Hydronephrosis alters cardiac ACE2 and Mas receptor expression in mice.

PubMed

Zhang, Yanling; Ma, Lulu; Wu, Junyan; Chen, Tingting

2015-06-01

Hydronephrosis is characterized by substantial loss of tubules and affects renin secretion in the kidney. However, whether alterations of angiotensin-converting enzyme (ACE), ACE2 and Mas receptor in the heart are observed in hydronephrosis is unknown. Thus, we assessed these components in hydronephrotic mice treated with AT1 receptor blockade and ACE inhibitor. Hydronephrosis was induced by left ureteral ligation in Balb/C mice except sham-operated animals. The levels of cardiac ACE, ACE2 and Mas receptor were measured after treatment of losartan or enalapril. Hydronephrosis led to an increase of ACE level and a decrease of ACE2 and Mas receptor in the heart. Losartan decreased cardiac ACE level, but ACE2 and Mas receptor levels significantly increased in hydronephrotic mice (p < 0.01). Enalapril increased ACE2 levels (p < 0.01), but did not affect Mas receptor in the heart. Plasma renin activity (PRA) and Ang II decreased in hydronephrotic mice, but significantly increased after treatment with losartan or enalapril. Hydronephrosis increased cardiac ACE and suppressed ACE2 and Mas receptor levels. AT1 blockade caused sustained activation of cardiac ACE2 and Mas receptor, but ACE inhibitor had the limitation of such activation of Mas receptor in hydronephrotic animals. © The Author(s) 2015.

Decomposition of adsorbed VX on activated carbons studied by {sup 31}P MAS NMR

DOE Office of Scientific and Technical Information (OSTI.GOV)

Ishay Columbus; Daniel Waysbort; Liora Shmueli

2006-06-15

The fate of the persistent OP nerve agent O-ethyl S-(2-(diisopropylamino)ethyl) methylphosphonothioate (VX) on granular activated carbons that are used for gas filtration was studied by means of 31P magic angle spinning (MAS) NMR spectroscopy. Four types of activated carbon were used, including coal-based BPL. VX as vapor or liquid was adsorbed on carbon granules, and MAS NMR spectra were recorded periodically. The results show that at least 90% of the adsorbed VX decomposes within 20 days or less to the nontoxic ethyl methylphosphonic acid (EMPA) and bis(S-2-diisopropylaminoethane) ((DES){sub 2}). Decomposition occurred irrespective of the phase from which VX was loaded,more » the presence of metal impregnation on the carbon surface, and the water content of the carbon. Theoretical and practical aspects of the degradation are discussed. 17 refs., 6 figs., 3 tabs.« less

FIRE_ACE_ER2_MAS

Atmospheric Science Data Center

2017-04-26

... Moderate Resolution Imaging Spectrometer (MODIS) Airborne Simulator (MAS) Data Project Title:  MAS ... IDL Code ER2 Flight Tracks Related Data:  MAS Additional Info:  ER-2 MODIS Airborne Simulator (MAS) Image Gallery:  ...

FIRE_AX_ER2_MAS

Atmospheric Science Data Center

2015-11-24

... Transition Experiment (ASTEX) ER-2 MODIS Airborne Simulator (MAS) Data Project Title:  MAS ... Temporal Resolution:  Each granule contains one flight track File Format:  HDF Tools:  ... Additional Info:  NASA ER-2 MODIS Airborne Simulator (MAS) SCAR-B Block:  SCAR-B ...

An activating G{sub s}{alpha} mutation is present in fibrous dysplasia of bone in the McCune-Albright syndrome

DOE Office of Scientific and Technical Information (OSTI.GOV)

Shenker, A.; Weinstein, L.S.; Spiegel, A.M.

1994-09-01

McCune-Albright syndrome (MAS) is a sporadic disease characterized by polyostotic fibrous dysplasia, cafe-au-lait spots, and multiple endocrinopathies. The etiology of fibrous dysplasia is unknown. Activating mutations of codon 201 in the gene encoding the {alpha}-subunit of G{sub s}, the G-protein that stimulates adenylyl cyclase, have been found in all affected MAS tissues that have been studied. Initial attempts to amplify DNA from decalcified paraffin-embedded bone specimens from frozen surgical bone specimens from five MAS patients using polymerase chain reaction and allele-specific oligonucleotide hybridization. Most of the cells in four specimens of dysplastic bone contained a heterozygous mutation encoding substitution ofmore » Arg{sup 201} of G{sub s}{alpha} with His, but the mutation was barely detectable in peripheral blood specimens from the patients. Only a small amount of mutant allele was detected in a specimen of normal cortical bone from the fifth patient, although this patients had a high proportion of mutation in other, affected tissues. The mosaic distribution of mutant alleles is consistent with an embryological somatic cell mutation of the G{sub s}{alpha} gene in MAS. The presence of an activating mutation of G{sub s}{alpha} in osteoblastic progenitor cells may cause them to exhibit increased proliferation and abnormal differentiation, thereby producing the lesions of fibrous dysplasia. 43 refs., 2 figs.« less

2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative.

PubMed

Ravelli, Angelo; Minoia, Francesca; Davì, Sergio; Horne, AnnaCarin; Bovis, Francesca; Pistorio, Angela; Aricò, Maurizio; Avcin, Tadej; Behrens, Edward M; De Benedetti, Fabrizio; Filipovic, Lisa; Grom, Alexei A; Henter, Jan-Inge; Ilowite, Norman T; Jordan, Michael B; Khubchandani, Raju; Kitoh, Toshiyuki; Lehmberg, Kai; Lovell, Daniel J; Miettunen, Paivi; Nichols, Kim E; Ozen, Seza; Pachlopnik Schmid, Jana; Ramanan, Athimalaipet V; Russo, Ricardo; Schneider, Rayfel; Sterba, Gary; Uziel, Yosef; Wallace, Carol; Wouters, Carine; Wulffraat, Nico; Demirkaya, Erkan; Brunner, Hermine I; Martini, Alberto; Ruperto, Nicolino; Cron, Randy Q

2016-03-01

To develop criteria for the classification of macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (JIA). A multistep process, based on a combination of expert consensus and analysis of real patient data, was conducted. A panel of 28 experts was first asked to classify 428 patient profiles as having or not having MAS, based on clinical and laboratory features at the time of disease onset. The 428 profiles comprised 161 patients with systemic JIA-associated MAS and 267 patients with a condition that could potentially be confused with MAS (active systemic JIA without evidence of MAS, or systemic infection). Next, the ability of candidate criteria to classify individual patients as having MAS or not having MAS was assessed by evaluating the agreement between the classification yielded using the criteria and the consensus classification of the experts. The final criteria were selected in a consensus conference. Experts achieved consensus on the classification of 391 of the 428 patient profiles (91.4%). A total of 982 candidate criteria were tested statistically. The 37 best-performing criteria and 8 criteria obtained from the literature were evaluated at the consensus conference. During the conference, 82% consensus among experts was reached on the final MAS classification criteria. In validation analyses, these criteria had a sensitivity of 0.73 and a specificity of 0.99. Agreement between the classification (MAS or not MAS) obtained using the criteria and the original diagnosis made by the treating physician was high (κ = 0.76). We have developed a set of classification criteria for MAS complicating systemic JIA and provided preliminary evidence of its validity. Use of these criteria will potentially improve understanding of MAS in systemic JIA and enhance efforts to discover effective therapies, by ensuring appropriate patient enrollment in studies. © 2015, American College of Rheumatology.

MAS2-8 radar and digital control unit

NASA Technical Reports Server (NTRS)

Oberg, J. M.; Ulaby, F. T.

1974-01-01

The design of the MAS 2-8 (2 to 8 GHz microwave-active spectrometer), a ground-based sensor system, is presented. A major modification in 1974 to the MAS 2-8, that of a control subsystem to automate the data-taking operation, is the prime focus. The digital control unit automatically changes all system parameters except FM rate and records the return signal on paper tape. The overall system operation and a detailed discussion of the design and operation of the digital control unit are presented.

Dental Perspectives in Fibrous Dysplasia and McCune-Albright Syndrome

PubMed Central

Akintoye, Sunday O.; Boyce, Alison M.; Collins, Michael T.

2013-01-01

McCune-Albright syndrome (MAS) is a rare multisystem disorder characterized by the triad of polyostotic fibrous dysplasia (PFD), endocrine disorders and café-au-lait skin pigmentation. Ninety percent of MAS patients have FD lesions in the craniofacial area, resulting in significant orofacial deformity, dental disorders, bone pain and compromised oral health. Maxillo-mandibular FD is also associated with dental developmental disorders, malocclusion, and high caries index. There is limited data on the outcomes of dental treatments in maxillo-mandibular FD/MAS patients, because clinicians and researchers have limited access to patients, and there are concerns that dental surgery may activate quiescent jaw FD lesions to grow aggressively. This report highlights current perspectives on dental management issues associated with maxillo-mandibular FD within the context of MAS. PMID:23953425

Reproductive disturbances in multiple neuroendocrine tumor syndromes.

PubMed

Lytras, Aristides; Tolis, George

2009-12-01

In the context of multiple neuroendocrine tumor syndromes, reproductive abnormalities may occur via a number of different mechanisms, such as hyperprolactinemia, increased GH/IGF-1 levels, hypogonadotropic hypogonadism, hypercortisolism, hyperandrogenism, hyperthyroidism, gonadotropin hypersecretion, as well as, tumorigenesis or functional disturbances in gonads or other reproductive organs. Precocious puberty and/or male feminization is a feature of McCune-Albright syndrome (MAS), neurofibromatosis type 1 (NF1), Carney complex (CNC), and Peutz-Jeghers syndrome (PJS), while sperm maturation and ovulation defects have been described in MAS and CNC. Although tumorigenesis of reproductive organs due to a multiple neuroendocrine tumor syndrome is very rare, certain lesions are characteristic and very unusual in the general population. Awareness leading to their recognition is important especially when other endocrine abnormalities coexist, as occasionally they may even be the first manifestation of a syndrome. Lesions such as certain types of ovarian cysts (MAS, CNC), pseudogynecomastia due to neurofibromas of the nipple-areola area (NF1), breast disease (CNC and Cowden disease (CD)), cysts and 'hypernephroid' tumors of the epididymis or bilateral papillary cystadenomas (mesosalpinx cysts) and endometrioid cystadenomas of the broad ligament (von Hippel-Lindau disease), testicular Sertoli calcifying tumors (CNC, PJS) monolateral or bilateral macroochidism and microlithiasis (MAS) may offer diagnostic clues. In addition, multiple neuroendocrine tumor syndromes may be complicated by reproductive malignancies including ovarian cancer in CNC, breast and endometrial cancer in CD, breast malignancies in NF1, and malignant sex-cord stromal tumors in PJS.

Fluid flow dynamics in MAS systems.

PubMed

Wilhelm, Dirk; Purea, Armin; Engelke, Frank

2015-08-01

The turbine system and the radial bearing of a high performance magic angle spinning (MAS) probe with 1.3mm-rotor diameter has been analyzed for spinning rates up to 67kHz. We focused mainly on the fluid flow properties of the MAS system. Therefore, computational fluid dynamics (CFD) simulations and fluid measurements of the turbine and the radial bearings have been performed. CFD simulation and measurement results of the 1.3mm-MAS rotor system show relatively low efficiency (about 25%) compared to standard turbo machines outside the realm of MAS. However, in particular, MAS turbines are mainly optimized for speed and stability instead of efficiency. We have compared MAS systems for rotor diameter of 1.3-7mm converted to dimensionless values with classical turbomachinery systems showing that the operation parameters (rotor diameter, inlet mass flow, spinning rate) are in the favorable range. This dimensionless analysis also supports radial turbines for low speed MAS probes and diagonal turbines for high speed MAS probes. Consequently, a change from Pelton type MAS turbines to diagonal turbines might be worth considering for high speed applications. CFD simulations of the radial bearings have been compared with basic theoretical values proposing considerably smaller frictional loss values. The discrepancies might be due to the simple linear flow profile employed for the theoretical model. Frictional losses generated inside the radial bearings result in undesired heat-up of the rotor. The rotor surface temperature distribution computed by CFD simulations show a large temperature gradient over the rotor. Copyright © 2015 Elsevier Inc. All rights reserved.

Fluid flow dynamics in MAS systems

NASA Astrophysics Data System (ADS)

Wilhelm, Dirk; Purea, Armin; Engelke, Frank

2015-08-01

The turbine system and the radial bearing of a high performance magic angle spinning (MAS) probe with 1.3 mm-rotor diameter has been analyzed for spinning rates up to 67 kHz. We focused mainly on the fluid flow properties of the MAS system. Therefore, computational fluid dynamics (CFD) simulations and fluid measurements of the turbine and the radial bearings have been performed. CFD simulation and measurement results of the 1.3 mm-MAS rotor system show relatively low efficiency (about 25%) compared to standard turbo machines outside the realm of MAS. However, in particular, MAS turbines are mainly optimized for speed and stability instead of efficiency. We have compared MAS systems for rotor diameter of 1.3-7 mm converted to dimensionless values with classical turbomachinery systems showing that the operation parameters (rotor diameter, inlet mass flow, spinning rate) are in the favorable range. This dimensionless analysis also supports radial turbines for low speed MAS probes and diagonal turbines for high speed MAS probes. Consequently, a change from Pelton type MAS turbines to diagonal turbines might be worth considering for high speed applications. CFD simulations of the radial bearings have been compared with basic theoretical values proposing considerably smaller frictional loss values. The discrepancies might be due to the simple linear flow profile employed for the theoretical model. Frictional losses generated inside the radial bearings result in undesired heat-up of the rotor. The rotor surface temperature distribution computed by CFD simulations show a large temperature gradient over the rotor.

Angiotensin (1-7) ameliorates the structural and biochemical alterations of ovariectomy-induced osteoporosis in rats via activation of ACE-2/Mas receptor axis.

PubMed

Abuohashish, Hatem M; Ahmed, Mohammed M; Sabry, Dina; Khattab, Mahmoud M; Al-Rejaie, Salim S

2017-05-23

The local and systemic renin angiotensin system (RAS) influences the skeletal system micro-structure and metabolism. Studies suggested angiotensin 1-7 (Ang(1-7)) as the beneficial RAS molecule via Mas receptor activation. This study examines the function of Ang(1-7) in bone micro-architecture and metabolism in an ovariectomized (OVX) rodent model of osteoporosis. OVX rats showed structural and bone metabolic degeneration in parallel with suppressed expressions of the angiotensin converting enzyme-2 (ACE-2)/Ang(1-7)/Mas components. The infusion of Ang(1-7) markedly alleviated the altered bone metabolism and significantly enhanced both trabecular (metaphyseal) and cortical (metaphyseal-diaphyseal) morphometry. Urinary and bones minerals were also improved in OVX rats by Ang(1-7). The infusion of the heptapeptide enhanced ACE-2/Mas receptor expressions, while down-regulated AngII, ACE, and AngII type-1 receptor (AT1R) in OVX animals. Moreover, Ang(1-7) markedly improved osteoprotegerin (OPG) and lowered receptor activator NF-κB ligand (RANKL) expressions. The defensive properties of Ang(1-7) on bone metabolism, structure and minerals were considerably eradicated after blockage of Mas receptor with A-779. Ang(1-7)-induced up-regulated ACE-2/Ang(1-7)/Mas cascade and OPG expressions were abolished and the expressions of ACE/AngII/AT1R and RANKL were provoked by A-779. These findings shows for the first time the novel valuable therapeutic role of Ang(1-7) on bone health and metabolism through the ACE-2/Mas cascade.

Sealed rotors for in situ high temperature high pressure MAS NMR

DOE PAGES

Hu, Jian Z.; Hu, Mary Y.; Zhao, Zhenchao; ...

2015-07-06

Magic angle spinning (MAS) nuclear magnetic resonance (NMR) investigations on heterogeneous samples containing solids, semi-solids, liquid and gases or a mixture of them under non-conventional conditions of a combined high pressure and high temperature, or cold temperature suffer from the unavailability of a perfectly sealed rotor. Here, we report the design of reusable and perfectly-sealed all-zircornia MAS rotors. The rotors are easy to use and are suitable for operation temperatures from below 0 to 250 °C and pressures up to 100 bar. As an example of potential applications we performed in situ MAS NMR investigations of AlPO₄-5 molecular sieve crystallization,more » a kinetic study of the cyclohexanol dehydration reaction using 13C MAS NMR, and an investigation of the metabolomics of intact biological tissue at low temperature using 1H HR-MAS NMR spectroscopy. The in situ MAS NMR experiments performed using the reported rotors allowed reproduction of the results from traditional batch reactions, while offering more detailed quantitative information at the molecular level, as demonstrated for the molecular sieve synthesis and activation energy measurements for cyclohexanol dehydration. The perfectly sealed rotor also shows promising application for metabolomics studies using 1H HR-MAS NMR.« less

Sealed rotors for in situ high temperature high pressure MAS NMR

DOE Office of Scientific and Technical Information (OSTI.GOV)

Hu, Jian Z.; Hu, Mary Y.; Zhao, Zhenchao

Magic angle spinning (MAS) nuclear magnetic resonance (NMR) investigations on heterogeneous samples containing solids, semi-solids, liquid and gases or a mixture of them under non-conventional conditions of a combined high pressure and high temperature, or cold temperature suffer from the unavailability of a perfectly sealed rotor. Here, we report the design of reusable and perfectly-sealed all-zircornia MAS rotors. The rotors are easy to use and are suitable for operation temperatures from below 0 to 250 °C and pressures up to 100 bar. As an example of potential applications we performed in situ MAS NMR investigations of AlPO₄-5 molecular sieve crystallization,more » a kinetic study of the cyclohexanol dehydration reaction using 13C MAS NMR, and an investigation of the metabolomics of intact biological tissue at low temperature using 1H HR-MAS NMR spectroscopy. The in situ MAS NMR experiments performed using the reported rotors allowed reproduction of the results from traditional batch reactions, while offering more detailed quantitative information at the molecular level, as demonstrated for the molecular sieve synthesis and activation energy measurements for cyclohexanol dehydration. The perfectly sealed rotor also shows promising application for metabolomics studies using 1H HR-MAS NMR.« less

2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative.

PubMed

Ravelli, Angelo; Minoia, Francesca; Davì, Sergio; Horne, AnnaCarin; Bovis, Francesca; Pistorio, Angela; Aricò, Maurizio; Avcin, Tadej; Behrens, Edward M; De Benedetti, Fabrizio; Filipovic, Lisa; Grom, Alexei A; Henter, Jan-Inge; Ilowite, Norman T; Jordan, Michael B; Khubchandani, Raju; Kitoh, Toshiyuki; Lehmberg, Kai; Lovell, Daniel J; Miettunen, Paivi; Nichols, Kim E; Ozen, Seza; Pachlopnik Schmid, Jana; Ramanan, Athimalaipet V; Russo, Ricardo; Schneider, Rayfel; Sterba, Gary; Uziel, Yosef; Wallace, Carol; Wouters, Carine; Wulffraat, Nico; Demirkaya, Erkan; Brunner, Hermine I; Martini, Alberto; Ruperto, Nicolino; Cron, Randy Q

2016-03-01

To develop criteria for the classification of macrophage activation syndrome (MAS) in patients with systemic juvenile idiopathic arthritis (JIA). A multistep process, based on a combination of expert consensus and analysis of real patient data, was conducted. A panel of 28 experts was first asked to classify 428 patient profiles as having or not having MAS, based on clinical and laboratory features at the time of disease onset. The 428 profiles comprised 161 patients with systemic JIA-associated MAS and 267 patients with a condition that could potentially be confused with MAS (active systemic JIA without evidence of MAS, or systemic infection). Next, the ability of candidate criteria to classify individual patients as having MAS or not having MAS was assessed by evaluating the agreement between the classification yielded using the criteria and the consensus classification of the experts. The final criteria were selected in a consensus conference. Experts achieved consensus on the classification of 391 of the 428 patient profiles (91.4%). A total of 982 candidate criteria were tested statistically. The 37 best-performing criteria and 8 criteria obtained from the literature were evaluated at the consensus conference. During the conference, 82% consensus among experts was reached on the final MAS classification criteria. In validation analyses, these criteria had a sensitivity of 0.73 and a specificity of 0.99. Agreement between the classification (MAS or not MAS) obtained using the criteria and the original diagnosis made by the treating physician was high (κ=0.76). We have developed a set of classification criteria for MAS complicating systemic JIA and provided preliminary evidence of its validity. Use of these criteria will potentially improve understanding of MAS in systemic JIA and enhance efforts to discover effective therapies, by ensuring appropriate patient enrollment in studies. Published by the BMJ Publishing Group Limited. For permission to use (where not

A Case of Atypical McCune-Albright Syndrome with Vaginal Bleeding

PubMed Central

Rostampour, Noushin; Hashemipour, Mahin; Kelishadi, Roya; Hovsepian, Silva; Hekmatnia, Ali

2011-01-01

Background McCune-Albright syndrome (MAS) is a rare non-inherited disorder characterized by the clinical triad of precocious puberty, cafe-au-lait skin lesions, and fibrous dysplasia of bone. Case Presentation We report a girl with MAS, presenting initially with vaginal bleeding at the age of 17 months. Ultrasonography revealed unilateral ovarian cysts and ureteral and ovarian enlargement. Considering the clinical and paraclinical findings, the patient diagnosed as a case of gonadotropin-independent precocious puberty was treated with medroxy-progestrone acetate (MPA) for three months. During the follow up, recurrent episodes of bleeding, ovarian activation and cyst formation, as well as breast size development were reported. At the age of 5.5 years, fibrous dysplasia was detected, which in coexistence with precocious puberty confirmed the diagnosis of MAS. The patient had no cafe-au-lait skin macles during follow up. Conclusion Considering that clinical manifestations of MAS appear later in the course of recurrent periods of ovarian activation and cyst formation, a careful clinical observation and follow up of patients is necessary and the diagnosis of MAS must be kept in mind in cases with gonadotropin-independent precocious puberty. PMID:23056821

Hepatobiliary and Pancreatic neoplasms in patients with McCune-Albright syndrome.

PubMed

Gaujoux, Sébastien; Salenave, Sylvie; Ronot, Maxime; Rangheard, Anne-Sophie; Cros, Jérôme; Belghiti, Jacques; Sauvanet, Alain; Ruszniewski, Philippe; Chanson, Philippe

2014-01-01

McCune-Albright syndrome (MAS), which includes polycystic fibrous dysplasia, precocious puberty, and café au lait spots, is a rare disorder caused by somatic activating mutations of the GNAS gene. GNAS mutations have also been implicated in various sporadic tumors, including hepatobiliary and pancreatic neoplasms. The aim of this study was to assess the prevalence of hepatobiliary and pancreatic neoplasms in patients with McCune-Albright syndrome. Nineteen patients diagnosed between 1995 and 2012 with MAS in a tertiary referral center for rare growth disorders were screened with dedicated gadolinium-enhanced magnetic resonance imaging for hepatobiliary and pancreatic neoplasms between June 2011 and December 2012. Six (32%) of the 19 screened patients were found to have hepatic, pancreatic, or biliary lesions, excluding liver hemangiomas, liver cysts, and focal nodular hyperplasia. This includes pancreatic ductal lesions observed in 4 patients, including numerous branch-duct intraductal papillary mucinous neoplasms in 3 patients. Biliary lesions were observed in 1 patient, with a large choledochal cyst also involving the left biliary branch. Finally, multiple inflammatory/telangiectatic hepatic adenomas were observed in 2 patients, including 1 with proven somatic GNAS mutation. We describe the first observation of syndromic intraductal papillary mucinous neoplasms and the new association between MAS and pancreatic neoplasms, namely intraductal papillary mucinous neoplasms of the pancreas but also rare hepatobiliary neoplasms including liver adenomas and choledochal cysts. These findings strongly suggest that somatic activating GNAS mutations, possibly through cAMP pathway disorders, are involved in the tumorigenesis of hepatobiliary and pancreatic tissues originating from the foregut endoderm and have led us to use a routine screening by dedicated magnetic resonance imaging including both pancreatobiliary and liver sequences in patients with MAS.

Cardiac ACE2/angiotensin 1–7/Mas receptor axis is activated in thyroid hormone-induced cardiac hypertrophy

PubMed Central

Diniz, Gabriela P.; Senger, Nathalia; Carneiro-Ramos, Marcela S.; Santos, Robson A. S.; Barreto-Chaves, Maria Luiza M.

2015-01-01

Objectives: Thyroid hormone (TH) promotes marked effects on the cardiovascular system, including the development of cardiac hypertrophy. Some studies have demonstrated that the renin–angiotensin system (RAS) is a key mediator of the cardiac growth in response to elevated TH levels. Although some of the main RAS components are changed in cardiac tissue on hyperthyroid state, the potential modulation of the counter regulatory components of the RAS, such as angiotensin-converting enzyme type 2 (ACE2), angiotensin 1–7 (Ang 1–7) levels and Mas receptor induced by hyperthyroidism is unknown. The aim of this study was to investigate the effect of hyperthyroidism on cardiac Ang 1–7, ACE2 and Mas receptor levels. Methods: Hyperthyroidism was induced in Wistar rats by daily intraperitoneal injections of T4 for 14 days. Results: Although plasma Ang 1–7 levels were unchanged by hyperthyroidism, cardiac Ang 1–7 levels were increased in TH-induced cardiac hypertrophy. ACE2 enzymatic activity was significantly increased in hearts from hyperthyroid animals, which may be contributing to the higher Ang 1–7 levels observed in the T4 group. Furthermore, elevated cardiac levels of Ang 1–7 levels were accompanied by increased Mas receptor protein levels. Conclusion: The counter-regulatory components of the RAS are activated in hyperthyroidism and may be contributing to modulate the cardiac hypertrophy in response to TH. PMID:26715125

Impact of endocrine hyperfunction and phosphate wasting on bone in McCune-Albright syndrome.

PubMed

Lala, R; Matarazzo, P; Andreo, M; Defilippi, C; de Sanctis, C

2002-01-01

Skin dysplasia, as café-au-lait spots, bone fibrous dysplasia and peripheral endocrinopathies are the main clinical features of McCune-Albright syndrome (MAS). This illness is due to activating mutations of the Gsalpha protein and is spread with a mosaic pattern in affected tissues that consist of intermixed areas of normal and mutated cells. Peripheral endocrine secretion, free of hypothalamic pituitary control, is the hallmark of the endocrine syndromes: precocious puberty, Cushing's syndrome, hyperthyroidism and gigantism/acromegaly. In addition, phosphate wasting as hyperphosphaturia is often present. The impact of hormonal hypersecretion and phosphate loss on the bones of patients with MAS is poorly understood both in normal and fibrous bone tissue. As hypercortisolism and hyperthyroidism increase bone resorption, hyperestrogenism and growth hormone hypersecretion stimulate bone growth and mineralization, and phosphate wasting reduces bone mineral content. All these actions can be exerted at varying times and degrees in a single patient on lesional and non-lesional bones. Sonographic evidence of multiple diffused hyperechogenic spots in the testes of patients with MAS do not seem to be related to alterations in calcium-phosphate metabolism but rather to zonal dysplasia/hyperplasia of testicular tissue.

Characterization of active phosphorus surface sites at synthetic carbonate-free fluorapatite using single-pulse 1H, 31P, and 31P CP MAS NMR.

PubMed

Jarlbring, Mathias; Sandström, Dan E; Antzutkin, Oleg N; Forsling, Willis

2006-05-09

The chemically active phosphorus surface sites defined as PO(x), PO(x)H, and PO(x)H2, where x = 1, 2, or 3, and the bulk phosphorus groups of PO4(3-) at synthetic carbonate-free fluorapatite (Ca5(PO4)3F) have been studied by means of single-pulse 1H,31P, and 31P CP MAS NMR. The changes in composition and relative amounts of each surface species are evaluated as a function of pH. By combining spectra from single-pulse 1H and 31P MAS NMR and data from 31P CP MAS NMR experiments at varying contact times in the range 0.2-3.0 ms, it has been possible to distinguish between resonance lines in the NMR spectra originating from active surface sites and bulk phosphorus groups and also to assign the peaks in the NMR spectra to the specific phosphorus species. In the 31P CP MAS NMR experiments, the spinning frequency was set to 4.2 kHz; in the single-pulse 1H MAS NMR experiments, the spinning frequency was 10 kHz. The 31P CP MAS NMR spectrum of fluorapatite at pH 5.9 showed one dominating resonance line at 2.9 ppm assigned to originate from PO4(3-) groups and two weaker shoulder peaks at 5.4 and 0.8 ppm which were assigned to the unprotonated PO(x) (PO, PO2-, and PO3(2-)) and protonated PO(x)H (PO2H and PO3H-) surface sites. At pH 12.7, the intensity of the peak representing unprotonated PO(x) surface sites has increased 1.7% relative to the bulk peak, while the intensity of the peaks of the protonated species PO(x)H have decreased 1.4% relative to the bulk peak. At pH 3.5, a resonance peak at -4.5 ppm has appeared in the 31P CP MAS NMR spectrum assigned to the surface species PO(x)H2 (PO3H2). The results from the 1H MAS and 31P CP MAS NMR measurements indicated that H+, OH-, and physisorbed H2O at the surface were released during the drying process at 200 degrees C.

Characterization of solid polymer dispersions of active pharmaceutical ingredients by 19F MAS NMR and factor analysis

NASA Astrophysics Data System (ADS)

Urbanova, Martina; Brus, Jiri; Sedenkova, Ivana; Policianova, Olivia; Kobera, Libor

In this contribution the ability of 19F MAS NMR spectroscopy to probe structural variability of poorly water-soluble drugs formulated as solid dispersions in polymer matrices is discussed. The application potentiality of the proposed approach is demonstrated on a moderately sized active pharmaceutical ingredient (API, Atorvastatin) exhibiting extensive polymorphism. In this respect, a range of model systems with the API incorporated in the matrix of polvinylpyrrolidone (PVP) was prepared. The extent of mixing of both components was determined by T1(1H) and T1ρ(1H) relaxation experiments, and it was found that the API forms nanosized domains. Subsequently it was found out that the polymer matrix induces two kinds of changes in 19F MAS NMR spectra. At first, this is a high-frequency shift reaching 2-3 ppm which is independent on molecular structure of the API and which results from the long-range polarization of the electron cloud around 19F nucleus induced by electrostatic fields of the polymer matrix. At second, this is broadening of the signals and formation of shoulders reflecting changes in molecular arrangement of the API. To avoid misleading in the interpretation of the recorded 19F MAS NMR spectra, because both the contributions act simultaneously, we applied chemometric approach based on multivariate analysis. It is demonstrated that factor analysis of the recorded spectra can separate both these spectral contributions, and the subtle structural differences in the molecular arrangement of the API in the nanosized domains can be traced. In this way 19F MAS NMR spectra of both pure APIs and APIs in solid dispersions can be directly compared. The proposed strategy thus provides a powerful tool for the analysis of new formulations of fluorinated pharmaceutical substances in polymer matrices.

Macrophage activation syndrome in children with systemic lupus erythematosus and children with juvenile idiopathic arthritis.

PubMed

Bennett, Tellen D; Fluchel, Mark; Hersh, Aimee O; Hayward, Kristen N; Hersh, Adam L; Brogan, Thomas V; Srivastava, Rajendu; Stone, Bryan L; Korgenski, E Kent; Mundorff, Michael B; Casper, T Charles; Bratton, Susan L

2012-12-01

To describe patient demographics, interventions, and outcomes in hospitalized children with macrophage activation syndrome (MAS) complicating systemic lupus erythematosus (SLE) or juvenile idiopathic arthritis (JIA). We performed a retrospective cohort study using data recorded in the Pediatric Health Information System (PHIS) database from October 1, 2006 to September 30, 2010. Participants had International Classification of Diseases, Ninth Revision, Clinical Modification diagnosis codes for MAS and either SLE or JIA. The primary outcome was hospital mortality (for the index admission). Secondary outcomes included intensive care unit (ICU) admission, critical care interventions, and medication use. A total of 121 children at 28 children's hospitals met the inclusion criteria, including 19 children with SLE and 102 children with JIA. The index admission mortality rate was 7% (8 of 121 patients). ICU admission (33%), mechanical ventilation (26%), and inotrope/vasopressor therapy (26%) were common. Compared to children with JIA, those with SLE had a similar mortality rate (6% versus 11%, respectively; exact P = 0.6). More patients with SLE than those with JIA received ICU care (63% versus 27%; P = 0.002), received mechanical ventilation (53% versus 21%; P = 0.003), and had cardiovascular dysfunction (47% versus 23% received inotrope/vasopressor therapy; P = 0.02). Children with SLE and those with JIA received cyclosporine at similar rates, but more children with SLE received cyclophosphamide and mycophenolate mofetil, and more children with JIA received interleukin-1 antagonists. Organ system dysfunction is common in children with rheumatic diseases complicated by MAS, and more organ system support is required in children with underlying SLE than in children with JIA. Current treatment of pediatric MAS varies based on the underlying rheumatic disease. Copyright © 2012 by the American College of Rheumatology.

Chicken parvovirus and its associations with malabsorption syndrome.

PubMed

Finkler, F; Lima, D A; Cerva, C; Moraes, L B; Cibulski, S P; Teixeira, T F; Santos, H F; Almeida, L L; Roehe, P M; Franco, A C

2016-08-01

Malabsorption syndrome (MAS) is a multifactorial syndrome which is characterized by enteric disorders and reduced growth rates of broilers. Such condition is responsible for significant economic losses to the poultry industry. A possible association between chicken parvovirus (ChPV) infections and the occurrence of MAS has been proposed. However, such association has not to date been elucidated in view that ChPV has been detected in healthy as well as in MAS-affected chickens. This study aimed to detect and quantify ChPV loads in sera and tissues of MAS-affected, as well as in healthy broilers. Fifty nine, 39-day-old broilers (50 diseased, 9 healthy birds), obtained from the same flocks, were examined. The highest ChPV DNA loads were detected in MAS-affected broilers, particularly in fecal samples and intestinal tissues (~5500 genomic copies/300ng of total DNA). The average viral genome load in serum in MAS-affected birds was 1134copies/mL, whereas no viral DNA was found in sera and thymus tissues from healthy animals. These findings reveal that MAS-affected broilers consistently carry ChPV DNA is serum, whereas healthy animals do not. In addition, viral loads in tissues (bursa of Fabricius, spleen, intestine and liver) of MAS-affected birds were significantly higher in comparison to the same tissues from healthy broilers. Although preliminary, the results obtained here indicate an association between the detection of ChPV DNA in serum, in addition to high ChPV viral loads in tissues, and the occurrence of MAS in broilers. Further experiments should be performed to confirm such results. Copyright © 2016. Published by Elsevier Ltd.

Genetic and clinical features of cryopyrin-associated periodic syndromes in Turkish children.

PubMed

Eroglu, Fehime Kara; Kasapcopur, Ozgür; Beşbaş, Nesrin; Ozaltin, Fatih; Bilginer, Yelda; Barut, Kenan; Mensa-Vilaro, Anna; Nakagawa, Kenji; Heike, Toshio; Nishikomori, Ryuta; Arostegui, Juan; Ozen, Seza

2016-01-01

The aim of this study was to present the genetic and clinical data of the largest cohort of Turkish cryopyrin-associated periodic syndromes (CAPS) patients. This is a two-centre descriptive study of Turkish children with clinical diagnosis of CAPS. NLRP3 analyses were performed by Sanger sequencing and by massively parallel sequencing. ASC dependent NF-κB activation and transfection-induced THP-1 cell death assays determined the functional consequences of the detected variants. Disease activity and response to anti interleukin 1 (anti-IL-1) treatment was also assessed. Heterozygous germline NLRP3 mutation was detected in 8 of 14 enrolled patients (57.1%). Two novel somatic mutations Y560H and G307D were found which induced both THP-1 cell death and ASC dependent NF-kB activation. With anti-IL-1 treatment the disease activity was improved in all patients except one. Except two patients with macrophage activation syndrome (MAS) attack, there were no serious adverse events requiring hospitalisation. CAPS should be considered in all patients with typical symptoms even if Sanger-based genetic analysis is negative, since a considerable number of patients have mosaicism. Treatment should be patient-tailored and MAS should be considered as a rare complication.

Cassini's Maneuver Automation Software (MAS) Process: How to Successfully Command 200 Navigation Maneuvers

NASA Technical Reports Server (NTRS)

Yang, Genevie Velarde; Mohr, David; Kirby, Charles E.

2008-01-01

To keep Cassini on its complex trajectory, more than 200 orbit trim maneuvers (OTMs) have been planned from July 2004 to July 2010. With only a few days between many of these OTMs, the operations process of planning and executing the necessary commands had to be automated. The resulting Maneuver Automation Software (MAS) process minimizes the workforce required for, and maximizes the efficiency of, the maneuver design and uplink activities. The MAS process is a well-organized and logically constructed interface between Cassini's Navigation (NAV), Spacecraft Operations (SCO), and Ground Software teams. Upon delivery of an orbit determination (OD) from NAV, the MAS process can generate a maneuver design and all related uplink and verification products within 30 minutes. To date, all 112 OTMs executed by the Cassini spacecraft have been successful. MAS was even used to successfully design and execute a maneuver while the spacecraft was in safe mode.

BOREAS Level-1B MAS Imagery At-sensor Radiance, Relative X and Y Coordinates

NASA Technical Reports Server (NTRS)

Strub, Richard; Strub, Richard; Newcomer, Jeffrey A.; Ungar, Stephen

2000-01-01

For BOReal Ecosystem-Atmosphere Study (BOREAS), the MODIS Airborne Simulator (MAS) images, along with the other remotely sensed data, were collected to provide spatially extensive information over the primary study areas. This information includes detailed land cover and biophysical parameter maps such as fraction of Photosynthetically Active Radiation (fPAR) and Leaf Area Index (LAI). Collection of the MAS images occurred over the study areas during the 1994 field campaigns. The level-1b MAS data cover the dates of 21-Jul-1994, 24-Jul-1994, 04-Aug-1994, and 08-Aug-1994. The data are not geographically/geometrically corrected; however, files of relative X and Y coordinates for each image pixel were derived by using the C-130 INS data in a MAS scan model. The data are provided in binary image format files.

A Vision in Jeopardy: Royal Navy Maritime Autonomous Systems (MAS)

DTIC Science & Technology

2017-03-31

Chapter 6 will propose a new MAS vision for the RN. However, before doing so, a fresh look at the problem is required. Consensus of the Problem, Not the... assessment , the RN has failed to deliver any sustainable MAS operational capability. A vision for MAS finally materialized in 2014. Yet, the vision...continuous investment and assessment , the RN has failed to deliver any sustainable MAS operational capability. A vision for MAS finally materialized in

MAS NMR of HIV-1 protein assemblies

NASA Astrophysics Data System (ADS)

Suiter, Christopher L.; Quinn, Caitlin M.; Lu, Manman; Hou, Guangjin; Zhang, Huilan; Polenova, Tatyana

2015-04-01

The negative global impact of the AIDS pandemic is well known. In this perspective article, the utility of magic angle spinning (MAS) NMR spectroscopy to answer pressing questions related to the structure and dynamics of HIV-1 protein assemblies is examined. In recent years, MAS NMR has undergone major technological developments enabling studies of large viral assemblies. We discuss some of these evolving methods and technologies and provide a perspective on the current state of MAS NMR as applied to the investigations into structure and dynamics of HIV-1 assemblies of CA capsid protein and of Gag maturation intermediates.

Whole Exome Sequencing Reveals a Monogenic Cause of Disease in ≈43% of 35 Families With Midaortic Syndrome.

PubMed

Warejko, Jillian K; Schueler, Markus; Vivante, Asaf; Tan, Weizhen; Daga, Ankana; Lawson, Jennifer A; Braun, Daniela A; Shril, Shirlee; Amann, Kassaundra; Somers, Michael J G; Rodig, Nancy M; Baum, Michelle A; Daouk, Ghaleb; Traum, Avram Z; Kim, Heung Bae; Vakili, Khashayar; Porras, Diego; Lock, James; Rivkin, Michael J; Chaudry, Gulraiz; Smoot, Leslie B; Singh, Michael N; Smith, Edward R; Mane, Shrikant M; Lifton, Richard P; Stein, Deborah R; Ferguson, Michael A; Hildebrandt, Friedhelm

2018-04-01

Midaortic syndrome (MAS) is a rare cause of severe childhood hypertension characterized by narrowing of the abdominal aorta in children and is associated with extensive vascular disease. It may occur as part of a genetic syndrome, such as neurofibromatosis, or as consequence of a pathological inflammatory disease. However, most cases are considered idiopathic. We hypothesized that in a high percentage of these patients, a monogenic cause of disease may be detected by evaluating whole exome sequencing data for mutations in 1 of 38 candidate genes previously described to cause vasculopathy. We studied a cohort of 36 individuals from 35 different families with MAS by exome sequencing. In 15 of 35 families (42.9%), we detected likely causal dominant mutations. In 15 of 35 (42.9%) families with MAS, whole exome sequencing revealed a mutation in one of the genes previously associated with vascular disease ( NF1 , JAG1 , ELN , GATA6 , and RNF213 ). Ten of the 15 mutations have not previously been reported. This is the first report of ELN , RNF213 , or GATA6 mutations in individuals with MAS. Mutations were detected in NF1 (6/15 families), JAG1 (4/15 families), ELN (3/15 families), and one family each for GATA6 and RNF213 Eight individuals had syndromic disease and 7 individuals had isolated MAS. Whole exome sequencing can provide conclusive molecular genetic diagnosis in a high fraction of individuals with syndromic or isolated MAS. Establishing an etiologic diagnosis may reveal genotype/phenotype correlations for MAS in the future and should, therefore, be performed routinely in MAS. © 2018 American Heart Association, Inc.

Acrogigantism and facial asymmetry: McCune-Albright syndrome.

PubMed

Subbiah, Sridhar; Palikhe, Gaurav; Bhadada, Sanjay Kumar; Mukherjee, Kanchan Kumar; Bhansali, Anil

2011-01-01

McCune-Albright syndrome (MAS) is characterized by a triad of poly/monoostotic fibrous dysplasia, café-au-lait macules and hyperfunctioning endocrinopathies. Association of MAS with GH excess is rare, and in most of the instances somatotropinoma has not been documented. Treatment of patients of MAS with acromegaly is difficult because of thickened calvarium and dysplastic skull bone. We report a 17-year-old girl, who presented with cranio-facial fibrous dysplasia, café-au-lait macules and also had acromegaly due to pituitary macroadenoma, and treated with gamma knife radiosurgery.

McCune-Albright syndrome: radiological and MR findings.

PubMed

Yongjing, G; Huawei, L; Zilai, P; Bei, D; Hao, J; Kemin, C

2001-01-01

McCune-Albright syndrome (MAS) is a non-inherited disorder due to the GNAS1 gene mutation. The syndrome is characterized with the triad of polyostotic fibrous dysplasia, pigmented skin lesions, endocrinopathy, and precocious puberty. We report the case of a 14-year-old boy, presenting with sclerotic type of polyostotic fibrous dysplasia. Radiological methods including plain X-ray film, MR and whole body bone scintigraphy suggested the diagnosis of MAS. MRI provided more directly perceived images and it was more sensitive in demonstrating the lesion: its shape, contents, especially the size of the affected region. Histopathological study and the identification of mutant gene finally confirmed the diagnostic result.

A case of atypical McCune-Albright syndrome requiring optic nerve decompression.

PubMed

Yavuzer, R; Khilnani, R; Jackson, I T; Audet, B

1999-10-01

McCune-Albright syndrome (MAS) is a disease of noninheritable, genetic origin defined by the triad of café-au-lait pigmentation of the skin, precocious puberty, and polyostotic fibrous dysplasia. This syndrome, which affects young girls primarily, has also been reported with other endocrinopathies, and rarely with acromegaly and hyperprolactinemia. The fibrous dysplasia in MAS is of the polyostotic type and, apart from the characteristic sites such as the proximal aspects of the femur and the pelvis, the craniofacial region is frequently involved. A male patient with MAS presented with juvenile gigantism, precocious puberty, pituitary adenoma-secreting growth hormone and prolactin, hypothalamic pituitary gonadal and thyroid dysfunction, and polyostotic fibrous dysplasia causing optic nerve compression. Visual deterioration and its surgical management are presented.

The MicroMAS CubeSat Mission

NASA Astrophysics Data System (ADS)

Cahoy, K.; Blackwell, W. J.; Allen, G.; Bury, M.; Efromson, R.; Galbraith, C.; Hancock, T.; Leslie, V.; Osaretin, I.; Retherford, L.; Scarito, M.; Shields, M.; Toher, D.; Wight, K.; Miller, D.; Marinan, A.; Paek, S.; Peters, E.; Schmidt, F. H.; Alvisio, B.; Wise, E.; Masterson, R.; Franzim Miranda, D.; Crail, C.; Kingsbury, R.; Souffrant, A.; Orrego, L.; Eslinger, G.; Nicholas, A.; Pong, C.

2012-12-01

The recently published Midterm Assessment of NASA's Implementation of the Decadal Survey finds that, "The nation's Earth observing system is beginning a rapid decline in capability as long-running missions end and key new missions are delayed, lost, or canceled. The projected loss of observing capability could have significant adverse consequences for science and society." In this presentation, we explore low-cost, mission-flexible, and rapidly deployable spaceborne sensors that can meet stringent performance requirements pervading the NASA Earth Science measurement programs, including especially the recommended NRC Decadal Survey missions. New technologies have enabled a novel approach toward this science observational goal, and in this paper we describe recent technology develop efforts to address the challenges above through the use of CubeSat radiometers. The Micro-sized Microwave Atmospheric Satellite (MicroMAS) is a 3U cubesat (30x10x10 cm, ~4kg) hosting a passive microwave spectrometer operating near the 118.75-GHz oxygen absorption line. The focus of the first MicroMAS mission (hereafter, MicroMAS-1) is to observe convective thunderstorms, tropical cyclones, and hurricanes from a near-equatorial orbit at approximately 500-km altitude. A MicroMAS flight unit is currently being developed in anticipation of a 2014 launch to be provided by NASA. A parabolic reflector is mechanically rotated as the spacecraft orbits the earth, thus directing a cross-track scanned beam with FWHM beamwidth of 2.4-degrees, yielding an approximately 25-km diameter footprint from a nominal altitude of 500 km. Radiometric calibration is carried out using observations of cold space, the earth's limb, and an internal noise diode that is weakly coupled through the RF front-end electronics. A key technology feature is the development of an ultra-compact intermediate frequency processor module for channelization, detection, and A-to-D conversion. The antenna system and RF front

Chicken parvovirus viral loads in cloacal swabs from malabsorption syndrome-affected and healthy broilers.

PubMed

Finkler, Fabrine; de Lima, Diane Alves; Cerva, Cristine; Cibulski, Samuel Paulo; Teixeira, Thais Fumaco; Dos Santos, Helton Fernandes; de Almeida, Laura Lopes; Roehe, Paulo Michel; Franco, Ana Cláudia

2016-12-01

Chicken parvovirus (ChPV) has been associated with malabsorption syndrome (MAS) in broilers. However, the participation of this virus in such syndrome is unclear, since it may be detected in diseased and healthy chickens. In the course of these studies, it was argued whether ChPV genome loads might be correlated to the occurrence of MAS. To check such a hypothesis, a SYBR green-based quantitative polymerase chain reaction was developed to detect and quantify ChPV genomes. Cloacal swabs from 68 broilers with MAS and 59 from healthy animals were collected from different poultry farms. Genomes of ChPV were detected in all samples, regardless of their health status. However, viral genome loads in MAS-affected broilers were significantly higher (1 × 10 5 genome copies per 100 ng DNA) than in healthy animals (1.3 × 10 3 GC/100 ng DNA). These findings indicate that there is an association between high ChPV genome loads and the occurrence of MAS in broilers.

Advances in the Management of Meconium Aspiration Syndrome

PubMed Central

Swarnam, Kamala; Soraisham, Amuchou S.; Sivanandan, Sindhu

2012-01-01

Meconium aspiration syndrome (MAS) is a common cause of severe respiratory distress in term infants, with an associated highly variable morbidity and mortality. MAS results from aspiration of meconium during intrauterine gasping or during the first few breaths. The pathophysiology of MAS is multifactorial and includes acute airway obstruction, surfactant dysfunction or inactivation, chemical pneumonitis with release of vasoconstrictive and inflammatory mediators, and persistent pulmonary hypertension of newborn (PPHN). This disorder can be life threatening, often complicated by respiratory failure, pulmonary air leaks, and PPHN. Approaches to the prevention of MAS have changed over time with collaboration between obstetricians and pediatricians forming the foundations for care. The use of surfactant and inhaled nitric oxide (iNO) has led to the decreased mortality and the need for extracorporeal membrane oxygenation (ECMO) use. In this paper, we review the current understanding of the pathophysiology and management of MAS. PMID:22164183

Physical activity patterns and metabolic syndrome in Costa Rica

PubMed Central

Hastert, Theresa A.; Gong, Jian; Campos, Hannia; Baylin, Ana

2015-01-01

Objective To examine whether total physical activity or activity patterns are associated with metabolic syndrome and its components. Methods Participants include 1,994 controls from a case-control study of non-fatal myocardial infarction in Costa Rica (1994–2004). Physical activity was assessed via self-administered questionnaire and patterns were identified using principal components analysis. Metabolic syndrome was assessed via blood samples and anthropometry measurements from in-home study visits. Prevalence ratios (PR) and 95% confidence intervals (CI) were calculated using log binomial regression. Adjusted least squares means of metabolic syndrome components were calculated by quintile of total activity and pattern scores. Results Four activity patterns were identified: rest/sleep, agricultural, light indoor activity, and manual labor. Total activity was not associated with metabolic syndrome. Metabolic syndrome prevalence was 20% lower in participants with the highest scores on the agricultural job pattern compared to those with the lowest (PR: 0.80, 95% CI: 0.68–0.94). Higher total activity was associated with lower triglycerides and lower HDL cholesterol. Higher scores on each pattern were inversely associated with metabolic syndrome components, particularly waist circumference and fasting blood glucose. Conclusions Patterns or types of physical activity may be more strongly associated with metabolic syndrome and its components than total activity levels. PMID:25445330

McCune-Albright syndrome: a detailed pathological and genetic analysis of disease effects in an adult patient.

PubMed

Vasilev, Vladimir; Daly, Adrian F; Thiry, Albert; Petrossians, Patrick; Fina, Frederic; Rostomyan, Liliya; Silvy, Monique; Enjalbert, Alain; Barlier, Anne; Beckers, Albert

2014-10-01

McCune Albright syndrome (MAS) is a clinical association of endocrine and nonendocrine anomalies caused by postzygotic mutation of the GNAS1 gene, leading to somatic activation of the stimulatory α-subunit of G protein (Gsα). Important advances have been made recently in describing pathological characteristics of many MAS-affected tissues, particularly pituitary, testicular, and adrenal disease. Other rarer disease related features are emerging. The objective of the investigation was to study the pathological and genetic findings of MAS on a tissue-by-tissue basis in classically and nonclassically affected tissues. This was a comprehensive autopsy and genetic analysis. The study was conducted at a tertiary referral university hospital. An adult male patient with MAS and severe disease burden including gigantism was the subject of the study. Interventions included clinical, hormonal, and radiographic studies and gross and microscopic pathology analyses, conventional PCR, and droplet digital PCR analyses of affected and nonaffected tissues. Pathological findings and the presence of GNAS1 mutations were measured. The patient was diagnosed with MAS syndrome at 6 years of age based on the association of café-au-lait spots and radiological signs of polyostotic fibrous dysplasia. Gigantism developed and hyperprolactinemia, hypogonadotropic hypogonadism, and hyperparathyroidism were diagnosed throughout the adult period. The patient died at the age of 39 years from a pulmonary embolism. A detailed study revealed mosaiscism for the p.R201C GNAS1 mutation distributed across many endocrine and nonendocrine tissues. These genetically implicated tissues included rare or previously undescribed disease associations including primary hyperparathyroidism and hyperplasia of the thymus and endocrine pancreas. This comprehensive pathological study of a single patient highlights the complex clinical profile of MAS and illustrates important advances in understanding the

McCune-Albright syndrome.

PubMed

Dumitrescu, Claudia E; Collins, Michael T

2008-05-19

McCune-Albright syndrome (MAS) is classically defined by the clinical triad of fibrous dysplasia of bone (FD), café-au-lait skin spots, and precocious puberty (PP). It is a rare disease with estimated prevalence between 1/100,000 and 1/1,000,000. FD can involve a single or multiple skeletal sites and presents with a limp and/or pain, and, occasionally, a pathologic fracture. Scoliosis is common and may be progressive. In addition to PP (vaginal bleeding or spotting and development of breast tissue in girls, testicular and penile enlargement and precocious sexual behavior in boys), other hyperfunctioning endocrinopathies may be involved including hyperthyroidism, growth hormone excess, Cushing syndrome, and renal phosphate wasting. Café-au-lait spots usually appear in the neonatal period, but it is most often PP or FD that brings the child to medical attention. Renal involvement is seen in approximately 50% of the patients with MAS. The disease results from somatic mutations of the GNAS gene, specifically mutations in the cAMP regulating protein, Gs alpha. The extent of the disease is determined by the proliferation, migration and survival of the cell in which the mutation spontaneously occurs during embryonic development. Diagnosis of MAS is usually established on clinical grounds. Plain radiographs are often sufficient to make the diagnosis of FD and biopsy of FD lesions can confirm the diagnosis. The evaluation of patients with MAS should be guided by knowledge of the spectrum of tissues that may be involved, with specific testing for each. Genetic testing is possible, but is not routinely available. Genetic counseling, however, should be offered. Differential diagnoses include neurofibromatosis, osteofibrous dysplasia, non-ossifying fibromas, idiopathic central precocious puberty, and ovarian neoplasm. Treatment is dictated by the tissues affected, and the extent to which they are affected. Generally, some form of surgical intervention is recommended

McCune-Albright syndrome

PubMed Central

Dumitrescu, Claudia E; Collins, Michael T

2008-01-01

McCune-Albright syndrome (MAS) is classically defined by the clinical triad of fibrous dysplasia of bone (FD), café-au-lait skin spots, and precocious puberty (PP). It is a rare disease with estimated prevalence between 1/100,000 and 1/1,000,000. FD can involve a single or multiple skeletal sites and presents with a limp and/or pain, and, occasionally, a pathologic fracture. Scoliosis is common and may be progressive. In addition to PP (vaginal bleeding or spotting and development of breast tissue in girls, testicular and penile enlargement and precocious sexual behavior in boys), other hyperfunctioning endocrinopathies may be involved including hyperthyroidism, growth hormone excess, Cushing syndrome, and renal phosphate wasting. Café-au-lait spots usually appear in the neonatal period, but it is most often PP or FD that brings the child to medical attention. Renal involvement is seen in approximately 50% of the patients with MAS. The disease results from somatic mutations of the GNAS gene, specifically mutations in the cAMP regulating protein, Gs alpha. The extent of the disease is determined by the proliferation, migration and survival of the cell in which the mutation spontaneously occurs during embryonic development. Diagnosis of MAS is usually established on clinical grounds. Plain radiographs are often sufficient to make the diagnosis of FD and biopsy of FD lesions can confirm the diagnosis. The evaluation of patients with MAS should be guided by knowledge of the spectrum of tissues that may be involved, with specific testing for each. Genetic testing is possible, but is not routinely available. Genetic counseling, however, should be offered. Differential diagnoses include neurofibromatosis, osteofibrous dysplasia, non-ossifying fibromas, idiopathic central precocious puberty, and ovarian neoplasm. Treatment is dictated by the tissues affected, and the extent to which they are affected. Generally, some form of surgical intervention is recommended

Novel and rare functional genomic variants in multiple autoimmune syndrome and Sjögren's syndrome.

PubMed

Johar, Angad S; Mastronardi, Claudio; Rojas-Villarraga, Adriana; Patel, Hardip R; Chuah, Aaron; Peng, Kaiman; Higgins, Angela; Milburn, Peter; Palmer, Stephanie; Silva-Lara, Maria Fernanda; Velez, Jorge I; Andrews, Dan; Field, Matthew; Huttley, Gavin; Goodnow, Chris; Anaya, Juan-Manuel; Arcos-Burgos, Mauricio

2015-06-02

Multiple autoimmune syndrome (MAS), an extreme phenotype of autoimmune disorders, is a very well suited trait to tackle genomic variants of these conditions. Whole exome sequencing (WES) is a widely used strategy for detection of protein coding and splicing variants associated with inherited diseases. The DNA of eight patients affected by MAS [all of whom presenting with Sjögren's syndrome (SS)], four patients affected by SS alone and 38 unaffected individuals, were subject to WES. Filters to identify novel and rare functional (pathogenic-deleterious) homozygous and/or compound heterozygous variants in these patients and controls were applied. Bioinformatics tools such as the Human gene connectome as well as pathway and network analysis were applied to test overrepresentation of genes harbouring these variants in critical pathways and networks involved in autoimmunity. Eleven novel and rare functional variants were identified in cases but not in controls, harboured in: MACF1, KIAA0754, DUSP12, ICA1, CELA1, LRP1/STAT6, GRIN3B, ANKLE1, TMEM161A, and FKRP. These were subsequently subject to network analysis and their functional relatedness to genes already associated with autoimmunity was evaluated. Notably, the LRP1/STAT6 novel mutation was homozygous in one MAS affected patient and heterozygous in another. LRP1/STAT6 disclosed the strongest plausibility for autoimmunity. LRP1/STAT6 are involved in extracellular and intracellular anti-inflammatory pathways that play key roles in maintaining the homeostasis of the immune system. Further; networks, pathways, and interaction analyses showed that LRP1 is functionally related to the HLA-B and IL10 genes and it has a substantial impact within immunological pathways and/or reaction to bacterial and other foreign proteins (phagocytosis, regulation of phospholipase A2 activity, negative regulation of apoptosis and response to lipopolysaccharides). Further, ICA1 and STAT6 were also closely related to AIRE and IRF5, two very

Differential Mechanisms of Activation of the Ang Peptide Receptors AT1, AT2, and MAS: Using In Silico Techniques to Differentiate the Three Receptors

PubMed Central

Prokop, Jeremy W.; Santos, Robson A. S.; Milsted, Amy

2013-01-01

The renin-angiotensin system is involved in multiple conditions ranging from cardiovascular disorders to cancer. Components of the pathway, including ACE, renin and angiotensin receptors are targets for disease treatment. This study addresses three receptors of the pathway: AT1, AT2, and MAS and how the receptors are similar and differ in activation by angiotensin peptides. Combining biochemical and amino acid variation data with multiple species sequence alignments, structural models, and docking site predictions allows for visualization of how angiotensin peptides may bind and activate the receptors; allowing identification of conserved and variant mechanisms in the receptors. MAS differs from AT1 favoring Ang-(1–7) and not Ang II binding, while AT2 recently has been suggested to preferentially bind Ang III. A new model of Ang peptide binding to AT1 and AT2 is proposed that correlates data from site directed mutagenesis and photolabled experiments that were previously considered conflicting. Ang II binds AT1 and AT2 through a conserved initial binding mode involving amino acids 111 (consensus 325) of AT1 (Asn) interacting with Tyr (4) of Ang II and 199 and 256 (consensus 512 and 621, a Lys and His respectively) interacting with Phe (8) of Ang II. In MAS these sites are not conserved, leading to differential binding and activation by Ang-(1–7). In both AT1 and AT2, the Ang II peptide may internalize through Phe (8) of Ang II propagating through the receptors’ conserved aromatic amino acids to the final photolabled positioning relative to either AT1 (amino acid 294, Asn, consensus 725) or AT2 (138, Leu, consensus 336). Understanding receptor activation provides valuable information for drug design and identification of other receptors that can potentially bind Ang peptides. PMID:23755216

Angiotensin-(1-7)/Mas axis modulates fear memory and extinction in mice.

PubMed

Lazaroni, Thiago Luiz do Nascimento; Bastos, Cristiane Perácio; Moraes, Márcio Flávio Dutra; Santos, Robson Souza; Pereira, Grace Schenatto

2016-01-01

Inappropriate defense-alerting reaction to fear is a common feature of neuropsychiatric diseases. Therefore, impairments in brain circuits, as well as in molecular pathways underlying the neurovegetative adjustments to fear may play an essential role on developing neuropsychiatric disorders. Here we tested the hypothesis that interfering with angiotensin-(1-7) [Ang-(1-7)]/Mas receptor axis homeostasis, which appears to be essential to arterial pressure control, would affect fear memory and extinction. Mas knockout (MasKO) mice, in FVB/N background, showed normal cued fear memory and extinction, but increased freezing in response to context. Next, as FVB/N has poor performance in contextual fear memory, we tested MasKO in mixed 129xC57BL/6 background. MasKO mice behaved similarly to wild-type (WT), but memory extinction was slower in contextual fear conditioning to a weak protocol (1CS/US). In addition, delayed extinction in MasKO mice was even more pronounced after a stronger protocol (3CS/US). We showed previously that Angiotensin II receptor AT1 antagonist, losantan, rescued object recognition memory deficit in MasKO mice. Here, losartan was also effective. Memory extinction was accelerated in MasKO mice after treatment with losartan. In conclusion, we showed for the first time that Ang-(1-7)/Mas axis may modulate fear memory extinction. Furthermore, we suggest MasKO mice as an animal model to study post-traumatic stress disorder (PTSD). Copyright © 2015 Elsevier Inc. All rights reserved.

[EPIDEMIOLOGY OF OVER-ACTIVE BLADDER (OAB) SYNDROME].

PubMed

Eshkoli, Tamar; Yohai, David; Laron, Elad; Weintraub, Adi Y

2016-11-01

An over-active bladder is a common disorder which influences women's health and quality of life. There is difficulty defining the exact prevalence of the disorder since there are various definitions in the literature. The ICS definition from 2002 on the over-active bladder (OAB) syndrome enables more uniformity, by declaring that OAB syndrome is a symptomatic syndrome defined as presence of urgency with or without urinary incontinence, mostly accompanied by frequency and nocturia. In this article we reviewed the current medical literature on the prevalence of the OAB syndrome by focusing on relevant crosssectional and longitudinal studies, the trend changes during life, co-morbidities, the influence of quality of life and the economic burden following the disorder and its treatment. The authors' hope is that elevating awareness of the OAB syndrome will help improve its diagnosis and treatment.

Surgical treatment of middle aortic syndrome with Takayasu arteritis or midaortic dysplastic syndrome.

PubMed

Kim, S M; Jung, I M; Han, A; Min, S-I; Lee, T; Ha, J; Kim, S J; Min, S-K

2015-08-01

Middle aortic syndrome (MAS) is a rare condition characterized by severe stenosis of the distal thoracic or abdominal aorta. The aims of this study are to define the anatomic characteristics of MAS and to review the various surgical methods and their outcomes in terms of long-term durability Ten adult patients were diagnosed with MAS caused by Takayasu arteritis (TA) or midaortic dysplastic syndrome and underwent surgical treatment between July 1992 and January 2013. The aortic lesions were mostly suprarenal (n = 7) and stenoses were commonly found in the celiac axis (n = 6), SMA (n = 7), and renal artery (n = 6). Indications for operation were uncontrolled hypertension in six patients and lower extremity claudication in four. Eight aortic bypasses, one supraceliac aortic interposition graft, and one bilateral aorto-renal bypass were performed. Adjunctive renal bypass with saphenous vein graft (n = 4) and IMA reimplantation (n = 2) were performed simultaneously. There was no post-operative mortality, and one complication of iliac dissection at the distal anastomosis site was detected and treated by stenting. Hypertension was cured or improved in five of the six patients, and lower extremity claudication improved in all of them. With a median follow up of 60 months (range, 12-263), all the aortic bypasses were patent and one adjunctive renal artery bypass graft with aortic bypass was occluded 29 months post-operatively. Aortic bypass for MAS is safe and shows excellent long-term durability. Considering the patients are relatively young with a long life expectancy, aggressive surgical treatment could be beneficial. Lifelong follow up to monitor complications and disease progression is necessary. Copyright © 2015 European Society for Vascular Surgery. Published by Elsevier Ltd. All rights reserved.

McCune-Albright syndrome: clinical picture and natural history in children and adolescents.

PubMed

Völkl, Thomas M K; Dörr, Helmuth G

2006-05-01

The classical triad of McCune-Albright syndrome (MAS) consists of polyostotic fibrous dysplasia (FD), skin hyperpigmentation (café-au-lait spots), and endocrine dysfunction, frequently seen in females as precocious puberty. Patients with MAS display mosaicism of activating somatic mutations of the alpha-subunit of Gs. Thus, the clinical presentation of each individual is dependent on the particular distribution of affected cells, causing a broad spectrum of endocrine and non-endocrine manifestations. Typical endocrinopathies are precocious puberty, hyperthyroidism, growth hormone excess, hyperprolactemia, and hypercortisolism. The onset of these manifestations is usually during infancy and childhood. Since specific treatment is required, the prognosis depends on the severity of each individual endocrine manifestation. Additionally, there are non-endocrine manifestations, such as fibrous dysplasia of bone (FD), renal phosphate wasting, and skin hyperpigmentation, i.e. café-au-lait spots. FD, mostly polyostotic, causes fractures needing surgical and orthopedic treatment. Since previous studies have suggested the overall prognosis of patients with McCune-Albright syndrome to be non-fatal, recent data have drawn our attention to non-endocrine affections, including hepatobiliary dysfunction and cardiac disease, which are probably an important risk factor for early death. In summary, the clinical picture in MAS is related to its mosaic nature, i.e. any cell, tissue and organ in any site of the body could be affected to varying degrees, ranging from one or two mild clinical signs with excellent long-term prognosis to a severe life-threatening multiorgan disease.

McCune-Albright syndrome: growth hormone and prolactin hypersecretion.

PubMed

Christoforidis, Athanasios; Maniadaki, Ilianna; Stanhope, Richard

2006-05-01

McCune-Albright syndrome (MAS) has a special interest for endocrinologists as its pathogenesis results in hypersecretion of hormones in peripheral endocrine tissues. This can be expressed as precocious puberty, mainly in girls, primary hyperthyroidism, growth hormone (GH) and/or prolactin excess, hyperparathyroidism and hypercortisolism. The incidence of GH excess among patients with MAS has been assessed as up to 21%. The pathogenesis of GH hypersecretion in MAS is not completely understood, whereas it seems to be different from the aetiology of acromegaly/gigantism in non-MAS patients. The clinical expression of GH excess can be masked because of precocious puberty or craniofacial fibrous dysplasia, indicating the necessity for screening. Medical treatment is usually the only option in MAS patients with GH excess, as transsphenoidal surgery is usually restricted due to massive thickening of the skull base, whereas radiotherapy is contraindicated due to probable higher predisposition to sarcomatous transformation. The use of bromocriptine, cabergoline and octreotide, or the combination of these, has shown variable results, whereas pegvisomant, a GH receptor antagonist, is a new promising option, although not yet used in patients with MAS.

Activity syndromes and metabolism in giant deep-sea isopods

NASA Astrophysics Data System (ADS)

Wilson, Alexander D. M.; Szekeres, Petra; Violich, Mackellar; Gutowsky, Lee F. G.; Eliason, Erika J.; Cooke, Steven J.

2017-03-01

Despite growing interest, the behavioural ecology of deep-sea organisms is largely unknown. Much of this scarcity in knowledge can be attributed to deepwater animals being secretive or comparatively 'rare', as well as technical difficulties associated with accessing such remote habitats. Here we tested whether two species of giant marine isopod (Bathynomus giganteus, Booralana tricarinata) captured from 653 to 875 m in the Caribbean Sea near Eleuthera, The Bahamas, exhibited an activity behavioural syndrome across two environmental contexts (presence/absence of food stimulus) and further whether this syndrome carried over consistently between sexes. We also measured routine metabolic rate and oxygen consumption in response to a food stimulus in B. giganteus to assess whether these variables are related to individual differences in personality. We found that both species show an activity syndrome across environmental contexts, but the underlying mechanistic basis of this syndrome, particularly in B. giganteus, is unclear. Contrary to our initial predictions, neither B. giganteus nor B. tricarinata showed any differences between mean expression of behavioural traits between sexes. Both sexes of B. tricarinata showed strong evidence of an activity syndrome underlying movement and foraging ecology, whereas only male B. giganteus showed evidence of an activity syndrome. Generally, individuals that were more active and bolder, in a standard open arena test were also more active when a food stimulus was present. Interestingly, individual differences in metabolism were not related to individual differences in behaviour based on present data. Our study provides the first measurements of behavioural syndromes and metabolism in giant deep-sea isopods.

