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1.
Childs Nerv Syst ; 39(6): 1509-1518, 2023 06.
Artículo en Inglés | MEDLINE | ID: mdl-36790496

RESUMEN

PURPOSE: Atypical teratoid/rhabdoid tumours (ATRTs) are malignant embryonal tumours of childhood that affect the central nervous system (CNS). We aim to determine which factors, including patient age, extent of resection (EOR), presence of distal metastasis and use of adjuvant therapies, affect overall survival in children with atypical teratoid/rhabdoid tumours (ATRTs) treated at this single centre. METHODS: Retrospective cohort review of patients with histological diagnosis of ATRT treated over 21 years (1999-2020) was conducted. Data on demographics, tumour location, presence of metastasis, use of adjuvant therapy, extent of resection (EOR), complications, neurological outcome post-surgery, and overall survival were collected. Kaplan-Meier survival analysis was performed. RESULTS: A total of 45 children (mean age 2 years) underwent 64 operations. 25 patients were <1 year of age. Gross-total resection (GTR) pre-adjuvant therapy was achieved in 15, near-total resection (NTR) in 15, subtotal resection (STR) in 9, and biopsy in 6 children. Most children had good neurological outcomes post-operatively (28/45 with GOS 5). Fourteen patients survived longer than 4 years. Survival analysis showed a significant difference in median survival in favour of GTR and localised disease. There was no significant difference in median survival between patients <1 year vs >1 year of age (p=0.84). CONCLUSION: We find that presence of metastasis was an important factor in poor survival in patients with ATRT. GTR, where possible, may confer significant survival benefit in ATRT. Children aged <1 year appear to have performed as well as those >1 year and therefore should still be considered for radical surgery.


Asunto(s)
Neoplasias del Sistema Nervioso Central , Tumor Rabdoide , Teratoma , Niño , Humanos , Preescolar , Estudios Retrospectivos , Tumor Rabdoide/cirugía , Tumor Rabdoide/patología , Teratoma/cirugía , Teratoma/patología , Neoplasias del Sistema Nervioso Central/cirugía , Análisis de Supervivencia
2.
Artículo en Inglés | MEDLINE | ID: mdl-36518619

RESUMEN

Our objective was to develop a clinical practice guideline (CPG) for the treatment of acute lower extremity fractures in persons with a chronic spinal cord injury (SCI). Methods: Information from a previous systematic review that addressed lower extremity fracture care in persons with an SCI as well as information from interviews of physical and occupational therapists, searches of the literature, and expert opinion were used to develop this CPG. The Grading of Recommendations, Assessment, Development and Evaluations (GRADE) system was used to determine the quality of evidence and the strength of the recommendations. An overall GRADE quality rating was applied to the evidence. Conclusions: Individuals with a chronic SCI who sustain an acute lower extremity fracture should be provided with education regarding the risks and benefits of operative and nonoperative management, and shared decision-making for acute fracture management should be used. Nonoperative management historically has been the default preference; however, with the advent of greater patient independence, improved surgical techniques, and advanced therapeutics and rehabilitation, increased use of surgical management should be considered. Physical therapists, kinesiotherapists, and/or occupational therapists should assess equipment needs, skills training, and caregiver assistance due to changes in mobility resulting from a lower extremity fracture. Therapists should be involved in fracture management as soon as possible following fracture identification. Pressure injuries, compartment syndrome, heterotopic ossification, nonunion, malunion, thromboembolism, pain, and autonomic dysreflexia are fracture-related complications that clinicians caring for patients who have an SCI and a lower extremity fracture may encounter. Strategies for their treatment are discussed. The underlying goal is to return the patient as closely as possible to their pre-fracture functional level with operative or nonoperative management.

