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https://www.readbyqxmd.com/read/29457839/brincidofovir-as-salvage-therapy-for-adenovirus-disease-in-intestinal-transplant-recipients
#1
Nimisha Sulejmani, Shunji Nagai, Mohamed Safwan, Michael D Rizzari, Mohammad Raoufi, Marwan S Abouljoud, Mayur Ramesh
Adenoviruses are double-stranded DNA viruses that typically cause mild self-limiting respiratory, ocular, and gastrointestinal infections. In immunocompromised patients, especially transplant recipients, the infection can be severe, with dissemination and multiorgan failure. In intestinal transplant recipients, the incidence is as high as 57%. To our knowledge, no standardized guidelines or United States Food and Drug Administration-approved medications exist for the treatment of adenovirus disease. We describe two isolated intestinal transplant recipients who developed adenovirus disease (viremia with viral enteritis) that was managed with a new experimental drug, brincidofovir (an oral lipid conjugate prodrug of cidofovir), as salvage therapy...
February 19, 2018: Pharmacotherapy
https://www.readbyqxmd.com/read/29456340/acute-febrile-encephalopathy-in-children-a-prospective-study-of-clinical-features-etiology-mortality-and-risk-factors-from-western-india
#2
Kirti Gupta, Charul S Purani, Anirban Mandal, Amitabh Singh
Introduction: Acute febrile encephalopathy (AFE) in children is a medical emergency and could be a manifestation of many systemic and central nervous system pathologies. The clinical features of AFE are nonspecific and etiological spectrum variable depending on the studied population. Materials and Methods: A prospective, observational study was carried out including children aged between 1 month and 12 years with AFE admitted to the Pediatric Intensive Care Unit of a tertiary care hospital in Western India...
January 2018: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/29455859/heterozygous-mutations-in-oas1-cause-infantile-onset-pulmonary-alveolar-proteinosis-with-hypogammaglobulinemia
#3
Kazutoshi Cho, Masafumi Yamada, Kazunaga Agematsu, Hirokazu Kanegane, Noriko Miyake, Masahiro Ueki, Takuma Akimoto, Norimoto Kobayashi, Satoru Ikemoto, Mishie Tanino, Atsushi Fujita, Itaru Hayasaka, Satoshi Miyamoto, Mari Tanaka-Kubota, Koh Nakata, Masaaki Shiina, Kazuhiro Ogata, Hisanori Minakami, Naomichi Matsumoto, Tadashi Ariga
Pulmonary alveolar proteinosis (PAP) is characterized by accumulation of a surfactant-like substance in alveolar spaces and hypoxemic respiratory failure. Genetic PAP (GPAP) is caused by mutations in genes encoding surfactant proteins or genes encoding a surfactant phospholipid transporter in alveolar type II epithelial cells. GPAP is also caused by mutations in genes whose products are implicated in surfactant catabolism in alveolar macrophages (AMs). We performed whole-exome sequence analysis in a family affected by infantile-onset PAP with hypogammaglobulinemia without causative mutations in genes associated with PAP: SFTPB, SFTPC, ABCA3, CSF2RA, CSF2RB, and GATA2...
February 12, 2018: American Journal of Human Genetics
https://www.readbyqxmd.com/read/29455226/respiratory-support-attitudes-among-pediatric-intensive-care-staff-for-spinal-muscular-atrophy-patients-in-saudi-arabia
#4
Mohamad-Hani A Temsah, Fahad M Al-Sohime, Fahad A Bashiri, Ayman A Al-Eyadhy, Gamal M Hasan, Ali A Alhaboob
OBJECTIVE: To explore therapeutic attitude of healthcare providers practicing in pediatric critical care in Saudi Arabia toward patients with Spinal Muscular Atroph (SMA) Type I, and to explore their awareness about the International Consensus statement for SMA care. METHODS: A cross-sectional survey was conducted in April 2015 during 6th Saudi Critical Care Conference, targeting physicians and respiratory therapists practicing in Pediatric Critical Care. RESULTS: Sixty participants accepted to participate in this survey...
January 2018: Neurosciences: the Official Journal of the Pan Arab Union of Neurological Sciences
https://www.readbyqxmd.com/read/29455044/timing-of-surgical-site-infection-and-pulmonary-complications-after-laparotomy
#5
Ossian Gundel, Sofie Kirchhoff Gundersen, Rikke Maria Dahl, Lars Nannestad Jørgensen, Lars S Rasmussen, Jørn Wetterslev, Ditte Sæbye, Christian S Meyhoff
BACKGROUND: Surgical site infection (SSI) and other postoperative complications are associated with high costs, morbidity, secondary surgery, and mortality. Many studies have identified factors that may prevent SSI and pulmonary complications, but it is important to know when they in fact occur. The aim of this study was to investigate the diagnostic timing of surgical site infections and pulmonary complications after laparotomy. MATERIAL AND METHODS: This is a secondary analysis of the PROXI trial which was a randomized clinical trial conducted in 1400 patients undergoing elective or emergent laparotomy...