High field 27Al MAS NMR and TPD studies of active sites in ethanol dehydration using thermally treated transitional aluminas as catalysts

DOE Office of Scientific and Technical Information (OSTI.GOV)

Hu, Jian Zhi; Xu, Suochang; Kwak, Ja Hun

High field quantitative 27Al MAS NMR and temperature programmed desorption (TPD) of ethanol are used to study the surface and phase transformation of gamma-Al2O3 during calcination in the temperature range of 500 to 1300 degrees C. Following ethanol adsorption, ethylene is generated during TPD with a desorption temperature > 200 degrees C. With increasing calcination temperature prior to TPD, the amount of ethylene produced decreases monotonically. Significantly, 27Al MAS NMR reveals that the amount of penta-coordinate Al3+ ions (Lewis acid sites) also decreases with increasing calcination temperature. In fact, a strong correlation between the amount of penta-coordinate Al3+ ions andmore » the amount of strongly adsorbed ethanol molecules (i.e., the ones that convert to ethylene during TPD) is obtained. This result indicates that the penta-coordinate aluminum sites are the catalytic active sites on alumina surfaces during ethanol dehydration reaction across the entire course of gamma- to alpha-Al2O3 phase transformations.« less

McCune-Albright syndrome in a discordant monozygotic twin.

PubMed

Peleg, Roni; Luba, Avizov; Eliakim, Alon; Israeli-Shani, Lilach; Manor, Esther; Birk, Ruth; Parvari, Ruti

2009-06-01

McCune-Albright syndrome is a sporadic disorder characterized by polystotic fibrous dysplasia, pigmented patches of skin, and endocrinological abnormalities. To compare the genetic characteristics of the GNAS1 gene in a monozygotic pair of twins, one of whom was diagnosed with MAS while the other had no indication of the syndrome. We performed a molecular analysis of the GNAS1 gene in DNA extracted from peripheral blood cells and quantification of mRNA extracted from lymphoblastoid cells from both twins by quantitative real-time polymerase chain reaction. Monozygosity of the twins was confirmed by typing them to four highly polymorphic microsatellites. Molecular analysis of the GNAS1 gene extracted from both twins did not reveal the cause of this discordance. It is possible that the exact molecular mechanism for the MAS discordance can only be determined by sampling affected tissues.

Physical Activity Patterns of Youth with Down Syndrome

ERIC Educational Resources Information Center

Esposito, Phil E.; MacDonald, Megan; Hornyak, Joseph E.; Ulrich, Dale A.

2012-01-01

The purpose of this study was to examine the physical activity patterns of children with Down syndrome. A cross-sectional approach and accelerometry were used to measure the time children with Down syndrome (N = 104) spent in sedentary, light, and moderate-to-vigorous physical activity. Results indicated that adolescents from ages 14 to 15 years…

Control of adipogenesis by the autocrine interplays between angiotensin 1-7/Mas receptor and angiotensin II/AT1 receptor signaling pathways.

PubMed

Than, Aung; Leow, Melvin Khee-Shing; Chen, Peng

2013-05-31

Angiotensin II (AngII), a peptide hormone released by adipocytes, can be catabolized by adipose angiotensin-converting enzyme 2 (ACE2) to form Ang(1-7). Co-expression of AngII receptors (AT1 and AT2) and Ang(1-7) receptors (Mas) in adipocytes implies the autocrine regulation of the local angiotensin system upon adipocyte functions, through yet unknown interactive mechanisms. In the present study, we reveal the adipogenic effects of Ang(1-7) through activation of Mas receptor and its subtle interplays with the antiadipogenic AngII-AT1 signaling pathways. Specifically, in human and 3T3-L1 preadipocytes, Ang(1-7)-Mas signaling promotes adipogenesis via activation of PI3K/Akt and inhibition of MAPK kinase/ERK pathways, and Ang(1-7)-Mas antagonizes the antiadipogenic effect of AngII-AT1 by inhibiting the AngII-AT1-triggered MAPK kinase/ERK pathway. The autocrine regulation of the AngII/AT1-ACE2-Ang(1-7)/Mas axis upon adipogenesis has also been revealed. This study suggests the importance of the local regulation of the delicately balanced angiotensin system upon adipogenesis and its potential as a novel therapeutic target for obesity and related metabolic disorders.

McCune-Albright syndrome associated with pituitary microadenoma: patient report.

PubMed

Chen, Chun-Jung; Liu, Jah-Yao; Cheng, Shin-Nan; Chang, Fung-Wei; Yuh, Yeong-Seng

2004-03-01

McCune-Albright syndrome (MAS) is a rare disorder characterized by the classic triad of precocious puberty, polyostotic fibrous dysplasia and café-au-lait spots. Additional endocrine abnormalities may also be present, including hyperthyroidism, growth hormone excess and hyperprolactinemia. The most commonly encountered endocrine dysfunction is gonadal hyperfunction. Gonadotropin-independent precocious puberty is typically the initial manifestation of MAS in girls. Ovarian cysts may be detected on pelvic ultrasound. Our patient was also found to have pituitary microadenoma, evidenced by dynamic magnetic resonance imaging.

Medial abrasion syndrome: a neglected cause of knee pain in middle and old age.

PubMed

Lyu, Shaw-Ruey; Lee, Ching-Chih; Hsu, Chia-Chen

2015-04-01

Knee pain is a prevailing health problem of middle and old age. Medial plica-related medial abrasion syndrome (MAS), although a well-known cause of knee pain in younger individuals, has rarely been investigated in older individuals. This prospective study was conducted to investigate the prevalence and clinical manifestations of this syndrome as a cause of knee pain in middle and old age. The outcomes of arthroscopic treatment for this syndrome were also evaluated.A total of 232 knees of 169 patients >40 years of age (41-82, median: 63 years old) suffering from chronic knee pain were analyzed. The clinical diagnosis, predisposing factors, presenting symptoms, and physical signs were investigated. The sensitivity and specificity of each parameter of the clinical presentation for the diagnosis of MAS were evaluated after confirmation by arthroscopy. For patients with MAS, the roentgenographic and arthroscopic manifestations were investigated, and arthroscopic medial release (AMR) was performed. The outcomes were evaluated by the changes in the pain domain of the Knee Society scoring system and by patient satisfaction. The prevalence of medial plica was 95%, and osteoarthritis (OA) was the most common clinical diagnosis. Symptoms of pain and crepitus in motion and local tenderness during physical examination were the most sensitive parameters for the diagnosis. A history of a single knee injury combined with local tenderness and a palpable band found during physical examination were the most specific parameters for the diagnosis. The majority of patients suffering from this syndrome were successfully treated using AMR, yielding a satisfaction rate of 85.5% after a minimum of 3 years.MAS is a common cause of knee pain in middle and old age and can be effectively treated by AMR. Its concomitance with OA warrants further investigation.

Thalamic Activation in the Kleine-Levin Syndrome

PubMed Central

Engström, Maria; Karlsson, Thomas; Landtblom, Anne-Marie

2014-01-01

Study Objectives: The objective of this study was to investigate if combined measures of activation in the thalamus and working memory capacity could guide the diagnosis of Kleine-Levin Syndrome (KLS). A second objective was to obtain more insight into the neurobiological causes of KLS. Design: Matched group and consecutive recruitment. Setting: University hospital neurology department and imaging center. Patients or Participants: Eighteen patients with KLS diagnosed according to the International Classification of Sleep Disorders and 26 healthy controls were included. Interventions: N/A. Measurements and Results: Working memory capacity was assessed by the listening span task. A version of this task (reading span) was presented to the participants during functional magnetic resonance imaging (fMRI). Activation in the thalamus was measured in a region of interest analysis. A combination of the working memory capacity and the thalamic activation measures resulted in 80% prediction accuracy, 81% sensitivity, and 78% specificity regarding the ability to separate KLS patients from healthy controls. The controls had an inverse relation between working memory capacity and thalamic activation; higher performing participants had lower thalamic activation (r = -0.41). KLS patients showed the opposite relationship; higher performing participants had a tendency to higher thalamic activation (r = -0.35). Conclusions: This study shows that functional neuroimaging of the thalamus combined with neuropsychological assessment of working memory function provides a means to guide diagnosis of Kleine-Levin Syndrome. Results in this study also indicate that imaging of brain function and evaluation of cognitive capacity can give insights into the neurobiological mechanisms of Kleine-Levin Syndrome. Citation: Engström M; Karlsson T; Landtblom AM. Thalamic activation in the Kleine-Levin Syndrome. SLEEP 2014;37(2):379-386. PMID:24497666

Quartz Crystal Temperature Sensor for MAS NMR

NASA Astrophysics Data System (ADS)

Simon, Gerald

1997-10-01

Quartz crystal temperature sensors (QCTS) were tested for the first time as wireless thermometers in NMR MAS rotors utilizing the NMR RF technique itself for exiting and receiving electro-mechanical quartz resonances. This new tool in MAS NMR has a high sensitivity, linearity, and precision. When compared to the frequently used calibration of the variable temperature in the NMR system by a solid state NMR chemical shift thermometer (CST), such as lead nitrate, QCTS shows a number of advantages. It is an inert thermometer in close contact with solid samples operating parallel to the NMR experiment. QCTS can be manufactured for any frequency to be near a NMR frequency of interest (typically 1 to 2 MHz below or above). Due to the strong response of the crystal, signal detection is possible without changing the tuning of the MAS probe. The NMR signal is not influenced due to the relative sharp crystal resonance, restricted excitation by finite pulses, high probeQvalues, and commonly used audio filters. The quadratic dependence of the temperature increase on spinning speed is the same for the QCTS and for the CST lead nitrate and is discussed in terms of frictional heat in accordance with the literature about lead nitrate and with the results of a simple rotor speed jump experiment with differently radial located lead nitrate in the rotor.

Risk factors differentiating mild/moderate from severe meconium aspiration syndrome in meconium-stained neonates

PubMed Central

Choi, Woneui; Jeong, Heejeong; Choi, Suk-Joo; Kim, Jung-Sun; Roh, Cheong-Rae; Kim, Jong-Hwa

2015-01-01

Objective The aim of this study was to compare the risk factors associated with mild/moderate meconium aspiration syndrome (MAS) with those associated with severe in meconium-stained term neonates. Methods Consecutive singleton term neonates (n=671) with meconium staining at birth from all deliveries (n=14,666) in our institution from January 2006 to December 2012 were included. Both maternal and neonatal variables were examined. Among the study population, for women who underwent the trial of labor (n=644), variables associated with labor were also examined. These variables were compared between the mild/moderate MAS group, the severe MAS group, and the MAS-absent group. Results MAS developed in 10.6% (71/671) of neonates with meconium staining at birth. Among the neonates with MAS, 81.7% had mild MAS, 5.6% had moderate MAS, and 12.7% had severe MAS. The presence of minimal variability was significantly increased in both the mild/moderate and the severe MAS groups. The frequencies of nulliparity, fetal tachycardia, and intrapartum fever were significantly increased in the mild/moderate MAS group, but not in the severe MAS group. While a longer duration of the second stage of labor was significantly associated with mild/moderate MAS, severe MAS was associated with a shorter duration of the second stage. Notably, low mean cord pH (7.165 [6.850-7.375]) was significantly associated with mild/moderate MAS, but not with severe MAS (7.220 [7.021-7.407]) compared with the absence of MAS (7.268 [7.265-7.271]). Conclusion Our data suggest the development of severe MAS is not simply a linear extension of the same risk factors driving mild/moderate MAS. PMID:25629015

Solvent signal suppression for high-resolution MAS-DNP

NASA Astrophysics Data System (ADS)

Lee, Daniel; Chaudhari, Sachin R.; De Paëpe, Gaël

2017-05-01

Dynamic nuclear polarization (DNP) has become a powerful tool to substantially increase the sensitivity of high-field magic angle spinning (MAS) solid-state NMR experiments. The addition of dissolved hyperpolarizing agents usually results in the presence of solvent signals that can overlap and obscure those of interest from the analyte. Here, two methods are proposed to suppress DNP solvent signals: a Forced Echo Dephasing experiment (FEDex) and TRAnsfer of Populations in DOuble Resonance Echo Dephasing (TRAPDORED) NMR. These methods reintroduce a heteronuclear dipolar interaction that is specific to the solvent, thereby forcing a dephasing of recoupled solvent spins and leaving acquired NMR spectra free of associated resonance overlap with the analyte. The potency of these methods is demonstrated on sample types common to MAS-DNP experiments, namely a frozen solution (of L-proline) and a powdered solid (progesterone), both containing deuterated glycerol as a DNP solvent. The proposed methods are efficient, simple to implement, compatible with other NMR experiments, and extendable past spectral editing for just DNP solvents. The sensitivity gains from MAS-DNP in conjunction with FEDex or TRAPDORED then permits rapid and uninterrupted sample analysis.

Whole-Exome Sequencing Reveals Mutations in Genes Linked to Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome in Fatal Cases of H1N1 Influenza.

PubMed

Schulert, Grant S; Zhang, Mingce; Fall, Ndate; Husami, Ammar; Kissell, Diane; Hanosh, Andrew; Zhang, Kejian; Davis, Kristina; Jentzen, Jeffrey M; Napolitano, Lena; Siddiqui, Javed; Smith, Lauren B; Harms, Paul W; Grom, Alexei A; Cron, Randy Q

2016-04-01

Severe H1N1 influenza can be lethal in otherwise healthy individuals and can have features of reactive hemophagocytic lymphohistiocytosis (HLH). HLH is associated with mutations in lymphocyte cytolytic pathway genes, which have not been previously explored in H1N1 influenza. Sixteen cases of fatal influenza A(H1N1) infection, 81% with histopathologic hemophagocytosis, were identified and analyzed for clinical and laboratory features of HLH, using modified HLH-2004 and macrophage activation syndrome (MAS) criteria. Fourteen specimens were subject to whole-exome sequencing. Sequence alignment and variant filtering detected HLH gene mutations and potential disease-causing variants. Cytolytic function of the PRF1 p.A91V mutation was tested in lentiviral-transduced NK-92 natural killer (NK) cells. Despite several lacking variables, cases of influenza A(H1N1) infection met 44% and 81% of modified HLH-2004 and MAS criteria, respectively. Five subjects (36%) carried one of 3 heterozygous LYST mutations, 2 of whom also possessed the p.A91V PRF1 mutation, which was shown to decrease NK cell cytolytic function. Several patients also carried rare variants in other genes previously observed in MAS. This cohort of fatal influenza A(H1N1) infections confirms the presence of hemophagocytosis and HLH pathology. Moreover, the high percentage of HLH gene mutations suggests they are risk factors for mortality among individuals with influenza A(H1N1) infection. © The Author 2015. Published by Oxford University Press for the Infectious Diseases Society of America. All rights reserved. For permissions, e-mail journals.permissions@oup.com.

Growth hormone deficiency in treated acromegaly and active Cushing's syndrome.

PubMed

Formenti, Anna Maria; Maffezzoni, Filippo; Doga, Mauro; Mazziotti, Gherardo; Giustina, Andrea

2017-02-01

Growth hormone deficiency (GHD) in adults is characterized by reduced quality of life and physical fitness, skeletal fragility, increased weight and cardiovascular risk. It may be found in (over-) treated acromegaly as well as in active Cushing's syndrome. Hypopituitarism may develop in patients after definitive treatment of acromegaly, although the exact prevalence of GHD in this population is still uncertain because of limited awareness, and scarce and conflicting data so far available. Because GHD associated with acromegaly and Cushing's syndrome may yield adverse consequences on similar target systems, the final outcomes of some complications of both acromegaly and Cushing's syndrome may be further affected by the occurrence of GHD. It is still largely unknown, however, whether GHD in patients with post-acromegaly or active Cushing's syndrome (e.g. pharmacologic glucocorticoid treatment) may benefit from GH replacement. We review the diagnostic, clinical and therapeutic aspects of GHD in adults treated for acromegaly and in those with active Cushing's syndrome. Copyright © 2017 Elsevier Ltd. All rights reserved.

Moderate MAS enhances local (1)H spin exchange and spin diffusion.

PubMed

Roos, Matthias; Micke, Peter; Saalwächter, Kay; Hempel, Günter

2015-11-01

Proton NMR spin-diffusion experiments are often combined with magic-angle spinning (MAS) to achieve higher spectral resolution of solid samples. Here we show that local proton spin diffusion can indeed become faster at low (<10 kHz) spinning rates as compared to static conditions. Spin diffusion under static conditions can thus be slower than the often referred value of 0.8 nm(2)/ms, which was determined using slow MAS (Clauss et al., 1993). The enhancement of spin diffusion by slow MAS relies on the modulation of the orientation-dependent dipolar couplings during sample rotation and goes along with transient level crossings in combination with dipolar truncation. The experimental finding and its explanation is supported by density matrix simulations, and also emphasizes the sensitivity of spin diffusion to the local coupling topology. The amplification of spin diffusion by slow MAS cannot be explained by any model based on independent spin pairs; at least three spins have to be considered. Copyright © 2015 Elsevier Inc. All rights reserved.

Mas' Making and Pedagogy: Imagined Possibilities

ERIC Educational Resources Information Center

Fournillier, Janice B.

2009-01-01

In this article I draw on an ethnographic case study that examined mas' makers' perceptions of the learning/teaching practices at work in the production of costumes for Trinidad and Tobago's annual Carnival celebrations. During the 2005 Carnival season I spent four months in the field, my country of birth, and collected data through participant…

Hyperspectral Microwave Atmospheric Sounder (HyMAS) Architecture and Design Accommodations

NASA Technical Reports Server (NTRS)

Hilliard, Lawrence; Racette, Paul; Blackwell, William; Galbraith, Christopher; Thompson, Erik

2013-01-01

The Hyperspectral Microwave Atmospheric Sounder (HyMAS) is being developed at Lincoln Laboratories and accommodated by the Goddard Space Flight Center for a flight opportunity on a NASA research aircraft. The term "hyperspectral microwave" is used to indicate an all-weather sounding that performs equivalent to hyperspectral infrared sounders in clear air with vertical resolution of approximately 1 km. Deploying the HyMAS equipped scanhead with the existing Conical Scanning Microwave Imaging Radiometer (CoSMIR) shortens the path to a flight demonstration. Hyperspectral microwave is achieved through the use of independent RF antennas that sample the volume of the Earth s atmosphere through various levels of frequencies, thereby producing a set of dense, spaced vertical weighting functions. The simulations proposed for HyMAS 118/183-GHz system should yield surface precipitation rate and water path retrievals for small hail, soft hail, or snow pellets, snow, rainwater, etc. with accuracies comparable to those of the Advanced Technology Microwave Sounder. Further improvements in retrieval methodology (for example, polarization exploitation) are expected. The CoSMIR instrument is a packaging concept re-used on HyMAS to ease the integration features of the scanhead. The HyMAS scanhead will include an ultra-compact Intermediate Frequency Processor (IFP) module that is mounted inside the door to improve thermal management. The IFP is fabricated with materials made of Low-Temperature Co-fired Ceramic (LTCC) technology integrated with detectors, amplifiers, A/D conversion and data aggregation. The IFP will put out 52 channels of 16 bit data comprised of 4-9 channel data streams for temperature profiles and 2-8 channel streams for water vapor. With the limited volume of the existing CoSMIR scanhead and new HyMAS front end components, the HyMAS team at Goddard began preliminary layout work inside the new drum. Importing and re-using models of the shell, the scan head computer

Hyperspectral Microwave Atmospheric Sounder (HyMAS) architecture and design accommodations

NASA Astrophysics Data System (ADS)

Hilliard, L.; Racette, P.; Blackwell, W.; Galbraith, C.; Thompson, E.

The Hyperspectral Microwave Atmospheric Sounder (HyMAS) is being developed at Lincoln Laboratories and accommodated by the Goddard Space Flight Center for a flight opportunity on a NASA research aircraft. The term “ hyperspectral microwave” is used to indicate an all-weather sounding that performs equivalent to hyperspectral infrared sounders in clear air with vertical resolution of approximately 1 km. Deploying the HyMAS equipped scanhead with the existing Conical Scanning Microwave Imaging Radiometer (CoSMIR) shortens the path to a flight demonstration. Hyperspectral microwave is achieved through the use of independent RF antennas that sample the volume of the Earth's atmosphere through various levels of frequencies, thereby producing a set of dense, spaced vertical weighting functions. The simulations proposed for HyMAS 118/183-GHz system should yield surface precipitation rate and water path retrievals for small hail, soft hail, or snow pellets, snow, rainwater, etc. with accuracies comparable to those of the Advanced Technology Microwave Sounder. Further improvements in retrieval methodology (for example, polarization exploitation) are expected. The CoSMIR instrument is a packaging concept re-used on HyMAS to ease the integration features of the scanhead. The HyMAS scanhead will include an ultra-compact Intermediate Frequency Processor (IFP) module that is mounted inside the door to improve thermal management. The IFP is fabricated with materials made of Low-Temperature Co-fired Ceramic (LTCC) technology integrated with detectors, amplifiers, A/D conversion and data aggregation. The IFP will put out 52 channels of 16 bit data comprised of 4 - 9 channel data streams for temperature profiles and 2-8 channel streams for water vapor. With the limited volume of the existing CoSMIR scanhead and new HyMAS front end components, the HyMAS team at Goddard began preliminary layout work inside the new drum. Importing and re-using models of the shell, the s- an head

BOREAS Level-2 MAS Surface Reflectance and Temperature Images in BSQ Format

NASA Technical Reports Server (NTRS)

Hall, Forrest G. (Editor); Newcomer, Jeffrey (Editor); Lobitz, Brad; Spanner, Michael; Strub, Richard; Lobitz, Brad

2000-01-01

The BOReal Ecosystem-Atmosphere Study (BOREAS) Staff Science Aircraft Data Acquisition Program focused on providing the research teams with the remotely sensed aircraft data products they needed to compare and spatially extend point results. The MODIS Airborne Simulator (MAS) images, along with other remotely sensed data, were collected to provide spatially extensive information over the primary study areas. This information includes biophysical parameter maps such as surface reflectance and temperature. Collection of the MAS images occurred over the study areas during the 1994 field campaigns. The level-2 MAS data cover the dates of 21-Jul-1994, 24-Jul-1994, 04-Aug-1994, and 08-Aug-1994. The data are not geographically/geometrically corrected; however, files of relative X and Y coordinates for each image pixel were derived by using the C130 navigation data in a MAS scan model. The data are provided in binary image format files.

Physical activity patterns of youth with Down syndrome.

PubMed

Esposito, Phil E; MacDonald, Megan; Hornyak, Joseph E; Ulrich, Dale A

2012-04-01

The purpose of this study was to examine the physical activity patterns of children with Down syndrome. A cross-sectional approach and accelerometry were used to measure the time children with Down syndrome (N = 104) spent in sedentary, light, and moderate-to-vigorous physical activity. Results indicated that adolescents from ages 14 to 15 years were the most sedentary and spent the least amount of time in light and moderate-to-vigorous physical activity. A general trend of decreasing physical activity as children increase in age was found. This trend is similar to that found among typically developing youth. Participants in this study were found to spend a majority of their day engaged in sedentary activities. Results indicate that most participants were not accumulating the recommended 60 minutes of moderate or vigorous physical activity.

U.S. Geological Survey mineral databases; MRDS and MAS/MILS

USGS Publications Warehouse

McFaul, E.J.; Mason, G.T.; Ferguson, W.B.; Lipin, B.R.

2000-01-01

These two CD-ROM's contain the latest version of the Mineral Resources Data System (MRDS) database and the Minerals Availability System/Minerals Industry Location System (MAS/MILS) database for coverage of North America and the world outside North America. The records in the MRDS database each contain almost 200 data fields describing metallic and nonmetallic mineral resources, deposits, and commodities. The records in the MAS/MILS database each contain almost 100 data fields describing mines and mineral processing plans.

The Individual, Joint, and Additive Interaction Associations of Aerobic-Based Physical Activity and Muscle Strengthening Activities on Metabolic Syndrome.

PubMed

Dankel, Scott J; Loenneke, Jeremy P; Loprinzi, Paul D

2016-12-01

Previous research has demonstrated that physical activity and muscle strengthening activities are independently and inversely associated with metabolic syndrome. Despite a number of studies examining the individual associations, only a few studies have examined the joint associations, and to our knowledge, no previous studies have examined the potential additive interaction of performing muscle strengthening activities and aerobic-based physical activity and their association with metabolic syndrome. Using data from the 2003 to 2006 National Health and Nutrition Examination Survey (NHANES), we computed three separate multivariable logistic regression models to examine the individual, combined, and additive interaction of meeting guidelines for accelerometer-assessed physical activity and self-reported muscle strengthening activities, and their association with metabolic syndrome. We found that individuals meeting physical activity and muscle strengthening activity guidelines, respectively, were at 61 and 25 % lower odds of having metabolic syndrome. Furthermore, individuals meeting both guidelines had the lowest odds of having metabolic syndrome (70 %), in part due to the additive interaction of performing both modes of exercise. In this national sample, accelerometer-assessed physical activity and muscle strengthening activities were synergistically associated with metabolic syndrome.

Identifying facilitators and barriers to physical activity for adults with Down syndrome.

PubMed

Mahy, J; Shields, N; Taylor, N F; Dodd, K J

2010-09-01

Adults with Down syndrome are typically sedentary, and many do not participate in the recommended levels of physical activity per week. The aim of this study was to identify the facilitators and barriers to physical activity for this group. Semi-structured interviews were conducted to elicit the views of adults with Down syndrome and their support people about what factors facilitate physical activity and what factors are barriers to activity. A sample of 18 participants (3 men, 15 women) was recruited through two agencies providing services for adults with disabilities; six participants were adults with Down syndrome and 12 participants were support people (four were parents of adults with Down syndrome and eight participants were employed by day programmes attended by the adults with Down syndrome). The interviews were recorded, transcribed verbatim and independently coded by two researchers. Three themes around facilitators to physical activity were identified: (1) support from others; (2) that the physical activity was fun or had an interesting purpose; and (3) routine and familiarity. Three themes around barriers were also identified: (1) lack of support; (2) not wanting to engage in physical activity; and (3) medical and physiological factors. The results suggest that support people play a key role, both as facilitators and barriers, in the participation by adults with Down syndrome in physical activity. Many of the barriers and facilitators of activity for adults with Down syndrome indentified are similar to those reported for adults without impairment. Our findings are also consistent with established theories in the field of health behaviour change.

A Quantum Approach to Multi-Agent Systems (MAS), Organizations, and Control

DTIC Science & Technology

2003-06-01

interdependent interactions between individuals represented approximately as vocal harmonic I resonators. Then the growth rate of an organization fits ...A quantum approach to multi-agent systems (MAS), organizations , and control W.F. Lawless Paine College 1235 15th Street Augusta, GA 30901...AND SUBTITLE A quantum approach to multi-agent systems (MAS), organizations , and control 5a. CONTRACT NUMBER 5b. GRANT NUMBER 5c. PROGRAM ELEMENT

Intrarenal Mas and AT1 receptors play a role in mediating the excretory actions of renal interstitial angiotensin-(1-7) infusion in anaesthetized rats.

PubMed

O'Neill, Julie; Healy, Vincent; Johns, Edward J

2017-12-01

What is the central question of this study? Dietary sodium manipulation alters the magnitude of angiotensin-(1-7) [Ang-(1-7)]-induced natriuresis. The present study sought to determine whether this was related to relative changes in the activity of intrarenal Mas and/or AT 1 receptors. What is the main finding and its importance? Angiotensin-(1-7)-induced diuresis and natriuresis is mediated by intrarenal Mas receptors. However, intrarenal AT 1 receptor blockade also had an inhibitory effect on Ang-(1-7)-induced natriuresis and diuresis. Thus, Ang-(1-7)-induced increases in sodium and water excretion are dependent upon functional Mas and AT 1 receptors. We investigated whether angiotensin-(1-7) [Ang-(1-7)]-induced renal haemodynamic and excretory actions were solely dependent upon intrarenal Mas receptor activation or required functional angiotensin II type 1 (AT 1 ) receptors. The renin-angiotensin system was enhanced in anaesthetized rats by prior manipulation of dietary sodium intake. Angiotensin-(1-7) and AT 1 and Mas receptor antagonists were infused into the kidney at the corticomedullary border. Mas receptor expression was measured in the kidney. Mean arterial pressure, urine flow and fractional sodium excretion were 93 ± 4 mmHg, 46.1 ± 15.7 μl min -1  kg -1 and 1.4 ± 0.3%, respectively, in the normal-sodium group and 91 ± 2 mmHg, 19.1 ± 3.3 μl min -1  kg -1 and 0.7 ± 0.2%, respectively, in the low-sodium group. Angiotensin-(1-7) infusion had no effect on mean arterial pressure in rats receiving a normal-sodium diet but decreased it by 4 ± 5% in rats receiving a low-sodium diet (P < 0.05). Interstitial Ang-(1-7) infusion increased urine flow twofold and fractional sodium excretion threefold (P < 0.05) in rats receiving a normal-sodium diet and to a greater extent, approximately three- and fourfold, respectively, in rats receiving the low-sodium diet (both P < 0.05). Angiotensin-(1-7)-induced increases in urine flow and

An update on renal involvement in hemophagocytic syndrome (macrophage activation syndrome).

PubMed

Esmaili, Haydarali; Mostafidi, Elmira; Mehramuz, Bahareh; Ardalan, Mohammadreza; Mohajel-Shoja, Mohammadali

2016-01-01

Hemophagocytic syndrome (HPS) is mainly characterized by massive infiltration of bone marrow by activated macrophages and often presents with pancytopenia. Thrombotic microangiopathy (TMA) is also present with thrombocytopenia and renal involvement. Both conditions could coexist with each other and complicate the condition. Directory of Open Access Journals (DOAJ), EMBASE, Google Scholar, PubMed, EBSCO, and Web of Science with keywords relevant to; Hemophagocytic syndrome, macrophage activation syndrome, interferon-gamma and thrombotic microangiopathy, have been searched. Viral infection, rheumatologic disease and malignancies are the main underlying causes for secondary HPS. calcineurin inhibitors and viral infections are also the main underlying causes of TMA in transplant recipients. In this review, we discussed a 39-year-old male who presented with pancytopenia and renal allograft dysfunction. With the diagnosis of HPS induced TMA his renal condition and pancytopenia improved after receiving intravenous immunoglobulin (IVIG) and plasmapheresis therapy. HPS is an increasingly recognized disorder in the realm of different medical specialties. Renal involvement complicates the clinical picture of the disease, and this condition even is more complex in renal transplant recipients. We should consider the possibility of HPS in any renal transplant recipient with pancytopenia and allograft dysfunction. The combination of HPS with TMA future increases the complexity of the situation.

An update on renal involvement in hemophagocytic syndrome (macrophage activation syndrome)

PubMed Central

Esmaili, Haydarali; Mostafidi, Elmira; Mehramuz, Bahareh; Ardalan, Mohammadreza; Mohajel-Shoja, Mohammadali

2016-01-01

Context: Hemophagocytic syndrome (HPS) is mainly characterized by massive infiltration of bone marrow by activated macrophages and often presents with pancytopenia. Thrombotic microangiopathy (TMA) is also present with thrombocytopenia and renal involvement. Both conditions could coexist with each other and complicate the condition. Evidence Acquisition: Directory of Open Access Journals (DOAJ), EMBASE, Google Scholar, PubMed, EBSCO, and Web of Science with keywords relevant to; Hemophagocytic syndrome, macrophage activation syndrome, interferon-gamma and thrombotic microangiopathy, have been searched. Results: Viral infection, rheumatologic disease and malignancies are the main underlying causes for secondary HPS. calcineurin inhibitors and viral infections are also the main underlying causes of TMA in transplant recipients. In this review, we discussed a 39-year-old male who presented with pancytopenia and renal allograft dysfunction. With the diagnosis of HPS induced TMA his renal condition and pancytopenia improved after receiving intravenous immunoglobulin (IVIG) and plasmapheresis therapy. Conclusions: HPS is an increasingly recognized disorder in the realm of different medical specialties. Renal involvement complicates the clinical picture of the disease, and this condition even is more complex in renal transplant recipients. We should consider the possibility of HPS in any renal transplant recipient with pancytopenia and allograft dysfunction. The combination of HPS with TMA future increases the complexity of the situation. PMID:27047804

Valsartan attenuates intimal hyperplasia in balloon-injured rat aortic arteries through modulating the angiotensin-converting enzyme 2-angiotensin-(1-7)-Mas receptor axis.

PubMed

Li, Yonghong; Cai, Shanglang; Wang, Qixin; Zhou, Jingwei; Hou, Bo; Yu, Haichu; Ge, Zhiming; Guan, Renyan; Liu, Xu

2016-05-15

The role of the Mas receptor in the activity of valsartan against intimal hyperplasia is unclear. Herein, we investigated the role of the angiotensin-converting enzyme 2 (ACE2)-angiotensin-(1-7)-Mas receptor axis on the activity of valsartan against intimal hyperplasiain balloon-injured rat aortic arteries. Wistar rats were randomized equally into the sham control group, injured group, and injured plus valsartan (20 mg/kg/d)-treated group. Valsartan significantly attenuated the vascular smooth muscle cell proliferation and intimal and medial thickening on days 14 and 28 after injury. The angiotensin-(1-7) levels as well as ACE2 and Mas receptor mRNA/protein expression were significantly decreased in the injured rats, compared to the uninjured rats; meanwhile, the angiotensin II level as well as the ACE and AT1 receptor mRNA/protein expression were increased (all P < 0.05 or < 0.01). Additionally, the p-ERK protein expression was increased (P < 0.01). Treatment with valsartan significantly increased the angiotensin-(1-7) levels as well as ACE2 and Mas receptor mRNA/protein expression but decreased the angiotensin II level, ACE and AT1 receptor mRNA/protein expression, as well as the p-ERK protein expression, compared to the injured group (all P < 0.05 or < 0.01). These results suggest that valsartan attenuates neointimal hyperplasiain balloon-injured rat aortic arteries through activation of the ACE2-angiotensin-(1-7)-Mas axis as well as inhibition of the ACE-angiotensin II-AT1 and p-ERK pathways. Copyright © 2016 Elsevier Inc. All rights reserved.

Renoprotective Effects of AVE0991, a Nonpeptide Mas Receptor Agonist, in Experimental Acute Renal Injury

PubMed Central

Barroso, Lívia Corrêa; Silveira, Kátia Daniela; Lima, Cristiano Xavier; Borges, Valdinéria; Bader, Michael; Rachid, Milene; Santos, Robson Augusto Souza; Souza, Danielle Gloria; Simões e Silva, Ana Cristina; Teixeira, Mauro Martins

2012-01-01

Renal ischemia and reperfusion (I/R) is the major cause of acute kidney injury in hospitalized patients. Mechanisms underlying reperfusion-associated injury include recruitment and activation of leukocytes and release of inflammatory mediators. In this study, we investigated the renal effects of acute administration of AVE0991, an agonist of Mas, the angiotensin-(1–7) receptor, the angiotensin-(1–7) receptor, in a murine model of renal I/R. Male C57BL/6 wild-type or Mas−/− mice were subjected to 30 min of bilateral ischemia and 24 h of reperfusion. Administration of AVE0991 promoted renoprotective effects, as seen by improvement of function, decreased tissue injury, prevention of local and remote leucocyte infiltration, and release of the chemokine, CXCL1. I/R injury was similar in WT and Mas−/− mice, suggesting that endogenous activation of this receptor does not control renal damage under baseline conditions. In conclusion, pharmacological interventions using Mas receptor agonists may represent a therapeutic opportunity for the treatment of renal I/R injury. PMID:22319645

Negative Association of Domestic Activity and Active Commuting with Metabolic Syndrome in a Chinese Population Aged 35-64 Years.

PubMed

Chen, Xiao Rong; Zhang, Jian; Ding, Gang Qiang; Dong, Zhong; Zhang, Xin Wei; Li, Jian Hong; Chen, Bo; Yan, Liu Xia; Mi, Sheng Quan; Zhao, Wen Hua

2015-07-01

To understand the associations of physical activity domains with metabolic syndrome among a middle-aged Chinese population. In all, 3326 professional adults aged 35-64 years from Beijing and Zhejiang province were recruited with a cluster random sampling method. The Global Physical Activity Questionnaire was modified, and the recommended Asia-Pacific cut-offs of waist circumstance were introduced into the criteria for metabolic syndrome from the Adult Treatment Panel III. A binary logistic regression model was applied to examine the association of all physical activity domains with the risk of the syndrome. Participants who engaged in domestic activity for ⋜1176 MET-min/week had a 41.6% less chance of having metabolic syndrome [odds ratio (OR), 0.584; 95% confidence interval (CI), 0.480-0.710] than those without this activity. In adjusted models, adults who actively commuted for ⋜33 MET-min/week but <528 MET-min/week had a 25% less chance of having the syndrome (OR, 0.750; 95% CI, 0.582-0.966) than those who did not. No interaction was detected between the two domains of activity and the syndrome. This study highlighted the independently negative association of traffic and house activity with the prevalence of the syndrome in this sample with a generally low level of moderate activity. Copyright © 2015 The Editorial Board of Biomedical and Environmental Sciences. Published by China CDC. All rights reserved.

Three-year-old child with middle aortic syndrome treated by endovascular stent implantation.

PubMed

Moszura, Tomasz; Goreczny, Sebastian; Dryzek, Pawel; Niwald, Marek

2013-04-01

Middle aortic syndrome (MAS) is an extremely rare anomaly and represents both a diagnostic and therapeutic challenge, particularly in young children. A case of a 3.5 year-old child with MAS and arterial hypertension is reported, where owing to the patient's young age and the length of the hypoplastic aortic segment, surgical correction with end-to-end anastomosis was not feasible. Instead of palliative bypass grafting between the thoracic and abdominal aorta, successful percutaneous balloon angioplasty and stenting of the lesion was performed with the assistance of three-dimensional rotational angiography.

Case report of a rarely seen long-segment middle aortic syndrome.

PubMed

Yakut, Kahraman; Erdoğan, İlkay

2017-03-01

Middle aortic syndrome (MAS) follows a course with distal thoracic and abdominal aorta stenosis. It is a rare disease that is usually diagnosed after the first decade of life. Clinical reflection of MAS is often in the form of hypertension and claudication in the lower extremities. Its etiology is unclear, but is known to be associated with congenital or acquired diseases. This pathology, which is accompanied by malignant hypertension, often does not respond to medical treatment. In patients with MAS, surgical treatment is first line recommendation to prevent complications such as hypertension, heart failure, intracranial bleeding, or aortic rupture. In order to draw attention to this disease, presently described is case of high blood pressure detected during routine examination of a child who had no complaint, and discovery of long-segment stenosis in the abdominal aorta identified with echocardiography and conventional angiography.

Descending aorta-external iliac artery bypass for middle aortic syndrome.

PubMed

Okamoto, Yuki; Yamamoto, Kazuo; Sugimoto, Tsutomu; Asami, Fuyuki; Nagasawa, Ayako; Shiraiwa, Satoru; Nakamura, Norihito; Yoshii, Shinpei

2014-11-01

We encountered a surgical case of middle aortic syndrome (MAS) in a 56-year-old man who had resistant hypertension. Computed tomography showed severe stenosis of the abdominal aorta from below the superior mesenteric artery to above the inferior mesenteric artery. Although bilateral renal artery stenosis was confirmed, renal function was within normal limits. A 10-mm vascular prosthetic graft was used to perform a descending aorta to left external iliac artery bypass. His hypertension was well controlled without medication. This extra-anatomic bypass may be a simple and useful approach for treating MAS if it is not necessary to reconstruct the renal artery or visceral artery.

77 FR 43084 - Multiple Award Schedule (MAS) Program Continuous Open Season-Operational Change

Federal Register 2010, 2011, 2012, 2013, 2014

2012-07-23

... Award Schedule (MAS) Program Continuous Open Season- Operational Change AGENCY: Federal Acquisition... proposing this operational change to enhance the performance of and modernize the MAS program in three key program areas: Small business viability, operational efficiency, and cost control. The DBM will realign...

[Middle aortic syndrome: a report of three pediatric cases].

PubMed

Martínez León, M I; Alcaide Martín, D; García-Herrera Taillefer, P; Ramos Rodríguez, R

2013-09-01

The middle aortic syndrome (MAS) is caused by stenosis of the abdominal aorta, often accompanied by concomitant stenosis of the renal or visceral arteries. Although MAS is uncommon (0.5-2%), it is an important cause of hypertension in children and adolescents. It is thought to originate in a failure of the two dorsal aortas to fuse during embryological development, and a high percentage of cases are idiopathic. MAS affects the segment of the aorta between the renal arteries in 54% of cases. Clinically, it courses with symptomatic or asymptomatic arterial hypertension. On physical examination, findings include an abdominal bruit, diminished or absent femoral pulses, and a difference between the arterial pressure of the upper and lower limbs. Angiography is the technique of choice, although noninvasive MR angiography and CT angiography have similar diagnostic accuracy. Ultrasonography is the primary screening technique. Medical treatment consists of a combination of different antihypertensives. Surgical treatment can be curative. Copyright © 2010 SERAM. Published by Elsevier Espana. All rights reserved.

Indirect detection of infinite-speed MAS solid-state NMR spectra

NASA Astrophysics Data System (ADS)

Perras, Frédéric A.; Venkatesh, Amrit; Hanrahan, Michael P.; Goh, Tian Wei; Huang, Wenyu; Rossini, Aaron J.; Pruski, Marek

2017-03-01

Heavy spin-1/2 nuclides are known to possess very large chemical shift anisotropies that can challenge even the most advanced magic-angle-spinning (MAS) techniques. Wide manifolds of overlapping spinning sidebands and insufficient excitation bandwidths often obfuscate meaningful spectral information and force the use of static, low-resolution solid-state (SS)NMR methods for the characterization of materials. To address these issues, we have merged fast-magic-angle-turning (MAT) and dipolar heteronuclear multiple-quantum coherence (D-HMQC) experiments to obtain D-HMQC-MAT pulse sequences which enable the rapid acquisition of 2D SSNMR spectra that correlate isotropic 1H chemical shifts to the indirectly detected isotropic "infinite-MAS" spectra of heavy spin-1/2 nuclides. For these nuclides, the combination of fast MAS and 1H detection provides a high sensitivity, which rivals the DNP-enhanced ultra-wideline SSNMR. The new pulse sequences were used to determine the Pt coordination environments in a complex mixture of decomposition products of transplatin and in a metal-organic framework with Pt ions coordinated to the linker ligands.

The megaaortic syndrome: Progression of ascending aortic aneurysm or a disease of distinct origin?

PubMed

Baranyi, Ulrike; Stern, Christian; Winter, Birgitta; Türkcan, Adrian; Scharinger, Bernhard; Stelzmüller, Marie-Elisabeth; Aschacher, Thomas; Andreas, Martin; Ehrlich, Marek; Laufer, Günther; Bernhard, David; Messner, Barbara

2017-01-15

Thoracic aortic aneurysm (TAA) is an often asymptomatic disease with fatal outcome, such as dissection or rupture. The megaaortic syndrome (MAS) is an extensive dilatation of the whole aorta with low incidence but high lethal outcome with unknown pathophysiology so far. We compared aortic tissue of patients with sporadic TAAs and MAS of the ascending aorta with non-aneurysmal control tissues. Specimens of MAS patients showed a significantly reduced thickness of the media but an increased thickness of the intima compared to control tissue and TAAs with moderate dilatation. Advanced media degeneration however was detectable in both, TAAs with enhanced luminal diameter and MAS specimens, accompanied by reduced medial smooth muscle cell-density. Further specimens of MAS were characterized by massive atherosclerotic lesions in contrast to specimens of sporadic TAA patients. Infiltrations of macrophages in atherosclerotic lesions but also in the media adjacent to the adventitia were significantly elevated in tissue of TAAs with dilatation ≤6cm. Of note, atherosclerotic plaque-associated macrophages as well as those in the external media produce huge amounts of MMP-9 which is possibly involved in media degeneration and tissue destruction. Taken together these results demonstrate that the pathology of MAS shows similarities with that of TAAs but pathological differences in the ascending aorta, suggesting that MAS might be a disease of different origin. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Biochemical and nutritional markers and antioxidant activity in metabolic syndrome.

PubMed

Bernabé García, Juana; Zafrilla Rentero, Pilar; Mulero Cánovas, Juana; Gómez Jara, Purificación; Leal Hernández, Mariano; Abellán Alemán, José

2014-01-01

1) Nutritional assessment of the diet followed by patients with metabolic syndrome, and 2) biochemical analysis of the oxidation-reduction level in patients with metabolic syndrome. A cross-sectional study was conducted in patients with metabolic syndrome in Murcia. Fifty-three patients, 33 with and 20 without (control group) metabolic syndrome, were selected. The intervention consisted of completion of a recall survey and a test to nutritionally assess dietary intake. Anthropometric and laboratory variables, including those related to antioxidant activity, were also tested. Antioxidant activity was within normal limits in both groups (1.7 ± 0.2 mmol/L in the control group and 1.8 ± 0.1 mmol/L in the metabolic syndrome group) (NS). Superoxide dismutase levels were not significantly different between the groups. Mean glutathione reductase levels (U/L) were higher in the control group as compared to patients with metabolic syndrome (P<.05). As regards oxidative stress biomarkers, mean isoprostane levels were higher in the control group (4.9 ± 6.2 ng/mL) than in metabolic syndrome patients (3.5 ± 3.9 ng/mL) (P<.05). Oxidized LDL values tended to be higher in metabolic syndrome patients (96 ± 23.2U/L) as compared to the control group (86.2 ± 17.3 U/L), but differences were not significant. There is a trend to a poorer nutritional and biochemical profile in patients with metabolic syndrome, who also tend to have a greater degree of oxidative stress. Copyright © 2013 SEEN. Published by Elsevier Espana. All rights reserved.

Familial Aggregation and Segregation Analysis in Families Presenting Autoimmunity, Polyautoimmunity, and Multiple Autoimmune Syndrome

PubMed Central

Castiblanco, John; Sarmiento-Monroy, Juan Camilo; Mantilla, Ruben Dario; Rojas-Villarraga, Adriana; Anaya, Juan-Manuel

2015-01-01

Studies documenting increased risk of developing autoimmune diseases (ADs) have shown that these conditions share several immunogenetic mechanisms (i.e., the autoimmune tautology). This report explored familial aggregation and segregation of AD, polyautoimmunity, and multiple autoimmune syndrome (MAS) in 210 families. Familial aggregation was examined for first-degree relatives. Segregation analysis was implemented as in S.A.G.E. release 6.3. Data showed differences between late- and early-onset families regarding their age, age of onset, and sex. Familial aggregation of AD in late- and early-onset families was observed. For polyautoimmunity as a trait, only aggregation was observed between sibling pairs in late-onset families. No aggregation was observed for MAS. Segregation analyses for AD suggested major gene(s) with no clear discernible classical known Mendelian transmission in late-onset families, while for polyautoimmunity and MAS no model was implied. Data suggest that polyautoimmunity and MAS are not independent traits and that gender, age, and age of onset are interrelated factors influencing autoimmunity. PMID:26697508

The Marfan Syndrome. Fact Sheet [and] Physical Education and Activity Guidelines.

ERIC Educational Resources Information Center

National Marfan Foundation, Port Washington, NY.

This document consists of two brochures, the first explaining the Marfan Syndrome and a second providing guidelines for physical education and activity for people who have this syndrome are provided. The brochure on factual information about Marfan syndrome outlines the associated medical problems involving the cardiovascular system, the skeleton,…

The renin-angiotensin system: a target of and contributor to dyslipidemias, altered glucose homeostasis, and hypertension of the metabolic syndrome

PubMed Central

Putnam, Kelly; Shoemaker, Robin; Yiannikouris, Frederique

2012-01-01

The renin-angiotensin system (RAS) is an important therapeutic target in the treatment of hypertension. Obesity has emerged as a primary contributor to essential hypertension in the United States and clusters with other metabolic disorders (hyperglycemia, hypertension, high triglycerides, low HDL cholesterol) defined within the metabolic syndrome. In addition to hypertension, RAS blockade may also serve as an effective treatment strategy to control impaired glucose and insulin tolerance and dyslipidemias in patients with the metabolic syndrome. Hyperglycemia, insulin resistance, and/or specific cholesterol metabolites have been demonstrated to activate components required for the synthesis [angiotensinogen, renin, angiotensin-converting enzyme (ACE)], degradation (ACE2), or responsiveness (angiotensin II type 1 receptors, Mas receptors) to angiotensin peptides in cell types (e.g., pancreatic islet cells, adipocytes, macrophages) that mediate specific disorders of the metabolic syndrome. An activated local RAS in these cell types may contribute to dysregulated function by promoting oxidative stress, apoptosis, and inflammation. This review will discuss data demonstrating the regulation of components of the RAS by cholesterol and its metabolites, glucose, and/or insulin in cell types implicated in disorders of the metabolic syndrome. In addition, we discuss data supporting a role for an activated local RAS in dyslipidemias and glucose intolerance/insulin resistance and the development of hypertension in the metabolic syndrome. Identification of an activated RAS as a common thread contributing to several disorders of the metabolic syndrome makes the use of angiotensin receptor blockers and ACE inhibitors an intriguing and novel option for multisymptom treatment. PMID:22227126

Physical Activity Engagement in Young People with Down Syndrome: Investigating Parental Beliefs.

PubMed

Alesi, Marianna; Pepi, Annamaria

2017-01-01

Despite the wide documentation of the physical/psychological benefits derived from regular physical activity (PA), high levels of inactivity are reported among people with Down syndrome. This study aims to explore parental beliefs concerning involvement, facilitators/barriers and benefits of PA in young people. Semi-structured interviews were conducted with 13 parents of young people with Down syndrome. Three facilitation themes were identified: (i) the support derived from family; (ii) the availability of APA (Adapted Physical Activity) expert instructors and coaches; (iii) the challenging nature of sport activities. Three barrier themes were identified: (i) the lack of APA expert coaches and specialized gyms; (ii) the characteristics of Down syndrome; and (iii) the parental beliefs and worries. Family plays a key role, as facilitator and barrier, to the participation of their children with Down syndrome in PA. Crucial is the implementation of evidence-based exercise programmes involving people with Down syndrome and their families. © 2015 John Wiley & Sons Ltd.

Association of Objectively Measured Physical Activity and Metabolic Syndrome Among US Adults With Osteoarthritis.

PubMed

Liu, Shao-Hsien; Waring, Molly E; Eaton, Charles B; Lapane, Kate L

2015-10-01

To investigate the association between objectively measured physical activity and metabolic syndrome among adults with osteoarthritis (OA). Using cross-sectional data from the 2003-2006 National Health and Nutrition Examination Survey, we identified 566 adults with OA with available accelerometer data assessed using Actigraph AM-7164 and measurements necessary to determine metabolic syndrome by the Adult Treatment Panel III. Analysis of variance was conducted to examine the association between continuous variables in each activity level and metabolic syndrome components. Logistic models estimated the relationship of quartile of daily minutes of different physical activity levels to odds of metabolic syndrome adjusted for socioeconomic and health factors. Among persons with OA, most were women average age of 62.1 years and average disease duration of 12.9 years. Half of adults with OA had metabolic syndrome (51.0%; 95% confidence interval [95% CI] 44.2%-57.8%), and only 9.6% engaged in the recommended 150 minutes per week of moderate/vigorous physical activity. Total sedentary time was associated with higher rates of metabolic syndrome and its components, while light and objectively measured moderate/vigorous physical activity was inversely associated with metabolic syndrome and its components. Higher levels of light activity were associated with lower prevalence of metabolic syndrome (quartile 4 versus quartile 1: adjusted odds ratio 0.45, 95% CI 0.24-0.84, P for linear trend < 0.005). Most US adults with OA are sedentary. Increased daily minutes in physical activity, especially in light intensity, is more likely to be associated with decreasing prevalence of metabolic syndrome among persons with OA. © 2015, American College of Rheumatology.

A case of McCune-Albright syndrome associated with pituitary GH adenoma: therapeutic process and autopsy.

PubMed

Liu, Fuyi; Li, Wenting; Yao, Yong; Li, Guilin; Yang, Yi; Dou, Wanchen; Zhong, Dingrong; Wang, Lin; Zhu, Xiangdong; Hu, Hua; Zhang, Jianmin; Wang, Renzhi; Chen, Gao

2011-01-01

McCune-Albright syndrome (MAS) is a clinical syndrome with low incidence, and its concurrence with pituitary GH adenoma is rare. Little of the history, treatment and outcome has been studied. Follow-up of a 37-year-old male patient of MAS associated with pituitary GH adenoma was performed continuously recording the disease development and the treatment process until death, after which an autopsy was performed. Radiation therapy (RT) efficaciously controlled GH hypersecretion, however, it may have been the cause of the malignant transformation of the dysplastic bone tissue, which eventually caused brain hernia and death; autopsy demonstrated that the cranium had significant thickening (as much as 10 cm), the pathological diagnosis was fibrous dysplasia of bone associated with chondrosarcoma; and undifferentiated chondrosarcoma with malignant fibrous histocytoma subtype in the sellar region; nodular goiter with the thyroid gland, one nodus was pathologically demonstrated as papillary carcinoma. GH adenoma, present in a patient with MAS, might be cured by RT; but the risk of malignant transformation of the dysplastic bone tissue in the field of irradiation make it controversial. Lessons from the case reported here told us that we should take great caution when recommending RT for patients like this.

The relationship between physical activity and metabolic syndrome in people with chronic obstructive pulmonary disease.

PubMed

Park, Soo Kyung; Larson, Janet L

2014-01-01

The prevalence of metabolic syndrome has been reported to be 20% to 50% in people with chronic obstructive pulmonary disease (COPD). Because such people are sedentary and physically inactive, they are at risk of metabolic syndrome. The extent of this problem, however, is not fully understood. This study examined the relationship of sedentary time and physical activity to metabolic syndrome and the components of metabolic syndrome in a population-based sample of people with COPD. This was a secondary analysis of existing cross-sectional data. Subjects with COPD (n = 223) were drawn from the National Health and Nutrition Examination Survey data set (2003-2006). Physical activity was measured by accelerometry. Waist circumference, triglyceride level, high-density lipoprotein cholesterol level, blood pressure, and fasting glucose level were used to describe metabolic syndrome. Descriptive and inferential statistics were used for analysis. Fifty-five percent of the sample had metabolic syndrome. No significant differences in sedentary time and level of physical activity were found in people with COPD and metabolic syndrome and people with COPD only. However, those with a mean activity count of greater than 240 counts per minute had a lower prevalence of metabolic syndrome. Waist circumference and glucose level were significantly associated with the time spent in sedentary, light, and moderate to vigorous physical activity. Metabolic syndrome is highly prevalent in people with COPD, and greater physical activity and less sedentary time are associated with lower rates of metabolic syndrome. This suggests that interventions to decrease the risk of metabolic syndrome in people with COPD should include both reducing sedentary time and increasing the time and intensity of physical activity.

Identifying the barriers and facilitators to participation in physical activity for children with Down syndrome.

PubMed

Barr, M; Shields, N

2011-11-01

Many children with Down syndrome do not undertake the recommended amount of daily physical activity. The aim of this study was to explore the barriers and facilitators to physical activity for this group. Eighteen in-depth interviews were conducted with 20 parents (16 mothers, 4 fathers) of children with Down syndrome aged between 2 and 17 years to examine what factors facilitate physical activity and what factors are barriers to activity for their children. The participants were recruited through a community disability organisation that advocates for people with Down syndrome and their families. Interviews were recorded, transcribed and independently coded and analysed by two researchers using thematic analysis. Four themes on facilitators of physical activity were identified: (1) the positive role of the family; (2) opportunity for social interaction with peers; (3) structured accessible programmes that make adaptations for children with Down syndrome; and (4) children who were determined to succeed and physically skilled. Four themes on the barriers to physical activity were also identified: (1) characteristics commonly associated with Down syndrome; (2) competing family responsibilities; (3) reduced physical or behavioural skills; and (4) a lack of accessible programmes. The results highlight the important role of families in determining how much physical activity children with Down syndrome undertake and the effect that common characteristics associated with Down syndrome can have on maintaining an active lifestyle. Future research needs to concentrate on successful methods of encouraging physical activity, such as ensuring social interaction is part of the activity, and eliminating barriers to physical activity such as the a lack of appropriate programmes for children with Down syndrome. Implementing these strategies may encourage children with Down syndrome to participate more frequently in a physically active lifestyle. © 2011 The Authors. Journal of

Improved background suppression in 1H MAS NMR using composite pulses

NASA Astrophysics Data System (ADS)

Odedra, Smita; Wimperis, Stephen

2012-08-01

A well known feature of 1H MAS NMR spectroscopy, particularly of solids where the concentration of 1H nuclei is low, is the presence in the spectrum of a significant broad "background" signal arising from 1H nuclei that are outside the MAS rotor and radiofrequency coil, probably located on the surfaces of the static components of the probehead. A popular method of suppressing this unwanted signal is the "depth pulse" method, consisting of a 90° pulse followed by one or two 180° pulses that are phase cycled according to the "Exorcycle" scheme, which removes signal associated with imperfect 180° pulses. Consequently, only spins in the centre of the radiofrequency coil contribute to the 1H MAS spectrum, while those experiencing a low B1 field outside the coil are suppressed. Although very effective at removing background signal from the spectrum, one drawback with this approach is that significant loss of the desired signal from the sample also occurs. Here we investigate the 1H background suppression problem and, in particular, the use of novel antisymmetric passband composite pulses to replace the simple pulses in a depth pulse experiment. We show that it is possible to improve the intensity of the 1H signals of interest while still maintaining effective background suppression. We expect that these results will be relevant to 1H MAS NMR studies of, for example, nominally perdeuterated biological samples or nominally anhydrous inorganic materials.

The Mas receptor mediates modulation of insulin signaling by angiotensin-(1-7).

PubMed

Muñoz, Marina C; Giani, Jorge F; Burghi, Valeria; Mayer, Marcos A; Carranza, Andrea; Taira, Carlos A; Dominici, Fernando P

2012-08-20

Angiotensin (Ang)-(1-7) stimulates proteins belonging to the insulin signaling pathway and ameliorates the Ang II negative effects at this level. However, up to date, receptors involved and mechanisms behind these observations remain unknown. Accordingly, in the present study, we explored the in vivo effects of antagonism of the Ang-(1-7) specific Mas receptor on insulin signal transduction in rat insulin-target tissues. We evaluated the acute modulation of insulin-stimulated phosphorylation of Akt, GSK-3β (Glycogen synthase kinase-3β) and AS160 (Akt substrate of 160kDa) by Ang-(1-7) and/or Ang II in the presence and absence of the selective Mas receptor antagonist A-779 in insulin-target tissues of normal rats. Also using A-779, we determined whether the Mas receptor mediates the improvement of insulin sensitivity exerted by chronic Ang-(1-7) treatment in fructose-fed rats (FFR), a model of insulin resistance, dyslipidemia and mild hypertension. The two major findings of the present work are as follows; 1) Ang-(1-7) attenuates acute Ang II-mediated inhibition of insulin signaling components in normal rats via a Mas receptor-dependent mechanism; and 2). The Mas receptor appears to be involved in beneficial effects of Ang-(1-7) on the phosphorylation of crucial insulin signaling mediators (Akt, GSK-3β and AS160), in liver, skeletal muscle and adipose tissue of FFR. These results shed light into the mechanism by which Ang-(1-7) exerts its positive physiological modulation of insulin actions in classical metabolic tissues and reinforces the central role of Akt in these effects. Copyright © 2012 Elsevier B.V. All rights reserved.

Acromegaly and McCune-Albright Syndrome

PubMed Central

Salenave, Sylvie; Boyce, Alison M.; Collins, Michael T.

2014-01-01

Background: McCune-Albright syndrome (MAS) includes the triad of poly/monostotic fibrous dysplasia, café-au-lait spots, and hyperfunctioning endocrinopathies. Acromegaly affects around 20% of MAS patients. Aims: The objective was to review all reported cases of acromegaly associated with MAS. Methods: All studies and case reports of acromegaly in patients with MAS were systematically sought in the world literature up to January 2013. We also included new data (from three unreported cases) and updated data on 23 previously reported patients from our two centers. Results: We reviewed the cases of 112 patients (65 males). Mean age at diagnosis of acromegaly was 24.4 years (range, 3–64). Among the 40 pediatric patients, 23 (57%) had precocious puberty. GH/IGF-1 excess was suggested by accelerated growth in 85% of pediatric cases. Acromegaly was almost always associated with skull base fibrous dysplasia. Modern imaging techniques (computed tomography or magnetic resonance imaging) revealed an adenoma in 54% of the patients (macroadenoma in more than two-thirds). Median GH levels and mean IGF-1 SD score at diagnosis were 57 μg/L (2.8 to 291 μg/L) and 8 (2.3 to 24), respectively. Hyperprolactinemia was present in 81% (mean, 149 μg/L; range, 21–600). Pituitary surgery, performed in 25 cases, very rarely cured the GH/IGF-1 excess. Somatostatin analogs improved GH/IGF-1 levels in most patients but achieved control of acromegaly in only 17 (30%) of 56 patients. Pegvisomant achieved normal IGF-1 levels in 10 of 13 cases. Conclusion: Acromegaly, which is present in 20–30% of patients with MAS, raises particular diagnostic and therapeutic issues. PMID:24517150

Leisure-time exercise, physical activity during work and commuting, and risk of metabolic syndrome.

PubMed

Kuwahara, Keisuke; Honda, Toru; Nakagawa, Tohru; Yamamoto, Shuichiro; Akter, Shamima; Hayashi, Takeshi; Mizoue, Tetsuya

2016-09-01

Data are limited regarding effect of intensity of leisure-time physical activity on metabolic syndrome. Furthermore, no prospective data are available regarding effect of occupational and commuting physical activity on metabolic syndrome. We compared metabolic syndrome risk by intensity level of leisure-time exercise and by occupational and commuting physical activity in Japanese workers. We followed 22,383 participants, aged 30-64 years, without metabolic syndrome until 2014 March (maximum, 5 years of follow-up). Physical activity was self-reported. Metabolic syndrome was defined by the Joint Statement criteria. We used Cox regression models to estimate the hazard ratios (HRs) and 95 % confidence intervals (CIs) of metabolic syndrome. During a mean follow-up of 4.1 years, 5361 workers developed metabolic syndrome. After adjustment for covariates, compared with engaging in no exercise, the HRs (95 % CIs) for <7.5, 7.5 to <16.5, and ≥16.5 metabolic equivalent hours of exercise per week were 0.99 (0.90, 1.08), 0.99 (0.90, 1.10), and 0.95 (0.83, 1.08), respectively, among individuals engaging in moderate-intensity exercise alone; 0.93 (0.75, 1.14), 0.81 (0.64, 1.02), and 0.84 (0.66, 1.06), among individuals engaging in vigorous-intensity exercise alone; and 0.90 (0.70, 1.17), 0.74 (0.62, 0.89), and 0.81 (0.69, 0.96) among individuals engaging in the two intensities. Higher occupational physical activity was weakly but significantly associated with lower risk of metabolic syndrome. Walking to and from work was not associated with metabolic syndrome. Vigorous-intensity exercise alone or vigorous-intensity combined with moderate-intensity exercise and worksite intervention for physical activity may help prevent metabolic syndrome for Japanese workers.

Low-dose CT for quantitative analysis in acute respiratory distress syndrome

PubMed Central

2013-01-01

Introduction The clinical use of serial quantitative computed tomography (CT) to characterize lung disease and guide the optimization of mechanical ventilation in patients with acute respiratory distress syndrome (ARDS) is limited by the risk of cumulative radiation exposure and by the difficulties and risks related to transferring patients to the CT room. We evaluated the effects of tube current-time product (mAs) variations on quantitative results in healthy lungs and in experimental ARDS in order to support the use of low-dose CT for quantitative analysis. Methods In 14 sheep chest CT was performed at baseline and after the induction of ARDS via intravenous oleic acid injection. For each CT session, two consecutive scans were obtained applying two different mAs: 60 mAs was paired with 140, 15 or 7.5 mAs. All other CT parameters were kept unaltered (tube voltage 120 kVp, collimation 32 × 0.5 mm, pitch 0.85, matrix 512 × 512, pixel size 0.625 × 0.625 mm). Quantitative results obtained at different mAs were compared via Bland-Altman analysis. Results Good agreement was observed between 60 mAs and 140 mAs and between 60 mAs and 15 mAs (all biases less than 1%). A further reduction of mAs to 7.5 mAs caused an increase in the bias of poorly aerated and nonaerated tissue (-2.9% and 2.4%, respectively) and determined a significant widening of the limits of agreement for the same compartments (-10.5% to 4.8% for poorly aerated tissue and -5.9% to 10.8% for nonaerated tissue). Estimated mean effective dose at 140, 60, 15 and 7.5 mAs corresponded to 17.8, 7.4, 2.0 and 0.9 mSv, respectively. Image noise of scans performed at 140, 60, 15 and 7.5 mAs corresponded to 10, 16, 38 and 74 Hounsfield units, respectively. Conclusions A reduction of effective dose up to 70% has been achieved with minimal effects on lung quantitative results. Low-dose computed tomography provides accurate quantitative results and could be used to characterize lung compartment distribution and

Using Stable Isotopes to Assess Reduced Physical Activity of Individuals with Prader-Willi Syndrome.

ERIC Educational Resources Information Center

Davies, Peter S. W.; Joughlin, C.

1993-01-01

This study found that the physical activity levels of 10 children with Prader-Willi syndrome were significantly reduced in comparison to children without the syndrome. Increasing activity levels in children with Prader-Willi syndrome is suggested as a way to raise total energy expenditure and control weight gain. (Author/JDD)

Technology Development for a Hyperspectral Microwave Atmospheric Sounder (HyMAS)

NASA Technical Reports Server (NTRS)

Blackwell, W.; Galbraith, C.; Hilliard, L.; Racette, P.; Thompson, E.

2014-01-01

The Hyperspectral Microwave Atmospheric Sounder (HyMAS) is being developed at Lincoln Laboratories and accommodated by the Goddard Space Flight Center for a flight opportunity on a NASA research aircraft. The term hyperspectral microwave is used to indicate an all-weather sounding instrument that performs equivalent to hyperspectral infrared sounders in clear air with vertical resolution of approximately 1 km. Deploying the HyMAS equipped scanhead with the existing Conical Scanning Microwave Imaging Radiometer (CoSMIR) shortens the path to a flight demonstration. Hyperspectral microwave is achieved through the use of independent RF antennas that sample the volume of the Earths atmosphere through various levels of frequencies, thereby producing a set of dense, spaced vertical weighting functions.

Research on the theory and methodology of integrating GIS and MAS and its application in simulating of pedestrians flows in a crowd's activity centre of Shanghai metropolitan

NASA Astrophysics Data System (ADS)

Liu, Miaolong; Chen, Peng

2006-10-01

Based on the development trend of research on urban morphology and its evolution from macro scale to micro scale, a new tight-coupling integrating method of GIS and MAS has been discussed briefly in this paper. After analyzing the characteristics and mechanism of pedestrian's flows in a crowds' activity center in a metropolitan, a prototype and mathematical expression of pedestrian's flows simulation have been put forward in the paper. A few key expressions and techniques for treating the specific behaviors of pedestrians flows, especially how the individuals of the flows make a decision to follow a original designed direction, how to make a decision whether stop or change his movement and select a new direction when the individuals meet a obstacle have been explored and discussed in detail. Using some tools provided by general GIS systems (such as ArcGIS 9) and a few specific programming languages, a new software system integrating GIS and MAS applicable for simulating pedestrians flows in a crowd activity centre has been developed successfully. Under the environment supported by the software system, as an applicable case, a dynamic evolution process of the pedestrian's flows (dispersed process for the spectators) in a crowds' activity center - The Shanghai Stadium has been simulated successfully. The successful simulating of a case of emergence when one or more exits emerge accidents will be very useful for managing and treating crowds' safety in a lot of assembling centers. At the end of the paper, some new research problems have been pointed out for the future.

