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https://www.readbyqxmd.com/read/28087208/-child-langerhans-cell-histiocytosis
#1
Jean Donadieu, Sébastien Héritier
DEFINITION: Langerhans cell histiocytosis (LCH) is defined by the association of a clinical and radiological involvement and a biopsy of a pathological tissue. Extension: it can affect any organ or system of the body but most commonly the bone (80% of cases), the skin (33%) and the pituitary (25%). Other organs are concerned such as liver, spleen, hematopoietic system and the lungs (15% each), lymph nodes (5-10%) and central nervous system (CNS) excluding the pituitary (2-4%). Natural history: the natural history of the disease is very heterogeneous, ranging from auto-regressive lesions to a disease affecting multiple organs with fatal consequences, while some lesions may be responsible for permanent sequels...
January 10, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28087073/abnormal-mortality-of-octopus-after-a-storm-water-event-accumulated-lead-and-lead-isotopes-as-fingerprints
#2
J Raimundo, F Ruano, J Pereira, M Mil-Homens, P Brito, C Vale, M Caetano
Octopus vulgaris is a sedentary organism that inhabits coastal waters being exposed to anthropogenic compounds. Lead concentration in coastal environments reflects many processes and activities namely weathering, industrial and domestic discharges, and atmospheric deposition. Since lead isotopic composition is little affected by kinetic processes occurring between source and sink, its signature has been used to identify different Pb sources. After a short-term heavy rainfall, hundreds of octopus appeared dead in two Portuguese coastal areas...
January 10, 2017: Science of the Total Environment
https://www.readbyqxmd.com/read/28074774/the-dair-debridement-antibiotics-and-implant-retention-procedure-for-infected-total-knee-replacement-a-literature-review
#3
Sultan Naseer Qasim, Andrew Swann, Robert Ashford
Prosthetic joint infection (PJI) is a devastating complication in total knee arthroplasty (TKA) and third most common cause of revision of TKA with significant morbidity and surgical challenges. Treatment options include non-operative measures with long term antibiotic suppression, debridement and implant retention (DAIR), one- or two-stage revision arthroplasty, arthrodesis and amputation. Implant retention without infection is ideal and DAIR has been reported to have variable success rates depending on patient factors, duration of infection, infecting micro-organisms, choice of procedure, single or multiple debridement procedures, arthroscopic or open, antibiotic choice and duration of antibiotic use...
2017: SICOT-J
https://www.readbyqxmd.com/read/28066610/the-application-of-autologous-pulmonary-artery-in-surgical-correction-of-complicated-aortic-arch-anomaly
#4
Shusheng Wen, Jianzheng Cen, Jimei Chen, Gang Xu, Biaochuan He, Yun Teng, Jian Zhuang
BACKGROUND: In the patients with longer-segment aortic arch hypoplasia or interruption with ventricular septal defect, surgery with homograft vessel or autologous pericardial patch to augment descending aortic arch will not result in adverse reactions caused by end-to-end anastomosis. In this study, we retrospectively analyzed primary experience of surgical correction of complicated aortic arch anomaly with autologous main pulmonary artery. METHODS: From July 2010 to March 2016, the twenty-one cases of aortic arch complex anomalies were reconstructed with autologous main pulmonary artery...
November 2016: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28061988/treatment-of-high-risk-smoldering-myeloma
#5
REVIEW
Neha Korde
Multiple myeloma (MM) is a hematologic malignancy of the plasma cell that causes symptoms of bone pain, renal failure, and anemia. It is usually preceded by a precursor disease state, such as smoldering multiple myeloma (SMM) or monoclonal gammopathy of undetermined significance (MGUS), and traditional dogma dictates that treatment should be initiated on frank MM symptom development. Emerging evidence suggests that a defined group of "high-risk SMM" may benefit from early treatment, before organ damage and symptoms actually occur...
December 2016: Seminars in Oncology
https://www.readbyqxmd.com/read/28060934/a-cohort-study-of-adult-patients-with-severe-dengue-in-taiwanese-intensive-care-units-the-elderly-and-aptt-prolongation-matter-for-prognosis
#6
Chih-Cheng Hsieh, Cong-Tat Cia, Jen-Chieh Lee, Junne-Ming Sung, Nan-Yao Lee, Po-Lin Chen, Te-Hui Kuo, Jo-Yen Chao, Wen-Chien Ko
BACKGROUND: There was a large dengue outbreak in Taiwan in 2015, in which the ages of the affected individuals were higher than those in other countries. The aim of this study was to explore the characteristics and prognostic factors for adults with severe dengue in intensive care units (ICUs). METHODS: All adults admitted to ICUs with dengue virus infection (DENV) at a medical center from July 1, 2015 to December 31, 2015 were enrolled. DENV was diagnosed by the presence of serum NS1 antigen, IgM antibodies to dengue virus, or dengue virus RNA by real-time reverse transcriptase polymerase chain reaction...
