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https://www.readbyqxmd.com/read/28087540/international-evidence-based-consensus-diagnostic-criteria-for-hhv-8-negative-idiopathic-multicentric-castleman-disease
#1
David C Fajgenbaum, Thomas S Uldrick, Adam Bagg, Dale Frank, David Wu, Gordan Srkalovic, David Simpson, Amy Y Liu, David Menke, Shanmuganathan Chandrakasan, Mary Jo Lechowicz, Raymond S M Wong, Sheila Pierson, Michele Paessler, Jean-François Rossi, Makoto Ide, Jason Ruth, Michael Croglio, Alexander Suarez, Vera Krymskaya, Amy Chadburn, Gisele Colleoni, Sunita Nasta, Raj Jayanthan, Christopher S Nabel, Corey Casper, Angela Dispenzieri, Alexander Fosså, Dermot Kelleher, Razelle Kurzrock, Peter Voorhees, Ahmet Dogan, Kazuyuki Yoshizaki, Frits van Rhee, Eric Oksenhendler, Elaine S Jaffe, Kojo S J Elenitoba-Johnson, Megan S Lim
Human Herpesvirus-8(HHV-8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare and life-threatening disorder involving systemic inflammatory symptoms, polyclonal lymphoproliferation, cytopenias, and multiple organ system dysfunction due to a cytokine storm often including interleukin-6. iMCD accounts for one-third to one-half of all cases of MCD and can occur in individuals of any age. Accurate diagnosis is challenging, as no standard diagnostic criteria or diagnostic biomarkers currently exist, and there is significant overlap with malignant, autoimmune, and infectious disorders...
January 13, 2017: Blood
https://www.readbyqxmd.com/read/28087259/clinical-manifestations-outcomes-and-etiologies-of-perinatal-stroke-in-taiwan-comparisons-between-ischemic-and-hemorrhagic-stroke-based-on-10-year-experience-in-a-single-institute
#2
Chien-Chung Lee, Jainn-Jim Lin, Kuang-Lin Lin, Wai-Ho Lim, Kai-Hsiang Hsu, Jen-Fu Hsu, Ren-Huei Fu, Ming-Chou Chiang, Shih-Ming Chu, Reyin Lien
BACKGROUND: Perinatal stroke is a common cause of established neurological sequelae. Although several risk factors have been identified, many questions regarding causes and clinical outcomes remain unanswered. This study investigated the clinical manifestations and outcomes of perinatal stroke and identified its etiologies in Taiwan. METHODS: We searched the reports of head magnetic resonance imaging and computed tomography performed between January 2003 and December 2012...
November 19, 2016: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/28078984/rasburicase-induced-methemoglobinemia-in-a-patient-with-glucose-6-phosphate-dehydrogenase-deficiency
#3
Maliha Khan, Shilpa Paul, Saad Farooq, Thein Hlaing Oo, Priya Ramshesh, Nitin Jain
BACKGROUND: Rasburicase is commonly used in patients with hematologic malignancies for tumor lysis syndrome prophylaxis and management. Methemoglobinemia is a serious rare adverse effect of rasburicase, more common in patients with G6PD deficiency. Prompt diagnosis and appropriate management of this condition can make the difference between successful recovery and significant morbidity. Here we discuss the link of rasburicase with methemoglobinemia and the pathophysiology behind increased incidence of this side effect in G6PD deficient patients...
January 11, 2017: Current Drug Safety
https://www.readbyqxmd.com/read/28073139/-influence-of-comorbidities-on-revision-rate-within-the-first-year-after-primary-total-hip-arthroplasty
#4
Roman Nesslage, Kerstin Radtke, Lisa Hohloch, Thilo Flörkemeier, Henning Windhagen, Gabriela von Lewinski
: Background Total hip arthroplasty (THA) is very frequently performed. Despite low complication rates, revisions play an important clinical and economical role. The aim of this study was to identify comorbid diseases of patients undergoing primary THA and their potential influence on the survival of hip replacements. Patients/Material and Methods A total of 867 patients were included in this retrospective study. All revisions were reviewed that took place at our hospital within one year of primary implantation of THA...
