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Maternal diet influences breast milk nutritional profile; however, it is unclear which nutrients and contaminants are particularly responsive to short- and long-term changes in maternal intake, and the impact of specific exclusion diets, such as vegan or vegetarian. This study systematically reviewed the literature on the effects of maternal nutrient intake, including exclusion diets, on both the nutrient and contaminant content of breast milk. The electronic databases, PubMed, CENTRAL, Web of Science and CINALH were systematically searched until 4 June 2023, with additionally searches of reference lists (PROSPERO, CRD42020221577). The quality of the studies was examined using Cochrane Risk of Bias tool and Newcastle-Ottawa scale. Eighty-eight studies (n 6577) met the search criteria. Due to high heterogeneity, meta-analysis was not possible. There was strong evidence of response to maternal intakes for DHA and EPA, vitamins A, E and K, iodine and Se in breast milk composition, some evidence of response for α-linolenic acid, B vitamins, vitamin C and D, ovalbumin, tyrosine and contaminants, and insufficient evidence to identify the effects arachidonic acid, Cu, Fe, Zn and choline. The paucity of evidence and high heterogeneity among studies reflects the need for more high-quality trials. However, this review identified the importance of maternal intake in the nutritional content of breast milk for a wide range of nutrients and supports the recommendation for supplementation of DHA and vitamin B12 for those on restrictive diets.
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Lactação , Leite Humano , Humanos , Feminino , Lactação/fisiologia , Vitaminas , Dieta , Ingestão de AlimentosRESUMO
Plate tectonics successfully describes the surface of Earth as a mosaic of moving lithospheric plates. But it is not clear what happens at the base of the plates, the lithosphere-asthenosphere boundary (LAB). The LAB has been well imaged with converted teleseismic waves, whose 10-40-kilometre wavelength controls the structural resolution. Here we use explosion-generated seismic waves (of about 0.5-kilometre wavelength) to form a high-resolution image for the base of an oceanic plate that is subducting beneath North Island, New Zealand. Our 80-kilometre-wide image is based on P-wave reflections and shows an approximately 15° dipping, abrupt, seismic wave-speed transition (less than 1 kilometre thick) at a depth of about 100 kilometres. The boundary is parallel to the top of the plate and seismic attributes indicate a P-wave speed decrease of at least 8 ± 3 per cent across it. A parallel reflection event approximately 10 kilometres deeper shows that the decrease in P-wave speed is confined to a channel at the base of the plate, which we interpret as a sheared zone of ponded partial melts or volatiles. This is independent, high-resolution evidence for a low-viscosity channel at the LAB that decouples plates from mantle flow beneath, and allows plate tectonics to work.
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We present experimental results from the first systematic study of performance scaling with drive parameters for a magnetoinertial fusion concept. In magnetized liner inertial fusion experiments, the burn-averaged ion temperature doubles to 3.1 keV and the primary deuterium-deuterium neutron yield increases by more than an order of magnitude to 1.1×10^{13} (2 kJ deuterium-tritium equivalent) through a simultaneous increase in the applied magnetic field (from 10.4 to 15.9 T), laser preheat energy (from 0.46 to 1.2 kJ), and current coupling (from 16 to 20 MA). Individual parametric scans of the initial magnetic field and laser preheat energy show the expected trends, demonstrating the importance of magnetic insulation and the impact of the Nernst effect for this concept. A drive-current scan shows that present experiments operate close to the point where implosion stability is a limiting factor in performance, demonstrating the need to raise fuel pressure as drive current is increased. Simulations that capture these experimental trends indicate that another order of magnitude increase in yield on the Z facility is possible with additional increases of input parameters.
