Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 108
Filtrar
1.
Front Pediatr ; 12: 1310317, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38425665

RESUMO

Introduction: Pompe disease (PD) is a glycogen disorder caused by the deficient activity of acid alpha-glucosidase (GAA). We sought to review the latest available evidence on the safety and efficacy of recombinant human GAA enzyme replacement therapy (ERT) for infantile-onset PD (IOPD). Methods: We systematically searched the MEDLINE (via PubMed) and Embase databases for prospective clinical studies evaluating ERT for IOPD on pre-specified outcomes. Meta-analysis was also performed. Results: Of 1,722 articles identified, 16 were included, evaluating 316 patients. Studies were heterogeneous and with very low certainty of evidence for most outcomes. A moderate/high risk of bias was present for most included articles. The following outcomes showed improvements associated with alglucosidase alfa, over natural history of PD/placebo, for a mean follow-up of 48.3 months: left ventricular (LV) mass {mean change 131.3 g/m2 [95% confidence interval (CI) 81.02, 181.59]}, time to start ventilation (TSV) [HR 0.21 (95% CI: 0.12, 0.36)], and survival [HR 0.10 (95% CI: 0.05, 0.19)]. There were no differences between the pre- and post-ERT period for myocardial function and psychomotor development. Adverse events (AEs) after ERT were mild in most cases. Conclusion: Our data suggest that alglucosidase alfa potentially improves LV mass, TSV, and survival in IOPD patients, with no important safety issues. Systematic Review Registration: PROSPERO identifier (CRD42019123700).

2.
BMC Cancer ; 23(1): 493, 2023 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-37264321

RESUMO

BACKGROUND: Over the past decade, therapeutic options in head and neck supraglottic squamous cell carcinoma have constantly evolved. The classical total laryngectomy has been partially replaced by alternative organ- and function-sparing techniques with the same prognosis but less morbidity, such as Radiotherapy, Transoral Laser Microsurgery (TLM) and Trans-Oral Robotic Surgery (TORS). Up to now, a prospective comparison of these innovant techniques has not been conducted. METHODS/DESIGN: We will conduct an original international multicentric prospective nonrandomized clinical trial to compare the efficacy between these treatments (Arm 1: Radiotherapy ± chemotherapy; Arm 2: TLM and Arm 3: TORS) with 4 classes of outcomes: quality of life (QoL), oncological outcomes, functional outcomes and economic resources. The population will include cT1-T2 /cN0-N1/M0 supraglottic squamous cell carcinoma. The primary outcome is a Clinical Dysphagia QoL evaluation assessed by the MD Anderson Dysphagia questionnaire. Secondary outcomes include others QoL evaluation, oncological and functional measures and cost parameters. The sample size needs to reach 36 patients per arm (total 108). DISCUSSION: In the current literature, no prospective head-to-head trials are available to compare objectively these different treatments. With the increase of highly efficient treatments and the increase of oncological survival, it is imperative also to develop management strategies that optimize QoL and functional results. We will conduct this innovate prospective trial in order to obtain objective data in these two main issues. TRIAL REGISTRATION: NCT05611515 posted on 10/11/2022 (clinicaltrial.fgov).


Assuntos
Carcinoma de Células Escamosas , Transtornos de Deglutição , Neoplasias de Cabeça e Pescoço , Neoplasias Laríngeas , Humanos , Qualidade de Vida , Carcinoma de Células Escamosas/cirurgia , Análise Custo-Benefício , Carcinoma de Células Escamosas de Cabeça e Pescoço/terapia , Resultado do Tratamento , Neoplasias Laríngeas/cirurgia
3.
Ann Oncol ; 31(3): 422-429, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-32067684

