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Nelson syndrome

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https://www.readbyqxmd.com/read/29324621/immediate-delivery-compared-with-expectant-management-in-late-preterm-prelabor-rupture-of-membranes-an-individual-participant-data-meta-analysis
#1
Johanna Quist-Nelson, Annemijn A de Ruigh, Anna Lene Seidler, David P van der Ham, Christine Willekes, Vincenzo Berghella, Eva Pajkrt, Jillian Patterson, David Espinoza, Jonathan Morris, Ben Mol, Lisa Askie
OBJECTIVE: To compare the effects of immediate delivery an expectant management among women whose pregnancies were complicated by preterm prelabor rupture of membranes (PROM) in the late preterm period (from 34 0/7 weeks until 36 6/7 weeks of gestation). DATA SOURCES: PubMed, Scopus, ClinicalTrials.gov, EMBASE, and the Cochrane Central Register of Controlled Trials were searched from inception until December 2016. METHODS OF STUDY SELECTION: We included all randomized controlled trials with individual participant data reporting on late preterm PROM with randomization to immediate delivery or expectant management...
January 9, 2018: Obstetrics and Gynecology
https://www.readbyqxmd.com/read/29316189/acute-lumbar-paraspinal-compartment-syndrome-a-systematic-review
#2
REVIEW
William Alexander, Nelson Low, George Pratt
While still a rare entity, acute lumbar paraspinal compartment syndrome has an increasing incidence. Similar to other compartment syndromes, acute lumbar paraspinal compartment syndrome is defined by raised pressure within a closed fibro-osseous space, limiting tissue perfusion within that space. The resultant tissue ischaemia presents as acute pain, and if left untreated, it may result in permanent tissue damage. A literature search of 'paraspinal compartment syndrome' revealed 21 articles. The details from a case encountered by the authors are also included...
January 8, 2018: ANZ Journal of Surgery
https://www.readbyqxmd.com/read/29313180/a-prospective-longitudinal-study-of-pasireotide-in-nelson-s-syndrome
#3
Eleni Daniel, Miguel Debono, Sharon Caunt, Constantine Girio-Fragkoulakis, Stephen J Walters, Scott A Akker, Ashley B Grossman, Peter J Trainer, John Newell-Price
PURPOSE: Nelson's syndrome is a challenging condition that can develop following bilateral adrenalectomy for Cushing's disease, with high circulating ACTH levels, pigmentation and an invasive pituitary tumor. There is no established medical therapy. The aim of the study was to assess the effects of pasireotide on plasma ACTH and tumor volume in Nelson's syndrome. METHODS: Open labeled multicenter longitudinal trial in three steps: (1) a placebo-controlled acute response test; (2) 1 month pasireotide 300-600 μg s...
January 8, 2018: Pituitary
https://www.readbyqxmd.com/read/29311011/-screening-for-nephropathy-in-major-sickle-cell-syndromes-in-patients-monitored-at-the-national-reference-center-for-sickle-cell-disease-in-niamey-niger
#4
Moumouni Garba, Zeinabou Maiga Moussa Tondi, Hassan Diongoule, Samaila Aboubacar, Abdou Ide, Nadège Ruddy Biyao-Nelson, Illiassou Soumaila, Soumana Alido
BACKGROUND: Sickle cell anemia is the most common hereditary hemopathy in the world. It is a disease that attacks all the systems of the organism. The kidneys are among the most sensitive organs of this disease. The main objective of this study is to detect sickle cell nephropathy in patients followed at the National Reference Center for Sickle Cell Disease in Niamey. METHODS: It is a prospective study carried out over a period of one year (January to December 2016)...
