea in patients with fibrotic lung diseases.

Sleep Med 2019 Jan 26. Epub 2019 Jan 26.

Department of Pneumology of São João Hospital Center, Oporto, Portugal; Respiratory Sleep Disorders Unit, Department of Pneumology of São João Hospital Center, Oporto, Portugal.

Background And Aim: Several studies reported a high prevalence of Obstructive Sleep Apnoea (OSA) in patients with Idiopathic Pulmonary Fibrosis (IPF) or restrictive end-stage lung disease (ESLD). Besides the known risk factors for OSA like high Body Mass Index (BMI), reduced static and dynamic volumes for IPF patients and reduced DLCO and low minimal O saturation during sleep for ESLD patients were associated with higher Apnoea-Hypopnoea Index (AHI). The aim of our study was to determine potential predictive factors of OSA in patients with Fibrotic Lung Diseases (FLD). Read More

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http://dx.doi.org/10.1016/j.sleep.2019.01.020DOI Listing
January 2019
1 Read

Impaired pulmonary function and its association with clinical outcomes, exercise capacity and quality of life in children with congenital heart disease.

Int J Cardiol 2019 Jun 1;285:86-92. Epub 2019 Mar 1.

Paediatric and Congenital Cardiology Department, M3C Regional Reference Centre, Montpellier University Hospital, Montpellier, France; Center for Studies and Research on Health Services and Quality of Life, Aix-Marseille University, Marseille, France; PhyMedExp, University of Montpellier, INSERM, CNRS, France. Electronic address:

Background: Impaired pulmonary function is an independent predictor of mortality in adult congenital heart disease (CHD), but has been scarcely studied in the paediatric CHD population.

Aims: To compare the pulmonary function of children with CHD to healthy controls, and evaluate its association with clinical outcomes, exercise capacity, and quality of life.

Methods: Cross-sectional multicentre study among 834 children (555 CHD and 279 control subjects) who underwent a complete spirometry and a cardiopulmonary exercise test (CPET). Read More

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http://dx.doi.org/10.1016/j.ijcard.2019.02.069DOI Listing
June 2019
3 Reads

Manifestation of congenital pulmonary airway malformation in a 26-year-old adult, requiring surgery.

Interact Cardiovasc Thorac Surg 2019 Mar 7. Epub 2019 Mar 7.

Department of Thoracic Surgery, Kyoto University Hospital, Kyoto, Japan.

This is a case of type 1 congenital pulmonary airway malformation in the left lower lobe of a 26-year-old male. At the age of 1 year, he developed a pulmonary cystic lesion, which was considered to be bronchopulmonary sequestration. He grew up healthy and showed no impairment during exercise; however, giant bullous lesion development along with compressed left upper lobe and mediastinum was recently noted; consequently, the patient was referred to our hospital for further examination. Read More

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http://dx.doi.org/10.1093/icvts/ivz032DOI Listing
March 2019
2 Reads

Pulmonary function abnormalities are common in patients with multiple myeloma and are independently associated with worse outcome.

Ann Hematol 2019 Mar 5. Epub 2019 Mar 5.

Department of Clinical Therapeutics, School of Medicine, National and Kapodistrian University of Athens, 80 Vasilissis Sofias, 11258, Athens, Greece.

Pre-existing pulmonary disease may affect treatment choices, toxicity, and survival of patients with multiple myeloma (MM). However, data on the prognostic value of pulmonary function tests (PFTs) in myeloma patients' outcome, at the time of initial assessment of newly diagnosed patients, are scarce. Here, we prospectively performed PFTs in 121 newly diagnosed MM patients, before initiation of treatment, and we evaluated possible associations of lung function with their outcomes. Read More

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http://dx.doi.org/10.1007/s00277-019-03641-xDOI Listing
March 2019
10 Reads

Is early detection of late-onset Pompe disease a pneumologist's affair? A lesson from an Italian screening study.

Orphanet J Rare Dis 2019 Mar 4;14(1):62. Epub 2019 Mar 4.