RESUMO

BACKGROUND: Long-term survival of high-risk neuroblastoma patients is still below 50% despite intensive multimodal treatment. This trial aimed to address whether the addition of two topotecan-containing chemotherapy courses compared to standard induction therapy improves event-free survival (EFS) of these patients. PATIENTS AND METHODS: An open-label, multicenter, prospective randomized controlled trial was carried out at 58 hospitals in Germany and Switzerland. Patients aged 1-21 years with stage 4 neuroblastoma and patients aged 6 months to 21 years with MYCN-amplified tumors were eligible. The primary endpoint was EFS. Patients were randomly assigned to standard induction therapy with six chemotherapy courses or to experimental induction chemotherapy starting with two additional courses of topotecan, cyclophosphamide, and etoposide followed by standard induction chemotherapy (eight courses in total). After induction chemotherapy, all patients received high-dose chemotherapy with autologous hematopoietic stem cell rescue and isotretinoin for consolidation. Radiotherapy was applied to patients with active tumors at the end of induction chemotherapy. RESULTS: Of 536 patients enrolled in the trial, 422 were randomly assigned to the control arm (n = 211) and the experimental arm (n = 211); the median follow-up time was 3.32 years (interquartile range 1.65-5.92). At data lock, the 3-year EFS of experimental and control patients was 34% and 32% [95% confidence Interval (CI) 28% to 40% and 26% to 38%; P = 0.258], respectively. Similarly, the 3-year overall survival of the patients did not differ [54% and 48% (95% CI 46% to 62% and 40% to 56%), respectively; P = 0.558]. The response to induction chemotherapy was not different between the arms. The median number of non-fatal toxicities per patient was higher in the experimental group while the median number of toxicities per chemotherapy course was not different. CONCLUSION: While the burden for the patients was increased by prolonging the induction chemotherapy and the toxicity, the addition of two topotecan-containing chemotherapy courses did not improve the EFS of high-risk neuroblastoma patients and thus cannot be recommended. CLINICAL TRIALS. GOV NUMBER: NCT number 03042429.


Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica , Quimioterapia de Indução , Neuroblastoma , Adolescente , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Criança , Pré-Escolar , Intervalo Livre de Doença , Alemanha , Humanos , Lactente , Neuroblastoma/tratamento farmacológico , Estudos Prospectivos , Suíça , Resultado do Tratamento , Adulto Jovem
4.
Eur Radiol ; 29(11): 5941-5949, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31041562

RESUMO

OBJECTIVE: To evaluate feasibility and diagnostic performance of multi-level calcium suppression in spectral detector computed tomography (SDCT) for assessment of bone metastasis. MATERIALS AND METHODS: Retrospective IRB-approved study on 21 patients who underwent SDCT (120 kV, reference mAs 116) and MRI. Thoracic and lumbar vertebrae (n = 357) were included and categorized as normal (n = 133) or metastatic (n = 203) based on MRI (STIR, T1w, ±contrast). The multi-level virtual non-calcium (VNCa) algorithm computes dynamic soft tissue/calcium pairs allowing for computation of different suppression index levels to address inter-individual variance of prevalent calcium composition weights. We computed images with low, medium, and high calcium suppression indices and compared them with conventional images (VNCa_low/med/high and conventional images (CI)). For quantitative image analysis, regions of interest were placed in normal and metastatic bone. Two readers reviewed the datasets independently in multiple sessions. They determined the presence of vertebral metastases on a per vertebra basis using a binary scale. Statistic assessment was performed using ANOVA with Tukey HSD, Student's T test, and ROC analysis. RESULTS: Attenuation of both normal and metastatic bone was lower in VNCa images than that in conventional images (e.g., CI/VNCa_low, - 46.3 to 238.8 HU/343.3-60.2 HU; p ≤ 0.05). VNCa_low+med improved separation of normal and metastatic bone in ROC analysis (AUC, CI/VNCa_low/VNCa_med = 0.74/0.95/0.98; p ≤ 0.05). In subjective analysis, both sensitivity and specificity were clearly improved in VNCa_low as compared with CI (0.85/0.84 versus 0.78/0.82). Readers showed a good inter-rater reliability (kappa = 0.65). CONCLUSIONS: Multi-level VNCa reconstructed from SDCT improve quantitative separation of normal and metastatic bone and subjective determination of bone metastases when using low to intermediate calcium suppression indices. KEY POINTS: • Spectral detector CT allows for multi-level calcium suppression in CT images and low and medium calcium suppression indices improved separation of normal and metastatic bone. • Thus, multi-level calcium suppression allows to optimize image contrast in regard to dedicated pathologies. • Low-level virtual non-calcium images (index 25-50) improved diagnostic performance regarding detection of metastasis.


Assuntos
Doenças da Medula Óssea/diagnóstico por imagem , Cálcio , Neoplasias da Coluna Vertebral/secundário , Tomografia Computadorizada por Raios X/métodos , Adulto , Algoritmos , Medula Óssea/diagnóstico por imagem , Métodos Epidemiológicos , Feminino , Humanos , Vértebras Lombares/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Masculino , Pessoa de Meia-Idade , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Vértebras Torácicas/diagnóstico por imagem
5.
Eur J Hosp Pharm ; 25(e1): e66-e69, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31157070

RESUMO

BACKGROUND: Potential inappropriate use of direct oral anticoagulants (DOACs) increases the risk of thromboembolic and haemorrhagic events. PURPOSE: To determine the net cost benefit of clinical pharmacy interventions on the prescription of DOACs. METHOD: We constructed a decision tree model using a public payer perspective. The appropriateness of the prescription was assessed using the Medication Appropriateness Index. The theoretical risks were collected from the literature and the individual potential risks were calculated using the Nesbit risk assignment conducted by two independent clinical pharmacists. Different costs were included based on diagnosis-related group coding and data in the literature. A univariate sensitivity analysis was performed. RESULTS: Thirty-six of 75 patients had an inappropriate prescription of DOACs. The saved difference between avoided costs (7954€) and annualised medication costs and pharmacist cost (4323€) was 3631€ for 75 patients. CONCLUSIONS: In addition to the enhancement of the quality of the prescription, our results indicate that pharmacist interventions provide a positive net cost benefit.

