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Function Data (function + data)
Selected AbstractsLow Serum Biotinidase Activity in Children with Valproic Acid MonotherapyEPILEPSIA, Issue 10 2001K. H. Schulpis Summary: ,Purpose: Valproic acid (VPA) is an effective antiepileptic drug (AED), which is associated with dose-related adverse reactions such as skin rash, hair loss (alopecia), etc. Profound as well as partial biotinidase deficiency causes dermatologic manifestations similar these. Therefore, it was of interest to evaluate serum biotinidase activity in patients receiving VPA monotherapy. Methods: Seventy-five patients with seizures, mean age, 8.6 years (±1.9 years) were divided into three groups. Group A (n = 25) was treated with VPA 28.7 ± 8.5 mg/kg/24 h, group B (n = 25) with 41.6 ± 4.9 mg/kg/24 h, and group C with 54.5 ± 5.8 mg/kg/24 h. Their "trough" VPA serum levels were 40.9 ± 13.2, 86.25 ± 11.5, and 137 ± 14.5 ,g/ml, respectively. Fifty healthy children were the controls. Patients and controls underwent clinical and laboratory evaluations including liver function data, complete blood counts, NH3, and so on, after 45 days of VPA treatment. Biotinidase serum levels were evaluated fluorometrically. Results: Liver function data were found elevated in the groups B and C. On the contrary, biotinidase activity was significantly statistically lowered (p < 0.001) in groups B and C (1.22 ± 1.11, 0.97 ± 0.07 mmol/min/L respectively), as compared with controls (5.20 ± 0.90 mmol/min/L). Strong inverse correlations were observed between liver enzymes and VPA blood levels with the activity of the enzyme. Additionally, no inhibitory effect on biotinidase activity was found, when the enzyme was incubated in vitro with high (1.2 mM) concentrations of the drug. Skin lesions (seborrheic rash, alopecia) were improved in our patients after biotin (10 mg/day) supplementation. Conclusions: It is suggested that VPA impairs the liver mitochondrial function, resulting in a low biotinidase activity and or biotin deficiency. Biotin supplementation could restore some of the side effects of the drug. [source] Molecular physiology of SLC4 anion exchangersEXPERIMENTAL PHYSIOLOGY, Issue 1 2006Seth L. Alper Plasmalemmal Cl,,HCO3, exchangers regulate intracellular pH and [Cl,] and cell volume. In polarized epithelial cells, they contribute also to transepithelial secretion and reabsorption of acid,base equivalents and of Cl,. Members of both the SLC4 and SLC26 mammalian gene families encode Na+ -independent Cl,,HCO3, exchangers. Human SLC4A1/AE1 mutations cause either the erythroid disorders spherocytic haemolytic anaemia or ovalocytosis, or distal renal tubular acidosis. SLC4A2/AE2 knockout mice die at weaning. Human SLC4A3/AE3 polymorphisms have been associated with seizure disorder. Although mammalian SLC4/AE polypeptides mediate only electroneutral Cl,,anion exchange, trout erythroid AE1 also promotes osmolyte transport and increased anion conductance. Mouse AE1 is required for DIDS-sensitive erythroid Cl, conductance, but definitive evidence for mediation of Cl, conductance is lacking. However, a single missense mutation allows AE1 to mediate both electrogenic SO42,,Cl, exchange or electroneutral, H+ -independent SO42,,SO42, exchange. In the Xenopus oocyte, the AE1 C-terminal cytoplasmic tail residues reported to bind carbonic anhydrase II are dispensable for Cl,,Cl, exchange, but required for Cl,,HCO3, exchange. AE2 is acutely and independently inhibited by intracellular and extracellular H+, and this regulation requires integrity of the most highly conserved sequence of the AE2 N-terminal cytoplasmic domain. Individual missense mutations within this and adjacent regions identify additional residues which acid-shift pHo sensitivity. These regions together are modelled to form contiguous surface patches on the AE2 cytoplasmic domain. In contrast, the N-terminal variant AE2c polypeptide exhibits an alkaline-shifted pHo sensitivity, as do certain transmembrane domain His mutants. AE2-mediated anion exchange is also stimulated by ammonium and by hypertonicity by a mechanism sensitive to inhibition by chelation of intracellular Ca2+ and by calmidazolium. This growing body of structure,function data, together with increased structural information, will advance mechanistic understanding of SLC4 anion exchangers. [source] Ineffective Peripheral Tissue Perfusion: Clinical