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https://www.readbyqxmd.com/read/29143319/the-full-spectrum-of-castleman-disease-273-patients-studied-over-20%C3%A2-years
#1
Eric Oksenhendler, David Boutboul, David Fajgenbaum, Adrien Mirouse, Claire Fieschi, Marion Malphettes, Laetitia Vercellino, Véronique Meignin, Laurence Gérard, Lionel Galicier
The spectrum of Castleman disease (CD) has considerably extended since its first description in 1956. Recently, an international collaborative working group has reached consensus on the diagnostic criteria and classification of CD. We herein report 273 patients with lymph node histopathology consistent with CD and investigate the newly established diagnostic criteria. Twenty of these patients with Castleman-like histopathology were removed from analyses, because they were diagnosed with an exclusionary disorder (18 with haematological malignancy)...
November 16, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/29124835/clinical-characteristics-and-outcomes-of-castleman-disease-a-multicenter-study-of-185-chinese-patients
#2
Xuanye Zhang, Huilan Rao, Xiaolu Xu, Zhihua Li, Bing Liao, Hongmei Wu, Mei Li, Xiuzhen Tong, Juan Li, Qingqing Cai
Castleman disease (CD) is a rare lymphoproliferative disorder. To assess the clinical features, outcomes, and prognostic factors of this disease, we retrospectively analyzed 185 HIV-negative CD patients from four medical centers in southern China. The median age was 37 years. 121 patients (65.4%) were classified as unicentric CD (UCD) and 64 patients (34.6%) were classified as multicentric CD (MCD). The histology subtype was hyaline-vascular for 132 patients (71.4%), plasma cell for 50 patients (27%) and mixed type for 3 patients (1...
November 9, 2017: Cancer Science
https://www.readbyqxmd.com/read/29103724/mild-and-severe-udder-cleft-dermatitis-prevalence-and-risk-factors-in-swedish-dairy-herds
#3
L Ekman, A-K Nyman, H Landin, U Magnusson, K Persson Waller
Udder cleft dermatitis (UCD) is an inflammatory skin condition affecting the anterior parts of the udder of dairy cows. The lesions may present as mild or severe skin lesions and have been associated with mastitis and digital dermatitis. The full etiology and pathogenesis are not understood and no large-scale studies have investigated prevalence and risk factors. Therefore, the main objectives of the study were to investigate the prevalence of mild and severe UCD in Swedish dairy herds and to identify risk factors associated with such lesions...
November 2, 2017: Journal of Dairy Science
https://www.readbyqxmd.com/read/29044911/split-graft-liver-transplantation-from-an-adult-donor-with-an-unrecognized-ucd-to-a-pediatric-and-adult-recipient
#4
C H Lee, C Ellaway, A Shun, G Thomas, P Nair, J O'Neill, N Shakel, M O Stormon
We report the outcomes of an adult and pediatric split liver transplant from an adult male donor who died due to an unrecognized UCD, OTC deficiency. Recognizing inborn errors of metabolism can be challenging, especially in adult centers where such disorders are rarely encountered. Shortage of donors for liver transplantation has led to procedures to maximize donor utilization, such as split and live donor grafts. The cause of death should be ascertained before accepting a cadaveric donor organ.
October 16, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/29027067/human-heterologous-liver-cells-transiently-improve-hyperammonemia-and-ureagenesis-in-individuals-with-severe-urea-cycle-disorders
#5
Jochen Meyburg, Thomas Opladen, Ute Spiekerkötter, Andrea Schlune, Jens-Peter Schenk, Jan Schmidt, Jürgen Weitz, Jürgen Okun, Friederike Bürger, Tawfeg Ben Omran, Ghassan Abdoh, Hilal Al Rifai, Ahmad Monavari, Vassiliki Konstantopoulou, Stefan Kölker, Marc Yudkoff, Georg F Hoffmann
BACKGROUND: Urea cycle disorders (UCDs) still have a poor prognosis despite several therapeutic advancements. As liver transplantation can provide a cure, liver cell therapy (LCT) might be a new therapeutic option in these patients. METHODS: Twelve patients with severe UCDs were included in this prospective clinical trial. Patients received up to six infusions of cryopreserved human heterologous liver cells via a surgically placed catheter in the portal vein. Portal vein pressure, portal vein flow, and vital signs were monitored continuously...