6.
Crit Rev Oncol Hematol ; 115: 59-66, 2017 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-28602170

RESUMO

BACKGROUND: The survival of colorectal cancer patients is frequently determined by the extent of metastatic invasion to the liver; in cases of major involvement, therapeutic strategies are limited because the liver is necessary for drug metabolism. MATERIAL AND METHODS: We have reviewed articles about the pharmacokinetic profiles of each drug used in colorectal cancer patients with hepatic dysfunction to determine which of these treatments are most feasible. RESULTS: Some drugs appear to be feasible options for patients with hepatic insufficiency. Agents such as 5-fluorouracil and oxaliplatin, as well as monoclonal antibodies such as bevacizumab, cetuximab, and panitumumab, can potentially be used in these cases. On the other hand, irinotecan and regorafenib cannot be recommended because of the risk of increased toxicity. CONCLUSION: Treatment of patients with colorectal cancer and liver dysfunction represents a major challenge because the prognosis is usually very poor and alteration of liver function is normally an exclusion criterion in clinical trials. In this review, we present evidence regarding the use of each drug in patients with colorectal cancer and hepatic impairment.


Assuntos
Antineoplásicos/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Colorretais/tratamento farmacológico , Neoplasias Colorretais/fisiopatologia , Neoplasias Hepáticas/tratamento farmacológico , Neoplasias Hepáticas/secundário , Antineoplásicos/efeitos adversos , Antineoplásicos/farmacocinética , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Protocolos de Quimioterapia Combinada Antineoplásica/farmacocinética , Neoplasias Colorretais/metabolismo , Neoplasias Colorretais/patologia , Humanos , Hepatopatias , Neoplasias Hepáticas/metabolismo
7.
Clin Neuroradiol ; 27(1): 39-42, 2017 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-26242291

RESUMO

INTRODUCTION: Bacterial contamination during angiographic procedures is a potential source of bacteremia. It is largely unknown whether it is clinically relevant. Our aim was to evaluate the incidence of contamination of liquids during catheter-based neuroangiographic examinations, the spectrum of microorganisms, a comparison of two different trolley-settings, and a follow-up of all patients with regard to clinical and lab signs of infection. METHODS: A total of 170 patients underwent either diagnostic angiography (n = 111) or arterial neuroendovascular procedures (n = 59). To study the impact of airborne contamination of sterile liquids, we randomly assigned equal numbers of procedures to two distinct setups. Group A with standard open-surface bowls and group B with repetitive coverage of liquids throughout the procedure. Patient preparation was performed with utmost care. After each procedure, samples of the liquids were sent for microbiological evaluation. Patients were followed for signs of infection (fever, white blood cell count, C-reactive-protein). RESULTS: Of all samples, 25.3 % were contaminated. Contamination consisted of resident skin microbiota only and was more common with procedures (28.8 %) than with diagnostic angiography (23.4 %) and less common in uncovered (23.5 %) than in covered bowls (27.1 %). However, these differences were insignificant. None of the patients developed clinical or lab signs of infection. CONCLUSION: Contamination during diagnostic and interventional angiography does occur and cannot be avoided by intermittent coverage. Despite a surprisingly high incidence, our findings support the common strategy that antibiotic coverage is unnecessary in most patients undergoing arterial angiography as it lacks clinical impact. Airborne contamination does not appear to play a role.


