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Hunter syndrome

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https://www.readbyqxmd.com/read/29652291/whole-lung-lavage-in-a-patient-with-pulmonary-alveolar-proteinosis
#1
Lindsay R Hunter Guevara, Shane M Gillespie, Alan M Klompas, Norman E Torres, David W Barbara
Pulmonary alveolar proteinosis (PAP) is a rare syndrome in which phospholipoproteinaceous matter accumulates in the alveoli leading to compromised gas exchange. Whole-lung lavage is considered the gold standard for severe autoimmune PAP and offers favorable long-term outcomes. In this case report, we describe the perioperative management and procedural specifics of a patient undergoing WLL for PAP in which an anesthesiologist serves as the proceduralist and a separate anesthesiologist provides anesthesia care for the patient...
April 2018: Annals of Cardiac Anaesthesia
https://www.readbyqxmd.com/read/29651028/author-correction-mapping-the-epidemic-changes-and-risks-of-hemorrhagic-fever-with-renal-syndrome-in-shaanxi-province-china-2005-2016
#2
Weifeng Liang, Xu Gu, Xue Li, Kangjun Zhang, Kejian Wu, Miaomiao Pang, Jianhua Dong, Hunter R Merrill, Tao Hu, Kun Liu, Zhongjun Shao, Hong Yan
A correction to this article has been published and is linked from the HTML and PDF versions of this paper. The error has not been fixed in the paper.
April 13, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29625729/corrigendum-to-elevated-20-hete-in-metabolic-syndrome-regulates-arterial-stiffness-and-systolic-hypertension-via-mmp12-activation-j-mol-cell-cardiol-117-2018-88-99
#3
Amanda Soler, Ian Hunter, Gregory Joseph, Rebecca Hutcheson, Brenda Hutcheson, Jenny Yang, Frank Fan Zhang, Sachindra Raj Joshi, Chastity Bradford, Katherine H Gotlinger, Rachana Maniyar, John R Falck, Spencer Proctor, Michal Laniado Schwartzman, Sachin A Gupte, Petra Rocic
No abstract text is available yet for this article.
April 4, 2018: Journal of Molecular and Cellular Cardiology
https://www.readbyqxmd.com/read/29618310/genetics-and-gene-therapy-in-hunter-disease
#4
Sestito Simona, Francesca Falvo, Rosalbina Apa, Licia Pensabene, Giuseppe Bonapace, Maria Teresa Moricca, Daniela Concolino
Mucopolysaccharidosis type II or Hunter syndrome is a rare X-linked lysosomal storage disorder due to a mutation in the gene encoding the lysosomal enzyme iduronate-2-sulfatase. The consequent enzyme deficiency leads to a progressive, multisystem accumulation of glycosaminoglycans throughout the body, which is the cause of the clinical manifestations involving also Central Nervous System for patients with the severe form of disease. The limits of the current available therapies for Hunter syndrome, hematopoietic stem cell transplantation and recombinant enzyme replacement therapy, mainly regarding brain achievement, encouraged several studies which recognized gene therapy as a potential therapeutic option for this condition...
April 4, 2018: Current Gene Therapy
https://www.readbyqxmd.com/read/29614069/prediction-of-extended-high-viremia-among-newly-hiv-1-infected-persons-in-sub-saharan-africa
#5
Kimberly A Powers, Matthew A Price, Etienne Karita, Anatoli Kamali, William Kilembe, Susan Allen, Eric Hunter, Linda-Gail Bekker, Shabir Lakhi, Mubiana Inambao, Omu Anzala, Mary H Latka, Patricia E Fast, Jill Gilmour, Eduard J Sanders
OBJECTIVE: Prompt identification of newly HIV-infected persons, particularly those who are most at risk of extended high viremia (EHV), allows important clinical and transmission prevention benefits. We sought to determine whether EHV could be predicted during early HIV infection (EHI) from clinical, demographic, and laboratory indicators in a large HIV-1 incidence study in Africa. DESIGN: Adults acquiring HIV-1 infection were enrolled in an EHI study assessing acute retroviral syndrome (ARS) symptoms and viral dynamics...
2018: PloS One
https://www.readbyqxmd.com/read/29588555/insulin-autoimmune-syndrome-a-rare-case-of-hypoglycaemia-resolving-with-immunosuppression
#6
A Hunter, U Graham, J R Lindsay
We report a case of a 58-year-old male presenting with confusion and hypoglycaemia. There had been no prior exposure to oral hypoglycaemic agents or insulin. He was found to have inappropriate endogenous hyperinsulinaemia. Insulinoma was excluded by detailed endocrine assessment. Insulin antibodies were positive in keeping with a diagnosis of insulin autoimmune syndrome (IAS). He was treated with prednisolone 5mg once daily and nutritional supplements leading to resolution of acute confusion and hypoglycaemic episodes...