October 12, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29026518/castleman-s-disease-a-rare-presentation-in-a-retroperitoneal-accessory-spleen-treated-with-a-minimally-invasive-robotic-approach
#6
Fabio Sbrana, Dimin Zhou, Ina Zamfirova, Nathaniel Leonardi
Unicentric Castleman's disease (UCD) is a rare disorder of unknown etiology characterized by localized lymphoid tissue proliferation and interfollicular hypervascularity. A 33-year-old Caucasian female presented with vague abdominal discomfort and pain with pressure. Ultrasound and computed tomography detected a large peripancreatic mass. Robotic-assisted resection of the mass along with en bloc dissection of the encased left adrenal gland was done. Frozen section examination confirmed UCD hyaline vascular variant in a retroperitoneal accessory spleen...
October 2017: Journal of Surgical Case Reports
https://www.readbyqxmd.com/read/28962575/the-incomparability-of-cause-of-death-statistics-under-one-country-two-systems-shanghai-versus-hong-kong
#7
Jiaying Zhao, Edward Jow-Ching Tu, Chi-Kin Law
BACKGROUND: Valid and comparable cause of death (COD) statistics are crucial for health policy analyses. Variations in COD assignment across geographical areas are well-documented while socio-institutional factors may affect the process of COD and underlying cause of death (UCD) determination. This study examines the comparability of UCD statistics in Hong Kong and Shanghai, having two political systems within one country, and assesses how socio-institutional factors influence UCD comparability...
September 29, 2017: Population Health Metrics
https://www.readbyqxmd.com/read/28956617/the-use-of-user-centered-participatory-design-in-serious-games-for-anxiety-and-depression
#8
Maria R Dekker, Alishia D Williams
There is increasing interest in using serious games to deliver or complement healthcare interventions for mental health, particularly for the most common mental health conditions such as anxiety and depression. Initial results seem promising, yet variations exist in the effectiveness of serious games, highlighting the importance of understanding optimal design features. It has been suggested that the involvement of end-users in the design and decision-making process could influence game effectiveness. In user-centered design (UCD) or participatory design (PD), users are involved in stages of the process, including planning, designing, implementing, and testing the serious game...
September 28, 2017: Games for Health
https://www.readbyqxmd.com/read/28916119/safety-and-efficacy-of-glycerol-phenylbutyrate-for-management-of-urea-cycle-disorders-in-patients-aged-2months-to-2years
#9
Susan A Berry, Nicola Longo, George A Diaz, Shawn E McCandless, Wendy E Smith, Cary O Harding, Roberto Zori, Can Ficicioglu, Uta Lichter-Konecki, Beth Robinson, Jerry Vockley
INTRODUCTION: Glycerol phenylbutyrate (GPB) is approved in the US for the management of patients 2months of age and older with urea cycle disorders (UCDs) that cannot be managed with protein restriction and/or amino acid supplementation alone. Limited data exist on the use of nitrogen conjugation agents in very young patients. METHODS: Seventeen patients (15 previously on other nitrogen scavengers) with all types of UCDs aged 2months to 2years were switched to, or started, GPB...
September 8, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28912414/polish-experience-with-liver-transplantation-and-post-transplant-outcomes-in-children-with-urea-cycle-disorders
#10
Edyta Szymańska, Piotr Kaliciński, Joanna Pawłowska, Sylwia Szymańska, Maciej Pronicki, Marek Stefanowicz, Joanna Teisseyre, Dorota Broniszczak, Dariusz Rokicki
BACKGROUND Liver transplantation (LT) is recommended for various metabolic diseases, including urea cycle disorders (UCDs). The aim of this study was to determine indications and outcomes of LT for UCDs in the tertiary reference Children's Hospital in Warsaw, Poland. MATERIAL AND METHODS Medical charts of children with UCD who underwent LT between 2008 and July 2016 were retrospectively reviewed. The following parameters were analyzed: symptoms at time of diagnosis, age at diagnosis, age at transplantation, graft characteristics and survival, postsurgical complications, and biochemical and laboratory results before and after transplantation...