3.
Acta Neurol Scand ; 138(2): 137-142, 2018 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-29532475

RESUMEN

OBJECTIVES: Papilloedema is a clinical manifestation of chronically raised intracranial pressure (ICP), often seen in idiopathic intracranial hypertension (IIH). However, the extent of intracranial hypertension required to produce papilloedema is not known. We compare ICP values in IIH patients who developed papilloedema and those who did not. We aim to identify a pathological ICP threshold predictive of the development of papilloedema in IIH patients. MATERIALS AND METHODS: Single-centre cohort of IIH patients (2006-2016) who underwent 24-hour ICP monitoring (ICPM) and ophthalmology assessments, prior to intervention. Papilloedema was graded according to the Frisén scale. An unpaired t-test compared 24-hour ICPM between papilloedema and no-papilloedema groups. Fisher's exact test was used to determine predictive value of ICP. RESULTS: Thirty-six patients with IIH (35 F: 1M), mean age 32.5 ± 9.49 years (mean ± SD) were included. Patients with papilloedema had a mean median 24-hour ICP of 10.4 ± 5.32 mm Hg (n = 25), significantly higher than the group without papilloedema 6.31 ± 3.30 mm Hg (n = 11) (P < .05). The papilloedema group were exposed to higher pressures (10 mm Hg) for 30 minutes or more. Using 24-hour median ICP of 10 mm Hg as a minimum cut-off predictive value gives a specificity = 91%, sensitivity = 48%, PPV = 92% and NPV = 44% of detecting papilloedema. CONCLUSIONS: A 24-hour ICP of 10 mmHg or more is a good predictor for papilloedema and reflects a pathological threshold. The range varied widely suggesting papilloedema can occur at even lower pressures. These results are consistent with emerging evidence suggest that pathologically "high" 24 hours ICP is lower than previously quoted.


Asunto(s)
Papiledema/etiología , Seudotumor Cerebral/complicaciones , Adulto , Femenino , Humanos , Masculino , Monitorización Neurofisiológica , Papiledema/fisiopatología , Seudotumor Cerebral/fisiopatología , Curva ROC , Valores de Referencia , Estudios Retrospectivos , Adulto Joven
5.
Acta Neurochir (Wien) ; 159(3): 485-490, 2017 03.
Artículo en Inglés | MEDLINE | ID: mdl-28108854

RESUMEN

BACKGROUND: Intracranial pressure monitoring is commonly undertaken to assess and manage acute patients following head injury. However, ICP monitoring can also be a useful diagnostic tool in the management of CSF dynamics in elective patients. To date, there is little published research to suggest how long these elective patients require ICP monitoring in order to gain an accurate picture of a patient's ICP dynamics. At the author's institution, a minimum of 48-h data collection is currently undertaken in patients with a suspected ICP abnormality. METHODS: A retrospective audit was undertaken comparing overall median ICP and overall median pulse amplitude data at three time points, 24 h, 48 h and total time analysed (if longer than 48 h). Paired T-test was used to assess if there were statistically significant differences between 24-h versus 48-h monitoring and total duration of monitoring. All patients admitted over a 6-month period for ICPM who met the inclusion/exclusion criteria were included. RESULTS: Eighteen patients met the criteria. Median age was 45.8 years, range 22-83 years, 12 female and 6 male. No complications were experienced as a result of ICPM. Diagnosis included NPH, IIH, suspected shunt malfunction and Chiari malformation. The results demonstrated that there is no statistical difference between 24 h and 48 h or longer for both overall median ICP and pulse amplitude. CONCLUSION: The results of this study demonstrate that ICP monitoring of elective adult patients using a Spiegelberg intraparenchymal bolt for 24 h gives an accurate picture of a patient's ICP dynamics compared with longer periods of monitoring.


Asunto(s)
Presión Intracraneal/fisiología , Monitoreo Fisiológico/normas , Adulto , Anciano , Anciano de 80 o más Años , Malformación de Arnold-Chiari/diagnóstico , Femenino , Humanos , Hidrocéfalo Normotenso/diagnóstico , Masculino , Persona de Mediana Edad , Seudotumor Cerebral/diagnóstico , Estudios Retrospectivos , Factores de Tiempo , Adulto Joven
6.
Elife ; 5: e10167, 2016 Jan 14.
Artículo en Inglés | MEDLINE | ID: mdl-26783763

RESUMEN

We present a reanalysis of the stochastic model of organelle production and show that the equilibrium distributions for the organelle numbers predicted by this model can be readily calculated in three different scenarios. These three distributions can be identified as standard distributions, and the corresponding exact formulae for their mean and variance can therefore be used in further analysis. This removes the need to rely on stochastic simulations or approximate formulae (derived using the fluctuation dissipation theorem). These calculations allow for further analysis of the predictions of the model. On the basis of this we question the extent to which the model can be used to conclude that peroxisome biogenesis is dominated by de novo production when Saccharomyces cerevisiae cells are grown on glucose medium.


