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  1. Article ; Online: Relative Lung Perfusion on Ventilation-Perfusion Scans After Double Lung Transplant.

    Li, David J / Abele, Jonathan / Sunner, Parveen / Varughese, Rhea A / Hirji, Alim S / Weinkauf, Justin G / Nagendran, Jayan / Weatherald, Jason C / Lien, Dale C / Halloran, Kieran M

    Transplantation

    2023  Volume 107, Issue 10, Page(s) 2262–2270

    Abstract: Background: Pulmonary blood flow can be assessed on ventilation-perfusion (VQ) scan with relative lung perfusion, with a 55% to 45% (or 10%) right-to-left differential considered normal. We hypothesized that wide perfusion differential on routine VQ ... ...

    Abstract Background: Pulmonary blood flow can be assessed on ventilation-perfusion (VQ) scan with relative lung perfusion, with a 55% to 45% (or 10%) right-to-left differential considered normal. We hypothesized that wide perfusion differential on routine VQ studies at 3 mo posttransplant would be associated with an increased risk of death or retransplantation, chronic lung allograft (CLAD), and baseline lung allograft dysfunction.
    Methods: We conducted a retrospective cohort study on all patients who underwent double-lung transplant in our program between 2005 and 2016, identifying patients with a wide perfusion differential of >10% on a 3-mo VQ scan. We used Kaplan-Meier estimates and proportional hazards models to assess the association between perfusion differential and time to death or retransplant and time to CLAD onset. We used correlation and linear regression to assess the relationship with lung function at time of scan and with baseline lung allograft dysfunction.
    Results: Of 340 patients who met inclusion criteria, 169 (49%) had a relative perfusion differential of ≥ 10% on a 3-mo VQ scan. Patients with increased perfusion differential had increased risk of death or retransplantation ( P  = 0.011) and CLAD onset ( P  = 0.012) after adjustment for other radiographic/endoscopic abnormalities. Increased perfusion differential was associated with lower lung function at time of scan.
    Conclusions: Wide lung perfusion differential was common after lung transplant in our cohort and associated with increased risk of death, poor lung function, and CLAD onset. The nature of this abnormality and its use as a predictor of future risk warrant further investigation.
    MeSH term(s) Humans ; Retrospective Studies ; Ventilation-Perfusion Scan ; Lung/diagnostic imaging ; Lung Transplantation/adverse effects ; Perfusion/adverse effects ; Allografts
    Language English
    Publishing date 2023-06-09
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 208424-7
    ISSN 1534-6080 ; 0041-1337
    ISSN (online) 1534-6080
    ISSN 0041-1337
    DOI 10.1097/TP.0000000000004683
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article ; Online: Endobronchial hamartoma.

    Poonja, Zia / Sobey, Allan / Weinkauf, Justin G

    Journal of bronchology & interventional pulmonology

    2013  Volume 20, Issue 3, Page(s) 247–248

    Abstract: We present images of an endobronchial hamartoma visible by bronchoscopy in the left lower lobe. A 56-year-old woman with a late presentation of an acute anterior myocardial infarction requires urgent heart transplantation and subsequently has trouble ... ...

    Abstract We present images of an endobronchial hamartoma visible by bronchoscopy in the left lower lobe. A 56-year-old woman with a late presentation of an acute anterior myocardial infarction requires urgent heart transplantation and subsequently has trouble weaning off the ventilator. Bronchoscopic evaluation reveals a 1 cm endobronchial hamartoma obstruction the left lower lobe bronchus. A polypectomy snare was effectively used with electrocautery to excise the lesion. The patient was successfully able to wean off the ventilator once the left lower lobe was free from obstruction.
    MeSH term(s) Bronchial Neoplasms/pathology ; Bronchial Neoplasms/surgery ; Bronchoscopy ; Female ; Hamartoma/pathology ; Hamartoma/surgery ; Heart Transplantation ; Humans ; Middle Aged ; Ventilator Weaning
    Language English
    Publishing date 2013-07
    Publishing country United States
    Document type Case Reports ; Journal Article
    ZDB-ID 2478320-1
    ISSN 1948-8270 ; 1944-6586
    ISSN (online) 1948-8270
    ISSN 1944-6586
    DOI 10.1097/LBR.0b013e31829aad50
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article ; Online: Successful bilateral lung transplantation in a patient with end-stage lung disease and positive novel influenza virus (H1N1).