September 15, 2017: Annals of Transplantation: Quarterly of the Polish Transplantation Society
https://www.readbyqxmd.com/read/28900784/liver-involvement-in-urea-cycle-disorders-a-review-of-the-literature
#11
REVIEW
Adrien Bigot, Michel C Tchan, Benjamin Thoreau, Hélène Blasco, François Maillot
Urea cycle disorders (UCDs) are inborn errors of metabolism of the nitrogen detoxification pathway and encompass six principal enzymatic deficiencies. The aging of UCD patients leads to a better knowledge of the long-term natural history of the condition and to the reporting of previously unnoticed manifestations. Despite historical evidence of liver involvement in UCDs, little attention has been paid to this organ until recently. Hence, we reviewed the available scientific evidence on acute and chronic liver dysfunction and liver carcinogenesis in UCDs and discuss their pathophysiology...
November 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28892883/a-case-of-hyperargininaemia-presenting-at-unusually-low-age
#12
Vanita Lal, Daisy Khera, Garima Gupta, Kuldeep Singh, Praveen Sharma
Arginase or ARG1 gene deficiency is a Type V Urea Cycle Disorder (UCD) (catalysing the fifth reaction of urea cycle), associated with hyperammonaemia. Here, we discuss a rare case of a 13-month-old female, having Severe Acute Malnutrition (SAM) and failure to thrive, with serial high plasma ammonia, normal plasma lactate with high arginine and glutamine levels on Amino Acid Assay (AAA) which was performed on 1220 Agilent HPLC. She was admitted for about a month and eventually succumbed to her ailment after a month of discharge...
July 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28888854/quantitation-of-phenylbutyrate-metabolites-by-uplc-ms-ms-demonstrates-inverse-correlation-of-phenylacetate-phenylacetylglutamine-ratio-with-plasma-glutamine-levels
#13
Yi Jiang, Mohammed Almannai, V Reid Sutton, Qin Sun, Sarah H Elsea
Urea cycle disorders (UCDs) are genetic conditions characterized by nitrogen accumulation in the form of ammonia and caused by defects in the enzymes required to convert ammonia to urea for excretion. UCDs include a spectrum of enzyme deficiencies, namely n-acetylglutamate synthase deficiency (NAGS), carbamoyl phosphate synthetase I deficiency (CPS1), ornithine transcarbamylase deficiency (OTC), argininosuccinate lyase deficiency (ASL), citrullinemia type I (ASS1), and argininemia (ARG). Currently, sodium phenylbutyrate and glycerol phenylbutyrate are primary medications used to treat patients with UCDs, and long-term monitoring of these compounds is critical for preventing drug toxic levels...
August 31, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28877826/clinical-analysis-of-unicentric-castleman-s-disease-with-paraneoplastic-pemphigus-and-bronchiolitis-obliterans
#14
Jun-Feng Zhen, Lu Zhang, Xin-Xin Cao, Jun Feng, Dao-Bin Zhou, Song-Bai Lin, Jian Li
Objective To investigate the clinical features of unicentric Castleman's disease(UCD)with paraneoplastic pemphigus(PNP)and bronchiolitis obliterans(BO).Method Data of UCD patients with PNP and BO from Peking Union Medical College Hospital were retrospectively analyzed,along with literatures review. Results Totally 23 cases(11 males and 12 females)were enrolled.The median age was 31 years(13-56 years).The most common pathological type was hyaline-vascular variant(91.4%),and most tumors located in abdominopelvic cavity(69...
August 20, 2017: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
https://www.readbyqxmd.com/read/28845542/cofactors-associated-with-sudden-acquired-retinal-degeneration-syndrome-151-dogs-within-a-reference-population
#15
Candace R Auten, Sara M Thomasy, Philip H Kass, Kathryn L Good, Steven R Hollingsworth, David J Maggs
OBJECTIVE: To determine factors associated with sudden acquired retinal degeneration syndrome (SARDS) diagnosed within one referral population. ANIMALS STUDIED: 151 dogs diagnosed with SARDS. PROCEDURES: Breed, age, sex, and body weight were compared between dogs with electroretinogram-confirmed SARDS and dogs presented to the UC Davis Veterinary Medical Teaching Hospital (UCD-VMTH) from 1991 to 2014. RESULTS: SARDS was diagnosed in 151 dogs, representing 1...