January 5, 2018: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/29301447/defibrotide-sodium-for-the-treatment-of-hepatic-veno-occlusive-disease-sinusoidal-obstruction-syndrome
#5
Paul G Richardson, Brandon M Triplett, Vincent T Ho, Nelson Chao, Fiona L Dignan, Michelle Maglio, Mohamad Mohty
Hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is an unpredictable condition associated with endothelial-cell damage due to conditioning for hematopoietic stem-cell transplantation (HSCT) or chemotherapy without HSCT. Mortality in patients with VOD/SOS and multi-organ dysfunction (MOD) may be >80%. Areas covered: Defibrotide is the only approved drug for the treatment of severe hepatic VOD/SOS after HSCT in the European Union and hepatic VOD/SOS with renal or pulmonary dysfunction in the United States...
January 5, 2018: Expert Review of Clinical Pharmacology
https://www.readbyqxmd.com/read/29281843/liver-injury-in-hemolysis-elevated-liver-enzymes-and-low-platelets-syndrome-measured-by-diffusion-weighted-magnetic-resonance-imaging
#6
David B Nelson, Ambereen Khan, April Bailey, Takeshi Yokoo, Ivan Pedrosa, Donald D McIntire, F Gary Cunningham
OBJECTIVE:  The objective of this study was to evaluate acute liver injury (ALI) detected by diffusion-weighted magnetic resonance imaging (MRI) and the associated laboratory findings in women with hemolysis, elevated liver enzymes, and low platelet (HELLP) syndrome. STUDY DESIGN:  This was a retrospective, observational study of women with HELLP syndrome defined by serum aspartate aminotransferase (AST) levels ≥100 U/L and thrombocytopenia with platelets ≤100,000/µL...
December 27, 2017: American Journal of Perinatology
https://www.readbyqxmd.com/read/29278455/genetic-diagnosis-of-down-syndrome-in-an-underserved-community
#7
Andrew K Sobering, Joshua B Stevens, Janice L Smith, Beverly Nelson, Tyhiesia Donald, Sarah H Elsea
It is a matter of course that in high-income countries, infants born with features suggestive of Down syndrome (DS) are offered genetic testing for confirmation of a clinical diagnosis. Benefits of a definitive diagnosis include an end to the diagnostic odyssey, informed prognosis, opportunities for caregiver support, inclusion to social support networks, and more meaningful genetic counseling. The healthcare experience for families of children born with DS in low- and middle-income nations is in stark contrast with such a level of care...
December 26, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29246349/stippled-chondral-calcifications-of-the-patella-in-zellweger-syndrome
#8
Eileen Rife, Alston E Dunbar, Stephen L Nelson, Hans C Andersson
No abstract text is available yet for this article.
January 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29241647/safety-of-currently-licensed-hepatitis-b-surface-antigen-vaccines-in-the-united-states-vaccine-adverse-event-reporting-system-vaers-2005-2015
#9
Penina Haber, Pedro L Moro, Carmen Ng, Paige W Lewis, Beth Hibbs, Sarah F Schillie, Noele P Nelson, Rongxia Li, Brock Stewart, Maria V Cano
INTRODUCTION: Currently four recombinant hepatitis B (HepB) vaccines are in use in the United States. HepB vaccines are recommended for infants, children and adults. We assessed adverse events (AEs) following HepB vaccines reported to the Vaccine Adverse Event Reporting System (VAERS), a national spontaneous reporting system. METHODS: We searched VAERS for reports of AEs following single antigen HepB vaccine and HepB-containing vaccines (either given alone or with other vaccines), from January 2005 - December 2015...