January 10, 2017: Zeitschrift Für Orthopädie und Unfallchirurgie
https://www.readbyqxmd.com/read/28072956/-clinical-and-immunological-analysis-of-the-patient-with-autoimmunity-due-to-germline-stat3-gain-of-function-mutation
#5
Y Ding, Y Zhang, Y P Wang, H Y Zhao, X M Chen, X H Xue, X M Bai, Y F An, Z Y Zhang, X M Tang, X D Zhao
Objective: To investigate the clinical and immunological laboratory features and gene mutation in a female patient who carried a germline gain-of-function mutation in STAT3. Method: A patient with lymphadenopathy and pancytopenia, visited the Department of Rheumatology and Immunology of Children's Hospital of Chongqing Medical University in May 2016. The clinical and laboratory characteristics, results of immunophenotyping and exome sequencing were analyzed retrospectively and related literature was reviewed...
January 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28072603/dyserythropoiesis-of-myelodysplastic-syndromes
#6
Carine Lefèvre, Sabrina Bondu, Salomé Le Goff, Olivier Kosmider, Michaela Fontenay
PURPOSE OF REVIEW: Myelodysplastic syndromes (MDS) are heterogeneous diseases of the hematopoietic stem cell in the elderly. Anemia is the main symptom that mostly correlates with dysplastic erythropoiesis in the bone marrow. We will review the recent advances in understanding the diverse mechanisms of dyserythropoiesis. RECENT FINDINGS: Dyserythropoiesis defined as 10% dysplastic erythroid cells in the bone marrow is found in more than 80% of early MDS. Immature erythroblasts accumulate at the expense of mature erythroblasts due to differentiation arrest and apoptosis...
January 7, 2017: Current Opinion in Hematology
https://www.readbyqxmd.com/read/28070879/a-review-of-pharmacogenetics-of-antimalarials-and-associated-clinical-implications
#7
REVIEW
Hazem Elewa, Kyle John Wilby
Genetic variability in drug-metabolizing enzymes and drug transporters is known to influence the pharmacokinetics of many drugs. Antimalarial drugs are a class of agents known to utilize metabolic and elimination pathways prone to genetic variation. This paper aims to review the genetic variants affecting antimalarial medications and discuss their clinical implications. Data were identified for the genes coding for the cytochrome P450 (CYP) enzymes: CYP2C8, CYP2C19, CYP2A6, CYP2D6, CYP2B6, and the P-glycoprotein drug transporter...
January 9, 2017: European Journal of Drug Metabolism and Pharmacokinetics
https://www.readbyqxmd.com/read/28067165/gene-therapy-in-fanconi-anemia-a-matter-of-time-safety-and-gene-transfer-tool-efficiency
#8
Verhoeyen Els, Francisco José Román Rodríguez, François-Loïc Cosset, Camille Lévy, Paula Rio
Fanconi anemia (FA) is a rare genetic syndrome characterized by progressive marrow failure. Gene therapy by infusion of FA-corrected autologous hematopoietic stem cells (HSCs) may offer a potential cure since it is a monogenetic disease with mutations in the FANC genes, coding for DNA repair enzymes (See review[1]). However, the collection of hCD34 +-cells in FA patients implies particular challenges because of the reduced numbers of progenitor cells present in their bone marrow (BM)[2] or mobilized peripheral blood[3-5]...
January 9, 2017: Current Gene Therapy
https://www.readbyqxmd.com/read/28062362/induced-pluripotent-stem-cell-technology-a-window-for-studying-the-pathogenesis-of-acquired-aplastic-anemia-and-possible-applications
#9
REVIEW
Mahmoud Elbadry, J Luis Espinoza, Shinji Nakao
Recent progress in human induced pluripotent stem cells (iPSCs) has opened the door to better understand the biology of human diseases, especially in rare disorders, such as acquired aplastic anemia (AA), in which the target hematopoietic tissues are depleted. The advent of somatic cell reprogramming has presented new routes for generating hematopoietic stem cells (HSCs) from patient-derived iPSCs and their differentiation into hematopoietic lineages. The purpose of this review is to discuss the recent advances in iPSC research technology and its potential applications in disease modeling for understanding the pathogenesis of bone marrow failure syndrome (BMFS) and the potential clinical utility of iPSC-derived cells...
