RESUMEN
A series of gold(I) phosphine complexes of the type [AuCl{PR(2)(R(f))}] (R = Et, i-Pr, Cy; R(f) = CF = CF(2); R = Ph, R(f) = C = CFH, CCl = CF(2), C ≡ CCF(3), CF(3), i-C(3)F(7), s-C(4)F(9)) have been prepared and most have been structurally characterised. All of the complexes are monomeric in the solid state, and a number of secondary interactions are observed--including short intramolecular AuF distances, metal-bound Au-ClH non-classical hydrogen bonds, fluorous domains and phenyl embraces. Only in the case of [AuCl{PEt(2)(CF = CF(2))}] is an aurophilic interaction with an AuAu contact less than the sum of the van der Waals radii observed. Even then, the distance, 3.3458(10) Å, is longer than that previously observed for the related complex with R = Ph; R(f) = CF = CF(2).
Asunto(s)
Compuestos Orgánicos de Oro/química , Fosfinas/química , Cristalografía por Rayos X , Modelos Moleculares , Compuestos Orgánicos de Oro/síntesis química , Fosfinas/síntesis químicaRESUMEN
Twenty five human breast adipose tissue samples were collected in Porto Alegre, Brazil during 2004-2005 and analyzed for polybrominated diphenyl ethers (PBDEs) and polychlorinated biphenyls (PCBs). SigmaPBDE concentrations (sum of tri- to hepta-BDEs) ranged from 0.19 to 132 ng/g lipid with a median of 1.51 ng/g lipid. These concentrations are 3- to 100-times lower than those reported from other countries, with the exception of Japan, probably reflecting lower usage of PBDE-containing products or lower exposures to these chemicals. The predominant congener was BDE-47, followed by BDEs 99, 183, 153 and 100. One individual in the dataset had about 70-times higher PBDE concentrations than the rest of the participants. SigmaPCB (sum of PCBs 118, 138, 153, 180) ranged from 30 to 339 ng/g lipid, with a median of 51 ng/g lipid. No age dependency was found for PBDEs (r=-0.800-0.374, p>0.05) or PCB 180 (r=0.278, p>0.05). On the other hand, PCBs 118, 138 and 153 did show age dependency (r=0.410-0.458, p<0.05). This is the first study to report levels of PBDEs in human breast adipose from Brazil.
Asunto(s)
Tejido Adiposo/química , Mama/química , Éteres Difenilos Halogenados/análisis , Bifenilos Policlorados/análisis , Factores de Edad , Brasil , Femenino , HumanosRESUMEN
The coordination chemistry of the perfluorovinyl phosphines PEt2(CF=CF2), P(i)Pr2(CF=CF2), PCy,(CF=CF2) and PPh(CF=CF2)2 to rhodium(I), palladium(II), and platinum(II) centres has been investigated. The electronic properties of the ligands are estimated based on v(CO) and 1J(Rh-P) values. X-Ray diffraction data for the square-planar Pd(II) and Pt(II) perfluorovinyl-phosphine containing complexes allow estimates of the steric demand for the series of ligands PPh2(CF=CF2), PEt2(CF=CF2), P(i)Pr2(CF=CF2), PCy2(CF=CF2) and PPh(CF=CF2)2 to be determined. The (CF=CF2) fragment is found to be more electron withdrawing than (C6F5) yet sterically less demanding. These ligands therefore provide a range of electron-neutral to phosphite-like electronic properties combined with a range of steric demands. This study also reveals that short intramolecular interactions from the metal centre to the beta-fluorine atom cis to phosphorus of the CF=CF2 groups are observed in all-trans square planar complexes of the ligands. Unusually, the complex [PtCl2{P(i)Pr2(CF=CF2)}2] crystallises with both cis- and trans-isomers present in the unit cell. It appears that co-crystallisation of both isomers occurs in order to maximise fluorous regions in the crystal packing, and the extended structure displays short fluorine-fluorine contacts. The generation of mixed geometries seems to be a phenomenon of crystallisation, as solution phase NMR studies reveal the presence of only the trans-isomer.