Asunto(s)
Biogénesis de Organelos , Peroxisomas/metabolismo , Saccharomyces cerevisiae/fisiología , Medios de Cultivo/química , Glucosa/metabolismo , Modelos Teóricos , Saccharomyces cerevisiae/crecimiento & desarrollo
7.
Elife ; 42015 Dec 22.
Artículo en Inglés | MEDLINE | ID: mdl-26765560

RESUMEN

We present a reanalysis of the stochastic model of organelle production and show that the equilibrium distributions for the organelle numbers predicted by this model can be readily calculated in three different scenarios. These three distributions can be identified as standard distributions, and the corresponding exact formulae for their mean and variance can therefore be used in further analysis. This removes the need to rely on stochastic simulations or approximate formulae (derived using the fluctuation dissipation theorem). These calculations allow for further analysis of the predictions of the model. On the basis of this we question the extent to which the model can be used to conclude that peroxisome biogenesis is dominated by de novo production when Saccharomyces cerevisiae cells are grown on glucose medium.


Asunto(s)
Modelos Teóricos , Biogénesis de Organelos , Peroxisomas/metabolismo , Saccharomyces cerevisiae/crecimiento & desarrollo , Medios de Cultivo/química , Glucosa/metabolismo
8.
Appl Clin Inform ; 5(1): 92-117, 2014.
Artículo en Inglés | MEDLINE | ID: mdl-24734127

RESUMEN

UNLABELLED: The US government allocated $30 billion to implement electronic health records (EHRs) in hospitals and provider practices through policy addressing Meaningful Use (MU). Most small, rural hospitals, particularly those designated as Critical Access Hospitals (CAHs), comprising nearly a quarter of US hospitals, had not implemented EHRs before. Little is known about implementation in this setting. Socio-technical factors differ between larger hospitals and CAHs, which continue to lag behind other hospitals in EHR adoption. OBJECTIVE: The main objective is to provide EHR implementation advice for CAHs from a spectrum of experts with an emphasis on recommendations from their peers at CAHs that have undertaken the process. The secondary objective is to begin to identify implementation process differences at CAHs v. larger hospitals. METHODS: We interviewed 41 experts, including 16 CAH staff members from EHR teams at 10 CAHs that recently implemented EHRs. We qualitatively analyzed the interviews to ascertain themes and implementation recommendations. RESULTS: Nineteen themes emerged. Under each theme, comments by experts provide in-depth advice on all implementation stages including ongoing optimization and use. We present comments for three top themes as ranked by number of CAH peer experts commenting - EHR System Selection, EHR Team, and Preparatory Work - and for two others, Outside Partners/Resources and Clinical Decision Support (CDS)/Knowledge Management (KM). Comments for remaining themes are included in tables. DISCUSSION: CAH experts rank the themes differently from all experts, a likely indication of the differences between hospitals. Comments for each theme indicate the specific difficulties CAHs encountered. CAH staffs have little or no EHR experience before implementation. A factor across themes is insufficient system and process knowledge, compounded by compressed implementation schedules. Increased, proactive self-education, via available outside partners and information resources, will mitigate difficulties and aid CAHs in meeting increased CDS requirements in MU Stages 2 and 3.


Asunto(s)
Registros Electrónicos de Salud , Testimonio de Experto , Implementación de Plan de Salud , Accesibilidad a los Servicios de Salud , Hospitales Rurales , Grupo Paritario , Sistemas de Apoyo a Decisiones Clínicas , Humanos
9.
Eukaryot Cell ; 12(7): 998-1008, 2013 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-23666623

RESUMEN

In the hyphal tip of Candida albicans we have made detailed quantitative measurements of (i) exocyst components, (ii) Rho1, the regulatory subunit of (1,3)-ß-glucan synthase, (iii) Rom2, the specialized guanine-nucleotide exchange factor (GEF) of Rho1, and (iv) actin cortical patches, the sites of endocytosis. We use the resulting data to construct and test a quantitative 3-dimensional model of fungal hyphal growth based on the proposition that vesicles fuse with the hyphal tip at a rate determined by the local density of exocyst components. Enzymes such as (1,3)-ß-glucan synthase thus embedded in the plasma membrane continue to synthesize the cell wall until they are removed by endocytosis. The model successfully predicts the shape and dimensions of the hyphae, provided that endocytosis acts to remove cell wall-synthesizing enzymes at the subapical bands of actin patches. Moreover, a key prediction of the model is that the distribution of the synthase is substantially broader than the area occupied by the exocyst. This prediction is borne out by our quantitative measurements. Thus, although the model highlights detailed issues that require further investigation, in general terms the pattern of tip growth of fungal hyphae can be satisfactorily explained by a simple but quantitative model rooted within the known molecular processes of polarized growth. Moreover, the methodology can be readily adapted to model other forms of polarized growth, such as that which occurs in plant pollen tubes.


