RESUMO
BACKGROUND: In chronic obstructive pulmonary disease (COPD), there is a poor correlation between forced expiratory volume in 1 s (FEV1) and dyspnea following bronchodilator use. Better correlations have been observed between inspiratory lung function parameters (ILPs) and dyspnea, which drives our interest in ILPs. However, the acute and prolonged effects of long-acting bronchodilators and oral corticosteroids on ILPs have not been well investigated. Therefore, the aim of this study was to investigate the effects of these treatments on the ILPs, FEV1, dyspnea (visual analog scale (VAS)) and clinical COPD questionnaire (CCQ). METHODS: Twenty-eight stable COPD patients had their ILPs and FEV1 measured both before and 2 h after the use of a single dose of 18 mcg bronchodilator tiotropium and 50 mcg salmeterol. Thereafter, the patients were randomized to 2 weeks of treatment with 30 mg oral prednisolone once daily or oral placebo in combination with daily treatment with these two bronchodilators. Four weeks after the cessation of the randomized treatment, the ILPs and FEV1 were again measured. After each intervention, any change in the VAS score was assessed. RESULTS: With both bronchodilators, significant improvements in ILPs were demonstrated (p < 0.005), with the exception of changes in ILPs inspiratory capacity (IC) and forced inspiratory flow at 50% of the vital capacity (FIF50) after tiotropium inhalation. After 2 weeks of treatment with prednisolone, significant differences were found for ILP forced inspiratory volume in 1 s (FIV1) and FEV1 compared with placebo. These differences were no longer present 4 weeks after the cessation of prednisolone. Significant relationships between ILPs and VAS scores were only found after 2 weeks of treatment with prednisolone or placebo. CONCLUSIONS: After a single dose of long-acting bronchodilator salmeterol, significant improvements are observed in all ILPs and in FIV1 and PIF after tiotropium. Two weeks of oral corticosteroid treatment improved the FIV1 and FEV1. The dyspnea VAS score was only significantly correlated with the ILPs after 2 weeks of oral corticosteroid treatment.
Assuntos
Broncodilatadores/farmacologia , Dispneia/tratamento farmacológico , Prednisolona/farmacologia , Doença Pulmonar Obstrutiva Crônica/tratamento farmacológico , Administração Oral , Idoso , Idoso de 80 Anos ou mais , Albuterol/administração & dosagem , Albuterol/análogos & derivados , Albuterol/farmacologia , Albuterol/uso terapêutico , Broncodilatadores/administração & dosagem , Broncodilatadores/uso terapêutico , Preparações de Ação Retardada , Método Duplo-Cego , Dispneia/etiologia , Feminino , Volume Expiratório Forçado/efeitos dos fármacos , Glucocorticoides/administração & dosagem , Glucocorticoides/farmacologia , Glucocorticoides/uso terapêutico , Humanos , Capacidade Inspiratória/efeitos dos fármacos , Masculino , Pessoa de Meia-Idade , Prednisolona/administração & dosagem , Prednisolona/uso terapêutico , Doença Pulmonar Obstrutiva Crônica/fisiopatologia , Testes de Função Respiratória , Xinafoato de Salmeterol , Derivados da Escopolamina/administração & dosagem , Derivados da Escopolamina/farmacologia , Derivados da Escopolamina/uso terapêutico , Inquéritos e Questionários , Fatores de Tempo , Brometo de TiotrópioRESUMO
OBJECTIVE: To investigate the stability throughout the day of the protein to creatinine ratio (PCR) in spot urine, to demonstrate whether the PCR is a valid alternative for 24-hour protein investigation in pregnant women. DESIGN: Prospective study. SETTING: Tertiary referral university centre. POPULATION: Women suspected of having pre-eclampsia, admitted to the Erasmus Medical Centre. METHODS: Twenty-four-hour urine collections and simultaneously three single voided 5-ml aliquots were obtained at 8 a.m., 12 a.m. (noon) and 5 p.m. A PCR was measured in each specimen and compared with the 24-hour protein excretion. MAIN OUTCOME MEASURES: The 24-hour proteinuria and PCR measured in spontaneous voids. RESULTS: The PCRs correlated strongly with each other and with the 24-hour protein excretion but did show variation throughout the day (mean coefficient of variation 36%; 95% confidence interval 31-40%). The coefficient of variation was unrelated to the degree of 24-hour proteinuria. Receiver operating characteristics curves to discriminate between values below and greater than or equal to the threshold of 0.3 g protein per 24-hour had an area under the curve of respectively 0.94 (8 a.m.), 0.96 (noon) and 0.97 (5 p.m.). Sensitivities at 8 a.m., noon and 5 p.m. were respectively 89%, 96% and 94%; specificities were 75%, 78% and 78% with the proposed PCR cut-off of 30 mg/mmol (0.26 g/g) (National Institute for Health and Care Excellence guidelines).There is no evidence of a difference between the three measurement times regarding the sensitivities and specificities. CONCLUSION: The PCR determined in spot urine varies throughout the day but is a valid alternative for 24-hour urine collections in pregnant women. It is especially useful to rapidly identify clinically relevant proteinuria.