December 11, 2017: Vaccine
https://www.readbyqxmd.com/read/29228130/acute-retroviral-syndrome-is-associated-with-high-viral-burden-cd4-depletion-and-immune-activation-in-systemic-and-tissue-compartments
#10
Trevor A Crowell, Donn J Colby, Suteeraporn Pinyakorn, James L K Fletcher, Eugène Kroon, Alexandra Schuetz, Shelly J Krebs, Bonnie M Slike, Louise Leyre, Nicolas Chomont, Linda L Jagodzinski, Irini Sereti, Netanya S Utay, Robin Dewar, Rungsun Rerknimitr, Nitiya Chomchey, Rapee Trichavaroj, Victor G Valcour, Serena Spudich, Nelson L Michael, Merlin L Robb, Nittaya Phanuphak, Jintanat Ananworanich
Background: Many individuals with acute HIV infection (AHI) experience acute retroviral syndrome (ARS), which is associated with adverse long-term clinical outcomes. We characterized clinical, virologic, and immunologic features of ARS and described the impact of early antiretroviral therapy (ART) initiation. Methods: Participants presenting for voluntary HIV testing were enrolled during AHI in Bangkok, Thailand. ARS was defined by ≥3 qualifying signs/symptoms...
December 7, 2017: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
https://www.readbyqxmd.com/read/29198741/pregnancy-as-an-early-stress-test-for-cardiovascular-and-kidney-disease-diagnosis
#11
Thaís Alquezar Facca, Gianna Mastroianni-Kirsztajn, Amélia Rodrigues Pereira Sabino, Michelle Tiveron Passos, Larissa Fátima Dos Santos, Eduardo Augusto Brosco Famá, Sonia Kiyomi Nishida, Nelson Sass
OBJECTIVES: Pregnancy is a cardiometabolic and renal stress test for women, primarily when associated with hypertension syndrome, which can have deleterious effects in the long term. We undertook this study to make a long-term evaluation on these women. STUDY DESIGN: A retrospective cohort study was conducted to investigate voluntary women who had pregnancy-induced hypertension syndrome versus normal pregnancy. MAIN OUTCOME MEASURES: We evaluated a total of 85 women, divided in case (n = 25) and control (n = 60) groups, by clinical, anthropometric and epidemiological profiles, general, metabolic and renal tests, and risk stratification for cardiovascular disease (CVD) and chronic kidney disease (CKD)...
November 23, 2017: Pregnancy Hypertension
https://www.readbyqxmd.com/read/29195081/corrigendum-to-klinefelter-s-syndrome-47-xxy-is-in-excess-among-men-with-sj%C3%A3-gren-s-syndrome-clin-immunol-168-2016-25-29
#12
Valerie M Harris, Rohan Sharma, Joshua Cavett, Biji T Kurien, Ke Liu, Kristi A Koelsch, Astrid Rasmussen, Lida Radfar, David Lewis, Donald U Stone, C Erick Kaufman, Shibo Li, Barbara Segal, Daniel J Wallace, Michael H Weisman, Swamy Venuturupalli, Jennifer A Kelly, Marta E Alarcon-Riquelme, Bernardo Pons-Estel, Roland Jonsson, Xianglan Lu, Jacques-Eric Gottenberg, Juan-Manuel Anaya, Deborah S Cunninghame-Graham, Andrew J W Huang, Michael T Brennan, Pamela Hughes, Ilias Alevizos, Corinne Miceli-Richard, Edward C Keystone, Vivian P Bykerk, Gideon Hirschfield, Gang Xie, Katherine A Siminovitch, Wan-Fai Ng, Gunnel Nordmark, Sara Magnusson Bucher, Per Eriksson, Roald Omdal, Nelson L Rhodus, Maureen Rischmueller, Michael Rohrer, Marie Wahren-Herlenius, TorstenWitte, Xavier Mariette, Christopher J Lessard, John B Harley, Kathy L Sivils, R Hal Scofield
No abstract text is available yet for this article.