RESUMEN
OBJECTIVE: To determine the influence of alpha-linolenic acid (ALA; 18 : 3omega3) intake and, hence, the influence of plasma and/or erythrocyte phospholipid content of docosahexaenoic acid (DHA; 22 : 6omega3) during early infancy on neurodevelopmental outcome of term infants. METHODS: The Bayley Scales of Infant Development (second edition), the Clinical Adaptive Test/Clinical Linguistic and Auditory Milestone Scale (CAT/CLAMS) and the Gross Motor Scale of the Revised Gesell Developmental Inventory were administered at a mean age of 12.26 +/- 0.94 months to 44 normal term infants enrolled in a study evaluating the effects of infant formulas differing only in ALA content (0.4, 1.0, 1.7 and 3.2% of total fatty acids). RESULTS: As reported previously [Jensen et al., Lipids 13 (1996) 107; J. Pediatr. 131 (1997) 200], the group fed the formula with the lowest ALA content had the lowest mean plasma and erythrocyte phospholipid DHA contents at 4 months of age. This group also had the lowest mean score on every neurodevelopmental measure. The difference in mean gross motor developmental quotient of this group versus the group fed the formula with 1.0% ALA but not of the other groups was statistically significant (P < 0.05). Across the groups, motor indices correlated positively with each other and with the plasma phospholipid DHA content at 4 months of age (P=0.02-0.03). The CLAMS developmental quotient correlated with the erythrocyte phospholipid content of 20 : 5omega3 (P < 0.01) but not with DHA. CONCLUSIONS: These statistically significant correlations suggest that the omega3 fatty acid status during early infancy may be important with respect to neurodevelopmental status at 1 year of age and highlight the need for further studies of this possibility.
Asunto(s)
Desarrollo Infantil , Ácidos Docosahexaenoicos/sangre , Sistema Nervioso/crecimiento & desarrollo , Ácido alfa-Linolénico/administración & dosificación , Membrana Eritrocítica/química , Femenino , Humanos , Lactante , Alimentos Infantiles/análisis , Fenómenos Fisiológicos Nutricionales del Lactante , Recién Nacido , Masculino , Lípidos de la Membrana/análisis , Fenómenos Fisiológicos del Sistema Nervioso , Fosfolípidos/sangre , Ácido alfa-Linolénico/metabolismoAsunto(s)
Trastorno Autístico/psicología , Desarrollo Infantil , Cognición , Desarrollo del Lenguaje , Desempeño Psicomotor , Trastorno Autístico/diagnóstico , Niño , Trastornos Generalizados del Desarrollo Infantil/psicología , Preescolar , Femenino , Humanos , Lactante , Masculino , Pruebas Neuropsicológicas , Estudios Retrospectivos , Índice de Severidad de la EnfermedadAsunto(s)
Trastorno Autístico/diagnóstico , Encéfalo/patología , Aberraciones Cromosómicas/diagnóstico , Síndrome del Cromosoma X Frágil/diagnóstico , Pruebas Genéticas , Trastorno Autístico/genética , Trastorno Autístico/patología , Trastorno Autístico/fisiopatología , Encéfalo/metabolismo , Encéfalo/fisiopatología , Niño , Trastornos Generalizados del Desarrollo Infantil/diagnóstico , Preescolar , Aberraciones Cromosómicas/psicología , Trastornos de los Cromosomas , Diagnóstico Diferencial , Electroencefalografía , Femenino , Síndrome del Cromosoma X Frágil/psicología , Humanos , Lactante , Imagen por Resonancia Magnética , Masculino , Guías de Práctica Clínica como Asunto , Estudios RetrospectivosRESUMEN
A case illustrating a congenital anomaly in the development of the left carotid artery is presented. The common carotid artery was found to be absent, with both internal and external carotid arteries arising separately from the aortic arch. This is a rare occurrence that is described angiographically and embryologically.