    Al Aklabi, Mohammed M / Weinkauf, Justin G / Humar, Atul / Ghorpade, Nitin

    The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation

    2010  Volume 29, Issue 8, Page(s) 898–899

    Abstract: In 2009, a pandemic novel influenza virus (H1N1) outbreak was declared by the World Health Organization and resulted in significant worldwide illness. This report describes a 50-year-old male with end-stage lung disease secondary to alpha(1)-anti-trypsin ...

    Abstract In 2009, a pandemic novel influenza virus (H1N1) outbreak was declared by the World Health Organization and resulted in significant worldwide illness. This report describes a 50-year-old male with end-stage lung disease secondary to alpha(1)-anti-trypsin deficiency and chronic obstructive pulmonary disease. He was admitted for potential bilateral lung transplantation when suitable organs became available. Incidentally, he was found to have some non-specific symptoms, including malaise and myalgias. These findings were attributed to killed-virus H1N1 vaccine given 48 hours earlier. However, as a safety measure, a nasopharyngeal swab was taken, and anti-viral therapy with oseltamivir (Tamiflu) was started empirically. He underwent bilateral lung transplantation on the same day of admission. In the immediate post-operative period his nasopharyngeal swab came back positive for H1N1 influenza virus. Then, post-operatively, two consecutive bronchoalveolar lavage samples from the transplanted lungs were found to be positive for H1N1 virus. He received three-weeks of antiviral treatment post-operatively and he had uneventful procedure with favorable outcome.
    MeSH term(s) Antiviral Agents/therapeutic use ; Bronchoalveolar Lavage ; Humans ; Incidental Findings ; Influenza A Virus, H1N1 Subtype ; Influenza, Human/diagnosis ; Influenza, Human/drug therapy ; Influenza, Human/microbiology ; Lung/microbiology ; Lung/surgery ; Lung Transplantation/methods ; Male ; Middle Aged ; Oseltamivir/therapeutic use ; Pulmonary Disease, Chronic Obstructive/surgery ; Treatment Outcome
    Chemical Substances Antiviral Agents ; Oseltamivir (20O93L6F9H)
    Language English
    Publishing date 2010-08
    Publishing country United States
    Document type Case Reports ; Journal Article
    ZDB-ID 1062522-7
    ISSN 1557-3117 ; 1053-2498
    ISSN (online) 1557-3117
    ISSN 1053-2498
    DOI 10.1016/j.healun.2010.04.017
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Transcutaneous electrical nerve stimulation for severe gastroparesis after lung transplantation.

    Weinkauf, Justin G / Yiannopoulos, Anna / Faul, John L

    The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation

    2005  Volume 24, Issue 9, Page(s) 1444

    Abstract: Gastroparesis is a serious complication of lung transplantation that can lead to weight loss, gastroesophageal reflux disease, and recurrent aspiration pneumonia. We present 2 lung allograft recipients in whom gastroparesis resolved with the use of ... ...

