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Christina lampe

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
Christian J Hendriksz, Kenneth I Berger, Christina Lampe, Susanne G Kircher, Paul J Orchard, Rebecca Southall, Sarah Long, Stephen Sande, Jeffrey I Gold
The mucopolysaccharidoses (MPS) comprise a heterogeneous family of rare, genetic lysosomal storage disorders that result in severe morbidity and reduced life expectancy. Emerging treatments for several of these disorders have triggered the search for clinically relevant biomarkers and clinical markers associated with treatment efficacy in populations and individuals. However, biomedical measures do not tell the whole story when characterizing a complex chronic disorder such as MPS. Health-related quality of life (HRQoL) tools that utilize patient reported outcomes to address patient parameters such as symptoms (pain, fatigue, psychological health), functioning (activity and limitations), or quality of life, have been used to supplement traditional biomedical endpoints...
2016: Orphanet Journal of Rare Diseases
Wendy Wood, Jenna L Lampe, Christina A Logan, Amy R Metcalfe, Beth E Hoesly
BACKGROUND: There is a need for a conceptual practice model that explicates ecological complexities involved in using occupation to optimize the quality of life of institutionalized people with dementia. PURPOSE: This study aimed to prepare the Lived Environment Life Quality Model, a dementia-specific conceptual practice model of occupational therapy in institutional facilities, for publication and application to practice. METHOD: Interviews and focus groups with six expert occupational therapists were subjected to qualitative content analysis to confirm, disconfirm, and further develop the model...
July 18, 2016: Canadian Journal of Occupational Therapy. Revue Canadienne D'ergothérapie
Guirish A Solanki, Peter P Sun, Kenneth W Martin, Christian J Hendriksz, Christina Lampe, Nathalie Guffon, Annie Hung, Zlatko Sisic, Renée Shediac, Paul R Harmatz
OBJECTIVES: To gain insight into the frequency, age of onset, and management of cervical cord compression in mucopolysaccharidosis VI (MPS VI). METHODS: Cervical spine magnetic resonance imaging (MRI) data and/or cervical decompression surgery data collected between 30 June 2005 and 1 September 2015 were analyzed from subjects enrolled in the MPS VI Clinical Surveillance Program (CSP) ( NCT00214773), an ongoing multicenter, observational, retrospective and prospective registry...
August 2016: Molecular Genetics and Metabolism
Sandrine Morel, Christina Christoffersen, Lene N Axelsen, Fabrizio Montecucco, Viviane Rochemont, Miguel A Frias, Francois Mach, Richard W James, Christian C Naus, Marc Chanson, Paul D Lampe, Morten S Nielsen, Lars B Nielsen, Brenda R Kwak
AIM: Increasing evidence points to lipoprotein composition rather than reverse cholesterol transport in the cardioprotective properties of high-density lipoproteins (HDLs). HDL binding to receptors at the surface of cardiomyocytes activates signalling pathways promoting survival, but downstream targets are largely unknown. Here, we investigate the pathways by which the sphingosine-1-phosphate (S1P) constituent of HDL limits cell death induced by cardiac ischaemia-reperfusion (I/R). METHODS AND RESULTS: Apolipoprotein M (ApoM) transgenic (Apom-Tg) mice, in which plasma S1P is increased by 296%, and wild-type (WT) mice were subjected to in vivo I/R...
March 1, 2016: Cardiovascular Research
Taha Sachak, Michael A Arnold, Bita V Naini, Rondell P Graham, Sejal S Shah, Michael Cruise, Jason Y Park, Lindsey Clark, Laura Lamps, Wendy L Frankel, Nicole Theodoropoulos, Christina A Arnold
Neutropenic enterocolitis (NE) is a deadly ileocecal-based disease seen in patients with a recent history of chemotherapy. As histology is not included in the current diagnostic criteria, the pathologic features of NE are poorly understood. We undertook a multi-institutional study of NE, and report helpful clinical clues, such as immunosuppression (n=20/20), recent chemotherapy (n=17/18), neutropenia (n=16/18) gastrointestinal symptoms (n=19/19), abnormal imaging studies of the cecum/right colon (n=11/14), and positive microbiological studies (n=13/15)...
December 2015: American Journal of Surgical Pathology
Sandra Cristina Raimundo, Utku Avci, Christina Hopper, Sivakumar Pattathil, Michael G Hahn, Zoë A Popper
Land plant cell wall glycan epitopes are present in Fucus vesiculosus. RG-I/AG mAbs recognize distinct glycan epitopes in structurally different galactans, and 3-linked glucans are also present in the cell walls. Cell wall-directed monoclonal antibodies (mAbs) have given increased knowledge of fundamental land plant processes but are not extensively used to study seaweeds. We profiled the brown seaweed Fucus vesiculosus glycome employing 155 mAbs that recognize predominantly vascular plant cell wall glycan components...