Assuntos
Infecções Bacterianas/epidemiologia , Infecções Bacterianas/microbiologia , Angiografia Cerebral/instrumentação , Contaminação de Equipamentos/estatística & dados numéricos , Radiografia Intervencionista/estatística & dados numéricos , Dispositivos de Acesso Vascular/microbiologia , Angiografia Cerebral/estatística & dados numéricos , Feminino , Alemanha/epidemiologia , Humanos , Masculino , Prevalência , Radiografia Intervencionista/instrumentação , Estudos Retrospectivos , Fatores de Risco , Dispositivos de Acesso Vascular/estatística & dados numéricos
8.
Z Gastroenterol ; 54(7): 642-6, 2016 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-27429101

RESUMO

PURPOSE: To investigate the effect of an early contrast-enhanced computed tomography (CECT) on clinical course and complications of acute pancreatitis (AP). MATERIAL AND METHODS: 58 patients with AP who had at least one CECT examination were analyzed retrospectively. Laboratory as well as clinical data, and results from the assessment of disease severity (CT severity index (CTSI) and its modified (MCTSI) version) were analyzed. The primary endpoint was the development of severe complications, defined as death, respiratory failure, acute renal failure, and the need for invasive interventions. Patients were divided into two groups: an early group (CECT within the first 48 h after the onset of symptoms, n = 32) and a late group (CECT > 48 h after the onset of symptoms, n = 26). Multivariate regression analysis was performed to identify risk factors for severe complications. RESULTS: There were no statistically significant differences between both groups concerning baseline characteristics, CTSI, and MCTSI. Complications occurred more often in the early CECT group (p = 0.008). Multivariate logistic regression analysis identified an early CECT and a severe MCTSI as independent risk factors for the occurrence of severe complications (p = 0.02 and p = 0.002, respectively). CONCLUSION: CECT performed within the first 48 h after the onset of symptoms is associated with an unfavorable outcome in AP.


Assuntos
Injúria Renal Aguda/mortalidade , Diagnóstico Precoce , Pancreatite/diagnóstico por imagem , Pancreatite/mortalidade , Insuficiência Respiratória/mortalidade , Tomografia Computadorizada por Raios X/estatística & dados numéricos , Doença Aguda , Injúria Renal Aguda/diagnóstico , Adulto , Idoso , Idoso de 80 Anos ou mais , Meios de Contraste , Progressão da Doença , Feminino , Alemanha/epidemiologia , Humanos , Tempo de Internação/estatística & dados numéricos , Masculino , Pessoa de Meia-Idade , Pancreatectomia/mortalidade , Pancreatectomia/estatística & dados numéricos , Pancreatite/cirurgia , Reprodutibilidade dos Testes , Insuficiência Respiratória/diagnóstico , Estudos Retrospectivos , Fatores de Risco , Sensibilidade e Especificidade , Taxa de Sobrevida , Adulto Jovem
9.
Technol Health Care ; 23(6): 847-54, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26409517

RESUMO

INTRODUCTION: Changes in pelvic position can influence the sagittal alignment of the lumbar spine. The restoration of hip kinematics by hip replacement thus appears to offer the possibility of correcting sagittal alignment. This preliminary retrospective study used EOS imaging to investigate the influence of total hip arthroplasty on pelvic parameters in patients with normal preoperative pelvic parameters. METHODS: Twenty patients with hip osteoarthritis undergoing total hip arthroplasty (THA) between 2011 and 2012 received unilateral THA. To evaluate the preoperative and postoperative changes of the pelvic parameters, we analyzed EOS imaging of the patients to determine pelvic incidence, sacral slope, sacral tilt, pelvic tilt, anterior pelvic plane inclination and pelvic axial rotation. Additionally, anteversion and inclination of the acetabular cup position were determined. RESULTS: No statistically significant difference was found between the preoperative and postoperative measurements of pelvic parameters, although the change in pelvic tilt approached significance. Postoperatively, respective average values of 42.6° and 22.7° were measured for inclination and anteversion of the acetabular cup position. CONCLUSION: THA did not influence pelvic position and sagittal alignment in patients with normal preoperative pelvic parameters. A subsequent study will investigate whether corrections of pelvic parameters outside the norm in patients with OA are possible with THA.


Assuntos
Artroplastia de Quadril , Vértebras Lombares/anatomia & histologia , Interpretação de Imagem Radiográfica Assistida por Computador/instrumentação , Acetábulo/anatomia & histologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Osteoartrite do Quadril/cirurgia , Postura , Estudos Retrospectivos
10.
Colorectal Dis ; 15(11): e627-33, 2013 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-24192263

