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https://www.readbyqxmd.com/read/28914735/palliative-care-in-neuromuscular-diseases
#1
Marianne de Visser, David J Oliver
PURPOSE OF REVIEW: Palliative care is an approach that improves the quality of life of patients and their families facing the problem associated with life-threatening illness. Neuromuscular disorders (NMDs) are characterized by progressive muscle weakness, leading to pronounced and incapacitating physical disabilities. Most NMDs are not amenable to curative treatment and would thus qualify for palliative care. Amyotrophic lateral sclerosis is a relentlessly progressive disease, which leads to death about 2 years after onset due to respiratory muscle weakness...
September 13, 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28906030/pathological-and-immunoblot-analysis-of-phosphorylated-tdp-43-in-sporadic-amyotrophic-lateral-sclerosis-with-pallido-nigro-luysian-degeneration
#2
Akiko Uchino, Mieko Ogino, Junko Takahashi-Fujigasaki, Saori Oonuma, Naomi Kanazawa, Sabine Kajita, Masaaki Ichinoe, Masato Hasegawa, Kazutoshi Nishiyama, Shigeo Murayama
Transactivation response DNA-binding protein 43 kDa (TDP-43) is a key protein of sporadic amyotrophic lateral sclerosis (ALS), and phosphorylated form of TDP-43 (p-TDP-43) is a major pathological protein that accumulates in sporadic ALS. p-TDP-43 is found not only in primary motor neurons, but often propagates to non-motor systems as well. However, pallido-nigro-luysian (PNL) degeneration (PNLD) is rarely associated with ALS. We describe here a 68-year-old ALS patient presenting severe PNLD. He had difficulty walking due to poor movement of his right leg, and was diagnosed as having Parkinson's disease because of akinesia...
September 14, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28904174/in-hospital-outcomes-of-tumor-lysis-syndrome-a-population-based-study-using-the-national-inpatient-sample
#3
Urshila Durani, Nilay D Shah, Ronald S Go
The epidemiology and outcomes of tumor lysis syndrome (TLS) are understudied. We used the National Inpatient Sample (NIS), a nationally representative weighted sample of all U.S. hospital discharges, to study outcomes and predictors of mortality in hospitalized patients with TLS. The NIS was queried for patients with a discharge diagnosis of TLS (ICD-9 code 277.88) from 2010-2013. Baseline characteristics and outcomes were analyzed. A multivariable logistic regression analysis was performed to identify predictors of mortality...
September 13, 2017: Oncologist
https://www.readbyqxmd.com/read/28887757/survival-in-idursulfase-treated-and-untreated-patients-with-mucopolysaccharidosis-type-ii-data-from-the-hunter-outcome-survey-hos
#4
Barbara K Burton, Virginie Jego, Jaromir Mikl, Simon A Jones
Mucopolysaccharidosis type II (MPS II; Hunter syndrome; OMIM 309900) is a life-limiting, multisystemic disease with varying presentation and severity. Enzyme replacement therapy with intravenous idursulfase (EC 3.1.6.13) has been available since 2006. Data from the Hunter Outcome Survey (July 2016) were used to compare survival in idursulfase-treated (n = 800) and untreated (n = 95) male patients followed prospectively in this multinational, observational registry. Median age at symptom onset was similar for the treated and untreated groups (1...
September 8, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28882233/prognostic-significance-of-central-apneas-throughout-a-24-hour-period-in-patients%C3%A2-with-heart-failure
#5
RANDOMIZED CONTROLLED TRIAL
Michele Emdin, Gianluca Mirizzi, Alberto Giannoni, Roberta Poletti, Giovanni Iudice, Francesca Bramanti, Claudio Passino
BACKGROUND: Large trials using noninvasive mechanical ventilation to treat central apnea (CA) occurring at night ("sleep apnea") in patients with systolic heart failure (HF) have failed to improve prognosis. The prevalence and prognostic value of CA during daytime and over an entire 24-h period are not well described. OBJECTIVES: This study evaluated the occurrence and prognostic significance of nighttime, daytime, and 24-h CA episodes in a large cohort of patients with systolic HF...