Asunto(s)
Actinas/metabolismo , Candida albicans/crecimiento & desarrollo , Candida albicans/metabolismo , Hifa/crecimiento & desarrollo , Hifa/metabolismo , Vesículas Transportadoras/metabolismo , Pared Celular/metabolismo , Endocitosis , Proteínas Fúngicas/metabolismo , Humanos , Modelos Biológicos
10.
Spinal Cord ; 50(11): 840-3, 2012 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-22565550

RESUMEN

STUDY DESIGN: A multicenter Canadian cohort study. OBJECTIVES: The objective of this study is to evaluate the impact of early versus late surgical decompression on motor neurological recovery after traumatic spinal cord injury (SCI). SETTING: Canadian acute care and SCI rehabilitation facilities. METHODS: A prospective cohort study of patients within the Ontario Spinal Cord Injury Registry program was performed. We considered SCI patients with an admission American Spinal Injury Association (ASIA) Impairment Scale (AIS) grade of A through D, with magnetic resonance imaging-confirmed spinal cord compression. Grouped analysis was performed comparing the cohort of patients who received early surgery (<24 h after SCI) to those receiving delayed surgery (<24 h after SCI). The primary outcome was the change in ASIA motor score (AMS) occurring between hospital admission and rehabilitation discharge. RESULTS: A total of 35 (41.7%) patients underwent early surgery and 49 (58.3%) underwent late surgery. At admission, there was a greater proportion of patients within the early surgery group with more severe AIS grade A injuries. Of the 55 patients with neurological exam available at rehabilitation discharge, a greater proportion had at least a two-grade AIS improvement in the early-surgery group (P=0.01). The mean improvement in AMS at rehabilitation discharge was 20 points amongst early-surgery patients and 15 points amongst late-surgery patients (P=0.46). In the analysis investigating AMS improvement, adjusted for preoperative status and neurological level, there was a positive effect estimate for early surgical therapy that was statistically significant (P=0.01). CONCLUSION: The results here add weight to the growing body of literature, which supports the principle of early intervention in the setting of spinal trauma and SCI.


Asunto(s)
Descompresión Quirúrgica/métodos , Procedimientos Neuroquirúrgicos/métodos , Recuperación de la Función , Traumatismos de la Médula Espinal/cirugía , Adulto , Canadá , Estudios de Cohortes , Femenino , Humanos , Tiempo de Internación , Masculino , Persona de Mediana Edad , Tiempo , Resultado del Tratamiento
11.
Br J Radiol ; 85(1013): 523-38, 2012 May.
Artículo en Inglés | MEDLINE | ID: mdl-21343316

RESUMEN

OBJECTIVES: The Radiation Protection of Patients Unit of the International Atomic Energy Agency (IAEA) is concerned about the effectiveness of justification of diagnostic medical exposures. Recent published work and the report of an initial IAEA consultation in the area gave grounds for such concerns. There is a significant level of inappropriate usage, and, in some cases, a poor level of awareness of dose and risk among some key groups involved. This article aims to address this. METHODS: The IAEA convened a second group of experts in November 2008 to review practical and achievable actions that might lead to more effective justification. RESULTS: This report summarises the matters that this group considered and the outcome of their deliberations. There is a need for improved communication, both within professions and between professionals on one hand, and between professionals and the patients/public on the other. Coupled with this, the issue of consent to imaging procedures was revisited. The need for good evidence-based referral guidelines or criteria of acceptability was emphasised, as was the need for their global adaptation and dissemination. CONCLUSION: Clinical audit was regarded as a key tool in ensuring that justification becomes an effective, transparent and accountable part of normal radiological practice. In summary, justification would be facilitated by the "3 As": awareness, appropriateness and audit.