RESUMO

AIM: Treatment of locally advanced rectal cancer (LARC) includes preoperative radiation therapy with or without chemotherapy followed by radical surgery, but the clinical outcome is uncertain. A systemic review was carried out to determine the predictive value of (18)F-fluoro-2-deoxyglucose positron emission tomography ((18)FDG-PET) for assessing disease-free (DFS) and overall survival (OS) in LARC. METHOD: A literature search (PubMed/MEDLINE, EMBASE, Cochrane) up to January 2012 to identify full papers with sequential (18)FDG-PET and survival data, using indexing terms and free text words. The inclusion criteria were: a study of at least 10 patients, having sequential (18)FDG-PET imaging before and after adjuvant chemoradiation and a minimal follow-up of 24 months. Studies were selected by two of the authors. A meta-analysis was performed for DFS and OS using the hazard ratio (HR) as the primary outcome. RESULTS: Five eligible studies were identified including 330 patients (mean age 63 years, 64% men), in which PET-CT or PET imaging was used. The American Joint Committee on Cancer stage distribution was as follows: Stage I, 2%; Stage II, 44%; Stage III, 52%; Stage IV, 1%. The pooled HRs for complete metabolic response versus partial or no response were 0.39 (95% CI 0.18-0.86; P = 0.02) for OS and 0.70 (95% CI 0.16-3.14; P = 0.64) for DFS. The lack of significance for DFS might be explained by different follow-up characteristics. There was also clinical heterogeneity among the different studies. CONCLUSION: This systematic review indicates that complete metabolic response on sequential (18)FDG-PET data after preoperative chemoradiation of LARC is predictive of OS, but not of DFS.


Assuntos
Tomografia por Emissão de Pósitrons , Neoplasias Retais/terapia , Quimiorradioterapia Adjuvante , Intervalo Livre de Doença , Fluordesoxiglucose F18 , Humanos , Estimativa de Kaplan-Meier , Terapia Neoadjuvante , Valor Preditivo dos Testes , Compostos Radiofarmacêuticos
11.
Minerva Anestesiol ; 79(5): 498-503, 2013 May.
Artigo em Inglês | MEDLINE | ID: mdl-23511349

RESUMO

BACKGROUND: Mechanically ventilated patients are prone to develop ventilator associated pneumonia due to microaspirations of subglottic secretions around the endotracheal tube cuff (usually constructed of polyvinyl material). A novel polyurethane cuff has been designed to minimize these leakages. The aim of the study was to compare the tracheal sealing capacities between the two tubes. METHODS: Twenty-nine consecutive patients from whom tracheal intubation was necessary as part of their care were randomized to receive either a polyvinyl HI-LO Evac® or a polyurethane SEALGUARD Evac® endotracheal tube. Patients requiring emergency intubation, with unstable hemodynamics or history of tracheal/laryngeal disease were excluded. For the entire study, cuff pressure was set at 30 cmH2O, and ventilator parameters were adjusted for a plateau pressure ≤30 cmH2O; Patients were fasting, placed in a strict 45° position during 12 hours and sedated if needed. After injection of 74 MBq 99mTc-DTPA diluted in 5 mL 0.9% NaCl just above the cuff, tracheal radioactivity was assessed sequentially (hourly from T0 to T6, then T8 and T12 hours) using a scintillation camera. RESULTS: Sixteen polyurethane and 13 polyvinyl tubes were compared. Leakages were observed in 11/29 patients (38%) (5/16 polyurethane and 6/13 polyvinyl tubes [P=NS]). Leakages occurred more often in female (7/8) than in male patients (4/21) (P<0.001). Microaspirations were decreased with larger tubes (size 9 vs. ≤8.5: 24% vs. 75%; P=0.01), whatever the cuff membrane. CONCLUSION: These preliminary results suggest that both tubes are poorly effective in preventing microaspirations.


Assuntos
Intubação Intratraqueal/instrumentação , Manufaturas , Pneumonia Aspirativa/prevenção & controle , Poliuretanos , Polivinil , Adulto , Idoso , Método Duplo-Cego , Feminino , Humanos , Processamento de Imagem Assistida por Computador , Intubação Intratraqueal/métodos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Cintilografia , Traqueia/diagnóstico por imagem
12.
Eur J Radiol ; 82(4): 608-16, 2013 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-23265182

RESUMO

BACKGROUND: Our rationale was to evaluate whether a 64-slice CT scanner allows accurate measurement of computed tomographic (CT) changes in coronary artery flow profiles and whether CT flow measurements are suitable for classifying the significance and hemodynamic relevance of a stenosis and thereby supplement as a functional parameter for morphological stenosis analysis. METHODS: A total of 50 patients prospectively underwent computed tomography coronary angiography (coronary CTA) in a multidetector CT scanner (Brilliance 64, Philips)±1 day before or after invasive coronary angiography (ICA). Immediately thereafter, 2 radiologists reviewed the imaging data to detect any vessel segments with morphology poorly evaluable by coronary CTA. A locally constant cyclical measurement was acquired in these coronary arteries in breath-hold technique during the passage of a 50ml bolus of contrast media. For analysis, time-density curves of the bolus passage were registered in the coronary artery and the aorta (internal reference), the up-slopes were determined and correlated with each other. The results were compared with the ICA findings. RESULTS: 47 of 50 CT flow measurements were evaluable. A good correlation was found between the degrees of stenosis and slope ratios in aorta and coronary artery (R(2)=0.92). The threshold corridor was 0.55-0.77 for distinguishing hemodynamically (≥70%) from non-hemodynamically relevant stenoses. CONCLUSIONS: CT-based coronary artery flow measurements (CTFM) correlate well with the angiographically determined degree of stenosis and can elevate by non-invasive means the diagnostic accuracy of coronary CTA. From both a clinically diagnostic and scientific standpoint, CTFM proves a suitable method for quantifying coronary blood flow.