September 12, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28880805/a-prospective-one-year-microbiologic-survey-of-combined-pneumonia-and-respiratory-failure
#6
Kristen Fisher, Tracy Trupka, Scott T Micek, Paul Juang, Marin H Kollef
BACKGROUND: Pneumonia and respiratory failure are common problems in the intensive care unit (ICU) setting, often occurring together. The relative prevalence of pneumonia types (community acquired, hospital acquired, ventilator associated) and causative pathogens is not well described in patients with respiratory failure. METHODS: This was a prospective observational cohort study conducted in the medical ICU (34 beds) of Barnes-Jewish Hospital, an academic referral center of 1,300 beds from January 2016-December 2016...
September 7, 2017: Surgical Infections
https://www.readbyqxmd.com/read/28874089/outcome-of-surgeon-modified-fenestrated-branched-stent-grafts-for-symptomatic-complex-aortic-pathologies-or-contained-rupture
#7
Nikolaos Tsilimparis, Franziska Heidemann, Fiona Rohlffs, Holger Diener, Sabine Wipper, E Sebastian Debus, Tilo Kölbel
PURPOSE: To analyze the outcome of surgeon-modified fenestrated and branched stent-grafts (sm-FBSG) in high-risk patients with symptomatic complex aortic pathology or contained rupture. METHODS: A single-center retrospective analysis was conducted of 21 consecutive patients (mean age 70 years, range 58-87; 16 men) treated with a sm-FBSG from April 2014 to September 2016. The indications included 11 thoracoabdominal and 10 pararenal aortic pathologies, which presented as symptomatic in 8 and as contained rupture in 13 patients...
September 1, 2017: Journal of Endovascular Therapy
https://www.readbyqxmd.com/read/28871936/xanthoma-like-skin-changes-in-an-elderly-woman-with-a-normal-lipid-profile
#8
Piotr Nockowski, Zdzisław Woźniak, Adam Reich, Joanna Maj
Dear Editor, An 83-year-old woman developed yellow-brownish infiltrates, nodules, and tumors mimicking xanthomas, mostly involving the periorbital and chest area within three months (Figure 1). She had no abnormalities in serum cholesterol or triglycerides levels. A detailed laboratory analysis revealed the presence of mild monoclonal gammopathy with a presence of immunoglobulin G (IgG) kappa light chains; however, according to hematologist consultation, it did not require medical intervention. Imaging assessment and ultrasound examination did not show any specific involvement of internal organs...
July 2017: Acta Dermatovenerologica Croatica: ADC
https://www.readbyqxmd.com/read/28858213/recreational-exposure-during-algal-bloom-in-carrasco-beach-uruguay-a-liver-failure-case-report
#9
Flavia Vidal, Daniela Sedan, Daniel D'Agostino, María Lorena Cavalieri, Eduardo Mullen, María Macarena Parot Varela, Cintia Flores, Josep Caixach, Dario Andrinolo
In January 2015, a 20-month-old child and her family took part in recreational activities at Carrasco and Malvín beaches (Montevideo, Uruguay). An intense harmful algae bloom (HAB) was developing along the coast at that time. A few hours after the last recreational exposure episode, the family suffered gastrointestinal symptoms which were self-limited except in the child's case, who was admitted to hospital in Uruguay with diarrhea, vomiting, fatigue, and jaundice. The patient had increased serum levels of liver enzymes and bilirubin and five days later presented acute liver failure...
August 31, 2017: Toxins
https://www.readbyqxmd.com/read/28837149/idh3-mediates-apoptosis-of-alveolar-epithelial-cells-type-2-due-to-mitochondrial-ca-2-uptake-during-hypocapnia
#10
Martina Kiefmann, Sascha Tank, Paula Keller, Christian Börnchen, Jan L Rinnenthal, Marc-Oliver Tritt, Leonie Schulte-Uentrop, Cynthia Olotu, Alwin E Goetz, Rainer Kiefmann
In adult respiratory distress syndrome (ARDS) pulmonary perfusion failure increases physiologic dead-space (VD/VT) correlating with mortality. High VD/VT results in alveolar hypocapnia, which has been demonstrated to cause edema formation, atelectasis, and surfactant depletion, evoked, at least in part, by apoptosis of alveolar epithelial cells (AEC). However, the mechanism underlying the hypocapnia-induced AEC apoptosis is unknown. Here, using fluorescent live-cell imaging of cultured AEC type 2 we could show that in terms of CO2 sensing the tricarboxylic acid cycle enzyme isocitrate dehydrogenase (IDH) 3 seems to be an important player because hypocapnia resulted independently from pH in an elevation of IDH3 activity and subsequently in an increase of NADH, the substrate of the respiratory chain...