Validation in Patients With Hypertensive CardiomiopathyINTERNATIONAL JOURNAL OF NURSING TERMINOLOGIES AND CLASSIFICATION, Issue 2 2006Rita de Cassia Gengo de Silva MS PURPOSE.,To validate defining characteristics of ineffective peripheral tissue perfusion using vasomotor function assessment. METHODS.,Twenty-four patients with hypertensive cardiomiopathy were evaluated for 18 defining characteristics of ineffective peripheral tissue perfusion and underwent vasomotor function assessment with induction of reactive hyperemia, intra-arterial infusion of acetylcholine, and pulse wave velocity measurement. The Student's t test and Kruskall,Wallis test were used to assess the significance of relationships between defining characteristics and vasomotor function data. FINDINGS.,Diminished lower extremity pulses were associated with diminished forearm blood flow during acetylcholine infusion; left ventricular overload, intermittent claudication, and diminished skin moisture were associated with elevated pulse wave velocity values. CONCLUSION.,The defining characteristics of ineffective peripheral tissue perfusion were highly associated with vasomotor function data as "gold standards" for that diagnosis. PRACTICE IMPLICATIONS.,Nurses should be able to accurately assess diminished lower extremity pulses, intermittent claudication, and diminished skin moisture as relevant characteristics of ineffective peripheral tissue perfusion in patients with hypertensive cardiomiopathy. Irrigation Tissulaire Périphérique Inefficace: Validation Clinique Chez les Patients Atteints de Cardiomyopathie Hypertensive BUT.,Valider les caractéristiques de Irrigation tissulaire périphérique inefficace en utilisant l'évaluation de la fonction vasomotrice. MÉTHODES.,Vingt-quatre patients souffrant de cardiomyopathie hypertensive furent évalués au regard des 18 caractéristiques du diagnostic Irrigation vasculaire périphérique inefficace et de la fonction vasomotrice par induction d'une hyperémie réactionnelle, la perfusion intra-artérielle d'acétylcholine, et la mesure de la vélocité de l'onde du pouls. Les tests "Student t et Kruskall,Wallis" furent utilisés pour déterminer l'importance des liens entre les caractéristiques et les valeurs de la fonction vasomotrice. RÉSULTATS.,La diminution des pulsations périphériques des membres inférieurs fut associée à une diminution du flot sanguin pendant la perfusion d'acétylcholine; une surcharge ventriculaire gauche, de la claudication intermittente et une diminution de l'hydratation de la peau furent associées à des valeurs élevées de la vélocité de l'onde du pouls. CONCLUSION.,,Les caractéristiques de Irrigation tissulaire périphérique inefficace qui furent associées de manière significative à la fonction vasomotrice peuvent être considérées comme les "étalons or" de ce diagnostic. IMPLICATIONS POUR LA PRATIQUE.,Les infirmières devraient être capables d'évaluer correctement la diminution des pouls périphériques, la claudication intermittente, et la diminution de l'hydratation de la peau, car ce sont des caractéristiques pertinentes de l'irrigation tissulaire périphérique inefficace chez les patients atteints de cardiomyopathie hypertensive. Translation by Cécile Boisvert, MSN, RN Perfusão Tissular Periférica Ineficaz: Validação Clínica em Pacientes com Miocardiopatia Hipertensiva PROPÓSITO.,Validar as caraterísticas definidoras do diagnóstico de perfusão tissular periférica ineficaz usando a avaliação da função vasomotora. MÉTODO.,Vinte e quatro pacientes com miocardiopatia hipertensiva foram avaliados segundo 18 características definidoras de perfusão tissular periférica ineficaz e submetidos a avaliação da função vasomotora por indução de hiperemia reativa, infusão intra-arterial de acetilcolina e por mensuração da velocidade da onda de pulso. Testes T de Student e de Kruskall,Wallis foram aplicados para avaliar a significância das relações entre as características definidoras e os dados da função vasomotora. RESULTADOS.,Diminuição de pulso nas extremidades inferiores foi associada com o menor fluxo de sangue no antebraço durante a infusão de acetilcolina; sobrecarga ventricular esquerda, claudicação intermitente e diminuição da hidratação da pele foram associados com valores elevados de velocidade de onda de pulso. CONCLUSÃO.,Quatro características definidoras de perfusão tissular periférica ineficaz foram altamente associadas com função vasomotora alterada