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Síndrome de Bloom , Nanismo , Sequenciamento do Exoma/métodos , Hormônio do Crescimento/efeitos adversos , RecQ Helicases/genética , Síndrome de Bloom/diagnóstico , Síndrome de Bloom/genética , Síndrome de Bloom/fisiopatologia , Síndrome de Bloom/terapia , Braquidactilia/diagnóstico , Braquidactilia/genética , Pré-Escolar , Contraindicações de Medicamentos , Diagnóstico Diferencial , Nanismo/diagnóstico , Nanismo/tratamento farmacológico , Nanismo/genética , Feminino , Estudos de Associação Genética , Gráficos de Crescimento , Hormônio do Crescimento/uso terapêutico , Homozigoto , Humanos , Micrognatismo/diagnóstico , Micrognatismo/genética , Neoplasias/etiologia , Neoplasias/prevenção & controle , Comportamento de Redução do Risco , Anormalidades da Pele/diagnóstico , Anormalidades da Pele/genéticaRESUMO
BACKGROUND: Degenerative aortic stenosis is the most common valvular heart disease in the elderly, and many patients are not suitable for aortic valve replacement surgery. Transcatheter aortic valve implantation (TAVI) is a new therapeutic option for selected patients at high risk for surgery. AIM: To evaluate the safety and efficacy of TAVI in Australian patients. METHODS: This is a prospective study of patients undergoing TAVI for severe symptomatic aortic stenosis at The Prince Charles Hospital, Brisbane, Australia between August 2008 and July 2013. Patients were at high risk of surgical aortic valve replacement, or inoperable, as deemed by a multidisciplinary 'heart team'. Outcomes include procedural success and complications, 30-day and 1-year mortality and stroke, combined end-points as outlined by the Valve Academic Research Consortium 2 consensus document. RESULTS: Two hundred and nine patients underwent TAVI during the study period. The mean age was 83.7 ± 6.7 years, and 101 (48%) were men. The valve systems utilised were as follows: Edwards-SAPIEN valve in 104 (49.5%), Medtronic CoreValve in 86 (41.2%) and Boston Scientific Lotus valve in 19 (9.3%) patients. Thirty-day and 1-year mortality rates were 5.7% and 11.5% respectively. Thirty-day and 1-year stroke rates were 4.3% and 6.2% respectively. The composite end-points of device success, early safety and clinical efficacy occurred in 80.4%, 27.3% and 68.4%. CONCLUSIONS: TAVI with various valve systems, delivered through several approaches, is feasible in high surgical risk and inoperable patients with severe aortic stenosis, with acceptable outcomes at short-term and intermediate-term follow up.
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Estenose da Valva Aórtica/mortalidade , Estenose da Valva Aórtica/cirurgia , Substituição da Valva Aórtica Transcateter/mortalidade , Substituição da Valva Aórtica Transcateter/tendências , Idoso , Idoso de 80 Anos ou mais , Estenose da Valva Aórtica/diagnóstico , Austrália/epidemiologia , Estudos de Coortes , Feminino , Seguimentos , Implante de Prótese de Valva Cardíaca/mortalidade , Implante de Prótese de Valva Cardíaca/tendências , Humanos , Masculino , Mortalidade/tendências , Seleção de Pacientes , Estudos Prospectivos , Fatores de Risco , Resultado do TratamentoRESUMO
Site-specific and accurate prediction of daily minimum air and grass temperatures, made available online several hours before their occurrence, would be of significant benefit to several economic sectors and for planning human activities. Site-specific and reasonably accurate nowcasts of daily minimum temperature several hours before its occurrence, using measured sub-hourly temperatures hours earlier in the morning as model inputs, was investigated. Various temperature models were tested for their ability to accurately nowcast daily minimum temperatures 2 or 4 h before sunrise. Temperature datasets used for the model nowcasts included sub-hourly grass and grass-surface (infrared) temperatures from one location in South Africa and air temperature from four subtropical sites varying in altitude (USA and South Africa) and from one site in central sub-Saharan Africa. Nowcast models used employed either exponential or square root functions to describe the rate of nighttime temperature decrease but inverted so as to determine the minimum temperature. The models were also applied in near real-time using an open web-based system to display the nowcasts. Extrapolation algorithms for the site-specific nowcasts were also implemented in a datalogger in an innovative and mathematically consistent manner. Comparison of model 1 (exponential) nowcasts vs measured daily minima air temperatures yielded root mean square errors (RMSEs) <1 °C for the 2-h ahead nowcasts. Model 2 (also exponential), for which a constant model coefficient (b = 2.2) was used, was usually slightly less accurate but still with RMSEs <1 °C. Use of model 3 (square root) yielded increased RMSEs for the 2-h ahead comparisons between nowcasted and measured daily minima air temperature, increasing to 1.4 °C for some sites. For all sites for all models, the comparisons for the 4-h ahead air temperature nowcasts generally yielded increased RMSEs, <2.1 °C. Comparisons for all model nowcasts of the daily grass and grass-surface minima yielded increased RMSEs compared to those for air temperature at 2 m. The sufficiently small RMSEs using the 2-h ahead nowcasts of the air temperature minimum, for the exponential model, demonstrate that the methodology used may be applied operationally but with increased errors for grass minimum temperature and the 4-h nowcasts.