January 2018: Ulster Medical Journal
https://www.readbyqxmd.com/read/29576490/is-the-inclinometer-a-valid-measure-of-thoracic-kyphosis-a-cross-sectional-study
#7
Donald J Hunter, Darren A Rivett, Sharmain McKiernan, Ishanka Weerasekara, Suzanne J Snodgrass
BACKGROUND: Radiographs are used to monitor thoracic kyphosis in individuals with certain pathologies (e.g. osteoporosis), exposing patients to potentially harmful radiation. Thus, other measures for monitoring the progression of thoracic kyphosis are desirable. The gravity-dependent inclinometer has been shown to be reliable but its validity as a measure of thoracic kyphosis has not been investigated. OBJECTIVES: To determine the validity of the gravity-dependent (analogue) inclinometer for measuring thoracic kyphosis...
March 6, 2018: Brazilian Journal of Physical Therapy
https://www.readbyqxmd.com/read/29566313/spinal-cord-stimulation-for-the-treatment-of-failed-neck-surgery-syndrome-outcome-of-a-prospective-case-series
#8
Corey W Hunter, Jonathan Carlson, Ajax Yang, Timothy Deer
OBJECTIVES: Spinal cord stimulation (SCS) is an accepted, cost-effective treatment option for a variety of chronic pain syndromes, including failed back surgery syndrome (FBSS). The application of SCS in the cervical spine, particularly for pain after cervical spine surgery, has been drawn into question in recent years by payers due to a purported lack of clinical evidence. To challenge this claim, we analyzed data from a prospective registry to support the use of SCS in the cervical spine for pain after spine surgery...
March 22, 2018: Neuromodulation: Journal of the International Neuromodulation Society
https://www.readbyqxmd.com/read/29565946/anatomy-pathophysiology-and-interventional-therapies-for-chronic-pelvic-pain-a-review
#9
Corey W Hunter, Brad Stovall, Grant Chen, Jonathan Carlson, Robert Levy
BACKGROUND: Chronic pelvic pain (CPP) represents a group of poorly understood disorders that are often refractory to conventional treatment. Referral to pain management typically occurs later in the continuum of care; as such, many of the injections and nerve blocks commonly prescribed for such patients are potentially limited in efficacy. While neuromodulation is conventionally considered the next algorithmic step in the treatment of chronic pain after injections fail, there is a common perception that neuromodulation is largely ineffective for CPP conditions...
March 2018: Pain Physician
https://www.readbyqxmd.com/read/29550833/a-case-of-lown-ganong-levine-syndrome-due-to-an-accessory-pathway-of-james-fibers-or-enhanced-atrioventricular-nodal-conduction-eavnc
#10
Juanita Hunter, Emmanouil Tsounias, John Cogan, Ming-Lon Young
BACKGROUND Lown-Ganong-Levine syndrome, includes a short PR interval, normal QRS complex, and paroxysmal tachycardia. The pathophysiology of this syndrome includes an accessory pathway connecting the atria and the atrioventricular (AV) node (James fiber), or between the atria and the His bundle (Brechenmacher fiber). Similar features are seen in enhanced atrioventricular nodal conduction (EAVNC), with the underlying pathophysiology due to a fast pathway to the AV node, and with the diagnosis requiring specific electrophysiologic criteria...
March 18, 2018: American Journal of Case Reports
https://www.readbyqxmd.com/read/29527552/perioperative-airway-management-for-aortic-valve-replacement-in-an-adult-with-mucopolysaccharidosis-type-ii-hunter-syndrome
#11
Kazuchika Suzuki, Hiroaki Sakai, Kenji Takahashi
We herein report anesthetic management during aortic valve replacement for aortic valve regurgitation in a patient with adult mucopolysaccharidosis type II (MPS type 2) (Hunter syndrome). This disorder is rare and related to the accumulation of a mucopolysaccharide in lysosomes. It affects various organs, including the airways, heart, and central nerves. In children with MPS type 2, the risk of airway obstruction during anesthesia/sedation is high, and the degree of difficulty increases with aging. The patient described herein was a 33-year-old male without mental retardation...