February 2016: Planta
Joanna Wasielica-Poslednik, Christina Butsch, Christina Lampe, Heike Elflein, Julia Lamparter, Veronika Weyer, Susanne Pitz
AIMS: To investigate the feasibility and to compare three devices measuring intraocular pressure (IOP) in mucopolysaccharidosis patients (MPS): iCare rebound tonometer (RT), Perkins applanation tonometer (PAT) and ocular response analyzer (ORA). METHODS: MPS patients who underwent at least two examinations out of: RT, PAT and ORA at the same visit were identified and retrospectively analyzed in this study. RESULTS: 17 patients fulfilled the inclusion criterion...
2015: PloS One
Christina Bluemel, Andreas Cramer, Christoph Grossmann, Georg W Kajdi, Uwe Malzahn, Nora Lamp, Heinz-Jakob Langen, Jan Schmid, Andreas K Buck, Hanns-Jörg Grimminger, Ken Herrmann
PURPOSE: To prospectively evaluate the feasibility of 3-D radioguided occult lesion localization (iROLL) and to compare iROLL with wire-guided localization (WGL) in patients with early-stage breast cancer undergoing breast-conserving surgery and sentinel lymph node biopsy (SLNB). METHODS: WGL (standard procedure) and iROLL in combination with SLNB were performed in 31 women (mean age 65.1 ± 11.2 years) with early-stage breast cancer and clinically negative axillae...
October 2015: European Journal of Nuclear Medicine and Molecular Imaging
Maurizio Scarpa, Cinzia Maria Bellettato, Christina Lampe, David J Begley
Pharmacological research has always focused on developing new therapeutic strategies capable of modifying a disease's natural history and improving patients' quality of life. Despite recent advances within the fields of medicine and biology, some diseases still represent a major challenge for successful therapy. Neuronopathic lysosomal storage disorders, in particular, have high rates of morbidity and mortality and a devastating socio-economic effect. Many of the available therapies, such as enzyme replacement therapy, can reverse the natural history of the disease in peripheral organs but, unfortunately, are still unable to reach the central nervous system effectively because they cannot cross the blood-brain barrier that surrounds and protects the brain...
March 2015: Best Practice & Research. Clinical Endocrinology & Metabolism
Sabine M Schriefl, Christina Leydolt, Eva Stifter, Rupert Menapace
PURPOSE: To compare the incidence and intensity of posterior capsular opacification (PCO) and neodymium:yttrium-aluminium-garnet (Nd:YAG) capsulotomy rates between two microincision intra-ocular lenses (IOLs) 3 years after surgery. METHODS: Sixty-five patients randomly received a Y-60H IOL (HOYA Surgical Optics, Singapore) in one eye and a Micro AY IOL (PhysIOL, Liège, Belgium) in the contralateral eye during same-day bilateral cataract surgery. Eyes were examined 1 week, 20 months and 3 years after surgery...
June 2015: Acta Ophthalmologica
Roberto Giugliani, Christina Lampe, Nathalie Guffon, David Ketteridge, Elisa Leão-Teles, James E Wraith, Simon A Jones, Cheri Piscia-Nichols, Ping Lin, Adrian Quartel, Paul Harmatz
Mucopolysaccharidosis VI (MPS VI) is a clinically heterogeneous and progressive disorder with multiorgan manifestations caused by deficient N-acetylgalactosamine-4-sulfatase activity. A cross-sectional Survey Study in individuals (n = 121) affected with MPS VI was conducted between 2001 and 2002 to establish demographics, urinary glycosaminoglycan (GAG) levels, and clinical progression of disease. We conducted a Resurvey Study ( NCT01387854) to obtain 10-year follow-up data, including medical histories and clinical assessments (n = 59), and survival status over 12 years (n = 117)...
August 2014: American Journal of Medical Genetics. Part A
Julia K Götzl, Kohji Mori, Markus Damme, Katrin Fellerer, Sabina Tahirovic, Gernot Kleinberger, Jonathan Janssens, Julie van der Zee, Christina M Lang, Elisabeth Kremmer, Jean-Jacques Martin, Sebastiaan Engelborghs, Hans A Kretzschmar, Thomas Arzberger, Christine Van Broeckhoven, Christian Haass, Anja Capell
Heterozygous loss-of-function mutations in the progranulin (GRN) gene and the resulting reduction of GRN levels is a common genetic cause for frontotemporal lobar degeneration (FTLD) with accumulation of TAR DNA-binding protein (TDP)-43. Recently, it has been shown that a complete GRN deficiency due to a homozygous GRN loss-of-function mutation causes neuronal ceroid lipofuscinosis (NCL), a lysosomal storage disorder. These findings suggest that lysosomal dysfunction may also contribute to some extent to FTLD...
2014: Acta Neuropathologica
Christian J Hendriksz, Christine Lavery, Mahmut Coker, Sema Kalkan Ucar, Mohit Jain, Lisa Bell, Christina Lampe
BACKGROUND: Morquio A syndrome (or mucopolysaccharidosis IVa) is an ultra-rare multi-organ disease, resulting in significantly impaired functional capacity, mobility and quality of life (QoL). METHODS: This patient-reported outcomes survey evaluated the global burden of Morquio A among adults (≥18 years, N = 27) and children (7-17 years, N = 36), including the impact on mobility, QoL, pain and fatigue. QoL was assessed using the general Health-Related Quality of Life (HRQoL) questionnaire (the EuroQol [EQ]-5D-5L)...