Asunto(s)
Diagnóstico por Imagen/métodos , Directrices para la Planificación en Salud , Protección Radiológica/métodos , Radiología/métodos , Anciano de 80 o más Años , Femenino , Humanos , Lactante , Recién Nacido , Agencias Internacionales , Masculino , Persona de Mediana Edad , Radiología/normas , Derivación y Consulta , Informe de Investigación , Riesgo
12.
Eur Arch Otorhinolaryngol ; 269(1): 113-9, 2012 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-21706322

RESUMEN

Septoplasty is a procedure often associated with high levels of patient dissatisfaction post-operatively. This study examined whether rhinospirometry and a modified 'valve-stabilised' technique could pre-operatively predict the outcome of septal surgery and therefore have a role as an objective tool for selection of patients suffering from nasal obstruction due to septal deformity for the procedure of septoplasty. A prospective study was performed of patients attending Singleton Hospital, Swansea, for surgical treatment of nasal obstruction due to septal deformity. Participants were asked to undertake decongested visual analogue scoring (VAS) and rhinospirometric assessment of their nasal obstruction in both normal 'resting' and 'valve-stabilised' nasal positioning. These investigations were undertaken on the day of surgery and 6 weeks post-operatively. Results were converted into nasal partition ratio scores and were assessed statistically for difference, correlation and accuracy. A total of 29 individual participants were included in the study. Septal surgery produced a statistically significant improvement in nasal airflow symmetry in the normal 'resting' nasal position using rhinospirometry (Wilcoxon ranked p > 0.5). This difference was not seen between evaluations in 'valve-stabilised' position (Wilcoxon p < 0.001). No statistical difference was evident between pre-operative 'valve-stabilised' testing and post-operative 'resting position'. The finding was also apparent on VAS nasal assessment. Correlation studies showed a strong relationship between pre-operative 'valve-stabilised' and post-operative 'resting' rhinospirometry results (Spearman's rho = 0. 586, p < 0.002). The strength of this relationship between VAS assessments was less pronounced (Spearman's rho = 0.386 p = 0.07). 'Valve-stabilised' rhinospirometry and VAS appear to be useful pre-operatively to predict the outcome of septal surgery, in terms of airflow symmetry, for nasal obstruction due to septal deformity, with pre-operative 'valve-stabilised' results correlating well with the post-operative standard 'resting' outcomes. Valve-stabilised rhinospirometry may have a role in pre-operatively predicting of likely post-operative patient satisfaction, although further studies are required in this area.


Asunto(s)
Obstrucción Nasal/fisiopatología , Tabique Nasal/cirugía , Espirometría/métodos , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Obstrucción Nasal/etiología , Obstrucción Nasal/cirugía , Tabique Nasal/anomalías , Selección de Paciente , Ventilación Pulmonar , Adulto Joven
13.
Spinal Cord ; 50(1): 42-50, 2012 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-21808258

RESUMEN

BACKGROUND: Disability scales do not enable the transmission of concise, meaningful and daily function description for clinical purposes. STUDY DESIGN: Cross-sectional statistical analysis of 328 patients' Spinal Cord Independence Measure (SCIM) III item scores (SIS). OBJECTIVE: To develop a concise and clinically interpretable data-based characterization of daily task accomplishment for patients with spinal cord lesions (SCLs). SETTING: Multi-center study at 13 spinal units in 6 countries. METHODS: Patients were grouped into clusters characterized by smaller differences between the patients' SIS within the clusters than between their centers, using the k-medoides algorithm. The number of clusters (k) was chosen according to the percent of SIS variation they explained and the clinical distinction between them. RESULTS: Analysis showed that k=8 SIS clusters offer a good description of the patient population. The eight functional clusters were designated as A-H, each cluster (grade) representing a combination of task accomplishments. Higher grades were usually (but not always) associated with patients implementing more difficult tasks. Throughout rehabilitation, the patients' functional grade improved and the distribution of patients with similar functional grades within the total SCIM III score deciles remained stable. CONCLUSIONS: A new classification based on SIS clusters enables a concise description of overall functioning and task accomplishment distribution in patients with SCL. A software tool is used to identify the patients' functional grade. Findings support the stability and utility of the grades for characterizing the patients' functional status.