Respiratory Pathophysiology and Intensive Care Unit, Department of Cardio-Thoracic, University-City Hospital of Padova, Padova, Italy.

Background: Late-onset Pompe disease (LOPD) is a recessive disease caused by α-glucosidase (GAA) deficiency, leading to progressive muscle weakness and/or respiratory failure in children and adults. Respiratory derangement can be the first indication of LOPD, but the diagnosis may be difficult for pneumologists. We hypothesize that assessing the GAA activity in suspected patients by a dried blood spot (DBS) may help the diagnosis of LOPD in the pneumological setting. Read More

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http://dx.doi.org/10.1186/s13023-019-1037-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6399888PMC
March 2019
1 Read
3.358 Impact Factor

The pleural mesothelium and transforming growth factor-β pathways in restrictive allograft syndrome: A pre-clinical investigation.

J Heart Lung Transplant 2019 Feb 6. Epub 2019 Feb 6.

Leuven Lung Transplant Group, Department of Chronic Diseases, Metabolism & Ageing, KU Leuven, Leuven, Belgium. Electronic address:

Background: Chronic lung allograft dysfunction (CLAD) hampers long-term survival after lung transplantation. Common fibrosis-related mechanisms in idiopathic pulmonary fibrosis and CLAD instigated the consideration of investigating the differential regulation of pleural mesothelium and transforming growth factor-β (TGF-β) in restrictive allograft syndrome (RAS).

Methods: TGF-β was assessed in bronchoalveolar lavage (BAL) fluid using enzyme-linked immunoassay and via immune staining of explant biopsies. Read More

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http://dx.doi.org/10.1016/j.healun.2019.02.001DOI Listing
February 2019
1 Read

Prevalence of lung function impairment in cured pulmonary tuberculosis patients in Cotonou, Benin.

Int J Tuberc Lung Dis 2019 Feb;23(2):195-202

Bordeaux Population Health Centre, Centre Institut national de la Santé et de la Recherche médicale Unité 1219, Université de Bordeaux, Bordeaux, France.

Setting: National teaching hospital for the management of respiratory diseases, Cotonou, Benin.

Objective: 1) To estimate the prevalence of lung function impairment (LFI) and associated factors in patients cured of pulmonary tuberculosis (PTB); and 2) to determine the link between human immunodeficiency virus (HIV) infection and LFI occurrence.

Design: We performed a cross-sectional study in cured patients with smear-positive TB (PTB+) treated between 2012 and 2015. Read More

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http://dx.doi.org/10.5588/ijtld.18.0234DOI Listing
February 2019
4 Reads

Study of some pulmonary function tests in Egyptian children with end-stage renal disease under regular hemodialysis in correlation with dialysis duration.

Saudi J Kidney Dis Transpl 2019 Jan-Feb;30(1):119-128

Department of Pediatrics, Faculty of Medicine, Tanta University, Tanta City, Gharbia Governate, Egypt.

Pulmonary function tests can differentiate between obstructive and restrictive lung diseases and assess the severity of the disease in children. The aim of work was to study pulmonary function tests in children with end-stage renal disease (ESRD) undergoing hemodialysis (HD) and its correlation with dialysis duration. This study was conducted on 40 patients with ESRD on regular HD for at least six months selected from the Pediatric Nephrology unit of Pediatric Department of Tanta university hospital and 40 healthy children as a control group. Read More

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February 2019
1 Read

Lung disease in STAT3 hyper-IgE syndrome requires intense therapy.

Allergy 2019 Feb 21. Epub 2019 Feb 21.

Department of Pediatrics, Dr. von Hauner Children's Hospital, Ludwig Maximilian University, Munich, Germany.

Background: Pulmonary complications are responsible for high morbidity and mortality rates in patients with the rare immunodeficiency disorder STAT3 hyper-IgE syndrome (STAT3-HIES). The aim of this study was to expand knowledge about lung disease in STAT3-HIES.