Assuntos
Ritmo Circadiano , Creatinina/urina , Pré-Eclâmpsia/urina , Proteinúria , Coleta de Urina/métodos , Adulto , Feminino , Humanos , Pré-Eclâmpsia/diagnóstico , Gravidez , Estudos Prospectivos , Curva ROC , Sensibilidade e EspecificidadeRESUMO
OBJECTIVE: To compare microcirculatory perfusion in women with severe pre-eclampsia against that in healthy pregnant women, and secondly in women with severe pre-eclampsia with or without HELLP syndrome (haemolysis, elevated liver enzymes, and low platelets). DESIGN: Case-control study. SETTING: University Hospital Rotterdam, the Netherlands. POPULATION: Twenty-three women with severe pre-eclampsia and 23 healthy pregnant controls, matched for maternal and gestational age. Out of the 23 women with severe pre-eclampsia, ten presented with HELLP syndrome. METHODS: Microcirculation was analysed sublingually by a non-invasive sidestream dark-field imaging device (SDF). MAIN OUTCOME MEASURES: Perfused vessel density (PVD), microcirculatory flow index (MFI), and heterogeneity index (HI) were calculated for both small vessels (∅ < 20 µm; capillaries) and non-small vessels (∅ > 20 µm; venules and arterioles). RESULTS: There were no significant differences between women with severe pre-eclampsia and healthy controls. Women with pre-eclampsia and HELLP syndrome showed a reduced PVD (P = 0.045), MFI (P = 0.008), and increased HI (P = 0.002) for small vessels, as compared with women with pre-eclampsia but without HELLP syndrome. CONCLUSIONS: Sidestream dark-field is a novel, promising technique in obstetrics that permits the non-invasive evaluation of microcirculation. We did not observe major differences in sublingual microcirculatory perfusion between women with severe pre-eclampsia and healthy pregnant controls. In women with severe pre-eclampsia, the presence of HELLP syndrome is characterised by impaired capillary perfusion.
Assuntos
Síndrome HELLP/fisiopatologia , Microcirculação/fisiologia , Microvasos/fisiologia , Soalho Bucal/irrigação sanguínea , Pré-Eclâmpsia/fisiopatologia , Adulto , Estudos de Casos e Controles , Feminino , Humanos , Países Baixos , GravidezRESUMO
OBJECTIVE: To assess the effect of red blood cell (RBC) transfusion on quality of life in acutely anaemic women after postpartum haemorrhage. DESIGN: Randomised non-inferiority trial. SETTING: Thirty-seven Dutch university and general hospitals. POPULATION: Women with acute anaemia (haemoglobin 4.8-7.9 g/dl [3.0-4.9 mmol/l] 12-24 hours postpartum) without severe anaemic symptoms or severe comorbidities. METHODS: Women were allocated to RBC transfusion or non-intervention. MAIN OUTCOME MEASURES: Primary outcome was physical fatigue 3 days postpartum (Multidimensional Fatigue Inventory, scale 4-20; 20 represents maximal fatigue). Non-inferiority was demonstrated if the physical fatigue difference between study arms was maximal 1.3. Secondary outcomes were health-related quality of life and physical complications. Health-related quality of life questionnaires were completed at five time-points until 6 weeks postpartum. RESULTS: In all, 521 women were randomised to non-intervention (n = 262) or RBC transfusion (n = 259). Mean physical fatigue score at day 3 postpartum, adjusted for baseline and mode of delivery, was 0.8 lower in the RBC transfusion arm (95% confidence interval: 0.1-1.5, P = 0.02) and at 1 week postpartum was 1.06 lower (95% confidence interval: 0.3-1.8, P = 0.01). A median of two RBC units was transfused in the RBC transfusion arm. In the non-intervention arm, 33 women received RBC transfusion, mainly because of anaemic symptoms. Physical complications were comparable. CONCLUSIONS: Statistically, non-inferiority could not be demonstrated as the confidence interval crossed the non-inferiority boundary. Nevertheless, with only a small difference in physical fatigue and no differences in secondary outcomes, implementation of restrictive management seems clinically justified.