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Modelos Teóricos , Temperatura , Florida , Poaceae , África do SulRESUMO
PURPOSE: The IGFALS gene encodes the acid-labile subunit (ALS) protein, which regulates circulating IGF-1. Human IGFALS mutations cause growth hormone insensitivity (GHI) associated with ALS, IGF-1 and IGFBP-3 deficiencies and mild to moderate postnatal growth impairment (height SDS -2 to -4). Prenatal growth impairment is not a recognised feature of this disorder, but heterozygous carriers may show an intermediate phenotype. METHODS: We report a family of five subjects, including three children born small for gestational age, who were investigated for IGFALS gene mutations. RESULTS: The proband, an 8.7 years female with pre- and postnatal growth failure (BW SDS -3.04, Ht SDS -3.86) and biochemical features of GHI, had a homozygous mutation of IGFALS, c.401T>A; p.L134Q. Her 6.1 years brother (BW SDS -2.11, Ht SDS -2.0) had the same homozygous IGFALS mutation. Both parents [adult height SDS -1.76 (father) and -1.82 (mother)] and her 2.7 years sister (BW SDS -2.60, Ht SDS -2.04) were heterozygous for the IGFALS mutation. CONCLUSION: Significant phenotypic heterogeneity was observed between family members, in particular varying degrees of prenatal growth retardation were present in the three siblings, which may have contributed to the variation in the postnatal growth phenotype.
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Proteínas de Transporte/genética , Glicoproteínas/genética , Transtornos do Crescimento , Recém-Nascido Pequeno para a Idade Gestacional , Síndrome de Laron , Adulto , Criança , Pré-Escolar , Feminino , Retardo do Crescimento Fetal/genética , Retardo do Crescimento Fetal/metabolismo , Retardo do Crescimento Fetal/patologia , Transtornos do Crescimento/genética , Transtornos do Crescimento/metabolismo , Transtornos do Crescimento/patologia , Humanos , Síndrome de Laron/genética , Síndrome de Laron/metabolismo , Síndrome de Laron/patologia , Masculino , Núcleo Familiar , FenótipoRESUMO
We present the results of lattice QCD calculations of the magnetic moments of the lightest nuclei, the deuteron, the triton, and ^{3}He, along with those of the neutron and proton. These calculations, performed at quark masses corresponding to m_{π}â¼800 MeV, reveal that the structure of these nuclei at unphysically heavy quark masses closely resembles that at the physical quark masses. In particular, we find that the magnetic moment of ^{3}He differs only slightly from that of a free neutron, as is the case in nature, indicating that the shell-model configuration of two spin-paired protons and a valence neutron captures its dominant structure. Similarly a shell-model-like moment is found for the triton, µ_{^{3}H}â¼µ_{p}. The deuteron magnetic moment is found to be equal to the nucleon isoscalar moment within the uncertainties of the calculations. Furthermore, deviations from the Schmidt limits are also found to be similar to those in nature for these nuclei. These findings suggest that at least some nuclei at these unphysical quark masses are describable by a phenomenological nuclear shell model.