January 3, 2017: Experimental Hematology
https://www.readbyqxmd.com/read/28061988/treatment-of-high-risk-smoldering-myeloma
#10
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/28059982/morbidity-of-adult-spinal-deformity-surgery-in-elderly-has-declined-over-time
#11
Peter G Passias, Gregory W Poorman, Cyrus M Jalai, Brian Neuman, Rafael de la Garza-Ramos, Emily Miller, Amit Jain, Daniel Sciubba, Shearwood McClelland, Louis Day, Subbu Ramachadran, Shaleen Vira, Bassel Diebo, Evan Isaacs, Olivia Bono, Shay Bess, Michael Gerling, Virginie Lafage
STUDY DESIGN: A retrospective review of a prospectively collected database, the Nationwide Inpatient Sample (NIS), years 2003 to 2012. OBJECTIVES: The aim of this study was to examine trends in the management of scoliosis in elderly (age >75 yrs) patients from 2003 to 2012. SUMMARY OF BACKGROUND DATA: Scoliosis incidence rises with increasing age, and age has been shown to be an independent risk factor for surgical complications in scoliosis surgery...
January 4, 2017: Spine
https://www.readbyqxmd.com/read/28058081/fungal-esophagitis-presenting-with-esophagitis-dissecans-superficialis-cause-or-concurrence-a-diagnostic-conundrum
#12
Rashmee Patil, Tagore Sunkara, Mel A Ona, Vinaya Gaduputi, Madhavi Reddy
Esophagitis dissecans superficialis (EDS) is a rare desquamative disorder of the esophagus. It is characterized by sheets of sloughed squamous tissue with normal underlying mucosa. We present a case of a 68-year-old man with severe anemia and a positive fecal occult blood test who was found to have features suggestive of EDS during endoscopy. However, histological features were suggestive of both EDS and fungal esophagitis. To our knowledge, this is the first reported case in literature which discusses the clinical scenario of fungal infection being a possible etiology rather than a commonly accepted differential diagnosis of EDS...
December 2016: Gastroenterology Research
https://www.readbyqxmd.com/read/28054252/a-risk-factor-analysis-for-in-hospital-mortality-after-surgery-for-infective-endocarditis-and-a-proposal-of-a-new-predictive-scoring-system
#13
Giuseppe Gatti, Bernardo Benussi, Florida Gripshi, Alessio Della Mattia, Alberto Proclemer, Antonio Cannatà, Lorella Dreas, Roberto Luzzati, Gianfranco Sinagra, Aniello Pappalardo
PURPOSE: Risk stratification is of utmost importance for patients with infective endocarditis (IE) who need surgery. However, for these critically ill patients, aspecific scoring systems are used to predict the risk of death after surgery. The aim of this study was both to analyze the risk factors for in-hospital death, which complicates surgery for IE and to create a mortality risk score based on the results of this analysis. METHODS: Outcomes of 138 consecutive patients (mean age 60...
January 4, 2017: Infection
https://www.readbyqxmd.com/read/28042234/diagnosis-and-treatment-of-anemia-in-patients-with-inflammatory-bowel-disease
#14
REVIEW
Victoria Mücke, Marcus M Mücke, Tim Raine, Dominik Bettenworth
Anemia represents one of the most frequent complications in inflammatory bowel disease (IBD) and severely impairs the quality of life of affected patients. The etiology of anemia in IBD patients can be multifactorial, often involving a combination of iron deficiency (ID) and anemia of chronic disease (ACD). Although current guidelines recommend screening for and treatment of anemia in IBD patients, current observational data suggest that it still remains underdiagnosed and undertreated. Besides basic laboratory parameters (e...
2017: Annals of Gastroenterology: Quarterly Publication of the Hellenic Society of Gastroenterology
https://www.readbyqxmd.com/read/28041993/efficient-breathing-at-neonatal-ages-a-sex-and-epo-dependent-issue
#15
REVIEW
Pablo Iturri, Aida Bairam, Jorge Soliz
During postnatal life, the respiratory control system undergoes intense development and is highly responsive to stimuli emerging from the environment. In fact, interruption of breathing prevents gas exchange and results in systemic hypoxia that, if prolonged, can lead to cardio-respiratory failure or sudden infant death. Moreover, in newborns and infants, respiratory disorders related to neural control dysfunction show significant sexual dimorphism with a higher prevalence in males. To this day, the therapeutic tools available to alleviate these respiratory disorders remain limited...