Assuntos
Angiografia Coronária/métodos , Estenose Coronária/diagnóstico por imagem , Tomografia Computadorizada por Raios X/métodos , Idoso , Velocidade do Fluxo Sanguíneo , Meios de Contraste , Feminino , Humanos , Iopamidol/análogos & derivados , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos
13.
Public Health Genomics ; 13(1): 27-33, 2010.
Artigo em Inglês | MEDLINE | ID: mdl-19407439

RESUMO

OBJECTIVE: Gaucher disease (GD) is a genetic disease caused by glucocerebrosidase deficiency. GD is treated by enzyme replacement therapy (ERT) with imiglucerase, a high-cost drug provided by the Brazilian Ministry of Health (BMH). This study reports the implementation of the BMH guidelines for GD in the southernmost state of the country. METHODS: We review the clinical and laboratorial data for patients seen at the reference center for GD from Rio Grande do Sul, Brazil (July 2003 to June 2006). RESULTS: Twenty-five patients were included in this study. At baseline, 19/20 were on ERT (mean dosage of imiglucerase = 51.8 U/kg/infusion), 3/17 presented anemia, and 5/16 thrombocytopenia. The amount of imiglucerase prescribed to these patients was adjusted according to the guidelines in July 2003; out of them, 18 were receiving ERT in the reference center at month 36 (mean dosage of imiglucerase = 27.5 U/kg/infusion), 2/18 presented anemia, and 4/18 presented thrombocytopenia. The analysis of the liver, spleen, and bone data presented some limitations, but the available information suggests that patients did not deteriorate. GD patients who initiated ERT after July 2003 (n = 5) received lower dosage of imiglucerase since the beginning of the treatment; most of them demonstrated clinical and laboratorial response. From baseline to month 36, the consumption of imiglucerase by the reference center showed a significant reduction, which represented savings of USD 3 million to the public health system. CONCLUSIONS: The model of care of GD patients suggested by the BMH guidelines appears to be cost-effective and could be an example for management of rare diseases in underdeveloped countries.


Assuntos
Terapia de Reposição de Enzimas , Doença de Gaucher/tratamento farmacológico , Glucosilceramidase/uso terapêutico , Adolescente , Adulto , Brasil , Criança , Gerenciamento Clínico , Feminino , Seguimentos , Humanos , Fígado/efeitos dos fármacos , Fígado/patologia , Masculino , Pessoa de Meia-Idade , Cooperação do Paciente , Guias de Prática Clínica como Assunto , Baço/efeitos dos fármacos , Baço/patologia , Inquéritos e Questionários
15.
Psychol Med ; 38(6): 843-51, 2008 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-18387213

RESUMO

BACKGROUND: Verbal learning and memory deficits are frequent among patients with schizophrenia and correlate with reduced magnetic resonance imaging (MRI) volumes of the hippocampus in these patients. A crucial question is the extent to which interrelated structural-functional deficits of the hippocampus reflect a vulnerability to schizophrenia, as opposed to the disorder per se. METHOD: We combined brain structural measures and the Rey Auditory Verbal Learning Test (RAVLT) to assess hippocampal structure and function in 36 never-medicated individuals suspected to be in early (EPS) or late prodromal states (LPS) of schizophrenia relative to 30 healthy controls. RESULTS: Group comparisons revealed bilaterally reduced MRI hippocampal volumes in both EPS and LPS subjects. In LPS subjects but not in EPS subjects, these reductions were correlated with poorer performance in RAVLT delayed recall. CONCLUSIONS: Our findings suggest progressive and interrelated structural-functional pathology of the hippocampus, as prodromal symptoms and behaviours accumulate, and the level of risk for psychosis increases. Given the inverse correlation of learning and memory deficits with social and vocational functioning in established schizophrenia, our findings substantiate the rationale for developing preventive treatment strategies that maintain cognitive capacities in the at-risk mental state.