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This Letter presents results from the first fully integrated experiments testing the magnetized liner inertial fusion concept [S. A. Slutz et al., Phys. Plasmas 17, 056303 (2010)], in which a cylinder of deuterium gas with a preimposed 10 Taxial magnetic field is heated by Z beamlet, a 2.5 kJ, 1 TW laser, and magnetically imploded by a 19 MA, 100 ns rise time current on the Z facility. Despite a predicted peak implosion velocity of only 70 km = s, the fuel reaches a stagnation temperature of approximately 3 keV, with T(e) ≈ T(i), and produces up to 2 x 10(12) thermonuclear deuterium-deuterium neutrons. X-ray emission indicates a hot fuel region with full width at half maximum ranging from 60 to 120 µm over a 6 mm height and lasting approximately 2 ns. Greater than 10(10) secondary deuterium-tritium neutrons were observed, indicating significant fuel magnetization given that the estimated radial areal density of the plasma is only 2 mg = cm(2).
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Magnetizing the fuel in inertial confinement fusion relaxes ignition requirements by reducing thermal conductivity and changing the physics of burn product confinement. Diagnosing the level of fuel magnetization during burn is critical to understanding target performance in magneto-inertial fusion (MIF) implosions. In pure deuterium fusion plasma, 1.01 MeV tritons are emitted during deuterium-deuterium fusion and can undergo secondary deuterium-tritium reactions before exiting the fuel. Increasing the fuel magnetization elongates the path lengths through the fuel of some of the tritons, enhancing their probability of reaction. Based on this feature, a method to diagnose fuel magnetization using the ratio of overall deuterium-tritium to deuterium-deuterium neutron yields is developed. Analysis of anisotropies in the secondary neutron energy spectra further constrain the measurement. Secondary reactions also are shown to provide an upper bound for the volumetric fuel-pusher mix in MIF. The analysis is applied to recent MIF experiments [M. R. Gomez et al., Phys. Rev. Lett. 113, 155003 (2014)] on the Z Pulsed Power Facility, indicating that significant magnetic confinement of charged burn products was achieved and suggesting a relatively low-mix environment. Both of these are essential features of future ignition-scale MIF designs.
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INTRODUCTION: The incidence of STEMI and subsequent mortality has been reported to be higher in Indian populations compared to developed countries. However, there is limited data directly comparing contemporary primary percutaneous coronary intervention (pPCI) treatment strategies and clinical outcomes for STEMI patients between developed and developing countries. MATERIALS AND METHODS: We compared population demographics, procedural characteristics, times to reperfusion and mortality in STEMI patients treated with pPCI between two tertiary referral centers in India and Australia respectively over a 3-year period (1st Jan 2017-31st Dec 2019). RESULTS: A total of 1293 STEMI presentations (896 Indian vs 397 Australian) were included. On average, Indian patients had lower median BMI than Australian patients (BMI 25.4 vs 27.8; p < 0.001), were significantly younger (mean age 56.0 vs 63.2 years; p < 0.001), more likely male (84 % vs 80 %; p = 0.046) and diabetic (48 % vs 18 %); p < 0.001). Radial access (50 % vs 88 %; p < 0.001) and TIMI III flow post PCI was also significantly lower (85 % vs 96 %; p < 0.001) with median door-to-balloon time significantly shorter in the Indian cohort (20mins vs 43mins; p < 0.001); however, median symptom to balloon time was significantly longer (245mins vs 160mins; p < 0.001). No significant differences in 30-day mortality (4.0 % vs 2.8 % Australian; p = 0.209) or 1-year mortality (6.5 % vs 4.3 %; p = 0.120) were observed. CONCLUSION: Significant differences in demographics and presentation characteristics exist between Indian and Australian STEMI patients treated with pPCI. Indian patients had significantly longer pre-hospital delays and lower achievement of TIMI III flow post PCI, yet shorter in-hospital time to treatment.
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Intervenção Coronária Percutânea , Infarto do Miocárdio com Supradesnível do Segmento ST , Humanos , Infarto do Miocárdio com Supradesnível do Segmento ST/cirurgia , Infarto do Miocárdio com Supradesnível do Segmento ST/terapia , Infarto do Miocárdio com Supradesnível do Segmento ST/epidemiologia , Intervenção Coronária Percutânea/métodos , Masculino , Feminino , Pessoa de Meia-Idade , Índia/epidemiologia , Austrália/epidemiologia , Resultado do Tratamento , Estudos Retrospectivos , Taxa de Sobrevida/tendências , Incidência , Seguimentos , Mortalidade Hospitalar/tendências , Fatores de Risco , Tempo para o TratamentoRESUMO
Novel experimental data are reported that reveal helical instability formation on imploding z-pinch liners that are premagnetized with an axial field. Such instabilities differ dramatically from the mostly azimuthally symmetric instabilities that form on unmagnetized liners. The helical structure persists at nearly constant pitch as the liner implodes. This is surprising since, at the liner surface, the azimuthal drive field presumably dwarfs the axial field for all but the earliest stages of the experiment. These fundamentally 3D results provide a unique and challenging test for 3D-magnetohydrodynamics simulations.
