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https://www.readbyqxmd.com/read/28806300/improving-benchmarks-for-global-surgery-nationwide-enumeration-of-operations-performed-in-ghana
#1
Adam Gyedu, Barclay Stewart, Cameron Gaskill, Godfred Boakye, Ebenezer Appiah-Denkyira, Peter Donkor, Ronald Maier, Robert Quansah, Charles Mock
OBJECTIVE: To evaluate the operation rate in Ghana and characterize it by types of procedures and hospital level. BACKGROUND: The Lancet Commission on Global Surgery recommended an annual rate of 5000 operations/100,000 people as a benchmark at which low- and middle-income countries could achieve most of the population-wide benefits of surgery, but did not define procedure-type benchmarks. METHODS: Data on operations performed from June 2014 to May 2015 were obtained from representative samples of 48 of 124 district-level (first-level) hospitals, 9 of 11 regional (referral) hospitals, and 3 of 5 tertiary hospitals, and scaled-up to nationwide estimates...
August 10, 2017: Annals of Surgery
https://www.readbyqxmd.com/read/28804634/muscle-mri-findings-in-a-one-year-old-girl-with-merosin-deficient-congenital-muscular-dystrophy-type-1a-due-to-lama2-mutation-a-case-report
#2
Yingyin Liang, Guidian Li, Songlin Chen, Rongxing He, Xiangxue Zhou, Yingming Chen, Xue Xu, Ronglan Zhu, Cheng Zhang
The objective of the present study was to characterize the muscle magnetic resonance imaging (MRI) features of a 1-year-old girl with merosin-deficient congenital muscular dystrophy type 1A (MDC1A). Beginning as an infant, this patient exhibited severe hypotonia and proximal weakness, as well as delays in developmental milestones. Her serum creatine kinase levels at 3 months, 8 months and 1 year were 2,959, 1,621 and 1,659 U/l, respectively. Brain MRI indicated symmetric, mild T1WI low, mild T2WI and FLAIR high radial patterns in the white matter of the Cornu posterius of the ventricular lateral...
August 2017: Biomedical Reports
https://www.readbyqxmd.com/read/28804003/effect-and-proposed-mechanism-of-vitamin-c-modulating-amino-acid-regulation-of-autophagic-proteolysis
#3
Md Razaul Karim, Motoni Kadowaki
Autophagy is an intracellular bulk degradation process, induced under nutrient starvation. Failure of autophagy has been recognized as a contributor to aging and multiple age related neurodegenerative diseases. Improving autophagy is a beneficial anti-aging strategy, however very few physiological regulators have been identified. Here, we demonstrate that vitamin C is a nutritional stimulator of autophagy. Supplementation of fresh hepatocytes with vitamin C increased autophagic proteolysis significantly in the presence of amino acids in a dose- and time-dependent manner, although no effect was observed in the absence of amino acids...
August 10, 2017: Biochimie
https://www.readbyqxmd.com/read/28803880/vitamin-d-and-symptoms-of-depression-in-overweight-or-obese-adults-a-cross-sectional-study-and-randomized-placebo-controlled-trial
#4
Aya Mousa, Negar Naderpoor, Maximilian P J de Courten, Barbora de Courten
Recent evidence suggests that vitamin D deficiency may contribute to increased risk of depression. However, previous studies are limited by variability in participant characteristics including vitamin D deficiency status and presence of existing diseases, use of low doses of vitamin D supplementation for short durations, and use of co-interventions or psychotropic drugs. We examined whether 25-hydroxyvitamin D (25(OH)D) concentrations were associated with symptoms of depression, as well as whether vitamin D supplementation reduced symptoms of depression in overweight or obese and vitamin D-deficient, but otherwise healthy adults...
August 10, 2017: Journal of Steroid Biochemistry and Molecular Biology
https://www.readbyqxmd.com/read/28803389/gastrointestinal-manifestations-of-stat3-deficient-hyper-ige-syndrome
#5
Manish Arora, Preet Bagi, Anna Strongin, Jennifer Heimall, Xiongce Zhao, Monica G Lawrence, Apurva Trivedi, Carolyn Henderson, Amy Hsu, Martha Quezado, David E Kleiner, Aradhana M Venkatesan, Steven M Holland, Alexandra F Freeman, Theo Heller
OBJECTIVE: STAT 3 deficiency (autosomal dominant hyper immunoglobulin E syndrome (AD-HIES)) is a primary immunodeficiency disorder with multi-organ involvement caused by dominant negative signal transducer and activator of transcription gene 3 (STAT3) mutations. We sought to describe the gastrointestinal (GI) manifestations of this disease. METHODS: Seventy subjects aged five to 60 years with a molecular diagnosis of AD-HIES were evaluated at the National Institutes of Health (NIH)...