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Anemia/terapia , Transfusão de Eritrócitos/normas , Fadiga/terapia , Bem-Estar Materno , Hemorragia Pós-Parto/terapia , Adulto , Anemia/etiologia , Fadiga/etiologia , Feminino , Seguimentos , Hospitais Gerais , Hospitais Universitários , Humanos , Países Baixos , Guias de Prática Clínica como Assunto , Qualidade de Vida , Medição de Risco , Índice de Gravidade de Doença , Resultado do TratamentoRESUMO
Little is known about long-term effects of neonatal intensive care on exercise capacity, physical activity, and fatigue in term borns. We determined these outcomes in 57 young adults, treated for neonatal respiratory failure; 27 of them had congenital diaphragmatic hernia with lung hypoplasia (group 1) and 30 had normal lung development (group 2). Patients in group 2 were age-matched, with similar gestational age and birth weight, and similar neonatal intensive care treatment as patients in group 1. All patients were born before the era of extracorporeal membrane oxygenation, nitric oxide administration, and high frequency ventilation. Exercise capacity was measured by cycle ergometry, daily physical activity with an accelerometry-based activity monitor, and fatigue by the fatigue severity scale. Median (range) VO2peak in mL/kg/min was 35.4 (19.6-55.0) in group 1 and 37.6 (15.7-52.7) in group 2. There was a between-group P-value of 0.65 for exercise capacity. Daily activity and fatigue were also similar in both groups. So, residual lung hypoplasia did not play an important role in this cohort. There were no significant associations between exercise capacity and perinatal characteristics. Future studies need to elucidate whether exercise capacity is impaired in patients with more severe lung hypoplasia who nowadays survive.
Assuntos
Tolerância ao Exercício/fisiologia , Fadiga/fisiopatologia , Hérnias Diafragmáticas Congênitas , Atividade Motora/fisiologia , Consumo de Oxigênio/fisiologia , Síndrome do Desconforto Respiratório do Recém-Nascido/fisiopatologia , Acelerometria , Adulto , Estudos de Casos e Controles , Teste de Esforço , Feminino , Seguimentos , Hérnia Diafragmática/complicações , Hérnia Diafragmática/fisiopatologia , Humanos , Masculino , Síndrome do Desconforto Respiratório do Recém-Nascido/etiologia , Testes de Função Respiratória , Sobreviventes , Adulto JovemRESUMO
STUDY QUESTION: Is it possible to evaluate first trimester brain ventricle development in human pregnancies using an innovative virtual reality (VR) application and to analyze the relation of the embryonic volume (EV) and brain ventricle fluid volume (BVFV) with gestational age (GA), crown-rump length (CRL) and the Carnegie stage? SUMMARY ANSWER: Volumetry and staging of the human embryo using a VR application make it possible to obtain unique information about in-vivo embryonic normal and abnormal development and about the sizes of the ventricles and body. WHAT IS KNOWN ALREADY: Human brain development is complex and has a rapidly changing anatomy during the first trimester of pregnancy. New insights will enable early detection of cerebral pathology. STUDY DESIGN, SIZE, DURATION: In a prospective cohort study, we weekly performed three-dimensional (3D) ultrasound examinations in 112 uncomplicated pregnancies between 6 + 0 and 12 + 6 weeks GA. MATERIALS, SETTING, METHODS: The examinations resulted in 696 3D ultrasound scans that were transferred to the I-Space VR system and analyzed using V-Scope volume rendering software. V-Scope is used to create a 'hologram' of the ultrasound image and allows depth perception and interaction with the rendered objects. The CRL measurements were performed with a tracing tool, and the volume measurements were automatically performed with a segmentation algorithm. The embryos were staged according to the internal and external characteristics of the Carnegie staging system. All longitudinal outcomes were analyzed using repeated measures ANOVA. MAIN RESULTS AND THE ROLE OF CHANCE: CRL could be measured in 91% of the datasets and ranged from 2.5 to 79.0 mm. EV could be measured in 66% of the datasets and ranged from 2.4 to 23 812.0 mm³, whereas the BVFV could be measured in 38% of the datasets and ranged from 10.4 to 226.3 mm³. Finally, in 74% of the datasets, the embryos were staged according to the Carnegie criteria, starting as early as stage 12. Reference charts of volumes versus GA, CRL and stage were constructed. There was no significant relationship between the CRL or EV and the birthweight. LIMITATIONS, REASONS FOR CAUTIONS: The low success rate is a limitation of this study that can be explained mainly by non-targeted scanning of the embryonic head. WIDER IMPLICATIONS OF THE FINDINGS: The I-Space VR system and the V-Scope software enable automatic EV and BVFV measurements and 3D observations of embryonic development in the first trimester. This allows in-vivo staging of human embryos based on both internal and external morphological characteristics. STUDY FUNDING, COMPETING INTERESTS: None.
