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Radu Prejbeanu, Dan V Poenaru, Andrei Dan Balanescu, Mihail-Lazar Mioc
PURPOSE: The medial plica (MP) is a normal anatomic structure consisting of a fold in the synovial layer of the joint. Arthroscopic resection is currently used as a gold standard procedure in patients with medial plica syndrome (MPS), but there are few prospective studies that analyze the long-term functional outcomes of plica resection. METHODS: The purpose of this prospective study was to evaluate the long-term results of arthroscopic resection of the medial plicae of the knee...
October 20, 2016: International Orthopaedics
J Do Cao, A Wiedemann, T Quinaux, S F Battaglia-Hsu, L Mainard, R Froissart, C Bonnemains, S Ragot, B Leheup, P Journeau, F Feillet
: Patients under 5 years were not evaluated in the phase-3 study for enzyme replacement therapy (ERT) in MPS IV A. Here we describe the evolution of a severe Morquio A pediatric patient who was diagnosed at 19 months old and treated by ERT at 21 months old for the next 30 months. Applying the standard ERT protocol on this very young patient appeared to reduce his urinary excretion of glycosaminoglycans (GAGs); the improvements in both the 6 minute-walk test (6MWT) and the stair climb test, however, were no different than those reported in the nature history study...
December 2016: Molecular Genetics and Metabolism Reports
S Somanadhan, P J Larkin
BACKGROUND: Many rare diseases of childhood are life-threatening and chronically debilitating, so living with a rare disease is an on-going challenge for patients and their families. MPS is one of a range of rare inherited metabolic disorders (IMDs) that come under category 3 of life-limiting conditions, where there is no curative treatment available at present. Although the study of rare diseases is increasingly novel, and of clinical importance to the population, the lack of empirical data in the field to support policy and strategy development is a compelling argument for further research to be sought...
October 10, 2016: Orphanet Journal of Rare Diseases
Andrew T Lucas, Taylor F White, Allison M Deal, Leah B Herity, Gina Song, Charlene M Santos, William C Zamboni
The mononuclear phagocyte system (MPS) has previously been shown to significantly affect the clearance, tumor delivery, and efficacy of nanoparticles (NPs). This study profiled MPS cell infiltration in murine preclinical tumor models and evaluated how these differences may affect tumor disposition of PEGylated liposomal doxorubicin (PLD) in models sensitive and resistant to PLD. Significant differences in MPS presence existed between tumor types (e.g. ovarian versus endometrial), cell lines within the same tumor type, and location of tumor implantation (i...
October 5, 2016: Nanomedicine: Nanotechnology, Biology, and Medicine
Francyne Kubaski, Heidi H Kecskemethy, H Theodore Harcke, Shunji Tomatsu
To date, the only published reports of bone mineral density (BMD) in MPS IV involve patients with MPS IVA; no reports exist describing BMD for MPS IVB. In this prospective study of BMD in three patients with MPS IVB, BMD was acquired by dual-energy X-ray absorptiometry (DXA) at whole body (WB), lumbar spine (LS), and lateral distal femur (LDF). Functional abilities, ambulatory status, medical history, and height z-score were evaluated. Three patients with MPS IVB (two females), aged 17.7, 31.4 and 31.7 years, were evaluated...
September 2016: Molecular Genetics and Metabolism Reports
E Artoni, G L Sighinolfi, A M Gatti, M Sebastiani, M Colaci, D Giuggioli, C Ferri
BACKGROUND: Mixed cryoglobulinemia (MC) is a rare multisystem disease whose aetiopathogenesis is not completely understood. Hepatitis C virus (HCV) infection may have a causative role, and genetic and/or environmental factors may also contribute. AIMS: To investigate the presence and possible role of environmental agents in MC. METHODS: We recruited 30 HCV-infected MC patients with different clinical manifestations and a control group of 30 healthy, sex-/age-matched volunteers...
September 30, 2016: Occupational Medicine
Ainslie L K Derrick-Roberts, Kavita Panir, Carmen E Pyragius, Krystyna H Zarrinkalam, Gerald J Atkins, Sharon Byers
Severe, progressive skeletal dysplasia is a major symptom of multiple mucopolysaccharidoses (MPS) types. While a gene therapy approach initiated at birth has been shown to prevent the development of bone pathology in different animal models of MPS, the capacity to correct developed bone disease is unknown. In this study, ex vivo micro-computed tomography was used to demonstrate that bone mass and architecture of murine MPS VII L5 vertebrae were within the normal range at 1month of age but by 2months of age were significantly different to normal...