Methods: The course of pulmonary disease, radiological and histopathological interrelations, therapeutic management, and the outcome of 14 STAT3-HIES patients were assessed. Read More

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http://dx.doi.org/10.1111/all.13753DOI Listing
February 2019
2 Reads
6.028 Impact Factor

Severe Restrictive Lung Disease in One of the Oldest Documented Males With Coffin-Lowry Syndrome.

J Investig Med High Impact Case Rep 2019 Jan-Dec;7:2324709618820660

2 Rio Bravo Family Medicine Residency Program, Bakersfield, CA, USA.

Coffin-Lowry syndrome is expressed as different phenotypes in males and females. In males, it is characterized by facial abnormalities, marked developmental disability, and skeletal changes. Approximately 80% of cases are associated with kyphoscoliosis, which can be quite severe, as seen in our patient, causing paraplegia and restrictive lung disease. Read More

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http://dx.doi.org/10.1177/2324709618820660DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6350156PMC
February 2019
1 Read

Cell-Free DNA and CXCL10 Derived from Bronchoalveolar Lavage Predict Lung Transplant Survival.

J Clin Med 2019 Feb 13;8(2). Epub 2019 Feb 13.

Department of Surgery, University of California San Francisco, San Francisco, CA 94143, USA.

Standard methods for detecting chronic lung allograft dysfunction (CLAD) and rejection have poor sensitivity and specificity and have conventionally required bronchoscopies and biopsies. Plasma cell-free DNA (cfDNA) has been shown to be increased in various types of allograft injury in transplant recipients and CXCL10 has been reported to be increased in the lung tissue of patients undergoing CLAD. This study used a novel cfDNA and CXCL10 assay to evaluate the noninvasive assessment of CLAD phenotype and prediction of survival from bronchoalveolar lavage (BAL) fluid. Read More

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http://dx.doi.org/10.3390/jcm8020241DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6406976PMC
February 2019

Pulmonary functions in Egyptian children with transfusion-dependent β-thalassemia.

Transfus Med 2019 Feb 18;29(1):55-60. Epub 2019 Feb 18.

Pediatric Department, Faculty of Medicine, Minia University, El-Minya, Egypt.

Background: In β-thalassemia, there are varying degrees of ineffective haematopoiesis, intermittent haemolysis and iron overload. Excess iron is deposited in organs such as the heart, the liver, the endocrine glands and the lungs.

Objectives: To evaluate the pulmonary functions in asymptomatic beta thalassemic children on regular transfusion therapy and their relation to iron overload. Read More

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http://dx.doi.org/10.1111/tme.12539DOI Listing
February 2019
2 Reads

NK and NKT cells in the diagnosis of diffuse lung diseases presenting with a lymphocytic alveolitis.

BMC Pulm Med 2019 Feb 13;19(1):39. Epub 2019 Feb 13.

Department of Pulmonology, Hospital de São João and Faculty of Medicine, University of Porto, Porto, Portugal.

Background: Diffuse lung diseases (DLD) are characterized by different immunophenotypes in the bronchoalveolar lavage fluid (BALF). We aimed to evaluate the diagnostic value of BALF NK and NKT cell counts of patients with DLD and lymphocytic alveolitis.

Methods: We assessed 202 patients with DLD, who underwent BALF immunophenotyping. Read More

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http://dx.doi.org/10.1186/s12890-019-0802-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6373142PMC
February 2019
2 Reads

Back to the Box: Using Lung Volumes to Predict Susceptibility to Develop Chronic Obstructive Pulmonary Disease among Smokers.

Ann Am Thorac Soc 2018 Dec;15(Supplement_4):S286-S287

University of California San Francisco and San Francisco Veterans Affairs Medical Center, San Francisco, California.

Background: Abnormal lung volumes that represent air trapping are common in chronic obstructive pulmonary disease (COPD). However, their clinical significance in those without spirometrically defined COPD (normal forced expiratory volume in 1 s [FEV] to forced vital capacity [FVC] ratio) is unclear.