    Abstract Gastroparesis is a serious complication of lung transplantation that can lead to weight loss, gastroesophageal reflux disease, and recurrent aspiration pneumonia. We present 2 lung allograft recipients in whom gastroparesis resolved with the use of transcutaneous electrical nerve stimulation (TENS). In both patients, severe symptoms of gastroparesis refractory to medical therapy were completely ablated after 20 and 30 days of therapy. Both patients are currently asymptomatic with a normal diet, without the use of promotility agents. Lung transplant recipients with severe gastroparesis can derive significant benefit from TENS.
    MeSH term(s) Adult ; Female ; Gastroparesis/etiology ; Gastroparesis/therapy ; Humans ; Lung Transplantation ; Male ; Middle Aged ; Postoperative Complications/therapy ; Transcutaneous Electric Nerve Stimulation
    Language English
    Publishing date 2005-09
    Publishing country United States
    Document type Case Reports ; Journal Article
    ZDB-ID 1062522-7
    ISSN 1557-3117 ; 1053-2498
    ISSN (online) 1557-3117
    ISSN 1053-2498
    DOI 10.1016/j.healun.2004.09.006
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article ; Online: An international survey of cytomegalovirus management practices in lung transplantation.

    Zuk, Dalyce M / Humar, Atul / Weinkauf, Justin G / Lien, Dale C / Nador, Roland G / Kumar, Deepali

    Transplantation

    2010  Volume 90, Issue 6, Page(s) 672–676

    Abstract: Background: Cytomegalovirus (CMV) is an important infection in lung transplant recipients. Center-to-center variation in preventive and treatment strategies is unknown.: Methods: An electronic survey was sent to 102 lung transplant programs ... ...

    Abstract Background: Cytomegalovirus (CMV) is an important infection in lung transplant recipients. Center-to-center variation in preventive and treatment strategies is unknown.
    Methods: An electronic survey was sent to 102 lung transplant programs registered with the International Society of Heart and Lung Transplantation and United Network for Organ Sharing.
    Results: Fifty-nine (58%) programs responded to the survey. For CMV prevention (D+/R-), 56 of the 59 (94.9%) programs used prophylaxis and two (3.4%) of them used preemptive therapy. For R+ patients, 86.4% used prophylaxis and 13.6% used preemptive strategy. Duration of prophylaxis was extremely variable ranging from 3 months to indefinite. Adjunctive prophylactic strategies included routine viral monitoring (51% D+/R-; 44% R+) and CMV immunoglobulin (32% D+/R-; 14% R+). The medication used for prophylaxis was valganciclovir with approximately half starting with intravenous ganciclovir. 9 of the 59 (15.2%) centers reported using specific CMV prophylaxis in D-/R- patients. Methods for viral monitoring included peripheral blood polymerase chain reaction, antigenemia, bronchoalveolar lavage viral culture, and bronchoalveolar lavage polymerase chain reaction. For treatment of CMV viremia, valganciclovir or intravenous ganciclovir were used. A total of 47.5% of centers routinely decreased immunosuppression at the time of viremia. Secondary antiviral prophylaxis was used routinely by 36 of the 59 (61%) centers.
    Conclusions: Although prophylaxis is the most commonly used preventive strategy, significant variation exists in the way it is implemented. Specifically, duration of prophylaxis is extremely variable. Uniform international guidelines would be of value in this population.
    MeSH term(s) Antiviral Agents/therapeutic use ; Cytomegalovirus/genetics ; Cytomegalovirus/isolation & purification ; Cytomegalovirus Infections/drug therapy ; Cytomegalovirus Infections/epidemiology ; Cytomegalovirus Infections/prevention & control ; Drug Therapy, Combination ; Electronic Health Records ; Ganciclovir/analogs & derivatives ; Ganciclovir/therapeutic use ; Health Surveys ; Humans ; Internationality ; Lung Transplantation/adverse effects ; Practice Guidelines as Topic
    Chemical Substances Antiviral Agents ; valganciclovir (GCU97FKN3R) ; Ganciclovir (P9G3CKZ4P5)
    Language English
    Publishing date 2010-09-27
    Publishing country United States
    Document type Journal Article
    ZDB-ID 208424-7
    ISSN 1534-6080 ; 0041-1337
    ISSN (online) 1534-6080
    ISSN 0041-1337
    DOI 10.1097/TP.0b013e3181ea3955
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article: An unusual case of spinal column metastasis after orthotopic transplantation for cardiac sarcoma.