February 15, 2018: International Journal of Surgery
https://www.readbyqxmd.com/read/29453889/mitochondrial-dysfunction-and-axon-degeneration-in-progressive-multiple-sclerosis
#6
REVIEW
Graham Campbell, Don Mahad
The neuron is the target of inflammatory demyelinating processes in multiple sclerosis (MS). In progressive MS, however, there is a gathering body of evidence indicating that molecular changes converge on mitochondria within neuronal cell bodies. The most reproducible change relates to mitochondrial respiratory chain complex deficiency, which compromises the capacity of neurons to generate ATP. The resulting energy failure state is coupled with an increase in demand for energy by the demyelinated axon, being particularly relevant to the long tracts such as corticospinal tracts with long projection axons...
February 17, 2018: FEBS Letters
https://www.readbyqxmd.com/read/29453415/erlin1-mutations-cause-teenage-onset-slowly-progressive-als-in-a-large-turkish-pedigree
#7
Ceren Tunca, Fulya Akçimen, Cemre Coşkun, Aslı Gündoğdu-Eken, Cemile Kocoglu, Betül Çevik, Can Ebru Bekircan-Kurt, Ersin Tan, A Nazlı Başak
Amyotrophic lateral sclerosis (ALS) is a late-onset motor neuron disease with mostly dominant inheritance and a life expectancy of 2-5 years; however, a quite common occurrence of atypical forms of the disease, due to recessive inheritance, has become evident with the use of NGS technologies. In this paper, we describe a family with close consanguinity for at least four generations, suffering from a slowly progressive form of ALS. Spastic walking is observed since teenage years, while bulbar symptoms start much later, at the fifth or sixth decade of life...
February 16, 2018: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/29451695/decreased-early-mortality-associated-with-the-treatment-of-acute-myeloid-leukemia-at-national-cancer-institute-designated-cancer-centers-in-california
#8
Gwendolyn Ho, Ted Wun, Lori Muffly, Qian Li, Ann Brunson, Aaron S Rosenberg, Brian A Jonas, Theresa H M Keegan
BACKGROUND: To the authors' knowledge, few population-based studies to date have evaluated the association between location of care, complications with induction therapy, and early mortality in patients with acute myeloid leukemia (AML). METHODS: Using linked data from the California Cancer Registry and Patient Discharge Dataset (1999-2014), the authors identified adult (aged ≥18 years) patients with AML who received inpatient treatment within 30 days of diagnosis...
February 16, 2018: Cancer
https://www.readbyqxmd.com/read/29450782/-is-the-refusal-of-invasive-or-noninvasive-ventilation-in-a-child-with-spinal-muscular-atrophy-ethically-justified
#9
Martina Radner, Annette Henry
We report about an infant who was diagnosed with spinal muscular atrophy type 1 (Werdnig-Hoffmann) at the age of 7 weeks. In a detailed discussion with the physicians the parents decided against mechanical ventilation in the event of either acute or chronical respiratory failure. During care at home all palliative actions were taken to optimize the quality of life for the child. The boy died at the age of 7 months at home. Whether continuous mechanical ventilation in a child with spinal muscular atrophy type 1 should be the individual decision of the parents or whether it should never be offered by the physicians is discussed controversially in the present medical literature...
February 15, 2018: Wiener Medizinische Wochenschrift
https://www.readbyqxmd.com/read/29450594/position-paper-for-the-organization-of-ecmo-programs-for-cardiac-failure-in-adults
#10
Darryl Abrams, A Reshad Garan, Akram Abdelbary, Matthew Bacchetta, Robert H Bartlett, James Beck, Jan Belohlavek, Yih-Sharng Chen, Eddy Fan, Niall D Ferguson, Jo-Anne Fowles, John Fraser, Michelle Gong, Ibrahim F Hassan, Carol Hodgson, Xiaotong Hou, Katarzyna Hryniewicz, Shingo Ichiba, William A Jakobleff, Roberto Lorusso, Graeme MacLaren, Shay McGuinness, Thomas Mueller, Pauline K Park, Giles Peek, Vin Pellegrino, Susanna Price, Erika B Rosenzweig, Tetsuya Sakamoto, Leonardo Salazar, Matthieu Schmidt, Arthur S Slutsky, Christian Spaulding, Hiroo Takayama, Koji Takeda, Alain Vuylsteke, Alain Combes, Daniel Brodie
Extracorporeal membrane oxygenation (ECMO) has been used increasingly for both respiratory and cardiac failure in adult patients. Indications for ECMO use in cardiac failure include severe refractory cardiogenic shock, refractory ventricular arrhythmia, active cardiopulmonary resuscitation for cardiac arrest, and acute or decompensated right heart failure. Evidence is emerging to guide the use of this therapy for some of these indications, but there remains a need for additional evidence to guide best practices...
