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https://www.readbyqxmd.com/read/29115094/the-first-successful-lung-transplantation-in-a-korean-child-with-cystic-fibrosis
#1
Soo Ran Noh, Eun Lee, Jisun Yoon, Sungsu Jung, Song I Yang, Jinho Yu, Soo Jong Hong
Cystic fibrosis (CF) is an autosomal recessive inherited multisystem disorder caused by mutations of the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Respiratory failure remains the most frequent cause of morbidity and mortality. Lung transplantation is the only option to treat end-stage lung disease. Very few cases of CF occur in Koreans. We report the case of a 12-year-old girl with respiratory failure due to CF who underwent lung transplantation. She had been diagnosed with CF 8 years previously after being treated for recurrent Pseudomonas aeruginosa pneumonia and malnutrition based on sweat chloride concentrations and the CFTR protein gene mutation test...
December 2017: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/29064176/calciphylaxis-after-parathyroidectomy
#2
Sathish Karmegam, Anupkumar Shetty
A 60-year-old African American man with end stage renal disease on hemodialysis (HD) for the past 2.5 years developed severe hyperparathyroidism. Other past medical history included atrial fibrillation, type II diabetes mellitus, hypertension, gout, pericardial effusion needing pericardial window, deep vein thrombosis, mitral insufficiency, and cardiomyopathy with implantable cardioversion device placement. His parathyroid hormone (PTH) level peaked at 4,191 pg/mL despite being on cinacalcet, sevelamer, and paricalcitol...
October 2017: Hemodialysis International
https://www.readbyqxmd.com/read/29059075/extracorporeal-membrane-oxygenation-berlin-and-ventricular-assist-devices-a-primer-for-the-cardiologist
#3
Charlotte S Van Dorn, Devon O Aganga, Jonathan N Johnson
PURPOSE OF REVIEW: Mechanical circulatory support (MCS) has become an indispensable tool in the management of children with impending respiratory and cardiac failure. Though extracorporeal membrane oxygenation (ECMO) was classically the only form of support available to pediatric patients, considerable advances have allowed ventricular assist devices (VADs) to become increasingly utilized in children. This review provides an update of recent advances in ECMO and VAD management in children...
October 20, 2017: Current Opinion in Cardiology
https://www.readbyqxmd.com/read/29033442/efficacy-of-oxycodone-for-dyspnea-in-end-stage-heart-failure-with-renal-insufficiency-a-case-report
#4
Masayuki Tanaka, Hirofumi Maeba, Takeshi Senoo, Junji Iwasaka, Aki Ohkita, Haruna Kita, Kazuki Uchitani, Yasuhiko Hirota
A 67-year-old man with dilated cardiomyopathy and renal insufficiency was admitted to our hospital with dyspnea secondary to end-stage heart failure. We introduced oxycodone for medically refractory dyspnea instead of morphine because of the patient's renal insufficiency. After the administration of oxycodone, his dyspnea was alleviated without any adverse opioid effects, such as respiratory depression. After treating his heart failure, he was able to leave the intensive care unit. Oxycodone may therefore be a reliable agent for the treatment of dyspnea in patients with end-stage heart failure and renal insufficiency...
October 16, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28986001/a-bioprosthetic-total-artificial-heart-for-end-stage-heart-failure-results-from-a-pilot-study
#5
Christian Latrémouille, Alain Carpentier, Pascal Leprince, Jean-Christian Roussel, Bernard Cholley, Elodie Boissier, Eric Epailly, Antoine Capel, Piet Jansen, David M Smadja
BACKGROUND: The electro-hydraulically actuated Carmat total artificial heart (C-TAH) is designed to replace the heart in patients with end-stage heart failure, either as bridge to transplant or destination therapy. It provides pulsatile flow and contains bio-prosthetic blood contacting materials. A clinical feasibility study was conducted to evaluate the C-TAH safety and performance. METHODS: Hospitalized patients, at imminent risk of death from irreversible biventricular failure despite optimal medical management, and not eligible for transplant or eligible but on extracorporeal life support, were enrolled...