    McLoughlin, Gregory S / Sciubba, Daniel M / Ali, S Kaiser / Weinkauf, Justin G / Fourney, Daryl R

    Journal of neurosurgery. Spine

    2008  Volume 9, Issue 4, Page(s) 377–381

    Abstract: The authors describe a patient who underwent orthotopic cardiac transplantation after an undifferentiated cardiac sarcoma was diagnosed. While receiving immunosuppressive therapy, the patient developed spinal column metastases and cauda equina syndrome ... ...

    Abstract The authors describe a patient who underwent orthotopic cardiac transplantation after an undifferentiated cardiac sarcoma was diagnosed. While receiving immunosuppressive therapy, the patient developed spinal column metastases and cauda equina syndrome requiring surgical decompression and stabilization. This occurred despite an exhaustive search for metastatic disease prior to the transplantation. To the authors' knowledge, this represents the first reported case of an undifferentiated cardiac sarcoma metastasis to the spine. This previously healthy 18-year-old woman presented with a myocardial infarction. Investigations revealed a left atrial tumor, which was resected. Following local recurrence, the patient underwent extensive studies to rule out systemic disease. Orthotopic heart-lung transplantation was then performed. While receiving postoperative immunosuppressive therapy the patient presented with cauda equina syndrome secondary to metastatic tumor compression at the L-5 level. Despite a comprehensive screening process to exclude metastatic disease prior to transplantation, spinal metastases occurred while this patient was receiving immunosuppressive therapy. This represents a previously unreported and clinically significant complication for undifferentiated cardiac sarcoma.
    MeSH term(s) Adolescent ; Female ; Heart Neoplasms/pathology ; Heart Neoplasms/surgery ; Heart Transplantation ; Humans ; Immunosuppression ; Lumbar Vertebrae ; Sarcoma/secondary ; Sarcoma/surgery ; Spinal Neoplasms/pathology
    Language English
    Publishing date 2008-10
    Publishing country United States
    Document type Case Reports ; Journal Article
    ZDB-ID 2158643-3
    ISSN 1547-5646 ; 1547-5654
    ISSN (online) 1547-5646
    ISSN 1547-5654
    DOI 10.3171/SPI.2008.9.10.377
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article: Why do some countries publish more than others? An international comparison of research funding, English proficiency and publication output in highly ranked general medical journals.

    Man, Jonathan P / Weinkauf, Justin G / Tsang, Monica / Sin, Don D

    European journal of epidemiology

    2004  Volume 19, Issue 8, Page(s) 811–817

    Abstract: National factor(s) influencing publication output in the highest ranked medical journals are largely unknown. We sought to examine the relationship between national research funding and English proficiency on publication output. We identified all ... ...

    Abstract National factor(s) influencing publication output in the highest ranked medical journals are largely unknown. We sought to examine the relationship between national research funding and English proficiency on publication output. We identified all original research articles appearing in the five highest ranked general medical journals between 1997 and 2001. Using the country of the corresponding author as the source nation for each article, we determined a standardized publication rate across developed nations. We used multiple regression techniques to determine the influence of national expenditures on research and scores from the Test of English as a Foreign Language (TOEFL), a surrogate for English proficiency, on publication output. There was a significant relationship of national spending on research and TOEFL scores to publication output of developed countries (p = 0.04; p < 0.01, respectively). These two variables explained approximately 71.5% of the variation in publication rate across developed nations around the world (R = 0.85; p < 0.01). Normalized for population size, English-speaking nations and certain northern European countries such as Denmark, The Netherlands, Switzerland, and Sweden had the highest rate of publication in the five highest ranked general medical journals, while Asian countries had generally low rates of publication. Research spending and English proficiency were strongly associated with publication output in the highest ranked general medical journals. While these data cannot be considered definitive due to their observational nature, they do suggest that for English-language medical journals, research funding and English proficiency may be important determinants of publication.
    MeSH term(s) Bibliometrics ; Biomedical Research ; Internationality ; Language ; Periodicals as Topic/statistics & numerical data ; Publishing
    Language English
    Publishing date 2004-09-03
    Publishing country Netherlands
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 632614-6
    ISSN 1573-7284 ; 0393-2990
    ISSN (online) 1573-7284
    ISSN 0393-2990
    DOI 10.1023/b:ejep.0000036571.00320.b8
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: Reduced elastogenesis