February 15, 2018: Intensive Care Medicine
https://www.readbyqxmd.com/read/29450214/-mycobacterium-abscessus-complex-infections-a-retrospective-cohort-study
#11
Maroun Sfeir, Marissa Walsh, Rossana Rosa, Laura Aragon, Sze Yan Liu, Timothy Cleary, Marylee Worley, Corey Frederick, Lilian M Abbo
Background: Infections caused by Mycobacterium abscessus group strains are usually resistant to multiple antimicrobials and challenging to treat worldwide. We describe the risk factors, treatment, and clinical outcomes of patients in 2 large academic medical centers in the United States. Methods: A retrospective cohort study of hospitalized adults with a positive culture for M. abscessus in Miami, Florida (January 1, 2011, to December 31, 2014). Demographics, comorbidities, the source of infection, antimicrobial susceptibilities, and clinical outcomes were analyzed...
February 2018: Open Forum Infectious Diseases
https://www.readbyqxmd.com/read/29449947/reversible-platypnoea-orthodeoxia-syndrome-in-post-tuberculosis-bronchial-stenosis
#12
Geak Poh Tan, John Arputhan Abisheganaden, Soon Keng Goh, Akash Verma
Bronchial stenosis is known to complicate endobronchial tuberculosis despite medical therapy. It is often associated with dyspnoea. In severe cases, bronchial stenosis results in airflow obstruction, impaired secretion clearance, and can lead to respiratory failure. We present an unusual observation of platypnoea-orthodeoxia syndrome in a young woman with acute atelectasis due to post-tuberculosis bronchial stricture. Imaging revealed complete middle and right lower lobe atelectasis with a partially aerated right upper lobe...
April 2018: Respirology Case Reports
https://www.readbyqxmd.com/read/29449425/young-and-middle-aged-adults-with-airflow-limitation-according-to-lower-limit-of-normal-but-not-fixed-ratio-have-high-morbidity-and-poor-survival-a-population-based-prospective-cohort-study
#13
Yunus Çolak, Shoaib Afzal, Børge G Nordestgaard, Jørgen Vestbo, Peter Lange
A presumed consequence of using a fixed ratio for the definition of airflow limitation (AFL) has been overdiagnosis among older and underdiagnosis among younger individuals. However, the prognosis of younger individuals with potentially underdiagnosed airflow limitation is poorly described. We hypothesised that potential underdiagnosis of AFL at younger age is associated with poor prognosis.We assigned 95 288 participants aged 20-100 years from the Copenhagen General Population Study into: individuals without AFL with forced expiratory volume in 1 s (FEV 1 )/forced vital capacity (FVC)≥0...
February 15, 2018: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/29446472/hantavirus-induced-cardiopulmonary-syndrome-a-public-health-concern
#14
REVIEW
Sibghat Tul Llah, Sheema Mir, Sumaiya Sharif, Salman Khan, Mohammed A Mir
Hantavirus cardiopulmonary syndrome is characterized by pulmonary capillary leakage and alveolar flooding, resulting in 50% mortality due to fulminant hypoxic respiratory failure. In addition, depression of cardiac function ensues, which complicates the picture with cardiogenic shock. Early diagnosis and appropriate use of extracorporeal membrane oxygenation (ECMO) are amongst the lifesaving interventions in this fatal illness. However, a recent case report demonstrates that implementation of high volume continuous hemofilteration along with protective ventilation reverses the cardiogenic shock within few hours in hantavirus infected patients...
February 15, 2018: Journal of Medical Virology
https://www.readbyqxmd.com/read/29445539/the-clinical-usefulness-of-a-self-administered-questionnaire-for-sleep-disordered-breathing-in-patients-with-neuromuscular-disease
#15
Cathy Zhang, Michelle Ramsay, Panagis Drakatos, Joerg Steier
Background: Patients with neuromuscular disease (NMD) are at risk of developing sleep-disordered breathing (SDB) with hypercapnic respiratory failure. We hypothesised that a self-administered questionnaire (SiNQ-5 scores) may be useful to assess patients who are established on treatment for NMD with SDB. Methods: Patients attending a tertiary referral centre filled in the SiNQ-5 (range 0-10 points, lower scores indicating fewer symptoms). The questionnaire contains five questions related to breathlessness, sleep and posture...