December 29, 2016: Respiratory Physiology & Neurobiology
https://www.readbyqxmd.com/read/28040287/race-and-contrast-induced-nephropathy-in-patients-undergoing-coronary-angiography-and-cardiac-catheterization
#16
REVIEW
Raveen Chawla, Jeremy Turlington, Pradeep Arora, Ion S Jovin
Contrast-induced nephropathy (CIN) is an acute worsening of renal function after receiving intravascular contrast during a procedure. Some of the predisposing factors include underlying diabetes, chronic kidney disease, congestive heart failure, periprocedural hypotension, anemia, contrast volume, and osmolality of contrast; however, it remains unclear if risk varies for CIN with race and ethnicity. There is evidence in the literature showing the link between race/ethnicity and the discrepancies in the utilization of preventive care services and the resources related to cardiovascular and renal health...
December 23, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/28034892/how-i-treat-anemia-in-pregnancy-iron-cobalamin-and-folate
#17
Maureen M Achebe, Anat Gafter-Gvili
Anemia of pregnancy is considered a global health problem, affecting almost 50% of pregnant women. Anemia is an important risk factor for both fetal and maternal morbidity. The purpose of this article is to discuss diagnosis and management of the most frequent causes of anemia in pregnancy: iron, cobalamin and folate deficiencies. This paper considers three clinical cases. Iron deficiency is the most common cause. The laboratory tests that define iron deficiency, the recognition of developmental delays and cognitive abnormalities in iron-deficient neonates and the literature addressing the efficacy and safety of intravenous iron in pregnancy are reviewed...
December 29, 2016: Blood
https://www.readbyqxmd.com/read/28031963/folic-acid-supplementation-what-is-new-fetal-obstetric-long-term-benefits-and-risks
#18
REVIEW
Hind N Moussa, Susan Hosseini Nasab, Ziad A Haidar, Sean C Blackwell, Baha M Sibai
The association between folic acid supplementation, prior to conception and/or during pregnancy and pregnancy outcomes, has been the subject of numerous studies. The worldwide recommendation of folic acid is at least 0.4 mg daily for all women of reproductive age, and 4-5 mg in high-risk women. In addition, evidence shows that folic acid supplementation could modulate other adverse pregnancy outcomes, specifically, in pregnancies complicated by seizure disorders, preeclampsia, anemia, fetal growth restriction and autism...
June 2016: Future Science OA
https://www.readbyqxmd.com/read/28028427/effects-of-iron-on-vitamin-d-metabolism-a-systematic-review
#19
REVIEW
Fatemeh Azizi-Soleiman, Mohammadreza Vafa, Behnaz Abiri, Morteza Safavi
Vitamin D is a prohormone nutrient, which is involved in skeletal and extra-skeletal functions. Iron is another essential nutrient that is necessary for the production of red blood cells and oxygen transport. This element plays important roles in enzymatic systems including those required for Vitamin D activation. To the best of our knowledge, there is no exclusive review on the relationship between iron deficiency anemia (IDA), as the most prevalent type of anemia, and Vitamin D deficiency and the effect of recovery from iron deficiency on Vitamin D status...
2016: International Journal of Preventive Medicine
https://www.readbyqxmd.com/read/28028297/histopathological-features-of-coeliac-disease-in-a-sample-of-sudanese-patients
#20
M A N Mokhtar, S O Mekki, H M Y Mudawi, S H Sulaiman, M A Tahir, M A Tigani, I A Omer, B M Yousif, I A Fragalla, Z Mohammed, M Dafaalla
INTRODUCTION: Coeliac disease can occur at any age but is more common in children. Its diagnosis requires correlation between clinical presentations, serological results, endoscopic findings and histopathological classification using the modified Marsh grading system. This study of coeliac disease with biopsies received in the department of histopathology at Soba University Hospital, and Fedail Hospital aimed to gain insight into the demographic profile, clinical presentations and histopathological classification of patients with coeliac disease...
December 2016: Malaysian Journal of Pathology
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