Methods: Using the Veterans Administration Informatics and Computing Infrastructure (VINCI) database, we identified 7,479 patients at risk for COPD (smokers > 40 years of age without restrictive lung disease) who had preserved spirometry (FEV/FVC and FEV ≥ lower limit of normal) and also had concomitant lung volume measurements by plethysmography across 37 Veterans Affairs Medical Centers in the United States between 1985 and 2017, and examined their subsequent health records for clinical diagnoses of COPD, respiratory exacerbations, healthcare utilization, spirometry, and mortality. Read More

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http://dx.doi.org/10.1513/AnnalsATS.201807-475MGDOI Listing
December 2018
3 Reads

Postoperative respiratory failure in liver transplantation: Risk factors and effect on prognosis.

PLoS One 2019 11;14(2):e0211678. Epub 2019 Feb 11.

Università Cattolica del Sacro Cuore, Rome, Italy.

Background: Postoperative respiratory failure (PRF, namely mechanical ventilation >48 hours) significantly affects morbidity and mortality in liver transplantation (LTx). Previous studies analyzed only one or two categories of PRF risk factors (preoperative, intraoperative or postoperative ones). The aims of this study were to identify PRF predictors, to assess the length of stay (LoS) in ICU and the 90-day survival according to the PRF in LTx patients. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0211678PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6370207PMC
February 2019
6 Reads

Impact of neurological level and spinal curvature on pulmonary function in adults with spina bifida.

J Pediatr Rehabil Med 2018 ;11(4):243-254

Human Engineering Research Laboratories, Veterans Affairs Pittsburgh Healthcare System, Pittsburgh, PA, USA.

Purpose: To describe pulmonary function and determine the impact of neurological level, scoliosis, and obesity on pulmonary function in people with spina bifida (SB).

Methods: Participants with SB (N= 29) (15 females; age, 30 ± 12 years) completed spirometry and body plethysmographic lung volume testing. Univariate and multivariate regression analyses were used to describe the factors associated with pulmonary function in people with SB. Read More

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http://dx.doi.org/10.3233/PRM-179451DOI Listing
January 2018
6 Reads

Prospective observational study in patients with obstructive lung disease: NOVELTY design.

ERJ Open Res 2019 Feb 1;5(1). Epub 2019 Feb 1.

School of Biological Sciences, University of Manchester, Manchester, UK.

Asthma and chronic obstructive pulmonary disease (COPD) have overlapping clinical features and share pathobiological mechanisms but are often considered distinct disorders. Prospective, observational studies across asthma, COPD and asthma-COPD overlap are limited. NOVELTY is a global, prospective observational 3-year study enrolling ∼12 000 patients ≥12 years of age from primary and specialist clinical practices in 19 countries (ClinicalTrials. Read More

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http://dx.doi.org/10.1183/23120541.00036-2018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6355976PMC
February 2019
4 Reads

Phenotypical diversity of airway morphology in chronic lung graft vs. host disease after stem cell transplantation.

Mod Pathol 2019 Feb 5. Epub 2019 Feb 5.

Lung Transplant Unit, Department of Chronic diseases, Metabolism and Aging, KU Leuven, Leuven, Belgium.

Pulmonary graft vs. host disease is a diverse and underestimated complication following allogenic hematopoietic stem cell transplantation. We aimed to compare the airway architecture with chronic lung allograft dysfunction post lung transplantation. Read More

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http://dx.doi.org/10.1038/s41379-019-0203-2DOI Listing
February 2019
2 Reads

Interstitial lung abnormalities: erecting fences in the path towards advanced pulmonary fibrosis.

Thorax 2019 May 5;74(5):506-511. Epub 2019 Feb 5.