September 14, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/28985703/escalation-of-extracorporeal-life-support-as-a-bridge-to-lung-transplantation-in-end-stage-lung-disease
#6
Prashant N Mohite, Alexander Rosenberg, Clara Hernández Caballero, Simona Soresi, Javid Fatullayev, Anna Reed, Aron-Frederik Popov, Anton Sabashnikov, André R Simon
Extracorporeal membrane oxygenation (ECMO) as a bridge to lung transplant (LTx) is not uncommon, but it is not commonplace yet. We present a case of a 45-year-old man with cystic fibrosis with recent deterioration in lung function who was initially supported with veno-venous (VV) ECMO. However, he subsequently required conversion to veno-veno-arterial (VVA) ECMO. After 21 days of support, he underwent double lung transplantation, with an uneventful postoperative course. This case shows that, in patients with end-stage respiratory failure awaiting lung transplantation, extracorporeal life support may require escalation to improve gas exchange and address circulatory requirements...
October 2017: Perfusion
https://www.readbyqxmd.com/read/28932576/outcomes-after-lung-transplantation
#7
REVIEW
Gabriel Thabut, Herve Mal
With more than 50,000 procedures having been performed worldwide, lung transplantation (LT) has become the standard of care for patients with end-stage chronic respiratory failure. LT leads to dramatic improvements in both pulmonary function and health related quality of life. Survival after LTs has steadily improved, but still lags far behind that observed after other solid organ transplantations, as evidenced by a median survival rate that currently stands at 5.8 years. Because of these disappointing results, the ability of LT to expand survival has been questioned...
August 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28911947/administration-of-albumin-solution-increases-serum-levels-of-albumin-in-patients-with-chronic-liver-failure-in-a-single-arm-feasibility-trial
#8
Louise China, Simon S Skene, Zainib Shabir, Alexander Maini, Yvonne Sylvestre, Kate Bennett, Scott Bevan, James O'Beirne, Ewan Forrest, Jim Portal, Steve Ryder, Gavin Wright, Derek W Gilroy, Alastair O'Brien
BACKGROUND & AIMS: Infections are life-threatening to patients with acute decompensation and acute-on-chronic liver failure (AD/ACLF). Patients with AD/ACLF have prostaglandin E2-mediated immune suppression, which can be reversed by administration of albumin; infusion of 20% human albumin solution (HAS) might improve outcomes of infections. We performed a feasibility study to determine optimal trial design, assess safety, and validate laboratory assessments of immune function to inform design of a phase 3 trial...
September 12, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28906399/characteristics-of-infection-and-its-impact-on-short-term-outcome-in-patients-with-acute-on-chronic-liver-failure
#9
Junjun Cai, Mengchen Zhang, Tao Han, Hui-Qing Jiang
Bacterial infections are an important cause of mortality in liver failure. However, the type of infection, predictors of infection, and their impact on outcomes in patients with acute-on-chronic liver failure (ACLF) are limited.A total of 389 patients with ACLF were admitted in this retrospective, corhort study. Once admitted, clinical data including first infection site, type (community-acquired, healthcare-associated, or nosocomial), and second infection occurrence during hospitalization were collected. The outcome was mortality within 90 days...
September 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28893830/assessment-of-paravalvular-leakage-after-transcatheter-aortic-valve-implantation-add-clinical-signs-to-echocardiographic-data
#10
Corstiaan A den Uil, Mihai Strachinaru, Ben van der Hoven, J Han J Meeder
A 62-year old male with pulmonary fibrosis and aortic valve stenosis underwent TAVI (Corevalve Evolut XL 34 mm) in the build-up for lung transplantation (LuTx). Following post-dilation for a large annular perimeter, moderate paravalvular aortic regurgitation (AR) was observed. After 6 weeks, he was admitted to the ICU with respiratory failure(A). TTE demonstrated residual mild AR (based on a small excentric paravalvular AR jet, <10% circumferential extent, pressure half-time was not reliably measured) and together with borderline elevated NT-pro-BNP, the consultant cardiologist authorized LuTx listing...
