Delleman (Oculocerebrocutaneous) Syndrome: Case report

Delleman syndrome is an unusual entity, characterized by ocular cysts or microphthalmia, focal dermal anomalies and cerebral malformations. In the following article, we carry out a review of the disease and we present the case of a patient with microphthalmos and palpebral coloboma. As we could not put orbital expanders at an early stage, we performed reconstructive surgery.     

Tratamiento del lupus eritematoso cutáneo resistente

Lupus erythematosus (LE) is an autoimmune inflammatory disease that includes a broad spectrum of manifestations, ranging from systemic disease (systemic lupus erythematosus [SLE]) to purely cutaneous forms (cutaneous lupus erythematosus [CLE]).Cutaneous involvement occurs in 90% of patients with SLE.Based on morphological and histopathological features, CLE can be divided into three categories: chronic CLE, subacute CLE and acute CLE.The precise etiology of LE is not fully understood, but the disease occurs when environmental factors, drugs and infectious agents trigger an abnormal immune response in an individual with predisposing genetic factors.To assess cutaneous involvement, several scores have been developed over the years. A recent study, called CLASI (Cutaneous Lupus Erythematosus Disease Area and Severity Index), staged mucocutaneous damage and disease activity separately, allowing assessment of therapeutic response to be standardized.The management of CLE is challenging. Although treatment traditionally consists of photoprotection, topical steroids and antimalarial agents, these measures are sometimes ineffective in subgroup of patients, giving rise to what is called resistant CLE.This article reviews the topical and systemic treatment options, both the classical and new treatment alternatives currently available.     

Multisystemic Langerhans Cell Histiocytosis Mimicking Diffuse Neonatal Hemangiomatosis

We report the first case of multisystemic Langerhans cell histiocytes mimicking diffuse neonatal hemangiomatosis clinically. This has been described in patients with congenital self‐healing reticulohistiocytosis but not in patients with acute, disseminated, and multisystemic disease. In our experience, dermoscopic findings did not help to diagnose the condition.     

The Healthy Immigrant Effect on Mental Health: Determinants and Implications for Mental Health Policy in Spain

Since the mid-1990s, Spain has started to receive a great number of migrant populations. The migration process can have a significantly negative impact on mental health of immigrant population and, consequently, generate implications for the delivery of mental health services. The aim of this article is to provide empirical evidence to demonstrate that the mental health of immigrants in Spain deteriorates the longer they are resident in the country. An empirical approach to this relationship is carried out with data from the National Survey of Health of Spain 2011–2012 and poisson and negative binomial models. Results show that immigrants who reside <10 years in Spain appear to be in a better state of mental health than that observed for the national population. Studying health disparities in the foreign population and its evolution are relevant to ensure the population’s access to health services and care. The need for further research is especially true in the case of the immigrant population’s mental health in Spain because there is scant evidence available on their situation.     

Evaluación formativa mediante el portafolio de 4 promociones de residentes de medicina de familia y comunitaria de la Unidad Docente de Murcia

ObjectiveTo evaluate the performance and quality of the 10 groups of training tasks envisaged in the portfolio training model undertaken by all residents of the Primary Care Teaching Unit in Murcia.DesignA cross-sectional study was conducted on the portfolios provided and completed by all residents in May 2011.ParticipantsAll residents who were in training at that time (131).MethodTen groups of training tasks were established from those recommended by the National Commission for the specialty. The performance of each one in each of the portfolios was evaluated, and the compliance for each training task was calculated. The quality of the performance of each of the tasks was given a score, 0 points (very poor) to 10 points (excellent).ResultsAs regards compliance, the tasks that were most performed were: filling in the Resident book correctly and using the resident skills guide, both with 99.24%, followed by reflection reports on the training visits. All tasks had a compliance rate higher than 67%. The mean percentage of compliance was 86.49%. All tasks obtained an average score greater than 7 (outstanding). The overall mean score was 7,8 points.ConclusionsThe level of perfomance of the tasks set out in the portfolio by the residents was very satisfying. It is necessary to continue working on improving the performance of the portfolio.     

A Specific Angiographic View of Left Coronary Artery Bifurcation in the Left Main Percutaneous Coronary Intervention Era

Objectives

We propose a right lateral (90–120° RAO) with 30° cranial angiographic view to expose the bifurcation of the left main coronary artery (LMCA) when previously used routine projections were inefficient at clearly showing this region.

Background

Little has been published in the medical literature regarding angiographic projections dedicated to special anatomies.

Methods

A total of 84 patients were subjected to the proposed projections. A reproducibility study, conducted with the participation of 2 independent observers, judged the effectiveness of the proposed projection. The Prevalence and Bias Adjusted Kappa (PABAK) index, with a 95% confidence interval (CI), was used to demonstrate the intensity of intra/inter‐observer agreement.

Results

The proposed projection was efficient in 79% of the angiograms, with agreement of 0.76 (0.6–0.9; P ≤ 0.001). The origins and the proximal segments of: the anterior descending coronary artery were exposed in 89% of the angiograms, agreement of 0.86 (0.7–1.0; P ≤ 0.001); the circumflex artery were exposed in 83% of the angiograms, with agreement of 0.72 (0.5–1.0; P ≤ 0.001); and the intermediate branch, when present, were exposed in 89% of the angiograms, agreement of 0.79 (0.6–1.0; P ≤ 0.001).

Conclusion

The right lateral (90–120° RAO) with 30° cranial projection is effective, safe, and reproducible. In special situations where routine projections fail, this proposed projection can reveal important details of the anatomy of the bifurcation of the LMCA during conventional coronary angiography or be the working projection during coronary angioplasty. (J Interven Cardiol 2016;29:293–299)

     

Paraxis is required for somite morphogenesis and differentiation in Xenopus laevis

Background : In most vertebrates, the segmentation of the paraxial mesoderm involves the formation of metameric units called somites through a mesenchymal‐epithelial transition. However, this process is different in Xenopus laevis because it does not form an epithelial somite. Xenopus somitogenesis is characterized by a complex cells rearrangement that requires the coordinated regulation of cell shape, adhesion, and motility. The molecular mechanisms that control these cell behaviors underlying somite formation are little known. Although the Paraxis has been implicated in the epithelialization of somite in chick and mouse, its role in Xenopus somite morphogenesis has not been determined. Results : Using a morpholino and hormone‐inducible construction approaches, we showed that both gain and loss of function of paraxis affect somite elongation, rotation and alignment, causing a severe disorganization of somitic tissue. We further found that depletion or overexpression of paraxis in the somite led to the downregulation or upregulation, respectively, of cell adhesion expression markers. Finally, we demonstrated that paraxis is necessary for the proper expression of myotomal and sclerotomal differentiation markers. Conclusions : Our results demonstrate that paraxis regulates the cell rearrangements that take place during the somitogenesis of Xenopus by regulating cell adhesion. Furthermore, paraxis is also required for somite differentiation. Developmental Dynamics 244:973–987, 2015. © 2015 Wiley Periodicals, Inc.