Oclusión de la arteria central de la retina y endocarditis infecciosa: el rigor sí importa / Central retinal artery occlusion and infective endocarditis: Rigor does matter

CASO CLÍNICO: Paciente con amaurosis brusca debido a oclusión de arteria central de la retina (OACR), en el que se objetivó insuficiencia mitral y hemocultivos positivos para Streptococcus viridans. Con el estudio de ecografía transesofágica, se diagnosticó de endocarditis infecciosa subaguda sobre válvula nativa mitral que cursó sin fiebre y con la pérdida de visión como único síntoma. DISCUSIÓN: La OACR debida a endocarditis infecciosa es muy infrecuente, y hay escasos casos reportados en la literatura médica. La semiología y el estudio sistemático y exhaustivo de los pacientes con esta OACR, ayuda a descubrir enfermedades graves subyacentes. La endocarditis infecciosa tiene formas muy diversas de presentación y con frecuencia se requiere una alta sospecha clínica para llegar a su diagnóstico

CLINICAL CASE: A patient with acute amaurosis due central retinal artery occlusion (CRAO), who had mitral regurgitation and Streptococcus viridans positive blood cultures. Using transesophageal ultrasound, the patient was diagnosed with native valve infective endocarditis without fever, and with loss of vision as the only symptom. DISCUSSION: CRAO due to infective endocarditis is rare and there are few cases reported in the literature. Semiology and a systematic and comprehensive study of patients with this ophthalmological pathology helps uncover serious underlying medical conditions. Infective endocarditis has many different forms of presentation and a high clinical suspicion is often required to reach a diagnosis

Expression of Wilms’ tumor gene (WT1) is associated with survival in malignant pleural mesothelioma

INTRODUCTION: Calretinin and Wilms' tumor gene (WT1) are mesothelial markers routinely used to confirm the diagnosis of malignant pleural mesothelioma (MPM). We investigated the prognostic value of calretinin and WT1 expression in predicting survival in a series of patients diagnosed with MPM in our institution. MATERIALS AND METHODS: Fifty-two patients diagnosed of MPM were retrospectively reviewed. Calretinin and WT1 were stained for IHC analysis in formalin-fixed, paraffin-embedded sections and positivity was considered for tumors with >1 % of tumor cells stained. Survival data were calculated by the Kaplan-Meier method and Cox regression was used to evaluate the prognostic value of the variables. RESULTS: Calretinin IHC expression was positive in 83.7 % of patients and WT1 in 78.1 %. A significant association of calretinin and WT1 expression with epithelial histology was detected (p = 0.030 and p = 0.010). We found a significant increase in OS in patients with epithelial subtype, PS1 and neutrophil-lymphocyte ratio (NLR) ≤5 (p < 0.05). In the IHC markers analysis, we found a significant increase in OS for patients with WT1 positive expression (16.4 vs. 2.3 m, p = 0.013), but not differences for calretinin expression (16.6 vs. 5.0 months, p = 0.37). In the multivariate analysis, epithelial histology and WT1 remained as significant prognostic factors for survival (p = 0.004 and p = 0.010, respectively). CONCLUSION: In our series of 52 MPM patients, epithelial histology, PS, NLR and WT1 expression are significant prognostic factors for survival. We concluded that WT1, but not calretinin, is a useful prognostic factor in MPM. The role of WT1 assessment is worth of prospective validation in future studies on MPM (AU)

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Neumonitis por hipersensibilidad en el ámbito escolar / Hypersensitivity pneumonitis in the school environment

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Biomarkers of pulmonary rejection.

Immunologic complications after lung transplantation (LT) include acute cellular rejection (ACR), antibody-mediated rejection (AMR), and most forms of chronic allograft dysfunction (CAD). ACR is an inflammatory process in which the reaction is mediated by the T-cell population. Most episodes of ACR fully recover with treatment, but repeated bouts are considered to be a risk factor for CAD. Biomarker cytokines interleukin (IL)-10, IL-15, IL-6, CCL5, CCR2 and IFNγ may play significant roles in this complication. Formerly bronchiolitis obliterans syndrome (BOS) or chronic rejection or most forms of CAD were considered to be immunologic complications not amenable therapeutic measures. CAD, the main limitation for long-term survival in LT, is characterized histologically by airway epithelial cell apoptosis and luminal fibrosis in the respiratory bronchioles causing airflow obstruction and, in some cases, lung parenchymal affectations causing restrictive lung disease. Several biomarkers have been studied in CAD, IL-6, IL-8, IL-17, IL-23, IL-13, IFN γ, and TGF ß cytokines, pH, bile acid, and tripsine of gastroesophageal reflux and toll-like receptors of innate immunity. Herein we have reviewed the literature of biomarkers involved in lung rejection.