September 11, 2017: Echo Research and Practice
https://www.readbyqxmd.com/read/28838437/urgent-lung-transplantation-in-severe-acute-respiratory-failure-based-on-rapidly-progressive-interstitial-lung-disease-a-case-report
#11
I Madurka, J Elek, N Schönauer, T Bartók, K Kormosói-Tóth, É Zöllei, Á Ghimessy, G Lang, W Klepetko, F Rényi-Vámos
Lung transplantation (LUTX) became a worldwide accepted standard therapy for certain well-defined chronic end-stage lung diseases. Until recently, patients on mechanical ventilation or extracorporeal life support techniques were hardly eligible for LUTX because of the inferior short-term results. However, a paradigm shift has occurred, and now these techniques represent bridging options to LUTX for listed patients. In the current practice, transplantation from the intensive care unit (ICU) is not extraordinary in patients on the waiting list...
September 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28836192/the-bleomycin-model-of-pulmonary-fibrosis
#12
Tianju Liu, Francina Gonzalez De Los Santos, Sem H Phan
Interstitial lung disease (ILD) comprises a large number of chronic lung disease characterized by varying degrees of inflammation and fibrosis. Mostly they are idiopathic including idiopathic pulmonary fibrosis (IPF), which is a specific disorder characterized by progressive fibrosis leading commonly to end-stage lung disease, respiratory failure, and fatal outcome. IPF and many of these fibrotic ILDs lack effective therapy despite recent approval of two drugs to slow progression in certain IPF patients. Because there are no natural models for IPF, the use of animal models that reproduce key known features of the disease is warranted...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28822818/phrenic-long-term-facilitation-following-intrapleural-ctb-sap-induced-respiratory-motor-neuron-death
#13
Nicole L Nichols, Taylor A Craig, Miles A Tanner
Amyotrophic lateral sclerosis (ALS) is a devastating disease leading to progressive motor neuron degeneration and death by ventilatory failure. In a rat model of ALS (SOD1(G93A)), phrenic long-term facilitation (pLTF) following acute intermittent hypoxia (AIH) is enhanced greater than expected at disease end-stage but the mechanism is unknown. We suggest that one trigger for this enhancement is motor neuron death itself. Intrapleural injections of cholera toxin B fragment conjugated to saporin (CTB-SAP) selectively kill respiratory motor neurons and mimic motor neuron death observed in SOD1(G93A) rats...
August 16, 2017: Respiratory Physiology & Neurobiology
https://www.readbyqxmd.com/read/28816877/fractures-in-spinal-ankylosing-disorders-a-narrative-review-of-disease-and-injury-types-treatment-techniques-and-outcomes
#14
Tarush Rustagi, Doniel Drazin, Cumhur Oner, Jonathan York, Gregory D Schroeder, Alexander R Vaccaro, Rod J Oskouian, Jens R Chapman
INTRODUCTION: Spinal ankylosing disorders encompass ankylosing spondylitis (AS), disseminated hyperostosis of the spine, and end-stage spondylosis. All these result in a stiffened and frequently deformed spinal column. This makes the spinal column highly susceptible to severe injuries that are commonly associated with unfavorable outcomes. Improved understanding of the underlying disease processes and clinical comorbidities may alter the poor injury related morbidity and mortality outcomes...
September 2017: Journal of Orthopaedic Trauma
https://www.readbyqxmd.com/read/28813495/establishing-failure-predictors-for-the-planned-extubation-of-overweight-and-obese-patients
#15
Chien-Ming Chao, Chih-Cheng Lai, Ai-Chin Cheng, Shyh-Ren Chiang, Wei-Lun Liu, Chung-Han Ho, Shu-Chen Hsing, Chin-Ming Chen, Kuo-Chen Cheng
We investigated failure predictors for the planned extubation of overweight (body mass index [BMI] = 25.0-29.9) and obese (BMI ≥ 30) patients. All patients admitted to the adult intensive care unit (ICU) of a tertiary hospital in Taiwan were identified. They had all undergone endotracheal intubation for > 48 h and were candidates for extubation. During the study, 595 patients (overweight = 458 [77%]); obese = 137 [23%]) with planned extubation after weaning were included in the analysis; extubation failed in 34 patients (5...