Brain angiotensin-(1-7)/Mas axis: A new target to reduce the cardiovascular risk to emotional stress.

PubMed

Fontes, Marco Antônio Peliky; Martins Lima, Augusto; Santos, Robson Augusto Souza dos

2016-04-01

Emotional stress is now considered a risk factor for several diseases including cardiac arrhythmias and hypertension. It is well known that the activation of neuroendocrine and autonomic mechanisms features the response to emotional stress. However, its link to cardiovascular diseases and the regulatory mechanisms involved remain to be further comprehended. The renin-angiotensin system (RAS) plays an important role in homeostasis on all body systems. Specifically in the brain, the RAS regulates a number of physiological aspects. Recent data indicate that the activation of angiotensin-converting enzyme/angiotensin II/AT1 receptor axis facilitates the emotional stress responses. On the other hand, growing evidence indicates that its counterregulatory axis, the angiotensin-converting enzyme 2 (ACE2)/(Ang)iotensin-(1-7)/Mas axis, reduces anxiety and attenuates the physiological responses to emotional stress. The present review focuses on angiotensin-(1-7)/Mas axis as a promising target to attenuate the physiological response to emotional stress reducing the risk of cardiovascular diseases. Copyright © 2015 Elsevier Ltd. All rights reserved.

Operando MAS NMR Reaction Studies at High Temperatures and Pressures

DOE Office of Scientific and Technical Information (OSTI.GOV)

Walter, Eric D.; Qi, Long; Chamas, Ali

Operando MAS-NMR studies provide unique insights into the details of chemical reactions; comprehensive information about temperature- and time-dependent changes in chemical species is accompanied by similarly rich information about changes in phase and chemical environment. Here we describe a new MAS-NMR rotor (the WHiMS rotor) capable of achieving internal pressures up to 400 bar at 20 °C or 225 bar at 250 °C, a range which includes many reactions of interest. The MAS-NMR spectroscopy enabled by these rotors is ideal for studying the behavior of mixed-phase systems, such as reactions involving solid catalysts and volatile liquids, with the potential tomore » add gases at high pressure. The versatile operation of the new rotors is demonstrated by collecting operando 1H and 13C spectra during the hydrogenolysis of benzyl phenyl ether, catalyzed by Ni/-Al2O3 at ca. 250 ºC, both with and without H2 (g) supplied to the rotor. The 2-propanol solvent, which exists in the supercritical phase under these reaction conditions, serves as an internal source of H2. The NMR spectra provide detailed kinetic profiles for the formation of the primary products toluene and phenol, as well as secondary hydrogenation and etherification products.« less

Improved background suppression in ¹H MAS NMR using composite pulses.

PubMed

Odedra, Smita; Wimperis, Stephen

2012-08-01

A well known feature of ¹H MAS NMR spectroscopy, particularly of solids where the concentration of ¹H nuclei is low, is the presence in the spectrum of a significant broad "background" signal arising from ¹H nuclei that are outside the MAS rotor and radiofrequency coil, probably located on the surfaces of the static components of the probehead. A popular method of suppressing this unwanted signal is the "depth pulse" method, consisting of a 90° pulse followed by one or two 180° pulses that are phase cycled according to the "Exorcycle" scheme, which removes signal associated with imperfect 180° pulses. Consequently, only spins in the centre of the radiofrequency coil contribute to the ¹H MAS spectrum, while those experiencing a low B₁ field outside the coil are suppressed. Although very effective at removing background signal from the spectrum, one drawback with this approach is that significant loss of the desired signal from the sample also occurs. Here we investigate the ¹H background suppression problem and, in particular, the use of novel antisymmetric passband composite pulses to replace the simple pulses in a depth pulse experiment. We show that it is possible to improve the intensity of the ¹H signals of interest while still maintaining effective background suppression. We expect that these results will be relevant to ¹H MAS NMR studies of, for example, nominally perdeuterated biological samples or nominally anhydrous inorganic materials. Copyright © 2012 Elsevier Inc. All rights reserved.

Physical activity, cardiorespiratory fitness, and metabolic syndrome in adolescents: A cross-sectional study

PubMed Central

2011-01-01

Background In adults, there is a substantial body of evidence that physical inactivity or low cardiorespiratory fitness levels are strongly associated with the development of metabolic syndrome. Although this association has been studied extensively in adults, little is known regarding this association in adolescents. The aim of this study was to analyze the association between physical activity and cardiorespiratory fitness levels with metabolic syndrome in Brazilian adolescents. Methods A random sample of 223 girls (mean age, 14.4 ± 1.6 years) and 233 boys (mean age, 14.6 ± 1.6 years) was selected for the study. The level of physical activity was determined by the Bouchard three-day physical activity record. Cardiorespiratory fitness was estimated by the Leger 20-meter shuttle run test. The metabolic syndrome components assessed included waist circumference, blood pressure, HDL-cholesterol, triglycerides, and fasting plasma glucose levels. Independent Student t-tests were used to assess gender differences. The associations between physical activity and cardiorespiratory fitness with the presence of metabolic syndrome were calculated using logistic regression models adjusted for age and gender. Results A high prevalence of metabolic syndrome was observed in inactive adolescents (males, 11.4%; females, 7.2%) and adolescents with low cardiorespiratory fitness levels (males, 13.9%; females, 8.6%). A significant relationship existed between metabolic syndrome and low cardiorespiratory fitness (OR, 3.0 [1.13-7.94]). Conclusion The prevalence of metabolic syndrome is high among adolescents who are inactive and those with low cardiorespiratory fitness. Prevention strategies for metabolic syndrome should concentrate on enhancing fitness levels early in life. PMID:21878095

Effectiveness of physical activity intervention among government employees with metabolic syndrome.

PubMed

Huei Phing, Chee; Abu Saad, Hazizi; Barakatun Nisak, M Y; Mohd Nasir, M T

2017-12-01

Our study aimed to assess the effects of physical activity interventions via standing banners (point-of-decision prompt) and aerobics classes to promote physical activity among individuals with metabolic syndrome. We conducted a cluster randomized controlled intervention trial (16-week intervention and 8-week follow-up). Malaysian government employees in Putrajaya, Malaysia, with metabolic syndrome were randomly assigned by cluster to a point-of-decision prompt group (n = 44), an aerobics group (n = 42) or a control group (n = 103) based on sample size calculation formula. Step counts were evaluated by Lifecorder e-STEP accelerometers for all participants. Metabolic syndrome was defined according to the 'harmonizing' definition, in which individuals who have at least three of the five metabolic risk factors (waist circumference, high-density lipoprotein cholesterol, triglycerides, fasting glucose levels, systolic and diastolic blood pressure) will be classified as having metabolic syndrome. A total of 80% of the enrolled government employees with metabolic syndrome completed the programme. Data were analyzed using SPSS for Windows (version 20, SPSS, Chicago, IL). There were significantly higher step counts on average in the aerobics group compared to the control group over assessments. Assessments at baseline, post-intervention and follow-up showed a significant difference in step counts between the intervention and control groups. The greatest reductions in the proportions of individuals with metabolic syndrome were observed in the aerobics group with a reduction of 79.4% in the post-intervention assessment compared to the assessment at baseline. The findings of this study suggest that physical activity intervention via aerobics classes is an effective strategy for improving step counts and reducing the prevalence of metabolic syndrome.

Meconium Aspiration Syndrome: A Role for Fetal Systemic Inflammation

PubMed Central

LEE, JoonHo; ROMERO, Roberto; LEE, Kyung A; KIM, Eun Na; KORZENIEWSKI, Steven J; CHAEMSAITHONG, Piya; YOON, Bo Hyun

2017-01-01

Background Meconium aspiration syndrome (MAS) is a leading cause of morbidity and mortality in term infants. Meconium-stained amniotic fluid (MSAF) occurs in approximately one of every seven pregnancies, but only 5% of neonates exposed to MSAF develop MAS. Why some infants exposed to meconium develop MAS while others do not is a fundamental question. Patients with MSAF have a higher frequency of intra-amniotic infection/inflammation than those with clear fluid. We propose that fetal systemic inflammation is a risk factor for the development of MAS in patients with MSAF. Objective To investigate whether intra-amniotic inflammation and funisitis, the histopathologic landmark of a fetal inflammatory response, predispose to MAS. Study Design A prospective cohort study was conducted from 1995 through 2009. Amniotic fluid (AF) samples (n=1,281) were collected at the time of cesarean delivery from women who delivered singleton newborns at term (gestational age ≥38 weeks). Intra-amniotic inflammation was diagnosed if the AF concentration of matrix metalloproteinase-8 (MMP-8) was >23 ng/ml. Funisitis was diagnosed by histologic examination if inflammation was present in the umbilical cord. Results The prevalence of MSAF was 9.2% (118/1,281), and 10.2% (12/118) of neonates exposed to MSAF developed MAS. There were no significant differences in the median gestational age or umbilical cord arterial pH at birth between neonates who developed MAS and those who did not (each p > 0.1). Mothers whose newborns developed MAS had a higher median AF MMP-8 (456.8 ng/ml vs.157.2 ng/ml; p < 0.05). Newborns exposed to intra-amniotic inflammation had a higher rate of MAS than those who were not exposed to intra-amniotic inflammation [13.0% (10/77) vs. 0% (0/32), p = 0.03], as did those exposed to funisitis [31.3% (5/16) vs. 7.3% (6/82), relative risk, 4.3; 95% confidence interval, 1.5–12.3]. Among the 89 newborns for whom both AF and placental histology were available, MAS was more

ACE-2/Ang1-7/Mas cascade mediates ACE inhibitor, captopril, protective effects in estrogen-deficient osteoporotic rats.

PubMed

Abuohashish, Hatem M; Ahmed, Mohammed M; Sabry, Dina; Khattab, Mahmoud M; Al-Rejaie, Salim S

2017-08-01

The local role of the renin angiotensin system (RAS) was documented recently beside its conventional systemic functions. Studies showed that the effector angiotensin II (AngII) alters bone health, while inhibition of the angiotensin converting enzyme (ACE-1) preserved these effects. The newly identified Ang1-7 exerts numerous beneficial effects opposing the AngII. Thus, the current study examines the role of Ang1-7 in mediating the osteo-preservative effects of ACEI (captopril) through the G-protein coupled Mas receptor using an ovariectomized (OVX) rat model of osteoporosis. 8 weeks after the surgical procedures, captopril was administered orally (40mgkg -1 d -1 ), while the specific Mas receptor blocker (A-779) was delivered at infusion rate of 400ngkg -1 min -1 for 6 weeks. Bone metabolic markers were measured in serum and urine. Minerals concentrations were quantified in serum, urine and femoral bones by inductive coupled plasma mass spectroscopy (ICP-MS). Trabecular and cortical morphometry was analyzed in the right distal femurs using micro-CT. Finally, the expressions of RAS peptides, enzymes and receptors along with the receptor activator of NF-κB ligand (RANKL) and osteoprotegerin (OPG) were determined femurs heads. OVX animals markedly showed altered bone metabolism and mineralization along with disturbed bone micro-structure. Captopril significantly restored the metabolic bone bio-markers and corrected Ca 2+ and P values in urine and bones of estrogen deficient rats. Moreover, the trabecular and cortical morphometric features were repaired by captopril in OVX groups. Captopril also improved the expressions of ACE-2, Ang1-7, Mas and OPG, while abolished OVX-induced up-regulation of ACE-1, AngII, Ang type 1 receptor (AT1R) and RANKL. Inhibition of Ang1-7 cascade by A-779 significantly eradicated captopril protective effects on bone metabolism, mineralization and micro-structure. A-779 also restored OVX effects on RANKL expression and ACE-1/AngII/AT1R

Arginase activity and nitric oxide levels in patients with obstructive sleep apnea syndrome

PubMed Central

Yüksel, Meral; Okur, Hacer Kuzu; Pelin, Zerrin; Öğünç, Ayliz Velioğlu; Öztürk, Levent

2014-01-01

OBJECTIVE: Obstructive sleep apnea syndrome is characterized by repetitive obstruction of the upper airways, and it is a risk factor for cardiovascular diseases. There have been several studies demonstrating low levels of nitric oxide in patients with obstructive sleep apnea syndrome compared with healthy controls. In this study, we hypothesized that reduced nitric oxide levels would result in high arginase activity. Arginase reacts with L-arginine and produces urea and L-ornithine, whereas L-arginine is a substrate for nitric oxide synthase, which produces nitric oxide. METHODS: The study group consisted of 51 obstructive sleep apnea syndrome patients (M/F: 43/8; mean age 49±10 years of age) and 15 healthy control subjects (M/F: 13/3; mean age 46±14 years of age). Obstructive sleep apnea syndrome patients were divided into two subgroups based on the presence or absence of cardiovascular disease. Nitric oxide levels and arginase activity were measured via an enzyme-linked immunosorbent assay of serum samples. RESULTS: Serum nitric oxide levels in the control subjects were higher than in the obstructive sleep apnea patients with and without cardiovascular diseases (p<0.05). Arginase activity was significantly higher (p<0.01) in obstructive sleep apnea syndrome patients without cardiovascular diseases compared with the control group. Obstructive sleep apnea syndrome patients with cardiovascular diseases had higher arginase activity than the controls (p<0.001) and the obstructive sleep apnea syndrome patients without cardiovascular diseases (p<0.05). CONCLUSION: Low nitric oxide levels are associated with high arginase activity. The mechanism of nitric oxide depletion in sleep apnea patients suggests that increased arginase activity might reduce the substrate availability of nitric oxide synthase and thus could reduce nitric oxide levels. PMID:24714832

Total physical activity volume, physical activity intensity, and metabolic syndrome: 1999-2004 National Health and Nutrition Examination Survey.

PubMed

Churilla, James R; Fitzhugh, Eugene C

2012-02-01

This study examined the association of total physical activity volume (TPAV) and physical activity (PA) from three domains [leisure-time physical activity (LTPA), domestic, transportation] with metabolic syndrome. We also investigated the relationship between LTPA intensity and metabolic syndrome risk. Sample included adults who participated in the 1999-2004 National Health and Nutrition Examination Survey. Physical activity measures were created for TPAV, LTPA, domestic PA, and transportational PA. For each, a six-level measure based upon no PA (level 1) and quintiles (levels 2-6) of metabolic equivalents (MET)·min·wk(-1) was created. A three-level variable associated with the current Department of Health and Human Services (DHHS) PA recommendation was also created. SAS and SUDAAN were used for the statistical analysis. Adults reporting the greatest volume of TPAV and LTPA were found to be 36% [odds ratio (OR) 0.64; 95% confidence interval (CI) 0.49-0.83] and 42% (OR 0.58; 95% CI 0.43-0.77), respectively, less likely to have metabolic syndrome. Domestic and transportational PA provided no specific level of protection from metabolic syndrome. Those reporting a TPAV that met the DHHS PA recommendation were found to be 33% (OR 0.67; 95%; CI 0.55-0.83) less likely to have metabolic syndrome compared to their sedentary counterparts. Adults reporting engaging in only vigorous-intensity LTPA were found to be 37% (OR 0.63; 95 CI 0.42-0.96) to 56% (OR 0.44; 95% CI 0.29-0.67) less likely to have metabolic syndrome. Volume, intensity, and domain of PA may all play important roles in reducing the prevalence and risk of metabolic syndrome.

Physical Activity and Sedentary Behavior Associated with Components of Metabolic Syndrome among People in Rural China.

PubMed

Xiao, Jing; Shen, Chong; Chu, Min J; Gao, Yue X; Xu, Guang F; Huang, Jian P; Xu, Qiong Q; Cai, Hui

2016-01-01

Metabolic syndrome is prevalent worldwide and its prevalence is related to physical activity, race, and lifestyle. Little data is available for people living in rural areas of China. In this study we examined associations of physical activity and sedentary behaviors with metabolic syndrome components among people in rural China. The Nantong Metabolic Syndrome Study recruited 13,505 female and 6,997 male participants between 2007 and 2008. Data of socio-demographic characteristics and lifestyle were collected. The associations of physical activity and sedentary behaviors with metabolic syndrome components were analyzed. Prevalence of metabolic syndrome was 21.6%. It was significantly lower in men than in women. Low risks of metabolic syndrome were observed in those who did less sitting and engaged in more vigorous physical activity. The highest tertile of vigorous physical activity was associated with 15-40% decreased odds of metabolic syndrome and all of its components, except for low high-density lipoprotein cholesterol in men. Women with the highest tertile of moderate physical activity had 15-30% lower odds of central obesity, high glucose, and high triglycerides compared with those in the lowest tertile. Sitting time >42 hours per week had a 4%-12% attributable risk of metabolic syndrome, central obesity, and high triglycerides in both genders, and abnormal glucose and diastolic blood pressure in women. Sleeping for more than 8 hours per day was associated with risk of high serum glucose and lipids. Our data suggested that physical activity has a preventive effect against metabolic syndrome and all its abnormal components, and that longer sitting time and sleep duration are associated with an increased risk of metabolic syndrome components, including central obesity and high triglycerides, glucose, and diastolic blood pressure. This study could provide information for future investigation into these associations. Also, recommendations are developed to reduce

Reach a nonlinear consensus for MAS via doubly stochastic quadratic operators

NASA Astrophysics Data System (ADS)

Abdulghafor, Rawad; Turaev, Sherzod; Zeki, Akram; Al-Shaikhli, Imad

2018-06-01

This technical note addresses the new nonlinear protocol class of doubly stochastic quadratic operators (DSQOs) for coordination of consensus problem in multi-agent systems (MAS). We derive the conditions for ensuring that every agent reaches consensus on a desired rate of the group's decision where the group decision value in its agent's initial statuses varies. Besides that, we investigate a nonlinear protocol sub-class of extreme DSQO (EDSQO) to reach a consensus for MAS to a common value with nonlinear low-complexity rules and fast time convergence if the interactions for each agent are not selfish. In addition, to extend the results to reach a consensus and to avoid the selfish case we specify a general class of DSQO for reaching a consensus under any given case of initial states. The case that MAS reach a consensus by DSQO is if each member of the agent group has positive interactions of DSQO (PDSQO) with the others. The convergence of both EDSQO and PDSQO classes is found to be directed towards the centre point. Finally, experimental simulations are given to support the analysis from theoretical aspect.

Prevalence of metabolic syndrome and its relationship with physical activity in suburban Beijing, China.

PubMed

Zhang, Wei-Hong; Xue, Peng; Yao, Meng-Ying; Chang, Hai-Min; Wu, Yan; Zhang, Lei

2013-01-01

The present study aimed to estimate the up-to-date prevalence of metabolic syndrome and its relationship with physical activity among suburban adults in Beijing, China. A cross-sectional survey in a representative sample of 19,003 suburban adults aged 18-76 years was carried out in 2007-2008. Data was collected via questionnaires and blood pressure, anthropometric, and laboratory measurements. Of the residents aged 18-76 years in suburban Beijing, 25.9% (27.3% in men and 25.1% in women), 21.3% (19.4% in men and 22.9% in women), and 25.3% (24.2% in men and 26.1% in women) had 1 component, 2 components, and 3 or more components of metabolic syndrome, respectively. The age-standardized prevalence of metabolic syndrome and its components, including abdominal obesity, elevated triglycerides, reduced high-density lipoprotein cholesterol, elevated blood pressure, and elevated fasting plasma glucose, decreased across categories with increasing physical activity. After adjusting for age, sex, education level, smoking, and alcohol consumption, residents were more likely to have metabolic syndrome across categories with decreasing physical activity; a similar relationship also applied to components of metabolic syndrome. A high prevalence of metabolic syndrome and its components is commonly present in suburban Beijing. Increasing physical activity can reduce the relative risk of metabolic syndrome and it components.

Physical Activity, Metabolic Syndrome, and Overweight in Rural Youth

ERIC Educational Resources Information Center

Moore, Justin B.; Davis, Catherine L.; Baxter, Suzanne Domel; Lewis, Richard D.; Yin, Zenong

2008-01-01

Background: Research suggests significant health differences between rural dwelling youth and their urban counterparts with relation to cardiovascular risk factors. This study was conducted to (1) determine relationships between physical activity and markers of metabolic syndrome, and (2) to explore factors relating to physical activity in a…

Clinical utility of soluble interleukin-2 receptor in hemophagocytic syndromes: a systematic scoping review.

PubMed

Lin, Molly; Park, Sujin; Hayden, Anna; Giustini, Dean; Trinkaus, Martina; Pudek, Morris; Mattman, Andre; Schneider, Marion; Chen, Luke Y C

2017-08-01

The serum-soluble interleukin-2 receptor (sIL-2r) level is considered an important diagnostic test and disease marker in hemophagocytic syndromes/hemophagocytic lymphohistiocytosis (HPS/HLH). However, this cytokine receptor is rarely measured in clinical practice and has been excluded from recent diagnostic/classification criteria such as the HScore and macrophage activation syndrome (MAS) 16. We performed a systematic scoping review of 64 articles (1975-2016) examining the clinical utility of sIL-2r in HPS/HLH. Twenty-two articles describe sIL-2r as a sensitive diagnostic marker for HLH, but only three distinct datasets actually address sensitivity. The original HLH-2004 Guidelines reported sensitivity of 93% and specificity of 100% for sIL-2r ≥ 2400, based on a pediatric dataset (n = 152) which is published for the first time in this review. Two pediatric studies reported sensitivity of 89% for sIL-2r ≥ 2400 in diagnosis of MAS complicating juvenile idiopathic arthritis (JIA) (n = 27) and 88% for secondary HLH in acute liver failure (n = 9). Twenty articles described sIL-2r as a dynamic marker of disease activity that falls with response to treatment, and 15 described high initial sIL-2r levels >10,000 U/mL as a poor prognostic marker. The ability of sIL-2r to distinguish between subtypes of HPS/HLH was inconsistent. This review confirms the importance of soluble IL-2r as a diagnostic and disease marker in HPS/HLH, but also reveals the need for more primary data about its performance characteristics, particularly in adults. More emphasis should be made in including this simple, inexpensive test in clinical practice and studies of HPS/HLH.

An HR-MAS MR Metabolomics Study on Breast Tissues Obtained with Core Needle Biopsy

PubMed Central

Cho, Nariya; Chang, Jung Min; Koo, Hye Ryoung; Yi, Ann; Kim, Hyeonjin; Park, Sunghyouk; Moon, Woo Kyung

2011-01-01

Background Much research has been devoted to the development of new breast cancer diagnostic measures, including those involving high-resolution magic angle spinning (HR-MAS) magnetic resonance (MR) spectroscopic techniques. Previous HR-MAS MR results have been obtained from post-surgery samples, which limits their direct clinical applicability. Methodology/Principal Findings In the present study, we performed HR-MAS MR spectroscopic studies on 31 breast tissue samples (13 cancer and 18 non-cancer) obtained by percutaneous core needle biopsy. We showed that cancer and non-cancer samples can be discriminated very well with Orthogonal Projections to Latent Structure-Discriminant Analysis (OPLS-DA) multivariate model on the MR spectra. A subsequent blind test showed 69% sensitivity and 94% specificity in the prediction of the cancer status. A spectral analysis showed that in cancer cells, taurine- and choline-containing compounds are elevated. Our approach, additionally, could predict the progesterone receptor statuses of the cancer patients. Conclusions/Significance HR-MAS MR metabolomics on intact breast tissues obtained by core needle biopsy may have a potential to be used as a complement to the current diagnostic and prognostic measures for breast cancers. PMID:22028780

Leisure Activity and Caregiver Involvement in Middle-Aged and Older Adults With Down Syndrome.

PubMed

Mihaila, Iulia; Hartley, Sigan L; Handen, Benjamin L; Bulova, Peter D; Tumuluru, Rameshwari V; Devenny, Darlynne A; Johnson, Sterling C; Lao, Patrick J; Christian, Bradley T

2017-04-01

The present study examined leisure activity and its association with caregiver involvement (i.e., residence and time spent with primary caregiver) in 62 middle-aged and older adults with Down syndrome (aged 30-53 years). Findings indicated that middle-aged and older adults with Down syndrome frequently participated in social and passive leisure activities, with low participation in physical and mentally stimulating leisure activities. Residence and time spent with primary caregiver were associated with participation in physical leisure activity. The findings suggest a need for support services aimed at increasing opportunities for participating in physical and mentally stimulating leisure activity by middle-aged and older adults with Down syndrome. These support services should partner with primary caregivers in order to best foster participation in physical leisure activity.

The ACE-2/Ang1-7/Mas cascade enhances bone structure and metabolism following angiotensin-II type 1 receptor blockade.

PubMed

Abuohashish, Hatem M; Ahmed, Mohammed M; Sabry, Dina; Khattab, Mahmoud M; Al-Rejaie, Salim S

2017-07-15

The renin angiotensin system (RAS) regulates numerous systemic functions and is expressed locally in skeletal tissues. Angiotensin1-7 (Ang1-7) is a beneficial member of the RAS, and the therapeutic effects of a large number of angiotensin receptors blockers (ARBs) are mediated by an Ang1-7-dependent cascade. This study examines whether the reported osteo-preservative effects of losartan are mediated through the angiotensin converting enzyme2 (ACE-2)/Ang1-7/Mas pathway in ovariectomized (OVX) rats. Sham and OVX animals received losartan (10mg/kg/d p.o.) for 6 weeks. A specific Mas receptor blocker (A-779) was delivered via mini-osmotic pumps during the losartan treatment period. Serum and urine bone metabolism biomarker levels were measured. Bone trabecular and cortical morphometry were quantified in distal femurs, whereas mineral contents were estimated in ashed bones, serum and urine. Finally, the expression of RAS components, the receptor activator of NF-κB ligand (RANKL) and osteoprotegerin (OPG) was determined. Losartan significantly improved the elevated bone metabolism marker levels and altered trabecular and cortical structures in OVX animals, and restored normal urinary and skeletal mineral levels. Mas receptor inhibition significantly abolished all osteo-protective effects of losartan and enhanced the deleterious effects of OVX. Losartan enhanced OVX-induced up-regulation of ACE-1, AngII, angiotensin type 1 (AT 1 ) receptor and RANKL expression, and increased ACE-2, Ang1-7, Mas and OPG expression in OVX animals. However, A-779 significantly eradicated the effects of losartan on RAS components and RANKL/OPG expression. Thus, Ang1-7 are involved in the osteo-preservative effects of losartan via Mas receptor, which may add therapeutic value to this well-known antihypertensive agent. Copyright © 2017 Elsevier B.V. All rights reserved.

Activation of mas-related G-protein-coupled receptors by the house dust mite cysteine protease Der p1 provides a new mechanism linking allergy and inflammation.

PubMed

Reddy, Vemuri B; Lerner, Ethan A

2017-10-20

Cysteine and serine proteases function via protease-activated and mas-related G-protein-coupled receptors (Mrgprs) to contribute to allergy and inflammation. Der p1 is a cysteine protease and major allergen from the house dust mite and is associated with allergic rhinitis and allergic asthma. Der p1 activates protease-activated receptor 2 and induces the release of the pro-inflammatory cytokine IL-6 from cells. However, the possibility that Der p1 acts on Mrgprs has not been considered. We report here that ratiometric calcium imaging reveals that Der p1 activates the human receptor MRGPRX1 and the mouse homolog MrgprC11, implicated previously in itch. Der p1 cleavage of N-terminal receptor peptides followed by site-directed mutagenesis of the cleavage sites links receptor activation to specific amino acid residues. Der p1 also induced the release of IL-6 from heterologous cells expressing MRGPRX1. In summary, activation of Mrgprs by the allergen Der p1 may contribute to inflammation. © 2017 by The American Society for Biochemistry and Molecular Biology, Inc.

Psychometric Comparison of the Motivation Assessment Scale (MAS) and the Questions about Behavioral Function (QABF)

ERIC Educational Resources Information Center

Koritsas, S.; Iacono, T.

2013-01-01

Background: The Motivation Assessment Scale (MAS) and the Questions About Behavioral Function (QABF) are frequently used to assess the learned function of challenging behaviour in people with intellectual disability (ID). The aim was to explore and compare the psychometric properties of the MAS and the QABF. Method: Seventy adults with ID and…

Technology Enhanced Learning for People with Intellectual Disabilities and Cerebral Paralysis: The MAS Platform

NASA Astrophysics Data System (ADS)

Colomo-Palacios, Ricardo; Paniagua-Martín, Fernando; García-Crespo, Ángel; Ruiz-Mezcua, Belén

Education for students with disabilities now takes place in a wide range of settings, thus, including a wider range of assistive tools. As a result of this, one of the most interesting application domains of technology enhanced learning is related to the adoption of learning technologies and designs for people with disabilities. Following this unstoppable trend, this paper presents MAS, a software platform aimed to help people with severe intellectual disabilities and cerebral paralysis in their learning processes. MAS, as a technology enhanced learning platform, provides several tools that supports learning and monitoring for people with special needs, including adaptative games, data processing and monitoring tools. Installed in a special needs education institution in Madrid, Spain, MAS provides special educators with a tool that improved students education processes.

Life-threatening NLRC4-associated hyperinflammation successfully treated with Interleukin-18 inhibition

PubMed Central

Canna, Scott W.; Girard, Charlotte; Malle, Louise; de Jesus, Adriana; Romberg, Neil; Kelsen, Judith; Surrey, Lea F.; Russo, Pierre; Sleight, Andrew; Schiffrin, Eduardo; Gabay, Cem; Goldbach-Mansky, Raphaela; Behrens, Edward M.

2017-01-01

Capsule Summary NLRC4-inflammasome hyperactivity causes infantile-onset Macrophage Activation Syndrome and enterocolitis with extraordinary serum IL-18 elevation (NLRC4-MAS). Herein, we report a critically ill infant with severe, refractory NLRC4-MAS who showed sustained response to treatment with experimental IL-18 inhibition. PMID:27876626

Association between physical activity and metabolic syndrome among Malay adults in a developing country, Malaysia.

PubMed

Chu, Anne H Y; Moy, F M

2014-03-01

Metabolic syndrome is a highly prevalent health problem within the adult population in developing countries. We aimed to study the association of physical activity levels and metabolic risk factors among Malay adults in Malaysia. Cross-sectional. Body mass index, waist circumference, and systolic/diastolic blood pressure, fasting blood glucose, fasting triglyceride and high-density lipoprotein cholesterol levels were measured in 686 Malay participants (aged 35-74 years). Self-reported physical activity was obtained with the validated International Physical Activity Questionnaire (Malay version) and categorized into low, moderate or high activity levels. Individuals who were classified as overweight and obese predominated (65.6%). On the basis of the modified NCEP ATP III criteria, metabolic syndrome was diagnosed in 31.9% of all participants, of whom 46.1% were men and 53.9% were women. The prevalence of metabolic syndrome among participants with low, moderate or high activity levels was 13.3%, 11.7% and 7.0%, respectively (p<0.001). Statistically significant negative associations were found between a number of metabolic risk factors and activity categories (p<0.05). The odds ratios for metabolic syndrome in the moderate and high activity categories were 0.42 (95% CI: 0.27-0.65) and 0.52 (95% CI: 0.35-0.76), respectively, adjusted for gender. Moderate and high activity levels were each associated with reduced odds for metabolic syndrome independent of gender. Although a slightly lower prevalence of metabolic syndrome was associated with high activity than with moderate activity, potential health benefits were observed when moderate activity was performed. Copyright © 2013 Sports Medicine Australia. All rights reserved.

Ammonia Vapor Removal by Cu3(BTC)2 and Its Characterization by MAS NMR

DTIC Science & Technology

2009-01-01

Experimental Section Materials. Cu(acetate)2(H2O), Cu(L-tartrate)3(H2O)3, Cu( CO3 )- Cu(OH)2, 1,3,5-benzenetricarboxylic acid, NH4HCO3, and N,N...for 3 days. The crystals were activated under high vacuum at a temperature of 170 °C. Nitrogen Adsorption Equilibria. Nitrogen adsorption equi- libria...solid (NH4)3BTC. 13C CP-MAS NMR confirmed the identity and purity of the trisubstituted material. BTC-Cu( CO3 )Cu(OH)2-NH4HCO3 Reaction. BTC (100 mg, 480

Exploring high-resolution magic angle spinning (HR-MAS) NMR spectroscopy for metabonomic analysis of apples.

PubMed

Vermathen, Martina; Marzorati, Mattia; Vermathen, Peter

2012-01-01

Classical liquid-state high-resolution (HR) NMR spectroscopy has proved a powerful tool in the metabonomic analysis of liquid food samples like fruit juices. In this paper the application of (1)H high-resolution magic angle spinning (HR-MAS) NMR spectroscopy to apple tissue is presented probing its potential for metabonomic studies. The (1)H HR-MAS NMR spectra are discussed in terms of the chemical composition of apple tissue and compared to liquid-state NMR spectra of apple juice. Differences indicate that specific metabolic changes are induced by juice preparation. The feasibility of HR-MAS NMR-based multivariate analysis is demonstrated by a study distinguishing three different apple cultivars by principal component analysis (PCA). Preliminary results are shown from subsequent studies comparing three different cultivation methods by means of PCA and partial least squares discriminant analysis (PLS-DA) of the HR-MAS NMR data. The compounds responsible for discriminating organically grown apples are discussed. Finally, an outlook of our ongoing work is given including a longitudinal study on apples.

Leisure Activity and Caregiver Involvement in Middle-Aged and Older Adults With Down Syndrome

PubMed Central

Mihaila, Iulia; Hartley, Sigan L.; Handen, Benjamin L.; Bulova, Peter D.; Tumuluru, Rameshwari V.; Devenny, Darlynne A.; Johnson, Sterling C.; Lao, Patrick J.; Christian, Bradley T.

2017-01-01

The present study examined leisure activity and its association with caregiver involvement (i.e., residence and time spent with primary caregiver) in 62 middle-aged and older adults with Down syndrome (aged 30–53 years). Findings indicated that middle-aged and older adults with Down syndrome frequently participated in social and passive leisure activities, with low participation in physical and mentally stimulating leisure activities. Residence and time spent with primary caregiver were associated with participation in physical leisure activity. The findings suggest a need for support services aimed at increasing opportunities for participating in physical and mentally stimulating leisure activity by middle-aged and older adults with Down syndrome. These support services should partner with primary caregivers in order to best foster participation in physical leisure activity. PMID:28375797

Accelerometer-determined physical activity and walking capacity in persons with Down syndrome, Williams syndrome and Prader-Willi syndrome.

PubMed

Nordstrøm, Marianne; Hansen, Bjørge Herman; Paus, Benedicte; Kolset, Svein Olav

2013-12-01

In this study we describe by use of accelerometers the total physical activity (PA), intensity pattern and walking capacity in 87 persons age 16-45 years with Down syndrome (DS), Williams syndrome (WS) and Prader-Willi syndrome (PWS). Participants were recruited from all over Norway, and lived either with their parents or in community residences with support. On average the participants generated 294 counts per minute (cpm) or 6712 steps per day, with most of the day spent in sedentary activity, 522 min/day, followed by 212 min/day in light PA, 71 min/day in lifestyle activity and 27 min/day in moderate-to-vigorous physical activity (MVPA). Inactivity was prevalent, as only 12% meet the current Nordic recommendations for PA. When compared, no differences for total physical activity or time in MVPA were observed between the three groups. However, participant with DS spent a mean of 73 min/day less and 43 min/day less in sedentary activities compared to participants with PWS and WS, respectively, (p=0.011, 95% CI: -10.9; -80.1). In addition the DS-group spent a mean of 66 min/day more in light PA than the PWS-group and 41 min/day more than the WS-group, (p<0.001, 95% CI: 29.3; 79.7). Participants with PWS spent on average 30 min/day less in lifestyle activities compared to both participants with DS and WS, (p<0.001, 95% CI: -14.2; -45.4). No association between total PA and BMI were observed. Males were more active than females across all diagnoses. Males accumulated on average 85 counts per minutes more than females, (p=0.002, 95% CI: 33.3; 136.7), 2137 more steps per day, (p=0.002, 95% CI: 778; 3496). The mean walking capacity during six-minutes was 507 m (SD 112 m) for males and 466 m (SD 88 m) for females. Distance walked during testing decreased with 33.6 m when comparing normal or underweight participants to overweight participants, and 78.1 m when comparing overweight to obese participants (p<0.001 95% CI: -40.4; -85.8). When adjusted for BMI no differences in

Role of Mas receptor antagonist (A779) in renal hemodynamics in condition of blocked angiotensin II receptors in rats.

PubMed

Mansoori, A; Oryan, S; Nematbakhsh, M

2016-03-01

The vasodilatory effect of angiotensin 1-7 (Ang 1-7) is exerted in the vascular bed via Mas receptor (MasR) gender dependently. However, the crosstalk between MasR and angiotensin II (Ang II) types 1 and 2 receptors (AT1R and AT2R) may change some actions of Ang 1-7 in renal circulation. In this study by blocking AT1R and AT2R, the role of MasR in kidney hemodynamics was described. In anaesthetized male and female Wistar rats, the effects of saline as vehicle and MasR blockade (A779) were tested on mean arterial pressure (MAP), renal perfusion pressure (RPP), renal blood flow (RBF), and renal vascular resistance (RVR) when both AT1R and AT2R were blocked by losartan and PD123319, respectively. In male rats, when AT1R and AT2R were blocked, there was a tendency for the increase in RBF/wet kidney tissue weight (RBF/KW) to be elevated by A779 as compared with the vehicle (P=0.08), and this was not the case in female rats. The impact of MasR on renal hemodynamics appears not to be sexual dimorphism either when Ang II receptors were blocked. It seems that co-blockade of all AT1R, AT2R, and MasR may alter RBF/ KW in male more than in female rats. These findings support a crosstalk between MasR and Ang II receptors in renal circulation.

Physical Activity and Walking Onset in Infants with Down Syndrome

ERIC Educational Resources Information Center

Lloyd, Meghann; Burghardt, Amy; Ulrich, Dale A.; Angulo-Barroso, Rosa

2010-01-01

Infants with Down syndrome (DS) are described as being less active and they also experience significant delays in motor development. It is hypothesized that early infant physical activity may be influential for the acquisition of independent walking. Physical activity was monitored longitudinally in 30 infants with DS starting at an average age of…

Changes in objectively measured physical activity in adolescents with Down syndrome: the UP&DOWN longitudinal study.

PubMed

Izquierdo-Gomez, R; Martinez-Gómez, D; Esteban-Cornejo, I; Hallal, P C; García-Cervantes, L; Villagra, A; Veiga, O L

2017-04-01

It is a priority to understand that physical activity behaviour over time is a priority in Down syndrome population in order to design and promote succesfull interventions to maintain or increase levels of physical activity. We aimed to study 1 and 2-year changes in objectively measured physical activity among a relatively large sample of adolescents with Down syndrome. This study comprised a total of 99 adolescents with Down syndrome (38 girls) aged from 11 to 20 years old at baseline. Participants with valid accelerometer data at baseline and at least one of the follow-up visits were included in the analysis. Overall, levels of physical activity observed in adolescents with Down syndrome declined from baseline to follow-ups, but these changes were not significant (all P > 0.05). Moderate-to-moderately high tracking of physical activity was observed in adolescents with Down syndrome (all P < 0.001). Youths who met physical activity guidelines at baseline demonstrated a greater decline in physical activity in 1 and 2-year changes (P < 0.05), although they were also more likely to meet physical activity guidelines at 1 and 2-year follow-ups (P < 0.05). Adolescents with Down syndrome do not change their levels of physical activity at 2-year follow-ups, but those who met physical activity guidelines presented stronger declines in physical activity over time. © 2017 MENCAP and International Association of the Scientific Study of Intellectual and Developmental Disabilities and John Wiley & Sons Ltd.

Active smoking and risk of metabolic syndrome: a meta-analysis of prospective studies.

PubMed

Sun, Kan; Liu, Jianmin; Ning, Guang

2012-01-01

Epidemiological evidence suggests that smoking has been associated with emergence of metabolic syndrome. However, data on this issue are inconsistent and controversial. We therefore conducted a meta-analysis to examine the association between smoking and metabolic syndrome. We searched the Medline, Embase and the Cochrane Library database up to March 2012 to identify prospective cohort studies related to smoking and metabolic syndrome. Reference lists of retrieved articles were also reviewed. Summary effect estimates were derived using a random-effects model and stratified by gender, smoking dose, follow-up duration and geographical area. Primary analysis of 13 studies involving 56,691 participants and 8,688 cases detected a significant positive association between active smoking and risk of metabolic syndrome (pooled relative risk [RR] 1.26, 95% CI: 1.10-1.44). Estimates of effects were substantially consistent in the stratified analyses. In the dose-response analysis, risk of metabolic syndrome was stronger for active male smokers (pooled RR 1.34, 95% CI: 1.20-1.50) than it was for former male smokers (pooled RR 1.19, 95% CI: 1.00-1.42), and greater for heavy smokers (pooled RR 1.42, 95% CI: 1.27-1.59) compared with light smokers (pooled RR 1.10, 95% CI: 0.90-1.35). No evidence of statistical publication bias was found (Egger' s test P=0.227, Begg' s test P=0.113). Active smoking is associated with development of metabolic syndrome. Smoking cessation appears to reduce the risk of metabolic syndrome.

Prevalence and impact of active and passive cigarette smoking in acute respiratory distress syndrome.

PubMed

Hsieh, S Jean; Zhuo, Hanjing; Benowitz, Neal L; Thompson, B Taylor; Liu, Kathleen D; Matthay, Michael A; Calfee, Carolyn S

2014-09-01

Cigarette smoke exposure has recently been found to be associated with increased susceptibility to trauma- and transfusion-associated acute respiratory distress syndrome. We sought to determine 1) the incidence of cigarette smoke exposure in a diverse multicenter sample of acute respiratory distress syndrome patients and 2) whether cigarette smoke exposure is associated with severity of lung injury and mortality in acute respiratory distress syndrome. Analysis of the Albuterol for the Treatment of Acute Lung Injury and Omega Acute Respiratory Distress Syndrome Network studies. Acute Respiratory Distress Syndrome Network hospitals. Three hundred eighty-one patients with acute respiratory distress syndrome. None. 4-(Methylnitrosamino)-1-(3-pyridyl)-1-butanol, a validated tobacco-specific marker, was measured in urine samples from subjects enrolled in two National Heart, Lung, and Blood Institute Acute Respiratory Distress Syndrome Network randomized controlled trials. Urine 4-(methylnitrosamino)-1-(3-pyridyl)-1-butanol levels were consistent with active smoking in 36% of acute respiratory distress syndrome patients and with passive smoking in 41% of nonsmokers (vs 20% and 40% in general population, respectively). Patients with 4-(methylnitrosamino)-1-(3-pyridyl)-1-butanol levels in the active smoking range were younger and had a higher incidence of alcohol misuse, fewer comorbidities, lower severity of illness, and less septic shock at enrollment compared with patients with undetectable 4-(methylnitrosamino)-1-(3-pyridyl)-1-butanol levels. Despite this lower severity of illness, the severity of lung injury did not significantly differ based on biomarker-determined smoking status. Cigarette smoke exposure was not significantly associated with death after adjusting for differences in age, alcohol use, comorbidities, and severity of illness. In this first multicenter study of biomarker-determined cigarette smoke exposure in acute respiratory distress syndrome patients

Physical Activity Engagement in Young People with Down Syndrome: Investigating Parental Beliefs

ERIC Educational Resources Information Center

Alesi, Marianna; Pepi, Annamaria

2017-01-01

Background: Despite the wide documentation of the physical/psychological benefits derived from regular physical activity (PA), high levels of inactivity are reported among people with Down syndrome. Method: Semi-structured interviews were conducted with 13 parents of young people with Down syndrome. Results Three facilitation themes were…

Project MAS, 1982-1983. O.E.E. Evaluation Report.

ERIC Educational Resources Information Center

Villegas, Ana; Villegas, Jose

This multi-site instructional program, in its first year of a three-year funding cycle, provided instruction in English as a Second Language (ESL) and native language arts, as well as bilingual instruction in various content areas, to 400 Spanish speaking students of limited English proficiency in grades 3-8. The functional goal of Project MAS,…

Comparison of HR MAS MR spectroscopic profiles of breast cancer tissue with clinical parameters.

PubMed

Sitter, Beathe; Lundgren, Steinar; Bathen, Tone F; Halgunset, Jostein; Fjosne, Hans E; Gribbestad, Ingrid S

2006-02-01

Breast cancer is the most frequent form of cancer in women and improved diagnostic methods are desirable. Malignant cells have altered metabolism and metabolic mapping might become a tool in cancer diagnostics. High-resolution magic angle spinning (HR MAS) MR spectroscopy of tissue biopsies provides detailed information on metabolic composition. The 600 MHz 1H HR MAS spectra were acquired of breast cancer tissue from 85 patients and adjacent non-involved tissue from 18 of these patients. Tissue specimens were investigated by microscopy after MR analysis. The resulting spectra were examined by three different approaches. Relative intensities of glycerophosphocholine (GPC), phosphocholine (PC) and choline were compared for cancerous and non-involved specimens. Eight metabolites, choline, creatine, beta-glucose, GPC, glycine, myo-inositol, PC and taurine, were quantified from the recorded spectra and compared with tumor histological type and size, patient's lymph node status and tissue composition of sample. The spectra were also compared with tumor histological type and size, lymph node status and tissue composition of samples using principal component analysis (PCA). Tumor samples could be distinguished from non-involved samples (82% sensitivity, 100% specificity) based on relative intensities of signals from GPC, PC and choline in 1H HR MAS spectra. Tissue concentrations of metabolites showed few differences between groups of samples, which can be caused by limitations in the quantification procedure. Choline and glycine concentrations were found to be significantly higher in tumors larger than 2 cm compared with smaller tumors. PCA of MAS spectra from patients with invasive ductal carcinomas indicated a possible prediction of spread to axillary lymph nodes. Metabolite estimates and PCA of MAS spectra were influenced by the percentage of tumor cells in the investigated specimens. 2006 John Wiley & Sons, Ltd.

Disease Beyond the Arch: A Systematic Review of Middle Aortic Syndrome in Childhood.

PubMed

Rumman, Rawan K; Nickel, Cheri; Matsuda-Abedini, Mina; Lorenzo, Armando J; Langlois, Valerie; Radhakrishnan, Seetha; Amaral, Joao; Mertens, Luc; Parekh, Rulan S

2015-07-01

Middle aortic syndrome (MAS) is a rare clinical entity in childhood, characterized by a severe narrowing of the distal thoracic and/or abdominal aorta, and associated with significant morbidity and mortality. MAS remains a relatively poorly defined disease. This paper systematically reviews the current knowledge on MAS with respect to etiology, clinical impact, and therapeutic options. A systematic search of 3 databases (Embase, MEDLINE, and Cochrane Central Register of Controlled Trials) yielded 1,252 abstracts that were screened based on eligibility criteria resulting in 184 full-text articles with 630 reported cases of childhood MAS. Data extracted included patient characteristics, clinical presentation, vascular phenotype, management, and outcomes. Most cases of MAS are idiopathic (64%), 15% are associated with Mendelian disorders, and 17% are related to inflammatory diseases. Extra-aortic involvement including renal (70%), superior mesenteric (30%), and celiac (22%) arteries is common, especially among those with associated Mendelian disorders. Inferior mesenteric artery involvement is almost never reported. The majority of cases (72%) undergo endovascular or surgical management with residual hypertension reported in 34% of cases, requiring medication or reintervention. Clinical manifestations and extent of extra-aortic involvement are lacking. MAS presents with significant involvement of visceral arteries with over two thirds of cases having renal artery stenosis, and one third with superior mesenteric artery stenosis. The extent of disease is worse among those with genetic and inflammatory conditions. Further studies are needed to better understand etiology, long-term effectiveness of treatment, and to determine the optimal management of this potentially devastating condition. © American Journal of Hypertension, Ltd 2015. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Optimized Spectral Editing of 13C MAS NMR Spectra of Rigid Solids Using Cross-Polarization Methods

NASA Astrophysics Data System (ADS)

Sangill, R.; Rastrupandersen, N.; Bildsoe, H.; Jakobsen, H. J.; Nielsen, N. C.

Combinations of 13C magic-angle spinning (MAS) NMR experiments employing cross polarization (CP), cross polarization-depolarization (CPD), and cross polarization-depolarization-repolarization are analyzed quantitatively to derive simple and general procedures for optimized spectral editing of 13C CP/MAS NMR spectra of rigid solids by separation of the 13C resonances into CH n subspectra ( n = 0, 1, 2, and 3). Special attention is devoted to a differentiation by CPD/MAS of CH and CH 2 resonances since these groups behave quite similarly during spin lock under Hartmann-Hahn match and are therefore generally difficult to distinguish unambiguously. A general procedure for the design of subexperiments and linear combinations of their spectra to provide optimized signal-to-noise ratios for the edited subspectra is described. The technique is illustrated by a series of edited 13C CP/MAS spectra for a number of rigid solids ranging from simple organic compounds (sucrose and l-menthol) to complex pharmaceutical products (calcipotriol monohydrate and vitamin D 3) and polymers (polypropylene, polyvinyl alcohol, polyvinyl chloride, and polystyrene).

Identifying risk factors of immune reconstitution inflammatory syndrome in AIDS patients receiving highly active anti-retroviral therapy.

PubMed

He, Bo; Zheng, Yuhuang; Liu, Meng; Zhou, Guoqiang; Chen, Xia; Mamadou, Diallo; He, Yan; Zhou, Huaying; Chen, Zi

2013-01-01

Immune reconstitution inflammation syndrome typically occurs within days after patients undergo highly active anti-retroviral therapy and is a big hurdle for effective treatment of AIDS patients. In this study, we monitored immune reconstitution inflammation syndrome occurrence in 238 AIDS patients treated with highly active anti-retroviral therapy. Among them, immune reconstitution inflammation syndrome occurred in 47 cases (19.7%). Immune reconstitution inflammation syndrome patients had significantly higher rate of opportunistic infection (p<0.001) and persistently lower CD4(+) cell count (p<0.001) compared to the non-immune reconstitution inflammation syndrome patients. In contrast, no significant differences in HIV RNA loads were observed between the immune reconstitution inflammation syndrome group and non-immune reconstitution inflammation syndrome group. These data suggest that a history of opportunistic infection and CD4(+) cell counts at baseline may function as risk factors for immune reconstitution inflammation syndrome occurrence in AIDS patients as well as potential prognostic markers. These findings will improve the management of AIDS with highly active anti-retroviral therapy. Copyright © 2013 Elsevier Editora Ltda. All rights reserved.

Unlikely culprit: congenital middle aortic syndrome diagnosed in the sixth decade of life.

PubMed

Ali, Muhammad Sajawal; Tchernodrinski, Stefan; Mohananey, Divyanshu; Ali, Ahya Sajawal

2016-08-16

A 58-year-old woman was admitted with acute heart failure. She had a long history of resistant hypertension, with an unremarkable work up for secondary causes in the past. Her brachial blood pressure was 210/70 mm Hg, with ankle blood pressure of 100/70 mm Hg. CT angiogram revealed marked narrowing of the descending thoracic aorta between the left subclavian artery and the diaphragm, consistent with middle aortic syndrome (MAS). She was initially managed with diuretics and antihypertensives. Subsequently thoracotomy revealed a severely hypoplastic segment of the descending aorta. The diseased segment was resected and aortic reconstruction performed. Histopathology showed fragmentation of the medial elastic fibres and fibrosis of the medial and intimal layers. These findings along with gross aortic hypoplasia and absence of features of Takayasu's arteritis, suggest that our patient had congenital MAS. The patient has done well since her surgery. We believe this is the first case of congenital MAS reported in the sixth decade of life. 2016 BMJ Publishing Group Ltd.

Brattleboro Rats as the Model of Blood Hyperviscosity Syndrome for Testing Substances with Hemorheological Activity.

PubMed

Plotnikov, M B; Vasil'ev, A S; Aliev, O I; Zibareva, L N

2015-09-01

Hyperviscosity syndrome was described in Brattleboro rats. The aim of this study was to investigate the possibility of Brattleboro rats using, as a test system for the study of agents with hemorheological activity. Under conditions of this model of high blood viscosity syndrome in Brattleboro rats, Lychnis chalcedonica L. extract (150 mg/kg) administered intragastrically for 10 days exhibited hemorheological activity by modulating macro- (plasma viscosity, fibrinogen concentration) and microrheological (erythrocyte aggregation and deformability parameters. Hence, Brattleboro rats are an adequate model of hyperviscosity syndrome that can be used for search and testing of substances with hemorheological activity.

Antioxidant capacity of cornelian cherry (Cornus mas L.) - comparison between permanganate reducing antioxidant capacity and other antioxidant methods.

PubMed

Popović, Boris M; Stajner, Dubravka; Slavko, Kevrešan; Sandra, Bijelić

2012-09-15

Ethanol extracts (80% in water) of 10 cornelian cherry (Cornus mas L.) genotypes were studied for antioxidant properties, using methods including DPPH(), ()NO, O(2)(-) and ()OH antiradical powers, FRAP, total phenolic and anthocyanin content (TPC and ACC) and also one relatively new, permanganate method (permanganate reducing antioxidant capacity-PRAC). Lipid peroxidation (LP) was also determined as an indicator of oxidative stress. The data from different procedures were compared and analysed by multivariate techniques (correlation matrix calculation and principal component analysis (PCA)). Significant positive correlations were obtained between TPC, ACC and DPPH(), ()NO, O(2)(-), and ()OH antiradical powers, and also between PRAC and TPC, ACC and FRAP. PCA found two major clusters of cornelian cherry, based on antiradical power, FRAP and PRAC and also on chemical composition. Chemometric evaluation showed close interdependence between PRAC method and FRAP and ACC. There was a huge variation between C. mas genotypes in terms of antioxidant activity. Copyright © 2012 Elsevier Ltd. All rights reserved.

48 CFR 538.270 - Evaluation of multiple award schedule (MAS) offers.

Code of Federal Regulations, 2010 CFR

2010-10-01

... SERVICES ADMINISTRATION SPECIAL CATEGORIES OF CONTRACTING FEDERAL SUPPLY SCHEDULE CONTRACTING Establishing and Administering Federal Supply Schedules 538.270 Evaluation of multiple award schedule (MAS) offers... determining the Government's price negotiation objectives, consider the following factors: (1) Aggregate...

The syndrome of `continuous muscle-fibre activity' cured: further studies

PubMed Central

Isaacs, Hyam; Heffron, J. J. A.

1974-01-01

Two cases suffering from the syndrome of `continuous muscle-fibre activity' have been followed-up for 14 years. These patients have gradually gone into remission and no longer require therapy. The results of recent histology, histochemistry, and electronmicroscopy, as well as sural nerve biopsy studies, are presented. The sarcoplasmic reticulum calcium binding activity and ATPase activity are normal. Images PMID:4281819

Metabolic profiles of placenta in preeclampsia using HR-MAS MRS metabolomics.

PubMed

Austdal, Marie; Thomsen, Liv Cecilie Vestrheim; Tangerås, Line Haugstad; Skei, Bente; Mathew, Seema; Bjørge, Line; Austgulen, Rigmor; Bathen, Tone Frost; Iversen, Ann-Charlotte

2015-12-01

Preeclampsia is a heterogeneous gestational disease characterized by maternal hypertension and proteinuria, affecting 2-7% of pregnancies. The disorder is initiated by insufficient placental development, but studies characterizing the placental disease components are lacking. Our aim was to phenotype the preeclamptic placenta using high-resolution magic angle spinning nuclear magnetic resonance spectroscopy (HR-MAS MRS). Placental samples collected after delivery from women with preeclampsia (n = 19) and normotensive pregnancies (n = 15) were analyzed for metabolic biomarkers including amino acids, osmolytes, and components of the energy and phospholipid metabolism. The metabolic biomarkers were correlated to clinical characteristics and inflammatory biomarkers in the maternal sera. Principal component analysis showed inherent differences in placental metabolic profiles between preeclamptic and normotensive pregnancies. Significant differences in metabolic profiles were found between placentas from severe and non-severe preeclampsia, but not between preeclamptic pregnancies with fetal growth restricted versus normal weight neonates. The placental metabolites correlated with the placental stress marker sFlt-1 and triglycerides in maternal serum, suggesting variation in placental stress signaling between different placental phenotypes. HR-MAS MRS is a sensitive method for defining the placental disease component of preeclampsia, identifying several altered metabolic pathways. Placental HR-MAS MRS analysis may improve insight into processes affected in the preeclamptic placenta, and represents a novel long-required tool for a sensitive placental phenotyping of this heterogeneous disease. Copyright © 2015 Elsevier Ltd. All rights reserved.

The metabolic profile of lemon juice by proton HR-MAS NMR: the case of the PGI Interdonato Lemon of Messina.

PubMed

Cicero, Nicola; Corsaro, Carmelo; Salvo, Andrea; Vasi, Sebastiano; Giofré, Salvatore V; Ferrantelli, Vincenzo; Di Stefano, Vita; Mallamace, Domenico; Dugo, Giacomo

2015-01-01

We have studied by means of High Resolution Magic Angle Spinning Nuclear Magnetic Resonance (HR-MAS NMR) the metabolic profile of the famous Sicilian lemon known as 'Interdonato Lemon of Messina PGI'. The PGI Interdonato Lemon of Messina possesses high organoleptic and healthy properties and is recognised as one of the most nutrient fruits. In particular, some of its constituents are actively studied for their chemo-preventive and therapeutic properties. In this paper, we have determined by means of HR-MAS NMR spectroscopy the molar concentration of the main metabolites constituent the juice of PGI Interdonato Lemon of Messina in comparison with that of the not-PGI Interdonato Lemon of Turkey. Our aim is to develop an analytical technique, in order to determine a metabolic fingerprint able to reveal commercial frauds in national and international markets.

Magnesium deficiency and metabolic syndrome: stress and inflammation may reflect calcium activation.

PubMed

Rayssiguier, Yves; Libako, Patrycja; Nowacki, Wojciech; Rock, Edmond

2010-06-01

Magnesium (Mg) intake is inadequate in the western diet and metabolic syndrome is highly prevalent in populations around the world. Epidemiological studies suggest that high Mg intake may reduce the risk but the possibility of confounding factors exists, given the strong association between Mg and other beneficial nutriments (vegetables, fibers, cereals). The concept that metabolic syndrome is an inflammatory condition may explain the role of Mg.Mg deficiency results in a stress effect and increased susceptibility to physiological damage produced by stress. Stress activates the hypothalamic-pituitary-adrenal axis (HPA) axis and the sympathetic nervous system. The activation of the renin-angiotensin-aldosterone system is a factor in the development of insulin resistance by increasing oxidative stress. In both humans and rats, aldosteronism results in an immunostimulatory state and leads to an inflammatory phenotype. Stress response induces the release of large quantities of excitatory amino acids and activates the nuclear factor NFkappaB, promoting translation of molecules involved in cell regulation, metabolism and apoptosis. The rise in neuropeptides is also well documented. Stress-induced HPA activation has been identified to play an important role in the preferential body fat accumulation but evidence that Mg is involved in body weight regulation is lacking. One of the earliest events in the acute response to stress is endothelial dysfunction. Endothelial cells actively contribute to inflammation by elaborating cytokines, synthesizing chemical mediators and expressing adhesion molecules. Experimental Mg deficiency in rats induces a clinical inflammatory syndrome characterized by leukocyte and macrophage activation, synthesis of inflammatory cytokines and acute phase proteins, extensive production of free radicals. An increase in extracellular Mg concentration decreases inflammatory effects, while reduction in extracellular Mg results in cell activation. The

HR-MAS MR Spectroscopy of Breast Cancer Tissue Obtained with Core Needle Biopsy: Correlation with Prognostic Factors

PubMed Central

Choi, Ji Soo; Baek, Hyeon-Man; Kim, Suhkmann; Kim, Min Jung; Youk, Ji Hyun; Moon, Hee Jung; Kim, Eun-Kyung; Han, Kyung Hwa; Kim, Dong-hyun; Kim, Seung Il; Koo, Ja Seung

2012-01-01

The purpose of this study was to examine the correlation between high-resolution magic angle spinning (HR-MAS) magnetic resonance (MR) spectroscopy using core needle biopsy (CNB) specimens and histologic prognostic factors currently used in breast cancer patients. After institutional review board approval and informed consent were obtained for this study, CNB specimens were collected from 36 malignant lesions in 34 patients. Concentrations and metabolic ratios of various choline metabolites were estimated by HR-MAS MR spectroscopy using CNB specimens. HR-MAS spectroscopic values were compared according to histopathologic variables [tumor size, lymph node metastasis, histologic grade, status of estrogens receptor (ER), progesterone receptor (PR), HER2 (a receptor for human epidermal growth factor), and Ki-67, and triple negativity]. Multivariate analysis was performed with Orthogonal Projections to Latent Structure-Discriminant Analysis (OPLS-DA). HR-MAS MR spectroscopy quantified and discriminated choline metabolites in all CNB specimens of the 36 breast cancers. Several metabolite markers [free choline (Cho), phosphocholine (PC), creatine (Cr), taurine, myo-inositol, scyllo-inositol, total choline (tCho), glycine, Cho/Cr, tCho/Cr, PC/Cr] on HR-MAS MR spectroscopy were found to correlate with histologic prognostic factors [ER, PR, HER2, histologic grade, triple negativity, Ki-67, poor prognosis]. OPLS-DA multivariate models were generally able to discriminate the status of histologic prognostic factors (ER, PR, HER2, Ki-67) and prognosis groups. Our study suggests that HR-MAS MR spectroscopy using CNB specimens can predict tumor aggressiveness prior to surgery in breast cancer patients. In addition, it may be helpful in the detection of reliable markers for breast cancer characterization. PMID:23272149

27 Al MAS NMR Studies of HBEA Zeolite at Low to High Magnetic Fields

DOE Office of Scientific and Technical Information (OSTI.GOV)

Hu, Jian Zhi; Wan, Chuan; Vjunov, Aleksei

27Al single pulse (SP) MAS NMR spectra of HBEA zeolites with high Si/Al ratios of 71 and 75 were obtained at three magnetic field strengths of 7.05, 11.75 and 19.97 T. High field 27Al MAS NMR spectra acquired at 19.97 T show significantly improved spectral resolution, resulting in at least two well-resolved tetrahedral-Al NMR peaks. Based on the results obtained from 27Al MAS and MQMAS NMR acquired at 19.97 T, four different quadrupole peaks are used to deconvolute the 27Al SP MAS spectra acquired at vari-ous fields by using the same set of quadrupole coupling constants, asymmetric parameters and relativemore » integrated peak intensities for the tetrahedral Al peaks. The line shapes of individual peaks change from typical quadrupole line shape at low field to essentially symmetrical line shapes at high field. We demonstrate that for fully hydrated HBEA zeolites the effect of second order quadrupole interaction can be ignored and quantitative spectral analysis can be performed by directly fitting the high field spectra using mixed Gaussian/Lorentzian line shapes. Also, the analytical steps described in our work allow direct assignment of spectral intensity to individual Al tetrahedral sites (T-sites) of zeolite HBEA. Finally, the proposed concept is suggested generally applicable to other zeo-lite framework types, thus, allowing a direct probing of Al distributions by NMR spectroscopic methods in zeolites with high confi-dence.« less

High field 27Al MAS NMR and TPD studies of active sites in ethanol dehydration using thermally treated transitional aluminas as catalysts

DOE Office of Scientific and Technical Information (OSTI.GOV)

Hu, Jian Zhi; Xu, Suochang; Kwak, Ja Hun

Gamma-, sigma- and theta-Al2O3 are well known metastable “transitional” alumina structural polymorphs. Upon heating, Al2O3 transitions to the so-called and Al2O3 polymorphs and finally forms the thermally stable Al2O3. The poorly developed crystallinity and co-existence of the , , and Al2O3 prior to forming all Al2O3, making it difficult to characterize the structures as well as to quantify the various phases of the transition alumina. As a result, there are significant controversies in the literatures. In this work, a detailed NMR analysis was carried out at high magnetic field on three special aluminum oxide samples where the, , , Al2O3more » phases are made dominant, respectively, by controlling the synthesis conditions. The goal is to simplify, including making unambiguous, spectral assignments in 27Al MAS NMR spectra of transition alumina that have not yet been commonly agreed previously. Specifically, quantitative 1D 27Al MAS NMR was used to quantify the ratios of the different alumina structural units, 2D MQMAS 27Al MAS was used for obtaining the highest spectral resolution to guide the analysis of the 1D spectrum, and a saturation pulse sequence was integrated into the 1D NMR to select the amorphous structures, including obtain spectra where the penta-coordinate sites are observed with enhanced relative intensity. Collectively, this study uniquely assigns Al-peaks (both octahedral and tetrahedral) to the Al2O3 and the Al2O3 phases and offers a new way of understanding, including quantifying, the different structural units and sites in transition alumina samples.« less

Association between physical activity and metabolic syndrome: a cross sectional survey in adolescents in Ho Chi Minh City, Vietnam.

PubMed

Nguyen, Trang H H D; Tang, Hong K; Kelly, Patrick; van der Ploeg, Hidde P; Dibley, Michael J

2010-03-17

The emerging epidemic of overweight/obesity in adolescents in Ho Chi Minh City, Vietnam underlines the importance of studying the metabolic syndrome in Vietnamese adolescents who are becoming progressively more inactive. No study in Vietnam has examined the association of metabolic syndrome with moderate to vigorous physical activity (PA) levels among adolescents. We aimed to examine this association in a sample of urban adolescents from Ho Chi Minh City. A cross-sectional assessment was conducted in 2007 on a representative sample of 693 high-school students from urban districts in Ho Chi Minh City. Metabolic syndrome was defined according to the International Diabetes Federation criteria and physical activity was measured with Actigraph accelerometers. The association between physical activity and metabolic syndrome was assessed by using multiple logistic regression models. Overall 4.6% of the adolescents and 11.8% of the overweight/obese adolescents had metabolic syndrome. Elevated BP was the most common individual component of the metabolic syndrome (21.5%), followed by hypertriglyceridemia (11.1%). After adjusting for other study factors, the odds of metabolic syndrome among youth in the lowest physical activity group (<43 minutes of physical activity/day) were five times higher than those in the highest physical activity group (>103 minutes/day) (AOR = 5.3, 95% CI: 1.5, 19.1). Metabolic syndrome was also positively associated with socioeconomic status (AOR = 9.4, 95% CI: 2.1, 42.4). A more physically active lifestyle appears to be associated with a lower odds of metabolic syndrome in Vietnamese adolescents. Socio-economic status should be taken into account when planning interventions to prevent adolescent metabolic syndrome.

Studies of minute quantities of natural abundance molecules using 2D heteronuclear correlation spectroscopy under 100kHz MAS

DOE PAGES

Nishiyama, Y.; Kobayashi, T.; Malon, M.; ...

2015-02-16

Two-dimensional 1H{ 13C} heteronuclear correlation solid-state NMR spectra of naturally abundant solid materials are presented, acquired using the 0.75-mm magic angle spinning (MAS) probe at spinning rates up to 100 kHz. In spite of the miniscule sample volume (290 nL), high-quality HSQC-type spectra of bulk samples as well as surface-bound molecules can be obtained within hours of experimental time. The experiments are compared with those carried out at 40 kHz MAS using a 1.6-mm probe, which offered higher overall sensitivity due to a larger rotor volume. The benefits of ultrafast MAS in such experiments include superior resolution in 1H dimensionmore » without resorting to 1H– 1H homonuclear RF decoupling, easy optimization, and applicability to mass-limited samples. As a result, the HMQC spectra of surface-bound species can be also acquired under 100 kHz MAS, although the dephasing of transverse magnetization has significant effect on the efficiency transfer under MAS alone.« less

Recent Results from the MicroMAS Global Environmental MonitoringNanosatellite Mission

NASA Astrophysics Data System (ADS)

Blackwell, W. J.; Cahoy, K.