Asunto(s)
Actividades Cotidianas/clasificación , Evaluación de la Discapacidad , Traumatismos de la Médula Espinal/rehabilitación , Adulto , Anciano , Algoritmos , Estudios Transversales , Incontinencia Fecal/etiología , Incontinencia Fecal/rehabilitación , Femenino , Humanos , Masculino , Persona de Mediana Edad , Cuadriplejía/etiología , Cuadriplejía/rehabilitación , Reproducibilidad de los Resultados , Estudios Retrospectivos , Traumatismos de la Médula Espinal/complicaciones
14.
PLoS One ; 6(9): e25501, 2011.
Artículo en Inglés | MEDLINE | ID: mdl-21980479

RESUMEN

BACKGROUND: Many bacteria undergo transitions between environments with differing O2 availabilities as part of their natural lifestyles and during biotechnological processes. However, the dynamics of adaptation when bacteria experience changes in O2 availability are understudied. The model bacterium and facultative anaerobe Escherichia coli K-12 provides an ideal system for exploring this process. METHODS AND FINDINGS: Time-resolved transcript profiles of E. coli K-12 during the initial phase of transition from anaerobic to micro-aerobic conditions revealed a reprogramming of gene expression consistent with a switch from fermentative to respiratory metabolism. The changes in transcript abundance were matched by changes in the abundances of selected central metabolic proteins. A probabilistic state space model was used to infer the activities of two key regulators, FNR (O2 sensing) and PdhR (pyruvate sensing). The model implied that both regulators were rapidly inactivated during the transition from an anaerobic to a micro-aerobic environment. Analysis of the external metabolome and protein levels suggested that the cultures transit through different physiological states during the process of adaptation, characterized by the rapid inactivation of pyruvate formate-lyase (PFL), a slower induction of pyruvate dehydrogenase complex (PDHC) activity and transient excretion of pyruvate, consistent with the predicted inactivation of PdhR and FNR. CONCLUSION: Perturbation of anaerobic steady-state cultures by introduction of a limited supply of O2 combined with time-resolved transcript, protein and metabolite profiling, and probabilistic modeling has revealed that pyruvate (sensed by PdhR) is a key metabolic signal in coordinating the reprogramming of E. coli K-12 gene expression by working alongside the O2 sensor FNR during transition from anaerobic to micro-aerobic conditions.


Asunto(s)
Técnicas de Cultivo , Ambiente , Escherichia coli K12/genética , Escherichia coli K12/metabolismo , Transcriptoma , Adaptación Fisiológica/efectos de los fármacos , Adaptación Fisiológica/genética , Aerobiosis , Anaerobiosis , Reactores Biológicos , Escherichia coli K12/efectos de los fármacos , Escherichia coli K12/crecimiento & desarrollo , Proteínas de Escherichia coli/metabolismo , Fermentación/efectos de los fármacos , Fermentación/genética , Proteínas Hierro-Azufre/metabolismo , Metaboloma/efectos de los fármacos , Metaboloma/genética , Oxígeno/farmacología , Ácido Pirúvico/metabolismo , ARN Mensajero/genética , ARN Mensajero/metabolismo , Proteínas Represoras/metabolismo , Factores de Tiempo
15.
Biochem Soc Trans ; 39(4): 961-5, 2011 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-21787330

RESUMEN

PLUNC (palate, lung and nasal epithelium clone) proteins make up the largest branch of the BPI (bactericidal/permeability-increasing protein)/LBP (lipopolysaccharide-binding protein) family of lipid-transfer proteins. PLUNCs make up one of the most rapidly evolving mammalian protein families and exhibit low levels of sequence similarity coupled with multiple examples of species-specific gene acquisition and gene loss. Vertebrate genomes contain multiple examples of genes that do not meet our original definition of what is required to be a member of the PLUNC family, namely conservation of exon numbers/sizes, overall protein size, genomic location and the presence of a conserved disulfide bond. This suggests that evolutionary forces have continued to act on the structure of this conserved domain in what are likely to be functionally important ways.


Asunto(s)
Variación Genética , Glicoproteínas/genética , Fosfoproteínas/genética , Secuencias de Aminoácidos , Animales , Exones , Glicoproteínas/química , Glicoproteínas/metabolismo , Humanos , Modelos Moleculares , Fosfoproteínas/química , Fosfoproteínas/metabolismo
16.
Biochem Soc Trans ; 39(4): 977-83, 2011 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-21787333

RESUMEN

We present the BPIFAn/BPIFBn systematic nomenclature for the PLUNC (palate lung and nasal epithelium clone)/PSP (parotid secretory protein)/BSP30 (bovine salivary protein 30)/SMGB (submandibular gland protein B) family of proteins, based on an adaptation of the SPLUNCn (short PLUNCn)/LPLUNCn (large PLUNCn) nomenclature. The nomenclature is applied to a set of 102 sequences which we believe represent the current reliable data for BPIFA/BPIFB proteins across all species, including marsupials and birds. The nomenclature will be implemented by the HGNC (HUGO Gene Nomenclature Committee).