January 6, 2017: PLoS Neglected Tropical Diseases
https://www.readbyqxmd.com/read/28059970/artesunate-protects-against-the-organ-injury-and-dysfunction-induced-by-severe-hemorrhage-and-resuscitation
#7
Regina Sordi, Kiran K Nandra, Fausto Chiazza, Florence L Johnson, Claudia P Cabrera, Hew D Torrance, Noriaki Yamada, Nimesh S A Patel, Michael R Barnes, Karim Brohi, Massimo Collino, Christoph Thiemermann
OBJECTIVE: To evaluate the effects of artesunate on organ injury and dysfunction associated with hemorrhagic shock (HS) in the rat. BACKGROUND: HS is still a common cause of death in severely injured patients and is characterized by impairment of organ perfusion, systemic inflammatory response, and multiple organ failure. There is no specific therapy that reduces organ injury/dysfunction. Artesunate exhibits pharmacological actions beyond its antimalarial activity, such as anticancer, antiviral, and anti-inflammatory effects...
February 2017: Annals of Surgery
https://www.readbyqxmd.com/read/28057640/murine-systemic-thrombophilia-and-hemolytic-uremic-syndrome-from-a-factor-h-point-mutation
#8
Yoshiyasu Ueda, Imran Mohammed, Delu Song, Damodar Gullipalli, Lin Zhou, Sayaka Sato, Yuan Wang, Shuchi Gupta, Zhongjian Cheng, Hong Wang, Jialing Bao, Yingying Mao, Lawrence Brass, X Long Zheng, Takashi Miwa, Matthew Palmer, Joshua Dunaief, Wen-Chao Song
Complement plays a key role in host defense but its dysregulation can cause autologous tissue injury. Complement activation is normally controlled by regulatory proteins including factor H (FH) in plasma and membrane cofactor protein (MCP) on the cell surface. Mutations in FH and MCP are linked to atypical hemolytic uremic syndrome, a type of thrombotic microangiopathy (TMA) that causes renal failure in patients. We describe here that disruption of FH function on the cell surface can also lead to disseminated complement-dependent macrovascular thrombosis...
January 5, 2017: Blood
https://www.readbyqxmd.com/read/28053267/assessment-of-resuscitation-as-measured-by-markers-of-metabolic-acidosis-and-features-of-injury
#9
D S Weinberg, A S Narayanan, T A Moore, H A Vallier
AIMS: The best time for definitive orthopaedic care is often unclear in patients with multiple injuries. The objective of this study was make a prospective assessment of the safety of our early appropriate care (EAC) strategy and to evaluate the potential benefit of additional laboratory data to determine readiness for surgery. PATIENTS AND METHODS: A cohort of 335 patients with fractures of the pelvis, acetabulum, femur, or spine were included. Patients underwent definitive fixation within 36 hours if one of the following three parameters were met: lactate < 4...
January 2017: Bone & Joint Journal
https://www.readbyqxmd.com/read/28050463/cardiofaciocutaneous-syndrome-case-report-of-a-rare-disorder
#10
Soutrik Seth, Tanmoy Biswas, Biswajit Biswas, Atanu Roy, Asok Kumar Datta
Cardiofaciocutaneous syndrome or CFC syndrome is a rare genetic disorder first described in 1986. It is one of the RASopathies involving multiple organs particularly the heart, skin and face affecting males and females equally. The phenotypic features overlap with 2 other conditions, the Noonan and Costello syndrome. We report on a 22-month-old boy with CFC syndrome presenting with typical craniofacial appearance, heart defects, ectodermal abnormalities, growth failure and developmental delay. Estimated population of affected individuals worldwide is a few hundreds...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28043527/overview-of-drug-resistant-tuberculosis-worldwide
#11
Ali A Velayati, Parissa Farnia, Amir M Farahbod
Even in the 21st century, we are losing the battle against eradication of tuberculosis (TB). In 2015, 9.6 million people were estimated to have fallen ill with TB, of which 1.5 million people died. This is the real situation despite the well-structured treatment programs and availability of effective treatment options since the 1950s. The high mortality rate has been associated with other risk factors, such as the HIV epidemic, underlying diseases, and decline of socioeconomic standards. Furthermore, the problem of drug resistance that was recognized in the early days of the chemotherapeutic era raises serious concerns...