November 28, 2017: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://www.readbyqxmd.com/read/29194024/adapting-the-mullen-scales-of-early-learning-for-a-standardized-measure-of-development-in-children-with-rett-syndrome
#13
Tessa Clarkson, Jocelyn LeBlanc, Geneva DeGregorio, Vanessa Vogel-Farley, Katherine Barnes, Walter E Kaufmann, Charles A Nelson
Rett Syndrome (RTT) is characterized by severe impairment in fine motor (FM) and expressive language (EL) function, making accurate evaluations of development difficult with standardized assessm ents. In this study, the administration and scoring of the Mullen Scales of Early Learning (MSEL) were adapted to eliminate the confounding effects of FM and EL impairments in assessing development. Forty-seven girls with RTT were assessed with the Adapted-MSEL (MSEL-A), a subset (n = 30) was also assessed using the Vineland Adaptive Behavior Scales-Second Edition (Vineland-II) and a further subset (n = 17) was assessed using an eye-tracking version of the MSEL (MSEL-ET)...
December 2017: Intellectual and Developmental Disabilities
https://www.readbyqxmd.com/read/29170704/a-pre-exercise-dose-of-melatonin-can-alter-substrate-use-during-exercise
#14
Cardyl P Trionfante, Greggory R Davis, Tyler M Farney, Ryan W Miskowiec, Arnold G Nelson
Notwithstanding the lack of exercise research, several reviews have championed the use of melatonin to combat metabolic syndrome. Therefore, this study compared substrate utilization during a 30-minute (min) graded exercise protocol following the ingestion of either 6 mg melatonin (M) or a placebo (P). Participants (12 women, 12 men) performed stages 1-5 of the Naughton graded exercise protocol (6 min per stage). The protocol was repeated 4 times (2x M, 2x P) at the same time of day with one week separating each session...
2017: International Journal of Exercise Science
https://www.readbyqxmd.com/read/29148407/minor-salivary-gland-fibrosis-in-sj%C3%A3-gren-s-syndrome-is-elevated-associated-with-focus-score-and-not-solely-a-consequence-of-aging
#15
Kerry M Leehan, Nathan P Pezant, Astrid Rasmussen, Kiely Grundahl, Jacen S Moore, Lida Radfar, David M Lewis, Donald U Stone, Christopher J Lessard, Nelson L Rhodus, Barbara M Segal, R Hal Scofield, Kathy L Sivils, Courtney Montgomery, A Darise Farris
OBJECTIVES: Evaluate the presence of minor salivary gland (SG) fibrosis in primary Sjögren's syndrome (pSS) as a function of disease pathology or a consequence of ageing. METHODS: Subjects with sicca symptoms attending a Sjögren's research clinic were classified by American European Consensus Group (AECG) criteria as either pSS or non-SS (nSS). Discovery (n=34 pSS, n=28 nSS) and replication (n=35 pSS, n=31 nSS) datasets were evaluated. Minor SG cross-sections from haematoxylin and eosin stained slides were imaged, digitally reconstructed and analysed for percent area fibrosis...
October 23, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29142983/efficacy-and-safety-of-sparsentan-compared-with-irbesartan-in-patients-with-primary-focal-segmental-glomerulosclerosis-randomized-controlled-trial-design-duet
#16
Radko Komers, Debbie S Gipson, Peter Nelson, Sharon Adler, Tarak Srivastava, Vimal K Derebail, Kevin E Meyers, Pablo Pergola, Meghan E MacNally, Jennifer L Hunt, Alvin Shih, Howard Trachtman
Introduction: Primary focal segmental glomerulosclerosis (FSGS) is a leading cause of nephrotic syndrome and end-stage renal disease. There are no US Food and Drug Administration-approved therapies for FSGS, and treatment often fails to reduce proteinuria. Endothelin is an important factor in the pathophysiology of podocyte disorders, including FSGS. Sparsentan is a first-in-class, orally active, dual-acting angiotensin receptor blocker (ARB) and highly selective endothelin Type A receptor antagonist...