2014: Orphanet Journal of Rare Diseases
Christina Lampe, Ann-Kathrin Bosserhoff, Barbara K Burton, Roberto Giugliani, Carolina F de Souza, Camila Bittar, Nicole Muschol, Rebecca Olson, Nancy J Mendelsohn
INTRODUCTION: No published clinical trial data are available to inform the use of enzyme replacement therapy (ERT) in patients with the severe (neuropathic) phenotype of mucopolysaccharidosis II (MPS II). Current guidelines recommend ERT administered intravenously be used on a trial basis in this population. AIMS/METHODS: A retrospective chart review was conducted at five international centers for this case series of 22 patients with neuropathic MPS II who received intravenous idursulfase 0...
September 2014: Journal of Inherited Metabolic Disease
Christina Lampe, Andrea Atherton, Barbara K Burton, Maria Descartes, Roberto Giugliani, Dafne D G Horovitz, Sandra O Kyosen, Tatiana S P C Magalhães, Ana Maria Martins, Nancy J Mendelsohn, Joseph Muenzer, Laurie D Smith
Mucopolysaccharidosis (MPS) II, or Hunter syndrome, is a lysosomal storage disease characterized by multi-systemic involvement and a progressive clinical course. Enzyme replacement therapy with idursulfase has been approved in more than 50 countries worldwide; however, safety and efficacy data from clinical studies are currently only available for patients 1.4 years of age and older. Sibling case studies of infants with MPS I, II, and VI who initiated ERT in the first weeks or months of life have reported no new safety concerns and a more favorable clinical course for the sibling treated in infancy than for the later-treated sibling...
2014: JIMD Reports
Christina M Scheel, Yitian Zhou, Raquel C Theodoro, Bethany Abrams, S Arunmozhi Balajee, Anastasia P Litvintseva
Improved methods for the detection of Histoplasma capsulatum are needed in regions with limited resources in which the organism is endemic, where delayed diagnosis of progressive disseminated histoplasmosis (PDH) results in high mortality rates. We have investigated the use of a loop-mediated isothermal amplification (LAMP) assay to facilitate rapid inexpensive molecular diagnosis of this disease. Primers for LAMP were designed to amplify the Hcp100 locus of H. capsulatum. The sensitivity and limit of detection were evaluated using DNA extracted from 91 clinical isolates of known geographic subspecies, while the assay specificity was determined using DNA extracted from 50 other fungi and Mycobacterium tuberculosis...
February 2014: Journal of Clinical Microbiology
Ralph S Lachman, Barbara K Burton, Lorne A Clarke, Scott Hoffinger, Shiro Ikegawa, Dong-Kyu Jin, Hiroki Kano, Ok-Hwa Kim, Christina Lampe, Nancy J Mendelsohn, Renée Shediac, Pranoot Tanpaiboon, Klane K White
OBJECTIVE: Mucopolysaccharidosis IVA (MPS IVA, or Morquio A syndrome) and VI (MPS VI, or Maroteaux-Lamy syndrome) are autosomal recessive lysosomal storage disorders. Skeletal abnormalities are common initial presenting symptoms and, when recognized early, may facilitate timely diagnosis and intervention, leading to improved patient outcomes. Patients with slowly progressing disease and nonclassic phenotypes can be particularly challenging to diagnose. The objective was to describe the radiographic features of patients with a delayed diagnosis of MPS IVA or VI...
March 2014: Skeletal Radiology
Christina Patlaka, Maria Norgård, Staffan Paulie, Annica Nordvall-Bodell, Pernilla Lång, Göran Andersson
Adipogenesis depends on growth factors controlling proliferation/differentiation of mesenchymal stem cells (MSCs). Membrane binding and endocytosis of growth factors are often coupled to receptor activation and downstream signaling leading to specific cellular responses. The novel adipokine tartrate-resistant acid phosphatase (TRAP) 5a exhibits a growth factor-like effect on MSCs and pre-adipocytes and induces hyperplastic obesity in vivo. However its molecular interaction with pre-adipocytes remains unknown...
March 2014: Biochimica et Biophysica Acta
Christina M Scheel, Yitian Zhou, Raquel C Theodoro, Bethany Abrams, S Arunmozhi Balajee, Anastasia P Litvintseva
Improved methods for detection of Histoplasma capsulatum are needed in endemic regions with limited resources, where delayed diagnosis of progressive disseminated histoplasmosis (PDH) results in high mortality. We have investigated the use of a loop-mediated isothermal amplification (LAMP) assay to facilitate rapid, inexpensive, molecular diagnosis of this disease. Primers for LAMP were designed to amplify the Hcp100 locus of H. capsulatum. Sensitivity and limit of detection were evaluated using DNA extracted from 91 clinical isolates of known geographic subspecies, while assay specificity was determined using DNA extracted from 50 other fungi and Mycobacterium tuberculosis...
November 27, 2013: Journal of Clinical Microbiology
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