Asunto(s)
Aorta Torácica/anomalías , Arteria Carótida Externa/anomalías , Arteria Carótida Interna/anomalías , Anciano , Angiografía , Aorta Torácica/diagnóstico por imagen , Aortografía , Encéfalo/irrigación sanguínea , Arteria Carótida Común/anomalías , Arteria Carótida Externa/diagnóstico por imagen , Arteria Carótida Interna/diagnóstico por imagen , Femenino , HumanosRESUMEN
Williams syndrome is a genetic disorder linked to cognitive and behavioral patterns of varying consistency; this study was conducted to clarify further the strengths and weaknesses of children with Williams syndrome. Fifteen subjects with the characteristic features of Williams syndrome were evaluated using the Stanford-Binet Intelligence Scale for Children, Fourth Edition; the Vineland Adaptive Behavior Scales, Interview Edition; and the Child Behavior Checklist. Cognitive skills ranged from the Moderate Range of Mental Retardation to the Low Average range, with relative strengths in nonverbal and quantitative reasoning. Adaptive skills were delayed, with strengths in communication and socialization. Behaviorally, clinically significant levels of attention problems, borderline-significant levels of social and thought problems, and significantly low levels of social contacts and structured activities were found. In contrast to the findings of many other studies of Williams syndrome, language skills and short-term memory skills were weak. Children with Williams syndrome may present a more evenly developed intellectual profile, with verbal and nonverbal skills being commensurate. In conclusion, a variety of cognitive, adaptive, and behavioral patterns have been shown to be possible in Williams syndrome; therefore, a single predictable cognitive or behavioral phenotype cannot be assumed.
Asunto(s)
Adaptación Psicológica , Conducta del Adolescente , Conducta Infantil , Manifestaciones Neuroconductuales , Síndrome de Williams/psicología , Actividades Cotidianas , Adolescente , Niño , Preescolar , Femenino , Humanos , Masculino , Destreza Motora , Conducta Social , Prueba de Stanford-Binet , Conducta Verbal , Síndrome de Williams/genéticaRESUMEN
The peroxisomal disorders represent a group of inherited metabolic disorders that derive from defects of peroxisomal biogenesis and/or from dysfunction of single or multiple peroxisomal enzymes. We described earlier an 8 1/2 year-old with a history of progressive developmental delay, micronodular cirrhosis, and elevated very long chain fatty acids in plasma and skin fibroblasts. These findings were felt to be compatible with both neonatal adrenoleukodystrophy (nALD) and Zellweger syndrome (ZS). This patient is now 21 years old and his clinical course, inconsistent with either nALD or ZS, led us to examine his peroxisomal status in light of a possible new peroxisomal disease. The normal levels of bile acid precursors found in this patient suggest that peroxisomal beta-oxidation is functional. The activities of dihydroxyacetone phosphate acyltransferase and oxidation of lignoceric acid and phytanic acid were 14, 17, and 15% of the control, respectively. This partial activity for oxidation and the normal levels of bile acid precursors suggests that this patient has peroxisomes containing beta-oxidation enzymes. Western blot analysis of subcellular organelles showed that beta-oxidation enzyme proteins are present at normal levels in catalase-negative peroxisomes of density equivalent to normal peroxisomes. The presence of acyl-CoA oxidase and 3-ketoacyl-CoA thiolase in catalase-negative peroxisomes suggests that both peroxisomal targeting signal-1 (PTS-1), and peroxisomal targeting signal-2 (PTS-2)-mediated protein transport processes into peroxisomes are normal in this patient. These findings of catalase-negative peroxisomes of normal density and normal PTS-1 and PTS-2 import machinery with partial peroxisomal functions clearly demonstrate that this patient differs from those with known disorders of peroxisomal biogenesis.
Asunto(s)
Microcuerpos/metabolismo , Proteínas/metabolismo , Síndrome de Zellweger/metabolismo , Catalasa/metabolismo , Células Cultivadas , Niño , Complejo IV de Transporte de Electrones/metabolismo , Fibroblastos/metabolismo , Humanos , Masculino , NADH Deshidrogenasa/metabolismo , Síndrome de Zellweger/sangre , Síndrome de Zellweger/orinaRESUMEN
The combination of neurodevelopmental regression and adrenal insufficiency should alert practitioners or emergency room physicians about ALD. Although still unproven, early medical intervention with either gene therapy or bone marrow transplantation may offer more promise to these patients.
Asunto(s)
Adrenoleucodistrofia/diagnóstico , Adrenoleucodistrofia/psicología , Trasplante de Médula Ósea , Ácidos Erucicos/uso terapéutico , Discapacidades para el Aprendizaje/etiología , Trioleína/uso terapéutico , Adrenoleucodistrofia/terapia , Encéfalo/patología , Niño , Diagnóstico Diferencial , Grasas Insaturadas en la Dieta/uso terapéutico , Combinación de Medicamentos , Humanos , Aprendizaje , Imagen por Resonancia Magnética , Masculino , Pruebas Neuropsicológicas , Examen FísicoRESUMEN
An upward trend in breast feeding has been seen in recent years, and health care providers must take an active role in providing services to lactating women if this trend continues. A nurse managed, hospital-based, breast-feeding program that provides continuity of care and supportive services from the hospital to the community for lactating women is described.
