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Intravenous immunoglobulin in dermatology – clinical experience in Hospital de Braga
Get PDFIntrodução: Nos últimos anos tem aumentado a experiência clínica com o uso de Imunoglobulinas
Endovenosas (IgEv) em Dermatologia. Apesar da informação limitada na literatura, a utilização off-label das IgEv
tem demonstrado eficácia na terapêutica de várias dermatoses refratárias aos tratamentos convencionais. Material
e métodos: Efetuou-se um estudo retrospetivo dos doentes com patologia dermatológica tratados com IgEv entre
Janeiro de 2004 e Outubro de 2011 no Serviço de Dermatologia do Hospital de Braga. Foram analisadas as características
demográficas e clínicas, as terapêuticas efetuadas, a resposta clínica e o perfil de segurança. Resultados:
Foram tratados 21 doentes com IgEv em 10 diferentes patologias dermatológicas: quatro doentes com Pênfigo Vulgar
[2 com resposta completa (RC), um com resposta parcial (RP) e outro que interrompeu o tratamento por efeito
lateral grave]; dois doentes com Penfigóide Bolhoso (um com RC e outro com RP); três doentes com Necrólise Epidérmica
Tóxica (NET) com RC; dois doentes com Dermatomiosite (ambos com RP); quatro doentes com Urticária
Crónica (um com RC, um com RP, um que não respondeu e outro que suspendeu o tratamento por efeito lateral); dois
doentes com Vasculopatia Livedóide com RP; um doente com Síndrome CREST que não melhorou; um doente com
Escleromixedema com RP; um doente com Pioderma Gangrenoso com RC e uma doente com Dermite Atópica que
interrompeu o tratamento na sequência de gravidez. Com excepção dos 3 doentes com NET, em todos os outros a
doença havia sido refratária aos tratamentos sistémicos convencionais. Conclusões: Apesar de a nossa experiência
ser limitada, o tratamento com IgEv pode ser benéfico em determinadas patologias que não melhoram com o tratamento
clássico. Atendendo ao seu custo elevado e efeito terapêutico variável, o seu uso deve ser criterioso até que
mais estudos definam a relação risco-benefício
Variations of the soft tissue thicknesses external to the ribs in Pectus Excavatum patients
Get PDFBACKGROUND:
Surgical repair of pectus excavatum (PE) has become more popular due to improvements in the minimally invasive Nuss procedure. The pre-surgical assessment of PE patients requires Computerized Tomography (CT), as the malformation characteristics vary from patient to patient.
OBJECTIVE:
This work aims to characterize soft tissue thickness (STT) external to the ribs among PE patients. It also presents a comparative analysis between the anterior chest wall surface before and after surgical correction.
METHODS:
Through surrounding tissue segmentation in CT data, STT values were calculated at different lines along the thoracic wall, with a reference point in the intersection of coronal and median planes. The comparative analysis between the two 3D anterior chest surfaces sets a surgical correction influence area (SCIA) and a volume of interest (VOI) based on image processing algorithms, 3D surface algorithms, and registration methods.
RESULTS:
There are always variations between left and right side STTs (2.54±2.05mm and 2.95±2.97mm for female and male patients, respectively). STTs are dependent on age, sex, and body mass index of each patient. On female patients, breast tissue induces additional errors in bar manual conception. The distances starting at the deformity's largest depression point at the SCIA are similar in all directions. Some diverging measures and outliers were found, being difficult to find similar characteristics between them, especially in asymmetric patients.
CONCLUSION:
The Nuss procedure metal bar must be modeled according to each patient's special characteristics. The studied relationships between STT and chest surface could represent a step forward to eliminate the CT scan from PE pre-surgical evaluation
Phenotypic characterization and familial risk in hyperplastic polyposis syndrome
Get PDFBackground. Hyperplastic polyposis syndrome (HPS) is a rare condition characterized by numerous hyperplastic polyps (HP) with a pancolonic distribution. Genetic and environmental factors, including smoking, may be responsible for phenotypic differences. Objective. To characterize HPS patients' phenotype and to determine HPS risk and colorectal cancer (CRC) risk in the first-degree relatives (FDRs). Patients and methods. Eight HPS patients were followed at our Gastroenterology Department (2008-2012). The data included (1) macroscopic and histological analysis of polyps, (2) demographic information about patients and their families and (3) colonoscopy results of FDR that accepted a screening exam. Results. Six of the eight index cases (ICs) had family history of CRC. Of the 24 FDRs screened, 5 were diagnosed with HPS. In our study, HPS and CRC prevalence in FDR was 625 and 9 times higher than the risk of the general population. Polyps over 10 mm were preferentially located in proximal colon (p < 0.001). Advanced polyps were larger (p < 0.001) than HP and more frequent in older patients (p = 0.0054). Nonsmokers had smaller polyps (p = 0.037) preferentially in the proximal colon (p = 0.04) and a lower age at HPS diagnosis. Patients with CRC family history manifest HPS at an earlier age and patients whose relatives had CRC before 50 years had larger polyps (p = 0.0475). Smokers with CRC family history had larger polyps than nonsmokers (p = 0.048). Conclusion. Despite the small sample, the results reflect the phenotypic heterogeneity of HPS as well as the increased family risk of HPS and CRC. This study points out that CRC family history and smoking influence HPS expression
The Impact of Polymorphic Variations in the 5p15, 6p12, 6p21 and 15q25 Loci on the Risk and Prognosis of Portuguese Patients with Non-Small Cell Lung Cancer
Get PDFINTRODUCTION:
Polymorphic variants in the 5p15, 6p12, 6p21, and 15q25 loci were demonstrated to potentially contribute to lung cancer carcinogenesis. Therefore, this study was performed to assess the role of those variants in non-small cell lung cancer (NSCLC) risk and prognosis in a Portuguese population.