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The low-energy nΣ(-) interactions determine, in part, the role of the strange quark in dense matter, such as that found in astrophysical environments. The scattering phase shifts for this system are obtained from a numerical evaluation of the QCD path integral using the technique of lattice QCD. Our calculations, performed at a pion mass of m(π)~389 MeV in two large lattice volumes and at one lattice spacing, are extrapolated to the physical pion mass using effective field theory. The interactions determined from lattice QCD are consistent with those extracted from hyperon-nucleon experimental data within uncertainties and strengthen model-dependent theoretical arguments that the strange quark is a crucial component of dense nuclear matter.
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The implosions of initially solid beryllium liners (tubes) have been imaged with penetrating radiography through to stagnation. These novel radiographic data reveal a high degree of azimuthal correlation in the evolving magneto-Rayleigh-Taylor structure at times just prior to (and during) stagnation, providing stringent constraints on the simulation tools used by the broader high energy density physics and inertial confinement fusion communities. To emphasize this point, comparisons to 2D and 3D radiation magnetohydrodynamics simulations are also presented. Both agreement and substantial disagreement have been found, depending on how the liner's initial outer surface finish was modeled. The various models tested, and the physical implications of these models are discussed. These comparisons exemplify the importance of the experimental data obtained.
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BACKGROUND: Takotsubo cardiomyopathy (TC) is increasingly recognised in patients presenting with features of acute coronary syndrome. We present a single centre experience of TC with medium term follow up. METHODS: Fifty-two consecutive patients presenting with a diagnosis of TC were included. The clinical presentation, complications, baseline and follow-up echocardiograms and cardiac magnetic resonance imaging were analysed. RESULTS: Fifty-one patients were female. A stressful event preceded presentation in 37 (71%) patients. Chest pain was the most common symptom (83%). Two patients presented with an out-of-hospital cardiac arrest. ST segment elevation (40%) and global T wave inversion (44%) were the most frequent electrocardiogram changes. Left ventricular assessment demonstrated typical apical ballooning in 41 patients and 11 patients demonstrated the mid-wall variant. In-hospital complications occurred in 11 patients (21%) and included acute pulmonary oedema (n = 2), cardiogenic shock (n = 5); two of whom had a significant left ventricular outflow gradient, atrial fibrillation (n = 1), left ventricular thrombus (n = 2) and a cerebrovascular event (n = 2). Left ventricular function at presentation and follow up was compared in 40 patients. The mean ejection fraction in this group at presentation was 47% (20-70%) compared with that at follow up of 63% (44-76%). There were no significant complications or recurrences at follow up. CONCLUSIONS: While TC is a reversible condition with low rates of complications and recurrence at follow up it is, as demonstrated in our cohort, associated with significant in-hospital morbidity in a proportion of patients.
