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Nelson syndrome

Louis Sass, Juan P Borda, Luis Madeira, Elizabeth Pienkos, Barnaby Nelson
The self-disorder model offers a unifying way of conceptualizing schizophrenia's highly diverse symptoms (positive, negative, disorganized), of capturing their distinctive bizarreness, and of conceiving their longitudinal development. These symptoms are viewed as differing manifestations of an underlying disorder of ipseity or core-self: hyper-reflexivity/diminished-self-presence with accompanying disturbances of "grip" or "hold" on reality. Recent revision to this phenomenological theory, in particular distinguishing primary-vs-secondary factors, offers a bio-pheno-social model that is consistent with recent empirical findings and offers several advantages: (1) It helps account for the temporal variations of the symptoms or syndrome, including longitudinal progression, but also the shorter-term, situationally reactive, and sometimes defensive or quasi-intentional variability of symptom-expression that can occur in schizophrenia (consistent with understanding some aspects of ipseity-disturbance as dynamic and mutable, involving shifting attitudes or experiential orientations)...
February 5, 2018: Schizophrenia Bulletin
Luiz Felipe Lessa Ortiz, Julio Elito Júnior, Edward Araujo Júnior, Alberto Borges Peixoto, Nelson Sass, Antonio Fernandes Moron
We report the case of a 20-year-old patient, primigravida, with twin monochorionic pregnancy, with a weight gain of 6 kg in one week and increased blood pressure. During the ultrasound diagnostic investigation, placental edema and hydrops were identified in both fetuses, which, in association with maternal anasarca and pressure control, constitute the triad for mirror syndrome, also known as triple edema. In addition to being hydropic, one of the twins was an acardiac fetus, which is a rare combination of events...
2018: Case Reports in Obstetrics and Gynecology
Sarah Nelson, Andrea McDuffie, Amy Banasik, Robyn Tempero Feigles, Angela John Thurman, Leonard Abbeduto
This study examined the impact of a distance-delivered parent-implemented narrative language intervention on the use of inferential language during shared storytelling by school-aged boys with fragile X syndrome, an inherited neurodevelopmental disorder. Nineteen school-aged boys with FXS and their biological mothers participated. Dyads were randomly assigned to an intervention or a treatment-as-usual comparison group. Transcripts from all pre- and post-intervention sessions were coded for child use of prompted and spontaneous inferential language coded into various categories...
March 2018: Journal of Communication Disorders
Hideyuki Takeuchi, Derek Wong, Michael Schneider, Hudson H Freeze, Megumi Takeuchi, Steven J Berardinelli, Atsuko Ito, Hane Lee, Stanley F Nelson, Robert S Haltiwanger
Protein O-fucosyltransferase-1 (POFUT1) adds O-fucose monosaccharides to epidermal growth factor-like (EGF) repeats found on approximately 100 mammalian proteins, including Notch receptors. Haploinsufficiency of POFUT1 has been linked to adult-onset Dowling Degos Disease (DDD) with hyperpigmentation defects. Homozygous deletion of mouse Pofut1 results in embryonic lethality with severe Notch-like phenotypes including defects in somitogenesis, cardiogenesis, vasculogenesis, and neurogenesis, but the extent to which POFUT1 is required for normal human development is not yet understood...
February 14, 2018: Glycobiology
Hovik J Ashchyan, Daniel C Butler, Caroline A Nelson, Megan H Noe, William G Tsiaras, Stephen J Lockwood, William D James, Robert G Micheletti, Misha Rosenbach, Arash Mostaghimi
Importance: Pyoderma gangrenosum is an inflammatory neutrophilic dermatosis. Current knowledge of this rare disease is limited owing to a lack of validated diagnostic criteria and large population studies. Objective: To evaluate the association of age with the clinical presentation and comorbidities of pyoderma gangrenosum. Design, Setting, and Participants: This was a multicenter retrospective cohort study performed at tertiary academic referral centers in urban settings...
February 14, 2018: JAMA Dermatology
Nelson Lee, Kevin Wade Lee, Matthew Michael D'Ambrosio, Joseph Vaughan Banta, Apostolos Voudouris, Antonios Tsompanidis
Takotsubo syndrome is classically characterized by apical ballooning and left ventricle akinesis associated with an underlying catecholamine surge. In patients with suspected Takotsubo syndrome, clinicians should be vigilant for acute coronary syndrome and arrhythmias. Ventricular standstill with underlying Takotsubo syndrome should be managed with a dual-chambered pacemaker to improve patient outcome.