Interstitial lung abnormalities, when present in members of undiagnosed family members recruited on the basis of familial interstitial pneumonia, or in undiagnosed research participants, have been associated with a syndrome that includes distinct sets of imaging abnormalities, restrictive physiological and exercise impairments, and an increased prevalence of histopathological findings, and genetic predictors, that have been noted in patients with idiopathic pulmonary fibrosis. Recent longitudinal studies have demonstrated that qualitative and quantitative assessments of interstitial abnormalities are associated with accelerated lung function decline, an increased rate of clinical diagnoses of interstitial lung disease and an increased rate of mortality. In this perspective, in addition to reviewing the prior information, four major efforts that could help the field of early pulmonary fibrosis detection move forward are discussed. Read More

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http://dx.doi.org/10.1136/thoraxjnl-2018-212446DOI Listing
May 2019
3 Reads

An SFTPC BRICHOS mutant links epithelial ER stress and spontaneous lung fibrosis.

JCI Insight 2019 Mar 21;4(6). Epub 2019 Mar 21.

Pulmonary, Allergy, and Critical Care Division, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.

Alveolar type 2 (AT2) cell endoplasmic reticulum (ER) stress is a prominent feature in adult and pediatric interstitial lung disease (ILD and ChILD), but in vivo models linking AT2 cell ER stress to ILD have been elusive. Based on a clinical ChILD case, we identified a critical cysteine residue in the surfactant protein C gene (SFTPC) BRICHOS domain whose mutation induced ER stress in vitro. To model this in vivo, we generated a knockin mouse model expressing a cysteine-to-glycine substitution at codon 121 (C121G) in the Sftpc gene. Read More

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http://dx.doi.org/10.1172/jci.insight.126125DOI Listing
March 2019
5 Reads

"Small small interventions, big big roles"- a qualitative study of patient, care-giver and health-care worker experiences of a palliative care programme in Kerala, India.

BMC Palliat Care 2019 Feb 4;18(1):16. Epub 2019 Feb 4.

Department of Community Medicine, Government T.D Medical College Alappuzha, Vandanam P.O, Alappuzha, Kerala, India.

Background: Home-based palliative care is an essential resource for many communities. We conducted a qualitative study to explore perceptions of a home-based palliative care programme in Kerala, India, from the perspective of patients, their care-givers and the doctors, nurses and volunteers running the intervention.

Methods: A descriptive qualitative study was carried out. Read More

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http://dx.doi.org/10.1186/s12904-019-0400-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6362568PMC
February 2019
2 Reads

Comparative study of mid-thoracic spinal versus epidural anesthesia for open nephrectomy in patients with obstructive/restrictive lung disease: A randomized controlled study.

Saudi J Anaesth 2019 Jan-Mar;13(1):52-59

Department of Anesthesia, Faculty of Medicine, Cairo University, Cairo, Egypt.

Background: The aim of this randomized controlled study is to compare the safety and efficacy of thoracic spinal versus thoracic epidural anesthesia for open nephrectomy in patients with obstructive/restrictive lung disease.

Methods: Sixty patients with mild to moderate chronic obstructive/restrictive lung disease undergoing open nephrectomy were randomized into two groups, 30 patients each. The thoracic spinal group (TSA) group received ultrasound guided mid-thoracic spinal anesthesia, and the thoracic epidural group (TEA) group received thoracic epidural anesthesia. Read More

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http://dx.doi.org/10.4103/sja.SJA_415_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6329241PMC
January 2019
3 Reads

A prospective 5-year study of exercise performance following Melody valve implant.

Am Heart J 2019 Mar 29;209:47-53. Epub 2018 Dec 29.

Children's Hospital of Orange County, University of California, Irvine, CA. Electronic address:

Background: The long-term benefits of Melody valve implant for right ventricular outflow tract conduit obstruction or insufficiency on exercise capacity are undefined.

Methods: As part of the Melody valve clinical trial, 136 patients with congenital heart disease underwent serial cardiopulmonary exercise testing prior to, 6 months after, and annually for up to 5 years postimplant.

Results: Mean age at Melody valve implantation was 22. Read More

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http://dx.doi.org/10.1016/j.ahj.2018.12.014DOI Listing
March 2019
2 Reads

Gender- and Age-Specific Associations Between Body Fat Composition and C-Reactive Protein with Lung Function: A Cross-Sectional Study.

