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https://www.readbyqxmd.com/read/28088601/mid-term-outcomes-in-patients-with-congenitally-corrected-transposition-of-the-great-arteries-a-single-center-experience
#1
Luis E De León, Carlos M Mery, Raymond A Verm, Daniel Trujillo-Díaz, Ankita Patro, Francisco A Guzmán-Pruneda, Iki Adachi, Jeffrey S Heinle, Lauren C Kane, E Dean McKenzie, Charles D Fraser
INTRO: The optimal management of patients with congenitally-corrected transposition of the great arteries (ccTGA) is unclear. The goal of this study was to compare the outcomes in patients with ccTGA undergoing different management strategies. METHODS: Patients with ccTGA believed suitable for biventricular circulation treated in 1995-2016 were included. The cohort was divided into four groups: systemic right ventricle (RV) (patients without surgical intervention or with a classic repair), anatomic repair, Fontan palliation, and patients only receiving a pulmonary artery band (PAB) or a shunt...
January 11, 2017: Journal of the American College of Surgeons
https://www.readbyqxmd.com/read/28088585/nocardiosis-in-south-of-france-during-the-last-ten-years
#2
Delphine Haussaire, Pierre-Edouard Fournier, Karamoko Djiguiba, Valerie Moal, Tristan Legris, Rajsingh Purgus, Jeremy Bismuth, Xavier Elharrar, Martine Reynaud-Gaubert, Henri Vacher-Coponat
BACKGROUND: Nocardiosis is a rare disease with polymorphic presentations. Epidemiology and clinical presentation could change with the increasing number of immunocompromised patients. METHODS: Medical records and microbiologic data for patients affected by nocardiosis and treated in the university hospital of Marseille between 2004 and 2014 were retrospectively analyzed. RESULTS: We analyzed 34 patients infected by Nocardia spp during this period...
January 11, 2017: International Journal of Infectious Diseases: IJID
https://www.readbyqxmd.com/read/28087362/clinical-phenotypes-and-outcomes-of-heritable-and-sporadic-pulmonary-veno-occlusive-disease-a-population-based-study
#3
David Montani, Barbara Girerd, Xavier Jaïs, Marilyne Levy, David Amar, Laurent Savale, Peter Dorfmüller, Andrei Seferian, Edmund M Lau, Mélanie Eyries, Jérôme Le Pavec, Florence Parent, Damien Bonnet, Florent Soubrier, Elie Fadel, Olivier Sitbon, Gérald Simonneau, Marc Humbert
BACKGROUND: Bi-allelic mutations of the EIF2AK4 gene cause heritable pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis (PVOD/PCH). We aimed to assess the effect of EIF2AK4 mutations on the clinical phenotypes and outcomes of PVOD/PCH. METHODS: We did a population-based study using clinical, functional, and haemodynamic data from the registry of the French Pulmonary Hypertension Network. We reviewed the clinical data and outcomes from all patients referred to the French Referral Centre (Pulmonary Department, Hospital Kremlin-Bicêtre, University Paris-Sud) with either confirmed or highly probable PVOD/PCH with DNA available for mutation screening (excluding patients with other risk factors of pulmonary hypertension, such as chronic respiratory diseases)...
January 10, 2017: Lancet Respiratory Medicine
https://www.readbyqxmd.com/read/28079851/corticosteroid-and-cyclophosphamide-in-acute-exacerbation-of-idiopathic-pulmonary-fibrosis-a-single-center-experience-and-literature-review
#4
Luca Novelli, Roberto Ruggiero, Federica De Giacomi, Alice Biffi, Paola Faverio, Luca Bilucaglia, Silvia Gamberini, Grazia Messinesi, Alberto Pesci
Acute Exacerbation (AEx) is a frequent and severe complication of Idiopathic Pulmonary Fibrosis (IPF). In the absence of consensus regarding treatment, studies evaluating the efficacy of specific therapies, such as corticosteroids and immunosuppresant agents, are needed. In this case series we evaluated the outcome in terms of survival of intravenous pulse doses of high-dose corticosteroid (methylprednisolone 1000 mg per day for 3 consecutive days) followed by montlhy cyclophosphamide administration (maximum 6 doses) in a cohort of patients with AEx-IPF referred to the Respiratory Unit, San Gerardo University Hospital, Monza, Italy, from 2009 to 2013...