2018: JA Clinical Reports
https://www.readbyqxmd.com/read/29525821/effect-of-loading-dose-of-atorvastatin-prior-to-planned-percutaneous-coronary-intervention-on-major-adverse-cardiovascular-events-in-acute-coronary-syndrome-the-secure-pci-randomized-clinical-trial
#12
Otavio Berwanger, Eliana Vieira Santucci, Pedro Gabriel Melo de Barros E Silva, Isabella de Andrade Jesuíno, Lucas Petri Damiani, Lilian Mazza Barbosa, Renato Hideo Nakagawa Santos, Ligia Nasi Laranjeira, Flávia de Mattos Egydio, Juliana Aparecida Borges de Oliveira, Frederico Toledo Campo Dall Orto, Pedro Beraldo de Andrade, Igor Ribeiro de Castro Bienert, Carlos Eduardo Bosso, José Armando Mangione, Carisi Anne Polanczyk, Amanda Guerra de Moraes Rego Sousa, Renato Abdala Karam Kalil, Luciano de Moura Santos, Andrei Carvalho Sposito, Rafael Luiz Rech, Antônio Carlos Sobral Sousa, Felipe Baldissera, Bruno Ramos Nascimento, Roberto Rocha Corrêa Veiga Giraldez, Alexandre Biasi Cavalcanti, Sabrina Bernardez Pereira, Luiz Alberto Mattos, Luciana Vidal Armaganijan, Hélio Penna Guimarães, José Eduardo Moraes Rego Sousa, John Hunter Alexander, Christopher Bull Granger, Renato Delascio Lopes
Importance: The effects of loading doses of statins on clinical outcomes in patients with acute coronary syndrome (ACS) and planned invasive management remain uncertain. Objective: To determine if periprocedural loading doses of atorvastatin decrease 30-day major adverse cardiovascular events (MACE) in patients with ACS and planned invasive management. Design, Setting, and Participants: Multicenter, double-blind, placebo-controlled, randomized clinical trial conducted at 53 sites in Brazil among 4191 patients with ACS evaluated with coronary angiography to proceed with a percutaneous coronary intervention (PCI) if anatomically feasible...
March 11, 2018: JAMA: the Journal of the American Medical Association
https://www.readbyqxmd.com/read/29506582/early-childhood-onset-of-high-grade-atrioventricular-block-in-hunter-syndrome
#13
Meghan M Chlebowski, Bryce A Heese, Lindsey E Malloy-Walton
Cardiac involvement has been reported in various mucopolysaccharidoses syndromes. Cardiac valve pathology is the most prominent cardiac manifestation of patients with these syndromes. To date, there have been no reports of early childhood onset of high-grade atrioventricular block in patients with Hunter syndrome. We present a case of a 3-year-old boy with Hunter syndrome who was found to have various degrees of atrioventricular block. This case highlights the importance of early routine cardiac screening for conduction abnormalities and close follow-up in patients with mucopolysaccharidoses syndromes...
March 6, 2018: Cardiology in the Young
https://www.readbyqxmd.com/read/29492144/bow-hunter-s-syndrome-in-a-patient-with-a-right-hypoplastic-vertebral-artery-and-a-dynamically-compressible-left-vertebral-artery
#14
David Z Cai, Ryan P Roach, John P Weaver, Gerald T McGillicuddy, Zachary M Mansell, Jonathan P Eskander, Mark S Eskander
This is a case report of a 48-year-old man with multiple transient ischemic attacks and a known hypoplastic right vertebral artery (VA) who presented after a syncopal event while turning his head to the left. The objective of this study is to demonstrate the necessity of proper diagnosis and management of cerebrovascular pathology including imaging and surgical intervention in patients with known anatomical anomalies. This study was conducted at Massachusetts, United States of America. Our patient's history was significant for a hypoplastic right VA and a stenotic segment of the right VA at the C3-C4 junction...
January 2018: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/29483611/intragenic-dna-methylation-in-buccal-epithelial-cells-and-intellectual-functioning-in-a-paediatric-cohort-of-males-with-fragile-x
#15
Marta Arpone, Emma K Baker, Lesley Bretherton, Minh Bui, Xin Li, Simon Whitaker, Cheryl Dissanayake, Jonathan Cohen, Chriselle Hickerton, Carolyn Rogers, Mike Field, Justine Elliott, Solange M Aliaga, Ling Ling, David Francis, Stephen J C Hearps, Matthew F Hunter, David J Amor, David E Godler
Increased intragenic DNA methylation of the Fragile X Related Epigenetic Element 2 (FREE2) in blood has been correlated with lower intellectual functioning in females with fragile X syndrome (FXS). This study explored these relationships in a paediatric cohort of males with FXS using Buccal Epithelial Cells (BEC). BEC were collected from 25 males with FXS, aged 3 to 17 years and 19 age-matched male controls without FXS. Methylation of 9 CpG sites within the FREE2 region was examined using the EpiTYPER approach...