August 13, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28802375/post-tonsillectomy-hemorrhagic-outcomes-in-children-with-bleeding-disorders-at-a-single-institution
#6
Priyesh N Patel, Alexandra M Arambula, Allison P Wheeler, Edward B Penn
OBJECTIVE: To report on the post-tonsillectomy bleeding outcomes and factors associated with hemorrhage among children with pre- or post-operatively diagnosed bleeding disorders treated with an institutional protocol. METHODS: Retrospective cohort study of patients with hematologic disorders who underwent tonsillectomy between 2003 and 2016 and were treated with perioperative desmopressin or factor replacement and/or aminocaproic acid. Postoperative outcomes were compared to controls matched for age, sex, and indication for surgery...
September 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28801223/limited-benefits-of-presymptomatic-cord-blood-transplantation-in-neurovisceral-acid-sphingomyelinase-deficiency-asmd-intermediate-type
#7
Oriane Mercati, Samia Pichard, Marie Ouachée, Roseline Froissart, Odile Fenneteau, Bastien Roche, Monique Elmaleh-Bergès, Yves Bertrand, Hélène Ogier de Baulny, Marie T Vanier, Manuel Schiff
Acid sphingomyelinase (ASM) deficient Niemann-Pick disease is a lysosomal storage disorder resulting from mutations in the SMPD1 gene. The clinical spectrum distinguishes a severe infantile neurological form (type A), a non-neurological visceral form (type B) and a rare intermediate neurovisceral form. We report the first case of presymptomatic cord blood transplantation in a child with the intermediate type of ASM deficiency due to a homozygous Tyr369Cys mutation, whose affected elder brother had developed neurodevelopmental delay from 19 months of age, and had died from severe visceral complications at the age of 3...
July 29, 2017: European Journal of Paediatric Neurology: EJPN
https://www.readbyqxmd.com/read/28801073/newborn-screening-for-carnitine-palmitoyltransferase-ii-deficiency-using-c16-c18-1-c2-evaluation-of-additional-indices-for-adequate-sensitivity-and-lower-false-positivity
#8
Go Tajima, Keiichi Hara, Miyuki Tsumura, Reiko Kagawa, Satoshi Okada, Nobuo Sakura, Shinsuke Maruyama, Atsuko Noguchi, Tomonari Awaya, Mika Ishige, Nobuyuki Ishige, Ikuma Musha, Sayaka Ajihara, Akira Ohtake, Etsuo Naito, Yusuke Hamada, Tomotaka Kono, Tomoko Asada, Hideo Sasai, Toshiyuki Fukao, Ryoji Fujiki, Osamu Ohara, Ryosuke Bo, Kenji Yamada, Hironori Kobayashi, Yuki Hasegawa, Seiji Yamaguchi, Masaki Takayanagi, Ikue Hata, Yosuke Shigematsu, Masao Kobayashi
BACKGROUND: Carnitine palmitoyltransferase (CPT) II deficiency is one of the most common forms of mitochondrial fatty acid oxidation disorder (FAOD). However, newborn screening (NBS) for this potentially fatal disease has not been established partly because reliable indices are not available. METHODS: We diagnosed CPT II deficiency in a 7-month-old boy presenting with hypoglycemic encephalopathy, which apparently had been missed in the NBS using C16 and C18:1 concentrations as indices...
July 31, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28800383/epithelial-desquamation-observed-in-a-phase-i-study-of-an-oral-cathepsin-c-inhibitor-gsk2793660
#9
Bruce E Miller, Ruth J Mayer, Navin Goyal, Joanne Bal, Nigel Dallow, Malcolm Boyce, Donald Carpenter, Alison Churchill, Teresa Heslop, Aili L Lazaar
AIMS: Cathepsin C (CTSC) is necessary for the activation of several serine proteases including neutrophil elastase (NE), cathepsin G, and proteinase 3. GSK2793660 is an oral, irreversible inhibitor of CTSC that is hypothesized to provide an alternative route to achieve NE inhibition and was tested in a Phase I study. METHODS: Single escalating oral doses of GSK2793660 0.5 mg to 20 mg or placebo were administered in a randomized crossover design to healthy male subjects; a separate cohort received once daily doses of 12 mg or placebo for 21 days...