December 23, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
https://www.readbyqxmd.com/read/28079843/the-association-between-bnp-6mwd-test-dlco-and-pulmonary-hypertension-in-sarcoidosis
#5
Mehdi Mirsaeidi, Hesham R Omar, Robert Baughman, Roberto Machado, Nadera Sweiss
INTRODUCTION: Pulmonary hypertension (PH) is a feared complication in patients with sarcoidosis.It is an important negative prognostic factor which is reflected as a priority given to these subjects for orthotopic lung transplantation. We evaluated the relationship between BNP, 6MWD test, DLCO% values and the severity of sarcoidosis-associated pulmonary hypertension. METHODS: A retrospective chart review was conducted between January 2010 and January 2015 on consecutive confirmed adult subjects diagnosed with sarcoidosis...
December 23, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
https://www.readbyqxmd.com/read/28078796/hyperparathyroidism-caused-by-distant-pulmonary-lesions-and-parathyromatosis-after-ethanol-injection-parathyroidectomy-for-secondary-hyperparathyroidism
#6
Michio Nakamura, Kiho Tanaka, Takeshi Fujii
Secondary hyperparathyroidism (SHPT) treatment includes parathyroidectomy and percutaneous ethanol injection therapy (PEIT), which are invasive procedures. The condition in which benign hyperfunctioning parathyroid tissue is distributed throughout the neck and mediastinum is termed parathyromatosis. Here, we present the case of a 51-year-old woman who began hemodialysis in 1986 due to chronic kidney disease of unknown etiology and developed SHPT in 1999. She underwent 6 rounds of PEIT followed by total a parathyroidectomy with partial forearm autotransplantation...
January 11, 2017: Hemodialysis International
https://www.readbyqxmd.com/read/28074663/primary-graft-dysfunction-pathophysiology-to-guide-new-preventive-therapies
#7
Ciara M Shaver, Lorraine B Ware
Primary graft dysfunction (PGD) is a common complication of lung transplantation characterized by acute pulmonary edema associated with bilateral pulmonary infiltrates and hypoxemia in the first 3 post-operative days. Development of PGD is a predictor of poor short- and long-term outcomes after lung transplantation, but there are currently limited tools to prevent its occurrence. Areas covered: Several potentially modifiable donor, recipient, and operative risk factors for PGD have been identified. In addition, basic and translational studies in animals and ex vivo lung perfusion systems have identified several biomarkers and mechanisms of injury in PGD...
January 11, 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28070238/hematological-disorders-and-pulmonary-hypertension
#8
REVIEW
Rajamma Mathew, Jing Huang, Joseph M Wu, John T Fallon, Michael H Gewitz
Pulmonary hypertension (PH), a serious disorder with a high morbidity and mortality rate, is known to occur in a number of unrelated systemic diseases. Several hematological disorders such as sickle cell disease, thalassemia and myeloproliferative diseases develop PH which worsens the prognosis. Associated oxidant injury and vascular inflammation cause endothelial damage and dysfunction. Pulmonary vascular endothelial damage/dysfunction is an early event in PH resulting in the loss of vascular reactivity, activation of proliferative and antiapoptotic pathways leading to vascular remodeling, elevated pulmonary artery pressure, right ventricular hypertrophy and premature death...
December 26, 2016: World Journal of Cardiology
https://www.readbyqxmd.com/read/28068330/loss-of-p53-induces-leukemic-transformation-in-a-murine-model-of-jak2-v617f-driven-polycythemia-vera
#9
T Tsuruta-Kishino, J Koya, K Kataoka, K Narukawa, Y Sumitomo, H Kobayashi, T Sato, M Kurokawa
As leukemic transformation of myeloproliferative neoplasms (MPNs) worsens the clinical outcome, reducing the inherent risk of the critical event in MPN cases could be beneficial. Among genetic alterations concerning the transformation, the frequent one is TP53 mutation. Here we show that retroviral overexpression of Jak2 V617F mutant into wild-type p53 murine bone marrow cells induced polycythemia vera (PV) in the recipient mice, whereas Jak2 V617F-transduced p53-null mice developed lethal leukemia after the preceding PV phase...
January 9, 2017: Oncogene
https://www.readbyqxmd.com/read/28067884/reversal-of-pre-capillary-pulmonary-hypertension-in-a-patient-with-sickle-cell-anemia-who-underwent-haploidentical-peripheral-blood-stem-cell-transplantation
#10
C Pittman, M M Hsieh, W Coles, J F Tisdale, N A Weir, C D Fitzhugh
No abstract text is available yet for this article.