January 2018: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29445537/neural-respiratory-drive-and-cardiac-function-in-patients-with-obesity-hypoventilation-syndrome-following-initiation-of-non-invasive-ventilation
#16
Angelo Onofri, Maxime Patout, Georgios Kaltsakas, Elodie Lhuillier, Sitali Mushemi-Blake, Gill Arbane, Martino F Pengo, Philip Marino, Joerg Steier
Background: Chronic hypercapnic respiratory failure (HRF) in obesity hypoventilation syndrome (OHS) is commonly treated using non-invasive ventilation (NIV). We hypothesised that treatment of OHS would improve neural respiratory drive index (NRDI) and cardiac function. Methods: Fourteen patients (8 females) with OHS, who were admitted for initiation of domiciliary NIV, were prospectively studied. Patients had (mean ± SD): age (53±10 years), body mass index (BMI) (50...
January 2018: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29445532/sleep-disordered-breathing-in-motor-neurone-disease
#17
REVIEW
Rebecca F D'Cruz, Patrick B Murphy, Georgios Kaltsakas
Motor neurone disease (MND) is a neurodegenerative disease defined by axonal loss and gliosis of upper and lower motor neurones in the motor cortex, lower brainstem nuclei and ventral horn of the spinal cord. MND is currently incurable and has a poor prognosis, with death typically occurring 3 to 5 years after disease onset. The disease is characterised by rapidly progressive weakness leading to paralysis, fasciculations, bulbar symptoms (including dysarthria and dysphagia) and respiratory compromise. Respiratory complications arise as a result of weakness of upper airway (pharyngeal and laryngeal) muscles and respiratory muscles (diaphragm, intercostal and accessory muscles) leading to respiratory failure...
January 2018: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29445530/update-non-invasive-ventilation-in-chronic-obstructive-pulmonary-disease
#18
REVIEW
Neeraj Mukesh Shah, Rebecca Francesca D'Cruz, Patrick B Murphy
Chronic obstructive pulmonary disease (COPD) remains a common cause of morbidity and mortality worldwide. Patients with COPD and respiratory failure, whether acute or chronic have a poorer prognosis than patients without respiratory failure. Non-invasive ventilation (NIV) has been shown to be a useful tool in both the acute hospital and chronic home care setting. NIV has been well established as the gold standard therapy for acute decompensated respiratory failure complicating an acute exacerbation of COPD with reduced mortality and intubation rates compared to standard therapy...
January 2018: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29445509/a-case-of-paraplegia-that-developed-6-years-after-thoracic-endovascular-aortic-repair-for-blunt-traumatic-aortic-injury
#19
Hiroyuki Takahashi, Tomohisa Shoko, Fumino Taketazu, Keiichi Kuriyama, Kazuhide Yoshikawa, Yoshizumi Deguchi
Case: Thoracic endovascular aortic repair (TEVAR) is becoming the standard therapy for blunt thoracic aortic injury (BTAI). However, the long-term outcomes of TEVAR for BTAI remain unclear. A 36-year-old man was admitted to our emergency department with dyspnea. He had been involved in a serious traffic accident 6 years earlier, requiring TEVAR for BTAI. Outcome: Acute heart failure and pneumonia were diagnosed on this admission. His respiratory condition improved, but paraplegia developed 10 h after hospitalization...
January 2018: Acute Medicine & Surgery
https://www.readbyqxmd.com/read/29445001/interventional-treatment-of-severe-tricuspid-regurgitation-early-clinical-experience-in-a-multicenter-observational-first-in-man-study
#20
Alexander Lauten, Hans R Figulla, Axel Unbehaun, Neil Fam, Joachim Schofer, Torsten Doenst, Joerg Hausleiter, Marcus Franz, Christian Jung, Henryk Dreger, David Leistner, Brunilda Alushi, Anja Stundl, Ulf Landmesser, Volkmar Falk, Karl Stangl, Michael Laule
BACKGROUND: Transcatheter caval valve implantation is under evaluation as a treatment option for inoperable patients with severe tricuspid regurgitation (TR). The procedure involves the catheter-based implantation of bioprosthetic valves in the inferior vena cava and superior vena cava to treat symptoms associated with TR. This study is the first to evaluate the feasibility, safety, and efficacy of this interventional concept. METHODS AND RESULTS: Twenty-five patients (mean age, 73...
February 2018: Circulation. Cardiovascular Interventions
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