    Morimoto Marie / Yu Zhongxin / Stenzel Peter / Clewing J / Najafian Behzad / Mayfield Christy / Hendson Glenda / Weinkauf Justin G / Gormley Andrew K / Parham David M / Ponniah Umakumaran / André Jean-Luc / Asakura Yumi / Basiratnia Mitra / Bogdanović Radovan / Bokenkamp Arend / Bonneau Dominique / Buck Anna / Charrow Joel /
    Cochat Pierre / Cordeiro Isabel / Deschenes Georges / Fenkçi M / Frange Pierre / Fründ Stefan / Fryssira Helen / Guillen-Navarro Encarna / Keller Kory / Kirmani Salman / Kobelka Christine / Lamfers Petra / Levtchenko Elena / Lewis David B / Massella Laura / McLeod D / Milford David V / Nobili François / Saraiva Jorge M / Semerci C / Shoemaker Lawrence / Stajić Nataša / Stein Anja / Taha Doris / Wand Dorothea / Zonana Jonathan / Lücke Thomas / Boerkoel Cornelius F

    Orphanet Journal of Rare Diseases, Vol 7, Iss 1, p

    a clue to the arteriosclerosis and emphysematous changes in Schimke immuno-osseous dysplasia?

    2012  Volume 70

    Abstract: Abstract Background Arteriosclerosis and emphysema develop in individuals with Schimke immuno-osseous dysplasia (SIOD), a multisystem disorder caused by biallelic mutations in SMARCAL1 (SWI/SNF-related, matrix-associated, actin-dependent regulator of ... ...

    Abstract Abstract Background Arteriosclerosis and emphysema develop in individuals with Schimke immuno-osseous dysplasia (SIOD), a multisystem disorder caused by biallelic mutations in SMARCAL1 (SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily a-like 1). However, the mechanism by which the vascular and pulmonary disease arises in SIOD remains unknown. Methods We reviewed the records of 65 patients with SMARCAL1 mutations. Molecular and immunohistochemical analyses were conducted on autopsy tissue from 4 SIOD patients. Results Thirty-two of 63 patients had signs of arteriosclerosis and 3 of 51 had signs of emphysema. The arteriosclerosis was characterized by intimal and medial hyperplasia, smooth muscle cell hyperplasia and fragmented and disorganized elastin fibers, and the pulmonary disease was characterized by panlobular enlargement of air spaces. Consistent with a cell autonomous disorder, SMARCAL1 was expressed in arterial and lung tissue, and both the aorta and lung of SIOD patients had reduced expression of elastin and alterations in the expression of regulators of elastin gene expression. Conclusions This first comprehensive study of the vascular and pulmonary complications of SIOD shows that these commonly cause morbidity and mortality and might arise from impaired elastogenesis. Additionally, the effect of SMARCAL1 deficiency on elastin expression provides a model for understanding other features of SIOD.
    Keywords Schimke immuno-osseous dysplasia ; SMARCAL1 ; Elastin ; Vascular disease ; Pulmonary emphysema ; Medicine ; R
    Subject code 610
    Language English
    Publishing date 2012-09-01T00:00:00Z
    Publisher BMC
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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  9. Article ; Online: Reduced elastogenesis: a clue to the arteriosclerosis and emphysematous changes in Schimke immuno-osseous dysplasia?