Assuntos
Encéfalo/embriologia , Ventrículos Cerebrais/embriologia , Primeiro Trimestre da Gravidez , Ultrassonografia Pré-Natal , Adulto , Algoritmos , Ventrículos Cerebrais/patologia , Estatura Cabeça-Cóccix , Feminino , Idade Gestacional , Humanos , Processamento de Imagem Assistida por Computador , Imageamento Tridimensional , Recém-Nascido , Gravidez , Estudos Prospectivos , Software , Fatores de Tempo , Interface Usuário-Computador , Adulto JovemRESUMO
BACKGROUND: Although widely fragmented BMs have been associated with adverse outcome in several cancer types, comparatively little is known with respect to its effect on the prognosis of pancreatic cancer. The aim of the current study was therefore to determine the prognostic value of tumour basement membrane (BM) continuity in two anatomically closely related, however, prognostically different tumours, pancreatic head- and periampullary cancer. METHODS: Tumour BM continuity was determined by immunohistochemical staining of its two major components, laminin and collagen type IV. Associations were made with recurrence free survival (RFS), cancer-specific survival (CSS), overall survival (OS) and conventional prognostic factors. RESULTS: Fifty-nine and 61% of pancreatic head and periampullary tumours, respectively, showed limited BM laminin expression. Whereas 43% and 41% of pancreatic head and periampullary cancers, respectively, showed limited BM collagen type IV expression. Limited BM laminin was associated with poor outcome following curative resection of pancreatic head cancer (P=0.034, 0.013 and 0.017 for RFS, CSS and OS, respectively). Two and a half times as many patients with ≥ 25% BM laminin were recurrence free and alive 5 years following resection compared with those with limited BM laminin. Although staining patterns of both BM components were weakly correlated with each other, BM collagen type IV expression was not significantly associated with outcome in either tumour type. CONCLUSION: Discontinuous BMs, determined by laminin expression, are associated with poor outcome following curative resection of pancreatic head cancer.
Assuntos
Membrana Basal/metabolismo , Membrana Basal/patologia , Carcinoma Ductal Pancreático/metabolismo , Carcinoma Ductal Pancreático/patologia , Laminina/biossíntese , Neoplasias Pancreáticas/metabolismo , Neoplasias Pancreáticas/patologia , Adenocarcinoma/metabolismo , Adenocarcinoma/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Colágeno Tipo IV/metabolismo , Intervalo Livre de Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Prognóstico , Ensaios Clínicos Controlados Aleatórios como Assunto , Estudos RetrospectivosRESUMO
OBJECTIVE: Owing to the shortcomings of clinical examination and radiographs, injury to the syndesmotic ligaments is often misdiagnosed. When there is no indication requiring that the fractured ankle be operated on, the syndesmosis is not tested intra-operatively, and rupture of this ligamentous complex may be missed. Subsequently the patient is not treated properly leading to chronic complaints such as instability, pain, and swelling. We evaluated three fracture classification methods and radiographic measurements with respect to syndesmotic injury. MATERIALS AND METHODS: Prospectively the radiographs of 51 consecutive ankle fractures were classified according to Weber, AO-Müller, and Lauge-Hansen. Both the fracture type and additional measurements of the tibiofibular clear space (TFCS), tibiofibular overlap (TFO), medial clear space (MCS), and superior clear space (SCS) were used to assess syndesmotic injury. MRI, as standard of reference, was performed to evaluate the integrity of the distal tibiofibular syndesmosis. The sensitivity and specificity for detection of syndesmotic injury with radiography were compared to MRI. RESULTS: The Weber and AO-Müller fracture classification system, in combination with additional measurements, detected syndesmotic injury with a sensitivity of 47% and a specificity of 100%, and Lauge-Hansen with both a sensitivity and a specificity of 92%. TFCS and TFO did not correlate with syndesmotic injury, and a widened MCS did not correlate with deltoid ligament injury. CONCLUSION: Syndesmotic injury as predicted by the Lauge-Hansen fracture classification correlated well with MRI findings. With MRI the extent of syndesmotic injury and therefore fracture stage can be assessed more accurately compared to radiographs.
Assuntos
Traumatismos do Tornozelo/patologia , Ligamentos Laterais do Tornozelo/lesões , Ligamentos Laterais do Tornozelo/patologia , Imageamento por Ressonância Magnética/métodos , Doença Aguda , Adolescente , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Reprodutibilidade dos Testes , Sensibilidade e Especificidade , Adulto JovemRESUMO
BACKGROUND: Mechanical bowel preparation (MBP) has been shown to have no influence on the incidence of anastomotic leakage in overall colorectal surgery. The role of MBP in elective surgery in combination with an inflammatory component such as diverticulitis is yet unclear. This study evaluates the effects of MBP on anastomotic leakage and other septic complications in 190 patients who underwent elective surgery for colonic diverticulitis. METHODS: A subgroup analysis was performed in a prior multicenter (13 hospitals) randomized trial comparing clinical outcome of MBP versus no MBP in elective colorectal surgery. Primary endpoint was the occurrence of anastomotic leakage in patients operated on for diverticulitis, and secondary endpoints were septic complications and mortality. RESULTS: Out of a total of 1,354 patients, 190 underwent elective colorectal surgery (resection with primary anastomosis) for (recurrent or stenotic) diverticulitis. One hundred and three patients underwent MBP prior to surgery and 87 did not. Anastomotic leakage occurred in 7.8 % of patients treated with MBP and in 5.7 % of patients not treated with MBP (p = 0.79). There were no significant differences between the groups in septic complications and mortality. CONCLUSION: Mechanical bowel preparation has no influence on the incidence of anastomotic leakage, or other septic complications, and may be safely omitted in case of elective colorectal surgery for diverticulitis.