August 24, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28812466/florid-intussusceptive-like-microvascular-dysangiogenesis-in-a-preterm-lung
#11
Monique E De Paepe, Merline Kocheekkaran V Benny, Lauren Priolo, Francois I Luks, Svetlana Shapiro
The cellular mechanisms underlying the microvascular dysangiogenesis of bronchopulmonary dysplasia (chronic lung disease of the newborn) remain largely undetermined. We report unusual pulmonary vascular findings in a 27-week-gestation male newborn who died on the second day of life from intractable respiratory failure, following a pregnancy complicated by prolonged membrane rupture and persistent severe oligohydramnios. As expected, postmortem examination revealed pulmonary hypoplasia (lung/body weight ratio: 2...
September 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28808861/epidemiology-of-sleep-disordered-breathing-and-heart-failure-what-drives-what
#12
REVIEW
Sushma M Dharia, Lee K Brown
PURPOSE OF REVIEW: The bidirectional relationships that have been demonstrated between heart failure (HF) and central sleep apnea (CSA) demand further exploration with respect to the implications that each condition has for the other. This review discusses the body of literature that has accumulated on these relationships and how CSA and its potential treatment may affect outcomes in patients with CSA. RECENT FINDINGS: Obstructive sleep apnea (OSA) can exacerbate hypertension, type 2 diabetes, obesity, and atherosclerosis, which are known predicates of HF...
August 14, 2017: Current Heart Failure Reports
https://www.readbyqxmd.com/read/28799855/surgical-management-of-cervical-spine-deformity-in-chondrodysplasia-punctata
#13
Nobuhito Morota, Satoshi Ihara, Hideki Ogiwara, Goichiro Tamura
OBJECTIVE Chondrodysplasia punctata (CDP), a rare skeletal dysplasia, can lead to cervical spine instability and deformity. However, an optimal neurosurgical intervention has yet to be established. Thus, a retrospective study was conducted to assess the efficacy of various surgical interventions for children with CDP. METHODS The authors retrospectively reviewed 9 cases of CDP in which cervical decompression with or without posterior fusion was performed between April 2007 and May 2016. Patient demographics, preoperative clinical conditions, radiographic findings, surgical procedures, and the postoperative course were analyzed in detail...
August 11, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28794121/changes-in-primary-noncardiac-diagnoses-over-time-among-elderly-cardiac-intensive-care-unit-patients-in-the-united-states
#14
Shashank S Sinha, Michael W Sjoding, Devraj Sukul, Hallie C Prescott, Theodore J Iwashyna, Hitinder S Gurm, Colin R Cooke, Brahmajee K Nallamothu
BACKGROUND: Early reports suggest the number of cardiac intensive care unit (CICU) patients with primary noncardiac diagnoses is rising in the United States, but no national data currently exist. We examined changes in primary noncardiac diagnoses among elderly patients admitted to a CICU during the past decade. METHODS AND RESULTS: Using 2003 to 2013 Medicare data, we grouped elderly patients admitted to CICUs into 2 categories based on principal diagnosis at discharge: (1) primary noncardiac diagnoses and (2) primary cardiac diagnoses...
August 2017: Circulation. Cardiovascular Quality and Outcomes
https://www.readbyqxmd.com/read/28791006/regulation-of-lung-epithelial-sodium-channels-by-cytokines-and-chemokines
#15
REVIEW
Brandi M Wynne, Li Zou, Valerie Linck, Robert S Hoover, He-Ping Ma, Douglas C Eaton
Acute lung injury leading to acute respiratory distress (ARDS) is a global health concern. ARDS patients have significant pulmonary inflammation leading to flooding of the pulmonary alveoli. This prevents normal gas exchange with consequent hypoxemia and causes mortality. A thin fluid layer in the alveoli is normal. The maintenance of this thin layer results from fluid movement out of the pulmonary capillaries into the alveolar interstitium driven by vascular hydrostatic pressure and then through alveolar tight junctions...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28780379/proinflammatory-cytokine-mif-plays-a-role-in-the-pathogenesis-of-type-2-diabetes-mellitus-but-does-not-affect-hepatic-mitochondrial-function
#16
Miriam Rodriguez-Sosa, Tecilli Cabellos-Avelar, Yuriko Sanchez-Zamora, Imelda Juárez-Avelar, Esperanza García-Reyes, Alejandra Lira-León, José Del Carmen Benítez-Flores, Thalia Pacheco-Fernández, Marcia Hiriart, Emma Berta Gutiérrez-Cirlos
BACKGROUND: Macrophage migration inhibitory factor (MIF) is a proinflammatory cytokine that plays an important role in the pathogenesis of type 2 diabetes mellitus (T2DM). Although the effect of high glucose on liver function has been described, the role of MIF in hepatic mitochondrial function during T2DM has not been studied. OBJECTIVE: We examine the influence of MIF to hepatic mitochondrial function in T2DM mouse model. METHODS: WT and Mif(-/-) BALB/c mice were treated with a single dose of streptozotocin (STZ)...