September 24, 2016: Molecular Genetics and Metabolism
Frank R Wendt, David H Warshauer, Xiangpei Zeng, Jennifer D Churchill, Nicole M M Novroski, Bing Song, Jonathan L King, Bobby L LaRue, Bruce Budowle
Short tandem repeat (STR) loci are the traditional markers used for kinship, missing persons, and direct comparison human identity testing. These markers hold considerable value due to their highly polymorphic nature, amplicon size, and ability to be multiplexed. However, many STRs are still too large for use in analysis of highly degraded DNA. Small bi-allelic polymorphisms, such as insertions/deletions (INDELs), may be better suited for analyzing compromised samples, and their allele size differences are amenable to analysis by capillary electrophoresis...
September 20, 2016: Forensic Science International. Genetics
Angela S P Lin, Chelsea M Fechter, Mark Magill, Felix Wipf, Thomas Moore, Robert E Guldberg
BACKGROUND: Metallic reconstruction plates used for fracture stabilization typically require intraoperative contouring for patient-specific anatomical fit. Despite this, characterization of plate mechanical properties after contouring has previously been limited. The objective of this study was to assess whether contouring affects fatigue resistance for three types of Stryker seven-hole stainless steel (SS) 316LVM fracture fixation plates. The hypothesis was that for each plate type, more contouring repetitions would result in lower fatigue resistance...
September 26, 2016: Journal of Orthopaedic Surgery and Research
F J Bemelman, J W de Fijter, J Kers, C Meyer, H Peters-Sengers, E F de Maar, K A M I van der Pant, A P J de Vries, J-S Sanders, A Zwinderman, M M Idu, S Berger, M E J Reinders, C Krikke, I M Bajema, M C van Dijk, I J M Ten Berge, J Ringers, J Lardy, D Roelen, D-J Moes, S Florquin, J J Homan van der Heide
In renal transplantation, use of calcineurin inhibitors (CNIs) is associated with nephrotoxicity and immunosuppression with malignancies and infections. This trial aimed to minimize CNI exposure and total immunosuppression while maintaining efficacy. We performed a randomized controlled, open-label multicenter trial with early cyclosporine A (CsA) elimination. Patients started with basiliximab, prednisolone (P), mycophenolate sodium (MPS), and CsA. At 6 months, immunosuppression was tapered to P/CsA, P/MPS, or P/everolimus (EVL)...
September 17, 2016: American Journal of Transplantation
Sandra R Breyer, Nicole Muschol, Mona Schmidt, Martin Rupprecht, Kornelia Babin, Jochen Herrmann, Ralf Stücker
BACKGROUND: Hip dysplasia is common in mucopolysaccharidosis type-1H (MPS-1H) patients, but its morphology is not completely understood. No magnetic resonance imaging (MRI)-based studies have been reported in the literature. The purpose of this study was to improve knowledge of hip dysplasia pathology by describing the hip morphology of these patients in MRI scans, plain radiographs, and arthrograms. METHODS: We performed a retrospective chart review of 18 MPS-1H patients...
September 15, 2016: Journal of Pediatric Orthopedics
Taissia G Popova, Allison Teunis, Virginia Espina, Lance A Liotta, Serguei G Popov
In this study the hydrogel microparticles (MPs) were used to enhance migration of neutrophils in order to improve outcome of anthrax infection in a mouse model. Two MP formulations were tested. In the first one the polyacrylamide gel MPs were chemically coupled with Cibacron Blue (CB) affinity bait. In the second one the bait molecules within the MPs were additionally loaded with neutrophil-attracting chemokines (CKs), human CXCL8 and mouse CCL3. A non-covalent interaction of the bait with the CKs provided their gradual release after administration of the MPs to the host...
2016: PloS One
Liza Grosman-Rimon, William Parkinson, Suneel Upadhye, Hance Clarke, Joel Katz, John Flannery, Philip Peng, Dinesh Kumbhare
The aims of the present study were to compare levels of circulating inflammatory biomarkers and growth factors between patients with myofascial pain syndrome (MPS) and healthy control participants, and to assess the relationship among inflammatory markers and growth factors in the two groups.Biomarkers levels were assessed in patients (n = 37) with myofascial pain complaints recruited from the hospital emergency department and non-MPS controls (n = 21), recruited via advertisements in the hospital and community...
September 2016: Medicine (Baltimore)
L A Krueger, D C Beitz, S B Humphrey, J R Stabel
Peripheral blood mononuclear cells (PBMC) and mesenteric node lymphocytes (MNL) were obtained from 30 calves that were assigned randomly at birth to 1 of 6 treatment groups with 5 calves per treatment in a 14-d study: (1) colostrum-deprived (CD), no vitamins; (2) colostrum-replacer (CR), no vitamins; (3) CR, vitamin A; (4) CR, vitamin D3; (5) CR, vitamin E; (6) CR, vitamins A, D3, E. Calves were injected with appropriate vitamin supplements and fed pasteurized whole milk (CD calves) or fractionated colostrum replacer (CR calves) at birth...