Assuntos
Catárticos/administração & dosagem , Procedimentos Cirúrgicos do Sistema Digestório , Diverticulite/cirurgia , Procedimentos Cirúrgicos Eletivos , Cuidados Pré-Operatórios/métodos , Anastomose Cirúrgica , Fístula Anastomótica/epidemiologia , Distribuição de Qui-Quadrado , Incontinência Fecal/epidemiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/epidemiologia , Análise de Regressão , Fatores de Risco , Sepse/epidemiologia , Estatísticas não Paramétricas , Infecção da Ferida Cirúrgica/epidemiologia , Resultado do TratamentoRESUMO
Several tools are useful in detecting uncontrolled asthma in children. The aim of this study was to compare Global Initiative for Asthma (GINA) guidelines with the Childhood Asthma Control Test (C-ACT) and the Asthma Control Test (ACT) in detecting uncontrolled asthma in children. 145 children with asthma filled in a web-based daily diary card for 4 weeks on symptoms, use of rescue medication and limitations of activities, followed by either the C-ACT or ACT. For predicting uncontrolled asthma, score cut-off points of 19 were used for C-ACT and ACT. According to GINA guidelines, asthma was uncontrolled in 71 (51%) children and completely controlled in 19 (14%) children. The area under the curve in the receiver operating characteristic curves for C-ACT and ACT versus GINA guidelines were 0.89 and 0.92, respectively. Cut-off points of 19 for C-ACT and ACT resulted in a sensitivity of 33% and 66% in predicting uncontrolled asthma, respectively. C-ACT and ACT correlate well with GINA criteria in predicting uncontrolled asthma, but commonly used cut-off points for C-ACT and ACT seem to underestimate the proportion of children with uncontrolled asthma as defined by GINA.
Assuntos
Asma/classificação , Asma/diagnóstico , Pneumologia/métodos , Pneumologia/normas , Asma/terapia , Criança , Pré-Escolar , Feminino , Saúde Global , Guias como Assunto , Humanos , Internet , Masculino , Estudos Prospectivos , Inquéritos e Questionários , Resultado do TratamentoRESUMO
Extracorporeal membrane oxygenation (ECMO) provides life support in acute reversible cardiorespiratory failure. Assessment of long-term morbidity is essential to confirm survival advantage. This study aimed to assess exercise capacity in the first 12 yrs of life after neonatal ECMO, and to evaluate the effect of primary diagnosis, lung function or perinatal characteristics on exercise capacity. 120 children who, as neonates, underwent ECMO performed 191 reliable exercise tests according to the Bruce treadmill protocol at ages 5, 8 and/or 12 yrs between 2001 and 2010. Primary diagnoses were meconium aspiration syndrome (n=66), congenital diaphragmatic hernia (n=18) and other diagnoses (n=36). At ages 5, 8 and 12 yrs, ANOVA resulted in mean ± se standard deviation score endurance time on the treadmill of -0.5 ± 0.1, -1.1 ± 0.1, and -1.5 ± 0.2, respectively, all significantly less than zero (p<0.001). Exercise capacity declined significantly over time irrespective of the primary diagnosis. Neonates treated with ECMO are at risk of decreased exercise capacity at school age. We therefore propose prolonged follow-up. Proactive advice on sports participation or referral to a physical therapist is recommended, especially when either the parents or the children themselves report impaired exercise capacity.
Assuntos
Tolerância ao Exercício , Oxigenação por Membrana Extracorpórea/efeitos adversos , Monitorização Transcutânea dos Gases Sanguíneos , Criança , Pré-Escolar , Teste de Esforço , Feminino , Seguimentos , Volume Expiratório Forçado , Humanos , Recém-Nascido , Masculino , Espirometria , Capacidade VitalRESUMO
Better treatment of obstructed small airways is needed in cystic fibrosis. This study investigated whether efficient deposition of dornase alfa in the small airways improves small airway obstruction. In a multicentre, double-blind, randomised controlled clinical trial, cystic fibrosis patients on maintenance treatment with 2.5 mL dornase alfa once daily were switched to a smart nebuliser and randomised to small airway deposition (n = 24) or large airway deposition (n = 25) for 4 weeks. The primary outcome parameter was forced expiratory flow at 75% of forced vital capacity (FEF(75%)). FEF(75%) increased significantly by 0.7 sd (5.2% predicted) in the large airways group and 1.2 sd (8.8% pred) in the small airways group. Intention-to-treat analysis did not show a significant difference in treatment effect between groups. Per-protocol analysis, excluding patients not completing the trial or with adherence <70%, showed a trend (p = 0.06) in FEF(75%) Z-score and a significant difference (p = 0.04) between groups in absolute FEF(75%) (L · s(-1)) favouring small airway deposition. Improved delivery of dornase alfa using a smart nebuliser that aids patients in correct inhalation technique resulted in significant improvement of FEF(75%) in children with stable cystic fibrosis. Adherent children showed a larger treatment response for small airway deposition.