December 2016: International Journal of Mycobacteriology
https://www.readbyqxmd.com/read/28041799/eif2b-related-multisystem-disorder-in-two-sisters-with-atypical-presentations
#12
Jin Sook Lee, Sangmoon Lee, Murim Choi, Byung Chan Lim, Jieun Choi, Ki Joong Kim, Jung-Eun Cheon, In-One Kim, Jong-Hee Chae
BACKGROUND: Vanishing white matter disease (VWM) is a chronic progressive leukoencephalopathy that is characterized by cerebellar ataxia and spasticity, together with cystic degeneration of the cerebral white matter as evidenced by brain magnetic resonance imaging (MRI). Here, we report two sisters with EIF2B2 variants, who presented with delayed development and failure to thrive before 1 year of age, developed cataracts, and showed diffuse leukoencephalopathy. CASE PRESENTATION: The index case had a history of hepatomegaly and intermittent vomiting after upper respiratory infection at 11 months of age...
July 18, 2016: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28040752/predictors-for-quality-of-life-of-patients-with-a-portable-out-of-centre-implanted-extracorporeal-membrane-oxygenation-device
#13
Florian Rückert, Thomas Steinke, Lilit Flöther, Michael Bucher, Dietrich Metz, Stefan Frantz, Efstratios I Charitos, Hendrik Treede, Christoph Raspé
OBJECTIVES: Despite progress in the treatment of cardiopulmonary organ failure, the mortality rate for patients with acute respiratory distress syndrome (ARDS) and cardiogenic shock remains high. Extracorporeal membrane oxygenation (ECMO) is a promising treatment option, but long-term outcomes and health-related quality of life (HRQOL) are unknown. METHODS: Detailed information related to pre- and post-device data and outcomes from a consecutive sample of 71 patients treated with ECMO was analysed...
December 31, 2016: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/28040370/benefit-of-extracorporeal-membrane-oxygenation-in-major-burns-after-stun-grenade-explosion-experience-from-a-single-military-medical-center
#14
Po-Shun Hsu, Yi-Ting Tsai, Chih-Yuan Lin, Shyi-Gen Chen, Niann-Tzyy Dai, Cheng-Jung Chen, Jia-Lin Chen, Chien-Sung Tsai
INTRODUCTION: Explosion injury is very common on the battlefield and is associated with major burn and inhalation injuries and subsequent high mortality and morbidity rates. Here we report six victims who suffered from explosion injuries caused by stun grenade; all were treated with extracorporeal membrane oxygenation (ECMO) as salvage therapy. This study was aimed to evaluate the indications and efficacy of ECMO in acute and critically ill major burn patients. METHODS: This was a retrospective analysis of six patients from Tri-Service General Hospital, National Defense Medical Center in Taiwan...
December 28, 2016: Burns: Journal of the International Society for Burn Injuries
https://www.readbyqxmd.com/read/28040364/acute-intestinal-damage-following-severe-burn-correlates-with-the-development-of-multiple-organ-dysfunction-syndrome-a-prospective-cohort-study
#15
Akinori Osuka, Hirofumi Kusuki, Hiroshi Matsuura, Kentaro Shimizu, Hiroshi Ogura, Masashi Ueyama
BACKGROUND: Severely burned patients occasionally suffer intestinal ischemia leading to a fatal outcome, and the gut is considered a "motor" driving the development of multiple organ failure. However, in clinical settings, it has been difficult to assess acute intestinal damage following burn and its consequence to patient outcome. Intestinal fatty acid binding protein (I-FABP) is a known biomarker for diagnosing intestinal ischemia/damage. This study aimed to assess the extent of intestinal damage using serial I-FABP measurements following severe burn and to clarify the association between intestinal damage and the development of organ dysfunctions...