como "padrão ouro" para este diagnóstico. IMPLICAÇÕES PARA A PRÁTICA.,As enfermeiras devem ser capazes de avaliar com precisão a diminuição dos pulsos das extremidades inferiores, claudicação intermitente e diminuição na hidratação da pele como características definidoras relevantes da perfusão tissular periférica ineficaz em pacientes com miocardiopatia hipertensiva. Translation by Alba Leite de Barros, PhD, RN Perfusión Tisular Periférica Inefectiva: Validación Clínica en Pacientes que Presentan Miocardiopatía Hipertensiva PROPÓSITO.,Validar las características definitorias del diagnóstico Perfusión tisular periférica inefectiva utilizando una valoración de la función vasomotora. METODOLOGÍA.,Veinticuatro pacientes diagnosticados de Miocardiopatía Hipertensiva fueron evaluados con respecto a las 18 características definitorias del diagnóstico Perfusión tisular periférica inefectiva y sometidos a una valoración de la función vasomotora con inducción de Hiperemia reactiva, infusión intra-arterial de acetilcolina, y medida de la velocidad de la onda del pulso. Se utilizaron los análisis estadísticos de t-Student y Kruskall,Wallis para valorar el significado de las relaciones entre las características definitorias y los datos obtenidos de la valoración de la función vasomotora. HALLAZGOS.,Durante la infusión de acetilcolina se detectó disminución de los pulsos en la extremidad inferior relacionado con la disminución del volumen de sangre en el antebrazo; la sobrecarga ventricular izquierda, claudicación intermitente y disminución de la hidratación de la piel fueron asociadas con la elevación de los valores de la velocidad de la onda del pulso. CONCLUSIÓN.,Las características definitorias del diagnóstico Perfusión tisular periférica inefectiva estaban fuertemente asociadas a los datos procedentes de la valoración de la función vasomotora identificándose como "estándares fundamentales" para este diagnóstico. IMPLICACIONES PARA LA PRÁCTICA.,Las enfermeras deberían ser capaces de valorar cuidadosamente la presencia de pulsos disminuidos en las extremidades inferiores, y la disminución de la hidratación de la piel ya que son características relevantes del diagnóstico Perfusión tisular periférica inefectiva en pacientes que presentan Miocardiopatía Hipertensiva. Translation by Adolf Guirao, RN [source] A method for interleaved acquisition of a vascular input function for dynamic contrast-enhanced MRI in experimental rat tumoursNMR IN BIOMEDICINE, Issue 3 2004Dominick J. O. McIntyre Abstract Dynamic contrast-enhanced MRI is widely used for the evaluation of the response of experimental rodent tumours to antitumour therapy, particularly for the newly developing antiangiogenic and antivascular agents. However, standard models require a time-course for the plasma concentration of contrast agent (usually referred to as the arterial input function) to calculate the transfer constant Ktrans from the dynamic time-course data. Ideally, the plasma concentration time-course should be measured during each experiment to obtain the most accurate measure of Ktrans. This is technically difficult in rodents, so assumed values are generally used. A method is presented here using interleaved acquisitions from a tail coil to obtain the plasma concentration simultaneously with DCE-MRI data obtained from a solenoid coil around the tumour. The SNR of the resulting vascular input function data is high compared with methods using a volume coil to acquire plasma concentrations from the aorta and vena cava. Copyright © 2004 John Wiley & Sons, Ltd. [source] Does presenting with meconium ileus affect the prognosis of children with cystic fibrosis?PEDIATRIC PULMONOLOGY, Issue 10 2010Jo-Anne Johnson MBChB Abstract It is a matter of debate as to what extent the long-term outcome of cystic fibrosis (CF) is affected by presenting with meconium ileus (MI). We compared long-term clinical outcomes of CF children who presented with MI, to those presenting with other symptoms (non-MI) in an era of non new-born-screening (NBS). We collected annual lung function data between the ages of 8,15 years in terms of percent predicted first second forced expired volume (FEV1%pr), percent predicted forced vital capacity (FVC%pr), and between the ages of 2,15 years annual height and weight Z-scores (HtZ and WtZ respectively) for children attending the Royal Brompton Hospital CF clinic. To be included in the