2014-12-01

The Micro-sized Microwave Atmospheric Satellite (MicroMAS) is a dual-spinning 3U CubeSat equipped with apassive microwave radiometer that observes in nine channels near the 118.75-GHz oxygen absorption line.MicroMAS is designed to observe convective thunderstorms, tropical cyclones, and hurricanes from a midinclinationorbit. The MicroMAS flight unit was developed by MIT Lincoln Laboratory and the MIT Space SystemsLaboratory and was launched to the International Space Station on July 13, 2014, and scheduled for an earlySeptember deployment for a ~90-day mission. The payload is housed in the "lower" 1U of the dual-spinning 3UCubeSat and mechanically rotated approximately once per second as the spacecraft orbits the Earth, resulting in across-track scanned beam with a full-width half-max (FWHM) beamwidth of 2.4 degrees and an approximately 17-km diameter footprint at nadir incidence from a nominal altitude of 400 km. The relatively low cost of MicroMASenables the deployment of a constellation of sensors, spaced equally around several orbit planes. A small fleet ofMicroMAS systems could yield high-resolution global temperature and water vapor profiles, as well as cloudmicrophysical and precipitation parameters.Significant advancements were made in the Assembly, Integration, and Test phase of the project developmentlifecycle. The flight software and communications architecture was refined and tested in relevant lab facilities. Thepower subsystem was modified to include additional required inhibits for the ISS launch. Hardware in the loop testsas well as simulations of the attitude determination and control system (ADCS) were performed to validate theunique dual-spinning, local vertical, local horizontal (LVLH) stabilized flight design. ADCS algorithms were testedon a 3-axis air bearing and custom rig inside a 3-axis programmable Helmholtz cage. Finally, the integratedspacecraft underwent a series of environmental tests in order to verify the results of thermal modeling

Parents' Perceptions of Health and Physical Activity Needs of Children with Down Syndrome

ERIC Educational Resources Information Center

Menear, Kristi Sayers

2007-01-01

Individuals with Down syndrome typically have low fitness levels and obesity despite data that indicate physiological gains from physical activity and exercise interventions. Low fitness levels and obesity in individuals with Down syndrome may be related to sedentary lifestyles, social and recreational opportunities, or low motivation to be…

Physical activity and the risk of colorectal cancer in Lynch syndrome.

PubMed

Dashti, S Ghazaleh; Win, Aung Ko; Hardikar, Sheetal S; Glombicki, Stephen E; Mallenahalli, Sheila; Thirumurthi, Selvi; Peterson, Susan K; You, Y Nancy; Buchanan, Daniel D; Figueiredo, Jane C; Campbell, Peter T; Gallinger, Steven; Newcomb, Polly A; Potter, John D; Lindor, Noralane M; Le Marchand, Loic; Haile, Robert W; Hopper, John L; Jenkins, Mark A; Basen-Engquist, Karen M; Lynch, Patrick M; Pande, Mala

2018-06-14

Greater physical activity is associated with a decrease in risk of colorectal cancer for the general population; however, little is known about its relationship with colorectal cancer risk for people with Lynch syndrome, carriers of inherited pathogenic mutations in genes affecting DNA mismatch repair (MMR). We studied a cohort of 2,042 MMR gene mutations carriers (n=807, diagnosed with colorectal cancer), from the Colon Cancer Family Registry. Self-reported physical activity in three age-periods (20-29, 30-49, and ≥50 years) was summarized as average metabolic equivalent of task hours per week (MET-h/week) during the age-period of cancer diagnosis or censoring (near-term exposure), and across all age-periods preceding cancer diagnosis or censoring (long-term exposure). Weighted Cox regression was used to estimate the hazard ratio (HR) and 95% confidence intervals (CI) for the association between physical activity and colorectal cancer risk. Near-term physical activity was associated with a small reduction in the risk of colorectal cancer (HR ≥35 vs. <3.5 MET-h/week, 0.71; 95% CI, 0.53 - 0.96). The strength and direction of associations were similar for long-term physical activity, although the associations were not nominally significant. Our results suggest that physical activity is inversely associated with the risk of colorectal cancer for people with Lynch syndrome, however, further confirmation is warranted. The potential modifying effect of physical activity on colorectal cancer risk for people with Lynch syndrome could be useful for risk prediction and support counseling advice for lifestyle modification to reduce cancer risk. This article is protected by copyright. All rights reserved. © 2018 UICC.

Identifying the Barriers and Facilitators to Participation in Physical Activity for Children with Down Syndrome

ERIC Educational Resources Information Center

Barr, M.; Shields, N.

2011-01-01

Background: Many children with Down syndrome do not undertake the recommended amount of daily physical activity. The aim of this study was to explore the barriers and facilitators to physical activity for this group. Methods: Eighteen in-depth interviews were conducted with 20 parents (16 mothers, 4 fathers) of children with Down syndrome aged…

Role of Mas receptor in renal blood flow response to angiotensin-(1-7) in ovariectomized estradiol treated rats.

PubMed

Saberi, Shadan; Dehghani, Aghdas; Nematbakhsh, Mehdi

2016-01-01

The angiotensin 1-7 (Ang 1-7), is abundantly produced in kidneys and antagonizes the function of angiotensin II through Mas receptor (MasR) or other unknown mechanisms. In the current study, the role of MasR and steroid hormone estrogen on renal blood flow response to Ang 1-7 administration was investigated in ovariectomized (OV) female rats. OV female Wistar-rats received estradiol (500 μg/kg/week) or vehicle for two weeks. In the day of the experiment, the animals were anesthetized, cannulated, and the responses including mean arterial pressure, renal blood flow (RBF), and renal vascular resistance at the constant level of renal perfusion pressure to graded infusion of Ang 1-7 at 0, 100 and 300 ng/kg/min were determined in OV and OV estradiol-treated (OVE) rats, treated with vehicle or MasR antagonist; A779. RBF response to Ang 1-7 infusion increased dose-dependently in vehicle (Pdose <0.001) and A779-treated (Pdose <0.01) animals. However, when MasR was blocked, the RBF response to Ang 1-7 significantly increased in OV animals compared with OVE rats (P<0.05). When estradiol was limited by ovariectomy, A779 increased RBF response to Ang 1-7 administration, while this response was attenuated in OVE animals.

The demands and benefits of ergonomics in Sri Lankan apparel industry: A case study at MAS holdings.

PubMed

Abeysekera, John; Illankoon, Prasanna

2016-10-17

Apparel exports bring in sizeable foreign income to Sri Lanka. To protect and promote this industry is a paramount need. This can be carried out by applying Human Factors/Ergonomics (HFE) which has proved to control negative effects at work places. This paper reports a case study which describes the demands and benefits of HFE in MAS Holdings which owns a large share of the apparel industry in Sri Lanka. The study consisted of walk through observation survey, a questionnaire survey and ergonomic work place analysis followed by a training programme to selected employees in three companies. Positive responses to questionnaires revealed good ergonomic practices in the work places surveyed. Ergonomically unfit chairs and potential hazards e.g. exposure to noise and hot environment were detected. It is seen that MAS have introduced strategies originated by Toyota Production System viz. 5S, Kaizen, six sigma etc., which are in fact ergonomic methods. A progressive project MAS boast of viz. 'MAS Operating System' (MOS) empowers training and development to employees. MAS Holdings has adequately realized the benefits of applying HFE as evident by the number of awards received. Relevant companies were advised to take appropriate corrective measures to control the potential hazards.

1H MAS NMR (magic-angle spinning nuclear magnetic resonance) techniques for the quantitative determination of hydrogen types in solid catalysts and supports.

PubMed

Kennedy, Gordon J; Afeworki, Mobae; Calabro, David C; Chase, Clarence E; Smiley, Randolph J

2004-06-01

Distinct hydrogen species are present in important inorganic solids such as zeolites, silicoaluminophosphates (SAPOs), mesoporous materials, amorphous silicas, and aluminas. These H species include hydrogens associated with acidic sites such as Al(OH)Si, non-framework aluminum sites, silanols, and surface functionalities. Direct and quantitative methodology to identify, measure, and monitor these hydrogen species are key to monitoring catalyst activity, optimizing synthesis conditions, tracking post-synthesis structural modifications, and in the preparation of novel catalytic materials. Many workers have developed several techniques to address these issues, including 1H MAS NMR (magic-angle spinning nuclear magnetic resonance). 1H MAS NMR offers many potential advantages over other techniques, but care is needed in recognizing experimental limitations and developing sample handling and NMR methodology to obtain quantitatively reliable data. A simplified approach is described that permits vacuum dehydration of multiple samples simultaneously and directly in the MAS rotor without the need for epoxy, flame sealing, or extensive glovebox use. We have found that careful optimization of important NMR conditions, such as magnetic field homogeneity and magic angle setting are necessary to acquire quantitative, high-resolution spectra that accurately measure the concentrations of the different hydrogen species present. Details of this 1H MAS NMR methodology with representative applications to zeolites, SAPOs, M41S, and silicas as a function of synthesis conditions and post-synthesis treatments (i.e., steaming, thermal dehydroxylation, and functionalization) are presented.

Assessment of Objectively Measured Physical Activity Levels in Individuals with Intellectual Disabilities with and without Down's Syndrome

PubMed Central

Phillips, Alexander C.; Holland, Anthony J.

2011-01-01

Objective To investigate, using accelerometers, the levels of physical activity being undertaken by individuals with intellectual disabilities with and without Down's syndrome. Methods One hundred and fifty two individuals with intellectual disabilities aged 12–70 years from East and South-East England. Physical activity levels in counts per minute (counts/min), steps per day (steps/day), and minutes of sedentary, light, moderate, vigorous, and moderate to vigorous physical activity (MVPA) measured with a uni-axial accelerometer (Actigraph GT1M) for seven days. Results No individuals with intellectual disabilities met current physical activity recommendations. Males were more active than females. There was a trend for physical activity to decline and sedentary behaviour to increase with age, and for those with more severe levels of intellectual disability to be more sedentary and less physically active, however any relationship was not significant when adjusted for confounding variables. Participants with Down's syndrome engaged in significantly less physical activity than those with intellectual disabilities without Down's syndrome and levels of activity declined significantly with age. Conclusions Individuals with intellectual disabilities, especially those with Down's syndrome may be at risk of developing diseases associated with physical inactivity. There is a need for well-designed, accessible, preventive health promotion strategies and interventions designed to raise the levels of physical activity for individuals with intellectual disabilities. We propose that there are physiological reasons why individuals with Down's syndrome have particularly low levels of physical activity that also decline markedly with age. PMID:22205957

Bronchoalveolar lavage with diluted porcine surfactant in mechanically ventilated term infants with meconium aspiration syndrome.

PubMed

Lista, Gianluca; Bianchi, Silvia; Castoldi, Francesca; Fontana, Paola; Cavigioli, Francesco

2006-01-01

To evaluate the efficacy and safety of bronchoalveolar lavage (BAL) with diluted porcine surfactant in mechanically ventilated term infants with severe acute respiratory distress syndrome (ARDS) due to meconium aspiration syndrome (MAS). Eight consecutive mechanically ventilated term infants with severe ARDS due to MAS underwent BAL with 15 mL/kg of diluted (5.3mg phospholipid/mL) surfactant saline suspension (porcine surfactant [Curosurf]). Treatment was administered slowly in aliquots of 2.5 mL. The mean age of neonates at treatment was 3.5 (range 1-8) hours. Heart rate, systemic blood pressure and oxygen saturation were monitored continuously. Arterial blood gases were measured immediately before treatment, and again at 3 and 6 hours post-treatment. Chest x-rays were taken 6 and 24 hours after treatment. Radiological improvement was evident in all eight patients 6 hours post-treatment. Compared with pre-BAL values, significant improvements (p < 0.05) in mean values for partial pressure of oxygen in arterial blood, partial pressure of carbon dioxide in arterial blood, pH, arterial/alveolar O2 ratio and oxygenation index were documented at 3 and 6 hours after BAL. In all patients, tracheal fluids that had been meconium-stained prior to BAL were clear of meconium after BAL. Only one patient required nitric oxide therapy for transient pulmonary hypertension. No adverse sequelae of treatment occurred during the study. BAL with dilute porcine surfactant administered slowly in 2.5 mL aliquots improved oxygenation and chest x-ray findings, without causing major adverse effects, in mechanically ventilated term infants with ARDS due to MAS.

Hydration properties of regioselectively etherified celluloses monitored by 2H and 13C solid-state MAS NMR spectroscopy.

PubMed

Larsen, Flemming H; Schöbitz, Michael; Schaller, Jens

2012-06-20

The hydration properties of 2,3-O-hydroxypropylcellulose (HPC) and 2,3-O-hydroxyethylcellulose (HEC) were analyzed by multi-nuclear solid-state MAS NMR spectroscopy. By 13C single-pulse (SP) MAS and cross-polarization (CP) MAS NMR, differences between the immobile regions and all parts of the polysaccharides were detected as a function of hydration. Complementary information about the water environments was observed by 2H MAS NMR. By this approach it was demonstrated that side chains in 2,3-O-HPC and 2,3-O-HEC were easier to hydrate than the cellulose backbone. Furthermore the motion of water was more restricted (slower) in 2,3-O-HPC than in 2,3-O-HEC. For both polysaccharides the hydration could be explained by a two-step process: in step one increased ordering of the immobile regions occurs after which the entire polymer is hydrated in step two. Copyright © 2012 Elsevier Ltd. All rights reserved.

An exploratory study of the association between physical activity, cardiovascular fitness and body size in children with Down syndrome.

PubMed

Shields, Nora; Hussey, Juliette; Murphy, Joan; Gormley, John; Hoey, Hilary

2017-02-01

To explore the association between physical activity, cardiovascular fitness and body size among children with Down syndrome. Physical activity, cardiovascular fitness and body size were measured by accelerometry, maximal fitness test and anthropometric measurements (BMI, waist circumference), respectively. Fourteen children with Down syndrome (8 boys, 6 girls; mean age 12.9 years) participated. There was no significant correlation between physical activity and cardiovascular fitness or physical activity and body size. Children with Down syndrome who were fitter, had lower BMIs (r = -0.77, 95% confidence interval (CI) -0.41 to -0.93) and smaller waist circumference (r = -0.75, 95% CI -0.36 to -0.92). Preliminary evidence suggests physical activity may not be associated with either body size or fitness in children with Down syndrome. Body size appears to be inversely related to fitness in children with Down syndrome.

Differential renal effects of candesartan at high and ultra-high doses in diabetic mice–potential role of the ACE2/AT2R/Mas axis

PubMed Central

Callera, Glaucia E.; Antunes, Tayze T.; Correa, Jose W.; Moorman, Danielle; Gutsol, Alexey; He, Ying; Cat, Aurelie Nguyen Dinh; Briones, Ana M.; Montezano, Augusto C.; Burns, Kevin D.; Touyz, Rhian M.

2016-01-01

High doses of Ang II receptor (AT1R) blockers (ARBs) are renoprotective in diabetes. Underlying mechanisms remain unclear. We evaluated whether high/ultra-high doses of candesartan (ARB) up-regulate angiotensin-converting enzyme 2 (ACE2)/Ang II type 2 receptor (AT2R)/Mas receptor [protective axis of the of the renin–angiotensin system (RAS)] in diabetic mice. Systolic blood pressure (SBP), albuminuria and expression/activity of RAS components were assessed in diabetic db/db and control db/+ mice treated with increasing candesartan doses (intermediate, 1 mg/kg/d; high, 5 mg/kg/d; ultra-high, 25 and 75 mg/kg/d; 4 weeks). Lower doses candesartan did not influence SBP, but ultra-high doses reduced SBP in both groups. Plasma glucose and albuminuria were increased in db/db compared with db/+ mice. In diabetic mice treated with intermediate dose candesartan, renal tubular damage and albuminuria were ameliorated and expression of ACE2, AT2R and Mas and activity of ACE2 were increased, effects associated with reduced ERK1/2 phosphorylation, decreased fibrosis and renal protection. Ultra-high doses did not influence the ACE2/AT2R/Mas axis and promoted renal injury with increased renal ERK1/2 activation and exaggerated fibronectin expression in db/db mice. Our study demonstrates dose-related effects of candesartan in diabetic nephropathy: intermediate–high dose candesartan is renoprotective, whereas ultra-high dose candesartan induces renal damage. Molecular processes associated with these effects involve differential modulation of the ACE2/AT2R/Mas axis: intermediate–high dose candesartan up-regulating RAS protective components and attenuating pro-fibrotic processes, and ultra-high doses having opposite effects. These findings suggest novel mechanisms through the protective RAS axis, whereby candesartan may ameliorate diabetic nephropathy. Our findings also highlight potential injurious renal effects of ultra-high dose candesartan in diabetes. PMID:27612496

Naringin ameliorates metabolic syndrome by activating AMP-activated protein kinase in mice fed a high-fat diet.

PubMed

Pu, Peng; Gao, Dong-Mei; Mohamed, Salim; Chen, Jing; Zhang, Jing; Zhou, Xiao-Ya; Zhou, Nai-Jing; Xie, Jing; Jiang, Hong

2012-02-01

Metabolic syndrome is a low-grade inflammatory state in which oxidative stress is involved. Naringin, isolated from the Citrussinensis, is a phenolic compound with anti-oxidative and anti-inflammatory activities. The aim of this study was to explore the effects of naringin on metabolic syndrome in mice. The animal models, induced by high-fat diet in C57BL/6 mice, developed obesity, dyslipidemia, fatty liver, liver dysfunction and insulin resistance. These changes were attenuated by naringin. Further investigations revealed that the inhibitory effect on inflammation and insulin resistance was mediated by blocking activation of the MAPKs pathways and by activating IRS1; the lipid-lowering effect was attributed to inhibiting the synthesis way and increasing fatty acid oxidation; the hypoglycemic effect was due to the regulation of PEPCK and G6pase. The anti-oxidative stress of naringin also participated in the improvement of insulin resistance and lipogenesis. All of these depended on the AMPK activation. To confirm the results of the animal experiment, we tested primary hepatocytes exposed to high glucose system. Naringin was protective by phosphorylating AMPKα and IRS1. Taken together, these results suggested that naringin protected mice exposed to a high-fat diet from metabolic syndrome through an AMPK-dependent mechanism involving multiple types of intracellular signaling and reduction of oxidative damage. Copyright © 2011 Elsevier Inc. All rights reserved.

1H line width dependence on MAS speed in solid state NMR - Comparison of experiment and simulation

NASA Astrophysics Data System (ADS)

Sternberg, Ulrich; Witter, Raiker; Kuprov, Ilya; Lamley, Jonathan M.; Oss, Andres; Lewandowski, Józef R.; Samoson, Ago

2018-06-01

Recent developments in magic angle spinning (MAS) technology permit spinning frequencies of ≥100 kHz. We examine the effect of such fast MAS rates upon nuclear magnetic resonance proton line widths in the multi-spin system of β-Asp-Ala crystal. We perform powder pattern simulations employing Fokker-Plank approach with periodic boundary conditions and 1H-chemical shift tensors calculated using the bond polarization theory. The theoretical predictions mirror well the experimental results. Both approaches demonstrate that homogeneous broadening has a linear-quadratic dependency on the inverse of the MAS spinning frequency and that, at the faster end of the spinning frequencies, the residual spectral line broadening becomes dominated by chemical shift distributions and susceptibility effects even for crystalline systems.

Participation and Performance Reporting for the Alternate Assessment Based on Modified Achievement Standards (AA-MAS). Technical Report 58

ERIC Educational Resources Information Center

Albus, Deb; Thurlow, Martha L.; Lazarus, Sheryl S.

2011-01-01

This report examines publicly reported participation and performance data for the alternate assessment based on modified achievement standards (AA-MAS). The authors' analysis of these data included all states publicly reporting AA-MAS data, regardless of whether they had received approval to use the results for Title I accountability calculations.…

Mas-allatotropin in the developing antennal lobe of the sphinx moth Manduca sexta: distribution, time course, developmental regulation, and colocalization with other neuropeptides.

PubMed

Utz, Sandra; Huetteroth, Wolf; Vömel, Matthias; Schachtner, Joachim

2008-01-01

The paired antennal lobes (ALs) of the sphinx moth Manduca sexta serve as a well-established model for studying development of the primary integration centers for odor information in the brain. To further reveal the role of neuropeptides during AL development, we have analyzed cellular distribution, developmental time course, and regulation of the neuropeptide M. sexta allatotropin (Mas-AT). On the basis of morphology and appearance during AL formation, seven major types of Mas-AT-immunoreactive (ir) cells could be distinguished. Mas-AT-ir cells are identified as local, projection, and centrifugal neurons, which are either persisting larval or newly added adult-specific neurons. Complementary immunostaining with antisera against two other neuropeptide families (A-type allatostatins, RFamides) revealed colocalization within three of the Mas-AT-ir cell types. On the basis of this neurochemistry, the most prominent type of Mas-AT-ir neurons, the local AT neurons (LATn), could be divided in three subpopulations. The appearance of the Mas-AT-ir cell types occurring during metamorphosis parallels the rising titer of the developmental hormone 20-hydroxyecdysone (20E). Artificially shifting the 20E titer to an earlier developmental time point resulted in the precocious occurrence of Mas-AT immunostaining. This result supports the hypothesis that the pupal rise of 20E is causative for Mas-AT expression during AL development. Comparing localization and developmental time course of Mas-AT and other neuropeptides with the time course of AL formation suggests various functions for these neuropeptides during development, including an involvement in the formation of the olfactory glomeruli.

A comparison of the function, activity and participation and quality of life between down syndrome children and typically developing children.

PubMed

Jung, Hee-Kyoung; Chung, EunJung; Lee, Byoung-Hee

2017-08-01

[Purpose] To compare function, activity, participation, and quality of life of Down syndrome children and typically developing children according to age. [Subjects and Methods] A total of 16 Down syndrome children and 20 children with typical development were included as subjects for this study. International Classification of Functioning, Disability, and Health (ICF) Child and Youth version (CY) developed by the World Health Organization (WHO) and a questionnaire were used to measure children's functioning, activity, and participation. To measure quality of life, KIDSCREEN 52-HRQOL questionnaire was used in this study. [Results] ICF-CY function, activity, participation, and quality of life showed statistically significant differences between Down syndrome children and typically developing children. Down syndrome children with higher functions showed higher activities and participation. Higher function, activity and participation features were correlated with better quality of life. Higher function resulted in better quality of life. [Conclusion] Function, activity, participation, quality of life, and several common factors of Down syndrome children depend on the ability of children. Function of Down syndrome children affects their activity, participation, and quality of life. Activities and participations also affect quality of life. Therefore, children's functional aspect is the foundation for quality of life.

Activities that girls and women with Rett syndrome liked or did not like to do.

PubMed

Sernheim, Åsa-Sara; Hemmingsson, Helena; Witt Engerström, Ingegerd; Liedberg, Gunilla

2016-11-06

Activities occur in all people's lives. This study investigated over a period of time, 15 years, what activities were enjoyed or not enjoyed and what activities parents and staff liked to do with girls/women with Rett syndrome. A descriptive study was conducted using secondary data from three earlier questionnaires at the Swedish National Rett Center. The first questionnaire provided data on 123 girls/women with Rett syndrome, the second on 52 and the third questionnaire, on 39. Informants were parents and/or staff, in total 365. Open-ended questions were analysed using a content analysis approach. Three categories appeared: Being in motion, receiving impressions and having contact. Bathing/swimming, listening to music and being outdoors/walking were the most enjoyed activities over the years. Of the few activities that were reported as being unenjoyable, most were daily care activities. The activities that the parents/staff enjoyed doing with the girls/women were similar to those the girls/women themselves liked to do. A preliminary overview for both liked and disliked activities of girls/women with Rett syndrome was presented. This knowledge could facilitate the choice and use of activities.

Do adults with Down syndrome do the same amount of physical activity as adults without disability? A proof of principle study.

PubMed

Shields, Nora; Plant, Samantha; Warren, Catherine; Wollersheim, Dennis; Peiris, Casey

2018-05-01

This study compared levels of physical activity completed by adults with and without Down syndrome. Fifteen adults with and 15 adults without Down syndrome matched for age and gender, took part. The intensity and duration of physical activity were measured using RT3 accelerometers worn for seven days. Only, 12 participants with Down syndrome had complete physical activity data, and these participants and their matched controls (total: six females, 18 males; aged 25.8 ± 9.7) were included in the analyses. There were significantly lower levels of moderate and vigorous physical activity per day for people with Down syndrome (median = 27 min) compared to those without (median = 101 min) (p < .001). Participants without disability were twice more likely to achieve recommended levels of physical activity than people with Down syndrome. Adults with Down syndrome appear to participate in lower levels of physical activity than adults without Down syndrome. Further research should validate these estimates. © 2017 John Wiley & Sons Ltd.

Activation of the Alternative NFκB Pathway Improves Disease Symptoms in a Model of Sjogren's Syndrome

PubMed Central

Gilboa-Geffen, Adi; Wolf, Yochai; Hanin, Geula; Melamed-Book, Naomi; Pick, Marjorie; Bennett, Estelle R.; Greenberg, David S.; Lester, Susan; Rischmueller, Maureen; Soreq, Hermona

2011-01-01

The purpose of our study was to understand if Toll-like receptor 9 (TLR9) activation could contribute to the control of inflammation in Sjogren's syndrome. To this end, we manipulated TLR9 signaling in non-obese diabetic (NOD) and TLR9−/− mice using agonistic CpG oligonucleotide aptamers, TLR9 inhibitors, and the in-house oligonucleotide BL-7040. We then measured salivation, inflammatory response markers, and expression of proteins downstream to NF-κB activation pathways. Finally, we labeled proteins of interest in salivary gland biopsies from Sjogren's syndrome patients, compared to Sicca syndrome controls. We show that in NOD mice BL-7040 activates TLR9 to induce an alternative NF-κB activation mode resulting in increased salivation, elevated anti-inflammatory response in salivary glands, and reduced peripheral AChE activity. These effects were more prominent and also suppressible by TLR9 inhibitors in NOD mice, but TLR9−/− mice were resistant to the salivation-promoting effects of CpG oligonucleotides and BL-7040. Last, salivary glands from Sjogren's disease patients showed increased inflammatory and decreased anti-inflammatory biomarkers, in addition to decreased levels of alternative NF-κB pathway proteins. In summary, we have demonstrated that activation of TLR9 by BL-7040 leads to non-canonical activation of NF-κB, promoting salivary functioning and down-regulating inflammation. We propose that BL-7040 could be beneficial in treating Sjogren's syndrome and may be applicable to additional autoimmune syndromes. PMID:22174879

Metabolic Syndrome Remodels Electrical Activity of the Sinoatrial Node and Produces Arrhythmias in Rats

PubMed Central

Albarado-Ibañez, Alondra; Avelino-Cruz, José Everardo; Velasco, Myrian; Torres-Jácome, Julián; Hiriart, Marcia

2013-01-01

In the last ten years, the incidences of metabolic syndrome and supraventricular arrhythmias have greatly increased. The metabolic syndrome is a cluster of alterations, which include obesity, hypertension, hypertriglyceridemia, glucose intolerance and insulin resistance, that increase the risk of developing, among others, atrial and nodal arrhythmias. The aim of this study is to demonstrate that metabolic syndrome induces electrical remodeling of the sinus node and produces arrhythmias. We induced metabolic syndrome in 2-month-old male Wistar rats by administering 20% sucrose in the drinking water. Eight weeks later, the rats were anesthetized and the electrocardiogram was recorded, revealing the presence of arrhythmias only in treated rats. Using conventional microelectrode and voltage clamp techniques, we analyzed the electrical activity of the sinoatrial node. We observed that in the sinoatrial node of “metabolic syndrome rats”, compared to controls, the spontaneous firing of all cells decreased, while the slope of the diastolic depolarization increased only in latent pacemaker cells. Accordingly, the pacemaker currents If and Ist increased. Furthermore, histological analysis showed a large amount of fat surrounding nodal cardiomyocytes and a rise in the sympathetic innervation. Finally, Poincaré plot denoted irregularity in the R-R and P-P ECG intervals, in agreement with the variability of nodal firing potential recorded in metabolic syndrome rats. We conclude that metabolic syndrome produces a dysfunction SA node by disrupting normal architecture and the electrical activity, which could explain the onset of arrhythmias in rats. PMID:24250786

Metabolic syndrome remodels electrical activity of the sinoatrial node and produces arrhythmias in rats.

PubMed

Albarado-Ibañez, Alondra; Avelino-Cruz, José Everardo; Velasco, Myrian; Torres-Jácome, Julián; Hiriart, Marcia

2013-01-01

In the last ten years, the incidences of metabolic syndrome and supraventricular arrhythmias have greatly increased. The metabolic syndrome is a cluster of alterations, which include obesity, hypertension, hypertriglyceridemia, glucose intolerance and insulin resistance, that increase the risk of developing, among others, atrial and nodal arrhythmias. The aim of this study is to demonstrate that metabolic syndrome induces electrical remodeling of the sinus node and produces arrhythmias. We induced metabolic syndrome in 2-month-old male Wistar rats by administering 20% sucrose in the drinking water. Eight weeks later, the rats were anesthetized and the electrocardiogram was recorded, revealing the presence of arrhythmias only in treated rats. Using conventional microelectrode and voltage clamp techniques, we analyzed the electrical activity of the sinoatrial node. We observed that in the sinoatrial node of "metabolic syndrome rats", compared to controls, the spontaneous firing of all cells decreased, while the slope of the diastolic depolarization increased only in latent pacemaker cells. Accordingly, the pacemaker currents If and Ist increased. Furthermore, histological analysis showed a large amount of fat surrounding nodal cardiomyocytes and a rise in the sympathetic innervation. Finally, Poincaré plot denoted irregularity in the R-R and P-P ECG intervals, in agreement with the variability of nodal firing potential recorded in metabolic syndrome rats. We conclude that metabolic syndrome produces a dysfunction SA node by disrupting normal architecture and the electrical activity, which could explain the onset of arrhythmias in rats.

Angiotensin (1-7) and its receptor Mas are expressed in the human testis: implications for male infertility.

PubMed

Reis, Augusto B; Araújo, Fabiano C; Pereira, Virginia M; Dos Reis, Adelina M; Santos, Robson A; Reis, Fernando M

2010-02-01

The presence of classical components of the renin-angiotensin system has been demonstrated in the male reproductive tract, mainly in the testes and epididymis. The objective of this study was to verify the localization of angiotensin (Ang)-(1-7) and its receptor Mas in human testis. The study included 12 men with previously proven fertility submitted to orchiectomy for prostate cancer and 20 infertile men submitted to testicular biopsy for infertility work-up, comprising a subgroup with obstructive azoospermia/normal spermatogenesis (n = 8) and another with non-obstructive azoospermia and severely impaired spermatogenesis (n = 12). Testicular tissue samples were processed by immunohistochemistry and real time polymerase chain reaction. Ang-(1-7) was strongly expressed in the interstitial compartment, mainly in Leydig cells, with similar intensity in all groups evaluated. The peptide was also detected in the seminiferous tubules, but with much less intensity compared to interstitial cells. The receptor Mas was equally distributed between interstitial and tubular compartments and was found in all layers of the normal seminiferous epithelium. However, neither Ang-(1-7) nor Mas were detected in the seminiferous tubules of samples with impaired spermatogenesis. The testicular samples of infertile men with impaired spermatogenesis (non-obstructive azoospermia) expressed Mas and ACE2 mRNA at lower concentrations (fold change = 0.06 and 0.04, respectively, P < 0.05) than samples with full spermatogenesis (obstructive azoospermia). This shows, for the first time, the immunolocalization of Ang-(1-7) and its receptor Mas in testes of fertile and infertile men, and suggests that this system may be altered when spermatogenesis is severely impaired.

Modified Ashworth Scale (MAS) Model based on Clinical Data Measurement towards Quantitative Evaluation of Upper Limb Spasticity

NASA Astrophysics Data System (ADS)

Puzi, A. Ahmad; Sidek, S. N.; Mat Rosly, H.; Daud, N.; Yusof, H. Md

2017-11-01

Spasticity is common symptom presented amongst people with sensorimotor disabilities. Imbalanced signals from the central nervous systems (CNS) which are composed of the brain and spinal cord to the muscles ultimately leading to the injury and death of motor neurons. In clinical practice, the therapist assesses muscle spasticity using a standard assessment tool like Modified Ashworth Scale (MAS), Modified Tardiue Scale (MTS) or Fugl-Meyer Assessment (FMA). This is done subjectively based on the experience and perception of the therapist subjected to the patient fatigue level and body posture. However, the inconsistency in the assessment is prevalent and could affect the efficacy of the rehabilitation process. Thus, the aim of this paper is to describe the methodology of data collection and the quantitative model of MAS developed to satisfy its description. Two subjects with MAS of 2 and 3 spasticity levels were involved in the clinical data measurement. Their level of spasticity was verified by expert therapist using current practice. Data collection was established using mechanical system equipped with data acquisition system and LABVIEW software. The procedure engaged repeated series of flexion of the affected arm that was moved against the platform using a lever mechanism and performed by the therapist. The data was then analyzed to investigate the characteristics of spasticity signal in correspondence to the MAS description. Experimental results revealed that the methodology used to quantify spasticity satisfied the MAS tool requirement according to the description. Therefore, the result is crucial and useful towards the development of formal spasticity quantification model.

Indirect detection of infinite-speed MAS solid-state NMR spectra

DOE Office of Scientific and Technical Information (OSTI.GOV)

Perras, Frédéric A.; Venkatesh, Amrit; Hanrahan, Michael P.

Heavy spin-1/2 nuclides are known to possess very large chemical shift anisotropies that can challenge even the most advanced magic-angle-spinning (MAS) techniques. Wide manifolds of overlapping spinning sidebands and insufficient excitation bandwidths often obfuscate meaningful spectral information and force the use of static, low-resolution solid-state (SS)NMR methods for the characterization of materials. In order to address these issues, we have merged fast-magic-angle-turning (MAT) and dipolar heteronuclear multiple-quantum coherence (D-HMQC) experiments to obtain D-HMQC-MAT pulse sequences which enable the rapid acquisition of 2D SSNMR spectra that correlate isotropic 1H chemical shifts to the indirectly detected isotropic “infinite-MAS” spectra of heavy spin-1/2more » nuclides. Furthermore, for these nuclides, the combination of fast MAS and 1H detection provides a high sensitivity, which rivals the DNP-enhanced ultra-wideline SSNMR. The new pulse sequences were used to determine the Pt coordination environments in a complex mixture of decomposition products of transplatin and in a metal-organic framework with Pt ions coordinated to the linker ligands.« less

Indirect detection of infinite-speed MAS solid-state NMR spectra

DOE PAGES

Perras, Frédéric A.; Venkatesh, Amrit; Hanrahan, Michael P.; ...

2017-01-18

Heavy spin-1/2 nuclides are known to possess very large chemical shift anisotropies that can challenge even the most advanced magic-angle-spinning (MAS) techniques. Wide manifolds of overlapping spinning sidebands and insufficient excitation bandwidths often obfuscate meaningful spectral information and force the use of static, low-resolution solid-state (SS)NMR methods for the characterization of materials. In order to address these issues, we have merged fast-magic-angle-turning (MAT) and dipolar heteronuclear multiple-quantum coherence (D-HMQC) experiments to obtain D-HMQC-MAT pulse sequences which enable the rapid acquisition of 2D SSNMR spectra that correlate isotropic 1H chemical shifts to the indirectly detected isotropic “infinite-MAS” spectra of heavy spin-1/2more » nuclides. Furthermore, for these nuclides, the combination of fast MAS and 1H detection provides a high sensitivity, which rivals the DNP-enhanced ultra-wideline SSNMR. The new pulse sequences were used to determine the Pt coordination environments in a complex mixture of decomposition products of transplatin and in a metal-organic framework with Pt ions coordinated to the linker ligands.« less

Role of Mas receptor in renal blood flow response to angiotensin (1-7) in male and female rats.

PubMed

Nematbakhsh, Mehdi; Safari, Tahereh

2014-01-01

Epidemiologic and clinical studies have shown that progression of renal disease in male is faster than that in female. However, the exact mechanisms are not well recognized. Angiotensin (1-7) (Ang 1-7) receptor, called "Mas", is an element in the depressor arm of renin angiotensin system (RAS), and its expression is enhanced in females. We test the hypothesis that Mas receptor (MasR) blockade (A779) attenuates renal blood flow (RBF) in response to infusion of graded doses of Ang 1-7 in female rats. Male and female Wistar rats were anesthetized and catheterized. Then, the mean arterial pressure (MAP), RBF, and controlled renal perfusion pressure (RPP) responses to infusion of graded doses of Ang 1-7 (100-1000 ng/kg/min i.v.) with and without A779 were measured in the animals. Basal MAP, RPP, RBF, and renal vascular resistance (RVR) were not significantly different between the two groups. After Ang 1-7 administration, RPP was controlled at a constant level. However, RBF increased in a dose-related manner in response to Ang 1-7 infusion in both male and female rats (Pdose<0.0001), but masR blockade significantly attenuated this response only in female (Pgroup=0.04) and not male (Pgroup=0.23). In addition, A779 increased the RBF response to Ang 1-7 to a greater extent. This is while the increase in male was not significant when compared with that in female (Pgender=0.08). RVR response to Ang 1-7 was insignificantly attenuated by A779 in both genders. The masR differently regulated RBF response to Ang 1-7 in the two genders, and the effect was greater in female rats. The masR may be a target for improvement of kidney circulation in renal diseases.

Leisure time sedentary behavior, occupational/domestic physical activity, and metabolic syndrome in U.S. men and women.

PubMed

Sisson, Susan B; Camhi, Sarah M; Church, Timothy S; Martin, Corby K; Tudor-Locke, Catrine; Bouchard, Claude; Earnest, Conrad P; Smith, Steven R; Newton, Robert L; Rankinen, Tuomo; Katzmarzyk, Peter T

2009-12-01

This study examines leisure time sedentary behavior (LTSB) and usual occupational/domestic activity (UODA) and their relationship with metabolic syndrome and individual cardiovascular disease (CVD) risk factors, independent of physical activity level. National Health and Nutrition Examination Survey (NHANES) 2003-2006 data from men (n = 1868) and women (n = 1688) with fasting measures were classified as having metabolic syndrome by the American Heart Association/National Heart, Lung, and Blood Institute (AHA/NHLBI) definition. LTSB was determined from self-reported television viewing and computer usage. UODA was self-reported daily behavior (sitting, standing, walking, carrying loads). LTSB >or=4 hours/day was associated with odds of having metabolic syndrome of 1.94 (95% confidence interval [CI], 1.24, 3.03) in men compared to or=4 hour/day was also associated with higher odds of elevated waist circumference (1.88, CI, 1.03, 3.41), low high-density lipoprotein cholesterol (HDL-C) (1.84, CI, 1.35, 2.51), and high blood pressure (1.55, CI, 1.07, 2.24) in men. LTSB 2-3 hours/day was associated with higher odds of elevated glucose (1.32, CI, 1.00, 1.75) in men. In women, odds of metabolic syndrome were 1.54 (CI, 1.00, 2.37) with >or=4 hours/day LTSB, but LTSB was not associated with risk of the individual CVD risk factors. Higher LTSB was associated with metabolic syndrome in inactive men (1.50, CI, 1.07, 2.09), active men (1.74, CI, 1.11, 2.71), inactive women (1.69, CI, 1.24, 2.33), but not active women (1.62, CI, 0.87,3.01). UODA was not strongly associated with metabolic syndrome or CVD risk factors in either men or women. In men, high LTSB is associated with higher odds of metabolic syndrome and individual CVD risk factors regardless of meeting physical activity recommendations. In women, high LTSB is associated with higher odds of metabolic syndrome only in those not meeting the physical activity recommendations.

Radiofrequency fields in MAS solid state NMR probes.

PubMed

Tošner, Zdeněk; Purea, Armin; Struppe, Jochem O; Wegner, Sebastian; Engelke, Frank; Glaser, Steffen J; Reif, Bernd

2017-11-01

We present a detailed analysis of the radiofrequency (RF) field over full volume of a rotor that is generated in a solenoid coil. On top of the usually considered static distribution of amplitudes along the coil axis we describe dynamic radial RF inhomogeneities induced by sample rotation. During magic angle spinning (MAS), the mechanical rotation of the sample about the magic angle, a spin packet travels through areas of different RF fields and experiences periodical modulations of both the RF amplitude and the phase. These modulations become particularly severe at the end regions of the coil where the relative RF amplitude varies up to ±25% and the RF phase changes within ±30°. Using extensive numerical simulations we demonstrate effects of RF inhomogeneity on pulse calibration and for the ramped CP experiment performed at a wide range of MAS rates. In addition, we review various methods to map RF fields using a B 0 gradient along the sample (rotor axis) for imaging purposes. Under such a gradient, a nutation experiment provides directly the RF amplitude distribution, a cross polarization experiment images the correlation of the RF fields on the two channels according to the Hartmann-Hahn matching condition, while a spin-lock experiment allows to calibrate the RF amplitude employing the rotary resonance recoupling condition. Knowledge of the RF field distribution in a coil provides key to understand its effects on performance of a pulse sequence at the spectrometer and enables to set robustness requirements in the experimental design. Copyright © 2017 Elsevier Inc. All rights reserved.

Radiofrequency fields in MAS solid state NMR probes

NASA Astrophysics Data System (ADS)

Tošner, Zdeněk; Purea, Armin; Struppe, Jochem O.; Wegner, Sebastian; Engelke, Frank; Glaser, Steffen J.; Reif, Bernd

2017-11-01

We present a detailed analysis of the radiofrequency (RF) field over full volume of a rotor that is generated in a solenoid coil. On top of the usually considered static distribution of amplitudes along the coil axis we describe dynamic radial RF inhomogeneities induced by sample rotation. During magic angle spinning (MAS), the mechanical rotation of the sample about the magic angle, a spin packet travels through areas of different RF fields and experiences periodical modulations of both the RF amplitude and the phase. These modulations become particularly severe at the end regions of the coil where the relative RF amplitude varies up to ±25% and the RF phase changes within ±30°. Using extensive numerical simulations we demonstrate effects of RF inhomogeneity on pulse calibration and for the ramped CP experiment performed at a wide range of MAS rates. In addition, we review various methods to map RF fields using a B0 gradient along the sample (rotor axis) for imaging purposes. Under such a gradient, a nutation experiment provides directly the RF amplitude distribution, a cross polarization experiment images the correlation of the RF fields on the two channels according to the Hartmann-Hahn matching condition, while a spin-lock experiment allows to calibrate the RF amplitude employing the rotary resonance recoupling condition. Knowledge of the RF field distribution in a coil provides key to understand its effects on performance of a pulse sequence at the spectrometer and enables to set robustness requirements in the experimental design.

The Association between Physical Activity and the Metabolic Syndrome among Type 2 Diabetes Patients in Gaza Strip, Palestine.

PubMed

El Bilbeisi, Abdel Hamid; Hosseini, Saeed; Djafarian, Kurosh

2017-05-01

Metabolic syndrome is a major health problem worldwide. Globally, the World Health Organization identified physical inactivity as the fourth leading risk factor for mortality. This study was conducted to evaluate the association between physical activities and metabolic syndrome and diabetes complications among type 2 diabetes patients in Gaza Strip, Palestine. This cross-sectional study was conducted among 1200 previously diagnosed type 2 diabetes mellitus patients (from both genders, aged 20 to 64 years) receiving care in the primary health care centers. Metabolic syndrome was defined based on the International Diabetes Federation criteria. The International Physical Activity Questionnaire was used to measure physical activity. Statistical analysis was performed using SPSS version 20. A significant inverse association was found between inactive patients and metabolic syndrome. In our study, 93.7% of inactive patients, 66.4% of active patients and 23.5% of very active patients had metabolic syndrome (OR .048 CI 95% (.03-.072)), (OR .787 CI 95% (.59-1.03)) and (OR 15.9 CI 95% (11.8-21.3)) respectively. Our results showed a significant inverse association between physical activity levels and anthropometric measurements in both gender. Moreover, a significant association was found between physical activity levels and triglycerides, HDL-cholesterol and blood pressure in both sexes (P value < 0.05 for all) and diabetes complications (P value < 0.05 for all). We conclude that low levels of physical activity are associated with increased prevalence of metabolic syndrome. Furthermore, inactive patients had a high percentage of diabetes complications among type 2 diabetes patients in Gaza Strip, Palestine.

The Stress-Metabolic Syndrome Relationship in Adolescents: An Examination of the Moderating Potential of Physical Activity.

PubMed

Holmes, Megan E; Pivarnik, Jim; Pfeiffer, Karin; Maier, Kimberly S; Eisenmann, Joey C; Ewing, Martha

2016-10-01

The role of psychosocial stress in the development of obesity and metabolic syndrome is receiving increased attention and has led to examination of whether physical activity may moderate the stress-metabolic syndrome relationship. The current study examined relationships among physical activity, stress, and metabolic syndrome in adolescents. Participants (N = 126; 57 girls, 69 boys) were assessed for anthropometry, psychosocial stress, physical activity, and metabolic syndrome variables; t tests were used to examine sex differences, and regression analysis was used to assess relationships among variables controlling for sex and maturity status. Mean body mass index approached the 75th percentile for both sexes. Typical sex differences were observed for systolic blood pressure, time spent in moderate and vigorous physical activity, and perceived stress. Although stress was not associated with MetS (β = -.001, P = .82), a modest, positive relationship was observed with BMI (β = .20, P = .04). Strong relationships between physical activity and stress with MetS or BMI were not found in this sample. Results may be partially explained by overall good physical health status of the participants. Additional research in groups exhibiting varying degrees of health is needed.

Prolonged hypothalamic-pituitary-adrenal axis activation after acute coronary syndrome in the GENESIS-PRAXY cohort.

PubMed

Tang, Andrew R; Rabi, Doreen M; Lavoie, Kim L; Bacon, Simon L; Pilote, Louise; Kline, Gregory A

2018-01-01

Background Glucocorticoid excess has been linked with cardiovascular disease. Little is known about the long-term cortisol response in patients after acute coronary syndrome. Design The objective of this study was to describe the distribution of salivary cortisol in the post-acute phase of acute coronary syndrome and to describe the association of late-night salivary cortisol with cardiovascular risk factors. Methods We used late-night salivary cortisol measurements post-discharge to estimate hypothalamic-pituitary-adrenal axis activity in 309 patients aged 18-55 years enrolled in the GENESIS-PRAXY study from January 2009-April 2013. We evaluated hypothalamic-pituitary-adrenal axis activity and its association with hypertension, dyslipidemia, diabetes, smoking, family history, prior acute coronary syndrome, psychiatric diseases, acute coronary syndrome severity, as well as mortality and rate of rehospitalization at 12 months. Results Persistently elevated late-night salivary cortisol>2.92 nmol/l was seen in 99 (32.0%) patients: within the range of what may be seen in Cushing's disease. Elevated late-night salivary cortisol was associated with previous acute coronary syndrome (13.3% vs 24.2%, p = 0.02), peripheral vascular disease (3.8% vs 13.1%, p = 0.002), and smoking (32.9% vs 46.5% p = 0.02). Elevated late-night salivary cortisol was associated with higher hemoglobin A1c values (5.6 ± 3.0 vs 6.1 ± 2.9, p = 0.008) and lower high density lipoprotein values (0.94 ± 0.53 vs 0.86 ± 0.50, p = 0.01). There were no differences in psychiatric symptom scores, acute coronary syndrome severity or mortality, and rate of rehospitalization at 12 months. Conclusions Many patients post-acute coronary syndrome have prolonged, marked activation of the hypothalamic-pituitary-adrenal axis. Late-night salivary cortisol co-associates with several cardiovascular risk factors. Further studies are needed to confirm the exact role of hypothalamic

Leisure Activity and Caregiver Involvement in Middle-Aged and Older Adults with Down Syndrome

ERIC Educational Resources Information Center

Mihaila, Iulia; Hartley, Sigan L.; Handen, Benjamin L.; Bulova, Peter D.; Tumuluru, Rameshwari V.; Devenny, Darlynne A.; Johnson, Sterling C.; Lao, Patrick J.; Christian, Bradley, T.

2017-01-01

The present study examined leisure activity and its association with caregiver involvement (i.e., residence and time spent with primary caregiver) in 62 middle-aged and older adults with Down syndrome (aged 30-53 years). Findings indicated that middle-aged and older adults with Down syndrome frequently participated in social and passive leisure…

In situ high temperature MAS NMR study of the mechanisms of catalysis. Ethane aromatization on Zn-modified zeolite BEA.

PubMed

Arzumanov, Sergei S; Gabrienko, Anton A; Freude, Dieter; Stepanov, Alexander G

2009-04-01

Ethane conversion into aromatic hydrocarbons over Zn-modified zeolite BEA has been analyzed by high-temperature MAS NMR spectroscopy. Information about intermediates (Zn-ethyl species) and reaction products (mainly toluene and methane), which were formed under the conditions of a batch reactor, was obtained by (13)C MAS NMR. Kinetics of the reaction, which was monitored by (1)H MAS NMR in situ at the temperature of 573K, provided information about the reaction mechanism. Simulation of the experimental kinetics within the frames of the possible kinetic schemes of the reaction demonstrates that a large amount of methane evolved under ethane aromatization arises from the stage of direct ethane hydrogenolysis.

Mas-related G protein coupled receptor-X2: A potential new target for modulating mast cell-mediated allergic and inflammatory diseases.

PubMed

Ali, Hydar

2016-12-01

Mast cells (MCs) are tissue resident immune cells that are best known for their roles in allergic and inflammatory diseases. In addition to the high affinity IgE receptor (FcεRI), MCs express numerous G protein coupled receptors (GPCRs), which are the most common targets of drug therapy. Neurokinin 1 receptor (NK-1R) is expressed on MCs and contributes to IgE and non-IgE-mediated responses in mice. Although NK-1R antagonists are highly effective in modulating experimental allergic and inflammatory responses in mice they lack efficacy in humans. This article reviews recent findings that demonstrate that while neuropeptides (NPs) activate murine MCs via NK-1R and Mas related G protein coupled receptor B2 (MrgprB2), they activate human MCs via Mas-related G protein coupled receptor X2 (MRGPRX2). Interestingly, conventional NK-1R antagonists have off-target activity against mouse MrgprB2 but not human MRGPRX2. These findings suggest that the failure to translate studies with NK-1R antagonists from in vivo mouse studies to the clinic likely reflects their lack of effect on human MRGPRX2. A unique feature of MRGPRX2 that distinguishes it from other GPCRs is that it is activated by a diverse group of ligands that include; neuropeptides, cysteine proteases, antimicrobial peptides and cationic proteins released from activated eosinophils. Thus, the development of small molecule MRGPRX2-specific antagonists or neutralizing antibodies may provide new targets for the treatment of MC-mediated allergic and inflammatory diseases.

The Gαo Activator Mastoparan-7 Promotes Dendritic Spine Formation in Hippocampal Neurons.

PubMed

Ramírez, Valerie T; Ramos-Fernández, Eva; Inestrosa, Nibaldo C

2016-01-01

Mastoparan-7 (Mas-7), an analogue of the peptide mastoparan, which is derived from wasp venom, is a direct activator of Pertussis toxin- (PTX-) sensitive G proteins. Mas-7 produces several biological effects in different cell types; however, little is known about how Mas-7 influences mature hippocampal neurons. We examined the specific role of Mas-7 in the development of dendritic spines, the sites of excitatory synaptic contact that are crucial for synaptic plasticity. We report here that exposure of hippocampal neurons to a low dose of Mas-7 increases dendritic spine density and spine head width in a time-dependent manner. Additionally, Mas-7 enhances postsynaptic density protein-95 (PSD-95) clustering in neurites and activates Gα(o) signaling, increasing the intracellular Ca(2+) concentration. To define the role of signaling intermediates, we measured the levels of phosphorylated protein kinase C (PKC), c-Jun N-terminal kinase (JNK), and calcium-calmodulin dependent protein kinase IIα (CaMKIIα) after Mas-7 treatment and determined that CaMKII activation is necessary for the Mas-7-dependent increase in dendritic spine density. Our results demonstrate a critical role for Gα(o) subunit signaling in the regulation of synapse formation.

Kinase-activating and kinase-impaired cardio-facio-cutaneous syndrome alleles have activity during zebrafish development and are sensitive to small molecule inhibitors.

PubMed

Anastasaki, Corina; Estep, Anne L; Marais, Richard; Rauen, Katherine A; Patton, E Elizabeth

2009-07-15

The Ras/MAPK pathway is critical for human development and plays a central role in the formation and progression of most cancers. Children born with germ-line mutations in BRAF, MEK1 or MEK2 develop cardio-facio-cutaneous (CFC) syndrome, an autosomal dominant syndrome characterized by a distinctive facial appearance, heart defects, skin and hair abnormalities and mental retardation. CFC syndrome mutations in BRAF promote both kinase-activating and kinase-impaired variants. CFC syndrome has a progressive phenotype, and the availability of clinically active inhibitors of the MAPK pathway prompts the important question as to whether such inhibitors might be therapeutically effective in the treatment of CFC syndrome. To study the developmental effects of CFC mutant alleles in vivo, we have expressed a panel of 28 BRAF and MEK alleles in zebrafish embryos to assess the function of human disease alleles and available chemical inhibitors of this pathway. We find that both kinase-activating and kinase-impaired CFC mutant alleles promote the equivalent developmental outcome when expressed during early development and that treatment of CFC-zebrafish embryos with inhibitors of the FGF-MAPK pathway can restore normal early development. Importantly, we find a developmental window in which treatment with a MEK inhibitor can restore the normal early development of the embryo, without the additional, unwanted developmental effects of the drug.

[Exaggerated somatomedin activity in the Beckwith-Wiedemann syndrome (author's transl)].

PubMed

Schabel, F; Frisch, H

1979-01-01

Beckwith and Wiedemann described the syndrome of exomphalos, macroglossia and gigantism with hypoglycemia and visceral organ hyperplasias. In some cases of severe hypoglycemias hyperplasia of beta cells of the pancreas was found. Hyperinsulinism, which has to date rarely been investigated, reacts strongly to beta cell stimulation and can hardly be suppressed. The cause of gigantism and organ hyperplasias is still unknown. After a short description of a case of hypoglycemias in the first two weeks of life a long-term profile of the endocrinologic abnormalities and carbohydrate metabolism is given. Growth hormone response to insulin is normal, tolbutamide is followed by severe hypoglycemias without an increase in the immunoreactive insulin levels; the activity of somatomedin is excessively increased. The high activity of somatomedin explains the high potency of growth in the different tissues and the hypoglycemic reactions and it seems reasonable to assume that somatomedin could create nesidioblastosis of the pancreas with hyperinsulinism and severe hypoglycemias. It is likely that the Beckwith-Wiedemann syndrome and the Laron type familial dwarfism with high plasma growth hormone, absent activity of somatomedin, and disorders in carbohydrate metabolism represent complementary diseases.

Physical Fitness and Physical Activity in Adolescents with Asperger Syndrome: A Comparative Study

ERIC Educational Resources Information Center

Borremans, Erwin; Rintala, Pauli; McCubbin, Jeffrey A.

2010-01-01

While physical activity is beneficial for youth with developmental disabilities, little is known about those individuals' fitness profile and levels of activity. Therefore the purpose of this study was to investigate the physical fitness profile and physical activity level of 30 adolescents with and without Asperger syndrome (AS). Evaluations were…

The Multidimensional Attitudes Scale toward Persons with Disabilities (MAS): Construction and Validation

ERIC Educational Resources Information Center

Findler, Liora; Vilchinsky, Noa; Werner, Shirli

2007-01-01

This study presents the development of a new instrument, the "Multidimensional Attitudes Scale Toward Persons With Disabilities" (MAS). Based on the multidimensional approach, it posits that attitudes are composed of three dimensions: affect, cognition, and behavior. The scale was distributed to a sample of 132 people along with a…

Synthesis and characterization of magnesium aluminate (MgAl2O4) spinel (MAS) thin films

NASA Astrophysics Data System (ADS)

Ahmad, Syed Muhammad; Hussain, Tousif; Ahmad, Riaz; Siddiqui, Jamil; Ali, Dilawar

2018-01-01

In a quest to identify more economic routes for synthesis of magnesium aluminate (MgAl2O4) spinel (MAS) thin films, dense plasma focus device was used with multiple plasma focus shots. Structural, bonding between composite films, surface morphological, compositional and hardness properties of MAS thin films were investigated by using x-ray diffraction (XRD), Fourier transform infrared spectroscopy (FTIR), scanning electron microscopy (SEM), energy dispersive x-rays (EDX) analysis and Vickers micro hardness test respectively. In XRD graph, the presence of MgAl2O4 diffraction peaks in crystallographic orientations (222), (400) and (622) pointed out the successful formation of polycrystalline thin films of MgAl2O4 with face centered cubic structure. The FTIR spectrums showed a major common transmittance band at 697.95 cm-1 which belongs to MgAl2O4. SEM micrographs illustrated a mesh type, granular and multi layers microstructures with significant melting effects. EDX spectrum confirmed the existence of magnesium, oxygen and aluminum in MAS films. A common increasing behavior in micro-hardness of composite MgAl2O4 films by increasing number of plasma focus shots was found.

Syndromic surveillance of influenza activity in Sweden: an evaluation of three tools.

PubMed

Ma, T; Englund, H; Bjelkmar, P; Wallensten, A; Hulth, A

2015-08-01

An evaluation was conducted to determine which syndromic surveillance tools complement traditional surveillance by serving as earlier indicators of influenza activity in Sweden. Web queries, medical hotline statistics, and school absenteeism data were evaluated against two traditional surveillance tools. Cross-correlation calculations utilized aggregated weekly data for all-age, nationwide activity for four influenza seasons, from 2009/2010 to 2012/2013. The surveillance tool indicative of earlier influenza activity, by way of statistical and visual evidence, was identified. The web query algorithm and medical hotline statistics performed equally well as each other and to the traditional surveillance tools. School absenteeism data were not reliable resources for influenza surveillance. Overall, the syndromic surveillance tools did not perform with enough consistency in season lead nor in earlier timing of the peak week to be considered as early indicators. They do, however, capture incident cases before they have formally entered the primary healthcare system.

Deuterium and lithium-6 MAS NMR studies of manganese oxide electrode materials

NASA Astrophysics Data System (ADS)

Paik, Younkee

Electrolytic manganese dioxide (EMD) is used world wide as the cathode materials in both lithium and alkaline primary (non-rechargeable) batteries. We have developed deuterium and lithium MAS NMR techniques to study EMD and related manganese oxides and hydroxides, where diffraction techniques are of limited value due to a highly defective nature of the structures. Deuterons in EMD, manganite, groutite, and deuterium-intercalated pyrolusite and ramsdellite were detected by NMR, for the first time, and their locations and motions in the structures were analyzed by applying variable temperature NMR techniques. Discharge mechanisms of EMD in alkaline (aqueous) electrolytes were studied, in conjunction with step potential electrochemical spectroscopic (SPECS) method, and five distinctive discharge processes were proposed. EMD is usually heat-treated at about 300--400°C to remove water to be used in lithium batteries. Details of the effects of heat-treatment, such as structural and compositional changes as a function of heat-treatment temperature, were studied by a combination of MAS NMR, XRD, and thermogravimetric analysis. Lithium local environments in heat-treated EMD (HEMD) that were discharged in lithium cells, were described in terms of related environments found in model compounds pyrolusite and ramsdellite where specific Li + sites were detected by MAS NMR and the hyperfine shift scale method of Grey et al. Acid-leaching of Li2MnO3 represents an approach for synthesizing new or modified manganese oxide electrode materials for lithium rechargeable batteries. Progressive removal of lithium from specific crystallographic sites, followed by a gradual change of the crystal structure, was monitored by a combination of NMR and XRD techniques.

Directly and indirectly detected through-bond heteronuclear correlation solid-state NMR spectroscopy under fast MAS

DOE Office of Scientific and Technical Information (OSTI.GOV)

Mao, Kanmi; Pruski, Marek

Two-dimensional through-bond {sup 1}H({sup 13}C) solid-state NMR experiments utilizing fast magic angle spinning (MAS) and homonuclear multipulse {sup 1}H decoupling are presented. Remarkable efficiency of polarization transfer can be achieved at MAS rates exceeding 40 kHz, which is instrumental in these measurements. Schemes utilizing direct and indirect detection of heteronuclei are compared in terms of resolution and sensitivity. A simple procedure for optimization of {sup 1}H homonuclear decoupling sequences under these conditions is proposed. The capabilities of these techniques were confirmed on two naturally abundant solids, tripeptide N-formyl-l-methionyl-l-leucyl-l-phenylalanine (f-MLF-OH) and brown coal.

Directly and indirectly detected through-bond heteronuclear correlation solid-state NMR spectroscopy under fast MAS

NASA Astrophysics Data System (ADS)

Mao, Kanmi; Pruski, Marek

2009-12-01

Two-dimensional through-bond 1H{ 13C} solid-state NMR experiments utilizing fast magic angle spinning (MAS) and homonuclear multipulse 1H decoupling are presented. Remarkable efficiency of polarization transfer can be achieved at MAS rates exceeding 40 kHz, which is instrumental in these measurements. Schemes utilizing direct and indirect detection of heteronuclei are compared in terms of resolution and sensitivity. A simple procedure for optimization of 1H homonuclear decoupling sequences under these conditions is proposed. The capabilities of these techniques were confirmed on two naturally abundant solids, tripeptide N- formyl- L-methionyl- L-leucyl- L-phenylalanine (f-MLF-OH) and brown coal.

Probing intermolecular interactions in a diethylcarbamazine citrate salt by fast MAS 1H solid-state NMR spectroscopy and GIPAW calculations.

PubMed

Venâncio, Tiago; Oliveira, Lyege Magalhaes; Ellena, Javier; Boechat, Nubia; Brown, Steven P

2017-10-01

Fast magic-angle spinning (MAS) NMR is used to probe intermolecular interactions in a diethylcarbamazine salt, that is widely used as a treatment against adult worms of Wuchereria bancrofti which cause a common disease in tropical countries named filariasis. Specifically, a dihydrogen citrate salt that has improved thermal stability and solubility as compared to the free form is studied. One-dimensional 1 H, 13 C and 15 N and two-dimensional 1 H- 13 C and 14 N- 1 H heteronuclear correlation NMR experiments under moderate and fast MAS together with GIPAW (CASTEP) calculations enable the assignment of the 1 H, 13 C and 14 N/ 15 N resonances. A two-dimensional 1 H- 1 H double-quantum (DQ) -single-quantum (SQ) MAS spectrum recorded with BaBa recoupling at 60kHz MAS identifies specific proton-proton proximities associated with citrate-citrate and citrate-diethylcarbamazine intermolecular interactions. Copyright © 2017 Elsevier Inc. All rights reserved.

El Proyecto Sismico "LARSE" - Trabajando Hacia un Futuro con Mas Seguridad para Los Angeles

USGS Publications Warehouse

Henyey, Thomas L.; Fuis, Gary S.; Benthien, Mark L.; Burdette, Thomas R.; Christofferson, Shari A.; Clayton, Robert W.; Criley, Edward E.; Davis, Paul M.; Hendley, James W.; Kohler, Monica D.; Lutter, William J.; McRaney, John K.; Murphy, Janice M.; Okaya, David A.; Ryberg, Trond; Simila, Gerald W.; Stauffer, Peter H.

1999-01-01

La region de Los Angeles contiene una red de fallas activas, incluyendo muchas fallas por empuje que son profundas y no rompen la superficie de la tierra. Estas fallas ocultas incluyen la falla anteriormente desconocida que fue responsable por la devastacion que ocurrio durante el terremoto de Northridge en enero de 1994, el terremoto mas costoso en la historia de los Estados Unidos. El Experimento Sismico en la Region de Los Angeles (Los Angeles Region Seismic Experiment, LARSE), esta localizando los peligros ocultos de los terremotos debajo de la region de Los Angeles para mejorar la construccion de las estructuras que pueden apoyar terremotos que son inevitables en el futuro, y que ayudaran a los cientificos determinar donde occurira el sacudimento mas fuerte y poderoso.

An Analysis of the Rise and Fall of the AA-MAS Policy

ERIC Educational Resources Information Center

Lazarus, Sheryl S.; Thurlow, Martha L.; Ysseldyke, James E.; Edwards, Lynn M.

2015-01-01

In 2005, to address concerns about students who might fall in the "gap" between the regular assessment and the alternate assessment based on alternate achievement standards (AA-AAS), the U.S. Department of Education announced that states could develop alternate assessments based on modified achievement standards (AA-MAS). This article…

Heterogeneous Coordination Environments in Lithium-Neutralized Ionomers Identified Using 1H and 7Li MAS NMR

PubMed Central

Alam, Todd M.; Jenkins, Janelle E.; Bolintineanu, Dan S.; Stevens, Mark J.; Frischknecht, Amalie L.; Buitrago, C. Francisco; Winey, Karen I.; Opper, Kathleen L.; Wagener, Kenneth B.

2012-01-01

The carboxylic acid proton and the lithium coordination environments for precise and random Li-neutralized polyethylene acrylic acid P(E-AA) ionomers were explored using high speed solid-state 1H and 7Li MAS NMR. While the 7Li NMR revealed only a single Li coordination environment, the chemical shift temperature variation was dependent on the precise or random nature of the P(E-AA) ionomer. The 1H MAS NMR revealed two different carboxylic acid proton environments in these materials. By utilizing 1H-7Li rotational echo double resonance (REDOR) MAS NMR experiments, it was demonstrated that the proton environments correspond to different average 1H-7Li distances, with the majority of the protonated carboxylic acids having a close through space contact with the Li. Molecular dynamics simulations suggest that the shortest 1H-7Li distance corresponds to un-neutralized carboxylic acids directly involved in the coordination environment of Li clusters. These solid-state NMR results show that heterogeneous structural motifs need to be included when developing descriptions of these ionomer materials.

Cardiovascular Structure and Function in Children With Middle Aortic Syndrome and Renal Artery Stenosis.

PubMed

Rumman, Rawan K; Slorach, Cameron; Hui, Wei; Matsuda-Abedini, Mina; Langlois, Valerie; Radhakrishnan, Seetha; Lorenzo, Armando J; Amaral, Joao; Mertens, Luc; Parekh, Rulan S

2017-12-01

Middle aortic syndrome (MAS) is a narrowing of the abdominal aorta, often in conjunction with renal artery stenosis (RAS). Structure and function of the cardiovascular system are not well understood. In a prospective cross-sectional study, 35 children with MAS or RAS or both (MAS/RAS) were compared with 140 age-, sex-, and body surface area-matched healthy children. Vascular assessment included carotid intima-media thickness and carotid distensibility using B-mode ultrasound and central and peripheral pulse wave velocities using applanation tonometry. Left ventricular structure and function were assessed by 2-dimensional and speckle-tracking echocardiography. Children with MAS or RAS were 12.5±3.0 years old at enrollment, and 50% were men. Carotid intima-media thickness (0.54±0.10 versus 0.44±0.05 mm; P <0.001) and central pulse wave velocities (5.58±1.83 versus 5.00±0.90 m/s; P =0.01) were significantly higher in children with disease compared with healthy children; however, after adjustment for systolic blood pressure z score, only carotid intima-media thickness remained significantly higher in the MAS/RAS group compared with the controls (β=0.07 [0.03, 0.10]). Peripheral pulse wave velocities and carotid distensibility were normal. Children with disease had significantly increased left ventricular mass and changes in diastolic function (lower E/a ratio and lower e' velocities). Systolic parameters, including ejection fraction, global longitudinal and circumferential strain, were similar to controls. Our findings demonstrate that children with MAS or RAS have evidence of carotid and left ventricular remodeling, without peripheral arterial involvement, which suggests a localized disease process. Left ventricular systolic function is preserved; however, subtle changes in diastolic function are observed. Carotid vessel changes are consistent with a 5- to 10-year aging, which underscores the importance of blood pressure control. © 2017 American Heart

Airway Management during Thyroidectomy for a Giant Goitre due to McCune-Albright Syndrome

PubMed Central

2018-01-01

There have been no case reports to date describing the technical aspects of tracheal intubation in a patient with a goitre associated with McCune-Albright syndrome (MAS), even though goitre is frequently observed in this condition. I describe a case of resection of a giant goitre in a patient with MAS, with difficult airway management. Preoperative investigation showed that the trachea was shifted to the right by the goitre, with the narrowest part of the tracheal lumen 4 mm in diameter. There was dome-shaped protuberance of the posterior pharyngeal wall into the airway. The patient had an S-shaped total spine, a short neck, and a relatively large jaw, which interfered with airway visualisation during intubation. Anaesthesia was induced with light sedation and supplemental oxygen. Endotracheal intubation was successfully performed using a fiberoptic laryngoscope and a flexible, spiral-wound, obtuse-tipped tracheal tube. PMID:29854469

Identifying Facilitators and Barriers to Physical Activity for Adults with Down Syndrome

ERIC Educational Resources Information Center

Mahy, J.; Shields, N.; Taylor, N. F.; Dodd, K. J.