Assuntos
Hipocampo/fisiopatologia , Transtornos da Memória/fisiopatologia , Testes Neuropsicológicos/estatística & dados numéricos , Esquizofrenia/fisiopatologia , Psicologia do Esquizofrênico , Transtorno da Personalidade Esquizotípica/fisiopatologia , Adulto , Estudos Transversais , Progressão da Doença , Dominância Cerebral/fisiologia , Feminino , Hipocampo/patologia , Humanos , Processamento de Imagem Assistida por Computador , Imageamento por Ressonância Magnética , Masculino , Programas de Rastreamento , Transtornos da Memória/diagnóstico , Transtornos da Memória/psicologia , Rememoração Mental/fisiologia , Escalas de Graduação Psiquiátrica/estatística & dados numéricos , Psicometria/estatística & dados numéricos , Reprodutibilidade dos Testes , Retenção Psicológica/fisiologia , Fatores de Risco , Esquizofrenia/diagnóstico , Transtorno da Personalidade Esquizotípica/diagnóstico , Aprendizagem Verbal/fisiologia
16.
Vasa ; 36(4): 282-4, 2007 Nov.
Artigo em Alemão | MEDLINE | ID: mdl-18357922

RESUMO

We report on a 66-years-old oncological patient with a spontaneous dislocation of a portcatheter into the pulmonary artery. The catheter fragment had a loop formation, both ends were located in the proximal right segment arteries. The first attempt of a removal of the catheter via the right heart into the inferior caval vein by means of a hooked pulmonalis-catheter failed. In a second manoeuvre, the catheter fragment was successfully fixed by means of a catheter with an extendable loop configuration (goose-neck) and retrieved from the pulmonal artery into the inferior caval vein. Then, the entire system could be removed together with the introducer sheet under mild traction from the femoral vein without any complications. In conclusion, the percutaneous interventional method appears to be a minimally-invasive approach to deal with catheter fragments dislocated in the central veins and the pulmonary arteries.


Assuntos
Carcinoma/tratamento farmacológico , Cateterismo Venoso Central/instrumentação , Cateteres de Demora , Neoplasias Esofágicas/tratamento farmacológico , Corpos Estranhos/terapia , Artéria Pulmonar , Idoso , Angiografia , Cateterismo de Swan-Ganz , Corpos Estranhos/diagnóstico por imagem , Humanos , Masculino , Terapia Neoadjuvante , Embolia Pulmonar/diagnóstico por imagem , Embolia Pulmonar/etiologia , Embolia Pulmonar/terapia , Tomografia Computadorizada por Raios X
17.
Rofo ; 177(7): 955-61, 2005 Jul.
Artigo em Alemão | MEDLINE | ID: mdl-15973597

RESUMO

PURPOSE: To compare the image quality of digital X-ray mammographies obtained with wet imagers with that of standard dry imaging technology. MATERIAL AND METHODS: Beginning 03/08/2003, 200 X-ray mammographies with a digital fullfield mammography system (Lorad Selenia, Lorad/Hologic) were prospectively and consecutively documented with a wet laser imager (Scopix LR 5200, Agfa), a dry infrared laser imager (DryView 8610, Kodak) and a dry imager using the principle of direct thermography (Drystar 4500M, Agfa, N = 166). One X-ray exposure was systematically chosen from each examination and was presented in an anonymous and randomized form to three radiologists who evaluated the films using a structured questionnaire. RESULTS: The visualization of normal anatomic structures was considered being good to excellent for all imagers with the mean assessments 1.0 - 2.4 for the Drystar 4500M, 1.0 - 2.1 for the DryView 8610 and 1.1 - 2.0 for the Scopix LR 5200. The mean assessments were 0.1 - 0.6 points lower in dense than in normal parenchyma, thus, the parenchymal density is the predominant factor for image quality. CONCLUSION: In view of the comparable image quality obtained with the different imagers used in the study, individual decisions to purchase a specific imager will be based on economics rather than on diagnostic points of view.


Assuntos
Periféricos de Computador , Análise de Falha de Equipamento , Lasers , Mamografia/instrumentação , Impressão/instrumentação , Intensificação de Imagem Radiográfica/instrumentação , Desenho de Equipamento , Humanos , Mamografia/métodos , Papel , Intensificação de Imagem Radiográfica/métodos , Reprodutibilidade dos Testes , Sensibilidade e Especificidade , Termografia/instrumentação
18.
Acta Anaesthesiol Scand ; 49(6): 784-91, 2005 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-15954960