2017: PloS One
https://www.readbyqxmd.com/read/28772036/implantation-of-a-left-ventricular-assist-device-to-provide-long-term-support-for-end-stage-duchenne-muscular-dystrophy-associated-cardiomyopathy
#16
Douglas Stoller, Faris Araj, Alpesh Amin, Catherine Fitzsimmons, Robert Morlend, Jennifer T Thibodeau, Claudio Ramaciotti, Mark H Drazner, Dan M Meyer, Pradeep P A Mammen
A young man with Duchenne muscular dystrophy presented to the UT Southwestern Neuromuscular Cardiomyopathy Clinic with advanced heart failure. Despite maximal medical therapy, his cardiac function continued to decline requiring initiation of inotrope therapy. Given the patient's clinical deterioration, a left ventricular assist device (LVAD) was implanted as destination therapy after undergoing a multidisciplinary assessment. The patient tolerated the surgical implantation of the LVAD without any significant complications, and he has had a relatively unremarkable course 38 months post-LVAD implantation...
August 2017: ESC Heart Failure
https://www.readbyqxmd.com/read/28754440/to-intubate-or-not-to-intubate-emergency-medicine-physicians-perspective-on-intubating-critically-ill-terminal-cancer-patients
#17
Kenneth Kim, Bharath Chakravarthy, Craig Anderson, Solomon Liao
CONTEXT: Emergency physicians (EPs) often need to make a decision whether or not to intubate a terminal cancer patient. OBJECTIVE: The objective of this study was to explore EPs' attitudes about intubating critically ill, terminal cancer patients. METHODS: Fifty EPs at three emergency departments (one university based, one community, and one Health Maintenance Organization) in Southern California participated in an anonymous survey that presented a hypothetical case of an end-stage lung cancer patient in pending respiratory failure...
November 2017: Journal of Pain and Symptom Management
https://www.readbyqxmd.com/read/28736018/extracorporeal-life-support-as-a-bridge-to-lung-transplantation-in-patients-with-acute-respiratory-failure
#18
H J Yeo, S Lee, S H Yoon, S E Lee, W H Cho, D Jeon, Y S Kim, D Kim
BACKGROUND: Extracorporeal membrane oxygenation (ECMO) is being used more often as a bridge to transplantation (BTT) in patients with acutely decompensated end-stage lung disease in Korea. ECMO as a BTT may be the only rescue strategy for severe acute respiratory failure, but many centers still consider it to be a relative contraindication to lung transplantation because of its poor outcome. Because there are not enough lung donors, it is important to determine their optimal use. We reviewed and analyzed our experiences with the use of ECMO as a BTT in patients with acute respiratory failure...
July 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28557861/heartmate-3-in-lowest-intermacs-profile-cohort-the-swiss-experience
#19
Piergiorgio Tozzi, Carlo Banfi, Kameran Ahmadov, Roger Hullin, Philippe Meyer, Raphael Giraud, Lucas Liaudet, Fabrizio Gronchi, Christophe Huber, Matthias Kirsch
New generation devices for long-term mechanical circulatory support are centrifugal pumps having fully magnetically levitated rotors to reduce blood trauma. Recently, the novel HeartMate 3 was cleared for clinical application in Switzerland. In two Swiss University Hospitals part of the "Lausanne-Geneva Transplantation Network," 10 consecutive patients in end-stage heart failure received the HeartMate 3 (Thoratec Corporation, Pleasanton, CA). Device implantation criteria were persistent low output syndrome despite optimal medical treatment...
November 2017: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
https://www.readbyqxmd.com/read/28526246/impaired-mitophagy-facilitates-mitochondrial-damage-in-danon-disease
#20
Sherin I Hashem, Anne N Murphy, Ajit S Divakaruni, Matthew L Klos, Bradley C Nelson, Emily C Gault, Teisha J Rowland, Cynthia N Perry, Yusu Gu, Nancy D Dalton, William H Bradford, Eric J Devaney, Kirk L Peterson, Kenneth L Jones, Matthew R G Taylor, Ju Chen, Neil C Chi, Eric D Adler
RATIONALE: Lysosomal associated membrane protein type-2 (LAMP-2) is a highly conserved, ubiquitous protein that is critical for autophagic flux. Loss of function mutations in the LAMP-2 gene cause Danon disease, a rare X-linked disorder characterized by developmental delay, skeletal muscle weakness, and severe cardiomyopathy. We previously found that human induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) from Danon patients exhibited significant mitochondrial oxidative stress and apoptosis...
May 16, 2017: Journal of Molecular and Cellular Cardiology
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