September 7, 2016: Journal of Dairy Science
Liushiqi Borjigin, Tomoyuki Shimazu, Yuki Katayama, Kouichi Watanabe, Haruki Kitazawa, Sang-Gun Roh, Hisashi Aso, Kazuo Katoh, Masahiro Satoh, Yoshihito Suda, Akiko Sakuma, Mituru Nakajo, Keiichi Suzuki
To clarify the genetic influence of mycoplasmal pneumonia of swine (MPS) lesion-selected Landrace (La) on MPS resistance and immune characteristics in three-way crossbred pigs (LaWaDa), the LaWaDa pigs were compared with the non-selected crossbred (LbWbDb) and purebred (La) pigs. The MPS lesion score in the three lines was as follows: La line < LaWaDa line < LbWbDb line, with significant differences among the lines. The proportions of myeloid cells and T cells were lower and higher, respectively, in the LaWaDa pigs compared with those in the other two lines...
September 9, 2016: Animal Science Journal, Nihon Chikusan Gakkaihō
Christoph Kampmann, Tariq Abu-Tair, Seyfullah Gökce, Christina Lampe, Jörg Reinke, Eugen Mengel, Julia B Hennermann, Christiane M Wiethoff
BACKGROUND: Mucopolysaccharidosis (MPS) IVA is a rare lysosomal storage disorder with multiple skeletal and non-skeletal abnormalities requiring multiple surgical interventions. It is well known that patients with MPS IVA suffer from tachycardia, but cardiac and hemodynamic alterations have not been reported to date. We investigated the cardiovascular and hemodynamic alterations in patients with MPS IVA and developed a possible patho-mechanism for cardiovascular deterioration during anesthesia...
2016: PloS One
K V Truxal, H Fu, D M McCarty, K A McNally, K L Kunkler, N A Zumberge, L Martin, S C Aylward, L N Alfano, K M Berry, L P Lowes, M Corridore, C McKee, K L McBride, K M Flanigan
Mucopolysaccharidosis type III is a group of four autosomal recessive enzyme deficiencies leading to tissue accumulation of heparan sulfate. Central nervous system disease is prominent, with initial normal development followed by neurocognitive decline leading to death. In order to define outcome measures suitable for gene transfer trials, we prospectively assessed disease progression in MPS IIIA and IIIB subjects >2years old at three time points over one year (baseline, 6 and 12months). Fifteen IIIA (9 male, 6 female; age 5...
August 18, 2016: Molecular Genetics and Metabolism
Julia B Hennermann, Seyfullah Gökce, Alexander Solyom, Eugen Mengel, Edward H Schuchman, Calogera M Simonaro
Current treatment options for MPS I have limited effects on some organs, including the skeletal system. In MPS animal models pentosan polysulphate (PPS) reduces the concentrations of glycosaminoglycans (GAGs) in tissues and body fluids and improves cartilaginous and osseous pathologies. The goals of this study were to investigate primarily the safety and secondary the clinical effects, concerning mobility and pain, of PPS treatment in MPS I patients. Four MPS I-Hurler-Scheie/-Scheie patients aged 35.6 ± 6...
November 2016: Journal of Inherited Metabolic Disease
John P Greenwood, David P Ripley, Colin Berry, Gerry P McCann, Sven Plein, Chiara Bucciarelli-Ducci, Erica Dall'Armellina, Abhiram Prasad, Petra Bijsterveld, James R Foley, Kenneth Mangion, Mark Sculpher, Simon Walker, Colin C Everett, David A Cairns, Linda D Sharples, Julia M Brown
IMPORTANCE: Among patients with suspected coronary heart disease (CHD), rates of invasive angiography are considered too high. OBJECTIVE: To test the hypothesis that among patients with suspected CHD, cardiovascular magnetic resonance (CMR)-guided care is superior to National Institute for Health and Care Excellence (NICE) guidelines-directed care and myocardial perfusion scintigraphy (MPS)-guided care in reducing unnecessary angiography. DESIGN, SETTING, AND PARTICIPANTS: Multicenter, 3-parallel group, randomized clinical trial using a pragmatic comparative effectiveness design...
September 13, 2016: JAMA: the Journal of the American Medical Association
G Ciancio, P Tryphonopoulos, J J Gaynor, G Guerra, J Sageshima, D Roth, L Chen, W Kupin, A Mattiazzi, L Tueros, S Flores, L Hanson, R H Powell, P Ruiz, R Vianna, G W Burke
BACKGROUND: Recent studies suggest that the combination of tacrolimus (TAC) and everolimus (EVL) could become a viable option for use as standard maintenance immunosuppression in non-highly sensitized kidney transplant recipients. METHODS: We conducted a single-center, open-label, randomized pilot trial comparing two maintenance immunosuppression regimens in non-highly sensitized, adult, primary kidney transplant recipients: (TAC/EVL, Group A) vs our standard maintenance regimen of TAC plus enteric-coated mycophenolate mofetil (TAC/EC-MPS, Group B)...
July 2016: Transplantation Proceedings
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