August 2, 2017: Cytokine
https://www.readbyqxmd.com/read/28754713/ubap2l-is-amplified-in-a-large-subset-of-human-lung-adenocarcinoma-and-is-critical-for-epithelial-lung-cell-identity-and-tumor-metastasis
#17
Romain Aucagne, Simon Girard, Nadine Mayotte, Bernhard Lehnertz, Stéphane Lopes-Paciencia, Patrick Gendron, Geneviève Boucher, Jalila Chagraoui, Guy Sauvageau
The ubiquitin-associated protein 2-like (UBAP2L) gene remains poorly studied in human and mouse development. UBAP2L interacts with the Polycomb group protein B lymphoma Mo-MLV insertion region 1 homolog (BMI1) and determines the activity of mouse hematopoietic stem cells in vivo Here we show that loss of Ubap2l leads to disorganized respiratory epithelium of mutant neonates, which die of respiratory failure. We also show that UBAP2L overexpression leads to epithelial-mesenchymal transition-like phenotype in a non-small cell lung carcinoma (NSCLC) cell line...
July 28, 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/28741975/antibiotic-switch-during-treatment-with-antibiotics-against-respiratory-tract-infections-in-ambulatory-care-in-norway
#18
Mari Blandhol, Thekla Tysland, Hege Salvesen Blix, Sigurd Høye
OBJECTIVES: To compare antibiotic treatment failure evaluated as switch from one type of antibiotics to another in ambulatory care. METHODS: Data on all dispensed doxycycline, amoxicillin, phenoxymethylpenicillin and macrolides in Norway June 2013 - May 2015, was retrieved from the Norwegian Prescription Database. We computed switch rates for the selected antibiotics on day 1-28 after initial dispensing, and the corresponding odds-ratios, adjusted for patients´ age and gender, and prescribers´ specialty...
July 25, 2017: Infectious Diseases
https://www.readbyqxmd.com/read/28731281/glycaemia-control-and-the-risk-of-hospitalisation-for-infection-in-patients-with-type-2-diabetes-hong-kong-diabetes-registry
#19
Andrea O Y Luk, Eric S H Lau, Kitty K T Cheung, Alice P S Kong, Ronald C W Ma, Risa Ozaki, Francis C C Chow, Wing-Yee So, Juliana C N Chan
BACKGROUND: Infection occurs more commonly in diabetic patients compared with the general population and is an under-recognised but important morbidity in patients with diabetes. We examined the impact of glycaemic control on hospitalisation for infection in a large prospective cohort of Chinese adults with type 2 diabetes. METHODS: Between July 1994 and June 2014, 22 846 patients with type 2 diabetes underwent detailed assessment of metabolic control and diabetes complications...
July 21, 2017: Diabetes/metabolism Research and Reviews
https://www.readbyqxmd.com/read/28712563/trends-in-comorbidity-in-patients-hospitalised-for-cardiovascular-disease
#20
Josefien Buddeke, Michiel L Bots, Ineke van Dis, Anho Liem, Frank L J Visseren, Ilonca Vaartjes
BACKGROUND: We determined trends over time in cardiovascular and non-cardiovascular comorbidity in patients hospitalised for cardiovascular disease (CVD). METHODS: The Dutch nationwide hospital register was used to identify patients hospitalised for CVD during 2000-2010. Comorbidity was defined as a previous hospital admission for CVD other than the index CVD, cancer, diabetes, musculoskeletal and connective tissue disorders, respiratory disorders, thyroid gland disorders, kidney disorders and dementia in the five years previous to hospital admittance for the index CVD...
July 10, 2017: International Journal of Cardiology
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