2010-01-01

Background: Adults with Down syndrome are typically sedentary, and many do not participate in the recommended levels of physical activity per week. The aim of this study was to identify the facilitators and barriers to physical activity for this group. Method: Semi-structured interviews were conducted to elicit the views of adults with Down…

The Gα o Activator Mastoparan-7 Promotes Dendritic Spine Formation in Hippocampal Neurons

PubMed Central

Ramírez, Valerie T.; Ramos-Fernández, Eva; Inestrosa, Nibaldo C.

2016-01-01

Mastoparan-7 (Mas-7), an analogue of the peptide mastoparan, which is derived from wasp venom, is a direct activator of Pertussis toxin- (PTX-) sensitive G proteins. Mas-7 produces several biological effects in different cell types; however, little is known about how Mas-7 influences mature hippocampal neurons. We examined the specific role of Mas-7 in the development of dendritic spines, the sites of excitatory synaptic contact that are crucial for synaptic plasticity. We report here that exposure of hippocampal neurons to a low dose of Mas-7 increases dendritic spine density and spine head width in a time-dependent manner. Additionally, Mas-7 enhances postsynaptic density protein-95 (PSD-95) clustering in neurites and activates Gα o signaling, increasing the intracellular Ca2+ concentration. To define the role of signaling intermediates, we measured the levels of phosphorylated protein kinase C (PKC), c-Jun N-terminal kinase (JNK), and calcium-calmodulin dependent protein kinase IIα (CaMKIIα) after Mas-7 treatment and determined that CaMKII activation is necessary for the Mas-7-dependent increase in dendritic spine density. Our results demonstrate a critical role for Gα o subunit signaling in the regulation of synapse formation. PMID:26881110

Associations Between Physical Activity and Metabolic Syndrome: Comparison Between Self-Report and Accelerometry.

PubMed

Tucker, Jared M; Welk, Gregory J; Beyler, Nicholas K; Kim, Youngwon

2016-01-01

To assess the relationship between self-reported and objectively measured physical activity (PA) and metabolic syndrome and its risk factors in U.S. adults. A cross-sectional design was used for this study. The study was set among a nationally representative sample of U.S. adults. Adults, ages 20 years and older, from the National Health and Nutrition Examination Survey (NHANES) 2003-2006 (n = 5580) participated in the study. PA measures included minutes per week of moderate plus vigorous PA estimated by self-report (MVPAsr), total 7-day accelerometry (MVPAa), and accelerometer-based MVPA performed in 10-minute bouts (MVPAb). Risk factors for metabolic syndrome included blood pressure, high-density lipoprotein cholesterol, triglycerides, glucose, and waist circumference. Odds ratios (ORs) for having metabolic syndrome were calculated for men and women who met the Physical Activity Guidelines for Americans compared to those who did not. Women who did not meet the PA guidelines had significantly greater odds of having metabolic syndrome according to MVPAsr (OR = 2.20; 95% confidence interval [CI] = 1.65-2.94), MVPAa (OR = 4.40; 95% CI = 2.65-7.31), and MVPAb (OR = 2.91; 95% CI = 1.42-5.96). Men had significantly higher odds of having metabolic syndrome according to MVPAa (OR = 2.57; 95% CI = 1.91-3.45) and MVPAb (OR = 2.83; 95% CI = 1.55-5.17), but not MVPAsr. These ORs remained significant after adjusting for all potential confounders except body mass index, after which only MVPAsr in women and MVPAb in men remained significant. Individuals who do not meet the PA guidelines exhibited greater odds of having metabolic syndrome. This relationship tended to be stronger for objective PA measures than for self-report.

Activation of the ACE2/Ang-(1-7)/Mas pathway reduces oxygen-glucose deprivation-induced tissue swelling, ROS production, and cell death in mouse brain with angiotensin II overproduction.

PubMed

Zheng, J; Li, G; Chen, S; Bihl, J; Buck, J; Zhu, Y; Xia, H; Lazartigues, E; Chen, Y; Olson, J E

2014-07-25

We previously demonstrated that mice which overexpress human renin and angiotensinogen (R+A+) show enhanced cerebral damage in both in vivo and in vitro experimental ischemia models. Angiotensin-converting enzyme 2 (ACE2) counteracts the effects of angiotensin (Ang-II) by transforming it into Ang-(1-7), thus reducing the ligand for the AT1 receptor and increasing stimulation of the Mas receptor. Triple transgenic mice, SARA, which specifically overexpress ACE2 in neurons of R+A+ mice were used to study the role of ACE2 in ischemic stroke using oxygen and glucose deprivation (OGD) of brain slices as an in vitro model. We examined tissue swelling, the production of reactive oxygen species (ROS), and cell death in the cerebral cortex (CX) and the hippocampal CA1 region during OGD. Expression levels of NADPH oxidase (Nox) isoforms, Nox2 and Nox4 were measured using western blots. Results show that SARA mice and R+A+ mice treated with the Mas receptor agonist Ang-(1-7) had less swelling, cell death, and ROS production in CX and CA1 areas compared to those in R+A+ animals. Treatment of slices from SARA mice with the Mas antagonist A779 eliminated this protection. Finally, western blots revealed less Nox2 and Nox4 expression in SARA mice compared with R+A+ mice both before and after OGD. We suggest that reduced brain swelling and cell death observed in SARA animals exposed to OGD result from diminished ROS production coupled with lower expression of Nox isoforms. Thus, the ACE2/Ang-(1-7)/Mas receptor pathway plays a protective role in brain ischemic damage by counteracting the detrimental effects of Ang-II-induced ROS production. Copyright © 2014 IBRO. Published by Elsevier Ltd. All rights reserved.

Cantú Syndrome Resulting from Activating Mutation in the KCNJ8 Gene

PubMed Central

Cooper, Paige E.; Reutter, Heiko; Woelfle, Joachim; Engels, Hartmut; Grange, Dorothy K.; van Haaften, Gijs; van Bon, Bregje W.; Hoischen, Alexander; Nichols, Colin G.

2014-01-01

ATP-sensitive potassium (KATP) channels, composed of inward-rectifying potassium channel subunits (Kir6.1 and Kir6.2, encoded by KCNJ8 and KCNJ11, respectively) and regulatory sulfonylurea receptor (SUR1 and SUR2, encoded by ABCC8 and ABCC9, respectively), couple metabolism to excitability in multiple tissues. Mutations in ABCC9 cause Cantú syndrome, a distinct multi-organ disease, potentially via enhanced KATP channel activity. We screened KCNJ8 in an ABCC9 mutation-negative patient who also exhibited clinical hallmarks of Cantú syndrome (hypertrichosis, macrosomia, macrocephaly, coarse facial appearance, cardiomegaly, and skeletal abnormalities). We identified a de novo missense mutation encoding Kir6.1[p.Cys176Ser] in the patient. Kir6.1[p.Cys176Ser] channels exhibited markedly higher activity than wild-type channels, as a result of reduced ATP sensitivity, whether co-expressed with SUR1 or SUR2A subunits. Our results identify a novel causal gene in Cantú syndrome, but also demonstrate that the cardinal features of the disease result from gain of KATP channel function, not from Kir6-independent SUR2 function. PMID:24700710

The effects of prednisolone and serum malondialdehyde levels in puppies with experimentally induced meconium aspiration syndrome.

PubMed

Kirimi, E; Tuncer, O; Kösem, M; Ceylan, E; Tas, A; Tasal, I; Balahoroğlu, R; Caksen, H

2003-01-01

The aim of this study was to investigate the effect of different doses of prednisolone in puppies experimentally induced with meconium aspiration syndrome (MAS). Meconium was collected from human babies in the first day of life and was released into the trachea of 11 newborn puppies to induce MAS. Puppies were treated with 2 mg/kg prednisolone (standard dose), 30 mg/kg prednisolone (megadose) or 0.9% saline, all administered intravenously. The study ended 20 h after meconium aspiration and the lungs were then scored for histopathology. Animals not treated with prednisolone deteriorated after 8 h while respiration rate, oxygenation, pH and partial pressure of carbon dioxide values were better in the prednisolone-treated groups. Histopathology scores were better in the treatment groups compared with the control group, with megadose giving the best result. At the end of the study, serum malondialdehyde levels were significantly higher in the megadose prednisolone group compared with the other two groups. In conclusion, we determined that prednisolone reduced physiological and histological changes in puppies with MAS and that a 30 mg/kg dose was more effective than 2 mg/kg.

Idiopathic Mast Cell Activation Syndrome With Associated Salicylate Intolerance.

PubMed

Rechenauer, Tobias; Raithel, Martin; Götze, Thomas; Siebenlist, Gregor; Rückel, Aline; Baenkler, Hanns-Wolf; Hartmann, Arndt; Haller, Florian; Hoerning, André

2018-01-01

Idiopathic mast cell activation syndrome can be a rare cause for chronic abdominal pain in children. It remains a diagnosis by exclusion that can be particularly challenging due to the vast variety of possible clinical manifestations. We present a 13-year-old boy who suffered from a multitude of unspecific complaints over a long period of time. In this case, an assessment of mast cell-derived metabolites and immunohistochemical analysis of bioptic specimen was worthwhile. After ruling out, primary (oncologic) and secondary causes for mast cell activation, pharmacologic treatment adapted to the patient's salicylate intolerance resulted in a major relief of symptoms.

Patients with metabolic syndrome exhibit higher platelet activity than those with conventional risk factors for vascular disease.

PubMed

Serebruany, Victor L; Malinin, Alex; Ong, Stephen; Atar, Dan

2008-04-01

The metabolic syndrome is a matter of ongoing debate with regard to its existence, classification, clinical meaningfulness, and associated risks for vessel occlusion. Considering that persistent platelet activation is a cornerstone for the development of acute vascular events, and that patients with type 2 diabetes consistently exhibit high platelet activity, these characteristics may be critical for distinguishing and triageing specific features of metabolic syndrome among established risk factors for vascular disease. We assessed the platelet activity by conventional aggregation, expression of major surface receptors by flow cytometry, and quantitatively by rapid bedside analyzers in 20 aspirin-naïve patients with documented metabolic syndrome, and compared these with 20 untreated subjects with multiple cardiovascular risk factors. Closure time by the PFA-100 analyzer was significantly (P = 0.002) shorter in patients with metabolic syndrome indicating platelet inhibition under high shear conditions. Ultegra analyzer readings revealed increased fibrinogen binding (P = 0.0003) what in combination with the increased expression of PAC-1 (P = 0.32) strongly suggest activation of platelet glycoprotein IIb/IIIa receptor. Surface expression of CD107a (P = 0.014), and SPAN-12 (P = 0.003) were also higher in patients with metabolic syndrome. In contrast, platelet aggregation induced by collagen or ADP, CD31, CD41, CD42b, CD51/61, CD62p, CD63, CD154, CD165, so as formation of platelet-monocyte aggregates, PAR-1 thrombin receptor, and thrombospondin did not differ between groups. Patients with metabolic syndrome exhibited a higher degree of platelet activation than subjects with conventional risk factors for vascular disease. Conceptually, applying adequate antiplatelet strategies may reduce the risk of acute thrombotic events in these patients. Further prospective studies exploring this notion are encouraged.

Physical activity disparities by socioeconomic status among metabolic syndrome patients: The Fifth Korea National Health and Nutrition Examination Survey.

PubMed

Lee, Hyo; Kim, Byung-Hoon

2016-02-01

Physical activity plays an important role in preventing further progression of metabolic syndrome conditions to cardiovascular disease and type-2 diabetes. This study investigated physical activity disparities by socioeconomic status among metabolic syndrome patients. The fifth Korea National Health and Nutrition Examination Survey (2010-2012) data were analyzed (n=19,831). A revised definition of the US National Cholesterol Education Program Adult Treatment Panel III was used for screening metabolic syndrome patients. Using International Physical Activity Questionnaire, physical activity adherence was defined as participating in 150+ minutes of moderate-intensity physical activity, 75+ minutes of vigorous-intensity physical activity, or an equivalent combination of moderate-to vigorous-intensity physical activity per week. Socioeconomic status was measured by level of education and house-hold income. Among metabolic syndrome patients, physical activity adherence rate of first (lowest), second, third, and fourth quartile house-hold income group were 28.31% (95% confidence interval [CI], 26.14-30.28%), 34.68% (95% CI, 32.71-36.70), 37.44% (95% CI, 35.66-39.25), and 43.79% (95% CI, 41.85-45.75). Physical activity adherence rate of groups with elementary or lower, middle-school, high-school, and college or higher education degree were 25.17% (95% CI, 22.95-27.54), 38.2% (95% CI, 35.13-41.00), 39.60% (95% CI, 38.24-41.77), and 36.89% (95% CI, 35.77-38.03), respectively. This study found that physical activity adherence rate was lower in socioeconomically disadvantaged metabolic syndrome patients, which may aggravate health inequity status of Korean society.

Upregulation of Mas-related G Protein coupled receptor X2 in asthmatic lung mast cells and its activation by the novel neuropeptide hemokinin-1.

PubMed

Manorak, Wichayapha; Idahosa, Chizobam; Gupta, Kshitij; Roy, Saptarshi; Panettieri, Reynold; Ali, Hydar

2018-01-03

Hemokinin-1 (HK-1) is a novel neuropeptide produced by human bronchial cells and macrophages and causes contraction of human bronchi ex vivo. It is also generated by antigen/IgE-activated murine mast cells (MCs) and contributes to experimental chronic allergic airway inflammation via the activation of the neurokinin receptor-1 (NK-1R) expressed on murine MCs. We found elevated MC numbers in the lungs of individuals who died from asthma (asthma) when compared to lungs of individuals who died from other causes (non-asthma). Mas-related G Protein coupled receptor X2 (MRGPRX2) is a novel G-protein coupled receptor (GPCR) that is expressed predominantly on human MCs. We detected low level of MRGPRX2 in non-asthma lung MCs but its expression was significantly upregulated in asthma lung MCs. HK-1 caused degranulation in a human MC line (LAD2) and RBL-2H3 cells stably expressing MRGPRX2 and this response was resistant to inhibition by an NK-1R antagonist. However, knockdown of MRGPRX2 in LAD2 cells resulted in substantial inhibition of HK-1-induced degranulation. These findings suggest that while HK-1 contributes to the development of experimental asthma in mice via NK-1R on murine MCs the effect of this neuropeptide on human bronchoconstriction likely reflects the activation of MRGPRX2 on lung MCs. Thus, development of selective MRGPRX2 antagonists could serve as novel target for the modulation of asthma.

McCune-Albright syndrome and the extraskeletal manifestations of fibrous dysplasia.

PubMed

Collins, Michael T; Singer, Frederick R; Eugster, Erica

2012-05-24

Fibrous dysplasia (FD) is sometimes accompanied by extraskeletal manifestations that can include any combination of café-au-lait macules, hyperfunctioning endocrinopathies, such as gonadotropin-independent precocious puberty, hyperthyroidism, growth hormone excess, FGF23-mediated renal phosphate wasting, and/or Cushing syndrome, as well as other less common features. The combination of any of these findings, with or without FD, is known as McCune-Albright syndrome (MAS). The broad spectrum of involved tissues and the unpredictable combination of findings owe to the fact that molecular defect is due to dominant activating mutations in the widely expressed signaling protein, Gsα, and the fact these mutations arises sporadically, often times early in development, prior to gastrulation, and can distribute across many or few tissues.The complexity can be mastered by a systematic screening of potentially involved tissues and cognizance that the pattern of involved tissues is established, to some degree, in utero. Thorough testing allows the clinician to establish, often times at presentation, the full extent of the disease, and importantly as well what tissues are unaffected. Treatment and follow-up can then be focused on affected systems and a meaningful prognosis can be offered to the patient and family. The authors outline screening and treatment strategies that allow for effective management of the extraskeletal manifestations of FD.

McCune-Albright syndrome and the extraskeletal manifestations of fibrous dysplasia

PubMed Central

2012-01-01

Fibrous dysplasia (FD) is sometimes accompanied by extraskeletal manifestations that can include any combination of café-au-lait macules, hyperfunctioning endocrinopathies, such as gonadotropin-independent precocious puberty, hyperthyroidism, growth hormone excess, FGF23-mediated renal phosphate wasting, and/or Cushing syndrome, as well as other less common features. The combination of any of these findings, with or without FD, is known as McCune-Albright syndrome (MAS). The broad spectrum of involved tissues and the unpredictable combination of findings owe to the fact that molecular defect is due to dominant activating mutations in the widely expressed signaling protein, Gsα, and the fact these mutations arises sporadically, often times early in development, prior to gastrulation, and can distribute across many or few tissues. The complexity can be mastered by a systematic screening of potentially involved tissues and cognizance that the pattern of involved tissues is established, to some degree, in utero. Thorough testing allows the clinician to establish, often times at presentation, the full extent of the disease, and importantly as well what tissues are unaffected. Treatment and follow-up can then be focused on affected systems and a meaningful prognosis can be offered to the patient and family. The authors outline screening and treatment strategies that allow for effective management of the extraskeletal manifestations of FD. PMID:22640971

Everyday physical activity and adiposity in Prader-Willi syndrome.

PubMed

van den Berg-Emons, Rita; Festen, Dederieke; Hokken-Koelega, Anita; Bussmann, Johannes; Stam, Henk

2008-11-01

The aim of this study was to assess the impact of Prader-Willi syndrome (PWS) on the level of everyday physical activity and to explore whether the activity level is related to adiposity. Measurements were performed with an accelerometry-based Activity Monitor during two consecutive schooldays in 12 children with PWS (7-16 years of age) and in 12 age- and gender-matched, healthy children. Adiposity was assessed by body mass index standard deviation scores and by percentage body fat (dual energy X-ray absorptiometry). Mean duration of dynamic activities (expressed as percentage of 24 h) was lower in children with PWS than in the comparison group (8.7 [2.5]% and 12.0 [3.1]%, respectively; p = 0.01). Six children with PWS had normal activity levels. Physical activity level was not related to adiposity. The results indicate that, as a group, children with PWS have an inactive lifestyle. However, children with PWS cannot be stereotyped as inactive since half of them had normal activity levels.

Targeting human Mas-related G protein-coupled receptor X1 to inhibit persistent pain

PubMed Central

Li, Zhe; Tseng, Pang-Yen; Tiwari, Vinod; Xu, Qian; He, Shao-Qiu; Wang, Yan; Zheng, Qin; Han, Liang; Wu, Zhiping; Blobaum, Anna L.; Cui, Yiyuan; Tiwari, Vineeta; Sun, Shuohao; Cheng, Yingying; Huang-Lionnet, Julie H. Y.; Geng, Yixun; Xiao, Bo; Peng, Junmin; Hopkins, Corey; Raja, Srinivasa N.; Guan, Yun; Dong, Xinzhong

2017-01-01

Human Mas-related G protein-coupled receptor X1 (MRGPRX1) is a promising target for pain inhibition, mainly because of its restricted expression in nociceptors within the peripheral nervous system. However, constrained by species differences across Mrgprs, drug candidates that activate MRGPRX1 do not activate rodent receptors, leaving no responsive animal model to test the effect on pain in vivo. Here, we generated a transgenic mouse line in which we replaced mouse Mrgprs with human MrgprX1. This humanized mouse allowed us to characterize an agonist [bovine adrenal medulla 8–22 (BAM8–22)] and a positive allosteric modulator (PAM), ML382, of MRGPRX1. Cellular studies suggested that ML382 enhances the ability of BAM8–22 to inhibit high-voltage-activated Ca2+ channels and attenuate spinal nociceptive transmission. Importantly, both BAM8–22 and ML382 effectively attenuated evoked, persistent, and spontaneous pain without causing obvious side effects. Notably, ML382 by itself attenuated both evoked pain hypersensitivity and spontaneous pain in MrgprX1 mice after nerve injury without acquiring coadministration of an exogenous agonist. Our findings suggest that humanized MrgprX1 mice provide a promising preclinical model and that activating MRGPRX1 is an effective way to treat persistent pain. PMID:28223516

[Severe Mast Cell Activation Syndrome in a 15-year-old patient with an hypermobile Ehlers-Danlos syndrome].

PubMed

Daens, S; Grossin, D; Hermanns-Lê, T; Peeters, D; Manicourt, D

2018-02-01

We report the history of a 15-year old patient with a hypermobile Ehlers-Danlos syndrome (hEDS) (his mother, his two brothers and his sister have the same phenotype as him). He suffers mainly from a severe mast cell activation syndrome (MCAS) with an overreaction of the skin to any kind of contact (water of the shower, clothes, bed sheets) but he has also fatigue, headaches, and rash. This impressive rash is exacerbated after the shower and he has the urge to rest («shower's sign»). We describe the MCAS and its easy, fast and very effective medication management, without any significant side effects as well as its frequent association with the hEDS. We finally introduce the original term of «MASED» to this MCAS, associated, linked or entangled to hEDS.

Growth hormone-Insulin-like growth factor 1 axis hyperactivity on bone fibrous dysplasia in McCune-Albright Syndrome.

PubMed

Tessaris, Daniele; Boyce, Alison M; Zacharin, Margaret; Matarazzo, Patrizia; Lala, Roberto; De Sanctis, Luisa; Collins, Michael T

2018-04-19

In fibrous dysplasia (BFD), normal bone and bone marrow are replaced by fibro-osseous tissue, leading to fracture, deformity and pain. BFD may be isolated, or in association with cutaneous hyperpigmentation and/or hyperfunctioning endocrinopathies, termed McCune-Albright syndrome (MAS). GH hypersecretion has been described in 10%-20% of MAS-BFD patients. Aim of the study was to determine the impact of GH-insulin like growth factor 1 (IGF1) axis hyperactivity on MAS-BFD morbidities and the efficacy of GH excess therapy. A multicentric cross-sectional analysis was conducted on three different MAS cohorts. From 195 MAS patients, 37 subjects (19%) with GH excess were identified and compared with 34 MAS controls without GH hypersecretion. Mean head circumference SDS was significantly higher in GH excess: 4.025 SDS vs 0.683 SDS (P < .0001). The risk of optic neuropathy (Odds ratio 4.231; P = .039), hearing deficit (Odds ratio 2.961; P = .0481), facial asymmetry (Odds ratio 6.563; P = .0192), malignancies (Odds ratio 15.24; P = .0173) were higher in GH excess group. Overall, pharmacotherapy (octreotide alone 10-30 mg/mo or with pegvisomant 10-20 mg/d) was effective in IGF1 normalization (IGF1 Z-score between -2 and +2 SDS) in 21/29 patients (72.4%) with good compliance to the regimen. Late diagnosis and GH excess treatment after 16 years old of age was associated with an increased risk of optic neuropathy (Odds ratio 4.500; P = .0491) and growth of pituitary adenomas (Odds ratio 7.846; P = .050). GH-IGF1 hyperactivity increases risk of morbidities in MAS. Medical therapy is effective in normalizing IGF1 in most patients, and early treatment during paediatric age is associated with a decreased risk of optic neuropathy and GH-secreting adenomas growth. © 2018 John Wiley & Sons Ltd.

Matriptase initiates epidermal prokallikrein activation and disease onset in a mouse model of Netherton syndrome

PubMed Central

Sales, Katiuchia Uzzun; Masedunskas, Andrius; Bey, Alexandra L.; Rasmussen, Amber; Weigert, Roberto; List, Karin; Szabo, Roman; Overbeek, Paul A.; Bugge, Thomas H.

2010-01-01

Deficiency in the serine protease inhibitor LEKTI is the etiological origin of Netherton syndrome. The principal morbidities of the disease are stratum corneum detachment and chronic inflammation. We show that the membrane protease, matriptase, initiates Netherton syndrome in a LEKTI-deficient mouse model by premature activation of a pro-kallikrein-related cascade. Auto-activation of pro-inflammatory and stratum corneum detachment-associated pro-kallikrein-related peptidases was either low or undetectable, but they were efficiently activated by matriptase. Ablation of matriptase from LEKTI-deficient mice dampened inflammation, eliminated aberrant protease activity, prevented stratum corneum detachment, and improved epidermal barrier function. The study uncovers a pathogenic matriptase-pro-kallikrein pathway that could be operative in several human skin and inflammatory diseases. PMID:20657595

Prevalence of Burnout Syndrome in patients admitted with acute coronary syndrome.

PubMed

Prosdócimo, Ana Cláudia Giaxa; Lucina, Luciane Boreki; Marcia, Olandoski; Jobs, Priscila Megda João; Schio, Nicolle Amboni; Baldanzi, Fernanda Fachin; Costantini, Costantino Ortiz; Benevides-Pereira, Ana Maria Teresa; Guarita-Souza, Luiz Cesar; Faria-Neto, José Rocha

2015-03-01

Burnout Syndrome is the extreme emotional response to chronic occupational stress, manifesting as physical and mental exhaustion. Although associated with higher prevalence of cardiovascular risk factors, no study so far has evaluated whether the Burnout Syndrome could be a prevalent factor in non-elderly individuals active in the labor market, admitted for acute coronary syndrome (ACS). To evaluate the prevalence of the Burnout Syndrome in non-elderly, economically active patients, hospitalized with ACS. Cross-sectional study conducted in a tertiary and private cardiology center, with economically active patients aged <65 years, hospitalized with diagnosis of ACS. The Burnout Syndrome was evaluated with the Burnout Syndrome Inventory (BSI), which assesses workplace conditions and four dimensions that characterize the syndrome: emotional exhaustion (EE), emotional distancing (EmD), dehumanization (De) and professional fulfillment (PF). The Lipp's Stress Symptoms Inventory for Adults (LSSI) was applied to evaluate global stress. Of 830 patients evaluated with suspected ACS, 170 met the study criteria, 90% of which were men, overall average age was 52 years, and 40.5% had an average income above 11 minimum wages. The prevalence of the Burnout Syndrome was 4.1%. When we evaluated each dimension individually, we found high EE in 34.7%, high De in 52.4%, high EDi in 30.6%, and low PF in 5.9%. The overall prevalence of stress was 87.5%. We found a low prevalence of Burnout Syndrome in an economically active, non-elderly population among patients admitted for ACS in a tertiary and private hospital.

Prevalence of Burnout Syndrome in Patients Admitted with Acute Coronary Syndrome

PubMed Central

Prosdócimo, Ana Cláudia Giaxa; Lucina, Luciane Boreki; Marcia, Olandoski; Jobs, Priscila Megda João; Schio, Nicolle Amboni; Baldanzi, Fernanda Fachin; Costantini, Costantino Ortiz; Benevides-Pereira, Ana Maria Teresa; Guarita-Souza, Luiz Cesar; Faria-Neto, José Rocha

2015-01-01

Background Burnout Syndrome is the extreme emotional response to chronic occupational stress, manifesting as physical and mental exhaustion. Although associated with higher prevalence of cardiovascular risk factors, no study so far has evaluated whether the Burnout Syndrome could be a prevalent factor in non-elderly individuals active in the labor market, admitted for acute coronary syndrome (ACS). Objective To evaluate the prevalence of the Burnout Syndrome in non-elderly, economically active patients, hospitalized with ACS. Methods Cross-sectional study conducted in a tertiary and private cardiology center, with economically active patients aged <65 years, hospitalized with diagnosis of ACS. The Burnout Syndrome was evaluated with the Burnout Syndrome Inventory (BSI), which assesses workplace conditions and four dimensions that characterize the syndrome: emotional exhaustion (EE), emotional distancing (EmD), dehumanization (De) and professional fulfillment (PF). The Lipp’s Stress Symptoms Inventory for Adults (LSSI) was applied to evaluate global stress. Results Of 830 patients evaluated with suspected ACS, 170 met the study criteria, 90% of which were men, overall average age was 52 years, and 40.5% had an average income above 11 minimum wages. The prevalence of the Burnout Syndrome was 4.1%. When we evaluated each dimension individually, we found high EE in 34.7%, high De in 52.4%, high EDi in 30.6%, and low PF in 5.9%. The overall prevalence of stress was 87.5%. Conclusion We found a low prevalence of Burnout Syndrome in an economically active, non-elderly population among patients admitted for ACS in a tertiary and private hospital. PMID:25517388

Validation of the Auto-Inflammatory Diseases Activity Index (AIDAI) for hereditary recurrent fever syndromes

PubMed Central

Piram, Maryam; Koné-Paut, Isabelle; Lachmann, Helen J; Frenkel, Joost; Ozen, Seza; Kuemmerle-Deschner, Jasmin; Stojanov, Silvia; Simon, Anna; Finetti, Martina; Sormani, Maria Pia; Martini, Alberto; Gattorno, Marco; Ruperto, Nicolino

2014-01-01

Objectives To validate the Auto-Inflammatory Diseases Activity Index (AIDAI) in the four major hereditary recurrent fever syndromes (HRFs): familial Mediterranean fever (FMF), mevalonate kinase deficiency (MKD), tumour necrosis factor receptor-associated periodic syndrome (TRAPS) and cryopyrin-associated periodic syndromes (CAPS). Methods In 2010, an international collaboration established the content of a disease activity tool for HRFs. Patients completed a 1-month prospective diary with 12 yes/no items before a clinical appointment during which their physician assessed their disease activity by a questionnaire. Eight international experts in auto-inflammatory diseases evaluated the patient's disease activity by a blinded web evaluation and a nominal group technique consensus conference, with their consensus judgement considered the gold standard. Sensitivity/specificity/accuracy measures and the ability of the score to discriminate active from inactive patients via the best cut-off score were calculated by a receiver operating characteristic analysis. Results Consensus was achieved for 98/106 (92%) cases (39 FMF, 35 CAPS, 14 TRAPS and 10 MKD), with 26 patients declared as having inactive disease and 72 as having active disease. The median total AIDAI score was 14 (range=0–175). An AIDAI cut-off score ≥9 discriminated active from inactive patients, with sensitivity/specificity/accuracy of 89%/92%/90%, respectively, and an area under the curve of 98% (95% CI 96% to 100%). Conclusions The AIDAI score is a valid and simple tool for assessing disease activity in FMF/MKD/TRAPS/CAPS. This tool is easy to use in clinical practice and has the potential to be used as the standard efficacy measure in future clinical trials. PMID:24026675

Surfactant therapy and antibiotics in neonates with meconium aspiration syndrome: a systematic review and meta-analysis

PubMed Central

Natarajan, C K; Sankar, M J; Jain, K; Agarwal, R; Paul, V K

2016-01-01

Meconium aspiration syndrome (MAS), a common cause of respiratory failure in neonates, is associated with high mortality and morbidity. The objectives of this review were to evaluate the effects of administration of (a) surfactant—either as lung lavage (SLL) or bolus surfactant (BS) and (b) antibiotics on mortality and severe morbidities in neonates with MAS. We searched the following databases: MEDLINE via PubMed, Cochrane CENTRAL, WHOLIS and CABI using sensitive search strategies. We included eight studies on use of surfactant and three studies on use of antibiotics. Neither SLL nor BS reduced the risk of mortality in neonates with MAS (relative risk (RR) 0.38, 95% confidence interval (CI) 0.09 to 1.57; and RR 0.80, 95% CI 0.39 to 1.66, respectively). Both SLL and BS reduced the duration of hospital stay (mean difference −2.0, 95% CI −3.66 to −0.34; and RR −4.68, 95% CI −7.11 to −2.24 days, respectively) and duration of mechanical ventilation (mean difference −1.31, 95% CI −1.91 to −0.72; and mean difference 5.4, 95% CI −9.76 to −1.03 days). Neonates who received BS needed extracorporeal membrane oxygenation (ECMO) less often than the controls (RR 0.64, 95% CI 0.46 to 0.91). Use of antibiotics for MAS did not result in significant reduction in the risk of mortality, sepsis or duration of hospital stay. Surfactant administration either as SLL or BS for MAS was found to reduce the duration of mechanical ventilation and hospital stay; BS also reduced the need for ECMO. Administration of antibiotics did not show any significant clinical benefits in neonates with MAS and no evidence of sepsis. Given the limited number of studies and small number of neonates enrolled, there is an urgent need to generate more evidence on the efficacy and cost-effectiveness of these two treatment modalities before recommending them in routine clinical practice. PMID:27109092

Surfactant therapy and antibiotics in neonates with meconium aspiration syndrome: a systematic review and meta-analysis.

PubMed

Natarajan, C K; Sankar, M J; Jain, K; Agarwal, R; Paul, V K

2016-05-01

Meconium aspiration syndrome (MAS), a common cause of respiratory failure in neonates, is associated with high mortality and morbidity. The objectives of this review were to evaluate the effects of administration of (a) surfactant-either as lung lavage (SLL) or bolus surfactant (BS) and (b) antibiotics on mortality and severe morbidities in neonates with MAS. We searched the following databases: MEDLINE via PubMed, Cochrane CENTRAL, WHOLIS and CABI using sensitive search strategies. We included eight studies on use of surfactant and three studies on use of antibiotics. Neither SLL nor BS reduced the risk of mortality in neonates with MAS (relative risk (RR) 0.38, 95% confidence interval (CI) 0.09 to 1.57; and RR 0.80, 95% CI 0.39 to 1.66, respectively). Both SLL and BS reduced the duration of hospital stay (mean difference -2.0, 95% CI -3.66 to -0.34; and RR -4.68, 95% CI -7.11 to -2.24 days, respectively) and duration of mechanical ventilation (mean difference -1.31, 95% CI -1.91 to -0.72; and mean difference 5.4, 95% CI -9.76 to -1.03 days). Neonates who received BS needed extracorporeal membrane oxygenation (ECMO) less often than the controls (RR 0.64, 95% CI 0.46 to 0.91). Use of antibiotics for MAS did not result in significant reduction in the risk of mortality, sepsis or duration of hospital stay. Surfactant administration either as SLL or BS for MAS was found to reduce the duration of mechanical ventilation and hospital stay; BS also reduced the need for ECMO. Administration of antibiotics did not show any significant clinical benefits in neonates with MAS and no evidence of sepsis. Given the limited number of studies and small number of neonates enrolled, there is an urgent need to generate more evidence on the efficacy and cost-effectiveness of these two treatment modalities before recommending them in routine clinical practice.

Sensitivity enhancements in MQ-MAS NMR of spin-5/2 nuclei using modulated rf mixing pulses

NASA Astrophysics Data System (ADS)

Vosegaard, Thomas; Massiot, Dominique; Grandinetti, Philip J.

2000-08-01

An X- overlineX pulse train with stepped modulation frequency was employed to enhance the multiple-quantum to single-quantum coherence transfer in the mixing period of the multiple-quantum magic-angle spinning (MQ-MAS) experiment for spin I=5/2 nuclei. Two MQ-MAS pulse sequences employing this mixing scheme for the triple-to-single and quintuple-to-single quantum coherence transfers have been designed and their performance is demonstrated for 27Al on samples of NaSi 3AlO 8 and 9Al 2O 3·2B 2O 3 . Compared to the standard single-pulse mixing sequences, the sensitivity is approximately doubled in the present experiments.

Food Waste Composting Study from Makanan Ringan Mas

NASA Astrophysics Data System (ADS)

Kadir, A. A.; Ismail, S. N. M.; Jamaludin, S. N.

2016-07-01

The poor management of municipal solid waste in Malaysia has worsened over the years especially on food waste. Food waste represents almost 60% of the total municipal solid waste disposed in the landfill. Composting is one of low cost alternative method to dispose the food waste. This study is conducted to compost the food waste generation in Makanan Ringan Mas, which is a medium scale industry in Parit Kuari Darat due to the lack knowledge and exposure of food waste recycling practice. The aim of this study is to identify the physical and chemical parameters of composting food waste from Makanan Ringan Mas. The physical parameters were tested for temperature and pH value and the chemical parameter are Nitrogen, Phosphorus and Potassium. In this study, backyard composting was conducted with 6 reactors. Tapioca peel was used as fermentation liquid and soil and coconut grated were used as the fermentation bed. Backyard composting was conducted with six reactors. The overall results from the study showed that the temperature of the reactors were within the range which are from 30° to 50°C. The result of this study revealed that all the reactors which contain processed food waste tend to produce pH value within the range of 5 to 6 which can be categorized as slightly acidic. Meanwhile, the reactors which contained raw food waste tend to produce pH value within the range of 7 to 8 which can be categorized as neutral. The highest NPK obtained is from Reactor B that process only raw food waste. The average value of Nitrogen is 48540 mg/L, Phosphorus is 410 mg/L and Potassium is 1550 mg/L. From the comparison with common chemical fertilizer, it shows that NPK value from the composting are much lower than NPK of the common chemical fertilizer. However, comparison with NPK of organic fertilizer shown only slightly difference value in NPK.

¹H-MAS-NMR chemical shifts in hydrogen-bonded complexes of chlorophenols (pentachlorophenol, 2,4,6-trichlorophenol, 2,6-dichlorophenol, 3,5-dichlorophenol, and p-chlorophenol) and amine, and H/D isotope effects on ¹H-MAS-NMR spectra.

PubMed

Honda, Hisashi

2013-04-22

Chemical shifts (CS) of the ¹H nucleus in N···H···O type hydrogen bonds (H-bond) were observed in some complexes between chlorophenols [pentachlorophenol (PCP), 2,4,6-tricholorophenol (TCP), 2,6-dichlorophenol (26DCP), 3,5-dichlorophenol (35DCP), and p-chlorophenol (pCP)] and nitrogen-base (N-Base) by solid-state high-resolution ¹H-NMR with the magic-angle-spinning (MAS) method. Employing N-Bases with a wide range of pKa values (0.65-10.75), ¹H-MAS-NMR CS values of bridging H atoms in H-bonds were obtained as a function of the N-Base's pKa. The result showed that the CS values were increased with increasing pKa values in a range of DpKa < 0 [DpKa = pKa(N-Base)-pKa(chlorophenols)] and decreased when DpKa > 2: The maximum CS values was recorded in the PCP (pKa = 5.26)-4-methylpyridine (6.03), TCP (6.59)-imidazole (6.99), 26DCP (7.02)-2-amino-4-methylpyridine (7.38), 35DCP (8.04)-4-dimethylaminopyridine (9.61), and pCP (9.47)-4-dimethylaminopyridine (9.61) complexes. The largest CS value of 18.6 ppm was recorded in TCP-imidazole crystals. In addition, H/D isotope effects on ¹H-MAS-NMR spectra were observed in PCP-2-amino-3-methylpyridine. Based on the results of CS simulation using a B3LYP/6-311+G** function, it can be explained that a little changes of the N-H length in H-bond contribute to the H/D isotope shift of the ¹H-MAS-NMR peaks.

Understanding activity participation among individuals with Wolfram Syndrome.

PubMed

Bumpus, Emily; Hershey, Tamara; Doty, Tasha; Ranck, Samantha; Gronski, Meredith; Urano, Fumihko; Foster, Erin R

2018-06-01

Wolfram Syndrome (WFS) is a rare genetic disease associated with a variety of progressive metabolic and neurologic impairments. Previous research has focused on WFS-related impairments and biomarkers for disease progression; however, information about how WFS impacts participation in daily activities is lacking. WFS (n=45; 20 children, 25 adults) participants completed an online questionnaire about activity participation. Thirty-six non-WFS comparison participants (11 children; 25 adults) completed a portion of the questionnaire. Symptom data from a subset of WFS participants (n=20) were also examined in relation to participation data. WFS children and adults had lower participation than non-WFS children and adults in almost all activity domains, and social and exercise-related activities were the most problematic. In the subset of WFS adults with symptom data, poorer vision, balance, gait, hearing, and overall symptom severity related to lower participation. WFS appears to negatively impact participation in a variety of activities, and this effect may increase as people age and/or WFS progresses. The most functionally-pertinent WFS symptoms are those associated with neurodegeneration especially vision loss and walking and balance problems. This study revealed symptoms and activity domains that are most relevant for people with WFS and, thus, can inform current practice and treatment development research.

The Marfan Syndrome: Physical Activity Guidelines for Physical Educators, Coaches and Physicians.

ERIC Educational Resources Information Center

Romeo, Thomas J.

Intended for physical educators, this manual provides guidelines for providing safe and effective physical activity programs for children with Marfan syndrome, a congenital condition involving the connective tissues and the probable cause of sudden death by heart failure of some young competitive athletes in recent cases. The manual includes…

Antiphospholipid and antioangiogenic activity in females with recurrent miscarriage and antiphospholipid syndrome.

PubMed

Pelusa, Hector F; Pezzarini, Eleonora; Basiglio, Cecilia L; Musuruana, Jorge; Bearzotti, Mariela; Svetaz, María J; Daniele, Stella M; Bottai, Hebe; Arriaga, Sandra Mm

2017-09-01

Background Antiphospholipid syndrome is an autoimmune disease characterized by thrombosis, fetal losses and thrombocytopenia associated to antiphospholipid antibodies. They are directed to phospholipids, such as cardiolipins (anticardiolipin) and lupus anticoagulant or to complexes formed by phospholipids and protein cofactors, such as β2 glycoprotein 1 (a-β2GP1) and annexin V (a-annexin V). These auto-antibodies may be considered as a family of antibodies involved in thrombotic events and antiphospholipid activity. On the other hand, some proangiogenic factors are involved in the normal development of placental vasculature, such as the vascular endothelial growth factor. Overexpression of vascular endothelial growth factor receptor in its soluble form (sVEGFR-1) has been associated to a higher antiangiogenic activity. Our aim was to analyse the association between anticardiolipin, lupus anticoagulant, a-β2GP1, a-annexin V and sVEGFR-1 with recurrent miscarriage before week 10 of gestation in females with antiphospholipid syndrome. Methods We studied 24 females (primary or secondary antiphospholipid syndrome), who were divided into two groups: females with recurrent miscarriage before week 10 of gestation (M; n = 12) and females with no history of fetal loss (NM; n = 12). Anticardiolipin, a-β2GP1, a-annexin V and sVEGF-R1 concentrations were assessed by ELISA, while lupus anticoagulant was assessed by screening and confirmatory tests. Results A significant association was observed between the number of positive biomarkers and the belonging group ( P < 0.05). Besides, a positive result for lupus anticoagulant and a-β2GP1 was found to be significantly associated to the M group ( P < 0.05). Conclusions Lupus anticoagulant and a-β2GP1 may be implicated in pregnancies complicated by recurrent miscarriage in females with antiphospholipid syndrome.

Pharmacological significance of the interplay between angiotensin receptors: MAS receptors as putative final mediators of the effects elicited by angiotensin AT1 receptors antagonists.

PubMed

Pernomian, Larissa; Pernomian, Laena; Gomes, Mayara S; da Silva, Carlos H T P

2015-12-15

The interplay between angiotensin AT1 receptors and MAS receptors relies on several inward regulatory mechanisms from renin-angiotensin system (RAS) including the functional crosstalk between angiotensin II and angiotensin-(1-7), the competitive AT1 antagonism exhibited by angiotensin-(1-7), the antagonist feature assigned to AT1/MAS heterodimerization on AT1 signaling and the AT1-mediated downregulation of angiotensin-converting enzyme 2 (ACE2). Recently, such interplay has acquired an important significance to RAS Pharmacology since a few studies have supporting strong evidences that MAS receptors mediate the effects elicited by AT1 antagonists. The present Perspective provides an overview of the regulatory mechanisms involving AT1 and MAS receptors, their significance to RAS Pharmacology and the future directions on the interplay between angiotensin receptors. Copyright © 2015 Elsevier B.V. All rights reserved.

Macrophage activation syndrome as the initial manifestation of severe juvenile onset systemic lupus erythematosus. Favorable response to cyclophosphamide.

PubMed

Torres Jiménez, Alfonso; Solís Vallejo, Eunice; Zeferino Cruz, Maritza; Céspedes Cruz, Adriana; Sánchez Jara, Berenice

2014-01-01

The macrophage activation syndrome is a rare but potentially fatal complication of patients with autoimmune rheumatic diseases. This is a clinicopathological entity characterized by activation of histiocytes with prominent hemophagocytosis in the bone marrow and other reticuloendothelial systems. In patients with lupus it may mimic an exacerbation of the disease or infection. We report the case of a 7-year-old girl in whom the diagnosis of lupus erythematosus and macrophage activation syndrome was simultaneously made with response to the use of cyclophosphamide. Copyright © 2013 Elsevier España, S.L. All rights reserved.

Acute Inhibition of MEK Suppresses Congenital Melanocytic Nevus Syndrome in a Murine Model Driven by Activated NRAS and Wnt Signaling.

PubMed

Pawlikowski, Jeffrey S; Brock, Claire; Chen, Sheau-Chiann; Al-Olabi, Lara; Nixon, Colin; McGregor, Fiona; Paine, Simon; Chanudet, Estelle; Lambie, Wendy; Holmes, William M; Mullin, James M; Richmond, Ann; Wu, Hong; Blyth, Karen; King, Ayala; Kinsler, Veronica A; Adams, Peter D

2015-08-01

Congenital melanocytic nevus (CMN) syndrome is the association of pigmented melanocytic nevi with extra-cutaneous features, classically melanotic cells within the central nervous system, most frequently caused by a mutation of NRAS codon 61. This condition is currently untreatable and carries a significant risk of melanoma within the skin, brain, or leptomeninges. We have previously proposed a key role for Wnt signaling in the formation of melanocytic nevi, suggesting that activated Wnt signaling may be synergistic with activated NRAS in the pathogenesis of CMN syndrome. Some familial pre-disposition suggests a germ-line contribution to CMN syndrome, as does variability of neurological phenotypes in individuals with similar cutaneous phenotypes. Accordingly, we performed exome sequencing of germ-line DNA from patients with CMN to reveal rare or undescribed Wnt-signaling alterations. A murine model harboring activated NRAS(Q61K) and Wnt signaling in melanocytes exhibited striking features of CMN syndrome, in particular neurological involvement. In the first model of treatment for this condition, these congenital, and previously assumed permanent, features were profoundly suppressed by acute post-natal treatment with a MEK inhibitor. These data suggest that activated NRAS and aberrant Wnt signaling conspire to drive CMN syndrome. Post-natal MEK inhibition is a potential candidate therapy for patients with this debilitating condition.

White matter alterations in the brains of patients with active, remitted, and cured cushing syndrome: a DTI study.

PubMed

Pires, P; Santos, A; Vives-Gilabert, Y; Webb, S M; Sainz-Ruiz, A; Resmini, E; Crespo, I; de Juan-Delago, M; Gómez-Anson, B

2015-06-01

Cushing syndrome appears after chronic exposure to elevated glucocorticoid levels. Cortisol excess may alter white matter microstructure. Our purpose was to study WM changes in patients with Cushing syndrome compared with controls by using DTI and the influence of hypercortisolism. Thirty-five patients with Cushing syndrome and 35 healthy controls, matched for age, education, and sex, were analyzed through DTI (tract-based spatial statistics) for fractional anisotropy, mean diffusivity, axial diffusivity, and radial diffusivity (general linear model, family-wise error, and threshold-free cluster enhancement corrections, P < .05). Furthermore, the influence of hypercortisolism on WM DTI changes was studied by comparing 4 subgroups: 8 patients with Cushing syndrome with active hypercortisolism, 7 with Cushing syndrome with medication-remitted cortisol, 20 surgically cured, and 35 controls. Cardiovascular risk factors were used as covariates. In addition, correlations were analyzed among DTI values, concomitant 24-hour urinary free cortisol levels, and disease duration. There were widespread alterations (reduced fractional anisotropy, and increased mean diffusivity, axial diffusivity, and radial diffusivity values; P < .05) in patients with Cushing syndrome compared with controls, independent of the cardiovascular risk factors present. Both active and cured Cushing syndrome subgroups showed similar changes compared with controls. Patients with medically remitted Cushing syndrome also had reduced fractional anisotropy and increased mean diffusivity and radial diffusivity values, compared with controls. No correlations were found between DTI maps and 24-hour urinary free cortisol levels or with disease duration. Diffuse WM alterations in patients with Cushing syndrome suggest underlying loss of WM integrity and demyelination. Once present, they seem to be independent of concomitant hypercortisolism, persisting after remission/cure. © 2015 by American Journal of

Determination of the structural changes by Raman and {sup 13}C CP/MAS NMR spectroscopy on native corn starch with plasticizers

DOE Office of Scientific and Technical Information (OSTI.GOV)

Cozar, O.; Filip, C.; Tripon, C.

The plasticizing - antiplasticizing effect of water and glycerol contents on native corn starch samples is investigated by FT-Raman and {sup 13}C CP/MAS NMR spectroscopy. The presence of both amorphous and crystalline structural phases was evidenced in pure native corn starch and also in the samples containing plasticizers. Among the crystalline starch structures, the A- and V- types were suggested by CP/MAS NMR spectra.

Errorless (re)learning of everyday activities in patients with Korsakoff's syndrome: A feasibility study.

PubMed

Rensen, Yvonne C M; Egger, Jos I M; Westhoff, Josette; Walvoort, Serge J W; Kessels, Roy P C

2017-10-02

Errorless learning has proven to be an effective method for (re)learning tasks in several patient groups with amnesia. However, so far only a handful of studies have examined the effects of errorless learning in patients with Korsakoff's syndrome. The aims of this feasibility study were to (a) examine the effects of errorless learning training on (re)learning tasks in a patient with Korsakoff's syndrome, (b) examine the effects of the nature of the training on the execution of the tasks, and (c) examine characteristics that may mediate learning outcome. Professional caregivers, who were trained in errorless learning principles, taught 51 patients with Korsakoff's syndrome two everyday tasks. Significant improvements in the performance were found after an errorless intervention for different types of trained tasks (activities of daily living, chores, mobility, housekeeping). Moreover, the results of this study suggest that all patients, despite of age, educational level, or level of cognitive functioning, may benefit from errorless learning. The results showed that, despite severe amnesia, patients with Korsakoff's syndrome have the potential to (re)learn everyday skills. Errorless learning might be beneficial for memory rehabilitation in patients with Korsakoff's syndrome in clinical practice. The results of this study are clinically relevant, as successfully learning tasks using errorless learning principles might improve autonomy and independence in the daily lives of patients with Korsakoff's syndrome.

Size-dependent scanning parameters (kVp and mAs) for photon-counting spectral CT system in pediatric imaging: simulation study

NASA Astrophysics Data System (ADS)

Chen, Han; Danielsson, Mats; Xu, Cheng

2016-06-01

We are developing a photon-counting spectral CT detector with a small pixel size of 0.4× 0.5 mm2, offering a potential advantage for better visualization of small structures in pediatric patients. The purpose of this study is to determine the patient size dependent scanning parameters (kVp and mAs) for pediatric CT in two imaging cases: adipose imaging and iodinated blood imaging. Cylindrical soft-tissue phantoms of diameters between 10-25 cm were used to mimic patients of different ages from 0 to 15 y. For adipose imaging, a 5 mm diameter adipose sphere was assumed as an imaging target, while in the case of iodinated imaging, an iodinated blood sphere of 1 mm in diameter was assumed. By applying the geometry of a commercial CT scanner (GE Lightspeed VCT), simulations were carried out to calculate the detectability index, {{d}\\prime 2} , with tube potentials varying from 40 to 140 kVp. The optimal kVp for each phantom in each imaging case was determined such that the dose-normalized detectability index, {{d}\\prime 2}/ dose, is maximized. With the assumption that the detectability index in pediatric imaging is required the same as in typical adult imaging, the value of mAs at optimal kVp for each phantom was selected to achieve a reference detectability index that was obtained by scanning an adult phantom (30 cm in diameter) in a typical adult CT procedure (120 kVp and 200 mAs) using a modeled energy-integrating system. For adipose imaging, the optimal kVps are 50, 60, 80, and 120 kVp, respectively, for phantoms of 10, 15, 20, and 25 cm in diameter. The corresponding mAs values required to achieve the reference detectability index are only 9%, 23%, 24%, and 54% of the mAs that is used for adult patients at 120 kVp, for 10, 15, 20, and 25 cm diameter phantoms, respectively. In the case of iodinated imaging, a tube potential of 60 kVp was found optimal for all phantoms investigated, and the mAs values required to achieve the reference detectability

Acquisition of an instrumental activity of daily living in patients with Korsakoff's syndrome: a comparison of trial and error and errorless learning.

PubMed

Oudman, Erik; Nijboer, Tanja C W; Postma, Albert; Wijnia, Jan W; Kerklaan, Sandra; Lindsen, Karen; Van der Stigchel, Stefan

2013-01-01

Patients with Korsakoff's syndrome show devastating amnesia and executive deficits. Consequently, the ability to perform instrumental activities such as making coffee is frequently diminished. It is currently unknown whether patients with Korsakoff's syndrome are able to (re)learn instrumental activities. A good candidate for an effective teaching technique in Korsakoff's syndrome is errorless learning as it is based on intact implicit memory functioning. Therefore, the aim of the current study was two-fold: to investigate whether patients with Korsakoff's syndrome are able to (re)learn instrumental activities, and to compare the effectiveness of errorless learning with trial and error learning in the acquisition and maintenance of an instrumental activity, namely using a washing machine to do the laundry. Whereas initial learning performance in the errorless learning condition was superior, both intervention techniques resulted in similar improvement over eight learning sessions. Moreover, performance in a different spatial layout showed a comparable improvement. Notably, in follow-up sessions starting after four weeks without practice, performance was still elevated in the errorless learning condition, but not in the trial and error condition. The current study demonstrates that (re)learning and maintenance of an instrumental activity is possible in patients with Korsakoff's syndrome.

High, but not low, exercise volume shifts the balance of renin-angiotensin system toward ACE2/Mas receptor axis in skeletal muscle in obese rats.

PubMed

Frantz, Eliete Dalla Corte; Giori, Isabele Gomes; Machado, Marcus Vinícius; Magliano, D'Angelo Carlo; Freitas, Fernanda Marques; Andrade, Mariana Sodré Boêta; Vieira, Aline Bomfim; Nóbrega, Antonio Claudio Lucas; Tibiriçá, Eduardo

2017-10-01

Metabolic syndrome is a cluster of metabolic risk factors that is linked to central obesity, elevated blood pressure, insulin resistance (IR), and dyslipidemia, where the renin-angiotensin system (RAS) may provide a link among them. This study aimed to evaluate volume exercise effects comparing low vs. high volume of chronic aerobic exercise on RAS axes in skeletal muscle in a diet-induced obesity (DIO) rat model. For this, male Wistar-Kyoto rats were fed a standard chow (SC) diet or a high-fat (HF) diet for 32 wk. Animals receiving the HF diet were randomly divided into low exercise volume (LEV, 150 min/wk) and high exercise volume (HEV, 300 min/wk) at the 20th week. After 12 wk of aerobic treadmill training, the body mass and composition, blood pressure, glucose and lipid metabolism, RAS axes, insulin signaling, and inflammatory pathway were performed. HEV slowed the body mass gain, reduced intra-abdominal fat pad and leptin levels, improved total and peripheral body composition and inflammatory cytokine, reduced angiotensin II type 1 receptor expression, and increased Mas receptor protein expression compared with the HF animals. Sedentary groups (SC and HF) presented lower time to exhaustion and maximal velocity compared with the LEV and HEV groups. Both exercise training groups showed reduced resting systolic blood pressure and heart rate, improved glucose tolerance, IR, insulin signaling, and lipid profile. We conclude that the HEV, but not LEV, shifted the balance of RAS toward the ACE2/Mas receptor axis in skeletal muscle, presenting protective effects against the DIO model. Copyright © 2017 the American Physiological Society.

Setting of commercial glass ionomer cement Fuji IX by (27)Al and (19)F MAS-NMR.

PubMed

Munhoz, Tais; Karpukhina, Natalia; Hill, Robert G; Law, Robert V; De Almeida, L H

2010-04-01

To investigate the long term setting reaction in the glass ionomer restorative, Fuji IX, by monitoring the structural evolution of aluminium and fluorine species using (27)Al and (19)F MAS-NMR spectroscopy. Fuji IX cements aged from 5min up to 3 months were prepared according to the manufacturer instructions from the commercial material. The (27)Al and (19)F MAS-NMR studies were carried out on powders made after terminating the setting reaction. (27)Al MAS-NMR results show conversion of aluminium from the glass phase, where it has coordination number four, Al(IV), into the cement matrix where it has a coordination number of six, Al(VI). At least two different Al(VI) species were detected at short ageing time cements. Assignment of these species is discussed and compared with the data from other sources. The possibility for a condensed aluminium species [Al(13)(OH)(24)(H(2)O)(12)](7+) to form is considered. The ratio of aluminium in the cement, Al(VI), to the remaining unreacted in glass has been evaluated by deconvolution of the spectra. Various theoretical ratios of aluminium species in the cement matrix to the unreacted ones remaining in glass have been estimated. The (19)F MAS-NMR spectra are identical for the glass and cements at the early times and contain a dominant signal assigned to Al-F-Sr(n). The data confirms that the conversion of aluminium is a diffusion-controlled process at early stage less than 1h and it is largely complete between 1 and 6h. The comparison with the experimental data shows that the majority of aluminium cations do not form tricarboxylates but are coordinated with one or two carboxylic groups and other ligands. Insufficient amount of water and excess of glass in this cement formulation affect glass degradation mechanism. Crown Copyright 2009. Published by Elsevier Ltd. All rights reserved.

Active Rehabilitation of Concussion and Post-concussion Syndrome.

PubMed

Leddy, John J; Baker, John G; Willer, Barry

2016-05-01

Concussion is a physiological brain injury with physical, cognitive, and emotional sequelae. The macrophysiological insult to the brain affects the autonomic nervous system and its control of cerebral blood flow. Most patients recover within 2 weeks, but some do not. Persistence of symptoms beyond the generally accepted time frame for recovery is called post-concussion syndrome (PCS). PCS is not a single entity; it is a group of disorders that requires specific forms of therapy. Rest has been the mainstay of the treatment for concussion and PCS. This article discusses the rationale for the active treatment of concussion and PCS. Copyright © 2016 Elsevier Inc. All rights reserved.

Locomotor activity, object exploration and space preference in children with autism and Down syndrome.

PubMed

Kawa, Rafał; Pisula, Ewa

2010-01-01

There have been ambiguous accounts of exploration in children with intellectual disabilities with respect to the course of that exploration, and in particular the relationship between the features of explored objects and exploratory behaviour. It is unclear whether reduced exploratory activity seen with object exploration but not with locomotor activity is autism-specific or if it is also present in children with other disabilities. The purpose of the present study was to compare preschool children with autism with their peers with Down syndrome and typical development in terms of locomotor activity and object exploration and to determine whether the complexity of explored objects affects the course of exploration activity in children with autism. In total there were 27 children in the study. The experimental room was divided into three zones equipped with experimental objects providing visual stimulation of varying levels of complexity. Our results indicate that children with autism and Down syndrome differ from children with typical development in terms of some measures of object exploration (i.e. looking at objects) and time spent in the zone with the most visually complex objects.

Omega-6 polyunsaturated fatty acids, serum zinc, delta-5- and delta-6-desaturase activities and incident metabolic syndrome.

PubMed

Yary, T; Voutilainen, S; Tuomainen, T-P; Ruusunen, A; Nurmi, T; Virtanen, J K

2017-08-01

The associations of n-6 polyunsaturated fatty acids (PUFA) with metabolic syndrome have been poorly explored. We investigated the associations of the serum n-6 PUFA and the activities of enzymes involved in the PUFA metabolism, delta-5-desaturase (D5D) and delta-6-desaturase (D6D) with risk of incident metabolic syndrome. We also investigated whether zinc, a cofactor for these enzymes, modifies these associations. A prospective follow-up study was conducted on 661 men who were aged 42-60 years old at baseline in 1984-1989 and who were re-examined in 1998-2001. Men in the highest versus the lowest serum total omega-6 PUFA tertile had a 70% lower multivariate-adjusted risk of incident metabolic syndrome [odds ratio (OR) = 0.30; 95% confidence interval (CI) = 0.18-0.51, P trend < 0.001]. Inverse associations were also observed for linoleic acid, arachidonic acid and D5D activity. By contrast, men in the highest tertile of D6D activity had an 84% higher risk (OR = 1.84; 95% CI = 1.15-2.94, P trend = 0.008). Similar associations were observed with many of the metabolic syndrome components at the re-examinations. Most associations were attenuated after adjustment for body mass index. Finally, the associations of D6D and LA were stronger among those with a higher serum zinc concentration. Higher serum total n-6 PUFA, linoleic acid and arachidonic acid concentrations and D5D activity were associated with a lower risk of developing metabolic syndrome and higher D6D activity was associated with a higher risk. The role of zinc also needs to be investigated in other populations. © 2016 The British Dietetic Association Ltd.

Magnetoencephalographic Analysis of Cortical Activity in Adults with and without Down Syndrome

ERIC Educational Resources Information Center

Virji-Babul, N.; Cheung, T.; Weeks, D.; Herdman, A. T.; Cheyne, D.

2007-01-01

Background: This preliminary study served as a pilot for an ongoing analysis of spectral power in adults with Down syndrome (DS) using a 151 channel whole head magnetoencephalography (MEG). The present study is the first step for examining and comparing cortical responses during spontaneous and task related activity in DS. Method: Cortical…

[A new variant of Munchausen's syndrome by proxy: the father in an active role].

PubMed

Prakken, A B; den Hartog, L; Waelkens, J J

1991-06-01

The case is described of an eleven-year-old boy with abdominal pain and hematuria. The hematuria appears to be fabricated by the father of the boy by adding cooked meat to the urine samples. The medical history of the father is very suspect for the Münchhausen syndrome. The case of the son differs in some respects from the normal pattern of the Münchhausen by proxy syndrome: the age of the son, the role of the father as active inducer and the low social class of the family.

Effects of Mirror Therapy in Stroke Patients With Complex Regional Pain Syndrome Type 1: A Randomized Controlled Study.

PubMed

Pervane Vural, Secil; Nakipoglu Yuzer, Guldal Funda; Sezgin Ozcan, Didem; Demir Ozbudak, Sibel; Ozgirgin, Nese

2016-04-01

To investigate the effects of mirror therapy on upper limb motor functions, spasticity, and pain intensity in patients with hemiplegia accompanied by complex regional pain syndrome type 1. Randomized controlled trial. Training and research hospital. Adult patients with first-time stroke and simultaneous complex regional pain syndrome type 1 of the upper extremity at the dystrophic stage (N=30). Both groups received a patient-specific conventional stroke rehabilitation program for 4 weeks, 5 d/wk, for 2 to 4 h/d. The mirror therapy group received an additional mirror therapy program for 30 min/d. We evaluated the scores of the Brunnstrom recovery stages of the arm and hand for motor recovery, wrist and hand subsections of the Fugl-Meyer Assessment (FMA) and motor items of the FIM-motor for functional status, Modified Ashworth Scale (MAS) for spasticity, and visual analog scale (VAS) for pain severity. After 4 weeks of rehabilitation, both groups had significant improvements in the FIM-motor and VAS scores compared with baseline scores. However, the scores improved more in the mirror therapy group than the control group (P<.001 and P=.03, respectively). Besides, the patients in the mirror therapy arm showed significant improvement in the Brunnstrom recovery stages and FMA scores (P<.05). No significant difference was found for MAS scores. In patients with stroke and simultaneous complex regional pain syndrome type 1, addition of mirror therapy to a conventional stroke rehabilitation program provides more improvement in motor functions of the upper limb and pain perception than conventional therapy without mirror therapy. Copyright © 2016 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.

Loss-of-function FERMT1 mutations in kindler syndrome implicate a role for fermitin family homolog-1 in integrin activation.

PubMed

Lai-Cheong, Joey E; Parsons, Maddy; Tanaka, Akio; Ussar, Siegfried; South, Andrew P; Gomathy, Sethuraman; Mee, John B; Barbaroux, Jean-Baptiste; Techanukul, Tanasit; Almaani, Noor; Clements, Suzanne E; Hart, Ian R; McGrath, John A

2009-10-01

Kindler syndrome is an autosomal recessive disorder characterized by skin atrophy and blistering. It results from loss-of-function mutations in the FERMT1 gene encoding the focal adhesion protein, fermitin family homolog-1. How and why deficiency of fermitin family homolog-1 results in skin atrophy and blistering are unclear. In this study, we investigated the epidermal basement membrane and keratinocyte biology abnormalities in Kindler syndrome. We identified altered distribution of several basement membrane proteins, including types IV, VII, and XVII collagens and laminin-332 in Kindler syndrome skin. In addition, reduced immunolabeling intensity of epidermal cell markers such as beta1 and alpha6 integrins and cytokeratin 15 was noted. At the cellular level, there was loss of beta4 integrin immunolocalization and random distribution of laminin-332 in Kindler syndrome keratinocytes. Of note, active beta1 integrin was reduced but overexpression of fermitin family homolog-1 restored integrin activation and partially rescued the Kindler syndrome cellular phenotype. This study provides evidence that fermitin family homolog-1 is implicated in integrin activation and demonstrates that lack of this protein leads to pathological changes beyond focal adhesions, with disruption of several hemidesmosomal components and reduced expression of keratinocyte stem cell markers. These findings collectively provide novel data on the role of fermitin family homolog-1 in skin and further insight into the pathophysiology of Kindler syndrome.

[Malabsorption is a leading clinical sign of small bowel disease].

PubMed

Parfenov, A I; Krums, L M

The paper presents a variety of clinical manifestations of malabsorption syndrome (MAS) in celiac disease, collagenous sprue, Whipple's disease, Crohn's disease, intestinal lymphangiectasia, amyloidosis, common variable immune deficiency, and treatment of short bowel syndrome. It shows the specific features of the pathophysiology, diagnosis, and treatment of MAS in small bowel diseases.

Tissue expander-stimulated lengthening of arteries for the treatment of midaortic syndrome in children.

PubMed

Kim, Heung Bae; Vakili, Khashayar; Ramos-Gonzalez, Gabriel J; Stein, Deborah R; Ferguson, Michael A; Porras, Diego; Lock, James E; Chaudry, Gulraiz; Alomari, Ahmad; Fishman, Steven J

2018-01-17

Midaortic syndrome (MAS) is a rare condition characterized by stenosis of the abdominal aorta. Patients with disease refractory to medical management will usually require either endovascular therapy or surgery with use of prosthetic graft material for bypass or patch angioplasty. We report our early experience with a novel approach using a tissue expander (TE) to lengthen the normal native arteries in children with MAS, allowing primary aortic repair without the need for prosthetic graft material. We conducted a retrospective review of patients with MAS undergoing the TE-stimulated lengthening of arteries (TESLA) procedure at our institution from 2010 to 2014. Data are presented as mean (range). Five patients aged 4.8 years (3-8 years) underwent the TESLA procedure. Stages of this procedure include the following: stage I, insertion of retroaortic TE; stage II, serial TE injections; and stage III, final repair with excision of aortic stenosis and primary end-to-end aortic anastomosis. Stage II was completed in 4 months (1-9 months) with 12 (7-20) TE injections. Goal lengthening was achieved in all patients. Stage III could not be completed in one patient because of extreme aortic inflammation, which precluded safe excision of the aortic stenosis and required use of a prosthetic bypass graft. The other four patients completed stage III with two (one to three) additional vessels also requiring reconstruction (renal or mesenteric arteries). At 3.2 years (1-6 years) of follow-up, all patients are doing well. The TESLA procedure allows surgical correction of MAS without the need for prosthetic grafts in young children who are still growing. Copyright © 2017 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.