Hamartoma mesenquimal rabdomiomatoso / Rhabdomyomatous mesenchymal hamartoma

El hamartoma mesenquimal rabdomiomatoso es una lesión congénita rara que consiste en la presencia de fibras musculares estriadas dispuestas al azar a nivel de dermis como consecuencia de una migración anómala durante la embriogénesis. Se presenta el caso de un paciente de un año de edad que mostró una lesión bilobulada en línea cervical media que tras la extirpación se diagnosticó de hamartoma mesenquimal rabdomiomatoso sin encontrarse malformaciones congénitas asociadas. Se describen las características clínicas e histopatológicas de esta entidad(AU)

Rhabdomyomatous mesenchymal hamartoma is a rare congenital lesion which consists of randomly arranged striated muscle fibers interspersed with mesenchymal elements. We describe the clinical and histopathological features of a rhabdomyomatous mesenchymal hamartoma in a one year-old patient presenting a bilobulated lesion in the mid-cervical line. No associated congenital malformations were observed(AU)

Fluorescence in situ hybridization and immunohistochemistry as diagnostic methods for ALK positive non-small cell lung cancer patients.

BACKGROUND: Anaplastic Lymphoma Kinase (ALK) positivity represents a novel molecular target in a subset of Non-Small Cell Lung Cancers (NSCLC). We explore Fluorescence in situ Hybridization (FISH) and Immunohistochemistry (IHC) as diagnostic methods for ALK positive patients and to describe its prevalence and outcomes in a population of NSCLC patients. METHODS: NSCLC patients previously screened for Epidermal Growth Factor Receptor (EGFR) at our institution were selected. ALK positive patients were identified by FISH and the value of IHC (D5F3) was explored. RESULTS: ninety-nine patients were identified. Median age was 61.5 years (range 35-83), all were caucasians, eighty percent were adenocarcinomas, fifty-one percent were male and thirty-eight percent were current smokers. Seven (7.1%) patients were ALK positive by FISH, thirteen (13.1%) were EGFR mutant, and 65 (65.6%) were negative/Wild Type (WT) for both ALK and EGFR. ALK positivity and EGFR mutations were mutually exclusive. ALK positive patients tend to be younger than EGFR mutated or wt patients. ALK positive patients were predominantly never smokers (71.4%) and adenocarcinoma (71.4%). ALK positive and EGFR mutant patients have a better outcome than negative/WT. All patients with ALK FISH negative tumours were negative for ALK IHC. Out of 6 patients positive for ALK FISH with more tissue available, 5 were positive for ALK IHC and 1 negative. CONCLUSIONS: ALK positive patients represent 7.1% of a population of selected NSCLC. ALK positive patients have different clinical features and a better outcome than EGFR WT and ALK negative patients. IHC is a promising method for detecting ALK positive NSCLC patients.

Natalizumab use in pediatric patients with relapsing-remitting multiple sclerosis.

BACKGROUND: Not all pediatric patients with relapsing-remitting multiple sclerosis (MS) may respond to traditional disease-modifying therapies. Natalizumab has been shown to be effective but is currently only approved in adults. OBJECTIVE: To analyze the safety and efficacy of natalizumab in patients under 18 years of age diagnosed with MS. METHOD: Data for pediatric patients with MS treated with natalizumab in a compassionate use setting were retrospectively collected and analyzed. RESULTS: Valid data were obtained for nine patients under 18 years from seven different centers (mean age, 15 years 4 months [range 9.8-17.7]; 5 were boys). Patients received a median of 17 infusions of natalizumab (range, 2-31) and eight received at least 12 infusions. For these 8 patients, the median score on the Expanded Disability Status Scale decreased from 3.0 to 1.0 and the median annualized relapse rate decreased from 3.0 to 0. After 12 months, no patients reported gadolinium-enhancing lesions compared to seven at baseline. Four post-baseline adverse events occurred and one patient discontinued due to hypersensitivity reaction. CONCLUSION: Natalizumab is a highly effective treatment as a second-line option in pediatric patients. In as far as the limited numbers allowed comparisons, the safety and efficacy of natalizumab in children was in line with the experience published in adult populations.