Asunto(s)
Proteínas/clasificación , Secuencias de Aminoácidos , Animales , Humanos , Filogenia , Proteínas/genética , Homología de Secuencia de Aminoácido , Terminología como Asunto
17.
Eur Arch Otorhinolaryngol ; 268(10): 1469-74, 2011 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-21559810

RESUMEN

The objective of this study is to investigate the appropriateness of the NV1 rhinospirometer in the assessment of asymmetrical nasal airflow using a nasal cavity model. The Study is a laboratory-based basic-science study using an artificial model of nasal airflow. It is conducted in Medical Physics Department, Singleton Hospital, Swansea, Wales. A nasal cavity model was created with a series of parallel flow symmetry/asymmetries that were each assessed using standard flow volume measurements. The results were converted into Nasal Partition Ratios (NPRs) for each trial scenario and were examined against a mathematically calculated NPR derived using Pouseille's law. Experimental scenario results were assessed for correlation, accuracy and precision against the mathematically derived result. In this study 300 individual test scenarios were completed using 2 different flow volumes and 15 different symmetry/asymmetry combinations. Correlation of the attained results against the mathematically derived figure gave a very strong correlation, using Spearman's Rho = 0.975. Accuracy was excellent within one Standard deviation of the expected results. It was concluded that the NV1 rhinospirometer is an accurate and precise objective marker of airflow symmetry in the nasal cavity model giving strong correlation, accuracy, precision and reproducibility. The rhinospirometer, as a precision tool, has displayed potential to become an effective objective marker of nasal airflow in the assessment of nasal obstruction; however, clinical trials are required to examine whether the accurate results of this laboratory study are transferable to clinical practice.


Asunto(s)
Modelos Biológicos , Cavidad Nasal/fisiopatología , Obstrucción Nasal/diagnóstico , Rinomanometría/instrumentación , Espirometría/instrumentación , Diseño de Equipo , Espiración , Humanos , Obstrucción Nasal/fisiopatología , Reproducibilidad de los Resultados
18.
Spinal Cord ; 49(8): 893-6, 2011 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-21483443

RESUMEN

STUDY DESIGN: Multicenter international cohort study. OBJECTIVE: The objective of this study was to establish target values for Spinal Cord Independence Measure (SCIM) III scoring in rehabilitation for clinically complete spinal cord lesion (SCL) neurological levels. SETTING: In total, 13 spinal cord units in six countries from North America, Europe and the Middle East were taken. METHODS: Total SCIM III scores and gain at discharge from rehabilitation were calculated for SCL levels in 128 patients with American Spinal Injury Association Impairment Scale (AIS) grade A on admission to rehabilitation. RESULTS: Median, quartiles, mean and s.d., values of discharge SCIM III scores and SCIM III gain for the various SCL levels are presented. Total SCIM III scores and gain were significantly correlated with the SCL level (r=0.730, r=0.579, P<0.001). CONCLUSIONS: Calculated discharge SCIM III scores can be used as target values for functional achievements at various neurological levels in patients after AIS A SCL. They are generally, but not always, inversely correlated with SCL level.


Asunto(s)
Evaluación de la Discapacidad , Evaluación de Resultado en la Atención de Salud/métodos , Traumatismos de la Médula Espinal/complicaciones , Traumatismos de la Médula Espinal/diagnóstico , Adulto , Europa (Continente) , Humanos , Cooperación Internacional , Persona de Mediana Edad , Medio Oriente , Examen Neurológico , América del Norte , Recuperación de la Función/fisiología , Reproducibilidad de los Resultados , Traumatismos de la Médula Espinal/rehabilitación , Adulto Joven
19.
J Biol Chem ; 286(24): 21254-65, 2011 Jun 17.
Artículo en Inglés | MEDLINE | ID: mdl-21524997