Assuntos
Fibrose Cística/tratamento farmacológico , Desoxirribonuclease I/uso terapêutico , Expectorantes/uso terapêutico , Administração por Inalação , Adolescente , Criança , Desoxirribonuclease I/administração & dosagem , Método Duplo-Cego , Expectorantes/administração & dosagem , Feminino , Humanos , Masculino , Cooperação do Paciente , Testes de Função Respiratória , Resultado do TratamentoRESUMO
Respiratory insufficiency is a serious threat to patients with Pompe disease, a neuromuscular disorder caused by lysosomal acid alpha-glucosidase deficiency. Innovative therapeutic options which may stabilize pulmonary function have recently become available. We therefore determined proportion and severity of pulmonary involvement in patients with Pompe disease, the rate of progression of pulmonary dysfunction, and predictive factors for poor respiratory outcome. In a single-center, prospective, cohort study, we measured vital capacity (VC) in sitting and supine positions, as well as maximum inspiratory (MIP) and expiratory (MEP) mouth pressures, and end expiratory CO(2) in 17 children and 75 adults with Pompe disease (mean age 42.7 years, range 5-76 years). Seventy-four percent of all patients, including 53% of the children, had some degree of respiratory dysfunction. Thirty-eight percent had obvious diaphragmatic weakness. Males appeared to have more severe pulmonary involvement than females: at a group level, their mean VC was significantly lower than that of females (p<0.001), they used mechanical ventilation more often than females (p=0.042) and the decline over the course of the disease was significantly different between males and females (p=0.003). Apart from male gender, severe skeletal muscle weakness and long disease duration were the most important predictors of poor respiratory status. During follow-up (average 1.6 years, range 0.5-4.2 years), three patients became ventilator dependent. Annually, there were average decreases in VC in upright position of 0.9% points (p=0.09), VC in supine position of 1.2% points (p=0.049), MIP of 3.2% points (p=0.018) and MEP of 3.8% points (p<0.01). We conclude that pulmonary dysfunction in Pompe disease is much more common than generally thought. Males, patients with severe muscle weakness, and those with advanced disease duration seem most at risk.
Assuntos
Progressão da Doença , Doença de Depósito de Glicogênio Tipo II/patologia , Doença de Depósito de Glicogênio Tipo II/fisiopatologia , Pulmão/fisiopatologia , Adolescente , Adulto , Idoso , Capnografia , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Lactente , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Força Muscular/fisiologia , Prognóstico , Espirometria , Decúbito Dorsal , Resultado do Tratamento , Capacidade Vital/fisiologia , Adulto JovemRESUMO
BACKGROUND: Methotrexate and fumarates are effective systemic therapies for moderate to severe psoriasis according to the European S3 guidelines. OBJECTIVES: We conducted a randomized controlled trial comparing the effectiveness and the adverse events of methotrexate and fumarates. METHODS: Sixty patients with moderate to severe psoriasis vulgaris were randomly assigned to treatment for 16 weeks with either methotrexate (30 patients; 15 mg per week) or fumarates (30 patients; 30 mg, followed by 120 mg according to a standard progressive dosage regimen) and were followed up for 4 weeks. The primary endpoint with respect to the efficacy was the difference in mean change from baseline in Psoriasis Area and Severity Index (PASI) after 12 weeks of treatment. The study was powered to detect a difference of five points. Analyses were by intention to treat. RESULTS: Six patients were excluded because five were not eligible and one withdrew consent. Two patients in the methotrexate group and one in the fumarate group dropped out during the 12 weeks of treatment because of nonappearance at the outpatient clinic. In total, 25 patients in the methotrexate group and 26 in the fumarate group were evaluated in the primary analysis. After 12 weeks of treatment, the mean ± SD PASI decreased from 14·5 ± 3·0 at baseline to 6·7 ± 4·5 in the 25 patients treated with methotrexate, whereas it decreased from 18·1 ± 7·0 to 10·5 ± 6·7 in the 26 patients treated with fumarates. After adjustment for baseline values, the absolute difference (fumarates minus methotrexate) in the mean values at 12 weeks was 1·4 (95% confidence interval -2·0 to 4·7; P = 0·417). CONCLUSIONS: In this randomized trial methotrexate and fumarates were found to be equally effective in the treatment of patients with moderate to severe psoriasis. No serious or irreversible adverse events were observed in any of the patients.