Development of microsatellites from Cornus mas L. (Cornaceae) and characterization of genetic diversity of cornelian cherries from China, central Europe, and the United States

USDA-ARS?s Scientific Manuscript database

Cornelian cherry (Cornus mas L.) is indigenous to central and southeastern Europe and is an ecologically and economically important shrub or small tree. The aim of this study was to develop molecular tools for assessing genetic diversity and provide unique molecular identification of C. mas cultivar...

Adherence to medications by patients after acute coronary syndromes.

PubMed

Sud, Anchal; Kline-Rogers, Eva M; Eagle, Kim A; Fang, Jianming; Armstrong, David F; Rangarajan, Krishna; Otten, Richard F; Stafkey-Mailey, Dana R; Taylor, Stephanie D; Erickson, Steven R

2005-11-01

Nonadherence to medication may lead to poor medical outcomes. To describe medication-taking behavior of patients with a history of acute coronary syndromes (ACS) for 4 classes of drugs and determine the relationship between self-reported adherence and patient characteristics. Consenting patients with the diagnosis of ACS were interviewed by telephone approximately 10 months after discharge. The survey elicited data characterizing the patient, current medication regimens, beliefs about drug therapy, reasons for discontinuing medications, and adherence. The survey included the Beliefs About Medicine Questionnaire providing 4 scales: Specific Necessity, Specific Concerns, General Harm, and General Overuse, and the Medication Adherence Scale (MAS). Multivariate regression was used to determine the independent variables with the strongest association to the MAS. A p value < or = 0.05 was considered significant for all analyses. Two hundred eight patients were interviewed. Mean +/- SD age was 64.9 +/- 13.0 years, with 60.6% male, 95.7% white, 57.3% with a college education, 87.9% living with > or =1 other person, and 42% indicating excellent or very good health. The percentage of patients continuing on medication at the time of the survey category ranged from 87.4% (aspirin) to 66.0% (angiotensin-converting enzyme inhibitors). Reasons for stopping medication included physician discontinuation or adverse effects. Of patients still on drug therapy, the mean MAS was 1.3 +/- 0.4, with 53.8% indicating nonadherence (score >1). The final regression model showed R(2) = 0.132 and included heart-related health status and Specific Necessity as significant predictor variables. After ACS, not all patients continue their drugs or take them exactly as prescribed. Determining beliefs about illness and medication may be helpful in developing interventions aimed at improving adherence.

Conformation analysis and molecular mobility of ethylene and tetrafluoroethylene copolymer using solid-state 19F MAS and 1H --> 19F CP/MAS NMR spectroscopy.

PubMed

Aimi, Keitaro; Ando, Shinji

2004-07-01

The changes in the conformation and molecular mobility accompanied by a phase transition in the crystalline domain were analyzed for ethylene (E) and tetrafluoroethylene (TFE) copolymer, ETFE, using variable-temperature (VT) solid-state 19F magic angle spinning (MAS) and 1H --> 19F cross-polarization (CP)/MAS NMR spectroscopy. The shifts of the signals for fluorines in TFE units to higher frequency and the continuing decrease and increase in the T1rho(F) values suggest that conformational exchange motions exist in the crystalline domain between 42 and 145 degrees C. Quantum chemical calculations of magnetic shielding constants showed that the high-frequency shift of TFE units should be induced by trans to gauche conformational changes at the CH2-CF2 linkage in the E-TFE unit. Although the 19F signals of the crystalline domain are substantially overlapped with those of the amorphous domain at ambient probe temperature (68 degrees C), they were successfully distinguished by using the dipolar filter and spin-lock pulse sequences at 145 degrees C. The dipolar coupling constants for the crystalline domain, which can be estimated by fitting the dipolar oscillation behaviors in the 1H --> 19F CP curve, showed a significant decrease with increasing temperature from 42 to 145 degrees C. This is due to the averaging of 1H-19F dipolar interactions originating from the molecular motion in the crystalline domain. The increase in molecular mobility in the crystalline domain was clearly shown by VT T1rho(F) and 1H --> 19F CP measurements in the phase transition temperature range. Copyright 2004 John Wiley & Sons, Ltd.

A Bio-Economic Case Study of Canadian Honey Bee (Hymenoptera: Apidae) Colonies: Marker-Assisted Selection (MAS) in Queen Breeding Affects Beekeeper Profits

PubMed Central

Baylis, Kathy; Hoover, Shelley E.; Currie, Rob W.; Melathopoulos, Andony P.; Pernal, Stephen F.; Foster, Leonard J.; Guarna, M. Marta

2017-01-01

Abstract Over the past decade in North America and Europe, winter losses of honey bee (Hymenoptera: Apidae) colonies have increased dramatically. Scientific consensus attributes these losses to multifactorial causes including altered parasite and pathogen profiles, lack of proper nutrition due to agricultural monocultures, exposure to pesticides, management, and weather. One method to reduce colony loss and increase productivity is through selective breeding of queens to produce disease-, pathogen-, and mite-resistant stock. Historically, the only method for identifying desirable traits in honey bees to improve breeding was through observation of bee behavior. A team of Canadian scientists have recently identified markers in bee antennae that correspond to behavioral traits in bees and can be tested for in a laboratory. These scientists have demonstrated that this marker-assisted selection (MAS) can be used to produce hygienic, pathogen-resistant honey bee colonies. Based on this research, we present a beekeeping case study where a beekeeper’s profit function is used to evaluate the economic impact of adopting colonies selected for hygienic behavior using MAS into an apiary. Our results show a net profit gain from an MAS colony of between 2% and 5% when Varroa mites are effectively treated. In the case of ineffective treatment, MAS generates a net profit benefit of between 9% and 96% depending on the Varroa load. When a Varroa mite population has developed some treatment resistance, we show that MAS colonies generate a net profit gain of between 8% and 112% depending on the Varroa load and degree of treatment resistance. PMID:28334400

Dynamic Behaviour of Tetramethylethylene Diamine (TMEDA) Ligands in Solid Organolithium Compounds: A Variable Temperature 13C and I5N CP/MAS NMR Study

NASA Astrophysics Data System (ADS)

Baumann, Wolfgang; Oprunenko, Yuri; Günther, Harald

1995-05-01

The dynamic behaviour of tetramethylethylene diamine (TMEDA) ligands in three organometallic complexes, dimeric phenyllithium, [Li(tmeda)μ-Ph]2 (1), lithium cyclopentadienide, [Li(tmeda)]C5H5 (2), and dilithium naphthalendiide, trans-[Li(tmeda)]2C10H8 (3), has been studied by CP/MAS 13C and 15N as well as 7Li MAS NMR spectroscopy of powdered samples. Two dynamic processes with free activation enthalpies of 40 and 68 kJ mol-1, respectively, were detected for 1. The first one can be assigned to ring inversion of the five-membered Li-TMEDA rings, while the second is caused by a complete rotation of the TMEDA ligands or a ring inversion of the central four-membered C-Li-C-Li metallacycle. Fast rotation of the ligands on the NMR time scale was found for 2, while 3 shows 180° ring flips of the Li-TMEDA groups, which are characterized by an energy barrier ΔG" (317) of 64 kJ mol-1

Improving patient outcomes in fibrous dysplasia/McCune-Albright syndrome: an international multidisciplinary workshop to inform an international partnership.

PubMed

Boyce, A M; Turner, A; Watts, L; Forestier-Zhang, L; Underhill, A; Pinedo-Villanueva, R; Monsell, F; Tessaris, D; Burren, C; Masi, L; Hamdy, N; Brandi, M L; Chapurlat, R; Collins, M T; Javaid, Muhammad Kassim

2017-12-01

To develop consensus on improving the management of patients, we convened an international workshop involving patients, clinicians, and researchers. Key findings included the diagnostic delay and variability in subsequent management with agreement to develop an international natural history study. We now invite other stakeholders to join the partnership. The aim of this study was develop a consensus on how to improve the management of patients with fibrous dysplasia and prioritize areas for research METHODS: An international workshop was held over 3 days involving patients, clinicians, and researchers. Each day had a combination of formal presentations and facilitated discussions that focused on clinical pathways and research. The patient workshop day highlighted the variability of patients' experience in getting a diagnosis, the knowledge of general clinical staff, and understanding long-term outcomes. The research workshop prioritized collaborations that improved understanding of the contemporary natural history of fibrous dysplasia/McCune-Albright syndrome (FD/MAS). The clinical workshop outlined the key issues around diagnostics, assessment of severity, treatment and monitoring of patients. In spite of advances in understanding the genetic and molecular underpinnings of fibrous dysplasia/McCune-Albright syndrome, clinical management remains a challenge. From the workshop, a consensus was reached to create an international, multi-stakeholder partnership to advance research and clinical care in FD/MAS. We invite other stakeholders to join the partnership.

Considerations for Consortia as States Transition Away from AA-MAS. NCEO Brief. Number 7

ERIC Educational Resources Information Center

National Center on Educational Outcomes, 2014

2014-01-01

States with an alternate assessment based on modified achievement standards (AA-MAS) that received a flexibility waiver from some of the requirements of No Child Left Behind are required to phase out their use of this assessment. And, on August 23, 2013, the U.S. Department of Education published a proposed rollback of regulation that allowed the…

Association between physical activity and metabolic syndrome in older adults in Korea: analysis of data from the Korean National Health and Nutrition Examination Survey IV.

PubMed

Choi, Mona; Yeom, Hye-A; Jung, Dukyoo

2013-09-01

The prevalence of metabolic syndrome is consistently increasing among Korean adults and is reported to be particularly high among older adults in Korea. This paper reports the prevalence of metabolic syndrome and identifies the association between metabolic syndrome and physical activity in Korean older adults. Subjects of this study were 3653 older adults who participated in the fourth Korean National Health and Nutrition Examination Survey during the years 2007-2009. The prevalence of metabolic syndrome in the study population was 46.84%. The prevalences of abdominal obesity, elevated fasting glucose, elevated triglycerides, reduced high-density lipoprotein cholesterol, and elevated blood pressure were 39.51, 45.53, 39.55, 48.24, and 69.14%, respectively, in the study population. Compared to subjects who reported low levels of physical activity, the odds ratios of metabolic syndrome for those who were moderately active and highly active were 0.93 and 0.63, respectively. Nurses should develop metabolic syndrome management programs that are tailored to the needs of the targeted group and that include individually adapted physical activity programs to promote health. © 2013 Wiley Publishing Asia Pty Ltd.

Peroxisome proliferator-activated receptor delta-agonist, GW501516, ameliorates insulin resistance, improves dyslipidaemia in monosodium L-glutamate metabolic syndrome mice.

PubMed

Chen, Wei; Wang, Li-Li; Liu, Hong-Ying; Long, Long; Li, Song

2008-09-01

We evaluated the effects of GW501516, a specific peroxisome proliferator-activated receptor beta/delta (PPARdelta) agonist in metabolic syndrome mice, obtained by perinatal injection of monosodium L-glutamate, to investigate the efficacy of GW501516 against metabolic syndrome and the effectiveness of PPARdelta activation as therapeutic target for metabolic syndrome. After 14 days treatment, GW501516 effectively improved the glucose intolerance, normalized the fasted blood glucose, and increased the serum high-density lipoprotein cholesterol (HDL-C) level. Postprandial blood glucose, serum insulin, leptin, free fatty acid (FFA) levels, and total cholesterol/HDL-C ratio were also significantly decreased. Moreover, semiquantitative reverse transcription-polymerase chain reaction results indicated that the above phenotypes might be due to (i) enhancement of fatty acid oxidation in muscle, adipose tissue and the liver; (ii) improvement of insulin-stimulated glucose transportation in skeletal muscle and adipose tissue; and (iii) reduced local glucocorticoid synthesis. Therefore, GW501516 could significantly ameliorate dyslipidaemia and insulin resistance in monosodium L-glutamate mice and activation of PPARdelta could be envisioned as a useful strategy against human metabolic syndrome and related diseases.

Autosomal dominant hypocalcemia with Bartter syndrome due to a novel activating mutation of calcium sensing receptor, Y829C.

PubMed

Choi, Keun Hee; Shin, Choong Ho; Yang, Sei Won; Cheong, Hae Il

2015-04-01

The calcium sensing receptor (CaSR) plays an important role in calcium homeostasis. Activating mutations of CaSR cause autosomal dominant hypocalcemia by affecting parathyroid hormone secretion in parathyroid gland and calcium resorption in kidney. They can also cause a type 5 Bartter syndrome by inhibiting the apical potassium channel in the thick ascending limb of the loop of Henle in the kidney. This study presents a patient who had autosomal dominant hypocalcemia with Bartter syndrome due to an activating mutation Y829C in the transmembrane domain of the CaSR. Symptoms of hypocalcemia occurred 12 days after birth and medication was started immediately. Medullary nephrocalcinosis and basal ganglia calcification were found at 7 years old and at 17 years old. Three hypercalcemic episodes occurred, one at 14 years old and two at 17 years old. The Bartter syndrome was not severe while the serum calcium concentration was controlled, but during hypercalcemic periods, the symptoms of Bartter syndrome were aggravated.

Autosomal dominant hypocalcemia with Bartter syndrome due to a novel activating mutation of calcium sensing receptor, Y829C

PubMed Central

Choi, Keun Hee; Yang, Sei Won; Cheong, Hae Il

2015-01-01

The calcium sensing receptor (CaSR) plays an important role in calcium homeostasis. Activating mutations of CaSR cause autosomal dominant hypocalcemia by affecting parathyroid hormone secretion in parathyroid gland and calcium resorption in kidney. They can also cause a type 5 Bartter syndrome by inhibiting the apical potassium channel in the thick ascending limb of the loop of Henle in the kidney. This study presents a patient who had autosomal dominant hypocalcemia with Bartter syndrome due to an activating mutation Y829C in the transmembrane domain of the CaSR. Symptoms of hypocalcemia occurred 12 days after birth and medication was started immediately. Medullary nephrocalcinosis and basal ganglia calcification were found at 7 years old and at 17 years old. Three hypercalcemic episodes occurred, one at 14 years old and two at 17 years old. The Bartter syndrome was not severe while the serum calcium concentration was controlled, but during hypercalcemic periods, the symptoms of Bartter syndrome were aggravated. PMID:25932037

Thermal maturity of type II kerogen from the New Albany Shale assessed by13C CP/MAS NMR

USGS Publications Warehouse

Werner-Zwanziger, U.; Lis, G.; Mastalerz, Maria; Schimmelmann, A.

2005-01-01

Thermal maturity of oil and gas source rocks is typically quantified in terms of vitrinite reflectance, which is based on optical properties of terrestrial woody remains. This study evaluates 13C CP/MAS NMR parameters in kerogen (i.e., the insoluble fraction of organic matter in sediments and sedimentary rocks) as proxies for thermal maturity in marine-derived source rocks where terrestrially derived vitrinite is often absent or sparse. In a suite of samples from the New Albany Shale (Middle Devonian to the Early Mississippian, Illinois Basin) the abundance of aromatic carbon in kerogen determined by 13C CP/MAS NMR correlates linearly well with vitrinite reflectance. ?? 2004 Elsevier Inc. All rights reserved.

Loss of ADAMTS3 activity causes Hennekam lymphangiectasia-lymphedema syndrome 3.

PubMed

Brouillard, Pascal; Dupont, Laura; Helaers, Raphael; Coulie, Richard; Tiller, George E; Peeden, Joseph; Colige, Alain; Vikkula, Miikka

2017-11-01

Primary lymphedema is due to developmental and/or functional defects in the lymphatic system. It may affect any part of the body, with predominance for the lower extremities. Twenty-seven genes have already been linked to primary lymphedema, either isolated, or as part of a syndrome. The proteins that they encode are involved in VEGFR3 receptor signaling. They account for about one third of all primary lymphedema cases, underscoring the existence of additional genetic factors. We used whole-exome sequencing to investigate the underlying cause in a non-consanguineous family with two children affected by lymphedema, lymphangiectasia and distinct facial features. We discovered bi-allelic missense mutations in ADAMTS3. Both were predicted to be highly damaging. These amino acid substitutions affect well-conserved residues in the prodomain and in the peptidase domain of ADAMTS3. In vitro, the mutant proteins were abnormally processed and sequestered within cells, which abolished proteolytic activation of pro-VEGFC. VEGFC processing is also affected by CCBE1 mutations that cause the Hennekam lymphangiectasia-lymphedema syndrome syndrome type1. Our data identifies ADAMTS3 as a novel gene that can be mutated in individuals affected by the Hennekam syndrome. These patients have distinctive facial features similar to those with mutations in CCBE1. Our results corroborate the recent in vitro and murine data that suggest a close functional interaction between ADAMTS3 and CCBE1 in triggering VEGFR3 signaling, a cornerstone for the differentiation and function of lymphatic endothelial cells. © The Author 2017. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

A Bio-Economic Case Study of Canadian Honey Bee (Hymenoptera: Apidae) Colonies: Marker-Assisted Selection (MAS) in Queen Breeding Affects Beekeeper Profits.

PubMed

Bixby, Miriam; Baylis, Kathy; Hoover, Shelley E; Currie, Rob W; Melathopoulos, Andony P; Pernal, Stephen F; Foster, Leonard J; Guarna, M Marta

2017-06-01

Over the past decade in North America and Europe, winter losses of honey bee (Hymenoptera: Apidae) colonies have increased dramatically. Scientific consensus attributes these losses to multifactorial causes including altered parasite and pathogen profiles, lack of proper nutrition due to agricultural monocultures, exposure to pesticides, management, and weather. One method to reduce colony loss and increase productivity is through selective breeding of queens to produce disease-, pathogen-, and mite-resistant stock. Historically, the only method for identifying desirable traits in honey bees to improve breeding was through observation of bee behavior. A team of Canadian scientists have recently identified markers in bee antennae that correspond to behavioral traits in bees and can be tested for in a laboratory. These scientists have demonstrated that this marker-assisted selection (MAS) can be used to produce hygienic, pathogen-resistant honey bee colonies. Based on this research, we present a beekeeping case study where a beekeeper's profit function is used to evaluate the economic impact of adopting colonies selected for hygienic behavior using MAS into an apiary. Our results show a net profit gain from an MAS colony of between 2% and 5% when Varroa mites are effectively treated. In the case of ineffective treatment, MAS generates a net profit benefit of between 9% and 96% depending on the Varroa load. When a Varroa mite population has developed some treatment resistance, we show that MAS colonies generate a net profit gain of between 8% and 112% depending on the Varroa load and degree of treatment resistance. © The Authors 2017. Published by Oxford University Press on behalf of Entomological Society of America.

In Situ High Temperature High Pressure MAS NMR Study on the Crystallization of AlPO 4 -5

DOE Office of Scientific and Technical Information (OSTI.GOV)

Zhao, Zhenchao; Xu, Suochang; Hu, Mary Y.

2016-01-28

A damped oscillating crystallization process of AlPO4-5 at the presence of small amount of water is demonstrated by in situ high temperature high pressure multinuclear MAS NMR. Crystalline AlPO4-5 is formed from an intermediate semicrystalline phase via continuous rearrangement of the local structure of amorphous precursor gel. Activated water catalyzes the rearrangement via repeatedly hydrolysis and condensation reaction. Strong interactions between organic template and inorganic species facilitate the ordered rearrangement. During the crystallization process, excess water, phosphate, and aluminums are expelled from the precursor. The oscillating crystallization reflects mass transportation between the solid and liquid phase during the crystallization process.more » This crystallization process is also applicable to AlPO4-5 crystallized in the presence of a relatively large amount of water.« less

FGF-23 in fibrous dysplasia of bone and its relationship to renal phosphate wasting

PubMed Central

Riminucci, Mara; Collins, Michael T.; Fedarko, Neal S.; Cherman, Natasha; Corsi, Alessandro; White, Kenneth E.; Waguespack, Steven; Gupta, Anurag; Hannon, Tamara; Econs, Michael J.; Bianco, Paolo; Gehron Robey, Pamela

2003-01-01

FGF-23, a novel member of the FGF family, is the product of the gene mutated in autosomal dominant hypophosphatemic rickets (ADHR). FGF-23 has been proposed as a circulating factor causing renal phosphate wasting not only in ADHR (as a result of inadequate degradation), but also in tumor-induced osteomalacia (as a result of excess synthesis by tumor cells). Renal phosphate wasting occurs in approximately 50% of patients with McCune-Albright syndrome (MAS) and fibrous dysplasia of bone (FD), which result from postzygotic mutations of the GNAS1 gene. We found that FGF-23 is produced by normal and FD osteoprogenitors and bone-forming cells in vivo and in vitro. In situ hybridization analysis of FGF-23 mRNA expression identified “fibrous” cells, osteogenic cells, and cells associated with microvascular walls as specific cellular sources of FGF-23 in FD. Serum levels of FGF-23 were increased in FD/MAS patients compared with normal age-matched controls and significantly higher in FD/MAS patients with renal phosphate wasting compared with those without, and correlated with disease burden bone turnover markers commonly used to assess disease activity. Production of FGF-23 by FD tissue may play an important role in the renal phosphate–wasting syndrome associated with FD/MAS. PMID:12952917

Correlation between Glutathione Peroxidase Activity and Anthropometrical Parameters in Adolescents with Down Syndrome

ERIC Educational Resources Information Center

Ordonez, F. J.; Rosety-Rodriguez, M.

2007-01-01

Since we have recently found that regular exercise increased erythrocyte antioxidant enzyme activities such as glutathione peroxidase (GPX) in adolescents with Down syndrome, these programs may be recommended. This study was designed to assess the role of anthropometrical parameters as easy, economic and non-invasive biomarkers of GPX. Thirty-one…

Nevoid Basal Cell Carcinoma Syndrome (Gorlin Syndrome).

PubMed

Bresler, Scott C; Padwa, Bonnie L; Granter, Scott R

2016-06-01

Nevoid basal cell carcinoma syndrome, or basal cell nevus syndrome (Gorlin syndrome), is a rare autosomal dominantly inherited disorder that is characterized by development of basal cell carcinomas from a young age. Other distinguishing clinical features are seen in a majority of patients, and include keratocystic odontogenic tumors (formerly odontogenic keratocysts) as well as dyskeratotic palmar and plantar pitting. A range of skeletal and other developmental abnormalities are also often seen. The disorder is caused by defects in hedgehog signaling which result in constitutive pathway activity and tumor cell proliferation. As sporadic basal cell carcinomas also commonly harbor hedgehog pathway aberrations, therapeutic agents targeting key signaling constituents have been developed and tested against advanced sporadically occurring tumors or syndromic disease, leading in 2013 to FDA approval of the first hedgehog pathway-targeted small molecule, vismodegib. The elucidation of the molecular pathogenesis of nevoid basal cell carcinoma syndrome has resulted in further understanding of the most common human malignancy.

Activating mutations in STIM1 and ORAI1 cause overlapping syndromes of tubular myopathy and congenital miosis

PubMed Central

Nesin, Vasyl; Wiley, Graham; Kousi, Maria; Ong, E-Ching; Lehmann, Thomas; Nicholl, David J.; Suri, Mohnish; Shahrizaila, Nortina; Katsanis, Nicholas; Gaffney, Patrick M.; Wierenga, Klaas J.; Tsiokas, Leonidas

2014-01-01

Signaling through the store-operated Ca2+ release-activated Ca2+ (CRAC) channel regulates critical cellular functions, including gene expression, cell growth and differentiation, and Ca2+ homeostasis. Loss-of-function mutations in the CRAC channel pore-forming protein ORAI1 or the Ca2+ sensing protein stromal interaction molecule 1 (STIM1) result in severe immune dysfunction and nonprogressive myopathy. Here, we identify gain-of-function mutations in the cytoplasmic domain of STIM1 (p.R304W) associated with thrombocytopenia, bleeding diathesis, miosis, and tubular myopathy in patients with Stormorken syndrome, and in ORAI1 (p.P245L), associated with a Stormorken-like syndrome of congenital miosis and tubular aggregate myopathy but without hematological abnormalities. Heterologous expression of STIM1 p.R304W results in constitutive activation of the CRAC channel in vitro, and spontaneous bleeding accompanied by reduced numbers of thrombocytes in zebrafish embryos, recapitulating key aspects of Stormorken syndrome. p.P245L in ORAI1 does not make a constitutively active CRAC channel, but suppresses the slow Ca2+-dependent inactivation of the CRAC channel, thus also functioning as a gain-of-function mutation. These data expand our understanding of the phenotypic spectrum of dysregulated CRAC channel signaling, advance our knowledge of the molecular function of the CRAC channel, and suggest new therapies aiming at attenuating store-operated Ca2+ entry in the treatment of patients with Stormorken syndrome and related pathologic conditions. PMID:24591628

Successfully Transitioning from the AA-MAS to the General Assessment. NCEO Policy Directions. Number 22

ERIC Educational Resources Information Center

Lazarus, Sheryl; Thurlow, Martha; Christensen, Laurene; Shyyan, Vitaliy

2014-01-01

Federal policy initiatives such as the flexibility waivers for accountability are requiring that states transition away from the use of an alternate assessment based on modified achievement standards (AA-MAS). It is expected that those students who had participated in that assessment will instead participate in the state's general assessment (or a…

P-Glycoprotein Activity in Steroid-Responsive vs. Steroid-Resistant Nephrotic Syndrome.

PubMed

Badr, Hassan S; El-Hawy, Mahmoud A; Helwa, Mohammed A

2016-11-01

To explore the expression of P-glycoprotein (P-gp) in the peripheral blood nucleated cells (PBNCs) of children with nephrotic syndrome in relation to their clinical response to glucocorticoid treatment. Thirty-six children with nephrotic syndrome (20 cases of steroid-responsive and 16 cases of steroid-resistant) were examined. All the participants were subjected to complete history taking, thorough clinical examination, laboratory investigations (24-h urinary protein, serum albumin, complete blood count with differential white blood cell count, serum cholesterol, serum urea, serum creatinine) and functional assay of P-gp using FACS Calibur flowcytometry. P-gp assay was done in both groups during remission. P-gp activity was significantly higher in steroid-resistant than steroid-sensitive cases. P-gp can be used as a predictor of outcome, as a part of laboratory evaluation of the cases before starting steroid therapy, so as to determine whether to use alternative line of therapy or use one of the P-gp inhibitors with steroid therapy.

Effects of exercise pressor reflex activation on carotid baroreflex function during exercise in humans

NASA Technical Reports Server (NTRS)

Gallagher, K. M.; Fadel, P. J.; Stromstad, M.; Ide, K.; Smith, S. A.; Querry, R. G.; Raven, P. B.; Secher, N. H.

2001-01-01

1. This investigation was designed to determine the contribution of the exercise pressor reflex to the resetting of the carotid baroreflex during exercise. 2. Ten subjects performed 3.5 min of static one-legged exercise (20 % maximal voluntary contraction) and 7 min dynamic cycling (20 % maximal oxygen uptake) under two conditions: control (no intervention) and with the application of medical anti-shock (MAS) trousers inflated to 100 mmHg (to activate the exercise pressor reflex). Carotid baroreflex function was determined at rest and during exercise using a rapid neck pressure/neck suction technique. 3. During exercise, the application of MAS trousers (MAS condition) increased mean arterial pressure (MAP), plasma noradrenaline concentration (dynamic exercise only) and perceived exertion (dynamic exercise only) when compared to control (P < 0.05). No effect of the MAS condition was evident at rest. The MAS condition had no effect on heart rate (HR), plasma lactate and adrenaline concentrations or oxygen uptake at rest and during exercise. The carotid baroreflex stimulus-response curve was reset upward on the response arm and rightward to a higher operating pressure by control exercise without alterations in gain. Activation of the exercise pressor reflex by MAS trousers further reset carotid baroreflex control of MAP, as indicated by the upward and rightward relocation of the curve. However, carotid baroreflex control of HR was only shifted rightward to higher operating pressures by MAS trousers. The sensitivity of the carotid baroreflex was unaltered by exercise pressor reflex activation. 4. These findings suggest that during dynamic and static exercise the exercise pressor reflex is capable of actively resetting carotid baroreflex control of mean arterial pressure; however, it would appear only to modulate carotid baroreflex control of heart rate.

Physical Activity and Metabolic Syndrome among Ethiopian Adults

PubMed Central

2013-01-01

BACKGROUND The global prevalence of chronic noncommunicable diseases (NCDs) is on the rise, with the majority of the growth occurring among populations in developing countries. Few studies have quantified the health benefits for physical activity among sub-Saharan African adults. We examined associations of physical activity with the prevalence of metabolic syndrome (MetS) and its components in Ethiopian men and women. METHODS This cross-sectional study of 1,843 individuals (1,117 men and 726 women) was conducted among working adults (public schools and bank employees) in Addis Ababa, Ethiopia. The study was conducted in accordance with the STEPwise approach of the World Health Organization. Physical activity was assessed using a previously validated Global Physical Activity Questionnaire. MetS was defined according to the International Diabetes Federation criteria. Multivariable logistic regression was used to estimate odds ratios (ORs) and 95% confidence intervals (CI). RESULTS The odds of MetS was inversely associated with physical activity in men (P trend = 0.02) but not women (P trend = 0.85). Among men, the OR of MetS comparing those with high vs. low levels of physical activity was 0.56 (95% CI = 0.33–0.97). For women, the corresponding OR was 1.07 (95% CI = 0.57–2.01). Physical activity was significantly and inversely associated with high waist circumference and hypertriglyceridemia among men, but no such associations were observed among women. CONCLUSIONS Higher levels of physical activity were inversely associated with MetS and several individual components among men. No similar trends were observed among women in this cohort, in part because of the small sample size. PMID:23422933

13C and 15N CP/MAS, 1H-15N SCT CP/MAS and FTIR spectroscopy as tools for qualitative detection of the presence of zwitterionic and non-ionic forms of ansa-macrolide 3-formylrifamycin SV and its derivatives in solid state.

PubMed

Przybylski, Piotr; Pyta, Krystian; Klich, Katarzyna; Schilf, Wojciech; Kamieński, Bohdan

2014-01-01

(13)C, (15)N CP/MAS, including (1)H-(13)C and (1)H-(15)N short contact time CP/MAS experiments, and FTIR methods were applied for detailed structural characterization of ansa-macrolides as 3-formylrifamycin SV (1) and its derivatives (2-6) in crystal and in powder forms. Although HPLC chromatograms for 2/CH3 OH and 2/CH3 CCl3 were the same for rifampicin crystals dissolved in respective solvents, the UV-vis data recorded for them were different in 300-375 nm region. Detailed solid state (13)C and (15)N CP/MAS NMR and FTIR studies revealed that rifampicin (2), in contrast to 3-formylrifamycin SV (1) and its amino derivatives (3-6), can occur in pure non-ionic or zwitterionic forms in crystal and in pure these forms or a mixture of them in a powder. Multinuclear CP/MAS and FTIR studies demonstrated also that 3-6 derivatives were present exclusively in pure zwitterionic forms, both in powder and in crystal. On the basis of the solid state NMR and FTIR studies, two conformers of 3-formylrifamycin SV were detected in powder form due to the different orientations of carbonyl group of amide moiety. The PM6 molecular modeling at the semi-empirical level of theory, allowed visualization the most energetically favorable non-ionic and zwitterionic forms of 1-6 antibiotics, strongly stabilized via intramolecular H-bonds. FTIR studies indicated that the originally adopted forms of these type antibiotics in crystal or in powder are stable in standard laboratory conditions in time. The results presented point to the fact that because of a possible presence of two forms of rifampicin (compound 2), quantification of the content of this antibiotic in relevant pharmaceuticals needs caution. Copyright © 2013 John Wiley & Sons, Ltd.

QuBiLS-MAS, open source multi-platform software for atom- and bond-based topological (2D) and chiral (2.5D) algebraic molecular descriptors computations.

PubMed

Valdés-Martiní, José R; Marrero-Ponce, Yovani; García-Jacas, César R; Martinez-Mayorga, Karina; Barigye, Stephen J; Vaz d'Almeida, Yasser Silveira; Pham-The, Hai; Pérez-Giménez, Facundo; Morell, Carlos A

2017-06-07

chemical structure defined by diagonal coefficients in matrix representations; and (f) several aggregation operators (invariants) applied over atom/bond-level descriptors in order to compute global indices. This software permits the parallel computation of the indices, contains a batch processing module and data curation functionalities. This program was developed in Java v1.7 using the Chemistry Development Kit library (version 1.4.19). The QuBiLS-MAS software consists of two components: a desktop interface (GUI) and an API library allowing for the easy integration of the latter in chemoinformatics applications. The relevance of the novel extensions and generalizations implemented in this software is demonstrated through three studies. Firstly, a comparative Shannon's entropy based variability study for the proposed QuBiLS-MAS and the DRAGON indices demonstrates superior performance for the former. A principal component analysis reveals that the QuBiLS-MAS approach captures chemical information orthogonal to that codified by the DRAGON descriptors. Lastly, a QSAR study for the binding affinity to the corticosteroid-binding globulin using Cramer's steroid dataset is carried out. From these analyses, it is revealed that the QuBiLS-MAS approach for atom-pair relations yields similar-to-superior performance with regard to other QSAR methodologies reported in the literature. Therefore, the QuBiLS-MAS approach constitutes a useful tool for the diversity analysis of chemical compound datasets and high-throughput screening of structure-activity data.

Metabolic Portraits of Breast Cancer by HR MAS MR Spectroscopy of Intact Tissue Samples.

PubMed

Haukaas, Tonje H; Euceda, Leslie R; Giskeødegård, Guro F; Bathen, Tone F

2017-05-16

Despite progress in early detection and therapeutic strategies, breast cancer remains the second leading cause of cancer-related death among women globally. Due to the heterogeneity and complexity of tumor biology, breast cancer patients with similar diagnosis might have different prognosis and response to treatment. Thus, deeper understanding of individual tumor properties is necessary. Cancer cells must be able to convert nutrients to biomass while maintaining energy production, which requires reprogramming of central metabolic processes in the cells. This phenomenon is increasingly recognized as a potential target for treatment, but also as a source for biomarkers that can be used for prognosis, risk stratification and therapy monitoring. Magnetic resonance (MR) metabolomics is a widely used approach in translational research, aiming to identify clinically relevant metabolic biomarkers or generate novel understanding of the molecular biology in tumors. Ex vivo proton high-resolution magic angle spinning (HR MAS) MR spectroscopy is widely used to study central metabolic processes in a non-destructive manner. Here we review the current status for HR MAS MR spectroscopy findings in breast cancer in relation to glucose, amino acid and choline metabolism.

Des schémas équivalents pour les circuits couplés multi-enroulements

NASA Astrophysics Data System (ADS)

Keradec, J. P.; Cogitore, B.; Laveuve, E.; Bensoam, M.

1994-04-01

The aim of this paper is to represent the electrical behaviour of any number of magnetically coupled windings with couplers and inductors. Two methods, mathematicaly justified, are proposed. The second one introduces only positive inductances. As an exemple, it is applied to the representation of a three column three phase transformer. The obtained circuits supply the requisite guide to design more complete circuits which allow the high frequency behaviour of wound components to be taken into account, especialy in electronics simulation softwares. Le but de cet article est de traduire le comportement électrique d'un nombre quelconque d'enroulements magnétiquement couplés, par des coupleurs et des inductances. Deux méthodes, établies mathématiquement, sont proposées. La seconde n'introduit que des inductances positives. A titre d'exemple, elle est appliquée à la représentation d'un transformateur triphasé à trois colonnes. Les schémas obtenus fournissent l'indispensable ossature de schémas plus complets, aptes à représenter le comportement haute fréquence des composants bobinés, notamment dans un logiciel de simulation électronique.

A cross-polarization based rotating-frame separated-local-field NMR experiment under ultrafast MAS conditions

NASA Astrophysics Data System (ADS)

Zhang, Rongchun; Damron, Joshua; Vosegaard, Thomas; Ramamoorthy, Ayyalusamy

2015-01-01

Rotating-frame separated-local-field solid-state NMR experiments measure highly resolved heteronuclear dipolar couplings which, in turn, provide valuable interatomic distances for structural and dynamic studies of molecules in the solid-state. Though many different rotating-frame SLF sequences have been put forth, recent advances in ultrafast MAS technology have considerably simplified pulse sequence requirements due to the suppression of proton-proton dipolar interactions. In this study we revisit a simple two-dimensional 1H-13C dipolar coupling/chemical shift correlation experiment using 13C detected cross-polarization with a variable contact time (CPVC) and systematically study the conditions for its optimal performance at 60 kHz MAS. In addition, we demonstrate the feasibility of a proton-detected version of the CPVC experiment. The theoretical analysis of the CPVC pulse sequence under different Hartmann-Hahn matching conditions confirms that it performs optimally under the ZQ (w1H - w1C = ±wr) condition for polarization transfer. The limits of the cross polarization process are explored and precisely defined as a function of offset and Hartmann-Hahn mismatch via spin dynamics simulation and experiments on a powder sample of uniformly 13C-labeled L-isoleucine. Our results show that the performance of the CPVC sequence and subsequent determination of 1H-13C dipolar couplings are insensitive to 1H/13C frequency offset frequency when high RF fields are used on both RF channels. Conversely, the CPVC sequence is quite sensitive to the Hartmann-Hahn mismatch, particularly for systems with weak heteronuclear dipolar couplings. We demonstrate the use of the CPVC based SLF experiment as a tool to identify different carbon groups, and hope to motivate the exploration of more sophisticated 1H detected avenues for ultrafast MAS.

Quality of Kelantan drinking water and knowledge, attitude and practice among the population of Pasir Mas, Malaysia.

PubMed

Ab Razak, N H; Praveena, S M; Aris, A Z; Hashim, Z

2016-02-01

Information about the quality of drinking water, together with analysis of knowledge, attitude and practice (KAP) analysis and health risk assessment (HRA) remain limited. The aims of this study were: (1) to ascertain the level of KAP regarding heavy metal contamination of drinking water in Pasir Mas; (2) to determine the concentration of heavy metals (Al, Cr, Cu, Fe, Ni, Pb, Zn and Cd) in drinking water in Pasir Mas; and (3) to estimate the health risks (non-carcinogenic and carcinogenic) caused by heavy metal exposure through drinking water using hazard quotient and lifetime cancer risk. Information on KAP was collected using a standardized questionnaire. Heavy metal analysis of drinking water samples was performed using graphite furnace atomic absorption spectrophotometry. The population of Pasir Mas has good knowledge (80%), a less positive attitude (93%) and good practice (81%) towards heavy metal contamination of drinking water. The concentrations of heavy metals analysed in this study were found to be below the permissible limits for drinking water set by the Malaysian Ministry of Health and the World Health Organization. The HRA showed no potential non-carcinogenic and carcinogenic risks from the intake of heavy metal through drinking water. By investigating the quality of drinking water, KAP and HRA, the results of this study will provide authorities with the knowledge and resources to improve the management of drinking water quality in the future. Copyright © 2015 The Royal Society for Public Health. Published by Elsevier Ltd. All rights reserved.

Who Are the Students Who May Qualify for an Alternate Assessment Based on Modified Academic Achievement Standards (AA-MAS)?: Focus Group Results. Synthesis Report 79

ERIC Educational Resources Information Center

Berndt, Sandra; Ebben, Barbara; Kubinski, Eva; Sim, Grant; Liu, Kristin; Lazarus, Sheryl; Thurlow, Martha; Christian, Elizabeth

2011-01-01

Beginning in 2007, federal regulations to two major education laws gave state departments of education the option to develop an alternate assessment based on modified achievement standards (AA-MAS) for some students with disabilities. The regulations stated that the AA-MAS was intended for students who were being instructed in the grade-level…

Role of peroxisome proliferator-activated receptors gene polymorphisms in type 2 diabetes and metabolic syndrome

PubMed Central

Dong, Chen; Zhou, Hui; Shen, Chong; Yu, Lu-Gang; Ding, Yi; Zhang, Yong-Hong; Guo, Zhi-Rong

2015-01-01

Metabolic syndrome (MetS) and type 2 diabetes mellitus (T2DM) are the serious public health problems worldwide. Moreover, it is estimated that MetS patients have about five-fold greater risk of the T2DM development compared with people without the syndrome. Peroxisome proliferator-activated receptors are a subgroup of the nuclear hormone receptor superfamily of ligand-activated transcription factors which play an important role in the pathogenesis of MetS and T2DM. All three members of the peroxisome proliferator-activated receptor (PPAR) nuclear receptor subfamily, PPARα, PPARβ/δ and PPARγ are critical in regulating insulin sensitivity, adipogenesis, lipid metabolism, and blood pressure. Recently, more and more studies indicated that the gene polymorphism of PPARs, such as Leu162Val and Val227Ala of PPARα, +294T > C of PPARβ/δ, Pro12Ala and C1431T of PPARγ, are significantly associated with the onset and progressing of MetS and T2DM in different population worldwide. Furthermore, a large body of evidence demonstrated that the glucose metabolism and lipid metabolism were influenced by gene-gene interaction among PPARs genes. However, given the complexity pathogenesis of metabolic disease, it is unlikely that genetic variation of a single locus would provide an adequate explanation of inter-individual differences which results in diverse clinical syndromes. Thus, gene-gene interactions and gene-environment interactions associated with T2DM and MetS need future comprehensive studies. PMID:25987964

Perinatally administered losartan augments renal ACE2 expression but not cardiac or renal Mas receptor in spontaneously hypertensive rats

PubMed Central

Klimas, Jan; Olvedy, Michael; Ochodnicka-Mackovicova, Katarina; Kruzliak, Peter; Cacanyiova, Sona; Kristek, Frantisek; Krenek, Peter; Ochodnicky, Peter

2015-01-01

Since the identification of the alternative angiotensin converting enzyme (ACE)2/Ang-(1-7)/Mas receptor axis, renin-angiotensin system (RAS) is a new complex target for a pharmacological intervention. We investigated the expression of RAS components in the heart and kidney during the development of hypertension and its perinatal treatment with losartan in young spontaneously hypertensive rats (SHR). Expressions of RAS genes were studied by the RT-PCR in the left ventricle and kidney of rats: normotensive Wistar, untreated SHR, SHR treated with losartan since perinatal period until week 9 of age (20 mg/kg/day) and SHR treated with losartan only until week 4 of age and discontinued until week 9. In the hypertrophied left ventricle of SHR, cardiac expressions of Ace and Mas were decreased while those of AT1 receptor (Agtr1a) and Ace2 were unchanged. Continuous losartan administration reduced LV weight (0.43 ± 0.02; P < 0.05 versus SHR) but did not influence altered cardiac RAS expression. Increased blood pressure in SHR (149 ± 2 in SHR versus 109 ± 2 mmHg in Wistar; P < 0.05) was associated with a lower renal expressions of renin, Agtr1a and Mas and with an increase in ACE2. Continuous losartan administration lowered blood pressure to control levels (105 ± 3 mmHg; P < 0.05 versus SHR), however, only renal renin and ACE2 were significantly up-regulated (for both P < 0.05 versus SHR). Conclusively, prevention of hypertension and LV hypertrophy development by losartan was unrelated to cardiac or renal expression of Mas. Increased renal Ace2, and its further increase by losartan suggests the influence of locally generated Ang-(1-7) in organ response to the developing hypertension in SHRs. PMID:25766467

Immune reconstitution inflammatory syndrome in acquired immunodeficiency syndrome.

PubMed

Jindal, A; Duggal, L; Jain, N; Malhotra, S

2008-01-01

A 33-year-old male presented with a history of fever and cough and was diagnosed to have pulmonary tuberculosis and acquired immunodeficiency syndrome (AIDS). He was started on antituberculosis therapy (ATT) followed by highly active anti-retroviral treatment (HAART) after one week. He developed an immune reconstitution inflammatory syndrome (IRIS) leading to an exacerbation of the tuberculosis disease. After HAART was stopped his condition improved dramatically.

Enzymatic Activity of Candida spp. from Oral Cavity and Urine in Children with Nephrotic Syndrome.

PubMed

Olczak-Kowalczyk, Dorota; Roszkowska-Blaim, Maria; Dąbkowska, Maria; Swoboda-Kopeć, Ewa; Gozdowski, Dariusz; Mizerska-Wasiak, Małgorzata; Demkow, Urszula; Pańczyk-Tomaszewska, Małgorzata

2017-01-01

Oral colonization with Candida spp. is not synonymous with a systemic active infection. The aim of the study was to evaluate enzymatic activity of Candida strains isolated from the oral cavity in patients with nephrotic syndrome (NS) and to compare it with the activity determined in urine. We studied 32 children with NS and 26 control healthy children. Children with NS were treated with glucocorticosteroids, cyclosporin A, mycophenolate mofetil or azathioprine. In all children, API-ZYM enzymatic tests were performed to evaluate hydrolytic enzymes of Candida isolated from the oral cavity and in urine. Candida spp. were isolated from the oral cavity in 11 patients with NS (34.4%), all receiving immunosuppressive treatment. All strains produced valine arylamidase, 9 alpha-glucosidase (E16), and 9 N-acetyl-beta-glucosaminidase (E18). A positive correlation between the presence of Candida in the oral cavity and E16 and E18 enzymatic activity in both oral cavity and urine was found. A dose of cyclosporin A had an effect on the enzymatic activity (p < 0.05). We conclude that immunosuppressive treatment of NS in children may predispose to systemic Candida invasion. The results of this study suggest that oral candida infection should be monitored in children with nephrotic syndrome, particularly those treated with immunosuppressive agents.

Mid-aortic syndrome with renovascular hypertension and multisystem involvement in a girl with familiar neurofibromatosis von Recklinghausen type 1.

PubMed

Petrak, Borivoj; Bendova, Sarka; Seeman, Tomas; Klein, Tibor; Lisy, Jiri; Zatrapa, Tomas; Marikova, Tana

2007-12-01

Neurofibromatosis von Recklinghausen type 1 (NF1) is an autosomal dominant neurocutaneous disorder affecting one in 3 000-4 000 individuals. Mid-aortic syndrome (MAS) is a rare condition characterized by segmental narrowing of abdominal aorta and stenosis of its major branches - mainly renal arteries, including manifestation of renovascular hypertension. MAS can be caused by different diseases, including NF1. A 9 years old girl with primary diagnosis of NF1 combined with renovascular hypertension due to MAS, suffered of bilateral optic and chiasm glioma, pubertas praecox, speech disorder, light mental retardation and scoliosis. We have found a mutation in exone 34 of the NF1 gene (17q11.2). Her father has been also diagnosed with NF1 and hypertension developed at early age. He has the same mutation in exone 34 of NF1 gene. The girl is currently treated with conservative antihypertensive medication with positive effect. Bilateral optic and chiasm glioma are asymptomatic at the time and they had been without progress over period of time. Any vascular surgery, neurosurgical and oncological therapy are not indicated at the present time. This article is a summary of clinical findings in patient with NF1 due to NF1 gene mutation in exone 34. It confirms the importance of complex multidisciplinar approach to examination and taking care of NF1 patients and their families.

Lung inflammatory and oxidative alterations after exogenous surfactant therapy fortified with budesonide in rabbit model of meconium aspiration syndrome.

PubMed

Mikolka, P; Kopincová, J; Košútová, P; Čierny, D; Čalkovská, A; Mokrá, D

2016-12-22

Meconium aspiration syndrome (MAS) triggers inflammatory and oxidative pathways which can inactivate both pulmonary surfactant and therapeutically given exogenous surfactant. Glucocorticoid budesonide added to exogenous surfactant can inhibit inflammation and thereby enhance treatment efficacy. Neonatal meconium (25 mg/ml, 4 ml/kg) was administered intratracheally (i.t.) to rabbits. When the MAS model was prepared, animals were treated with budesonide i.t. (Pulmicort, 0.25 mg/kg, M+B); with surfactant lung lavage (Curosurf®, 10 ml/kg, 5 mg phospholipids/ml, M+S) followed by undiluted Curosurf® i.t. (100 mg phospholipids/kg); with combination of budesonide and surfactant (M+S+B); or were untreated (M); or served as controls with saline i.t. instead of meconium (C). Animals were oxygen-ventilated for additional 5 h. Cell counts in the blood and bronchoalveolar lavage fluid (BAL), lung edema formation (wet/dry weight ratio), oxidative damage of lipids/ proteins and inflammatory expression profiles (IL-2, IL-6, IL-13, TNF-alpha) in the lung homogenate and plasma were determined. Combined surfactant+budesonide therapy was the most effective in reduction of neutrophil counts in BAL, oxidative damage, levels and mRNA expression of cytokines in the lung, and lung edema formation compared to untreated animals. Curosurf fortified with budesonide mitigated lung inflammation and oxidative modifications what indicate the perspectives of this treatment combination for MAS therapy.

Somatic GNAS Mutation Causes Widespread and Diffuse Pituitary Disease in Acromegalic Patients with McCune-Albright Syndrome

PubMed Central

Vortmeyer, Alexander O.; Gläsker, Sven; Mehta, Gautam U.; Abu-Asab, Mones S.; Smith, Jonathan H.; Zhuang, Zhengping; Collins, Michael T.

2012-01-01

Context: McCune-Albright syndrome (MAS) is caused by sporadic mutations of the GNAS. Patients exhibit features of acromegaly. In most patients, GH-secreting pituitary adenomas have been held responsible for this presentation. However, surgical adenomectomy rarely eliminates excess GH production. Objective: The aim of this study was to elucidate pituitary pathology in patients with MAS and to explain the basis of failure of adenomectomy to eliminate GH hypersecretion. Design and Setting: We conducted a case series at the National Institutes of Health. Intervention(s): Interventions included medical therapy and transsphenoidal surgery. Patients and Main Outcome Measures: We studied clinical and imaging features and the histology and molecular features of the pituitary of four acromegalic MAS patients. Results: We identified widespread and diffuse pituitary gland disease. The primary pathological changes were characterized by hyperplastic and neoplastic change, associated with overrepresentation of somatotroph cells in structurally intact tissue areas. Genetic analysis of multiple microdissected samples of any type of histological area consistently revealed identical GNAS mutations in individual patients. The only patient with remission after surgery received complete hypophysectomy in addition to removal of multiple GH-secreting tumors. Conclusions: These findings indicate developmental effects of GNAS mutation on the entire anterior pituitary gland. The pituitary of individual cases contains a spectrum of changes with regions of normal appearing gland, hyperplasia, and areas of fully developed adenoma formation, as well as transitional stages between these entities. The primary change underlying acromegaly in MAS patients is somatotroph hyperplasia involving the entire pituitary gland, with or without development of somatotroph adenoma. Thus, successful clinical management, whether it is medical, surgical, or via irradiation, must target the entire pituitary, not

Thrombomodulin Mutations in Atypical Hemolytic–Uremic Syndrome

PubMed Central

Delvaeye, Mieke; Noris, Marina; De Vriese, Astrid; Esmon, Charles T.; Esmon, Naomi L.; Ferrell, Gary; Del-Favero, Jurgen; Plaisance, Stephane; Claes, Bart; Lambrechts, Diether; Zoja, Carla; Remuzzi, Giuseppe; Conway, Edward M.

2012-01-01

BACKGROUND The hemolytic–uremic syndrome consists of the triad of microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. The common form of the syndrome is triggered by infection with Shiga toxin–producing bacteria and has a favorable outcome. The less common form of the syndrome, called atypical hemolytic–uremic syndrome, accounts for about 10% of cases, and patients with this form of the syndrome have a poor prognosis. Approximately half of the patients with atypical hemolytic–uremic syndrome have mutations in genes that regulate the complement system. Genetic factors in the remaining cases are unknown. We studied the role of thrombomodulin, an endothelial glycoprotein with anticoagulant, antiinflammatory, and cytoprotective properties, in atypical hemolytic–uremic syndrome. METHODS We sequenced the entire thrombomodulin gene (THBD) in 152 patients with atypical hemolytic–uremic syndrome and in 380 controls. Using purified proteins and cell-expression systems, we investigated whether thrombomodulin regulates the complement system, and we characterized the mechanisms. We evaluated the effects of thrombomodulin missense mutations associated with atypical hemolytic–uremic syndrome on complement activation by expressing thrombomodulin variants in cultured cells. RESULTS Of 152 patients with atypical hemolytic–uremic syndrome, 7 unrelated patients had six different heterozygous missense THBD mutations. In vitro, thrombomodulin binds to C3b and factor H (CFH) and negatively regulates complement by accelerating factor I–mediated inactivation of C3b in the presence of cofactors, CFH or C4b binding protein. By promoting activation of the plasma procarboxypeptidase B, thrombomodulin also accelerates the inactivation of anaphylatoxins C3a and C5a. Cultured cells expressing thrombomodulin variants associated with atypical hemolytic–uremic syndrome had diminished capacity to inactivate C3b and to activate procarboxypeptidase B and were

Ammonia Vapor Removal by Cu(3)(BTC)(2) and Its Characterization by MAS NMR.

PubMed

Peterson, Gregory W; Wagner, George W; Balboa, Alex; Mahle, John; Sewell, Tara; Karwacki, Christopher J

2009-07-01

Adsorption equilibria and NMR experiments were performed to study the adsorption and interactions of ammonia with metal-organic framework (MOF) HKUST-1, or Cu(3)(BTC)(2) (BTC = 1,3,5-benzenetricarboxylate). Ammonia capacities determined from chemical breakthrough measurements show significantly higher uptake capacities than from adsorption alone, suggesting a stronger interaction involving a potential reaction with the Cu(3)(BTC)(2) framework. Indeed, (1)H MAS NMR reveals that a major disruption of the relatively simple spectrum of Cu(3)(BTC)(2) occurs to generate a composite spectrum consistent with Cu(OH)(2) and (NH(4))(3)BTC species under humid conditions-the anticipated products of a copper(II) carboxylate reacted with limited ammonia. These species are not detected under dry conditions; however, reaction stoichiometry combined with XRD results suggests the partial formation of an indeterminate diammine copper (II) complex with some residual Cu(3)(BTC)(2) structure retained. Cu(II)-induced paramagnetic shifts exhibited by various species in (1)H and (13)C MAS NMR spectra are consistent with model compounds and previous literature. Although results show extensive ammonia capacity of Cu(3)(BTC)(2), much of the capacity is due to reaction with the structure itself, causing a permanent loss in porosity and structural integrity.

Ammonia Vapor Removal by Cu3(BTC)2 and Its Characterization by MAS NMR

PubMed Central

Peterson, Gregory W.; Wagner, George W.; Balboa, Alex; Mahle, John; Sewell, Tara; Karwacki, Christopher J.

2009-01-01

Adsorption equilibria and NMR experiments were performed to study the adsorption and interactions of ammonia with metal-organic framework (MOF) HKUST-1, or Cu3(BTC)2 (BTC = 1,3,5-benzenetricarboxylate). Ammonia capacities determined from chemical breakthrough measurements show significantly higher uptake capacities than from adsorption alone, suggesting a stronger interaction involving a potential reaction with the Cu3(BTC)2 framework. Indeed, 1H MAS NMR reveals that a major disruption of the relatively simple spectrum of Cu3(BTC)2 occurs to generate a composite spectrum consistent with Cu(OH)2 and (NH4)3BTC species under humid conditions—the anticipated products of a copper(II) carboxylate reacted with limited ammonia. These species are not detected under dry conditions; however, reaction stoichiometry combined with XRD results suggests the partial formation of an indeterminate diammine copper (II) complex with some residual Cu3(BTC)2 structure retained. Cu(II)-induced paramagnetic shifts exhibited by various species in 1H and 13C MAS NMR spectra are consistent with model compounds and previous literature. Although results show extensive ammonia capacity of Cu3(BTC)2, much of the capacity is due to reaction with the structure itself, causing a permanent loss in porosity and structural integrity. PMID:20161144

MAS-NMR investigations of the crystallization behaviour of lithium aluminum silicate (LAS) glasses containing P 2O 5 and TiO 2 nucleants

NASA Astrophysics Data System (ADS)

Ananthanarayanan, A.; Kothiyal, G. P.; Montagne, L.; Revel, B.

2010-06-01

Lithium aluminum silicate (LAS) glass of composition (mol%) 20.4Li 2O-4.0Al 2O 3-68.6SiO 2-3.0K 2O-2.6B 2O 3-0.5P 2O 5-0.9TiO 2 was prepared by melt quenching. The glass was then nucleated and crystallized based on differential thermal analysis (DTA) data and was characterized by 29Si, 31P, 11B and 27Al MAS-NMR. XRD and 29Si NMR showed that lithium metasilicate (Li 2SiO 3) is the first phase to c form followed by cristobalite (SiO 2) and lithium disilicate (Li 2Si 2O 5). 29Si MAS-NMR revealed a change in the network structure already for the glasses nucleated at 550 °C. Since crystalline Li 3PO 4, as observed by 31P MAS-NMR, forms concurrently with the silicate phases, we conclude that crystalline Li 3PO 4 does not act as a nucleating agent for lithium silicate phases. Moreover, 31P NMR indicates the formation of M-PO 4 ( M=B, Al or Ti) complexes. The presence of BO 3 and BO 4 structural units in all the glass/glass-ceramic samples is revealed through 11B MAS-NMR. B remains in the residual glass and the crystallization of silicate phases causes a reduction in the number of alkali ions available for charge compensation. As a result, the number of trigonally coordinated B (BO 3) increases at the expense of tetrahedrally coordinated B (BO 4). The 27Al MAS-NMR spectra indicate the presence of tetrahedrally coordinated Al species, which are only slightly perturbed by the crystallization.

Role of angiotensin-converting enzyme 2/angiotensin-(1-7)/Mas axis in the hypotensive effect of azilsartan.

PubMed

Iwanami, Jun; Mogi, Masaki; Tsukuda, Kana; Wang, Xiao-Li; Nakaoka, Hirotomo; Ohshima, Kousei; Chisaka, Toshiyuki; Bai, Hui-Yu; Kanno, Harumi; Min, Li-Juan; Horiuchi, Masatsugu

2014-07-01

The possible counteracting effect of angiotensin (Ang)-converting enzyme (ACE)2/Ang-(1-7)/Mas axis against the ACE/Ang II/Ang II type 1 (AT1) receptor axis in blood pressure control has been previously described. We examined the possibility that this pathway might be involved in the anti-hypertensive effect of a newly developed AT1 receptor blocker (ARB), azilsartan, and compared azilsartan's effects with those of another ARB, olmesartan. Transgenic mice carrying the human renin and angiotensinogen genes (hRN/hANG-Tg) were given azilsartan or olmesartan. Systolic and diastolic blood pressure, as determined by radiotelemetry, were significantly higher in hRN/hANG-Tg mice than in wild-type (WT) mice. Treatment with azilsartan or olmesartan (1 or 5 mg kg(-1) per day) significantly decreased systolic and diastolic blood pressure, and the blood pressure-lowering effect of azilsartan was more marked than that of olmesartan. The urinary Na concentration decreased in an age-dependent manner in hRN/hANG-Tg mice. Administration of azilsartan or olmesartan increased urinary Na concentration, and this effect was weaker with olmesartan than with azilsartan. Azilsartan decreased ENaC-α mRNA expression in the kidney and decreased the ratio of heart to body weight. Olmesartan had a similar but less-marked effect. ACE2 mRNA expression was lower in the kidneys and hearts of hRN/hANG-Tg mice than in WT mice. This decrease in ACE2 mRNA expression was attenuated by azilsartan, but not by olmesartan. These results suggest that the hypotensive and anti-hypertrophic effects of azilsartan may involve activation of the ACE2/Ang-(1-7)/Mas axis with AT1 receptor blockade.

Changes in jaw muscle activity and the physical properties of foods with different textures during chewing behaviors.

PubMed

Iguchi, Hiroko; Magara, Jin; Nakamura, Yuki; Tsujimura, Takanori; Ito, Kayoko; Inoue, Makoto

2015-12-01

This study aimed to investigate how the activity of the masseter (Mas) and suprahyoid (Hyoid) muscles is influenced by the physical properties of food, how changes in the rheological properties of food differ between different foods during the process of food reduction, and how different salivary flow rates affect bolus-making capability during masticatory behavior in healthy humans. Ten healthy adults participated in this study. Electromyographic (EMG) recordings were obtained from the Mas and Hyoid muscles, and 15 g of steamed rice and rice cake was prepared as test foods. In the ingestion test, the subjects were asked to eat each food in their usual manner. The chewing duration, number of chewing cycles before the first swallow, Mas and Hyoid EMG activity, and chewing cycle time were compared between the foods. Total chewing duration was divided into three substages: early, middle, and late; chewing cycle time and EMG activity per chewing cycle of each substage were compared between the foods and among the substages. In the spitting test, the rheological properties of the bolus at the end of each substage were compared between the foods and among the substages. Finally, stimulated salivary flow rates were measured and the relationships between salivary flow rate and chewing duration, EMG activity, and changes in physical food characteristics were investigated. There were significant differences in total chewing duration and the number of chewing cycles, but not in chewing cycle time, between the foods, which had similar hardness values. The EMG activity levels of the Mas and Hyoid per chewing cycle for the rice cake were significantly greater than for the steamed rice throughout the recording periods. While Mas activity did not change among the substages during chewing, Hyoid EMG activity decreased as chewing progressed. Chewing cycle time also gradually decreased as chewing progressed. The hardness of both foods initially increased, then gradually decreased

Activity of LPO Processes in Women with Polycystic Ovarian Syndrome and Infertility.

PubMed

Kolesnikova, L I; Kolesnikov, S I; Darenskaya, M A; Grebenkina, L A; Nikitina, O A; Lazareva, L M; Suturina, L V; Danusevich, I N; Druzhinina, E B; Semendyaev, A A

2017-01-01

Specific features of LPO processes and antioxidant defense were studied in patients with polycystic ovarian syndrome (PCOS) and infertility. Changes in LPO processes in patients with PCOS were compensatory, which manifested in increased α-tocopherol and retinol concentrations and moderate decrease in superoxide dismutase activity. Intensification of prooxidant processes was found in the group of patients with infertility without PCOS. The observed changes necessitate differentiated approach to the treatment of these patients.

Thrombocytose au cours d'un syndrome d'activation macrophagique compliquant un lupus érythémateux systémique

PubMed Central

Lekpa, Fernando Kemta; Ndongo, Souhaïbou; Fall, Seynabou; Pouye, Abdoulaye; Ka, Mamadou Mourtalla; Moreira-Diop, Thérèse

2014-01-01

Le syndrome d'activation macrophagique (SAM) est une complication du lupus érythémateux systémique (LES), due à l'activation et la prolifération incontrôlée des macrophages dans la moelle osseuse. La bycytopénie voire la pancytopénie est constante. Nous rapportons un cas atypique de SAM diagnostiqué au même moment qu'un LES chez une patiente noire de 17 ans. Le tableau initial associait une fièvre, un syndrome inflammatoire, une anémie, un taux normal de leucocytes et plus surprenant, une thrombocytose. PMID:25584123

Differential Regulation of PAI-1 in Hantavirus Cardiopulmonary Syndrome and Hemorrhagic Fever With Renal Syndrome.

PubMed

Bellomo, Carla; Korva, Miša; Papa, Anna; Mäkelä, Satu; Mustonen, Jukka; Avšič-Županc, Tatjana; Vaheri, Antti; Martinez, Valeria P; Strandin, Tomas

2018-02-01

We analyzed the levels of circulating tissue plasminogen activator (tPA) and plasminogen activator inhibitor (PAI)-1 in acute hantavirus cardiopulmonary syndrome (HCPS) and hemorrhagic fever with renal syndrome (HFRS). The levels of tPA commonly increased in both diseases, whereas PAI-1 correlated with disease severity in HCPS but not in HFRS.

Clustering of leptin and physical activity with components of metabolic syndrome in Iranian population: an exploratory factor analysis.

PubMed

Esteghamati, Alireza; Zandieh, Ali; Khalilzadeh, Omid; Morteza, Afsaneh; Meysamie, Alipasha; Nakhjavani, Manouchehr; Gouya, Mohammad Mehdi

2010-10-01

Metabolic syndrome (MetS), manifested by insulin resistance, dyslipidemia, central obesity, and hypertension, is conceived to be associated with hyperleptinemia and physical activity. The aim of this study was to elucidate the factors underlying components of MetS and also to test the suitability of leptin and physical activity as additional components of this syndrome. Data of the individuals without history of diabetes mellitus, aged 25-64 years, from third national surveillance of risk factors of non-communicable diseases (SuRFNCD-2007), were analyzed. Performing factor analysis on waist circumference, homeostasis model assessment of insulin resistance, systolic blood pressure, triglycerides (TG) and high-density lipoprotein cholesterol (HDL-C) led to extraction of two factors which explained around 59.0% of the total variance in both genders. When TG and HDL-C were replaced by TG to HDL-C ratio, a single factor was obtained. In contrast to physical activity, addition of leptin was consistent with one-factor structure of MetS and improved the ability of suggested models to identify obesity (BMI≥30 kg/m2, P<0.01), using receiver-operator characteristics curve analysis. In general, physical activity loaded on the first identified factor. Our study shows that one underlying factor structure of MetS is also plausible and the inclusion of leptin does not interfere with this structure. Further, this study suggests that physical activity influences MetS components via modulation of the main underlying pathophysiologic pathway of this syndrome.

Discriminating heavy aerosol, clouds, and fires during SCAR-B: Application of airborne multispectral MAS data

NASA Astrophysics Data System (ADS)

King, Michael D.; Tsay, Si-Chee; Ackerman, Steven A.; Larsen, North F.

1998-12-01

A multispectral scanning spectrometer was used to obtain measurements of the reflection function and brightness temperature of smoke, clouds, and terrestrial surfaces at 50 discrete wavelengths between 0.55 and 14.2 μm. These observations were obtained from the NASA ER-2 aircraft as part of the Smoke, Clouds, and Radiation-Brazil (SCAR-B) campaign, conducted over a 1500×1500 km region of cerrado and rain forest throughout Brazil between August 16 and September 11, 1995. Multispectral images of the reflection function and brightness temperature in 10 distinct bands of the MODIS airborne simulator (MAS) were used to derive a confidence in clear sky (or alternatively the probability of cloud), shadow, fire, and heavy aerosol. In addition to multispectral imagery, monostatic lidar data were obtained along the nadir ground track of the aircraft and used to assess the accuracy of the cloud mask results. This analysis shows that the cloud and aerosol mask being developed for operational use on the moderate-resolution imaging spectroradiometer (MODIS), and tested using MAS data in Brazil, is quite capable of separating cloud, aerosol, shadow, and fires during daytime conditions over land.

Gastric Activity and Gut Peptides in Patients With Functional Dyspepsia: Postprandial Distress Syndrome Versus Epigastric Pain Syndrome.

PubMed

Russo, Francesco; Chimienti, Guglielmina; Clemente, Caterina; Riezzo, Giuseppe; D'Attoma, Benedetta; Martulli, Manuela

2017-02-01

The goals of the study were to investigate in both postprandial distress syndrome (PDS) and epigastric pain syndrome (EPS) the gastric electrical activity and the gastric emptying (GE) time together with the circulating concentrations of motilin, somatostatin, corticotrophin-releasing factor, and neurotensin, and to establish whether the genetic variability in the neurotensin system genes differs between these 2 categories of functional dyspepsia (FD). The current FD classification is based on symptoms and it has been proven not to be completely satisfying because of a high degree of symptom overlap between subgroups. Gastric electrical activity was evaluated by cutaneous electrogastrography: the GE time by C-octanoic acid breast test. Circulating concentrations of gut peptides were measured by a radioimmunoassay. NTS 479 A/G and NTSR1 rs6090453 SNPs were evaluated by PCR and endonuclease digestion. Fifty-four FD patients (50 female/4 male) were studied. Using a symptom questionnaire, 42 patients were classified as PDS and 12 as EPS, although an overlap between the symptom profiles of the 2 subgroups was recorded. The electrogastrographic parameters (the postprandial instability coefficient of dominant frequency, the dominant power, and the power ratio) were significantly different between the subgroups, whereas the GE time did not differ significantly. In addition, EPS was characterized by a different gut peptide profile compared with PDS. Finally, neurotensin polymorphism was shown to be associated with neurotensin levels. This evidence deserves further studies in consideration of an analgesic role of neurotensin. Analysis of gut peptide profiles could represent an interesting tool to enhance FD diagnosis and overcome limitations due to a distinction based solely on symptoms.