RESUMEN

The PEB4 protein is an antigenic virulence factor implicated in host cell adhesion, invasion, and colonization in the food-borne pathogen Campylobacter jejuni. peb4 mutants have defects in outer membrane protein assembly and PEB4 is thought to act as a periplasmic chaperone. The crystallographic structure of PEB4 at 2.2-Å resolution reveals a dimer with distinct SurA-like chaperone and peptidyl-prolyl cis/trans isomerase (PPIase) domains encasing a large central cavity. Unlike SurA, the chaperone domain is formed by interlocking helices from each monomer, creating a domain-swapped architecture. PEB4 stimulated the rate of proline isomerization limited refolding of denatured RNase T(1) in a juglone-sensitive manner, consistent with parvulin-like PPIase domains. Refolding and aggregation of denatured rhodanese was significantly retarded in the presence of PEB4 or of an engineered variant specifically lacking the PPIase domain, suggesting the chaperone domain possesses a holdase activity. Using bioinformatics approaches, we identified two other SurA-like proteins (Cj1289 and Cj0694) in C. jejuni. The 2.3-Å structure of Cj1289 does not have the domain-swapped architecture of PEB4 and thus more resembles SurA. Purified Cj1289 also enhanced RNase T(1) refolding, although poorly compared with PEB4, but did not retard the refolding of denatured rhodanese. Structurally, Cj1289 is the most similar protein to SurA in C. jejuni, whereas PEB4 has most structural similarity to the Par27 protein of Bordetella pertussis. Our analysis predicts that Cj0694 is equivalent to the membrane-anchored chaperone PpiD. These results provide the first structural insights into the periplasmic assembly of outer membrane proteins in C. jejuni.


Asunto(s)
Proteínas Bacterianas/metabolismo , Campylobacter jejuni/metabolismo , Proteínas Portadoras/química , Proteínas de Escherichia coli/química , Chaperonas Moleculares/fisiología , Isomerasa de Peptidilprolil/química , Factores de Virulencia/metabolismo , Proteínas Bacterianas/genética , Proteínas Bacterianas/fisiología , Chaperoninas/química , Cristalografía por Rayos X/métodos , Genoma Bacteriano , Humanos , Interacciones Hidrofóbicas e Hidrofílicas , Espectroscopía de Resonancia Magnética/métodos , Chaperonas Moleculares/genética , Plásmidos/metabolismo , Conformación Proteica , Pliegue de Proteína , Propiedades de Superficie , Tiosulfato Azufretransferasa/química , Factores de Virulencia/genética , Factores de Virulencia/fisiología
20.
Spinal Cord ; 49(2): 292-6, 2011 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-20820178

RESUMEN

STUDY DESIGN: A multi-center international cohort study. OBJECTIVE: To evaluate the reliability and validity of the third version of the Spinal Cord Independence Measure (SCIM III), separately for patients with traumatic spinal cord lesions (SCLs). SETTING: A total of 13 spinal cord units in six countries from North America, Europe and the Middle-East. METHODS: SCIM III and Functional Independence Measure (FIM) were assessed for 261 patients with traumatic SCLs, on admission to rehabilitation and before discharge, by two raters. Conventional statistical measures were used to evaluate the SCIM III reliability and validity. RESULTS: In almost all SCIM III tasks, the total agreement between the paired raters was >80%. The κ coefficients were all >0.6 and statistically significant. Pearson's coefficients of the correlations between the paired raters were >0.9, the mean differences between raters were nonsignificant and the intraclass correlation coefficients (ICCs) were ≥ 0.95. Cronbach's α values for the entire SCIM III scale were 0.833-0.835. FIM and SCIM III total scores were correlated (r=0.84, P<0.001). SCIM III was more responsive to changes than FIM. In all subscales, SCIM III identified more changes in function than FIM, and in 3 of the 4 subscales, differences in responsiveness were statistically significant (P<0.02). CONCLUSION: The results confirm the reliability and validity of SCIM III for patients with traumatic SCLs in a number of countries.


Asunto(s)
Evaluación de la Discapacidad , Evaluación de Resultado en la Atención de Salud/métodos , Traumatismos de la Médula Espinal/diagnóstico , Traumatismos de la Médula Espinal/rehabilitación , Encuestas y Cuestionarios/normas , Actividades Cotidianas/clasificación , Adulto , Estudios de Cohortes , Femenino , Humanos , Vida Independiente/normas , Masculino , Persona de Mediana Edad , Adulto Joven
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