Assuntos
Fumaratos/uso terapêutico , Imunossupressores/uso terapêutico , Metotrexato/uso terapêutico , Psoríase/tratamento farmacológico , Adulto , Feminino , Fumaratos/efeitos adversos , Humanos , Imunossupressores/efeitos adversos , Masculino , Metotrexato/efeitos adversos , Pessoa de Meia-Idade , Estudos Prospectivos , Psoríase/patologia , Índice de Gravidade de DoençaRESUMO
While nifedipine is commonly used for tocolysis, the controversy on its safety remains. So far, the haemodynamic effects on maternal and fetal circulations have not been well documented. Fifteen normotensive women who received 20 mg nifedipine were included in this prospective observational study. The maternal and fetal haemodynamic effects were analysed using maternal echocardiography and fetal Doppler ultrasonography. Nifedipine induced a significant afterload reduction in all women. It triggered a compensatory increase in cardiac output, which maintained blood pressure. These maternal changes had no influence on the uteroplacental and fetal circulations.
Assuntos
Hemodinâmica/efeitos dos fármacos , Nifedipino/farmacologia , Vasodilatadores/farmacologia , Adulto , Pressão Sanguínea/efeitos dos fármacos , Débito Cardíaco/efeitos dos fármacos , Ecocardiografia , Feminino , Humanos , Recém-Nascido , Gravidez , Estudos Prospectivos , Ultrassonografia DopplerRESUMO
OBJECTIVES: To evaluate a virtual reality (VR) application for gestational sac fluid volume (GSFV) measurements in first-trimester pregnancies and to study the correlation between different embryonic growth parameters. METHODS: This was a prospective cohort study analyzing 180 three-dimensional (3D) ultrasound scans of 42 healthy women, performed between 5 + 5 and 12 + 6 weeks' gestational age (GA). The 3D datasets were transferred to the I-Space immersive VR system. The V-Scope application was used to create a 'hologram' of the ultrasound image, allowing depth perception and interaction with the rendered objects. Volumes were measured semi-automatically using a segmentation algorithm. In addition to the GSFV, the total gestational sac volume (GSV) and its diameter (GSD) were measured. The GSV was also calculated using the ellipsoid formula. Previously obtained measurements of embryonic volume and crown-rump length (CRL) were included in the study. The outcomes were analyzed using repeated-measures analysis of variance. RESULTS: The GSFV was measured in 78 scans, and varied from 434 to 81 491 mm(3). A positive correlation between GSFV and GA, CRL and GSD was found. Comparison of the GSD formula constructed in our study in relation to GA with a formula that is commonly used clinically showed an increasing difference with increasing GA either side of 8 + 5 weeks. The GSFV/embryonic volume ratio showed a decrease with GA. The GSV calculated using the ellipsoid formula was on average 19.8% larger compared with the GSV measured in VR. CONCLUSION: New charts for first-trimester GSFV were constructed using VR. These growth charts could be promising tools for studying normal and abnormal embryonic development.
Assuntos
Líquido Amniótico/diagnóstico por imagem , Simulação por Computador , Saco Gestacional/diagnóstico por imagem , Imageamento Tridimensional , Ultrassonografia Pré-Natal , Algoritmos , Estudos de Coortes , Simulação por Computador/normas , Estatura Cabeça-Cóccix , Feminino , Idade Gestacional , Humanos , Gravidez , Primeiro Trimestre da Gravidez , Estudos Prospectivos , Interface Usuário-ComputadorRESUMO
AIM: Short-term survival after emergency surgery for perforated diverticulitis is poor. Less is known about long-term survival. The aims of this study were to evaluate long-term survival after discharge from hospital and to identify factors associated with prognosis. METHOD: All patients who underwent emergency surgery for perforated diverticulitis in five hospitals in Rotterdam, the Netherlands, between 1990 and 2005, were included. The association between type of surgery (Hartmann's procedure or primary anastomosis) and long-term survival was analysed using multivariate Cox regression analysis, taking into account age American Society of Anesthesiology (ASA) classification, Hinchey score, Mannheim Peritonitis Index (MPI) and surgeon's experience. In addition, survival of the patients was compared with that of the matched general Dutch population. RESULTS: Of 340 patients included in the study, 250 were discharged alive from hospital. The overall 5-year survival was 53%. Survival was significantly impaired compared with the expected matched gender-, age- and calendar time-specific survival. Overall survival was significantly related to age and ASA classification. Hinchey score, MPI, number of re-interventions, the surgeon's experience and type of procedure did not influence long-term survival, although a trend was found for Hartmann's procedure to be a risk factor for poorer survival compared with primary anastomosis (hazard ratio for mortality: 1.88; 95% confidence interval, 0.96-3.67; P = 0.07). CONCLUSION: Long-term survival of patients after perforated diverticulitis is limited and mainly caused by the poor general condition of the patients, rather than by the severity of the primary disease or calendar-time and type of procedure.