study, subjects had to have at least five pulmonary function tests and five anthropometric measurements recorded over this period. Thirty-eight MI and 76 non-MI subjects were compared. There were no significant differences in genotype, sex, chronic Pseudomonas infection, or pancreatic enzyme use between the two groups. The median age of diagnosis was 1 day (MI) versus 7 months (non-MI). There was a decline in spirometry and anthropometric variables over the study period for both MI and non-MI groups apart from WtZ score in the non-MI group. Mixed model analysis adjusting for potential confounders including genotype, pancreatic status, sex, chronic Pseudomonas aeruginosa lung infection, and age of diagnosis revealed no difference between the two groups in terms of lung function and growth during the time period of the study, however there was a non-significant trend for subjects presenting with MI to do better in all four parameters. We conclude that babies presenting with MI have no worse long-term outcome than those presenting symptomatically later in infancy, despite having undergone invasive procedures in the newborn period. This underscores the importance of early diagnosis and treatment in CF. Pediatr Pulmonol. 2010; 45:951,958. © 2010 Wiley-Liss, Inc. [source] Scaling law and critical exponent for ,0 at the 3D Anderson transitionANNALEN DER PHYSIK, Issue 12 2009L.J. Vasquez Abstract We use high-precision, large system-size wave function data to analyse the scaling properties of the multifractal spectra around the disorder-induced three-dimensional Anderson transition in order to extract the critical exponents of the transition. Using a previously suggested scaling law, we find that the critical exponent , is significantly larger than suggested by previous results. We speculate that this discrepancy is due to the use of an oversimplified scaling relation. [source] Characterization and peripheral blood biomarker assessment of anti,Jo-1 antibody,positive interstitial lung diseaseARTHRITIS & RHEUMATISM, Issue 7 2009Thomas J. Richards Objective Using a combination of clinical, radiographic, functional, and serum protein biomarker assessments, this study was aimed at defining the prevalence and clinical characteristics of interstitial lung disease (ILD) in a large cohort of patients with anti,Jo-1 antibodies. Methods A review of clinical records, pulmonary function test results, and findings on imaging studies determined the existence of ILD in anti,Jo-1 antibody,positive individuals whose data were accumulated in the University of Pittsburgh Myositis Database from 1982 to 2007. Multiplex enzyme-linked immunosorbent assays (ELISAs) for serum inflammation markers, cytokines, chemokines, and matrix metalloproteinases in different patient subgroups were performed to assess the serum proteins associated with anti,Jo-1 antibody,positive ILD. Results Among the 90 anti,Jo-1 antibody,positive individuals with sufficient clinical, radiographic, and/or pulmonary function data, 77 (86%) met the criteria for ILD. While computed tomography scans revealed a variety of patterns suggestive of underlying usual interstitial pneumonia (UIP) or nonspecific interstitial pneumonia, a review of the histopathologic abnormalities in a subset of patients undergoing open lung biopsy or transplantation or whose lung tissue was obtained at autopsy (n = 22) demonstrated a preponderance of UIP and diffuse alveolar damage. Analysis by multiplex ELISA yielded statistically significant associations between anti,Jo-1 antibody,positive ILD and elevated serum levels of C-reactive protein (CRP), CXCL9, and CXCL10, which distinguished this disease entity from idiopathic pulmonary fibrosis and anti,signal recognition particle antibody,positive myositis. Recursive partitioning further demonstrated that combinations of these and other serum protein biomarkers can distinguish these disease subgroups at high levels of sensitivity and specificity. Conclusion In this large cohort of anti,Jo-1 antibody,positive individuals, the incidence of ILD approached 90%. Multiplex ELISA demonstrated disease-specific associations between anti,Jo-1 antibody,positive ILD and serum levels of CRP as well as the interferon-,,inducible chemokines CXCL9 and CXCL10, highlighting the potential of this approach to define biologically active molecules contributing to the pathogenesis of myositis-associated ILD. [source] | ||||||||||