Primary Immunodeficiency Diseases in Latin America: The Second Report of the LAGID Registry

This is the second report on the continuing efforts of LAGID to increase the recognition and registration of patients with primary immunodeficiency diseases in 12 Latin American countries: Argentina, Brazil, Chile, Colombia, Costa Rica, Honduras, Mexico, Panama, Paraguay, Peru, Uruguay, and Venezuela. This report reveals that from a total of 3321 patients registered, the most common form of primary immunodeficiency disease was predominantly antibody deficiency (53.2%) with IgA deficiency reported as the most frequent phenotype. This category was followed by 22.6% other well-defined ID syndromes, 9.5% combined T- and B-cell inmunodeficiency, 8.6% phagocytic disorders, 3.3% diseases of immune dysregulation, and 2.8% complement deficiencies. All countries that participated in the first publication in 1998 reported an increase in registered primary immunodeficiency cases, ranging between 10 and 80%. A comparison of the estimated minimal incidence of X-linked agammaglobulinemia, chronic granulomatous disease, and severe combined immunodeficiency between the first report and the present one shows an increase in the reporting of these diseases in all countries. In this report, the estimated minimal incidence of chronic granulomatous disease was between 0.72 and 1.26 cases per 100,000 births in Argentina, Chile, Costa Rica, and Uruguay and the incidence of severe combined immunodeficiency was 1.28 and 3.79 per 100,000 births in Chile and Costa Rica, respectively. However, these diseases are underreported in other participating countries. In addition to a better diagnosis of primary immunodeficiency diseases, more work on improving the registration of patients by each participating country and by countries that have not yet joined LAGID is still needed. Latin American Group for Primary Immunodeficiency Diseases     

Fracture healing following high energy tibial trauma: Ilizarov versus Taylor Spatial Frame

Introduction

The optimal treatment of high energy tibial fractures remains controversial and a challenging orthopaedic problem. The role of external fixators for all these tibial fractures has been shown to be crucial.

Methods

A five-year consecutive series was reviewed retrospectively, identifying two treatment groups: Ilizarov and Taylor Spatial Frame (TSF; Smith & Nephew, Memphis, TN, US). Fracture healing time was the primary outcome measure.

Results

A total of 112 patients (85 Ilizarov, 37 TSF) were identified for the review with a mean age of 45 years. This was higher in women (57 years) than in men (41 years). There was no significant difference between frame types (p=0.83). The median healing time was 163 days in both groups. There was no significant difference in healing time between smokers and non-smokers (180 vs 165 days respectively, p=0.07), open or closed fractures (p=0.13) or age and healing time (Spearman''s r=0.12, p=0.18). There was no incidence of non-union or re-fracture following frame removal in either group.

Conclusions

Despite the assumption of the rigid construct of the TSF, the median time to union was similar to that of the Ilizarov frame and the TSF therefore can play a significant role in complex tibial fractures.     

PROPERTIES OF NUCLEAR RNA IN SEA URCHIN EMBRYOS

Pulse-labeled RNA in early sea urchin embryos (64 cells to mesenchyme blastulase) is found almost exclusively associated with the nucleus and turns over very rapidly. The rate of labeling of the cytoplasm is much slower and is consistent with the transfer of 6 per cent of the nuclear RNA made every 15-20 minutes. Most of the pulse-labeled nuclear RNA exists in membrane-bound polyribosomes.     

Essential steps in developing best practices to assess reflective skill: A comparison of two rubrics

Purpose: Medical education lacks best practices for evaluating reflective writing skill. Reflection assessment rubrics include the holistic, reflection theory-based Reflection-on-Action and the analytic REFLECT developed from both reflection and narrative-medicine literatures. To help educators move toward best practices, we evaluated these rubrics to determine (1) rater requirements; (2) score comparability; and (3) response to an intervention.

Methods: One-hundred and forty-nine third-year medical students wrote reflections in response to identical prompts. Trained raters used each rubric to score 56 reflections, half written with structured guidelines and half without. We used Pearson’s correlation coefficients to associate overall rubric levels and independent t-tests to compare structured and unstructured reflections.

Results: Reflection-on-Action training required for two hours; two raters attained an interrater-reliability?=?0.91. REFLECT training required six hours; three raters achieved an interrater-reliability?=?0.84. Overall rubric correlation was 0.53. Students given structured guidelines scored significantly higher (p?<?0.05) on both rubrics.

Conclusions: Reflection-on-Action and REFLECT offer unique educational benefits and training challenges. Reflection-on-Action may be preferred for measuring overall quality of reflection given its ease of use. Training on REFLECT takes longer but it yields detailed data on multiple dimensions of reflection that faculty can reference when providing feedback.     

The role of industry in improving quality of life for persons with an ostomy: a Canadian consensus

Financial and clinical resources (which include 260 enterostomal therapy nurses nationwide) to provide clinical and rehabilitative expertise to the estimated 50,000 to 60,000 Canadians living with an ostomy are limited. Hence, many persons with an ostomy fall through the cracks in the early pre- and postoperative period or are lost to follow-up afterdischarge. Problems and issues related to ostomy management continue to surface throughout their lives. As a result,the time required for rehabilitation is often lengthy, affecting the person's quality of life. The First Canadian Enterostomal Therapy Nurses' Advisory Council consisting of 10 Canadian enterostomal therapy nurses met in February 2004 to identify key issues and concerns for Canadians living the ostomy experience and to identify gaps in information and services available before surgery, during the immediate postoperative period, and as the time from surgery lengthened and patients moved through age-related life issues. The two most important gaps identified were the development of quality, pertinent resources and the dissemination of those resources. The Council also identified potential strategies to bridge these gaps, including utilizing the resources of manufacturer Customer Service departments to follow a communications protocol to provide information to patients, retailers, and providers. Finally, a process map from which individual ostomy manufacturers can build programs and processes for improving patient/provider/manufacturer communication was developed.