January 9, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28067706/impact-of-systemic-vascular-resistance-on-the-accuracy-of-the-pulsioflex-device
#11
Matthieu Biais, Elodie Mazocky, Laurent Stecken, Bruno Pereira, Musa Sesay, Stéphanie Roullet, Alice Quinart, François Sztark
BACKGROUND: The accuracy of currently available devices using pulse contour analysis without external calibration for cardiac index (CI) estimation is negatively impacted by hyperdynamic states, low systemic vascular resistance (SVR), and abrupt changes in SVR. The aim of this study was to evaluate the accuracy of a new device, the Pulsioflex (Pulsion Medical System), in patients undergoing liver transplantation. METHODS: Thirty consecutive patients scheduled for liver transplantation were included...
January 5, 2017: Anesthesia and Analgesia
https://www.readbyqxmd.com/read/28066854/live-donor-liver-transplantation-for-antitubercular-drug-induced-acute-liver-failure
#12
Akshay P Bavikatte, S Sudhindran, Puneet Dhar, O V Sudheer, G Unnikrishnan, Dinesh Balakrishnan, Ramachandran N Menon
Antitubercular therapy (ATT)-induced hepatotoxicity is often over looked and active tuberculosis is considered a contraindication for liver transplantation, however it might be the only lifesaving option to certain patients of acute liver failure (ALF) due to ATT. We have assessed the outcome of live donor liver transplantation in ATT-induced ALF. A retrospective analysis of all the cases of ALF that underwent liver transplantation from 2006 to 2014 at the Amrita Institute of Medical Sciences was done. A total of seven (7...
January 9, 2017: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
https://www.readbyqxmd.com/read/28065839/cardiovascular-function-in-long-term-hematopoietic-cell-transplantation-survivors
#13
Saro H Armenian, David Horak, Jessica M Scott, George Mills, Aida Siyahian, Jennifer Berano Teh, Pamela S Douglas, Stephen J Forman, Smita Bhatia, Lee W Jones
Peak oxygen consumption (VO2peak), as measured by cardiopulmonary exercise testing (CPET), is a powerful independent predictor of cardiovascular disease (CVD) and all-cause mortality in a broad range of populations. We assessed the safety and feasibility of CPET in aging long-term HCT survivors, a population at high risk for premature-onset of CVD. Next we examined how organ-specific impairments (e.g. cardiac, pulmonary, hematologic) impact VO2peak after HCT. Twenty consecutive HCT survivors underwent a comprehensive assessment of cardiopulmonary health that included CPET, echocardiography with strain, pulmonary function testing, six-minute walk test, and timed-up and go...
January 5, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28061469/diagnosis-of-cytomegalovirus-pneumonia-by-quantitative-polymerase-chain-reaction-using-bronchial-washing-fluid-from-patients-with-hematologic-malignancies
#14
Hwa Young Lee, Chin Kook Rhee, Joon Young Choi, Hea Yon Lee, Jong Wook Lee, Dong Gun Lee
BACKGROUND: The incidence of cytomegalovirus (CMV) pneumonia is increasing in patients diagnosed with hematologic malignancies. The utility of CMV-DNA viral load measurement has not been standardized, and viral cut-off values have not been established. This study was designed to investigate the utility of CMV quantitative real-time PCR (qRT-PCR) using bronchial washing fluid. METHODS: We retrospectively reviewed the microbiologic and pathologic results of bronchial washing fluid and biopsy specimens in addition to the patients' clinical characteristics...
January 4, 2017: Oncotarget
https://www.readbyqxmd.com/read/28058524/determination-of-residual-dimethylsulfoxide-in-cryopreserved-cardiovascular-allografts
#15
R Díaz Rodríguez, B Van Hoeck, S De Gelas, F Blancke, R Ngakam, K Bogaerts, R Jashari
Dimethylsulfoxide (DMSO) is a solvent which protects the structure of allografts during the cryopreservation and thawing process. However, several toxic effects of DMSO in patients after transplantation of cryopreserved allografts have been described. The aim of this study is to determine the residual DMSO in the cardiovascular allografts after thawing and preparation of cryopreserved allografts for clinical application following guidelines of the European Pharmacopoeia for DMSO detection. Four types of EHB allografts (aortic valve-AV, pulmonary valve-PV, descending thoracic aorta-DA, and femoral artery-FA) are cryopreserved using as cryoprotecting solution a 10% of DMSO in medium 199...