Asunto(s)
Lactancia Materna , Servicios de Salud Materna/organización & administración , Madres/educación , Continuidad de la Atención al Paciente , Femenino , Humanos , Lactante , Recién Nacido , Madres/psicología , Apoyo SocialRESUMEN
The purpose of this descriptive pilot study was to discover and correct problems with data collection in the identification of variables that are the strongest predictors of the occurrence of four nosocomial infections in cardiac patients. Data from 29 subjects who were admitted to a critical care unit (CCU) were tracked at a health care facility located in upstate New York. Data were obtained from daily care flow sheets and patients' charts. Although most results were not statistically significant, trends were identified in the data. The 7 patients who developed nosocomial infections were men who were older than 50 years, underwent a cardiac surgical or other invasive procedure, had more than one intravenous catheter, experienced a significant event in the CCU, and received prophylactic antibiotics. It is recommended that the number of independent variables tracked be reduced to 20. In addition, readmission of subjects whose data are being tracked should be included.
Asunto(s)
Infección Hospitalaria/epidemiología , Infección Hospitalaria/etiología , Cardiopatías/complicaciones , Adulto , Anciano , Infección Hospitalaria/prevención & control , Femenino , Cardiopatías/terapia , Humanos , Control de Infecciones , Masculino , Persona de Mediana Edad , Proyectos Piloto , Factores de RiesgoRESUMEN
The peroxisomal disorders represent a group of inherited metabolic disorders that derive from defects of peroxisomal biogenesis and/or from dysfunction of single or multiple peroxisomal enzymes. Because peroxisomes are involved in the metabolism of lipids critical to the functioning of the nervous system, many of the peroxisomal disorders manifest with significant degrees of progressive psychomotor dysfunction. These disorders should be considered in the differential diagnosis of the infant with hypotonia and psychomotor delay (especially if accompanied by facial dysmorphisms, hepatomegaly, cataracts and/or retinitis, calcific stippling, short limbs, or combinations of these features), in the school-aged child with progressive neurologic dysfunction, and in adults with slowly progressive motor dysfunction. Current knowledge of peroxisomal biochemical and enzymatic processes permits precise identification of particular disorders within the peroxisomal disorder grouping. An effort should be made to identify the specific peroxisomal disorder to provide a precise explanation for neurodevelopmental deficits, to potentially prevent recurrence through genetic counseling, and to provide appropriate therapies when available.
Asunto(s)
Adrenoleucodistrofia/metabolismo , Condrodisplasia Punctata/metabolismo , Microcuerpos/metabolismo , Enfermedad de Refsum/metabolismo , Proteínas Represoras , Proteínas de Saccharomyces cerevisiae , Síndrome de Zellweger/metabolismo , Acetil-CoA C-Aciltransferasa/metabolismo , Acil-CoA Oxidasa , Adolescente , Adrenoleucodistrofia/diagnóstico , Adrenoleucodistrofia/genética , Adrenoleucodistrofia/terapia , Adulto , Niño , Preescolar , Condrodisplasia Punctata/diagnóstico , Condrodisplasia Punctata/genética , Condrodisplasia Punctata/terapia , Coenzima A Ligasas/metabolismo , Diagnóstico Diferencial , Humanos , Lactante , Recién Nacido , Microcuerpos/enzimología , Microcuerpos/patología , Oxidorreductasas/metabolismo , Enfermedad de Refsum/diagnóstico , Enfermedad de Refsum/genética , Enfermedad de Refsum/terapia , Síndrome de Zellweger/diagnóstico , Síndrome de Zellweger/genética , Síndrome de Zellweger/terapiaRESUMEN
The transport efficiency of the particle beam liquid chromatography/mass spectrometer interface is influenced by analyte concentration contributing to a widely reported non-linearity. In this work, coeluting, isotope-labeled internal standards were investigated as 'carriers' to improve the transport efficiency and linearity. Three styrene metabolites--mandelic, phenylglyoxylic and hippuric acids--and their pentadeutero analogs were separated by reversed-phase liquid chromatography (LC) with an ammonium acetate-acetonitrile mobile phase. Selected positive ions produced by electron ionization were monitored to generate particle beam LC/MS calibration curves. The present study demonstrates that particle beam LC/MS not only is non-linear, but also is subject to a matrix effect presumably by the same mechanism responsible for non-linearity. Coeluting, isotope-labeled internal standards were ineffective at linearizing the particle beam liquid chromatograph/mass spectrometer detector response. Isotope dilution quantification, however, compensates for variable transport efficiencies, linearizes calibration and compensates for the matrix effect, affording reliable quantification of the styrene metabolites.