December 28, 2016: Burns: Journal of the International Society for Burn Injuries
https://www.readbyqxmd.com/read/28040257/the-2016-update-of-the-international-study-group-isgps-definition-and-grading-of-postoperative-pancreatic-fistula-11-years-after
#16
REVIEW
Claudio Bassi, Giovanni Marchegiani, Christos Dervenis, Micheal Sarr, Mohammad Abu Hilal, Mustapha Adham, Peter Allen, Roland Andersson, Horacio J Asbun, Marc G Besselink, Kevin Conlon, Marco Del Chiaro, Massimo Falconi, Laureano Fernandez-Cruz, Carlos Fernandez-Del Castillo, Abe Fingerhut, Helmut Friess, Dirk J Gouma, Thilo Hackert, Jakob Izbicki, Keith D Lillemoe, John P Neoptolemos, Attila Olah, Richard Schulick, Shailesh V Shrikhande, Tadahiro Takada, Kyoichi Takaori, William Traverso, Charles R Vollmer, Christopher L Wolfgang, Charles J Yeo, Roberto Salvia, Marcus Buchler
BACKGROUND: In 2005, the International Study Group of Pancreatic Fistula developed a definition and grading of postoperative pancreatic fistula that has been accepted universally. Eleven years later, because postoperative pancreatic fistula remains one of the most relevant and harmful complications of pancreatic operation, the International Study Group of Pancreatic Fistula classification has become the gold standard in defining postoperative pancreatic fistula in clinical practice. The aim of the present report is to verify the value of the International Study Group of Pancreatic Fistula definition and grading of postoperative pancreatic fistula and to update the International Study Group of Pancreatic Fistula classification in light of recent evidence that has emerged, as well as to address the lingering controversies about the original definition and grading of postoperative pancreatic fistula...
December 28, 2016: Surgery
https://www.readbyqxmd.com/read/28036136/maternal-source-of-variability-in-the-embryo-development-of-an-annual-killifish
#17
Matej Polačik, Carl Smith, Martin Reichard
Organisms inhabiting unpredictable environments often evolve diversified reproductive bet-hedging strategies, expressed as production of multiple offspring phenotypes, thereby avoiding complete reproductive failure. To cope with unpredictable rainfall, African annual killifish from temporary savannah pools lay drought-resistant eggs that vary widely in the duration of embryo development. We examined the sources of variability in the duration of individual embryo development, egg production and fertilization rate in Nothobranchius furzeri...
December 30, 2016: Journal of Evolutionary Biology
https://www.readbyqxmd.com/read/28031087/life-threatening-spontaneous-retroperitoneal-haemorrhage-role-of-multidetector-ct-angiography-for-the-emergency-management
#18
Zhi-Wei Wang, Hua-Dan Xue, Xiao-Guang Li, Jie Pan, Xiao-Bo Zhang, Zheng-Yu Jin
Objective To evaluate the role of multidetector computed tomography-angiography (MDCTA) for management of life-threatening spontaneous retroperitoneal haemorrhage (SRH).Methods A retrospective analysis of the medical records of all SRH patients admitted to our hospital from January 1, 2005 to November 31, 2014 was performed. SRH was defined as unrelated to invasive procedures, surgery, trauma, abdominal tumor or aortic aneurysm. Life-threatening SRH was defined as hemodynamic instability with hypotension. Sixteen cases met the inclusive criteria...
March 20, 2016: Chinese Medical Sciences Journal, Chung-kuo i Hsüeh K'o Hsüeh Tsa Chih
https://www.readbyqxmd.com/read/28030507/defining-multiple-organ-failure-after-major-trauma-a-comparison-of-the-denver-sequential-organ-failure-assessment-and-marshall-scoring-systems
#19
Lynn Hutchings, Peter Watkinson, J Duncan Young, Keith Willett
BACKGROUND: Postinjury multiple organ failure (MOF) remains a significant cause of morbidity and mortality. A large number of scoring systems have been proposed to define MOF, with no gold-standard. The purpose of this study was to compare three commonly used scores - the Denver PostInjury Multiple Organ Failure Score, the Sequential Organ Failure Assessment (SOFA) and the Marshall Multiple Organ Dysfunction Score - by descriptive analysis of the populations described by each score, and their predictive ability for mortality...
December 23, 2016: Journal of Trauma and Acute Care Surgery
https://www.readbyqxmd.com/read/28028977/the-effectiveness-of-corticosteroid-usage-in-complex-therapy-for-severe-sepsis-and-acute-respiratory-distress-syndrome-in-cases-of-severe-traumatic-brain-injury
#20
Oleksandr V Oliynyk, Bohdana O Pereviznyk, Oleh V Yemiashev, Anna Shlifirchyk
BACKGROUND: Severe traumatic brain injury (STBI) is an important issue in contemporary medicine and treatment strategies are still in need of improvement. The most dangerous complications of STBI are multiple organ failure and severe sepsis. As many as 80% of STBI patients with multiple organ failure have acute respiratory distress syndrome (ARDS). The need for better treatment strategies for STBI has led to investigations of the positive therapeutic effects of corticosteroids (CS). About 10 to 15 years ago research showed the inexpediency of CS in STBI therapy, but there were also contradictory findings showing their effectiveness...
November 2016: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
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