August 11, 2017: British Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/28800252/progressive-changes-in-walking-kinematics-and-kinetics-after-anterior-cruciate-ligament-injury-and-reconstruction-a-review-and-meta-analysis
#10
Lindsay V Slater, Joseph M Hart, Adam R Kelly, Christopher M Kuenze
CONTEXT:   Anterior cruciate ligament (ACL) injury and ACL reconstruction (ACLR) result in persistent alterations in lower extremity movement patterns. The progression of lower extremity biomechanics from the time of injury has not been described. OBJECTIVE:   To compare the 3-dimensional (3D) lower extremity kinematics and kinetics of walking among individuals with ACL deficiency (ACLD), individuals with ACLR, and healthy control participants from 3 to 64 months after ACLR...
August 11, 2017: Journal of Athletic Training
https://www.readbyqxmd.com/read/28798412/a-time-varying-biased-random-walk-approach-to-human-growth
#11
Béla Suki, Urs Frey
Growth and development are dominated by gene-environment interactions. Many approaches have been proposed to model growth, but most are either descriptive or describe population level phenomena. We present a random walk-based growth model capable of predicting individual height, in which the growth increments are taken from time varying distributions mimicking the bursting behaviour of observed saltatory growth. We derive analytic equations and also develop a computational model of such growth that takes into account gene-environment interactions...
August 10, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28797127/the-resqu-index-a-new-instrument-to-appraise-the-quality-of-research-on-birth-place
#12
Saraswathi Vedam, Chris Rossiter, Caroline S E Homer, Kathrin Stoll, Vanessa L Scarf
OBJECTIVE: Place of birth is a known determinant of health care outcomes, interventions and costs. Many studies have examined the maternal and perinatal outcomes when women plan to give birth in hospitals compared with births in birth centres or at home. However, these studies vary substantially in rigour; assessing their quality is challenging. Existing research appraisal tools do not always capture important elements of study design that are critical when comparing outcomes by planned place of birth...
2017: PloS One
https://www.readbyqxmd.com/read/28796048/clinicopathologic-study-of-succinate-dehydrogenase-deficient-gastrointestinal-stromal-tumors-a-single-institutional-experience-in-china
#13
Weizhen Liu, Xiangyu Zeng, Xiuli Wu, Jun He, Jinbo Gao, Xiaoming Shuai, Guobin Wang, Peng Zhang, Kaixiong Tao
Gastrointestinal stromal tumors (GISTs) that are not driven by kinase mutations, as are most GISTs, often show loss of function of the succinate dehydrogenase (SDH) complex and are considered SDH-deficient GISTs. SDH-deficient GISTs share many distinct characteristics compared with conventional GISTs. However, data regarding these characteristics, particularly among Asian people, are relatively limited. The objective of this study was to characterize the clinicopathologic characteristics, treatment, and prognosis of these uncommon GISTs...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28793868/clinical-and-laboratory-profiles-of-patients-with-early-spontaneous-healing-in-cutaneous-localized-leishmaniasis-a-historical-cohort-study
#14
Carla Oliveira-Ribeiro, Maria Inês Fernandes Pimentel, Raquel de Vasconcellos Carvalhaes Oliveira, Aline Fagundes, Maria de Fatima Madeira, Cintia Xavier Mello, Eliame Mouta-Confort, Claudia Maria Valete-Rosalino, Erica de Camargo Ferreira Vasconcellos, Marcelo Rosandiski Lyra, Leonardo Pereira Quintella, Liliane de Fatima Antonio, Armando Schubach, Fatima Conceição-Silva
BACKGROUND: Skin ulcers in American cutaneous leishmaniasis (ACL) may heal spontaneously after months/years. However, few cases may present quick heal even during diagnosis procedure (early spontaneous healing- ESH). The main objective of this study was to compare ESH patients with cases requiring specific treatment [non-ESH (NESH)]. METHODS: A historical cohort study of ACL patients (n = 445) were divided into 2 groups: ESH - spontaneously healed patients (n = 13; 2...
August 10, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28793824/dual-therapy-deflazacort-doxycyclyne-is-better-than-deflazacort-monotherapy-to-alleviate-cardiomyopathy-in-dystrophin-deficient-mdx-mice
#15
Juliano Alves Pereira, Adriana Fogagnolo Mauricio, Maria Julia Marques, Humberto Santo Neto
Cardiomyopathy related to the absence of dystrophin is an important feature in Duchenne muscular dystrophy (DMD) and in the mdx mouse. Doxycycline (DOX) could be a potential therapy for mdx skeletal muscles dystrophy. We investigated whether the corticoid deflazacort (DFZ) plus DOX could improve cardiac mdx dystrophy better than DFZ alone, later (17 months) in dystrophy. Mdx mice (8 months old) received DFZ/DOX or DFZ for 9 months. The combined therapy was greater than DFZ in reducing fibrosis (60% decrease with DFZ/DOX and 40% with DFZ alone) in the right ventricle and transforming growth factor β levels (6...