February 2018: Clinical Case Reports
Flávia Mariel Steckling, Juliano Boufleur Farinha, Felipe da Cunha Figueiredo, Daniela Lopes Dos Santos, Guilherme Bresciani, Nélson Alexandre Kretzmann, Sílvio Terra Stefanello, Aline Alves Courtes, Maristela de Oliveira Beck, Manuela Sangoi Cardoso, Marta Maria Medeiros Frescura Duarte, Rafael Noal Moresco, Félix Alexandre Antunes Soares
This study investigate the effects of high-intensity interval training (HIIT) on systemic levels of inflammatory and hormonal markers in postmenopausal women with metabolic syndrome (MS). Fifteen postmenopausal women with MS completed the training on treadmills. Functional, body composition parameters, maximal oxygen uptake (VO 2 max), and lipid profile were assessed before and after HIIT. Serum or plasma levels of cytokines and hormonal markers were measured along the intervention. The analysis of messenger RNA (mRNA) expression of these cytokines was performed in peripheral blood mononuclear cells (PBMC)...
February 12, 2018: Archives of Physiology and Biochemistry
Enrique Diaz-Convalia, Miguel Angel Arrabal-Polo, Maria Del Carmen Cano-Garcia, Alejandro Dominguez-Amillo, Nelson Canales-Casco, Miguel Arrabal-Martin
PURPOSE: To determine whether androgen blockade produces metabolic changes in urine and increases the risk of calculi after 1 year of treatment. MATERIALS AND METHODS: The study included 38 patients, from the period April 2015 to June 2016, diagnosed with locally advanced prostate cancer or lymph node metastasis, and with an indication of androgen blockade. Androgen blockade was started with luteinising hormone-releasing hormone (LHRH) analogues, and a blood specimen, a fasting urine and 24-h urine were collected at the time of inclusion, and then at 1 year of follow-up...
January 27, 2018: International Urology and Nephrology
K Li, E Ochoa, T Lipsey, T Nelson
Background: It has been documented that cardiovascular disease is the leading cause of mortality in on-duty firefighters and little is known about the association between cardiorespiratory fitness and 10-year risk of atherosclerotic cardiovascular disease (ASCVD). Aims: To estimate the probability of 10-year ASCVD risk and its association with per cent body fat (%BF), metabolic syndrome (MetS) and physical fitness in Colorado firefighters. Methods: Ten-year ASCVD risk was calculated using the newly developed pooled cohort equations in firefighters...
January 17, 2018: Occupational Medicine
Alec J Hirsch, Victoria H J Roberts, Peta L Grigsby, Nicole Haese, Matthias C Schabel, Xiaojie Wang, Jamie O Lo, Zheng Liu, Christopher D Kroenke, Jessica L Smith, Meredith Kelleher, Rebecca Broeckel, Craig N Kreklywich, Christopher J Parkins, Michael Denton, Patricia Smith, Victor DeFilippis, William Messer, Jay A Nelson, Jon D Hennebold, Marjorie Grafe, Lois Colgin, Anne Lewis, Rebecca Ducore, Tonya Swanson, Alfred W Legasse, Michael K Axthelm, Rhonda MacAllister, Ashlee V Moses, Terry K Morgan, Antonio E Frias, Daniel N Streblow
Zika virus (ZIKV) infection during pregnancy leads to an increased risk of fetal growth restriction and fetal central nervous system malformations, which are outcomes broadly referred to as the Congenital Zika Syndrome (CZS). Here we infect pregnant rhesus macaques and investigate the impact of persistent ZIKV infection on uteroplacental pathology, blood flow, and fetal growth and development. Despite seemingly normal fetal growth and persistent fetal-placenta-maternal infection, advanced non-invasive in vivo imaging studies reveal dramatic effects on placental oxygen reserve accompanied by significantly decreased oxygen permeability of the placental villi...
January 17, 2018: Nature Communications
Ana Beatriz D Grisolia, Christine C Nelson
Distichiasis is a challenging condition that may require multiple surgical interventions. Besides ophthalmologic concerns in children, distichiasis may be part of the lymphedema-distichiasis syndrome, which presents with lymphedema of variable time of onset. Other significant systemic disorders such as coarctation of the aorta and varicose veins have been reported in association with this syndrome and must be reviewed for proper patient care. The authors report the case of a 22-year-old male patient who had been treated for distichiasis and followed for 16 years...
January 16, 2018: Ophthalmic Plastic and Reconstructive Surgery
Virginie Bussières, Shreyas Roy, Johnny Deladoey, Élisabeth Rousseau, Dickens St-Vil, Nelson Piché
AIM OF THE STUDY: The aim of the study was to evaluate the outcomes of prophylactic thyroidectomies performed in an academic setting in the context of multiple endocrine neoplasia type 2 (MEN2) syndrome. METHODS: A chart review of patients <18years old who underwent prophylactic thyroidectomy for a MEN2 syndrome at a children's hospital between 2006 and 2015 was performed. MAIN RESULTS: The study included 21 patients (57% female) with a mean age of 6...
November 13, 2017: Journal of Pediatric Surgery
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,, 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...
February 2018: Obstetrics and Gynecology
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
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
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
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
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
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
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
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