Sci Rep 2019 Jan 23;9(1):384. Epub 2019 Jan 23.

Department of Family Medicine, Chang-Gung Memorial Hospital at Linkou, Taoyuan, Taiwan.

Lung function deterioration is frequently observed in overweight and obese patients. In the current study, we explored the contribution of body fat (BF) composition, particularly visceral and nonvisceral adiposity, to lung function deterioration. In addition, we examined gender- and age-specific differences in the association between the joint effects of BF% and C-reactive protein (CRP) concentrations with lung function. Read More

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http://dx.doi.org/10.1038/s41598-018-36860-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6344558PMC
January 2019
3 Reads

Surfactant protein D as a marker for pulmonary complications in pediatric patients with sickle cell disease: Relation to lung function tests.

Pediatr Pulmonol 2019 Jan 22. Epub 2019 Jan 22.

Faculty of Medicine, Pediatrics Department, Ain Shams University, Cairo, Egypt.

Background: Surfactant protein D (SP-D) is considered a candidate biomarker for lung integrity and for disease progression.

Aim: We determined the level of SP-D in children and adolescents with SCD and assessed its possible relation to pulmonary complications and lung function.

Methods: Serum SP-D levels were assessed in 50 SCD patients compared with 30 healthy controls. Read More

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http://dx.doi.org/10.1002/ppul.24257DOI Listing
January 2019
5 Reads

Restrictive spirometry pattern is associated with low physical activity levels. A population based international study.

Respir Med 2019 Jan 15;146:116-123. Epub 2018 Dec 15.

ISGlobal, Barcelona, Spain; Universitat Pompeu Fabra (UPF), Barcelona, Spain; CIBER Epidemiología y Salud Pública (CIBERESP), Barcelona, Spain. Electronic address:

Introduction: Restrictive spirometry pattern is an under-recognised disorder with a poor morbidity and mortality prognosis. We compared physical activity levels between adults with a restrictive spirometry pattern and with normal spirometry.

Methods: Restrictive spirometry pattern was defined as a having post-bronchodilator FEV/FVC ≥ Lower Limit of Normal and a FVC<80% predicted in two population-based studies (ECRHS-III and SAPALDIA3). Read More

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http://dx.doi.org/10.1016/j.rmed.2018.11.017DOI Listing
January 2019
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"Structure-Function Imaging of Lung Disease Using Ultrashort Echo Time MRI".

Acad Radiol 2019 Mar 16;26(3):431-441. Epub 2019 Jan 16.

Department of Medical Physics, School of Medicine and Public Health, University of Wisconsin - Madison, Rm. 2488, 1111 Highland Ave., Madison, WI; Department of Radiology, School of Medicine and Public Health, University of Wisconsin - Madison, Madison, WI; Department of Medicine, School of Medicine and Public Health, University of Wisconsin - Madison, Madison, WI; Department of Biomedical Engineering, College of Engineering, University of Wisconsin - Madison, Madison, WI. Electronic address:

Rationale And Objectives: The purpose of this review is to acquaint the reader with recent advances in ultrashort echo time (UTE) magnetic resonance imaging (MRI) of the lung and its implications for pulmonary MRI when used in conjunction with functional MRI technique.

Materials And Methods: We provide an overview of recent technical advances of UTE and explore the advantages of combined structure-function pulmonary imaging in the context of restrictive and obstructive pulmonary diseases such as idiopathic pulmonary fibrosis (IPF) and cystic fibrosis (CF).

Results: UTE MRI clearly shows the lung parenchymal changes due to IPF and CF. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10766332183056
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http://dx.doi.org/10.1016/j.acra.2018.12.007DOI Listing
March 2019
15 Reads

Montelukast in chronic lung allograft dysfunction after lung transplantation.

J Heart Lung Transplant 2018 Dec 6. Epub 2018 Dec 6.