January 5, 2017: Cell and Tissue Banking
https://www.readbyqxmd.com/read/28056830/development-of-aspergillosis-in-a-cohort-of-non-neutropenic-non-transplant-patients-colonised-by-aspergillus-spp
#16
José Barberán, Francisco-Javier García-Pérez, Victoria Villena, Alberto Fernández-Villar, Eduardo Malmierca, Cristina Salas, María-José Giménez, Juan-José Granizo, Lorenzo Aguilar
BACKGROUND: A previous study explored factors discriminating colonization and true infection among non-transplant, non-neutropenic patients with repeated Aspergillus spp. isolation from lower respiratory samples. The present study explored the evolution of patients with Aspergillus colonization in that study to determine the percentage of cases progressing to aspergillosis and time to development. METHODS: Clinical records were retrospectively reviewed (for each patient from his end date in the past study) and data from all respiratory processes suffered by patients up to April 2015 were recorded...
January 6, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28056816/optimization-of-pressure-settings-during-adaptive-servo-ventilation-support-using-real-time-heart-rate-variability-assessment-initial-case-report
#17
Teruhiko Imamura, Daisuke Nitta, Koichiro Kinugawa
BACKGROUND: Adaptive servo-ventilation (ASV) therapy is a recent non-invasive positive pressure ventilation therapy that was developed for patients with heart failure (HF) refractory to optimal medical therapy. However, it is likely that ASV therapy at relatively higher pressure setting worsens some of the patients' prognosis compared with optimal medical therapy. Therefore, identification of optimal pressure settings of ASV therapy is warranted. CASE PRESENTATION: We present the case of a 42-year-old male with HF, which was caused by dilated cardiomyopathy, who was admitted to our institution for evaluating his eligibility for heart transplantation...
January 5, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28056498/bone-marrow-stem-cell-therapy-partially-ameliorates-pathological-consequences-in-livers-of-mice-expressing-mutant-human-%C3%AE-1-antitrypsin
#18
Prakash Baligar, Veena Kochat, Shailendra K Arindkar, Zaffar Equbal, Snehashish Mukherjee, Swati Patel, Perumal Nagarajan, Sujata Mohanty, Jeffrey H Teckman, Asok Mukhopadhyay
: Alpha1-antitrypsin deficiency (AATD) is a genetic disease, caused by mutation of the AAT gene. Accumulation of mutated AAT protein aggregates in hepatocytes leads to endoplasmic reticulum (ER) stress resulting in impairment of liver functions and in some cases hepatocellular carcinoma, whereas decline of AAT levels in sera is responsible for pulmonary emphysema. In critical cases of liver ailments, the only option for treatment is liver transplantation, whereas AAT replacement therapy is followed in case of emphysema...
January 5, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28054732/liver-transplantation-is-associated-with-good-clinical-outcome-in-patients-with-active-tuberculosis-and-acute-liver-failure-due-to-anti-tubercular-treatment
#19
REVIEW
Michele Bartoletti, Giulia Martelli, Sara Tedeschi, Mariacristina Morelli, Valentine Bertuzzo, Marina Tadolini, Paolo Pianta, Francesco Cristini, Maddalena Giannella, Russell E Lewis, Antonio D Pinna, Pierluigi Viale
BACKGROUND: Active tuberculosis (TB) is commonly considered a contraindication for liver transplantation (LT). However, in patients with TB who develop acute liver failure (ALF) due to toxicity induced by anti-tubercular treatment (ATT), LT could be the only opportunity. The aim of this study was to evaluate the feasibility of LT in this scenario. METHODS: We described 2 cases and comprehensively reviewed the literature finding 26 cases of LT performed in patients having a concomitant active TB and liver failure secondary to ATT toxicity...
January 5, 2017: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/28054207/mortality-after-hematopoietic-stem-cell-transplantation-for-severe-mucopolysaccharidosis-type-i-the-30-year-university-of-minnesota-experience
#20
Nathan J Rodgers, Alexander M Kaizer, Weston P Miller, Kyle D Rudser, Paul J Orchard, Elizabeth A Braunlin
BACKGROUND AND AIM: Mucopolysaccharidosis IH (MPS IH, Hurler syndrome) naturally leads to death within the first decade of life, primarily from cardiac and pulmonary causes. To determine how hematopoietic stem cell transplantation (HSCT) has altered mortality, we analyzed our institution's 30-year experience of patients with MPS IH undergoing HSCT. METHODS: Using chart review and the National Death Index, we determined survival status of 134 patients (males = 69) with MPS IH transplanted between 9/16/1983 and 7/25/2013 on 12/31/2013...
January 4, 2017: Journal of Inherited Metabolic Disease
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