September 2017: Journal of Cardiovascular Pharmacology and Therapeutics
https://www.readbyqxmd.com/read/28793284/growth-hormone-therapy-in-children-with-kabuki-syndrome-1-year-treatment-results
#16
Dina A Schott, Willem J M Gerver, Constance T R M Stumpel
BACKGROUND/AIMS: Kabuki syndrome (KS) is a rare genetic malformation syndrome, resulting in characteristic features such as short stature. We investigate whether growth hormone (GH) treatment increases linear height and influences body proportions in KS children. METHODS: In this prospective study, 18 genetically confirmed prepubertal KS children (9 females and 9 males) aged from 3.8 to 10.1 years (mean 6.8 ± 2.1 years) were treated with recombinant human GH (rhGH) for 1 year...
August 9, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/28791864/melting-of-oxygen-vacancy-order-at-oxide-heterostructure-interface
#17
Kei Hirai, Ryotaro Aso, Yusuke Ozaki, Daisuke Kan, Mitsutaka Haruta, Noriya Ichikawa, Hiroki Kurata, Yuichi Shimakawa
Modifications of oxygen coordination environments in heterostructures consisting of dissimilar oxides often emerge and lead to unusual properties of the constituent materials. While lots of attention has been paid on slight modifications of rigid oxygen octahedra of perovskite-based heterointerfaces, revealing the modification behaviors of the oxygen coordination environments in the heterostructures containing oxides with oxygen vacancies has been challenging. Here we show that a significant modification of oxygen coordination environments -melting of oxygen vacancy order- is induced at the heterointerface between SrFeO2...
August 9, 2017: ACS Applied Materials & Interfaces
https://www.readbyqxmd.com/read/28791817/binder-syndrome-clinical-findings-and-surgical-treatment-of-18-patients-at-the-department-of-plastic-surgery-in-polanica-zdr%C3%A3-j
#18
Piotr H Drozdowski, Ireneusz Łątkowski, Mateusz G Zachara, Piotr Wójcicki
BACKGROUND: Binder syndrome (BS) is an uncommon congenital underdevelopment of the maxilla and nasal skeleton. Other clinical features include a hypoplastic or absent anterior nasal spine; a short, flat nose with short columella; an acute nasolabial angle; a convex upper lip and class III malocclusion. OBJECTIVES: The aim of the study was to outline the major characteristics of BS and to present a variety of surgical treatment methods. MATERIAL AND METHODS: The study included 18 patients treated in the authors' department from 1989 to 2013...
May 2017: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
https://www.readbyqxmd.com/read/28791025/the-ido-ahr-axis-controls-th17-treg-immunity-in-a-pulmonary-model-of-fungal-infection
#19
Eliseu Frank de Araújo, Claudia Feriotti, Nayane Alves de Lima Galdino, Nycolas Willian Preite, Vera Lúcia Garcia Calich, Flávio Vieira Loures
In infectious diseases, the enzyme indoleamine 2,3 dioxygenase-1 (IDO1) that catalyzes the tryptophan (Trp) degradation along the kynurenines (Kyn) pathway has two main functions, the control of pathogen growth by reducing available Trp and immune regulation mediated by the Kyn-mediated expansion of regulatory T (Treg) cells via aryl hydrocarbon receptor (AhR). In pulmonary paracoccidioidomycosis (PCM) caused by the dimorphic fungus Paracoccidioides brasiliensis, IDO1 was shown to control the disease severity of both resistant and susceptible mice to the infection; however, only in resistant mice, IDO1 is induced by TGF-β signaling that confers a stable tolerogenic phenotype to dendritic cells (DCs)...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28789730/iodine-status-of-postpartum-women-and-their-infants-in-australia-after-the-introduction-of-mandatory-iodine-fortification
#20
Dao Huynh, Dominique Condo, Robert Gibson, Beverly Muhlhausler, Philip Ryan, Sheila Skeaff, Maria Makrides, Shao J Zhou
Mandatory I fortification in bread was introduced in Australia in 2009 in response to the re-emergence of biochemical I deficiency based on median urinary I concentration (UIC)<100 µg/l. Data on the I status of lactating mothers and their infants in Australia are scarce. The primary aim of this study was to assess the I status, determined by UIC and breast milk I concentration (BMIC), of breast-feeding mothers in South Australia and UIC of their infants. The secondary aim was to assess the relationship between the I status of mothers and their infants...
June 2017: British Journal of Nutrition
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