August 27, 2017: Veterinary Ophthalmology
https://www.readbyqxmd.com/read/28752144/is-there-any-relationship-between-low-papp-a-levels-and-measures-of-umbilical-vein-and-placental-thickness-during-first-trimester-of-pregnancy
#16
Gulsum Uysal, Sadan Tutus, Fulya Cagli, Cevdet Adiguzel
OBJECTIVE: Low pregnancy-associated plasma protein A (PAPP-A) level is associated with adverse perinatal outcomes. The purpose of this study was to evaluate relationship between umbilical cord diameter (UCD), umbilical vein and artery diameters (UVD, UAD), placental thickness, and PAPP-A level at gestational age of between 11 and 14 weeks. METHODS: UCD, UVD, UAD, and placental thickness of 246 women were assessed during ultrasound examination at between 11 and 14 weeks of gestation, as well as measurement of nuchal translucency (NT) and crown-rump length (CRL)...
2017: Northern Clinics of Istanbul
https://www.readbyqxmd.com/read/28725569/psychiatric-adult-onset-of-urea-cycle-disorders-a-case-series
#17
Adrien Bigot, Paul Brunault, Christian Lavigne, François Feillet, Sylvie Odent, Elsa Kaphan, Christel Thauvin, Vanessa Leguy, Pierre Broué, Michel C Tchan, François Maillot
Adult onset urea cycle disorders (UCD) may present with psychiatric symptoms, occasionally as the initial presentation. We aimed to describe the characteristics of patients presenting with a psychiatric adult-onset of UCDs, to discuss which signs could suggest this diagnosis in such a situation, and to determine which tests should be conducted. A survey of psychiatric symptoms occurring in teenagers or adults with UCD was conducted in 2010 among clinicians involved in the French society for the study of inborn errors of metabolism (SFEIM)...
September 2017: Molecular Genetics and Metabolism Reports
https://www.readbyqxmd.com/read/28709037/finite-element-analysis-and-clinical-complications-in-mandibular-implant-overdentures-opposing-maxillary-dentures
#18
Tameem Khuder, Norsiah Yunus, Eshamsul Sulaiman, Ali Dabbagh
Denture fracture is a common clinical complication caused by improper material selection, design, or fabrication technique. This study aimed to investigate the effect of two attachment systems on fracture risk of the implant-overdentures (IOD) via finite element analysis (FEA), using the force distributions obtained from patients' occlusal analyses and to compare the obtained results with the clinical complications associated with these attachments. A three-dimensional jaw model comprised of the edentulous bones was constructed...
July 4, 2017: Journal of the Mechanical Behavior of Biomedical Materials
https://www.readbyqxmd.com/read/28643185/keeping-disability-in-mind-a-case-study-in-implantable-brain-computer-interface-research
#19
Laura Specker Sullivan, Eran Klein, Tim Brown, Matthew Sample, Michelle Pham, Paul Tubig, Raney Folland, Anjali Truitt, Sara Goering
Brain-Computer Interface (BCI) research is an interdisciplinary area of study within Neural Engineering. Recent interest in end-user perspectives has led to an intersection with user-centered design (UCD). The goal of user-centered design is to reduce the translational gap between researchers and potential end users. However, while qualitative studies have been conducted with end users of BCI technology, little is known about individual BCI researchers' experience with and attitudes towards UCD. Given the scientific, financial, and ethical imperatives of UCD, we sought to gain a better understanding of practical and principled considerations for researchers who engage with end users...
June 22, 2017: Science and Engineering Ethics
https://www.readbyqxmd.com/read/28629683/the-relationship-between-dietary-intake-growth-and-body-composition-in-inborn-errors-of-intermediary-protein-metabolism
#20
Maureen Evans, Helen Truby, Avihu Boneh
OBJECTIVES: To examine relationships between dietary intake, growth and body composition patterns in patients with inborn errors of intermediary protein metabolism and to determine a safe protein:energy ratio (P:E ratio) associated with optimal growth outcomes. STUDY DESIGN: Retrospective longitudinal data of growth and dietary intake in patients (n = 75) with isovaleric acidemia (IVA; n = 7), methylmalonic acidemia/propionic acidemia (MMA/PA; n = 14), urea cycle defects (UCD; n = 44), classical maple syrup urine disease (MSUD; n = 10) were collected...
September 2017: Journal of Pediatrics
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