February 26, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29478001/attenuated-form-of-type-ii-mucopolysaccharidoses-hunter-syndrome-pitfalls-and-potential-clues-in-diagnosis
#16
Partha Pratim Chakraborty, Shinjan Patra, Sugata Narayan Biswas, Himanshu Barman
No abstract text is available yet for this article.
February 23, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29466866/chromosome-7q11-23-duplication-syndrome-presenting-as-neuropsychiatric-regression-in-late-adolescence-a-new-manifestation-of-a-new-syndrome
#17
Julanne Frater, Ian G Williams, Colleen Hunter
No abstract text is available yet for this article.
February 1, 2018: Australian and New Zealand Journal of Psychiatry
https://www.readbyqxmd.com/read/29465674/the-etiology-of-vaginal-discharge-syndrome-in-zimbabwe-results-from-the-zimbabwe-sti-etiology-study
#18
Z Mike Chirenje, Nicholas Dhibi, H Hunter Handsfield, Elizabeth Gonese, Beth Tippett Barr, Lovemore Gwanzura, Ahmed S Latif, Dumisili Venessa Maseko, Ranmini S Kularatne, Mufuta Tshimanga, Peter H Kilmarx, Anna Machiha, Owen Mugurungi, Cornelis A Rietmeijer
INTRODUCTION: Symptomatic vaginal discharge is a common gynecological condition managed syndromically in most developing countries. In Zimbabwe, women presenting with symptomatic vaginal discharge are treated with empirical regimens that commonly cover both sexually transmitted infections (STI) and reproductive tract infections, typically including a combination of an intramuscular injection of kanamycin, and oral doxycycline and metronidazole regimens. This study was conducted to determine the current etiology of symptomatic vaginal discharge and assess adequacy of current syndromic management guidelines...
November 29, 2017: Sexually Transmitted Diseases
https://www.readbyqxmd.com/read/29432500/large-animals-as-potential-models-of-human-mental-and-behavioral-disorders
#19
REVIEW
Michał Danek, Janusz Danek, Aleksander Araszkiewicz
Many animal models in different species have been developed for mental and behavioral disorders. This review presents large animals (dog, ovine, swine, horse) as potential models of this disorders. The article was based on the researches that were published in the peer-reviewed journals. Aliterature research was carried out using the PubMed database. The above issues were discussed in the several problem groups in accordance with the WHO International Statistical Classification of Diseases and Related Health Problems 10thRevision (ICD-10), in particular regarding: organic, including symptomatic, disorders; mental disorders (Alzheimer's disease and Huntington's disease, pernicious anemia and hepatic encephalopathy, epilepsy, Parkinson's disease, Creutzfeldt-Jakob disease); behavioral disorders due to psychoactive substance use (alcoholic intoxication, abuse of morphine); schizophrenia and other schizotypal disorders (puerperal psychosis); mood (affective) disorders (depressive episode); neurotic, stress-related and somatoform disorders (posttraumatic stress disorder, obsessive-compulsive disorder); behavioral syndromes associated with physiological disturbances and physical factors (anxiety disorders, anorexia nervosa, narcolepsy); mental retardation (Cohen syndrome, Down syndrome, Hunter syndrome); behavioral and emotional disorders (attention deficit hyperactivity disorder)...
December 30, 2017: Psychiatria Polska
https://www.readbyqxmd.com/read/29428638/elevated-20-hete-in-metabolic-syndrome-regulates-arterial-stiffness-and-systolic-hypertension-via-mmp12-activation
#20
Amanda Soler, Ian Hunter, Gregory Joseph, Rebecca Hutcheson, Brenda Hutcheson, Jenny Yang, Frank Fan Zhang, Sachindra Raj Joshi, Chastity Bradford, Katherine H Gotlinger, Rachana Maniyar, John R Falck, Spencer Proctor, Michal Laniado Schwartzman, Sachin A Gupte, Petra Rocic
Arterial stiffness plays a causal role in development of systolic hypertension. 20-hydroxyeicosatetraeonic acid (20-HETE), a cytochrome P450 (CYP450)-derived arachidonic acid metabolite, is known to be elevated in resistance arteries in hypertensive animal models and loosely associated with obesity in humans. However, the role of 20-HETE in the regulation of large artery remodeling in metabolic syndrome has not been investigated. We hypothesized that elevated 20-HETE in metabolic syndrome increases matrix metalloproteinase 12 (MMP12) activation leading to increased degradation of elastin, increased large artery stiffness and increased systolic blood pressure...
February 8, 2018: Journal of Molecular and Cellular Cardiology
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