MATERIALS AND METHODS:
Blood from patients with NSCLC was prospectively collected. To perform an association study, DNA from these patients and healthy controls were genotyped for a panel of 19 SNPs using a Sequenom® MassARRAY platform. Kaplan-Meier curves were used to assess the overall survival (OS) and progression-free survival (PFS).
RESULTS:
One hundred and forty-four patients with NSCLC were successfully consecutively genotyped for the 19 SNPs. One SNP was associated with NSCLC risk: rs9295740 G/A. Two SNPs were associated with non-squamous histology: rs3024994 (VEGF intron 2) T/C and rs401681 C/T. Three SNPs were associated with response rate: rs3025035 (VEGF intron 7) C/T, rs833061 (VEGF -460) C/T and rs9295740 G/A. One SNP demonstrated an influence on PFS: rs401681 C/T at 5p15, p = 0.021. Four SNPs demonstrated an influence on OS: rs2010963 (VEGF +405 G/C), p = 0.042; rs3025010 (VEGF intron 5 C/T), p = 0.047; rs401681 C/T at 5p15, p = 0.046; and rs31489 C/A at 5p15, p = 0.029.
CONCLUSIONS:
Our study suggests that SNPs in the 6p12, 6p21, and 5p15 loci may serve as risk, predictive and prognostic NSCLC biomarkers. In the future, SNPs identified in the genomes of patients may improve NSCLC screening strategies and therapeutic management as well
Risk and clinical-outcome indicators of delirium in an emergency department intermediate care unit (EDIMCU): an observational prospective study
Get PDFBACKGROUND:
Identification of delirium in emergency departments (ED) is often underestimated; within EDs, studies on delirium assessment and relation with patient outcome in Intermediate Care Units (IMCU) appear missing in European hospital settings. Here we aimed to determine delirium prevalence in an EDIMCU (Hospital de Braga, Braga, Portugal) and assessed routine biochemical parameters that might be delirium indicators.
METHODS:
The study was prospective and observational. Sedation level was assessed via the Richmond Agitation-Sedation Scale and delirium status by the Confusion Assessment Method for the ICU. Information collected included age and gender, admission type, Charlson Comorbidity Index combined condition score (Charlson score), systemic inflammatory response syndrome criteria (SIRS), biochemical parameters (blood concentration of urea nitrogen, creatinine, hemoglobin, sodium and potassium, arterial blood gases, and other parameters as needed depending on clinical diagnosis) and EDIMCU length of stay (LOS). Statistical analyses were performed as appropriate to determine if baseline features differed between the 'Delirium' and 'No Delirium' groups. Multivariate logistic regression was performed to assess the effect of delirium on the 1-month outcome.
RESULTS:
Inclusion and exclusion criteria were met in 283 patients; 238 were evaluated at 1-month for outcome follow-up after EDIMCU discharge ("good" recovery without complications requiring hospitalization or institutionalization; "poor" institutionalization in permanent care-units/assisted-living or death). Delirium was diagnosed in 20.1% patients and was significantly associated with longer EDIMCU LOS. At admission, Delirium patients were significantly older and had significantly higher blood urea, creatinine and osmolarity levels and significantly lower hemoglobin levels, when compared with No Delirium patients. Delirium was an independent predictor of increased EDIMCU LOS (odds ratio 3.65, 95% CI 1.97-6.75) and poor outcome at 1-month after discharge (odds ratio 3.51, CI 1.84-6.70), adjusted for age, gender, admission type, presence of SIRS criteria, Charlson score and osmolarity at admission.
CONCLUSIONS:
In an EDIMCU setting, delirium was associated with longer LOS and poor outcome at 1-month post-discharge. Altogether, findings support the need for delirium screening and management in emergency settings
Automatic localization of the optic disc by combining vascular and intensity information
No full textThis paper describes a new methodology for automatic location of the optic disc in retinal images, based on the combination of information taken from the blood vessel network with intensity data. The distribution of vessel orientations around an image point is quantified using the new concept of entropy of vascular directions. The robustness of the method for OD localization is improved by constraining the search for maximal values of entropy to image areas with high intensities. The method was able to obtain a valid location for the optic disc in 1357 out of the 1361 images of the four datasets