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Dor no Peito/etiologia , Cardiomiopatia de Takotsubo/epidemiologia , Síndrome Coronariana Aguda/diagnóstico , Adulto , Idoso , Idoso de 80 Anos ou mais , Fibrilação Atrial/epidemiologia , Fibrilação Atrial/etiologia , Cateterismo Cardíaco , Fármacos Cardiovasculares/uso terapêutico , Diagnóstico Diferencial , Eletrocardiografia , Feminino , Seguimentos , Parada Cardíaca/etiologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Inibidores da Agregação Plaquetária/uso terapêutico , Edema Pulmonar/epidemiologia , Edema Pulmonar/etiologia , Queensland/epidemiologia , Choque Cardiogênico/epidemiologia , Choque Cardiogênico/etiologia , Choque Cardiogênico/terapia , Estresse Psicológico/complicações , Volume Sistólico , Cardiomiopatia de Takotsubo/complicações , Cardiomiopatia de Takotsubo/diagnóstico , Cardiomiopatia de Takotsubo/diagnóstico por imagem , Cardiomiopatia de Takotsubo/tratamento farmacológico , Cardiomiopatia de Takotsubo/fisiopatologia , Ultrassonografia , Disfunção Ventricular Esquerda/epidemiologia , Disfunção Ventricular Esquerda/etiologiaRESUMO
We present evidence for the existence of a bound H dibaryon, an I=0, J=0, s=-2 state with valence quark structure uuddss, at a pion mass of m(π)â¼389 MeV. Using the results of lattice QCD calculations performed on four ensembles of anisotropic clover gauge-field configurations, with spatial extents of Lâ¼2.0, 2.5, 3.0, and 3.9 fm at a spatial lattice spacing of b(s)â¼0.123 fm, we find an H dibaryon bound by B(∞)(H)=16.6±2.1±4.6 MeV at a pion mass of m(π)â¼389 MeV.
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The Joint British Diabetes Societies guidelines for the management of diabetic ketoacidosis (these do not cover Hyperosmolar Hyperglycaemic Syndrome) are available in full at: (i) http://www.diabetes.org.uk/About_us/Our_Views/Care_recommendations/The-Management-of-Diabetic-Ketoacidosis-in-Adults; (ii) http://www.diabetes.nhs.uk/publications_and_resources/reports_and_guidance; (iii) http://www.diabetologists-abcd.org.uk/JBDS_DKA_Management.pdf. This article summarizes the main changes from previous guidelines and discusses the rationale for the new recommendations. The key points are: Monitoring of the response to treatment (i) The method of choice for monitoring the response to treatment is bedside measurement of capillary blood ketones using a ketone meter. (ii) If blood ketone measurement is not available, venous pH and bicarbonate should be used in conjunction with bedside blood glucose monitoring to assess treatment response. (iii) Venous blood should be used rather than arterial (unless respiratory problems dictate otherwise) in blood gas analysers. (iv) Intermittent laboratory confirmation of pH, bicarbonate and electrolytes only. Insulin administration (i) Insulin should be infused intravenously at a weight-based fixed rate until the ketosis has resolved. (ii) When the blood glucose falls below 14 mmol/l, 10% glucose should be added to allow the fixed-rate insulin to be continued. (iii) If already taking, long-acting insulin analogues such as insulin glargine (Lantus(®), Sanofi Aventis, Guildford, Surry, UK) or insulin detemir (Levemir(®), Novo Nordisk, Crawley, West Sussex, UK.) should be continued in usual doses. Delivery of care (i) The diabetes specialist team should be involved as soon as possible. (ii) Patients should be nursed in areas where staff are experienced in the management of ketoacidosis.
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Glicemia/metabolismo , Diabetes Mellitus Tipo 1/diagnóstico , Cetoacidose Diabética/diagnóstico , Cetoacidose Diabética/tratamento farmacológico , Hipoglicemiantes/uso terapêutico , Insulina/uso terapêutico , Peso Corporal , Diabetes Mellitus Tipo 1/tratamento farmacológico , Diabetes Mellitus Tipo 1/epidemiologia , Cetoacidose Diabética/epidemiologia , Gerenciamento Clínico , Humanos , Injeções Subcutâneas , Cetonas/sangue , Reino Unido/epidemiologiaRESUMO
The impact of disordered mineral and bone metabolism following kidney transplantation is not well defined. We studied the association of serum phosphate and calcium concentrations, and surrogate measures of arterial stiffness (augmentation index: AIx and Timing of the reflected wave: Tr), with long-term kidney transplant recipient and allograft survival. Prevalent adult renal transplant patients (n = 270) were prospectively studied over a median 88-month follow-up. Detailed demographic, clinical and laboratory data, in addition to both peripheral and central non-invasive blood pressure measurements, were recorded. Higher serum phosphate and calcium levels were associated with increased all-cause mortality (HR: 1.21; 95% CI 1.09,1.35, p < 0.001 and HR: 1.22; 95% CI 1.01,1.48; p < 0.04, respectively; adjusted Cox model) and death-uncensored graft loss (p < 0.001 and p = 0.03, respectively). In addition, serum calcium and phosphate were associated with death-censored graft loss on univariable analysis (p < 0.001 and p = 0.02, respectively), but did not retain significance on multivariable analysis. AIx and Tr were not associated with mortality or graft loss on multivariable analysis. This is the first report to demonstrate that both higher serum phosphate and calcium levels are associated with increased mortality in kidney transplant recipients. It highlights the need for randomized trials assessing current interventions available for improving disordered mineral-bone metabolism post transplantation.