Modulation of autonomic activity in neurological conditions: Epilepsy and Tourette Syndrome

PubMed Central

Nagai, Yoko

2015-01-01

This manuscript considers the central but neglected role of the autonomic nervous system in the expression and control of seizures in epilepsy (small) and tics in Tourette Syndrome (TS). In epilepsy, consideration of autonomic involvement is typically confined to differential diagnoses (e.g., syncope), or in relation to Sudden Unexpected Death in Epilepsy (SUDEP). Investigation is more limited in Tourette Syndrome. The role of the autonomic nervous system in the generation and prevention of epileptic seizures is largely overlooked. Emotional stimuli such as anxiety and stress are potent causes of seizures and tic activity in epilepsy and TS, respectively. This manuscript will describe a possible neural mechanism by which afferent autonomic projections linked to cognition and behavior influence central thalamo-cortical regulation, which appears to be an important means for controlling both seizure and tic activity. It also summarizes the link between the integrity of the default mode network and autonomic regulation in patients with epilepsy as well as the link between impaired motor control and autonomic regulation in patients with TS. Two neurological conditions; epilepsy and TS were chosen, as seizures and tics represent parameters that can be easily measured to investigate influences of autonomic functions. The EDA biofeedback approach is anticipated to gain a strong position within the next generation of treatment for epilepsy, as a non-invasive technique with minimal side effects. This approach also takes advantage of the current practical opportunity to utilize growing digital health technology. PMID:26441491

Modulation of autonomic activity in neurological conditions: Epilepsy and Tourette Syndrome.

PubMed

Nagai, Yoko

2015-01-01

This manuscript considers the central but neglected role of the autonomic nervous system in the expression and control of seizures in epilepsy (small) and tics in Tourette Syndrome (TS). In epilepsy, consideration of autonomic involvement is typically confined to differential diagnoses (e.g., syncope), or in relation to Sudden Unexpected Death in Epilepsy (SUDEP). Investigation is more limited in Tourette Syndrome. The role of the autonomic nervous system in the generation and prevention of epileptic seizures is largely overlooked. Emotional stimuli such as anxiety and stress are potent causes of seizures and tic activity in epilepsy and TS, respectively. This manuscript will describe a possible neural mechanism by which afferent autonomic projections linked to cognition and behavior influence central thalamo-cortical regulation, which appears to be an important means for controlling both seizure and tic activity. It also summarizes the link between the integrity of the default mode network and autonomic regulation in patients with epilepsy as well as the link between impaired motor control and autonomic regulation in patients with TS. Two neurological conditions; epilepsy and TS were chosen, as seizures and tics represent parameters that can be easily measured to investigate influences of autonomic functions. The EDA biofeedback approach is anticipated to gain a strong position within the next generation of treatment for epilepsy, as a non-invasive technique with minimal side effects. This approach also takes advantage of the current practical opportunity to utilize growing digital health technology.

MRP8 and MRP14, phagocyte-specific danger signals, are sensitive biomarkers of disease activity in cryopyrin-associated periodic syndromes.

PubMed

Wittkowski, Helmut; Kuemmerle-Deschner, Jasmin B; Austermann, Judith; Holzinger, Dirk; Goldbach-Mansky, Raphaela; Gramlich, Katharina; Lohse, Peter; Jung, Thomas; Roth, Johannes; Benseler, Susanne M; Foell, Dirk

2011-12-01

To assess the sensitivity of the phagocyte-specific molecules myeloid-related protein (MRP) 8 and MRP14 (calprotectin) for monitoring disease activity during anti-interleukin (IL)-1 therapies in patients with cryopyrin-associated periodic syndromes (CAPS), including familial cold autoinflammatory syndrome (FCAS), Muckle-Wells syndrome (MWS) and chronic infantile neurological, cutaneous and articular (CINCA) syndrome. A total of 39 patients with CAPS, including 5 FCAS, 16 MWS and 18 CINCA syndrome, received anti-IL-1 therapy. All patients with CINCA and 12 with MWS were treated with IL-1Ra (anakinra), 14 patients with MWS with a monoclonal anti-IL-1β antibody (canakinumab) and patients with FCAS received IL-1 Trap (rilonacept). During serial clinical visits serum amyloid A, C-reactive protein, erythrocyte sedimentation rate and MRP8/14 serum levels were analysed. Untreated patients with CAPS had significantly elevated MRP8/14 values. In response to treatment there was a significant reduction of MRP8/14 levels in CINCA (2,830 (range 690 - 8,480) ng/ml to 670 ng/ml, p < 0.001) and MWS patients (anakinra-treated: 4,390 (1790 - 9780) ng/ml to 1,315 ng/ml (p = 0.003); canakinumab-treated: 3,000 (500 - 13060) ng/ml to 630 ng/ml (p=0.001)). However, in many patients with CAPS, MRP8/14 levels were still elevated compared with healthy individuals, reflecting residual disease activity. However, canakinumab-treated patients with CAPS showed normalised MRP8/14 levels, suggesting control of phagocyte activation. Monitoring of cellular systems involved in inflammatory cascades of the innate immunity was successfully applied to the IL-1-driven CAPS diseases. This is the first study illustrating different states of subclinical disease activity in all types of CAPS depending on the type of anti-IL-1 therapy. MRP8/14 is a sensitive biomarker for monitoring disease activity, status of inflammation and response to IL-1 blockade in patients with CAPS.

MRP8 and MRP14, phagocyte-specific danger signals, are sensitive biomarkers of disease activity in cryopyrin-associated periodic syndromes

PubMed Central

Austermann, Judith; Holzinger, Dirk; Goldbach-Mansky, Raphaela; Gramlich, Katharina; Lohse, Peter; Jung, Thomas; Roth, Johannes; Benseler, Susanne M; Foell, Dirk

2014-01-01

Objectives To assess the sensitivity of the phagocyte-specific molecules myeloid-related protein (MRP) 8 and MRP14 (calprotectin) for monitoring disease activity during anti-interleukin (IL)-1 therapies in patients with cryopyrin-associated periodic syndromes (CAPS), including familial cold autoinflammatory syndrome (FCAS), Muckle–Wells syndrome (MWS) and chronic infantile neurological, cutaneous and articular (CINCA) syndrome. Methods A total of 39 patients with CAPS, including 5 FCAS, 16 MWS and 18 CINCA syndrome, received anti-IL-1 therapy. All patients with CINCA and 12 with MWS were treated with IL-1Ra (anakinra), 14 patients with MWS with a monoclonal anti-IL-1β antibody (canakinumab) and patients with FCAS received IL-1 Trap (rilonacept). During serial clinical visits serum amyloid A, C-reactive protein, erythrocyte sedimentation rate and MRP8/14 serum levels were analysed. Results Untreated patients with CAPS had significantly elevated MRP8/14 values. In response to treatment there was a significant reduction of MRP8/14 levels in CINCA (2,830 (range 690 – 8,480) ng/ml to 670 ng/ml, p < 0.001) and MWS patients (anakinra-treated: 4,390 (1790 – 9780) ng/ml to 1,315 ng/ml (p = 0.003); canakinumab-treated: 3,000 (500 – 13060) ng/ml to 630 ng/ml (p=0.001)). However, in many patients with CAPS, MRP8/14 levels were still elevated compared with healthy individuals, reflecting residual disease activity. However, canakinumab-treated patients with CAPS showed normalised MRP8/14 levels, suggesting control of phagocyte activation. Conclusions Monitoring of cellular systems involved in inflammatory cascades of the innate immunity was successfully applied to the IL-1-driven CAPS diseases. This is the first study illustrating different states of subclinical disease activity in all types of CAPS depending on the type of anti-IL-1 therapy. MRP8/14 is a sensitive biomarker for monitoring disease activity, status of inflammation and response to IL-1 blockade in

Comparison of the Results of Studies of Air Pollution Fungi Using the SAS Super 100, MAS 100, and Air IDEAL.

PubMed

Łukaszuk, Cecylia; Krajewska-Kułak, Elżbieta; Guzowski, Andrzej; Kułak, Wojciech; Kraszyńska, Bogumiła

2017-07-20

Although several air sampling devices for identifying and enumerating airborne microorganisms are commercially available, each poses some limitations. The aim of this study was to evaluate air pollution fungi using three such samplers: SAS Super 100, Microbiological Air Sampler (MAS) 100, and Air IDEAL. Mycological air was taken from the cellars of a 17th-century church in Siemiatycze, Poland, and the nearby outdoor environment. With samplers placed 1.5 m above the floor, microbial flora in air samples collected inside and outside the cellar were detected. The number of colony-forming units (CFU) of fungi obtained with the three samplers from the cellars and outdoor environment differed; the most CFU were obtained with the Air IDEAL and the least with the SAS Super 100. Significant differences emerged in CFUs collected from air samples with the MAS 100 and SAS Super 100, on the one hand, and the SAS Super 100 and Air IDEAL, on the other. Otherwise, results among the samplers were different. More Cladosporium species were collected with the MAS 100 sampler, whereas more Fusarium and Aspergillus species were collected with the Air IDEAL sampler. Significant differences among CFU/m³ values among the tested sites depended on the sampler used.

Perinatally administered losartan augments renal ACE2 expression but not cardiac or renal Mas receptor in spontaneously hypertensive rats.

PubMed

Klimas, Jan; Olvedy, Michael; Ochodnicka-Mackovicova, Katarina; Kruzliak, Peter; Cacanyiova, Sona; Kristek, Frantisek; Krenek, Peter; Ochodnicky, Peter

2015-08-01

Since the identification of the alternative angiotensin converting enzyme (ACE)2/Ang-(1-7)/Mas receptor axis, renin-angiotensin system (RAS) is a new complex target for a pharmacological intervention. We investigated the expression of RAS components in the heart and kidney during the development of hypertension and its perinatal treatment with losartan in young spontaneously hypertensive rats (SHR). Expressions of RAS genes were studied by the RT-PCR in the left ventricle and kidney of rats: normotensive Wistar, untreated SHR, SHR treated with losartan since perinatal period until week 9 of age (20 mg/kg/day) and SHR treated with losartan only until week 4 of age and discontinued until week 9. In the hypertrophied left ventricle of SHR, cardiac expressions of Ace and Mas were decreased while those of AT1 receptor (Agtr1a) and Ace2 were unchanged. Continuous losartan administration reduced LV weight (0.43 ± 0.02; P < 0.05 versus SHR) but did not influence altered cardiac RAS expression. Increased blood pressure in SHR (149 ± 2 in SHR versus 109 ± 2 mmHg in Wistar; P < 0.05) was associated with a lower renal expressions of renin, Agtr1a and Mas and with an increase in ACE2. Continuous losartan administration lowered blood pressure to control levels (105 ± 3 mmHg; P < 0.05 versus SHR), however, only renal renin and ACE2 were significantly up-regulated (for both P < 0.05 versus SHR). Conclusively, prevention of hypertension and LV hypertrophy development by losartan was unrelated to cardiac or renal expression of Mas. Increased renal Ace2, and its further increase by losartan suggests the influence of locally generated Ang-(1-7) in organ response to the developing hypertension in SHRs. © 2015 The Authors. Journal of Cellular and Molecular Medicine published by John Wiley & Sons Ltd and Foundation for Cellular and Molecular Medicine.

Cornus mas L. (cornelian cherry), an important European and Asian traditional food and medicine: Ethnomedicine, phytochemistry and pharmacology for its commercial utilization in drug industry.

PubMed

Dinda, Biswanath; Kyriakopoulos, Anthony M; Dinda, Subhajit; Zoumpourlis, Vassilis; Thomaidis, Nikolaos S; Velegraki, Aristea; Markopoulos, Charlambos; Dinda, Manikarna

2016-12-04

Cornus mas L. (cornelian cherry) fruits have been used for centuries as traditional cuisine and folk medicine in various countries of Europe and Asia. In folk medicines, the fruits and other parts of the plant have been used for prevention and treatment of a wide range of diseases such as diabetes, diarrhea, gastrointestinal disorders, fevers, rheumatic pain, skin and urinary tract infections, kidney and liver diseases, sunstroke, among others. This review provides a systematic and constructive overview of ethnomedicinal uses, chemical constituents and pharmacological activities of this plant as well as future research need for its commercial utilization as nutraceutical food supplement and medicine. This review is based on available literature on ethnomedicinal uses, phytochemical, pharmacological, toxicity and clinical studies on Cornus mas L. (cornelian cherry) fruits and other organs that was collected from electronic (SciFinder, PubMed, Science Direct and ACS among others) and library searches of books and journals. Versatile ethnomedicinal uses of the plant in different European and Asian countries have been reported. Phytochemical investigations on different parts of this plant have resulted in the identification of 101 compounds, among which anthocyanins, flavonoids and iridoids are the predominant groups. The crude extracts of fruits and other parts of the plant and their pure isolates exhibit a broad spectrum of pharmacological activities such as anti-microbial, anti-diabetic, anti-atherosclerotic, cyto-, hepato-, neuro- and renalprotective, antiplatelet and antiglaucomic activities. Anthocyanins, flavonoids, iridoids and vitamin C are the major bioactive constituents of the fruits. Fruits are non-toxic and safe food on acute toxicity studies in rat and human models. Clinical trials in diabetic type2 and hyperlipidemic patients showed significant trends of amelioration in sugar level, insulin secretion in diabetic patients and amelioration of lipid

Physical activity but not sedentary activity is reduced in primary Sjögren's syndrome.

PubMed

Ng, Wan-Fai; Miller, Ariana; Bowman, Simon J; Price, Elizabeth J; Kitas, George D; Pease, Colin; Emery, Paul; Lanyon, Peter; Hunter, John; Gupta, Monica; Giles, Ian; Isenberg, David; McLaren, John; Regan, Marian; Cooper, Annie; Young-Min, Steven A; McHugh, Neil; Vadivelu, Saravanan; Moots, Robert J; Coady, David; MacKay, Kirsten; Dasgupta, Bhaskar; Sutcliffe, Nurhan; Bombardieri, Michele; Pitzalis, Costantino; Griffiths, Bridget; Mitchell, Sheryl; Miyamoto, Samira Tatiyama; Trenell, Michael

2017-04-01

The aim of the study was to evaluate the levels of physical activity in individuals with primary Sjögren's syndrome (PSS) and its relationship to the clinical features of PSS. To this cross-sectional study, self-reported levels of physical activity from 273 PSS patients were measured using the International Physical Activity Questionnaire-short form (IPAQ-SF) and were compared with healthy controls matched for age, sex and body mass index. Fatigue and other clinical aspects of PSS including disease status, dryness, daytime sleepiness, dysautonomia, anxiety and depression were assessed using validated tools. Individuals with PSS had significantly reduced levels of physical activity [median (interquartile range, IQR) 1572 (594-3158) versus 3708 (1732-8255) metabolic equivalent of task (MET) × min/week, p < 0.001], but similar levels of sedentary activity [median (IQR) min 300 (135-375) versus 343 (223-433) (MET) × min/week, p = 0.532] compared to healthy individuals. Differences in physical activity between PSS and controls increased at moderate [median (IQR) 0 (0-480) versus 1560 (570-3900) MET × min/week, p < 0.001] and vigorous intensities [median (IQR) 0 (0-480) versus 480 (0-1920) MET × min/week, p < 0.001]. Correlation analysis revealed a significant association between physical activity and fatigue, orthostatic intolerance, depressive symptoms and quality of life. Sedentary activity did not correlate with fatigue. Stepwise linear regression analysis identified symptoms of depression and daytime sleepiness as independent predictors of levels of physical activity. Physical activity is reduced in people with PSS and is associated with symptoms of depression and daytime sleepiness. Sedentary activity is not increased in PSS. Clinical care teams should explore the clinical utility of targeting low levels of physical activity in PSS.

Metabolic Syndrome and Sexual Function in Postmenopausal Women.

PubMed

Trompeter, Susan E; Bettencourt, Ricki; Barrett-Connor, Elizabeth

2016-12-01

Limited literature suggests that sexual dysfunction in women covaries with the metabolic syndrome. This study examined the association of sexual function with metabolic syndrome and cardiovascular disease in healthy older women. There were 376 postmenopausal, community-dwelling women from the Rancho Bernardo Study (mean baseline age = 73 years) that completed a clinic visit during 1999-2002 and returned the Female Sexual Function Index (FSFI) questionnaire mailed in 2002. Thirty-nine percent reported being sexually active; 41.5% met a diagnosis of metabolic syndrome. The number of metabolic syndrome components was strongly associated with decreased sexual activity, desire, and low sexual satisfaction. Waist girth, diabetes, and hypertension were associated with decreased sexual activity. Elevated triglycerides were associated with low desire. Among the cardiovascular endpoints, heart attack, coronary artery bypass, and angina were associated with decreased sexual activity, but not with sexual desire or satisfaction. Past diagnosis of heart failure, poor circulation, and stroke were not associated with sexual function. Sexually active women with metabolic syndrome met criteria for sexual dysfunction in desire, arousal, orgasm, and satisfaction domains. The FSFI Total Score did not differ significantly between sexually active and inactive women. Metabolic syndrome was associated with decreased sexual activity, desire, and satisfaction in all women and with sexual dysfunction in most domains in sexually active women. Coronary artery disease was more prevalent in women with low sexual activity. Copyright © 2016 Elsevier Inc. All rights reserved.

Alimentary habits, physical activity, and Framingham global risk score in metabolic syndrome.

PubMed

Soares, Thays Soliman; Piovesan, Carla Haas; Gustavo, Andréia da Silva; Macagnan, Fabrício Edler; Bodanese, Luiz Carlos; Feoli, Ana Maria Pandolfo

2014-04-01

Metabolic syndrome is a complex disorder represented by a set of cardiovascular risk factors. A healthy lifestyle is strongly related to improve Quality of Life and interfere positively in the control of risk factors presented in this condition. To evaluate the effect of a program of lifestyle modification on the Framingham General Cardiovascular Risk Profile in subjects diagnosed with metabolic syndrome. A sub-analysis study of a randomized clinical trial controlled blind that lasted three months. Participants were randomized into four groups: dietary intervention + placebo (DIP), dietary intervention + supplementation of omega 3 (fish oil 3 g/day) (DIS3), dietary intervention + placebo + physical activity (DIPE) and dietary intervention + physical activity + supplementation of omega 3 (DIS3PE). The general cardiovascular risk profile of each individual was calculated before and after the intervention. The study included 70 subjects. Evaluating the score between the pre and post intervention yielded a significant value (p < 0.001). We obtained a reduction for intermediate risk in 25.7% of subjects. After intervention, there was a significant reduction (p < 0.01) on cardiovascular age, this being more significant in groups DIP (5.2%) and DIPE (5.3%). Proposed interventions produced beneficial effects for reducing cardiovascular risk score. This study emphasizes the importance of lifestyle modification in the prevention and treatment of cardiovascular diseases.

RAAS inhibition and cardiorenal syndrome.

PubMed

Onuigbo, Macaulay Amechi C

2014-01-01

The consensus conference on cardio-renal syndromes (2008) defined 'cardio-renal syndromes' as 'disorders of the heart and kidneys whereby acute or chronic dysfunction in one organ may induce acute or chronic dysfunction of the other' and identified five subtypes of the syndromes. Various pathophysiologic mechanisms underlie cardiorenal syndrome including hemodynamic derangements, reduced cardiac output leading to impaired renal perfusion, reduced stroke volume, raised atrial filling pressures, elevated atrial pressures, sodium and water retention, venous congestion, right ventricular dysfunction and venous hypertension causing increased renal venous pressure, intra-abdominal hypertension, various neurohormonal adaptations including activation of the renin-angiotensin-aldosterone system, adaptive activation of the sympathetic nervous system, cytokine release and oxidative stress. Although there are standardized clinical guidelines for the management of heart failure, and chronic kidney disease, respectively, there are no similar consensus clinical guidelines for the management of the cardiorenal syndromes. RAAS inhibition is advocated in treating systolic heart failure. There is evidence that RAAS inhibition is also useful in cardiorenal syndrome. However, RAAS inhibition, while potentially useful in the management of cardiorenal syndrome, is not the 'magic bullet', is sometimes limited by adverse renal events, is not applicable to all patients, and must be applied by physicians with due diligence and caution. Nevertheless, a more comprehensive multidisciplinary multipronged approach to managing patients with cardiorenal syndrome is even more pragmatic and commonsense given the multiple mechanisms and pathogenetic pathways implicated in the causation and perpetuation of cardiorenal syndrome.

BaHaMAS A Bash Handler to Monitor and Administrate Simulations

NASA Astrophysics Data System (ADS)

Sciarra, Alessandro

2018-03-01

Numerical QCD is often extremely resource demanding and it is not rare to run hundreds of simulations at the same time. Each of these can last for days or even months and it typically requires a job-script file as well as an input file with the physical parameters for the application to be run. Moreover, some monitoring operations (i.e. copying, moving, deleting or modifying files, resume crashed jobs, etc.) are often required to guarantee that the final statistics is correctly accumulated. Proceeding manually in handling simulations is probably the most error-prone way and it is deadly uncomfortable and inefficient! BaHaMAS was developed and successfully used in the last years as a tool to automatically monitor and administrate simulations.

The Effect of Antitumor Glycosides on Glioma Cells and Tissues as Studied by Proton HR-MAS NMR Spectroscopy

PubMed Central

García-Álvarez, Isabel; Garrido, Leoncio; Romero-Ramírez, Lorenzo; Nieto-Sampedro, Manuel; Fernández-Mayoralas, Alfonso; Campos-Olivas, Ramón

2013-01-01

The effect of the treatment with glycolipid derivatives on the metabolic profile of intact glioma cells and tumor tissues, investigated using proton high resolution magic angle spinning (1H HR-MAS) nuclear magnetic resonance (NMR) spectroscopy, is reported here. Two compounds were used, a glycoside and its thioglycoside analogue, both showing anti-proliferative activity on glioma C6 cell cultures; however, only the thioglycoside exhibited antitumor activity in vivo. At the drug concentrations showing anti-proliferative activity in cell culture (20 and 40 µM), significant increases in choline containing metabolites were observed in the 1H NMR spectra of the same intact cells. In vivo experiments in nude mice bearing tumors derived from implanted C6 glioma cells, showed that reduction of tumor volume was associated with significant changes in the metabolic profile of the same intact tumor tissues; and were similar to those observed in cell culture. Specifically, the activity of the compounds is mainly associated with an increase in choline and phosphocholine, in both the cell cultures and tumoral tissues. Taurine, a metabolite that has been considered a biomarker of apoptosis, correlated with the reduction of tumor volume. Thus, the results indicate that the mode of action of the glycoside involves, at least in part, alteration of phospholipid metabolism, resulting in cell death. PMID:24194925

Severe Acute Respiratory Syndrome Coronavirus Envelope Protein Ion Channel Activity Promotes Virus Fitness and Pathogenesis

PubMed Central

Nieto-Torres, Jose L.; DeDiego, Marta L.; Verdiá-Báguena, Carmina; Jimenez-Guardeño, Jose M.; Regla-Nava, Jose A.; Fernandez-Delgado, Raul; Castaño-Rodriguez, Carlos; Alcaraz, Antonio; Torres, Jaume; Aguilella, Vicente M.; Enjuanes, Luis

2014-01-01

Deletion of Severe Acute Respiratory Syndrome Coronavirus (SARS-CoV) envelope (E) gene attenuates the virus. E gene encodes a small multifunctional protein that possesses ion channel (IC) activity, an important function in virus-host interaction. To test the contribution of E protein IC activity in virus pathogenesis, two recombinant mouse-adapted SARS-CoVs, each containing one single amino acid mutation that suppressed ion conductivity, were engineered. After serial infections, mutant viruses, in general, incorporated compensatory mutations within E gene that rendered active ion channels. Furthermore, IC activity conferred better fitness in competition assays, suggesting that ion conductivity represents an advantage for the virus. Interestingly, mice infected with viruses displaying E protein IC activity, either with the wild-type E protein sequence or with the revertants that restored ion transport, rapidly lost weight and died. In contrast, mice infected with mutants lacking IC activity, which did not incorporate mutations within E gene during the experiment, recovered from disease and most survived. Knocking down E protein IC activity did not significantly affect virus growth in infected mice but decreased edema accumulation, the major determinant of acute respiratory distress syndrome (ARDS) leading to death. Reduced edema correlated with lung epithelia integrity and proper localization of Na+/K+ ATPase, which participates in edema resolution. Levels of inflammasome-activated IL-1β were reduced in the lung airways of the animals infected with viruses lacking E protein IC activity, indicating that E protein IC function is required for inflammasome activation. Reduction of IL-1β was accompanied by diminished amounts of TNF and IL-6 in the absence of E protein ion conductivity. All these key cytokines promote the progression of lung damage and ARDS pathology. In conclusion, E protein IC activity represents a new determinant for SARS-CoV virulence. PMID:24788150

The role of endothelial activation in dengue hemorrhagic fever and hantavirus pulmonary syndrome.

PubMed

Spiropoulou, Christina F; Srikiatkhachorn, Anon

2013-08-15

The loss of the endothelium barrier and vascular leakage play a central role in the pathogenesis of hemorrhagic fever viruses. This can be caused either directly by the viral infection and damage of the vascular endothelium, or indirectly by a dysregulated immune response resulting in an excessive activation of the endothelium. This article briefly reviews our knowledge of the importance of the disruption of the vascular endothelial barrier in two severe disease syndromes, dengue hemorrhagic fever and hantavirus pulmonary syndrome. Both viruses cause changes in vascular permeability without damaging the endothelium. Here we focus on our understanding of the virus interaction with the endothelium, the role of the endothelium in the induced pathogenesis, and the possible mechanisms by which each virus causes vascular leakage. Understanding the dynamics between viral infection and the dysregulation of the endothelial cell barrier will help us to define potential therapeutic targets for reducing disease severity.

Impaired factor XIIa-dependent activation of fibrinolysis in treated antiphospholipid syndrome gestations developing late-pregnancy complications.

PubMed

Carmona, Francisco; Lázaro, Isabel; Reverter, Juan C; Tàssies, Dolors; Font, Josep; Cervera, Ricard; Balasch, Juan

2006-02-01

The objective of the study was to investigate the potential role of impaired factor XII-dependent activation of fibrinolysis in treated antiphospholipid syndrome gestations developing late-pregnancy complications. This was a prospective study in a third-level teaching hospital, including 75 patients: 25 pregnant patients having the antiphospholipid syndrome and carrying their pregnancies until 26 weeks' gestation or later (group 1); 25 pregnant patients having normal term pregnancies and delivery and no previous miscarriage (group 2); and 25 pregnant patients being diagnosed as having severe pre-eclampsia and/or intrauterine growth restriction but testing negative for antiphospholipid antibodies (group 3). Hemostatic evaluation was carried out from patients in groups 1 and 2 between 6 and 10 weeks, between 18 and 22 weeks, and between 28 and 32 weeks' gestation. Patients in group 3 were sampled between 28 and 32 weeks. An additional blood sample was obtained 4 to 6 months after delivery (baseline). The Mann-Whitney U test, the Friedman test, and the chi2 test were used. Patients in group 1 were characterized by increased factor VIIa levels, increased prothrombin fragment 1+2 levels, reduced factor XIIa levels, diminished functional urokinase-type plasminogen activator levels, and decreased levels of plasmin/alpha-2-plasmin inhibitor complexes. These abnormalities were more evident in patients in group 1 developing pre-eclampsia and/or intrauterine growth restriction. Impaired factor XIIa-dependent activation of fibrinolysis seems to be a key mechanism related to late-pregnancy complications in patients with the antiphospholipid syndrome.

Comparison of erector spinae and hamstring muscle activities and lumbar motion during standing knee flexion in subjects with and without lumbar extension rotation syndrome.

PubMed

Kim, Si-hyun; Kwon, Oh-yun; Park, Kyue-nam; Kim, Moon-Hwan

2013-12-01

The aim of this study was to compare the activity of the erector spinae (ES) and hamstring muscles and the amount and onset of lumbar motion during standing knee flexion between individuals with and without lumbar extension rotation syndrome. Sixteen subjects with lumbar extension rotation syndrome (10 males, 6 females) and 14 healthy subjects (8 males, 6 females) participated in this study. During the standing knee flexion, surface electromyography (EMG) was used to measure muscle activity, and surface EMG electrodes were attached to both the ES and hamstring (medial and lateral) muscles. A three-dimensional motion analysis system was used to measure kinematic data of the lumbar spine. An independent-t test was conducted for the statistical analysis. The group suffering from lumbar extension rotation syndrome exhibited asymmetric muscle activation of the ES and decreased hamstring activity. Additionally, the group with lumbar extension rotation syndrome showed greater and earlier lumbar extension and rotation during standing knee flexion compared to the control group. These data suggest that asymmetric ES muscle activation and a greater amount of and earlier lumbar motion in the sagittal and transverse plane during standing knee flexion may be an important factor contributing to low back pain. Copyright © 2013 Elsevier Ltd. All rights reserved.

Characterization of Mast Cell Activation Syndrome

PubMed Central

Afrin, Lawrence B.; Self, Sally; Menk, Jeremiah; Lazarchick, John

2016-01-01

Background Mast cell activation syndrome (MCAS), a recently recognized non-neoplastic mast cell (MC) disease driving chronic multisystem inflammation ± allergy, appears prevalent and thus important. We report the first systematic characterization of a large MCAS population. Method Demographics, comorbidities, symptoms, family histories, and physical exam and laboratory findings were reviewed in 298 retrospective and 115 prospective MCAS patients. Blood samples from prospective subjects were examined by flow cytometry for clonal MC disease and tested for cytokines potentially driving the monocytosis frequent in MCAS. Results Demographically, white females dominated. Median ages at symptom onset/diagnosis were 9/49 years (ranges 0–88/16–92); median time from symptom onset to diagnosis was 30 years (range 1–85). Median numbers of comorbidities/symptoms/family medical issues were 11/20/4 (ranges 1–66/2–84/0–33). Gastroesophageal reflux, fatigue, and dermatographism were the most common comorbidity, symptom, and exam finding. Abnormalities in routine labs were common and diverse but typically modest. The most useful diagnostic markers were heparin, prostaglandin D2, histamine, and chromogranin A. Flow cytometric and cytokine assessments were unhelpful. Conclusions Our study highlights MCAS’s morbidity burden and challenging heterogeneity. Recognition is important given good survival and treatment prospects. PMID:28262205

Azilsartan ameliorates diabetic cardiomyopathy in young db/db mice through the modulation of ACE-2/ANG 1-7/Mas receptor cascade.

PubMed

Sukumaran, Vijayakumar; Tsuchimochi, Hirotsugu; Tatsumi, Eisuke; Shirai, Mikiyasu; Pearson, James T

2017-11-15

Hyperglycemia up-regulates intracellular angiotensin II (ANG-II) production in cardiac myocytes. This study investigated the hemodynamic and metabolic effects of azilsartan (AZL) treatment in a mouse model of diabetic cardiomyopathy and whether the cardioprotective effects of AZL are mediated by the angiotensin converting enzyme (ACE)-2/ANG 1-7/Mas receptor (R) cascade. Control db/+ and db/db mice (n=5 per group) were treated with vehicle or AZL (1 or 3mg/kg/d oral gavage) from the age of 8 to 16weeks. Echocardiography was then performed and myocardial protein levels of ACE-2, Mas R, AT 1 R, AT 2 R, osteopontin, connective tissue growth factor (CTGF), atrial natriuretic peptide (ANP) and nitrotyrosine were measured by Western blotting. Oxidative DNA damage and inflammatory markers were assessed by immunofluorescence of 8-hydroxy-2'-deoxyguanosine (8-OHdG), tumor necrosis factor (TNF)-α and interleukin 6 (IL-6). Compared with db/+ mice, the vehicle-treated db/db mice developed obesity, hyperglycemia, hyperinsulinemia and diastolic dysfunction along with cardiac hypertrophy and fibrosis. AZL treatment lowered blood pressure, fasting blood glucose and reduced peak plasma glucose during an oral glucose tolerance test. AZL-3 treatment resulted in a significant decrease in the expression of cytokines, oxidative DNA damage and cardiac dysfunction. Moreover, AZL-3 treatment significantly abrogated the downregulation of ACE-2 and Mas R protein levels in db/db mice. Furthermore, AZL treatment significantly reduced cardiac fibrosis, hypertrophy and their marker molecules (osteopontin, CTGF, TGF-β1 and ANP). Short-term treatment with AZL-3 reversed abnormal cardiac structural remodeling and partially improved glucose metabolism in db/db mice by modulating the ACE-2/ANG 1-7/Mas R pathway. Copyright © 2017 Elsevier Inc. All rights reserved.

Differences in Knee and Hip Adduction and Hip Muscle Activation in Runners With and Without Iliotibial Band Syndrome.

PubMed

Baker, Robert L; Souza, Richard B; Rauh, Mitchell J; Fredericson, Michael; Rosenthal, Michael D

2018-04-26

Iliotibial band syndrome has been associated with altered hip and knee kinematics in runners. Previous studies have recommended further research on neuromuscular factors at the hip. The frontal plane hip muscles have been a strong focus in strength comparison but not for electromyography investigation. To compare hip surface electromyography, and frontal plane hip and knee kinematics, in runners with and without iliotibial band syndrome. Observational cross-sectional study. Thirty participants were tested for motion capture at the hip and knee and muscle activation in the lateral and posterior hip. Biomechanics research laboratory within a university. Thirty subjects were recruited consisting of 15 injured runners with iliotibial band syndrome and 15 gender-, age-, and body mass index-matched controls. In each group, 8 were male runners and 7 were female runners. Inclusion criteria for the injured group were pain within 2 months related to iliotibial band syndrome and a positive Noble compression test. Participants were excluded if they reported other lower extremity diagnoses within the last year or active lower extremity or low back pain not related to iliotibial band syndrome. Controls were excluded if they reported a history of iliotibial band syndrome. Convenience sampling was used based on referrals from local running clinics and orthopedic clinics. Three-dimensional motion capture was performed with 10 high-speed cameras synchronized with wireless surface electromyography during a 30-minute run. The first data point was at 3 minutes, using a constant speed of 2.74 meters per second. A second data point was at 30 minutes, using a self-selected pace by the participant to allow for a challenging run until completion at 30 minutes. Motion capture was reported as peak kinematic values from heel strike to peak knee flexion for hip adduction and knee adduction. Surface electromyography was reported as a percentage of maximal voluntary contraction for the gluteus

Leisure activities in Prader-Wili syndrome: implications for health, cognition and adaptive functioning.

PubMed

Dykens, Elisabeth M

2014-02-01

Although hyperphagia and compulsivity in Prader-Willi syndrome (PWS) are well described, recreation and adaptive skills are relatively unexplored. Parents of 123 participants with PWS (4-48 years) completed measures of their child's adaptive, recreation, and problem behaviors. Offspring received cognitive testing. Watching TV was the most frequent recreational activity, and was associated with compulsivity and skin picking. BMIs were negatively correlated with physical play, and highest in those who watched TV and played computer games. Computer games and physical activities were associated with higher IQ and adaptive scores. People with PWS and other disabilities need to watch less TV and be more engaged in physical activities, games, and leisure pursuits that are fun, and may bring cognitive or adaptive advantages.

Optical mapping of the brain activity in children with Down's syndrome

NASA Astrophysics Data System (ADS)

Yuan, Zhen; Lu, Fengmei

2018-02-01

Down's syndrome (DS) has been shown to be associated with many neurological complications, including cognitive deficits, seizures, early-onset dementia that resembles Alzheimer's disease, and neurological complications of systemic disorders. DS patients show to have poor performance in executive functions (EF) and fine motor skills. In this study, we examined the brain hemodynamic responses and brain activation patterns of DS children during the completion of EF tasks. Revealing its neural mechanism of DS is not only able to contribute to the early intervention of this children with DS, but also increase understanding of developmental cascades in childhood.

Watery diarrhea syndrome in an adult with ganglioneuroma-pheochromocytoma: identification of vasoactive intestinal peptide, calcitonin, and catecholamines and assessment of their biologic activity.

PubMed

Trump, D L; Livingston, J N; Baylin, S B

1977-10-01

A case of adult ganglioneuroma-pheochromocytoma with an associated watery diarrhea syndrome is reported. High levels of vasoactive intestinal peptide (VIP) were found in preoperative serum and in tumor tissue. The serum VIP levels fell to normal, and the watery diarrhae syndrome completely ceased following removal of the tumor. In addition to containing VIP, the tumor was rich in catecholamines, and calcitonin. Peptide hormone-containing extracts and catecholamine extracts from the tumor both activated the adenyl cyclase system and increased lipolytic activity in a preparation of isolated rat fat cells. The findings in this patient further link VIP with neural crest tissues, and suggest the importance of determining catecholamine levels in patients with the watery diarrhea syndrome.

Cultivation of activated sludge using sea mud as seed to treat industrial phenolic wastewater with high salinity.

PubMed

Tan, Songwen; Cui, Chunzhi; Hou, Yang; Chen, Xuncai; Xu, Aiqin; Li, Weiguo; You, Hong

2017-01-30

A technique is proposed to treat saline hazardous wastewater by using marine activated sludge, cultivated with sea mud as seed. Since the developed marine activated sludge had phenol-tolerant microorganisms (MAS-1, MAS-2 and MAS-3) which originated from the ocean, it was envisaged that these bacteria could survive and breakdown phenol in saline environments. In this work, typical phenol-tolerant microorganisms were isolated from the marine activated sludge and identified. After a hierarchical acclimation process, the marine activated sludge was used to treat the industrial phenolic wastewater with high salinity. The marine activated sludge was able to break down phenol and other organic components effectively and efficiently in treating the wastewater with salinity of 5.7% w/v. The results showed a high removal of phenol (99%), COD (80%) and NH 3 -N (68%). Copyright © 2016 Elsevier Ltd. All rights reserved.

Inhibition of interleukin-2-mediated lymphocyte activation in patients with Cushing's syndrome: a comparison with hypocortisolemic patients.

PubMed

Sauer, J; Stalla, G K; Müller, O A; Arzt, E

1994-02-01

We investigated the lymphocyte interleukin-2 (IL-2) system, which is critically involved in lymphocyte activation, in patients with disorders or the hypothalamic-pituitary-adrenal (HPA) axis. Patients with Cushing's syndrome (n = 9) showed a significant (p < 0.05) inhibition of phytohemagglutinin (PHA)-stimulated IL-2 secretion by peripheral lymphocytes and a decrease of sensitivity to cortisol inhibition in vitro compared to normal subjects (n = 9). Circulating soluble interleukin-2 receptor (sIL-2R) levels were significantly decreased (p < 0.05), whereas no significant difference was observed in PHA-induced sIL-2R release in vitro. In patients with hypocortisolism (n = 12), in vitro IL-2 synthesis was increased compared to normal subjects and to patients with Cushing's syndrome (p < 0.01). In vitro sIL-2R release was significantly higher (p < 0.01) compared to patients with Cushing's syndrome. In contrast to patients with secondary adrenal insufficiency (n = 7), patients with an adrenal origin of hypocortisolism (Addison's disease, bilateral adrenalectomy; n = 5) showed significantly elevated circulating sIL-2R levels compared to normal subjects and patients with Cushing's syndrome (p < 0.005). There was no significant difference between the study groups in mitogen-induced DNA synthesis. This is the first description of alterations of cytokine secretion in patients with HPA axis disorders. The contrary effects of long-term hypercortisolism and insufficient or absent adrenal glucocorticoid secretion on IL-2-mediated lymphocyte activation could account for the immune states previously observed in these patients.

Geriatric Sexuality Breakdown Syndrome.

ERIC Educational Resources Information Center

Kaas, Merrie Jean

1981-01-01

Focuses on the relationship between social environment and the older individual. By utilizing the Social Breakdown Syndrome a cycle of events is defined by the Geriatric Sexuality Breakdown Syndrome, in which an older individual is initially predisposed to diminished sexual activity to the end point of self-identification as nonsexual. (Author)

Physical activity and nutrition behaviour outcomes of a cluster-randomized controlled trial for adults with metabolic syndrome in Vietnam.

PubMed

Tran, Van Dinh; Lee, Andy H; Jancey, Jonine; James, Anthony P; Howat, Peter; Mai, Le Thi Phuong

2017-01-13

Metabolic syndrome is prevalent among Vietnamese adults, especially those aged 50-65 years. This study evaluated the effectiveness of a 6 month community-based lifestyle intervention to increase physical activity levels and improve dietary behaviours for adults with metabolic syndrome in Vietnam. Ten communes, involving participants aged 50-65 years with metabolic syndrome, were recruited from Hanam province in northern Vietnam. The communes were randomly allocated to either the intervention (five communes, n = 214) or the control group (five communes, n = 203). Intervention group participants received a health promotion package, consisting of an information booklet, education sessions, a walking group, and a resistance band. Control group participants received one session of standard advice during the 6 month period. Data were collected at baseline and after the intervention to evaluate programme effectiveness. The International Physical Activity Questionnaire - Short Form and a modified STEPS questionnaire were used to assess physical activity and dietary behaviours, respectively, in both groups. Pedometers were worn by the intervention participants only for 7 consecutive days at baseline and post-intervention testing. To accommodate the repeated measures and the clustering of individuals within communes, multilevel mixed regression models with random effects were fitted to determine the impacts of intervention on changes in outcome variables over time and between groups. With a retention rate of 80.8%, the final sample comprised 175 intervention and 162 control participants. After controlling for demographic and other confounding factors, the intervention participants showed significant increases in moderate intensity activity (P = 0.018), walking (P < 0.001) and total physical activity (P = 0.001), as well as a decrease in mean sitting time (P < 0.001), relative to their control counterparts. Significant improvements in dietary

Paramagnet induced signal quenching in MAS-DNP experiments in frozen homogeneous solutions

NASA Astrophysics Data System (ADS)

Corzilius, Björn; Andreas, Loren B.; Smith, Albert A.; Ni, Qing Zhe; Griffin, Robert G.

2014-03-01

The effects of nuclear signal quenching induced by the presence of a paramagnetic polarizing agent are documented for conditions used in magic angle spinning (MAS)-dynamic nuclear polarization (DNP) experiments on homogeneous solutions. In particular, we present a detailed analysis of three time constants: (1) the longitudinal build-up time constant TB for 1H; (2) the rotating frame relaxation time constant T1ρ for 1H and 13C and (3) T2 of 13C, the transverse relaxation time constant in the laboratory frame. These relaxation times were measured during microwave irradiation at a magnetic field of 5 T (140 GHz) as a function of the concentration of four polarizing agents: TOTAPOL, 4-amino-TEMPO, trityl (OX063), and Gd-DOTA and are compared to those obtained for a sample lacking paramagnetic doping. We also report the EPR relaxation time constants T1S and T2S, the DNP enhancements, ε, and the parameter E, defined below, which measures the sensitivity enhancement for the four polarizing agents as a function of the electron concentration. We observe substantial intensity losses (paramagnetic quenching) with all of the polarizing agents due to broadening mechanisms and cross relaxation during MAS. In particular, the monoradical trityl and biradical TOTAPOL induce ∼40% and 50% loss of signal intensity. In contrast there is little suppression of signal intensity in static samples containing these paramagnetic species. Despite the losses due to quenching, we find that all of the polarizing agents provide substantial gains in signal intensity with DNP, and in particular that the net enhancement is optimal for biradicals that operate with the cross effect. We discuss the possibility that much of this polarization loss can be regained with the development of instrumentation and methods to perform electron decoupling.

Unmasking Diogenes Syndrome

PubMed Central

Nayak, Kashinath; Gopinath, Hima; Kini, Hema; Kumar, Pramod

2015-01-01

Diogenes syndrome is characterized by extreme self-neglect, social withdrawal, and poor personal and domestic hygiene. We report a case of Diogenes syndrome presenting with dermatitis passivata. An unusual “mask” of dirt resembling a carapace, onset of neglect after awareness of a breast lump and resumption of personal grooming and social activities after removal of the lump and counseling were seen. PMID:26120158

The role of endothelial activation in dengue hemorrhagic fever and hantavirus pulmonary syndrome

PubMed Central

Spiropoulou, Christina F; Srikiatkhachorn, Anon

2013-01-01

The loss of the endothelium barrier and vascular leakage play a central role in the pathogenesis of hemorrhagic fever viruses. This can be caused either directly by the viral infection and damage of the vascular endothelium, or indirectly by a dysregulated immune response resulting in an excessive activation of the endothelium. This article briefly reviews our knowledge of the importance of the disruption of the vascular endothelial barrier in two severe disease syndromes, dengue hemorrhagic fever and hantavirus pulmonary syndrome. Both viruses cause changes in vascular permeability without damaging the endothelium. Here we focus on our understanding of the virus interaction with the endothelium, the role of the endothelium in the induced pathogenesis, and the possible mechanisms by which each virus causes vascular leakage. Understanding the dynamics between viral infection and the dysregulation of the endothelial cell barrier will help us to define potential therapeutic targets for reducing disease severity. PMID:23841977

Metabolic and cardiovascular outcomes in patients with Cushing's syndrome of different aetiologies during active disease and 1 year after remission.

PubMed

Giordano, Roberta; Picu, Andreea; Marinazzo, Elisa; D'Angelo, Valentina; Berardelli, Rita; Karamouzis, Ioannis; Forno, Daniela; Zinnà, Domenico; Maccario, Mauro; Ghigo, Ezio; Arvat, Emanuela

2011-09-01

Cushing's syndrome is associated with several comorbidities responsible for the increased cardiovascular risk, not only during the active phase but also after disease remission. In 29 patients with Cushing's syndrome (14 Cushing's diseases and 15 adrenal adenomas), waist circumference, fasting and 2-h glucose after oral glucose tolerance test (OGTT), lipid profile and blood pressure were evaluated during the active disease and 1 year after remission and compared with those in 29 sex-, age- and BMI-matched controls. During the active disease, waist circumference, 2-h glucose after OGTT, total and LDL cholesterol were higher in patients with Cushing's syndrome than in controls (P < 0·001) but similar in Cushing's disease and adrenal adenomas. The prevalence of impaired glucose tolerance (IGT), diabetes mellitus, dyslipidaemia and hypertension was higher (P < 0·001) in patients with Cushing's syndrome (27%, 24%, 59% and 72%) than in controls (10%, 0%, 21% and 10%), with no significant difference between Cushing's disease and adrenal adenomas. One year following hormonal remission, waist circumference persisted higher than in controls (P < 0·05) in both Cushing's disease and adrenal adenomas. Metabolic and cardiovascular abnormalities were still present in both groups, although with a lower prevalence, as well as with a more marked decrease in adrenal adenomas (P < 0·05 vs active disease for IGT, dyslipidaemia and hypertension). These results show that chronic hypercortisolism, independently of its aetiology, contributes to metabolic impairment and increased cardiovascular risk, while these abnormalities mostly persist in patients with previous Cushing's disease after hormonal remission. Pituitary hormonal deficiencies, hormonal replacement treatments and/or incomplete cure from Cushing's disease may account for these findings. © 2011 Blackwell Publishing Ltd.

Meigs Syndrome Superimposed on Gorlin Syndrome in a 14-Year-Old Girl.

PubMed

Iwasaki, Keita; Matsushita, Hiroshi; Murakami, Hideki; Watanabe, Kazushi; Wakatsuki, Akihiko

2016-10-01

Meigs syndrome is a rare complication associated with ovarian fibromas. Although ovarian fibromas are rare in children, they are common in women with Gorlin syndrome after puberty. A 14-year-old girl with Gorlin syndrome was admitted to our hospital for ablation of basal cell carcinoma. A chest x-ray revealed pleural effusion. Ultrasonography revealed bilateral multinodular ovarian masses. Meigs syndrome associated with ovarian fibromas was considered. A laparotomy revealed bilateral ovarian masses, which were resected. Microscopically, the masses were composed of mitotically active fibroma and areas resembling hemangiopericytoma and luteinized thecoma. The pleural effusion disappeared soon after the surgery. Physicians should consider the possibility that pleural effusion might precede the diagnosis of ovarian fibroma in patients with Gorlin syndrome. Copyright © 2016 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

{sup 13}C chemical shift anisotropies for carbonate ions in cement minerals and the use of {sup 13}C, {sup 27}Al and {sup 29}Si MAS NMR in studies of Portland cement including limestone additions

DOE Office of Scientific and Technical Information (OSTI.GOV)

Sevelsted, Tine F.; Herfort, Duncan; Skibsted, Jørgen, E-mail: jskib@chem.au.dk

2013-10-15

{sup 13}C isotropic chemical shifts and chemical shift anisotropy parameters have been determined for a number of inorganic carbonates relevant in cement chemistry from slow-speed {sup 13}C MAS or {sup 13}C({sup 1}H) CP/MAS NMR spectra (9.4 T or 14.1 T) for {sup 13}C in natural abundance. The variation in the {sup 13}C chemical shift parameters is relatively small, raising some doubts that different carbonate species in Portland cement-based materials may not be sufficiently resolved in {sup 13}C MAS NMR spectra. However, it is shown that by combining {sup 13}C MAS and {sup 13}C({sup 1}H) CP/MAS NMR carbonate anions in anhydrousmore » and hydrated phases can be distinguished, thereby providing valuable information about the reactivity of limestone in cement blends. This is illustrated for three cement pastes prepared from an ordinary Portland cement, including 0, 16, and 25 wt.% limestone, and following the hydration for up to one year. For these blends {sup 29}Si MAS NMR reveals that the limestone filler accelerates the hydration for alite and also results in a smaller fraction of tetrahedrally coordinated Al incorporated in the C-S-H phase. The latter result is more clearly observed in {sup 27}Al MAS NMR spectra of the cement–limestone blends and suggests that dissolved aluminate species in the cement–limestone blends readily react with carbonate ions from the limestone filler, forming calcium monocarboaluminate hydrate. -- Highlights: •{sup 13}C chemical shift anisotropies for inorganic carbonates from {sup 13}C MAS NMR. •Narrow {sup 13}C NMR chemical shift range (163–171 ppm) for inorganic carbonates. •Anhydrous and hydrated carbonate species by {sup 13}C MAS and {sup 13}C({sup 1}H) CP/MAS NMR. •Limestone accelerates the hydration for alite in Portland – limestone cements. •Limestone reduces the amount of aluminium incorporated in the C-S-H phase.« less

Surgical treatment of chronic exertional compartment syndrome of the leg: failure rates and postoperative disability in an active patient population.

PubMed

Waterman, Brian R; Laughlin, Matthew; Kilcoyne, Kelly; Cameron, Kenneth L; Owens, Brett D

2013-04-03

Chronic exertional compartment syndrome of the leg is a frequent source of lower-extremity pain in military personnel, competitive athletes, and runners. We are not aware of any previous study in which the authors rigorously evaluated the rates of return to full activity, persistent disability, and surgical revision after operative management of chronic exertional compartment syndrome of the leg in a large, physically active population. Individuals who had undergone surgical fasciotomy of the anterior, lateral, and/or posterior compartments (current procedural terminology [CPT] codes 27600, 27601, and 27602) for nontraumatic compartment syndrome of the lower extremity (International Classification of Diseases, Ninth Revision [ICD-9] code 729.72) between 2003 and 2010 were identified from the Military Health System Management Analysis and Reporting Tool (M2). Demographic variables including age, sex, and rank were extracted, and rates of postoperative complications, activity limitations, and revision surgery or medical discharge were obtained from the electronic medical record and U.S. Army Physical Disability Agency database. A total of 611 patients underwent 754 surgical procedures. The average patient age was 28.0 years, and 91.8% of the patients were male. Of the surgical procedures, 77.4% involved only anterior and lateral compartment releases; 19.4% addressed the anterior, lateral, and posterior compartments; and 2.2% addressed the posterior compartments alone. Symptom recurrence was reported by 44.7% of the patients, and 27.7% were unable to return to full activity. Surgical complications were documented for 15.7% of the patients, 5.9% underwent surgical revision, and 17.3% were referred for medical discharge because of chronic exertional compartment syndrome. Univariate analysis of prognostic factors revealed that surgical failure was associated with bilateral involvement (odds ratio [OR], 1.64), perioperative complications (OR, 2.12), activity limitations

Rett syndrome and menstruation.

PubMed

Hamilton, Amy; Marshal, Michael P; Sucato, Gina S; Murray, Pamela J

2012-04-01

Describe the experience that girls with Rett syndrome have with menstruation including menstrual hygiene, dysmenorrhea, premenstrual syndrome (PMS), and attempts at treatment. Anonymous web-based survey. Convenience sample recruited from Rett syndrome LISTSERV in July of 2009. Mothers of girls with Rett syndrome between the ages of 10-25 who have had at least one menses. Prevalence, frequency, and severity of dysmenorrhea and PMS; hygiene concerns; and treatments attempts and perceived effectiveness. Dysmenorrhea and PMS are common problems among young women with Rett syndrome. Despite their frequency and severity they do not routinely limit activities. Multiple treatment attempts are common. Hormonal contraception is used mostly for menstrual cycle control with oral contraceptive pills the most commonly used method. Young women with Rett syndrome have standard symptoms of dysmenorrhea and PMS as well as autism spectrum specific PMS symptoms. Hormonal contraception is commonly used for menstrual management. Copyright © 2012 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

Angiotensin-converting enzyme 2 activation improves endothelial function.

PubMed

Fraga-Silva, Rodrigo A; Costa-Fraga, Fabiana P; Murça, Tatiane M; Moraes, Patrícia L; Martins Lima, Augusto; Lautner, Roberto Q; Castro, Carlos H; Soares, Célia Maria A; Borges, Clayton L; Nadu, Ana Paula; Oliveira, Marilene L; Shenoy, Vinayak; Katovich, Michael J; Santos, Robson A S; Raizada, Mohan K; Ferreira, Anderson J

2013-06-01

Diminished release and function of endothelium-derived nitric oxide coupled with increases in reactive oxygen species production is critical in endothelial dysfunction. Recent evidences have shown that activation of the protective axis of the renin-angiotensin system composed by angiotensin-converting enzyme 2, angiotensin-(1-7), and Mas receptor promotes many beneficial vascular effects. This has led us to postulate that activation of intrinsic angiotensin-converting enzyme 2 would improve endothelial function by decreasing the reactive oxygen species production. In the present study, we tested 1-[[2-(dimetilamino)etil]amino]-4-(hidroximetil)-7-[[(4-metilfenil)sulfonil]oxi]-9H-xantona-9 (XNT), a small molecule angiotensin-converting enzyme 2 activator, on endothelial function to validate this hypothesis. In vivo treatment with XNT (1 mg/kg per day for 4 weeks) improved the endothelial function of spontaneously hypertensive rats and of streptozotocin-induced diabetic rats when evaluated through the vasorelaxant responses to acetylcholine/sodium nitroprusside. Acute in vitro incubation with XNT caused endothelial-dependent vasorelaxation in aortic rings of rats. This vasorelaxation effect was attenuated by the Mas antagonist D-pro7-Ang-(1-7), and it was reduced in Mas knockout mice. These effects were associated with reduction in reactive oxygen species production. In addition, Ang II-induced reactive oxygen species production in human aortic endothelial cells was attenuated by preincubation with XNT. These results showed that chronic XNT administration improves the endothelial function of hypertensive and diabetic rat vessels by attenuation of the oxidative stress. Moreover, XNT elicits an endothelial-dependent vasorelaxation response, which was mediated by Mas. Thus, this study indicated that angiotensin-converting enzyme 2 activation promotes beneficial effects on the endothelial function and it is a potential target for treating cardiovascular disease.

ANGIOTENSIN-CONVERTING ENZYME 2 ACTIVATION IMPROVES ENDOTHELIAL FUNCTION

PubMed Central

Fraga-Silva, Rodrigo A.; Costa-Fraga, Fabiana P.; Murça, Tatiane M.; Moraes, Patrícia L.; Lima, Augusto Martins; Lautner, Roberto Q.; Castro, Carlos H.; Soares, Célia Maria A.; Borges, Clayton L.; Nadu, Ana Paula; Oliveira, Marilene L.; Shenoy, Vinayak; Katovich, Michael J.; Santos, Robson A.S.; Raizada, Mohan K.; Ferreira, Anderson J.

2013-01-01

Diminished release and function of endothelium-derived nitric oxide (NO) coupled with increases in reactive oxygen species (ROS) production is critical in endothelial dysfunction. Recent evidences have shown that activation of the protective axis of the renin-angiotensin system composed by angiotensin-converting enzyme2 (ACE2), Angiotensin-(1-7) [Ang-(1-7)] and Mas receptor promotes many beneficial vascular effects. This has led us to postulate that activation of intrinsic ACE2 would improve endothelial function by decreasing the ROS production. In the present study, we tested 1-[[2-(dimetilamino)etil]amino]-4-(hidroximetil)-7-[[(4-metilfenil)sulfonil]oxi]-9H-xantona-9 (XNT), a small molecule ACE2 activator, on endothelial function to validate this hypothesis. In vivo treatment with XNT (1mg/kg/day for 4 weeks) improved the endothelial function of spontaneously hypertensive rats and of streptozotocin-induced diabetic rats when evaluated through the vasorelaxant responses to acetylcholine/sodium nitroprusside. Acute in vitro incubation with XNT caused endothelial-dependent vasorelaxation in aortic rings of rats. This vasorelaxation effect was attenuated by the Mas antagonist D-pro7-Ang-(1-7) and it was reduced in Mas knockout mice. These effects were associated with reduction in ROS production. In addition, Ang II-induced ROS production in human aortic endothelial cells was attenuated by pre-incubation with XNT. These results showed that chronic XNT administration improves the endothelial function of hypertensive and diabetic rat vessels by attenuation of the oxidative stress. Moreover, XNT elicits an endothelial-dependent vasorelaxation response, which was mediated by Mas. Thus, this study indicated that ACE2 activation promotes beneficial effects on the endothelial function and it is a potential target for treating cardiovascular disease. PMID:23608648

The MasPar MP-1 As a Computer Arithmetic Laboratory

PubMed Central

Anuta, Michael A.; Lozier, Daniel W.; Turner, Peter R.

1996-01-01

This paper is a blueprint for the use of a massively parallel SIMD computer architecture for the simulation of various forms of computer arithmetic. The particular system used is a DEC/MasPar MP-1 with 4096 processors in a square array. This architecture has many advantages for such simulations due largely to the simplicity of the individual processors. Arithmetic operations can be spread across the processor array to simulate a hardware chip. Alternatively they may be performed on individual processors to allow simulation of a massively parallel implementation of the arithmetic. Compromises between these extremes permit speed-area tradeoffs to be examined. The paper includes a description of the architecture and its features. It then summarizes some of the arithmetic systems which have been, or are to be, implemented. The implementation of the level-index and symmetric level-index, LI and SLI, systems is described in some detail. An extensive bibliography is included. PMID:27805123

Distributed Cooperation Solution Method of Complex System Based on MAS

NASA Astrophysics Data System (ADS)

Weijin, Jiang; Yuhui, Xu

To adapt the model in reconfiguring fault diagnosing to dynamic environment and the needs of solving the tasks of complex system fully, the paper introduced multi-Agent and related technology to the complicated fault diagnosis, an integrated intelligent control system is studied in this paper. Based on the thought of the structure of diagnostic decision and hierarchy in modeling, based on multi-layer decomposition strategy of diagnosis task, a multi-agent synchronous diagnosis federation integrated different knowledge expression modes and inference mechanisms are presented, the functions of management agent, diagnosis agent and decision agent are analyzed, the organization and evolution of agents in the system are proposed, and the corresponding conflict resolution algorithm in given, Layered structure of abstract agent with public attributes is build. System architecture is realized based on MAS distributed layered blackboard. The real world application shows that the proposed control structure successfully solves the fault diagnose problem of the complex plant, and the special advantage in the distributed domain.

The influence of various therapeutic regimens on early clinical and laboratory response and outcome of children with secondary hemophagocytic lymphohistiocytosis

PubMed Central

Gietka, Piotr; Książyk, Janusz B.; Machaczka, Maciej

2016-01-01

Introduction Secondary hemophagocytic lymphohistiocytosis (sHLH) is a life-threatening syndrome of severe hyperinflammation which is often triggered by infection or autoimmune disease (macrophage activation syndrome – MAS). The aim of our study was to assess the frequency of sHLH/MAS in children treated in our institution and to compare the effectiveness of various therapeutic interventions. Material and methods Between 2005 and 2013, 24 children (age: 1–17 years) were consecutively treated for sHLH/MAS. Therapy was based on glucocorticoids (GCs) in high or standard doses (hd-GCs or sd-GCs), intravenous immunoglobulin (IVIG), and cyclosporin A (CyA). A comparison of selected laboratory and clinical parameters during the first 72 h of treatment and after a week from the last intervention applied in the first 72 h after diagnosis was performed retrospectively. Results The majority of patients (14/24, 58%) suffered from sHLH/MAS in the course of an autoimmune disease (12 patients diagnosed with a systemic form of juvenile idiopathic arthritis). We found with a confidence level of 95% that the application of hd-GCs in the first 24 h caused rapid alleviation of fever, reduction of hepatosplenomegaly, and an increase in thrombocytes and s-fibrinogen concentrations. The use of combination therapy with hd-GCs, IVIG, and CyA in the first 72 h caused a faster increase in s-fibrinogen. All patients survived and were alive at the follow-up of 1–8 years. Conclusions The results indicate that treatment of sHLH/MAS based on hd-GCs, CyA and IVIG is an effective therapy in children. PMID:29379544

Elucidating proline dynamics in spider dragline silk fibre using 2H-13C HETCOR MAS NMR.

PubMed

Shi, Xiangyan; Yarger, Jeffery L; Holland, Gregory P

2014-05-14

(2)H-(13)C HETCOR MAS NMR is performed on (2)H/(13)C/(15)N-Pro enriched A. aurantia dragline silk. Proline dynamics are extracted from (2)H NMR line shapes and T1 in a site-specific manner to elucidate the backbone and side chain molecular dynamics for the MaSp2 GPGXX β-turn regions for spider dragline silk in the dry and wet, supercontracted states.

Novel toxic shock syndrome toxin-1 amino acids required for biological activity.

PubMed

Brosnahan, Amanda J; Schaefers, Matthew M; Amundson, William H; Mantz, Mary J; Squier, Christopher A; Peterson, Marnie L; Schlievert, Patrick M

2008-12-09

Superantigens interact with T lymphocytes and macrophages to cause T lymphocyte proliferation and overwhelming cytokine production, which lead to toxic shock syndrome. Staphylococcus aureus superantigen toxic shock syndrome toxin-1 is a major cause of menstrual toxic shock syndrome. In general, superantigen-secreting S. aureus remains localized at the vaginal surface, and the superantigen must therefore penetrate the vaginal mucosa to interact with underlying immune cells to cause toxic shock syndrome. A dodecapeptide region (toxic shock syndrome toxin-1 amino acids F119-D130), relatively conserved among superantigens, has been implicated in superantigen penetration of the epithelium. The purpose of this study was to determine amino acids within this dodecapeptide region that are required for interaction with vaginal epithelium. Alanine mutations were constructed in S. aureus toxic shock syndrome toxin-1 amino acids D120 to D130. All mutants maintained superantigenicity, and selected mutants were lethal when given intravenously to rabbits. Toxic shock syndrome toxin-1 induces interleukin-8 from immortalized human vaginal epithelial cells; however, three toxin mutants (S127A, T128A, and D130A) induced low levels of interleukin-8 compared to wild type toxin. When carboxy-terminal mutants (S127A to D130A) were administered vaginally to rabbits, D130A was nonlethal, while S127A and T128A demonstrated delayed lethality compared to wild type toxin. In a porcine ex vivo permeability model, mutant D130A penetrated the vaginal mucosa more quickly than wild type toxin. Toxic shock syndrome toxin-1 residue D130 may contribute to binding an epithelial receptor, which allows it to penetrate the vaginal mucosa, induce interleukin-8, and cause toxic shock syndrome.

Break-dance: an unusual cause of hammer syndrome.

PubMed

Schneider, Frédéric; Milesi, Ilaria; Haesler, Erik; Wicky, Stephan; Schnyder, P; Denys, Alban

2002-01-01

We report the case of a young break-dancer presenting with hammer syndrome. This syndrome has been correlated with many professional and recreational activities but this is, to our knowledge, the first description of hammer syndrome caused by break-dancing. The etiology, diagnosis and treatment modalities of this rare syndrome are considered.

Break-Dance: An Unusual Cause of Hammer Syndrome

DOE Office of Scientific and Technical Information (OSTI.GOV)

Schneider, Frederic; Milesi, Ilaria; Haesler, Erik

2002-08-15

We report the case of a young break-dancer presenting with hammer syndrome. This syndrome has been correlated with many professional and recreational activities but this is, to our knowledge, the first description of hammer syndrome caused by break-dancing. The etiology, diagnosis and treatment modalities of this rare syndrome are considered.

Characterization of Mast Cell Activation Syndrome.

PubMed

Afrin, Lawrence B; Self, Sally; Menk, Jeremiah; Lazarchick, John

2017-03-01

Mast cell activation syndrome (MCAS), a recently recognized nonneoplastic mast cell disease driving chronic multisystem inflammation and allergy, appears prevalent and thus important. We report the first systematic characterization of a large MCAS population. Demographics, comorbidities, symptoms, family histories, physical examination and laboratory findings were reviewed in 298 retrospective and 115 prospective patients with MCAS. Blood samples from prospective subjects were examined by flow cytometry for clonal mast cell disease and tested for cytokines potentially driving the monocytosis frequent in MCAS. Demographically, white females dominated. Median ages at symptom onset and diagnosis were 9 and 49 years, respectively (range: 0-88 and 16-92, respectively) and median time from symptom onset to diagnosis was 30 years (range: 1-85). Median numbers of comorbidities, symptoms, and family medical issues were 11, 20, and 4, respectively (range: 1-66, 2-84, and 0-33, respectively). Gastroesophageal reflux, fatigue and dermatographism were the most common comorbidity, symptom and examination finding. Abnormalities in routine laboratories were common and diverse but typically modest. The most useful diagnostic markers were heparin, prostaglandin D 2 , histamine and chromogranin A. Flow cytometric and cytokine assessments were unhelpful. Our study highlights MCAS׳s morbidity burden and challenging heterogeneity. Recognition is important given good survival and treatment prospects. Copyright © 2017 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.

Dietary patterns as compared with physical activity in relation to metabolic syndrome among Chinese adults.

PubMed

He, Y; Li, Y; Lai, J; Wang, D; Zhang, J; Fu, P; Yang, X; Qi, L

2013-10-01

To examine the nationally-representative dietary patterns and their joint effects with physical activity on the likelihood of metabolic syndrome (MS) among 20,827 Chinese adults. CNNHS was a nationally representative cross-sectional observational study. Metabolic syndrome was defined according to the Joint Interim Statement definition. The "Green Water" dietary pattern, characterized by high intakes of rice and vegetables and moderate intakes in animal foods was related to the lowest prevalence of MS (15.9%). Compared to the "Green Water" dietary pattern, the "Yellow Earth" dietary pattern, characterized by high intakes of refined cereal products, tubers, cooking salt and salted vegetable was associated with a significantly elevated odds of MS (odds ratio 1.66, 95%CI: 1.40-1.96), after adjustment of age, sex, socioeconomic status and lifestyle factors. The "Western/new affluence" dietary pattern characterized by higher consumption of beef/lamb, fruit, eggs, poultry and seafood also significantly associated with MS (odds ratio: 1.37, 95%CI: 1.13-1.67). Physical activity showed significant interactions with the dietary patterns in relation to MS risk (P for interaction = 0.008). In the joint analysis, participants with the combination of sedentary activity with the "Yellow Earth" dietary pattern or the "Western/new affluence" dietary pattern both had more than three times (95%CI: 2.8-6.1) higher odds of MS than those with active activity and the "Green Water" dietary pattern. Our findings from the large Chinese national representative data indicate that dietary patterns affect the likelihood of MS. Combining healthy dietary pattern with active lifestyle may benefit more in prevention of MS. Copyright © 2012 Elsevier B.V. All rights reserved.

Effects of female sex hormones on expression of the Ang-(1-7)/Mas-R/nNOS pathways in rat brain.