    Morimoto, Marie / Yu, Zhongxin / Stenzel, Peter / Clewing, J Marietta / Najafian, Behzad / Mayfield, Christy / Hendson, Glenda / Weinkauf, Justin G / Gormley, Andrew K / Parham, David M / Ponniah, Umakumaran / André, Jean-Luc / Asakura, Yumi / Basiratnia, Mitra / Bogdanović, Radovan / Bokenkamp, Arend / Bonneau, Dominique / Buck, Anna / Charrow, Joel /
    Cochat, Pierre / Cordeiro, Isabel / Deschenes, Georges / Fenkçi, M Semin / Frange, Pierre / Fründ, Stefan / Fryssira, Helen / Guillen-Navarro, Encarna / Keller, Kory / Kirmani, Salman / Kobelka, Christine / Lamfers, Petra / Levtchenko, Elena / Lewis, David B / Massella, Laura / McLeod, D Ross / Milford, David V / Nobili, François / Saraiva, Jorge M / Semerci, C Nur / Shoemaker, Lawrence / Stajić, Nataša / Stein, Anja / Taha, Doris / Wand, Dorothea / Zonana, Jonathan / Lücke, Thomas / Boerkoel, Cornelius F

    Orphanet journal of rare diseases

    2012  Volume 7, Page(s) 70

    Abstract: Background: Arteriosclerosis and emphysema develop in individuals with Schimke immuno-osseous dysplasia (SIOD), a multisystem disorder caused by biallelic mutations in SMARCAL1 (SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, ...

    Abstract Background: Arteriosclerosis and emphysema develop in individuals with Schimke immuno-osseous dysplasia (SIOD), a multisystem disorder caused by biallelic mutations in SMARCAL1 (SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily a-like 1). However, the mechanism by which the vascular and pulmonary disease arises in SIOD remains unknown.
    Methods: We reviewed the records of 65 patients with SMARCAL1 mutations. Molecular and immunohistochemical analyses were conducted on autopsy tissue from 4 SIOD patients.
    Results: Thirty-two of 63 patients had signs of arteriosclerosis and 3 of 51 had signs of emphysema. The arteriosclerosis was characterized by intimal and medial hyperplasia, smooth muscle cell hyperplasia and fragmented and disorganized elastin fibers, and the pulmonary disease was characterized by panlobular enlargement of air spaces. Consistent with a cell autonomous disorder, SMARCAL1 was expressed in arterial and lung tissue, and both the aorta and lung of SIOD patients had reduced expression of elastin and alterations in the expression of regulators of elastin gene expression.
    Conclusions: This first comprehensive study of the vascular and pulmonary complications of SIOD shows that these commonly cause morbidity and mortality and might arise from impaired elastogenesis. Additionally, the effect of SMARCAL1 deficiency on elastin expression provides a model for understanding other features of SIOD.
    MeSH term(s) Adult ; Arteriosclerosis/genetics ; Arteriosclerosis/physiopathology ; Autopsy ; Child ; Child, Preschool ; DNA Helicases/genetics ; Emphysema/genetics ; Emphysema/physiopathology ; Female ; Humans ; Immunohistochemistry ; Immunologic Deficiency Syndromes/genetics ; Immunologic Deficiency Syndromes/physiopathology ; Male ; Nephrotic Syndrome/genetics ; Nephrotic Syndrome/physiopathology ; Osteochondrodysplasias/genetics ; Osteochondrodysplasias/physiopathology ; Primary Immunodeficiency Diseases ; Pulmonary Embolism/genetics ; Pulmonary Embolism/physiopathology
    Chemical Substances SMARCAL1 protein, human (EC 2.7.7.-) ; DNA Helicases (EC 3.6.4.-)
    Language English
    Publishing date 2012-09-22
    Publishing country England
    Document type Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
    ISSN 1750-1172
    ISSN (online) 1750-1172
    DOI 10.1186/1750-1172-7-70
    Database MEDical Literature Analysis and Retrieval System OnLINE

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