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Cálcio/sangue , Rejeição de Enxerto/mortalidade , Falência Renal Crônica/sangue , Falência Renal Crônica/mortalidade , Transplante de Rim/mortalidade , Fosfatos/sangue , Adulto , Estudos de Coortes , Feminino , Seguimentos , Rejeição de Enxerto/etiologia , Humanos , Transplante de Rim/efeitos adversos , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Prospectivos , Fatores de Risco , Taxa de SobrevidaRESUMO
Fifteen children and adolescents (4 male) with a median age of 5.4 yr (range 1.2 -13.6 yr) were entered into a screening protocol to identify lesions of von Hippel-Lindau (VHL) disease. Fourteen had an affected first-degree relative and one had a previous VHL lesion. Screening during the period of 2000 to 2008 followed published guidelines and consisted of measurement of urinary catecholamines, adrenal and renal imaging and ophthalmological and central nervous system examinations and imaging. Screening identified 8 VHL lesions in 6 asymptomatic patients with confirmed genetic mutations. Five patients had elevated urinary noradrenaline excretion and in each case the presence of a pheochromocytoma was identified on adrenal magnetic resonance imagin scan. In one patient a left-sided tumor was identified 1 yr after a right-sided tumor had been removed. In a sixth patient a retinal capillary hemangioma and a cerebellar hemangioblastoma were identified. Patient compliance with the screening protocol was variable reflecting its time-intensive nature. A formal screening programme for this at-risk population of pediatric patients, despite being intensive, can identify VHL lesions during a pre-morbid phase and may thus have a beneficial impact on prognosis in this serious disorder.
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Programas de Rastreamento , Cooperação do Paciente , Doença de von Hippel-Lindau/diagnóstico , Doença de von Hippel-Lindau/patologia , Adolescente , Neoplasias das Glândulas Suprarrenais/diagnóstico , Neoplasias das Glândulas Suprarrenais/genética , Neoplasias das Glândulas Suprarrenais/patologia , Neoplasias das Glândulas Suprarrenais/cirurgia , Catecolaminas/urina , Neoplasias Cerebelares/diagnóstico , Neoplasias Cerebelares/genética , Neoplasias Cerebelares/patologia , Neoplasias Cerebelares/cirurgia , Criança , Pré-Escolar , Feminino , Hemangioma/diagnóstico , Hemangioma/genética , Hemangioma/patologia , Hemangioma/cirurgia , Humanos , Lactente , Neoplasias Renais/diagnóstico , Neoplasias Renais/genética , Neoplasias Renais/patologia , Neoplasias Renais/cirurgia , Masculino , Feocromocitoma/diagnóstico , Feocromocitoma/genética , Feocromocitoma/patologia , Feocromocitoma/cirurgia , Neoplasias da Retina/diagnóstico , Neoplasias da Retina/genética , Neoplasias da Retina/patologia , Neoplasias da Retina/cirurgia , Estudos Retrospectivos , Resultado do Tratamento , Doença de von Hippel-Lindau/genética , Doença de von Hippel-Lindau/cirurgiaRESUMO
The first controlled experiments measuring the growth of the magneto-Rayleigh-Taylor instability in fast (â¼100 ns) Z-pinch plasmas are reported. Sinusoidal perturbations on the surface of an initially solid Al tube (liner) with wavelengths of 25-400 µm were used to seed the instability. Radiographs with 15 µm resolution captured the evolution of the outer liner surface. Comparisons with numerical radiation magnetohydrodynamic simulations show remarkably good agreement down to 50 µm wavelengths.