Department of Respiratory Diseases, University Hospitals Leuven, Leuven, Belgium; Department of Chronic Diseases, Metabolism & Ageing, Division of Respiratory Diseases, KU Leuven, Leuven, Belgium.

Background: Chronic lung allograft dysfunction (CLAD) is a major cause of post‒lung transplant mortality, with limited medical treatment options. In this study we assessed the association of montelukast treatment with pulmonary function and outcome in lung transplant recipients with progressive CLAD.

Methods: We performed a retrospective study of all lung transplant recipients transplanted between July 1991 and December 2016 at our center and who were treated for at least 3 months with montelukast for progressive CLAD, despite at least 3 months of prior azithromycin therapy. Read More

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http://dx.doi.org/10.1016/j.healun.2018.11.014DOI Listing
December 2018
6 Reads

Impact of presenting patient characteristics on surgical complications and morbidity in early onset scoliosis.

J Clin Neurosci 2019 Apr 8;62:105-111. Epub 2019 Jan 8.

Department of Orthopaedics. NYU Medical Center-Orthopaedic Hospital, New York, NY, USA. Electronic address:

This study sought to assess comorbidity profiles unique to early-onset-scoliosis (EOS) patients by employing cluster analytics and to determine the influence of isolated comorbidity clusters on perioperative complications, morbidity and mortality using a high powered administrative database. The KID database was queried for ICD-9 codes pertaining to congenital and idiopathic scoliosis from 2003, 2006, 2009, 2012. Patients <10 y/o (EOS group) were included. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S09675868183134
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http://dx.doi.org/10.1016/j.jocn.2018.12.007DOI Listing
April 2019
14 Reads
1.318 Impact Factor

Characteristic clinical and ultrastructural findings in nesprinopathies.

Eur J Paediatr Neurol 2019 Mar 29;23(2):254-261. Epub 2018 Dec 29.

Department of Pediatric Neurology, Developmental Neurology and Social Pediatrics, University of Essen, Germany.

Aims: To define the neurological and neuropathological alterations caused by SYNE1 mutations.

Methods: We describe 5 patients (3 males, 2 females; age 3-24 years) from 3 families. The diagnostic work-up included three muscle biopsies and two nerve biopsies in three of the cases. Read More

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http://dx.doi.org/10.1016/j.ejpn.2018.12.011DOI Listing
March 2019
4 Reads

Prolonged Survival in a Patient with Idiopathic Pulmonary Fibrosis Receiving Acupuncture and DHEA-Promoting Herbs with Conventional Management: A Case Report.

Perm J 2019 ;23:18-074

National University of Natural Medicine, Portland, OR.

Introduction: Idiopathic pulmonary fibrosis (IPF) is a lethal, chronic, degenerative disease most commonly affecting the elderly population. The pathologic finding of this restrictive lung condition is excessive deposition of fibrotic tissue in the alveolar interstitium. Conventional therapies minimally alter the course of disease, and mean survival time from diagnosis is only 2 to 4 years. Read More

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http://www.thepermanentejournal.org/issues/2019/winter/6927-
Publisher Site
http://dx.doi.org/10.7812/TPP/18-074DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6326563PMC
January 2019
17 Reads

Interstitial lung diseases in dogs and cats part I: The idiopathic interstitial pneumonias.

Authors:
Carol Reinero

Vet J 2019 Jan 20;243:48-54. Epub 2018 Nov 20.

Department of Veterinary Medicine, University of Missouri, Columbia, MO 65211, USA. Electronic address:

Interstitial lung diseases (ILDs), also called diffuse parenchymal lung diseases, are a large heterogenous group of non-infectious, non-neoplastic disorders characterized by varied patterns of inflammation and fibrosis (Travis et al., 2002). In humans, accurate classification of interstitial lung diseases (ILDs) requires multidisciplinary collaboration between clinicians, radiologists and pathologists. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10900233183017
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http://dx.doi.org/10.1016/j.tvjl.2018.11.010DOI Listing
January 2019
12 Reads