Asunto(s)
Cromatografía Liquida/instrumentación , Espectrometría de Masas/instrumentación , Técnica de Dilución de Radioisótopos/instrumentación , Adulto , Calibración , Glioxilatos/orina , Hipuratos/orina , Humanos , Indicadores y Reactivos , Masculino , Ácidos Mandélicos/orina , Estireno , Estirenos/orinaRESUMEN
The kinetics of the dissolution of copper metal in an aqueous solution containing copper(II) and an unsaturated organic ligand was followed by using an automated flow-injection analysis technique to determine the concentration of copper(I) in solution as a function of time. The results suggest that the rate of dissolution of the copper metal is dependent on electron transfer between the copper(II) and copper atoms on the surface of the copper metal, and on the stabilization of copper(I) by the unsaturated organic ligand in solution.
RESUMEN
Williams syndrome is characterized by cardiac defects, varying degrees of physical and developmental delay, stellate eye pattern, possible elevated serum calcium level, and elfin/pixie facial features. A problem perhaps unique to these children is hyperacusis that can be severe enough to disrupt many routine daily activities. Parental questionnaires were used to determine the prevalence of hyperacusis and otitis media in individuals with Williams syndrome. Prevalences of 95% for hyperacusis and 61% for otitis media were found. This was significantly higher than in the general population. Despite the prevalence of hyperacusis, parents of these children were not counseled about management of the problem. The audiologist may become involved with Williams syndrome patients through hearing assessment and management, parental counseling, and research.
Asunto(s)
Estenosis de la Válvula Aórtica/complicaciones , Cara/anomalías , Trastornos de la Audición/complicaciones , Discapacidad Intelectual/complicaciones , Otitis Media/complicaciones , Adolescente , Adulto , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , SíndromeRESUMEN
The cognitive and adaptive capacities of 130 individuals with Down syndrome were investigated as a function of age and environmental placement. Intellectual deterioration occurred whether individuals resided at home or in an institutional setting. Social/adaptive deterioration also occurred but with the least decline for those individuals who resided in institutional settings.
Asunto(s)
Síndrome de Down/rehabilitación , Ambiente , Actividades Cotidianas , Adolescente , Adulto , Factores de Edad , Niño , Preescolar , Femenino , Humanos , Lactante , Inteligencia , Masculino , Persona de Mediana Edad , Instituciones ResidencialesRESUMEN
To examine the proposal that myelin proteolipid protein underlies the adhesion of neighboring membranes in central nervous system (CNS) myelin, we carried out X-ray diffraction studies on the structure and interactions of model bilayers composed of total myelin lipids plus proteolipid apoprotein (PLP). Total myelin lipids were used because their heterogeneity was expected to provide an appropriate environment for the integral membrane protein to achieve its native conformation and establish appropriate contacts with the apposed bilayer. We found that incorporation of PLP into myelin lipid bilayers, whether organized into multilamellar vesicles or oriented multilayers, did not appreciably affect the lamellar period, which ranged from 65-71 A. In oriented multilayers, the wide-angle spacing at approximately 4.8 A, which arises from the lateral packing of lipid chains and is perpendicular to the lamellar diffraction, was less oriented and weaker in bilayers containing the protein. These results indicate that PLP was incorporated into the bilayers and had a disordering effect on the hydrocarbon chains but did not extend into the spaces between bilayers. Bilayer profiles calculated from the lamellar diffraction to about 15 A spacing did not show any major changes in the distribution of electron density, suggesting that to moderate resolution, the protein was distributed uniformly across the width of the lipid bilayer. Periodicities measured from osmotically stressed multilamellar vesicles did not depend on the presence of PLP, indicating that the protein did not form stabilizing contacts between bilayers.(ABSTRACT TRUNCATED AT 250 WORDS)