July 2017: KI Reports
https://www.readbyqxmd.com/read/29140951/platelet-aggregation-after-blunt-trauma-is-associated-with-the-acute-respiratory-distress-syndrome-and-altered-by-cigarette-smoke-exposure
#17
Farzad Moazed, Carolyn Hendrickson, Mary Nelson, Amanda Conroy, Mitchell J Cohen, Carolyn S Calfee
BACKGROUND: The risk of the acute respiratory distress syndrome (ARDS) is increased in passive and active smokers after blunt trauma. However, the mechanisms responsible, including the role of platelet aggregation, for this association are unknown. METHODS: We analyzed 215 patients with severe blunt trauma from a prospective observational cohort at a Level 1 trauma center between 2010 - 2015. Subjects underwent impedance-based platelet aggregometry in response to platelet agonists arachidonic acid (ASPI), adenosine diphosphate (ADP), collagen (COL) and thrombin receptor activating peptide-6 (TRAP)...
November 14, 2017: Journal of Trauma and Acute Care Surgery
https://www.readbyqxmd.com/read/29126394/prospective-study-of-autism-phenomenology-and-the-behavioural-phenotype-of-phelan-mcdermid-syndrome-comparison-to-fragile-x-syndrome-down-syndrome-and-idiopathic-autism-spectrum-disorder
#18
Caroline Richards, Laurie Powis, Jo Moss, Christopher Stinton, Lisa Nelson, Christopher Oliver
BACKGROUND: The limited behavioural phenotype literature on Phelan-McDermid syndrome (PMS) indicates atypically high levels of activity, impulsivity and autism spectrum disorder (ASD) behaviours. Divergent profiles of ASD in PMS are also reported, with some studies demonstrating similarities to idiopathic ASD and others indicating an uneven profile of social and communication impairments and repetitive behaviours. An evaluation of the behavioural phenotype of PMS and the prevalence and phenomenology of ASD is warranted, particularly given the causal involvement of the SHANK3 gene in the aetiology of PMS...
November 10, 2017: Journal of Neurodevelopmental Disorders
https://www.readbyqxmd.com/read/29118981/zika-virus-disease-microcephaly-and-guillain-barr%C3%A3-syndrome-in-colombia-epidemiological-situation-during-21%C3%A2-months-of-the-zika-virus-outbreak-2015-2017
#19
Nelson Méndez, Misael Oviedo-Pastrana, Salim Mattar, Isaac Caicedo-Castro, German Arrieta
Background: The Zika virus disease (ZVD) has had a huge impact on public health in Colombia for the numbers of people affected and the presentation of Guillain-Barre syndrome (GBS) and microcephaly cases associated to ZVD. Methods: A retrospective descriptive study was carried out, we analyze the epidemiological situation of ZVD and its association with microcephaly and GBS during a 21-month period, from October 2015 to June 2017. The variables studied were: (i) ZVD cases, (ii) ZVD cases in pregnant women, (iii) laboratory-confirmed ZVD in pregnant women, (iv) ZVD cases associated with microcephaly, (v) laboratory-confirmed ZVD associated with microcephaly, and (vi) ZVD associated to GBS cases...
2017: Archives of Public Health, Archives Belges de Santé Publique
https://www.readbyqxmd.com/read/29107342/sweet-syndrome-in-patients-with-and-without-malignancy-a-retrospective-analysis-of-83-patients-from-a-tertiary-academic-referral-center
#20
Caroline A Nelson, Megan H Noe, Christine M McMahon, Asha Gowda, Benedict Wu, Hovik J Ashchyan, Alexander E Perl, William D James, Robert G Micheletti, Misha Rosenbach
BACKGROUND: Sweet syndrome is a neutrophilic dermatosis that may be categorized into classic, malignancy-associated, and drug-induced subtypes. Few studies have systematically analyzed this rare disorder. OBJECTIVE: To describe the clinicopathologic characteristics and treatment of Sweet syndrome and identify characteristics associated with concurrent malignancy. METHODS: We retrospectively reviewed patients with Sweet syndrome at the University of Pennsylvania from 2005 to 2015...
February 2018: Journal of the American Academy of Dermatology
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