RESUMO

BACKGROUND: Functional magnetic resonance imaging (fMRI) using blood-oxygen-level-dependent (BOLD) contrasts is a common method for studying sensory or cognitive brain functions. The aim of the present study was to assess the effect of the intravenous anaesthetic propofol on auditory-induced brain activation using BOLD contrast fMRI. METHODS: In eight neurosurgical patients, musical stimuli were presented binaurally in a block design. Imaging was performed under five conditions: no propofol (or wakefulness) and propofol plasma target concentrations of 0.5, 1.0, 1.5, and 2.0 microg ml(-1). RESULTS: During wakefulness we found activations in the superior temporal gyrus (STG) corresponding to the primary and secondary auditory cortex as well as in regions of higher functions of auditory information processing. The BOLD response decreased with increasing concentrations of propofol but remained partially preserved in areas of basic auditory processing in the STG during propofol 2.0 microg ml(-1). CONCLUSIONS: Our results suggest a dose-dependent impairment of central processing of auditory information after propofol administration. These results are consistent with electrophysiological findings measuring neuronal activity directly, thus suggesting a dose-dependent impairment of central processing of auditory information after propofol administration. However, propofol did not totally blunt primary cortical responses to acoustic stimulation, indicating that patients may process auditory information under general anaesthesia.


Assuntos
Anestesia Intravenosa , Anestésicos Intravenosos , Córtex Auditivo/efeitos dos fármacos , Propofol , Estimulação Acústica , Adulto , Anestesia Geral , Anestésicos Intravenosos/sangue , Neoplasias Encefálicas/cirurgia , Interpretação Estatística de Dados , Relação Dose-Resposta a Droga , Feminino , Lateralidade Funcional/fisiologia , Hemodinâmica , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Procedimentos Neurocirúrgicos , Oxigênio/sangue , Propofol/sangue
19.
Rofo ; 177(1): 130-6, 2005 Jan.
Artigo em Alemão | MEDLINE | ID: mdl-15657832

RESUMO

PURPOSE: To assess the diagnostic value of postmortem computed tomography (CT) in comparison to autopsy. MATERIALS AND METHODS: Twenty-seven cadavers were examined by sequential cranial CT and helical CT through the neck, thorax and abdomen and subsequently underwent an autopsy with histomorphologic examination of the pathologic specimens. The findings of CT, autopsy and histology were registered and compared by three radiologists and one specialist for forensic medicine, using a data entry form. RESULTS: In 19 of 27 cases, the findings explaining the cause of death were concordant for CT and autopsy. Intracranial, intraspinal and intracardiac gas accumulations (n = 12) were registered by CT alone. The detection of skull fractures was equal for both methods (n = 3). CT showed diagnostic problems in the assessment of pneumonic infiltrations (n = 16) and pulmonary edema (n = 21). CONCLUSION: CT is a useful and complementary method to autopsy.


Assuntos
Autopsia , Causas de Morte , Medicina Legal , Mudanças Depois da Morte , Tomografia Computadorizada por Raios X , Adulto , Idoso , Idoso de 80 Anos ou mais , Cadáver , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Pneumonia/diagnóstico por imagem , Pneumonia/patologia , Estudos Prospectivos , Edema Pulmonar/diagnóstico por imagem , Edema Pulmonar/patologia , Fraturas Cranianas/diagnóstico por imagem , Fraturas Cranianas/patologia
20.
Acta Radiol ; 45(6): 641-5, 2004 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-15587422

RESUMO

PURPOSE: To investigate the diagnostic yield of core needle biopsy in patients with malignant lymphoma. MATERIAL AND METHODS: Computed-tomography-guided core needle biopsies in patients with malignant lymphoma performed in the period 1996 to 2001 were evaluated retrospectively. A biopsy was considered as "fully diagnostic" if a histological diagnosis, including the histologic subtype in the event of malignant lymphoma, was achieved and the clinical course and CT follow-up of at least 6 months confirmed the biopsy results. A biopsy was regarded as "partly diagnostic" if histological work-up defined malignant lymphoma but not the histological subtype, and if histological diagnosis bore therapeutic relevance. Diagnostic yield was correlated with features such as size of specimen, location and depth of the target lesion, and experience of the investigator. RESULTS: 45 biopsies were performed in 40 patients. With respect to definite histopathological diagnosis, 31 biopsies (68.9%) were diagnostic and 14 (31.1%) non-diagnostic. In 4 cases (8.8%), biopsies yielded partly diagnostic results, since therapy could be scheduled after biopsy without final sub-classification. Statistical analysis of biopsy parameters revealed that sample sizes were significantly larger in the diagnostic group. CONCLUSION: CT-guided biopsy can be considered as an alternative for lymphoma diagnosis and should be the first interventional procedure. The most important parameter for diagnostic success is the size of the specimen.


Assuntos
Biópsia por Agulha/métodos , Doença de Hodgkin/patologia , Linfoma não Hodgkin/patologia , Tomografia Computadorizada por Raios X , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA
...