Assuntos
Doença Diverticular do Colo/complicações , Perfuração Intestinal/etiologia , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Causas de Morte , Doença Diverticular do Colo/mortalidade , Doença Diverticular do Colo/cirurgia , Emergências , Feminino , Humanos , Perfuração Intestinal/mortalidade , Perfuração Intestinal/cirurgia , Masculino , Pessoa de Meia-Idade , Países Baixos/epidemiologia , Prognóstico , Modelos de Riscos Proporcionais , Taxa de SobrevidaRESUMO
AIM: Comparison of transanal excision (TE) and transanal endoscopic microsurgery (TEM) of rectal adenomas (RA) has rarely been performed. METHOD: From 1990 to 2007, the results of TE (43 RA) and TEM (216 RA) were compared. Rectal adenomas were matched for diameter and distance from the anal verge. RESULTS: Operation time was 47.5 min for TE and 35 min for TEM (P < 0.001). Morbidity was 10% after TE and 5.3% after TEM (P < 0.001). Negative resection margins were observed in 50% after TE and 88% after TEM (P < 0.001). Fragmentation of the excised specimen was observed in 23.8% after TE and 1.4% after TEM (P < 0.001). In cases of fragmentation, positive resection margins were observed more frequently. Recurrence was 28.7% after TE and 6.1% after TEM (P < 0.001). After TE, RA with a negative resection margin had a local recurrence rate of 0%, compared with 59.6% with a positive margin (P < 0.001), and after TEM these rates were 3.2 and 7.7% (P = 0.3), respectively. CONCLUSION: Transanal endoscopic microsurgery is superior to transanal excision of RA.
Assuntos
Adenoma/cirurgia , Microcirurgia , Recidiva Local de Neoplasia , Neoplasias Retais/cirurgia , Adenoma/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Colonoscopia/efeitos adversos , Colonoscopia/métodos , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Neoplasias Retais/patologia , Fatores de TempoRESUMO
BACKGROUND: Haemangioma of infancy (HOI) is the most frequently occurring benign tumour of infancy. A good, reliable and objective scoring system for haemangioma activity is not yet available. AIM: We have developed a simple system called the Haemangioma Activity Score (HAS) for scoring the (disease) proliferative activity of haemangiomas. The current study was undertaken to validate this system. METHODS: We validated the HAS in a comparative study of photographs taken during consultations from 2000 until 2008 (n = 78). Agreement between three observers was assessed at two different time points (t(0) and t(1)) with a minimum interval of 6 months between them, using interclass correlation coefficients (ICC). RESULTS: Agreement between observers was good. The average ICC of the HAS at t(0) and t(1) was 0.72 and 0.76, respectively. The average ICC of the HAS for the changes from baseline (HAS at t(0) minus HAS at t(1) ) was 0.69. CONCLUSIONS: We conclude that the HAS is a good system for scoring the proliferative activity of haemangiomas, and believe it to be useful in future investigations. The number of studies comparing different therapies for treating haemangiomas is steadily increasing, and the HAS (before and after treatment) may provide a valuable scoring system for evaluating such therapies.
Assuntos
Hemangioma/patologia , Progressão da Doença , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Variações Dependentes do Observador , Projetos Piloto , Reprodutibilidade dos Testes , Índice de Gravidade de DoençaRESUMO
BACKGROUND: The recent introduction of virtual reality (VR) enables us to use all three dimensions in a three-dimensional (3D) image. The aim of this prospective study was to evaluate an innovative VR technique for automated 3D volume measurements of the human embryo and yolk sac in first trimester pregnancies. METHODS: We analysed 180 3D first trimester ultrasound scans of 42 pregnancies. Scans were transferred to an I-Space VR system and visualized as 3D 'holograms' with the V-Scope volume-rendering software. A semi-automatic segmentation algorithm was used to calculate the volumes. The logarithmically transformed outcomes were analysed using repeated measurements ANOVA. Interobserver and intraobserver agreement was established by calculating intraclass correlation coefficients (ICCs). RESULTS: Eighty-eight embryonic volumes (EVs) and 118 yolk sac volumes (YSVs) were selected and measured between 5(+5) and 12(+6) weeks of gestational age (GA). EV ranged from 14 to 29 877 mm(3) and YSV ranged from 33 to 424 mm(3). ANOVA calculations showed that when the crown-rump length (CRL) doubles, the mean EV increases 6.5-fold and when the GA doubles, the mean EV increases 500-fold (P < 0.001). Furthermore, it was found that a doubling in GA results in a 3.8-fold increase of the YSV and when the CRL doubles, the YSV increases 1.5-fold (P < 0.001). Interobserver and intraobserver agreement were both excellent with ICCs of 0.99. CONCLUSION: We measured the human EV and YSV in early pregnancy using a VR system. This innovative technique allows us to obtain unique information about the size of the embryo using all dimensions, which may be used to differentiate between normal and abnormal human development.