PubMed

Cheng, Yuan; Li, Qiaoying; Zhang, Yidan; Wen, Quan; Zhao, Jianjun

2015-11-01

Female sex hormones are considered to reduce the risk of ischemic stroke. As a part of the renin-angiotensin system, angiotensin-(1-7) [Ang-(1-7)] has recently been reported to play a role in protecting neuronal tissues from ischemic stroke. Thus, we examined the effects of female sex hormones on the levels of Ang-(1-7) and its downstream pathways in the brain. Female rats were ovariectomized and 17β-estradiol (17β-EST), progesterone (PGR), or a combination of 17β-EST plus PGR were administered. Our data demonstrated that lack of female sex hormones significantly decreased the levels of Ang-(1-7) in the cerebral cortex and hippocampal CA1 area. Also, we observed a linear relationship between cortex levels of Ang-(1-7) and plasma brain natriuretic peptide levels (as an indicator for risk of ischemic stroke). We further showed that lack of female sex hormones decreased the expression of Ang-(1-7), Mas-receptor (Mas-R), and neuronal nitric oxide synthase (nNOS). Overall, our findings show for the first time that Ang-(1-7) and Mas-R/nNOS in the cortex are influenced by circulating 17β-EST and (or) PGR, whereas Ang-(1-7) and its pathways in the hippocampal CA1 area are primarily altered by 17β-EST. This suggests that female sex hormones play a role in regulating the expression of Ang-(1-7) and its pathways during ischemic brain injuries.

A small and active ring X chromosome in a female with features of Kabuki syndrome.

PubMed

Rodríguez, L; Diego-Alvarez, D; Lorda-Sanchez, I; Gallardo, F L; Martínez-Fernández, M L; Arroyo-Muñoz, M E; Martínez-Frías, M L

2008-11-01

A ring X chromosome is found in about 6% of patients with Turner syndrome (TS), often with mosaicism for a 45,X cell line. Patients with this karyotype are reported to have a higher incidence of a more severe phenotype including mental retardation. In fact, some studies have shown a correlation between this severity and the presence or absence of an intact and functional X inactivation center (XIST). However, the phenotype of the individuals with r(X) cannot be entirely defined in terms of their X-inactivation patterns. Nevertheless, a small group of these patients have been described to manifest clinical features reminiscent of the Kabuki syndrome. Here we present a female patient with clinical features resembling Kabuki syndrome and a mos 45,X/46,X,r(X) karyotype. Methylation analyses of polymorphic alleles of the androgen receptor gene showed that both alleles were unmethylated suggesting an active ring chromosome. A specific X chromosome array CGH was performed estimating the size of the ring to be 17 Mb, lacking the XIST gene, and including some genes with possible implications in the phenotype of the patient. Copyright 2008 Wiley-Liss, Inc.

Obesity, Food Selectivity, and Physical Activity in Individuals with Fragile X Syndrome

ERIC Educational Resources Information Center

Raspa, Melissa; Bailey, Donald B., Jr.; Bishop, Ellen; Holiday, David; Olmsted, Murrey

2010-01-01

National survey data from 884 families were used to examine the overall health of children and adults with fragile X syndrome. Results indicate the rate of obesity in adults with fragile X syndrome is similar to the general population (30%). Male children with fragile X syndrome, however, had higher rates of obesity (31%) when compared with…

[Amotivational syndrome in organic solvent abusers].

PubMed

Ozaki, S; Wada, K

2001-01-01

Amotivational syndrome is a chronic psychiatric disorder characterized by a variety of changes in personality, emotions and cognitive functions such as lack of activity, inward-turning, avolition, apathy, incoherence, blunted affect, inability to concentrate and memory disturbance. The syndrome was first described among those patients with a history of longtime cannabis use in the 1960's. Since then, there have been several reports describing similar psychiatric disorders to amotivational syndrome among patients with the history of some other psychoactive substances use including solvents, methamphetamine and OTC cough syrups. Therefore, the syndrome has been recognized as one of the common psychiatric conditions that might develop in patients with a history of any psychoactive substance use. Recently, more attention has been paid to the biological basis of amotivational syndrome. Several studies using MRI, SPECT or neuropsychological measures have revealed white matter changes, hypoperfusion in the frontal cortex of the brain and impairment of frontal lobe function. Those findings suggest that amotivational syndrome might be related to "hypofrontality" of the brain. Although no specific treatments have been reported to be definitely effective for patients with amotivational syndrome, some neuroleptics with activating properties or antidepressants can be given appropriately to treat the chief symptoms of the patients.

Plasma plasminogen activator inhibitor-1 levels and nonalcoholic fatty liver in individuals with features of metabolic syndrome.

PubMed

de Larrañaga, Gabriela; Wingeyer, Silvia Perés; Graffigna, Mabel; Belli, Susana; Bendezú, Karla; Alvarez, Silvia; Levalle, Oscar; Fainboim, Hugo

2008-07-01

Fatty liver represents the liver component of metabolic syndrome and may be involved in plasminogen activator inhibitor-1 (PAI-1) synthesis. We studied plasma PAI-1 levels and relationships with risk factors for metabolic syndrome, including fatty liver, in 170 patients. Liver ultrasound scan was performed on all patients, and a liver biopsy was performed on those patients with chronically elevated transaminase levels. Plasma PAI-1 levels correlated significantly (P < .05) with body mass index, degree of steatosis, insulin resistance, insulin level, waist circumference, triglycerides, and high-density lipoprotein (HDL) -cholesterol. However, only body mass index (beta = .455) and HDL-cholesterol (beta = .293) remained predictors of PAI-1 levels. Liver biopsy revealed a significant correlation (P < .05) between insulin resistance (r = 0.381) or insulin level (r = 0.519) and liver fibrosis. In patients presenting features of metabolic syndrome, plasma PAI-1 levels were mainly conditioned by the whole-body fat content.

Noonan syndrome - a new survey.

PubMed

Tafazoli, Alireza; Eshraghi, Peyman; Koleti, Zahra Kamel; Abbaszadegan, Mohammadreza

2017-02-01

Noonan syndrome (NS) is an autosomal dominant disorder with vast heterogeneity in clinical and genetic features. Various symptoms have been reported for this abnormality such as short stature, unusual facial characteristics, congenital heart abnormalities, developmental complications, and an elevated tumor incidence rate. Noonan syndrome shares clinical features with other rare conditions, including LEOPARD syndrome, cardio-facio-cutaneous syndrome, Noonan-like syndrome with loose anagen hair, and Costello syndrome. Germline mutations in the RAS-MAPK (mitogen-activated protein kinase) signal transduction pathway are responsible for NS and other related disorders. Noonan syndrome diagnosis is primarily based on clinical features, but molecular testing should be performed to confirm it in patients. Due to the high number of genes associated with NS and other RASopathy disorders, next-generation sequencing is the best choice for diagnostic testing. Patients with NS also have higher risk for leukemia and specific solid tumors. Age-specific guidelines for the management of NS are available.

Activity Pacing Self-Management in Chronic Fatigue Syndrome: A Randomized Controlled Trial

PubMed Central

van Eupen, Inge; Meirte, Jill; Van Cauwenbergh, Deborah; Moorkens, Greta; Meeus, Mira; Nijs, Jo

2015-01-01

OBJECTIVE. The objective of this study was to evaluate the effectiveness of an activity pacing self-management (APSM) intervention in improving performance of daily life activities in women with chronic fatigue syndrome (CFS). METHOD. A total of 33 women with CFS (age 41.1 ± 11.2 yr) were randomly allocated to APSM (experimental group; n = 16) or relaxation (control group; n = 17). Main outcome measures included the Canadian Occupational Performance Measure (COPM; primary) and Checklist Individual Strength (CIS). RESULTS. COPM scores changed significantly over time in both groups (p = .03). The change in Satisfaction scores showed a significant difference in favor only of APSM (effect size = 0.74 [0.11, 1.4]). CIS scores decreased significantly in the experimental group only (p < .01). CONCLUSION. APSM was found to be feasible and effective in optimizing participation in desired daily life activities in women with CFS. Replication in a larger sample with long-term follow-up is required. PMID:26356665

Marfan Syndrome: A Clinical Update.

PubMed

Bitterman, Adam D; Sponseller, Paul D

2017-09-01

Marfan syndrome is a connective tissue disorder that can affect many organ systems. Affected patients present with orthopaedic manifestations of the syndrome during all phases of life. Pain caused by musculoskeletal abnormalities often requires definitive orthopaedic treatment. Orthopaedic surgeons must understand the phenotypes of Marfan syndrome so they can recognize when screening is warranted and can appropriately address the skeletal manifestations. Through medical advancements, patients with Marfan syndrome are living longer and more active lives. Knowledge of the latest diagnostic criteria for the disorder, as well as of advances in understanding the skeletal phenotype, clinical trials of medication therapy, and lifestyle considerations is important for orthopaedic surgeons who treat these patients because these clinicians often are the first to suspect Marfan syndrome and recommend screening.

Physical activity does not attenuate the relationship between daily cortisol and metabolic syndrome in obese youth.

PubMed

Guseman, Emily Hill; Pfeiffer, Karin A; Carlson, Joseph J; Stansbury, Kathy; Eisenmann, Joey C

2016-01-01

We examined the associations among daily cortisol, physical activity (MVPA) and continuous metabolic syndrome score (cMetS) in obese youth. Fifty adolescents (mean age 14.8 ± 1.9 years) were recruited from medical clinics. Daily MVPA (min/day) was assessed by accelerometry. Saliva was sampled at prescribed times: immediately upon waking; 30 min after waking; and 3, 6 and 9 h after waking. Fasting lipids, glucose, waist circumference and blood pressure were used to calculate a continuous metabolic syndrome score (cMetS). Multiple linear regression analysis was used to examine associations among variables. The mean cMetS score was 4.16 ± 4.30 and did not differ by clinic or sex. No significant relationship was found between cortisol area under the curve (cAUC) and cMetS, nor did the interaction of MVPA with cAUC significantly predict cMetS. Physical activity, cortisol, and metabolic risk were not associated in this sample of obese adolescents. Future research should examine the role of insulin sensitivity in these relationships.

Noonan syndrome gain-of-function mutations in NRAS cause zebrafish gastrulation defects

PubMed Central

Runtuwene, Vincent; van Eekelen, Mark; Overvoorde, John; Rehmann, Holger; Yntema, Helger G.; Nillesen, Willy M.; van Haeringen, Arie; van der Burgt, Ineke; Burgering, Boudewijn; den Hertog, Jeroen

2011-01-01

SUMMARY Noonan syndrome is a relatively common developmental disorder that is characterized by reduced growth, wide-set eyes and congenital heart defects. Noonan syndrome is associated with dysregulation of the Ras–mitogen-activated-protein-kinase (MAPK) signaling pathway. Recently, two mutations in NRAS were reported to be associated with Noonan syndrome, T50I and G60E. Here, we report a mutation in NRAS, resulting in an I24N amino acid substitution, that we identified in an individual bearing typical Noonan syndrome features. The I24N mutation activates N-Ras, resulting in enhanced downstream signaling. Expression of N-Ras-I24N, N-Ras-G60E or the strongly activating mutant N-Ras-G12V, which we included as a positive control, results in developmental defects in zebrafish embryos, demonstrating that these activating N-Ras mutants are sufficient to induce developmental disorders. The defects in zebrafish embryos are reminiscent of symptoms in individuals with Noonan syndrome and phenocopy the defects that other Noonan-syndrome-associated genes induce in zebrafish embryos. MEK inhibition completely rescued the activated N-Ras-induced phenotypes, demonstrating that these defects are mediated exclusively by Ras-MAPK signaling. In conclusion, mutations in NRAS from individuals with Noonan syndrome activated N-Ras signaling and induced developmental defects in zebrafish embryos, indicating that activating mutations in NRAS cause Noonan syndrome. PMID:21263000

Oral Colostrum Macrophage-activating Factor for Serious Infection and Chronic Fatigue Syndrome: Three Case Reports.

PubMed

Inui, Toshio; Kubo, Kentaro; Kuchiike, Daisuke; Uto, Yoshihiro; Nishikata, Takahito; Sakamoto, Norihiro; Mette, Martin

2015-08-01

Gc protein-derived macrophage-activating factor (GcMAF) immunotherapy has been steadily advancing over the last two decades. Oral colostrum macrophage-activating factor (MAF) produced from bovine colostrum has shown high macrophage phagocytic activity. GcMAF-based immunotherapy has a wide application for use in treating many diseases via macrophage activation or for use as supportive therapy. Three case studies demonstrate that oral colostrum MAF can be used for serious infection and chronic fatigue syndrome (CFS) without adverse effects. We demonstrate that colostrum MAF shows promising clinical results in patients with infectious diseases and for symptoms of fatigue, which is common in many chronic diseases. Copyright© 2015 International Institute of Anticancer Research (Dr. John G. Delinassios), All rights reserved.

Binding of a small molecule water channel inhibitor to aquaporin Z examined by solid-state MAS NMR.

PubMed

Phillips, Margaret; To, Janet; Yamazaki, Toshio; Nagashima, Toshio; Torres, Jaume; Pervushin, Konstantin

2018-06-18

Aquaporins are integral membrane proteins that facilitate water flow across biological membranes. Their involvement in multiple physiological functions and disease states has prompted intense research to discover water channel activity modulators. However, inhibitors found so far are weak and/or lack specificity. For organic compounds, which lack of high electron-dense atoms, the identification of binding sites is even more difficult. Nuclear magnetic resonance spectroscopy (NMR) requires large amounts of the protein, and expression and purification of mammalian aquaporins in large quantities is a difficult task. However, since aquaporin Z (AqpZ) can be purified and expressed in good quantities and has a high similarity to human AQP1 (~ 40% identity), it can be used as a model for studying the structure and function of human aquaporins. In the present study, we have used solid-state MAS NMR to investigate the binding of a lead compound [1-(4-methylphenyl)1H-pyrrole-2,5-dione] to AqpZ, through mapping of chemical shift perturbations in the presence of the compound.

[Foetal therapy for Down syndrome: a pro-active ethical reflection].

PubMed

de Wert, G M W R; Dondorp, W J

2016-01-01

Prenatal screening for Down syndrome has to date focused on facilitating the informed choice to continue or not with a pregnancy. The non-invasive prenatal test (NIPT) for Down syndrome does potentially offer the option to apply foetal neurocognitive therapy for Down syndrome (FTDS). Current research in animal models looks promising and therefore a proactive ethical reflection in relation to clinical trials is urgently needed. This discussion includes an exploration of the ethical aspects of FTDS. There seem to be no convincing a priori objections on the basis of the social model of disability. Arguments in terms of (respect for) autonomy, wellbeing and justice seem to in principle support such therapy. Still, both the conditions for sound clinical trials and the implications of possible effective therapy for current prenatal screening need further scrutiny.

Nakalanga Syndrome: Clinical Characteristics, Potential Causes, and Its Relationship with Recently Described Nodding Syndrome

PubMed Central

Föger, Kathrin; Gora-Stahlberg, Gina; Sejvar, James; Ovuga, Emilio; Jilek-Aall, Louise; Schmutzhard, Erich

2017-01-01

Nakalanga syndrome is a condition that was described in Uganda and various other African countries decades ago. Its features include growth retardation, physical deformities, endocrine dysfunction, mental impairment, and epilepsy, amongst others. Its cause remains obscure. Nodding syndrome is a neurological disorder with some features in common with Nakalanga syndrome, which has been described mainly in Uganda, South Sudan, and Tanzania. It has been considered an encephalopathy affecting children who, besides head nodding attacks, can also present with stunted growth, delayed puberty, and mental impairment, amongst other symptoms. Despite active research over the last years on the pathogenesis of Nodding syndrome, to date, no convincing single cause of Nodding syndrome has been reported. In this review, by means of a thorough literature search, we compare features of both disorders. We conclude that Nakalanga and Nodding syndromes are closely related and may represent the same condition. Our findings may provide new directions in research on the cause underlying this neurological disorder. PMID:28182652

[Tibial periostitis ("medial tibial stress syndrome")].

PubMed

Fournier, Pierre-Etienne

2003-06-01

Medial tibial stress syndrome is characterised by complaints along the posteromedial tibia. Runners and athletes involved in jumping activities may develop this syndrome. Increased stress to stabilize the foot especially when excessive pronation is present explain the occurrence this lesion.

[Social interactions of preschool children with Down syndrome during extracurricular activities].

PubMed

Lucisano, Renata Valdívia; Pfeifer, Luzia Iara; Pinto, Maria Paula Panuncio; Santos, Jair Lício Ferreira; Anhão, Patrícia Páfaro Gomes

2013-01-01

The aim of this research was to identify the process of social interaction of children with Down Syndrome (DS) during extracurricular activities in the regular early childhood education in Ribeirão Preto-SP, Brazil. Six children aged 3-6 years participated in this study. There were two recordings of each child in situations of social interaction during extracurricular activities, and analyzed by 15 behaviors, divided into two categories of social skills: interpersonal and self-expression. The results demonstrate that, in the interpersonal skill category, the higher occurrence was the behavior "occurs interaction with other children". In the self-expression skills category, only the behaviors "smiles" and "imitates other children" have significant occurrence. The behaviors more frequently identified in this study permit to understand that the school environment is a facilitator for the interaction of child with DS with the typical developmental children, allowing him/her to develop the expected social skills.

Severe Health-Related Quality of Life Impairment in Active Primary Sjögren's Syndrome and Patient-Reported Outcomes: Data From a Large Therapeutic Trial.

PubMed

Cornec, Divi; Devauchelle-Pensec, Valérie; Mariette, Xavier; Jousse-Joulin, Sandrine; Berthelot, Jean-Marie; Perdriger, Aleth; Puéchal, Xavier; Le Guern, Véronique; Sibilia, Jean; Gottenberg, Jacques-Eric; Chiche, Laurent; Hachulla, Eric; Yves Hatron, Pierre; Goeb, Vincent; Hayem, Gilles; Morel, Jacques; Zarnitsky, Charles; Dubost, Jean Jacques; Saliou, Philippe; Pers, Jacques Olivier; Seror, Raphaèle; Saraux, Alain

2017-04-01

To identify the principal determinants of health-related quality of life (HRQOL) impairment in patients with active primary Sjögren's syndrome (SS) participating in a large therapeutic trial, Tolerance and Efficacy of Rituximab in Primary Sjögren's Syndrome (TEARS). At the inclusion visit for the TEARS trial, 120 patients with active primary SS completed the Short Form 36 health survey (SF-36), a validated HRQOL assessment tool. Univariate then multivariate linear regression analyses were used to assess associations linking SF-36 physical and mental components to demographic data, patient-reported outcomes (symptom intensity assessments for dryness, pain, and fatigue, including the European League Against Rheumatism [EULAR] Sjögren's Syndrome Patient Reported Index [ESSPRI]), objective measures of dryness and autoimmunity, and physician evaluation of systemic activity (using the EULAR Sjögren's Syndrome Disease Activity Index [ESSDAI]). SF-36 scores indicated marked HRQOL impairments in our population with active primary SS. Approximately one-third of the patients had low, moderate, and high systemic activity according to the ESSDAI. ESSPRI and ESSDAI scores were moderately but significantly correlated. The factors most strongly associated with HRQOL impairment were patient-reported symptoms, best assessed using the ESSPRI, with pain and ocular dryness intensity showing independent associations with HRQOL. Conversely, systemic activity level was not associated with HRQOL impairment in multivariate analyses, even in the patient subset with ESSDAI values indicating moderate-to-high systemic activity. The cardinal symptoms of primary SS (dryness, pain, and fatigue, best assessed using the ESSPRI) are stronger predictors of HRQOL impairment than systemic involvement (assessed by the ESSDAI) and should be used as end points in future therapeutic trials focusing on patients' well-being. New consensual and data-driven response criteria are needed for primary SS

[Clinical efficacy of porcine pulmonary surfactant combined with budesonide suspension intratracheal instillation in the treatment of neonatal meconium aspiration syndrome].

PubMed

Tan, Xiu-Zhen; Wu, Shi-Guang; Zhang, Jian-Hua; Li, Xiao-Fen; Gao, Ping-Ming; Wang, Yu

2016-12-01

To study the clinical efficacy of porcine pulmonary surfactant (PS) combined with budesonide suspension intratracheal instillation in the treatment of neonatal meconium aspiration syndrome (MAS). Seventy neonates with MAS were enrolled for a prospective study. The neonates were randomly assigned to PS alone treatment group and PS+budesonide treatment group (n=35 each). The PS alone treatment group was given PS (100 mg/kg) by intratracheal instillation. The treatment group was given budesonide suspension (0.25 mg/kg) combined with PS (100 mg/kg). The rate of repeated use of PS in the PS+ budesonide group was significantly lower than that in the PS alone group 12 hours after treatment (p<0.05). The improvement of PaO 2 /FiO 2 , TcSaO 2 , PaO 2 , and PaCO 2 in the PS+ budesonide group was significantly greater than that in the PS alone group 6, 12, and 24 hours after treatment (p<0.05). The chest X-ray examination showed that the pulmonary inflammation absorption in the PS+ budesonide group was significantly better than that in the PS alone group 48 hours after treatment (p<0.05). The incidence of complications in the PS+budesonide group was significantly lower than that in the PS alone group (p<0.05), and the average hospitalization duration was significantly shorter than that in the PS alone group (p<0.01). PS combined with budesonide suspension intratracheal instillation for the treatment of neonatal MAS is effective and superior to PS alone treatment.

Experiences of daily activity in chronic fatigue syndrome/myalgic encephalomyelitis (CFS/ME) and their implications for rehabilitation programmes.

PubMed

Pemberton, Sue; Cox, Diane L

2014-01-01

Chronic Fatigue Syndrome, also known as Myalgic Encephalomyelitis (CFS/ME), has a significant impact upon daily functioning. Most recommended treatments aim to alter activity patterns based upon assumptions of activity avoidance. However, as there is limited research on the experience of activity and occupational beliefs in people with CFS/ME, this study took a qualitative approach to understand the meaning of activity in people with this disabling condition. This study applied a social constructivist grounded theory methodology. Semi-structured interviews took place with 14 participants attending a Specialist CFS/ME Service in England. The emergent themes described a premorbid state of constant action with difficulty stopping an activity once it had commenced. When this pattern was interrupted by illness, participants attempted to maintain their previous level of occupational engagement. Negative associations and emotions were described in response to the concept of doing nothing or limited activity. A recurring cycle was reported of increasing activity levels when symptoms improved, followed by post exertional symptoms. Consequently, participants' beliefs about concepts of both activity and inactivity need to be considered within the application of rehabilitation programmes for CFS/ME that aim to modify activity related behaviours. Chronic fatigue syndrome/myalgic encephalomyelitis (CFS/ME) is commonly treated in the UK using activity modification. In this small qualitative study, patients expressed negative feelings and beliefs towards the concept of doing nothing and therefore sought to push their activity levels when this was available, leading to recurring cycles of symptoms and activity. Rehabilitation programmes need to consider how people with CFS/ME engaged with activity and inactivity before the condition and how this may impact upon engagement with activity-based rehabilitation programmes.

[Capillary leak syndrome disclosing Ofuji's papuloerythroderma].

PubMed

Carsuzaa, F; Pierre, C; Morand, J J; Farnarier, C; Marrot, F; Kaplanski, G

1996-01-01

Capillary leak syndrome is a specific entity among syndromes with capillary hyperpermeability. Endothelial cell activation is related to the higt level of adhesion molecules (sICAM-1, sVCAM-&, sCD62E) possibly due to several cytokines (IL-2, TNF ...). An 84-year-old woman was hospitalized for erythroderma. Ofujui papuloerythroderma was diagnosed and edema was attributed to capillary leak. A kinetic study of several cytokines and adhesion molecules sCD62E, sVCAM-1 and sICAM-1 was done. Outcome was favorable with corticopuvatherapy. The capillary leak syndrome reported here is simlar to that described in other erythrodermas with or without lymphoma. The keratinocyte would be activated by the CD4 T lymphocyte via the gamma-interferon mediator. The T cell secretes cytokines (interleukin-1, tumor necrosis factor ...) which activates the endothelium and increases vascular permeability. The level of adhesion molecules and changes observed during the episode of edema demonstrated the endothelial activation.

Heart Activity and Autistic Behavior in Infants and Toddlers with Fragile X Syndrome

PubMed Central

Roberts, Jane E.; Tonnsen, Bridgette; Robinson, Ashley; Shinkareva, Svetlana V.

2014-01-01

The present study contrasted physiological arousal in infants and toddlers with fragile X syndrome to typically developing control participants and examined physiological predictors early in development to autism severity later in development in fragile X syndrome. Thirty-one males with fragile X syndrome (ages 8–40 months) and 25 age-matched control participants were included. The group with fragile X syndrome showed shorter interbeat intervals (IBIs), lower vagal tone (VT), and less modulation of IBI. Data suggested a nonlinear effect with IBI and autistic behavior; however, a linear effect with VT and autistic behavior emerged. These findings suggest that atypical physiological arousal emerges within the first year and predicts severity of autistic behavior in fragile X syndrome. These relationships are complex and dynamic, likely reflecting endogenous factors assumed to reflect atypical brain function secondary to reduced fragile X mental retardation protein. This research has important implications for the early identification and treatment of autistic behaviors in young children with fragile X syndrome. PMID:22515825

Determination of Structural Topology of a Membrane Protein in Lipid -Bilayers using Polarization Optimized Experiments (POE) for Static and MAS Solid State NMR Spectroscopy

PubMed Central

Mote, Kaustubh R.; Gopinath, T.; Veglia, Gianluigi

2013-01-01

The low sensitivity inherent to both the static and magic angle spinning techniques of solid-state NMR (ssNMR) spectroscopy has thus far limited the routine application of multidimensional experiments to determine the structure of membrane proteins in lipid bilayers. Here, we demonstrate the advantage of using a recently developed class of experiments, polarization optimized experiments (POE), for both static and MAS spectroscopy to achieve higher sensitivity and substantial time-savings for 2D and 3D experiments. We used sarcolipin, a single pass membrane protein, reconstituted in oriented bicelles (for oriented ssNMR) and multilamellar vesicles (for MAS ssNMR) as a benchmark. The restraints derived by these experiments are then combined into a hybrid energy function to allow simultaneous determination of structure and topology. The resulting structural ensemble converged to a helical conformation with a backbone RMSD ∼ 0.44 Å, a tilt angle of 24° ± 1°, and an azimuthal angle of 55° ± 6°. This work represents a crucial first step toward obtaining high-resolution structures of large membrane proteins using combined multidimensional O-ssNMR and MAS-ssNMR. PMID:23963722

Regulatory landscape of AGE-RAGE-oxidative stress axis and its modulation by PPARγ activation in high fructose diet-induced metabolic syndrome.

PubMed

Cannizzaro, Luca; Rossoni, Giuseppe; Savi, Federica; Altomare, Alessandra; Marinello, Cristina; Saethang, Thammakorn; Carini, Marina; Payne, D Michael; Pisitkun, Trairak; Aldini, Giancarlo; Leelahavanichkul, Asada

2017-01-01

The AGE-RAGE-oxidative stress (AROS) axis is involved in the onset and progression of metabolic syndrome induced by a high-fructose diet (HFD). PPARγ activation is known to modulate metabolic syndrome; however a systems-level investigation looking at the protective effects of PPARγ activation as related to the AROS axis has not been performed. The aim of this work is to simultaneously characterize multiple molecular parameters within the AROS axis, using samples taken from different body fluids and tissues of a rat model of HFD-induced metabolic syndrome, in the presence or absence of a PPARγ agonist, Rosiglitazone (RGZ). Rats were fed with 60% HFD for the first half of the treatment duration (21 days) then continued with either HFD alone or HFD plus RGZ for the second half. Rats receiving HFD alone showed metabolic syndrome manifestations including hypertension, dyslipidemia, increased glucose levels and insulin resistance, as well as abnormal kidney and inflammatory parameters. Systolic blood pressure, plasma triglyceride and glucose levels, plasma creatinine, and albuminuria were significantly improved in the presence of RGZ. The following molecular parameters of the AROS axis were significantly upregulated in our rat model: carboxymethyl lysine (CML) in urine and liver; carboxyethyl lysine (CEL) in urine; advanced glycation end products (AGEs) in plasma; receptor for advanced glycation end products (RAGE) in liver and kidney; advanced oxidation protein products (AOPP) in plasma; and 4-hydroxynonenal (HNE) in plasma, liver, and kidney. Conversely, with RGZ administration, the upregulation of AOPP and AGEs in plasma, CML and CEL in urine, RAGE in liver as well as HNE in plasma and liver was significantly counteracted/prevented. Our data demonstrate (i) the systems-level regulatory landscape of HFD-induced metabolic syndrome involving multiple molecular parameters, including HNE, AGEs and their receptor RAGE, and (ii) attenuation of metabolic syndrome by

Intravenous thrombolysis with recombinant tissue plasminogen activator in vascular warning syndromes.

PubMed

González Hernández, A; Fabre Pi, O; Cabrera Naranjo, F; López Veloso, A C

2014-01-01

Vascular warning syndromes constitute a neurological emergency due to their associated high risk of established stroke. At present, there is no strong evidence indicating the best treatment for these patients. The aim of this paper is to describe the function of intravenous rt-PA thrombolysis in the treatment of vascular warning syndromes. We reviewed our hospital records and the literature to find patients with neurologically fluctuating profiles and who underwent intravenous rt-PA thrombolysis. We retrieved 3 cases from our hospital records and 19 from the literature (15 males and 7 females). Mean age was 68.7±9 years (range: 52-84 years). The mean number of episodes before treatment was 4 (range: 2-15 episodes). The maximum NIHSS scores ranged from 6 to 22 in different patients. We obtained 24-hour post-treatment NIHSS scores in 8 cases; of these cases, 6 (75%) had a score of 0, and the other 2 (25%) had a score of 12. The Modified Rankin Score calculated at 3 months of treatment was 0 or 1 in 18 patients (81.8%); these 18 comprised 8 of the 10 patients with lacunar warning syndromes (80%), 6 of the 7 with basilar warning syndromes (85.7%), and 4 of the 5 with fluctuating non-lacunar, non-basilar warning syndromes (80%). Intravenous rt-PA treatment may constitute an effective and safe therapeutic alternative for patients with neurovascular fluctuations. However, well-designed studies are needed to determine the role of intravenous rt-PA thrombolysis in cases of vascular warning syndrome. Copyright © 2013 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.

Physical activity in prevention and treatment of the metabolic syndrome.

PubMed

Lakka, Timo A; Laaksonen, David E

2007-02-01

Randomised controlled trials have shown that exercise training has a mild or moderate favourable effect on many metabolic and cardiovascular risk factors that constitute or are related to the metabolic syndrome (MetS). Epidemiological studies suggest that regular physical activity prevents type 2 diabetes, cardiovascular disease, and premature mortality in large part through these risk factors. Although randomized controlled trials with the prevention or treatment of the MetS as the main outcome have not been published, several large randomized controlled trials provide strong evidence that favourable lifestyle changes, including regular physical activity, are effective in the prevention of type 2 diabetes in individuals who are overweight and have impaired glucose tolerance. Compliance with the current recommendations to increase the total volume of moderate-intensity physical activity and to maintain good cardiorespiratory and muscular fitness appears to markedly decrease the likelihood of developing the MetS, especially in high-risk groups. Walking is the most common form of physical activity--it improves health in many ways and is generally safe. Therefore, brisk walking for at least 30 min daily can be recommended as the principal form of physical activity at the population level. If there are no contraindications, more vigorous physical exercise or resistance training should also be considered to obtain additional health benefits. Unstructured and low-intensity physical activity may also decrease the likelihood of developing the MetS, especially when substituted for sedentary behaviours such as watching television. The measurement of maximal oxygen consumption may provide an efficient means to target even individuals with relatively few metabolic risk factors who may benefit from more intensive intervention.

Immunomodulating activity of Pidotimod in children with Down syndrome.

PubMed

Zuccotti, G V; Mameli, C; Trabattoni, D; Beretta, S; Biasin, M; Guazzarotti, L; Clerici, M

2013-01-01

Acute respiratory tract infections (ARTIs) are the most frequent illnesses in pediatric age, frequently experienced in children with Down Syndrome (DS) due to the associated immune defects of both specific and non-specific immunity. Pidotimod, a synthetic immunostimulant, was shown to reduce the rates of ARTIs in children with DS, however the mechanisms associated with this effect is currently unknown. We analyzed immune parameters in DS children who received the seasonal 2011–2012 virosomal-adjuvanted influenza vaccine. Eighteen children aged 3-10 years (mean age 7.1+/-2.6 years) were randomly assigned (1:1 ratio) to receive Pidotimod 400 mg, administered orally once a day for 90 days or placebo. At the recruitment (T0) all children received a single dose of virosomal-adjuvanted influenza vaccine (Flu). Blood samples were collected at T0 and 3 months after the recruitment (T3) in order to evaluate innate and adaptative immune responses pathway. Flu-specific IgG1 and IgG3 levels in plasma samples were determined at pre-vaccination (T0), and 1 (T1) and 3 months (T3) post-vaccination. The use of Pidotimod was associated with the upregulation of a number of genes involved in the activation of innate immune responses and in antimicrobial activity. Interestingly the ratio of Flu-specific IgG1/IgG3 was skewed in pidotimod-treated individuals, suggesting a preferential activation of complement-dependent effector mechanisms. Although preliminary these data suggest that Pidotimod can potentiate the beneficial effect of immunization, possibly resulting in a stronger activity of both innate and adaptive immune responses.

The cardiofaciocutaneous syndrome

PubMed Central

Roberts, A; Allanson, J; Jadico, S K; Kavamura, M I; Noonan, J; Opitz, J M; Young, T; Neri, G

2006-01-01

The cardiofaciocutaneous (CFC) syndrome is a condition of sporadic occurrence, with patients showing multiple congenital anomalies and mental retardation. It is characterised by failure to thrive, relative macrocephaly, a distinctive face with prominent forehead, bitemporal constriction, absence of eyebrows, hypertelorism, downward‐slanting palpebral fissures often with epicanthic folds, depressed nasal root and a bulbous tip of the nose. The cutaneous involvement consists of dry, hyperkeratotic, scaly skin, sparse and curly hair, and cavernous haemangiomata. Most patients have a congenital heart defect, most commonly pulmonic stenosis and hypertrophic cardiomyopathy. The developmental delay usually is moderate to severe. The syndrome is caused by gain‐of‐function mutations in four different genes BRAF, KRAS, mitogen‐activated protein/extracellular signal‐regulated kinase MEK1 and MEK2, all belonging to the same RAS–extracellular signal‐regulated kinase (ERK) pathway that regulates cell differentiation, proliferation and apoptosis. The CFC syndrome is a member of a family of syndromes that includes the Noonan and Costello syndromes, presenting with phenotypic similarities. Noonan syndrome is caused by mutations in the protein tyrosine phosphatase SHP‐2 gene (PTPN11), with a few people having a mutation in KRAS. Costello syndrome is caused by mutations in HRAS. The protein products of these genes also belong to the RAS–ERK pathway. Thus, the clinical overlap of these three conditions, which often poses a problem of differential diagnosis, is explained by their pathogenetic relatedness. PMID:16825433

States' Participation Guidelines for Alternate Assessments Based on Modified Academic Achievement Standards (AA-MAS) in 2008. Synthesis Report 71

ERIC Educational Resources Information Center

Lazarus, Sheryl S.; Rogers, Christopher; Cormier, Damien; Thurlow, Martha L.

2008-01-01

Federal regulations (U.S. Department of Education, 2007a) provide states with the flexibility to offer an alternate assessment based on modified academic achievement standards (AA-MAS). This assessment option is for a small group of students with disabilities who can make significant progress, but may not reach grade-level achievement within the…

Heart Activity and Autistic Behavior in Infants and Toddlers with Fragile X Syndrome

ERIC Educational Resources Information Center

Roberts, Jane E.; Tonnsen, Bridgette; Robinson, Ashley; Shinkareva, Svetlana V.

2012-01-01

The present study contrasted physiological arousal in infants and toddlers with fragile X syndrome to typically developing control participants and examined physiological predictors early in development to autism severity later in development in fragile X syndrome. Thirty-one males with fragile X syndrome (ages 8-40 months) and 25 age-matched…

Physical activity in women with polycystic ovary syndrome: prevalence, predictors, and positive health associations.

PubMed

Lamb, Julie D; Johnstone, Erica B; Rousseau, Julie-Anne; Jones, Christopher L; Pasch, Lauri A; Cedars, Marcelle I; Huddleston, Heather G

2011-04-01

The purpose of this study was to describe the prevalence and predictors of physical activity in women with polycystic ovary syndrome (PCOS) and to explore the potential health benefits that are associated with physical activity in this population. This was a cross-sectional assessment of 150 women with PCOS. Active women (those who met Department of Health and Human Services [DHHS] guidelines for exercise) were compared with inactive women with regards to demographic and psychosocial variables and health characteristics. Fifty-nine percent (88/150 women) met the DHHS guidelines for physical activity. Active women were more likely than inactive women to be nulliparous (64.1% vs 40.0%; P = .04) and white (71.6% vs 42.6%; P = .0004). Inactive women were more likely to have mild depression (adjusted odds ratio, 2.2; 95% confidence interval, 1.01-4.79; P = .048). Women with PCOS who met the DHHS guidelines for physical activity were more likely to enjoy a variety of health benefits. Our findings identify several groups that are at risk for inadequate physical activity. Copyright © 2011. Published by Mosby, Inc.

Endocrine manifestations of Down syndrome.

PubMed

Whooten, Rachel; Schmitt, Jessica; Schwartz, Alison

2018-02-01

To summarize the recent developments in endocrine disorders associated with Down syndrome. Current research regarding bone health and Down syndrome continues to show an increased prevalence of low bone mass and highlights the importance of considering short stature when interpreting dual energy x-ray absorptiometry. The underlying cause of low bone density is an area of active research and will shape treatment and preventive measures. Risk of thyroid disease is present throughout the life course in individuals with Down syndrome. New approaches and understanding of the pathophysiology and management of subclinical hypothyroidism continue to be explored. Individuals with Down syndrome are also at risk for other autoimmune conditions, with recent research revealing the role of the increased expression of the Autoimmune Regulatory gene on 21st chromosome. Lastly, Down-syndrome-specific growth charts were recently published and provide a better assessment of growth. Recent research confirms and expands on the previously known endocrinopathies in Down syndrome and provides more insight into potential underlying mechanisms.

Molecular genetics of Liddle's syndrome.

PubMed

Yang, Kun-Qi; Xiao, Yan; Tian, Tao; Gao, Ling-Gen; Zhou, Xian-Liang

2014-09-25

Liddle's syndrome, an autosomal dominant form of monogenic hypertension, is characterized by salt-sensitive hypertension with early penetrance, hypokalemia, metabolic alkalosis, suppression of plasma rennin activity and aldosterone secretion, and a clear-cut response to epithelial sodium channel (ENaC) blockers but not spironolactone therapy. Our understanding of ENaCs and Na(+) transport defects has expanded greatly over the past two decades and provides detailed insight into the molecular basis of Liddle's syndrome. In this review, we offer an overview of recent advances in understanding the molecular genetics of Liddle's syndrome, involving mutation analysis, molecular mechanisms and genetic testing. The ENaC in the distal nephron is composed of α, β and γ subunits that share similar structures. Mutations associated with Liddle's syndrome are positioned in either β or γ subunits and disturb or truncate a conserved proline-rich sequence (i.e., PY motif), leading to constitutive activation of the ENaC. Genetic testing has made it possible to make accurate diagnoses and develop tailored therapies for mutation carriers. Copyright © 2014 Elsevier B.V. All rights reserved.

Transport Properties of Ibuprofen Encapsulated in Cyclodextrin Nanosponge Hydrogels: A Proton HR-MAS NMR Spectroscopy Study.

PubMed

Ferro, Monica; Castiglione, Franca; Punta, Carlo; Melone, Lucio; Panzeri, Walter; Rossi, Barbara; Trotta, Francesco; Mele, Andrea

2016-08-15

The chemical cross-linking of β-cyclodextrin (β-CD) with ethylenediaminetetraacetic dianhydride (EDTA) led to branched polymers referred to as cyclodextrin nanosponges (CDNSEDTA). Two different preparations are described with 1:4 and 1:8 CD-EDTA molar ratios. The corresponding cross-linked polymers were contacted with 0.27 M aqueous solution of ibuprofen sodium salt (IP) leading to homogeneous, colorless, drug loaded hydrogels. The systems were characterized by high resolution magic angle spinning (HR-MAS) NMR spectroscopy. Pulsed field gradient spin echo (PGSE) NMR spectroscopy was used to determine the mean square displacement (MSD) of IP inside the polymeric gel at different observation times td. The data were further processed in order to study the time dependence of MSD: MSD = f(td). The proposed methodology is useful to characterize the different diffusion regimes that, in principle, the solute may experience inside the hydrogel, namely normal or anomalous diffusion. The full protocols including the polymer preparation and purification, the obtainment of drug-loaded hydrogels, the NMR sample preparation, the measurement of MSD by HR-MAS NMR spectroscopy and the final data processing to achieve the time dependence of MSD are here reported and discussed. The presented experiments represent a paradigmatic case and the data are discussed in terms of innovative approach to the characterization of the transport properties of an encapsulated guest within a polymeric host of potential application for drug delivery.

A heart breaking case of rapidly developing severe hemophagocytic syndrome secondary to chronic active EBV infection; a case report and review of the literature.

PubMed

Tawfik, Khoury; Liron, Yosha; Ayman, Abu Rmieleh; Schneider, Ronen; Wolf, D G; Ronen, Levi

2015-06-01

Epstein-Barr virus (EBV, HHV-4) is a gamma Herpesvirus with a 90% >seroprevalence in adults. Reactivations in non-immuno compromised individuals usually cause mild or no symptoms at all. Rarely, host immunity-virus balance is interrupted, resulting in a chronic active EBV infection. The following case illustrates the rapid development of severe hemophagocytic syndrome during chronic active EBV infection in a 73 year old woman who presented with lower extremity pain and edema, splenomegaly and abnormal liver enzymes. A diagnosis of chronic active EBV infection was made following an extensive investigation and the patient died secondary to rapidly progressive hemophagocytic syndrome. Copyright © 2015 Elsevier B.V. All rights reserved.

[Descriptive and comparative study of cardiovascular risk factors and physical activity in patients with acute coronary syndrome].

PubMed

Vazquez-Arce, Maria Isabel; Marques-Sule, Elena

2017-08-22

To analyse several cardiovascular risk factors by means of the physical activity performed by patients with acute coronary syndrome (ACS). Cross-sectional study. Cardiovascular prevention service (Health Department, Valencia, Spain). The study included 401 individuals with acute coronary syndrome and discharged from hospital 2-3months before the assessment. The inclusion criteria included age between 30 and 80years-old, no contraindication for physical activity, and no previous participation in cardiac rehabilitation programmes. Metabolic equivalent MET (Kcal/Kg) was calculated, based on the type of activity, frequency, duration and intensity. Participants were divided into two groups: sedentary group (<10METs/week) and physically active group (≥10METs/week). Several variables associated with cardiovascular risk factors were assessed: body mass index (BMI), waist circumference, lipid profile, blood glucose, and arterial pressure. The mean consumption was 8.24±12.5METs/week. Prevalent factors were overweight (77.05%), and dyslipidaemia (64.3%), whilst 64.8% were sedentary. The physically active group showed differences when compared to sedentary group in triglycerides (146.53±72.8 vs. 166.94±104.8mg/dL; 95%CI; P=.031), and BMI (27.65±3.86 vs. 28.50±4.38kg/m 2 ; 95%CI; P=.045). Physical activity was performed by a limited number of patients with ACS, with a prevalence of overweight and dyslipidaemia. Being physically active improved triglycerides levels and BMI. Therefore, health promotion from Primary Care and encouraging physical activity amongst patients with ACS is crucial. Copyright © 2017 Elsevier España, S.L.U. All rights reserved.

Physical activity improves symptoms in irritable bowel syndrome: a randomized controlled trial.

PubMed

Johannesson, Elisabet; Simrén, Magnus; Strid, Hans; Bajor, Antal; Sadik, Riadh

2011-05-01

Physical activity has been shown to be effective in the treatment of conditions, such as fibromyalgia and depression. Although these conditions are associated with irritable bowel syndrome (IBS), no study has assessed the effect of physical activity on gastrointestinal (GI) symptoms in IBS. The aim was to study the effect of physical activity on symptoms in IBS. We randomized 102 patients to a physical activity group and a control group. Patients of the physical activity group were instructed by a physiotherapist to increase their physical activity, and those of the control group were instructed to maintain their lifestyle. The primary end point was to assess the change in the IBS Severity Scoring System (IBS-SSS). A total of 38 (73.7% women, median age 38.5 (19-65) years) patients in the control group and 37 (75.7% women, median age 36 (18-65) years) patients in the physical activity group completed the study. There was a significant difference in the improvement in the IBS-SSS score between the physical activity group and the control group (-51 (-130 and 49) vs. -5 (-101 and 118), P=0.003). The proportion of patients with increased IBS symptom severity during the study was significantly larger in the control group than in the physical activity group. Increased physical activity improves GI symptoms in IBS. Physically active patients with IBS will face less symptom deterioration compared with physically inactive patients. Physical activity should be used as a primary treatment modality in IBS.

[Hypovitaminosis D and metabolic syndrome].

PubMed

Miñambres, Inka; de Leiva, Alberto; Pérez, Antonio

2014-12-23

Metabolic syndrome and hypovitaminosis D are 2 diseases with high prevalence that share several risk factors, while epidemiological evidence shows they are associated. Although the mechanisms involved in this association are not well established, hypovitaminosis D is associated with insulin resistance, decreased insulin secretion and activation of the renin-angiotensin system, mechanisms involved in the pathophysiology of metabolic syndrome. However, the apparent ineffectiveness of vitamin D supplementation on metabolic syndrome components, as well as the limited information about the effect of improving metabolic syndrome components on vitamin D concentrations, does not clarify the direction and the mechanisms involved in the causal relationship between these 2 pathologies. Overall, because of the high prevalence and the epidemiological association between both diseases, hypovitaminosis D could be considered a component of the metabolic syndrome. Copyright © 2013 Elsevier España, S.L.U. All rights reserved.

Dancing with Down Syndrome: A Phenomenological Case Study

ERIC Educational Resources Information Center

Reinders, Nicole; Bryden, Pamela J.; Fletcher, Paula C.

2015-01-01

"Dance for individuals with Down syndrome has many benefits; however, there is little research on this topic." Down syndrome is the most common "genetic condition," resulting in psychological, physical, and social impairments. There is research to suggest that dance may be a beneficial activity for people with Down syndrome;…

The Landau-Kleffner Syndrome

PubMed Central

Pearl, Phillip L.; Carrazana, Enrique J.; Holmes, Gregory L.

2001-01-01

Landau-Kleffner syndrome (LKS), or acquired epileptiform aphasia, is an epilepsy syndrome involving progressive neuropsychological impairment related to the appearance of paroxysmal electroencephalograph (EEG) activity. LKS appears to share a common pathophysiologic mechanism with continuous spike-wave of sleep (CSWS), acquired epileptic opercular syndrome (AEOS), and even benign childhood epilepsy with centrotemporal spikes (BECTS), with differentiating factors including age of onset, area of primary epileptogenicity, and severity of clinical presentation. This article covers the clinical, diagnostic, therapeutic, and prognostic features of LKS. In a child with autistic spectrum disorder, the presence of a fluctuating clinical course or regression should raise suspicion for the presence of associated epilepsy. PMID:15309183

Sensitivity Gains, Linearity, and Spectral Reproducibility in Nonuniformly Sampled Multidimensional MAS NMR Spectra of High Dynamic Range.

DOE Office of Scientific and Technical Information (OSTI.GOV)

Suiter, Christopher L.; Paramasivam, Sivakumar; Hou, Guangjin

Recently, we have demonstrated that considerable inherent sensitivity gains are attained in MAS NMR spectra acquired by nonuniform sampling (NUS) and introduced maximum entropy interpolation (MINT) processing that assures the linearity of transformation between the time and frequency domains. In this report, we examine the utility of the NUS/MINT approach in multidimensional datasets possessing high dynamic range, such as homonuclear 13C–13C correlation spectra. We demonstrate on model compounds and on 1–73-(U-13C,15N)/74–108-(U-15N) E. coli thioredoxin reassembly, that with appropriately constructed 50 % NUS schedules inherent sensitivity gains of 1.7–2.1-fold are readily reached in such datasets. We show that both linearity andmore » line width are retained under these experimental conditions throughout the entire dynamic range of the signals. Furthermore, we demonstrate that the reproducibility of the peak intensities is excellent in the NUS/MINT approach when experiments are repeated multiple times and identical experimental and processing conditions are employed. Finally, we discuss the principles for design and implementation of random exponentially biased NUS sampling schedules for homonuclear 13C–13C MAS correlation experiments that yield high quality artifact-free datasets.« less

Sensitivity gains, linearity, and spectral reproducibility in nonuniformly sampled multidimensional MAS NMR spectra of high dynamic range

PubMed Central

Suiter, Christopher L.; Paramasivam, Sivakumar; Hou, Guangjin; Sun, Shangjin; Rice, David; Hoch, Jeffrey C.; Rovnyak, David

2014-01-01

Recently, we have demonstrated that considerable inherent sensitivity gains are attained in MAS NMR spectra acquired by nonuniform sampling (NUS) and introduced maximum entropy interpolation (MINT) processing that assures the linearity of transformation between the time and frequency domains. In this report, we examine the utility of the NUS/MINT approach in multidimensional datasets possessing high dynamic range, such as homonuclear 13C–13C correlation spectra. We demonstrate on model compounds and on 1–73-(U-13C, 15N)/74–108-(U-15N) E. coli thioredoxin reassembly, that with appropriately constructed 50 % NUS schedules inherent sensitivity gains of 1.7–2.1-fold are readily reached in such datasets. We show that both linearity and line width are retained under these experimental conditions throughout the entire dynamic range of the signals. Furthermore, we demonstrate that the reproducibility of the peak intensities is excellent in the NUS/MINT approach when experiments are repeated multiple times and identical experimental and processing conditions are employed. Finally, we discuss the principles for design and implementation of random exponentially biased NUS sampling schedules for homonuclear 13C–13C MAS correlation experiments that yield high-quality artifact-free datasets. PMID:24752819

Quantitative rest activity in ambulatory monitoring as a physiological marker of restless legs syndrome: a controlled study.

PubMed

Tuisku, Katinka; Holi, Matti Mikael; Wahlbeck, Kristian; Ahlgren, Aulikki Johanna; Lauerma, Hannu

2003-04-01

An objective marker of restless legs syndrome (RLS) is needed for developing diagnostic tools and monitoring symptoms. Actometric ambulatory monitoring of 15 RLS patients and 15 healthy controls was undertaken in order to differentiate between RLS-related motor symptoms and normal motor activity. Nocturnal lower-limb activity per minute differentiated and discriminated between groups with no overlap, whereas the periodic limb movement index and the controlled rest activity during sitting showed less discriminative power. The naturalistic recording of nocturnal activity by actometry may prove useful for assessing the severity of RLS and for finding an objective marker to support the diagnosis of RLS. Copyright 2002 Movement Disorder Society

Features that exacerbate fatigue severity in joint hypermobility syndrome/Ehlers-Danlos syndrome - hypermobility type.

PubMed

Krahe, Anne Maree; Adams, Roger David; Nicholson, Leslie Lorenda

2018-08-01

To assess the prevalence, severity and impact of fatigue on individuals with joint hypermobility syndrome (JHS)/Ehlers-Danlos syndrome - hypermobility type (EDS-HT) and establish potential determinants of fatigue severity in this population. Questionnaires on symptoms and signs related to fatigue, quality of life, mental health, physical activity participation and sleep quality were completed by people with JHS/EDS-HT recruited through two social media sites. Multiple regression analysis was performed to identify predictors of fatigue in this population. Significant fatigue was reported by 79.5% of the 117 participants. Multiple regression analysis identified five predictors of fatigue severity, four being potentially modifiable, accounting for 52.3% of the variance in reported fatigue scores. Predictors of fatigue severity were: the self-perceived extent of joint hypermobility, orthostatic dizziness related to heat and exercise, levels of participation in personal relationships and community, current levels of physical activity and dissatisfaction with the diagnostic process and management options provided for their condition. Fatigue is a significant symptom associated with JHS/EDS-HT. Assessment of individuals with this condition should include measures of fatigue severity to enable targeted management of potentially modifiable factors associated with fatigue severity. Implications for rehabilitation Fatigue is a significant symptom reported by individuals affected by joint hypermobility syndrome/Ehlers-Danlos syndrome - hypermobility type. Potentially modifiable features that contribute to fatigue severity in this population have been identified. Targeted management of these features may decrease the severity and impact of fatigue in joint hypermobility syndrome/Ehlers-Danlos syndrome - hypermobility type.

A physical activity intervention to treat the frailty syndrome in older persons-results from the LIFE-P study

USDA-ARS?s Scientific Manuscript database

BACKGROUND: The frailty syndrome is as a well-established condition of risk for disability. Aim of the study is to explore whether a physical activity (PA) intervention can reduce prevalence and severity of frailty in a community-dwelling elders at risk of disability. METHODS: Exploratory analyses ...

A randomized controlled trial of qigong exercise on fatigue symptoms, functioning, and telomerase activity in persons with chronic fatigue or chronic fatigue syndrome.

PubMed

Ho, Rainbow T H; Chan, Jessie S M; Wang, Chong-Wen; Lau, Benson W M; So, Kwok Fai; Yuen, Li Ping; Sham, Jonathan S T; Chan, Cecilia L W

2012-10-01

Chronic fatigue is common in the general population. Complementary therapies are often used by patients with chronic fatigue or chronic fatigue syndrome to manage their symptoms. This study aimed to assess the effect of a 4-month qigong intervention program among patients with chronic fatigue or chronic fatigue syndrome. Sixty-four participants were randomly assigned to either an intervention group or a wait list control group. Outcome measures included fatigue symptoms, physical functioning, mental functioning, and telomerase activity. Fatigue symptoms and mental functioning were significantly improved in the qigong group compared to controls. Telomerase activity increased in the qigong group from 0.102 to 0.178 arbitrary units (p < 0.05). The change was statistically significant when compared to the control group (p < 0.05). Qigong exercise may be used as an alternative and complementary therapy or rehabilitative program for chronic fatigue and chronic fatigue syndrome.

Acute Motor-dominant Polyneuropathy as Guillain-Barré Syndrome and Multiple Mononeuropathies in a Patient with Sjögren's Syndrome.

PubMed

Tanaka, Kenichiro; Nakayasu, Hiroyuki; Suto, Yutaka; Takahashi, Shotaro; Konishi, Yoshihiro; Nishimura, Hirotake; Ueno, Rino; Kusunoki, Susumu; Nakashima, Kenji

A patient with xerostomia and xerophthalmia due to Sjögren's syndrome presented with acute motor-dominant polyneuropathy and multiple mononeuropathy with antiganglioside antibodies. Nerve conduction studies and a sural nerve biopsy revealed the neuropathy as a mixture of segmental demyelination and axonal degeneration. Positive results were obtained for several antiganglioside antibodies. Corticosteroid treatment proved effective. The neuropathy was considered to represent a mixture of polyneuropathy as Guillain-Barré syndrome and multiple mononeuropathy via Sjögren's syndrome. We speculate that Guillain-Barré syndrome occurred in the patient and Guillain-Barré syndrome itself activated multiple mononeuropathy via Sjögren's syndrome.

MELAS Syndrome and Kidney Disease Without Fanconi Syndrome or Proteinuria: A Case Report.

PubMed

Rudnicki, Michael; Mayr, Johannes A; Zschocke, Johannes; Antretter, Herwig; Regele, Heinz; Feichtinger, René G; Windpessl, Martin; Mayer, Gert; Pölzl, Gerhard

2016-12-01

Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS syndrome) represents one of the most frequent mitochondrial disorders. The majority of MELAS cases are caused by m.3243A>G mutation in the mitochondrial MT-TL1 gene, which encodes the mitochondrial tRNA Leu(UUR) . Kidney involvement usually manifests as Fanconi syndrome or focal segmental glomerulosclerosis. We describe a patient with MELAS mutation, cardiomyopathy, and chronic kidney disease without Fanconi syndrome, proteinuria, or hematuria. While the patient was waitlisted for heart transplantation, her kidney function deteriorated from an estimated glomerular filtration rate of 33 to 20mL/min/1.73m 2 within several months. Kidney biopsy was performed to distinguish decreased kidney perfusion from intrinsic kidney pathology. Histologic examination of the biopsy specimen showed only a moderate degree of tubular atrophy and interstitial fibrosis, but quantitative analysis of the m.3243A>G mitochondrial DNA mutation revealed high heteroplasmy levels of 89% in the kidney. Functional assessment showed reduced activity of mitochondrial enzymes in kidney tissue, which was confirmed by immunohistology. In conclusion, we describe an unusual case of MELAS syndrome with chronic kidney disease without apparent proteinuria or tubular disorders associated with Fanconi syndrome, but widespread interstitial fibrosis and a high degree of heteroplasmy of the MELAS specific mutation and low mitochondrial activity in the kidney. Copyright © 2016 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.

Recent Updates on Peroxisome Proliferator-Activated Receptor δ Agonists for the Treatment of Metabolic Syndrome.

PubMed

Grewal, Ajmer S; Beniwal, Meenu; Pandita, Deepti; Sekhon, Bhupinder S; Lather, Viney

2016-01-01

Metabolic syndrome is a disorder described by reduced insulin sensitivity, overweight, hyperlipidaemia, high blood pressure and myocardial disorders, mainly due to high fat diet and lack of physical activity. The peroxisome proliferator activated receptors (PPARs) are type II nuclear hormone receptors that regulate a number of processes in living systems, such as metabolism of carbohydrates and fatty acids, growth and differentiation of cell, and inflammatory reactions. Alpha, gamma and delta are the three distinct isoforms of PPAR. The stimulation of PPARδ alters body's energy fuel preference from glucose to fat. The PPARδ isoform is expressed ubiquitously in all tissues, especially in those tissues which involved in metabolism of lipids like adipose tissue, liver, kidney, and muscle. Currently, PPARδ is an emerging therapeutic target for the pharmacological therapy of disorders associated with metabolic syndrome. Several PPARδ selective agonists had been reported in last ten years, many of them had been advanced into the late phase of clinical trials such as Endurobol (GW501516). However, no PPARδ agonists are yet approved for human use. The present work had been planned to cover wide variety of PPARδ agonists reported till now along with their potential role to tackle various metabolic disorders. The present review has been planned to focus mainly the most popular PPARδ agonists.

Neural activity in relation to empirically derived personality syndromes in depression using a psychodynamic fMRI paradigm

PubMed Central

Taubner, Svenja; Wiswede, Daniel; Kessler, Henrik

2013-01-01

Objective: The heterogeneity between patients with depression cannot be captured adequately with existing descriptive systems of diagnosis and neurobiological models of depression. Furthermore, considering the highly individual nature of depression, the application of general stimuli in past research efforts may not capture the essence of the disorder. This study aims to identify subtypes of depression by using empirically derived personality syndromes, and to explore neural correlates of the derived personality syndromes. Materials and Methods: In the present exploratory study, an individually tailored and psychodynamically based functional magnetic resonance imaging paradigm using dysfunctional relationship patterns was presented to 20 chronically depressed patients. Results from the Shedler–Westen Assessment Procedure (SWAP-200) were analyzed by Q-factor analysis to identify clinically relevant subgroups of depression and related brain activation. Results: The principle component analysis of SWAP-200 items from all 20 patients lead to a two-factor solution: “Depressive Personality” and “Emotional-Hostile-Externalizing Personality.” Both factors were used in a whole-brain correlational analysis but only the second factor yielded significant positive correlations in four regions: a large cluster in the right orbitofrontal cortex (OFC), the left ventral striatum, a small cluster in the left temporal pole, and another small cluster in the right middle frontal gyrus. Discussion: The degree to which patients with depression score high on the factor “Emotional-Hostile-Externalizing Personality” correlated with relatively higher activity in three key areas involved in emotion processing, evaluation of reward/punishment, negative cognitions, depressive pathology, and social knowledge (OFC, ventral striatum, temporal pole). Results may contribute to an alternative description of neural correlates of depression showing differential brain activation dependent

Noonan syndrome – a new survey

PubMed Central

Tafazoli, Alireza; Eshraghi, Peyman; Koleti, Zahra Kamel

2016-01-01

Noonan syndrome (NS) is an autosomal dominant disorder with vast heterogeneity in clinical and genetic features. Various symptoms have been reported for this abnormality such as short stature, unusual facial characteristics, congenital heart abnormalities, developmental complications, and an elevated tumor incidence rate. Noonan syndrome shares clinical features with other rare conditions, including LEOPARD syndrome, cardio-facio-cutaneous syndrome, Noonan-like syndrome with loose anagen hair, and Costello syndrome. Germline mutations in the RAS-MAPK (mitogen-activated protein kinase) signal transduction pathway are responsible for NS and other related disorders. Noonan syndrome diagnosis is primarily based on clinical features, but molecular testing should be performed to confirm it in patients. Due to the high number of genes associated with NS and other RASopathy disorders, next-generation sequencing is the best choice for diagnostic testing. Patients with NS also have higher risk for leukemia and specific solid tumors. Age-specific guidelines for the management of NS are available. PMID:28144274

38 CFR 4.88a - Chronic fatigue syndrome.

Code of Federal Regulations, 2012 CFR

2012-07-01

... 38 Pensions, Bonuses, and Veterans' Relief 1 2012-07-01 2012-07-01 false Chronic fatigue syndrome... Deficiencies § 4.88a Chronic fatigue syndrome. (a) For VA purposes, the diagnosis of chronic fatigue syndrome requires: (1) new onset of debilitating fatigue severe enough to reduce daily activity to less than 50...

38 CFR 4.88a - Chronic fatigue syndrome.

Code of Federal Regulations, 2013 CFR

2013-07-01

... 38 Pensions, Bonuses, and Veterans' Relief 1 2013-07-01 2013-07-01 false Chronic fatigue syndrome... Deficiencies § 4.88a Chronic fatigue syndrome. (a) For VA purposes, the diagnosis of chronic fatigue syndrome requires: (1) new onset of debilitating fatigue severe enough to reduce daily activity to less than 50...

38 CFR 4.88a - Chronic fatigue syndrome.

Code of Federal Regulations, 2014 CFR

2014-07-01

... 38 Pensions, Bonuses, and Veterans' Relief 1 2014-07-01 2014-07-01 false Chronic fatigue syndrome... Deficiencies § 4.88a Chronic fatigue syndrome. (a) For VA purposes, the diagnosis of chronic fatigue syndrome requires: (1) new onset of debilitating fatigue severe enough to reduce daily activity to less than 50...

Experimental aspects in acquisition of wide bandwidth solid-state MAS NMR spectra of low-γ nuclei with different opportunities on two commercial NMR spectrometers

NASA Astrophysics Data System (ADS)

Jakobsen, Hans J.; Bildsøe, Henrik; Gan, Zhehong; Brey, William W.

2011-08-01

The acquisition and different appearances observed for wide bandwidth solid-state MAS NMR spectra of low-γ nuclei, using 14N as an illustrative nucleus and employing two different commercial spectrometers (Varian, 14.1 T and Bruker, 19.6 T), have been compared/evaluated and optimized from an experimental NMR and an electronic engineering point of view, to account for the huge differences in these spectra. The large differences in their spectral appearances, employing the recommended/standard experimental set-up for the two different spectrometers, are shown to be associated with quite large differences in the electronic design of the two types of preamplifiers, which are connected to their respective probes through a 50 Ω cable, and are here completely accounted for. This has led to different opportunities for optimum performances in the acquisition of nearly ideal wide bandwidth spectra for low-γ nuclei on the two spectrometers by careful evaluation of the length for the 50 Ω probe-to-preamp cable for the Varian system and appropriate changes to the bandwidth ( Q) of the NMR probe used on the Bruker spectrometer. Earlier, we reported quite distorted spectra obtained with Varian Unity INOVA spectrometers (at 11.4 and 14.1 T) in several exploratory wide bandwidth 14N MAS NMR studies of inorganic nitrates and amino acids. These spectra have now been compared/evaluated with fully analyzed 14N MAS spectra correspondingly acquired at 19.6 T on a Bruker spectrometer. It is shown that our upgraded version of the STARS simulation/iterative-fitting software is capable of providing identical sets for the molecular spectral parameters and corresponding fits to the experimental spectra, which fully agree with the electronic measurements, despite the highly different appearances for the MAS NMR spectra acquired on the Varian and Bruker spectrometers.

Structural brain abnormalities in Cushing's syndrome.

PubMed

Bauduin, Stephanie E E C; van der Wee, Nic J A; van der Werff, Steven J A

2018-05-08

Alongside various physical symptoms, patients with Cushing's disease and Cushing's syndrome display a wide variety of neuropsychiatric and cognitive symptoms, which are indicative of involvement of the central nervous system. The aim of this review is to provide an overview of the structural brain abnormalities that are associated with Cushing's disease and Cushing's syndrome and their relation to behavioral and cognitive symptomatology. In this review, we discuss the gray matter structural abnormalities found in patients with active Cushing's disease and Cushing's syndrome, the reversibility and persistence of these changes and the white matter structural changes related to Cushing's syndrome. Recent findings are of particular interest because they provide more detailed information on localization of the structural changes as well as possible insights into the underlying biological processes. Active Cushing's disease and Cushing's syndrome is related to volume reductions of the hippocampus and in a prefrontal region involving the anterior cingulate cortex (ACC) and medial frontal gyrus (MFG). Whilst there are indications that the reductions in hippocampal volume are partially reversible, the changes in the ACC and MFG appear to be more persistent. In contrast to the volumetric findings, changes in white matter connectivity are typically widespread involving multiple tracts.

Pseudo Gitelman Syndrome Associated With Pregnancy.

PubMed

Yoshihara, Masato; Sayo, Akira; Mayama, Michinori; Oguchi, Hidenori

2015-10-01

Gitelman syndrome is a rare inherited renal tubulopathy associated with metabolic alkalosis and electrolyte disorders. Pseudo Gitelman syndrome presents with the same clinical characteristics as Gitelman syndrome, yet without genetic mutations in SLC12A3. A 32-year-old woman with no remarkable medical and family history developed hypokalemia at 32 weeks of gestation. Laboratory findings were consistent with Gitelman syndrome and potassium supplementation was initiated. The patient delivered a healthy neonate at 40 weeks of gestation and the electrolyte disorders drastically improved. After delivery, genomic analysis revealed no evidence of mutations in SLC12A3, and pseudo Gitelman syndrome was finally diagnosed. Pseudo Gitelman syndrome, presenting with Gitelman syndrome-like renal tubulopathy without mutations in SLC12A3, can cause a temporary electrolyte imbalance based on the physiologic changes of pregnancy. Although pregnant women with isolated hypokalemia need not be evaluated for Gitelman or pseudo Gitelman syndrome, if it is accompanied by metabolic alkalosis, hypocalciuria, hypomagnesia, and activation of the renin-angiotensin-aldosterone system without hypertension, this evaluation should be considered.

Antiphospholipid antibody syndrome complicated by Grave's disease.

PubMed

Takahashi, Ayumi; Tamura, Atsushi; Ishikawa, Osamu

2002-12-01

The report describes a woman with primary antiphospholipid antibody syndrome complicated with Grave's disease. Developing symptoms included a small cutaneous nodule on her finger and subsequently ecchymotic purpura on the cheeks, ears, buttocks and lower legs. Histological examinations showed thrombosed vessels in the dermis without or with hemorrhage, respectively. Laboratory investigation revealed positive lupus anticoagulant and immunogenic hyperthyroidism due to Grave's disease. There is a close relationship between the cutaneous manifestation of antiphospholipid antibody syndrome and the activities of Grave's disease and a possible link of antiphospholipid antibody syndrome with Grave's disease was suggested both by the etiology of the disease as well as the disease activity.