Blomstrand lethal osteochondrodysplasia

We present the clinical, roentgenographic, and histologic abnormalities in a stillborn infant with Blomstrand osteochondrodysplasia. Parental consanguinity and multiplex occurrence in the patients' sibship confirm the hypothesis of autosomal recessive inheritance of this monogenic lethal entity. The unknown genetic defect interferes severely with skeletal growth through lack of chondrocyte multiplication and apparent uncoupling of the processes of enchondral ossification and skeletal growth. © 1996 Wiley-Liss, Inc.     

The efficacy of the back school: An analysis of the literature

One of the possible treatments for patients suffering from low back pain is the so-called back school, which is of Swedish origin. Back schools offer an education and skills program in a group setting, directed toward pain management and consisting of elements of education and/or training of skills. The contents and, briefly, the efficacy of all reported and evaluated back schools in a group setting are presented. Based on the different contents of back schools and the necessity of a multidimensional approach to treating low back pain, we designed the Maastricht Back School to be a combination of all those elements about which we consider a back school should give information and/or training.     

Performance characteristics of updated INNO-LiPA assays for molecular typing of human leukocyte antigen A (HLA-A), HLA-B, and HLA-DQB1 alleles

We carried out a multicenter performance evaluation of three new DNA-based human leukocyte antigen (HLA) typing assays: INNO-LiPA HLA-A Update, INNO-LiPA HLA-B Update, and INNO-LiPA HLA-DQB1 Update. After optimization, the accuracy rates were all 100%, and the final observed resolutions were 99.4, 92.4, and 85.6%, respectively. These rapid and easy-to-perform assays yielded results fully concordant with other DNA-based tissue typing tests.     

Teaching communication skills to medical students,a challenge in the curriculum?

INTRODUCTION: As communication skills become more and more important in medical practice, the new medical curriculum at Ghent University (1999) implemented a communication curriculum. METHOD: Communication training or experiences in 'real life' settings are provided every year of the medical curriculum. The training starts with simple basic skills but gradually slips into medical communication or consultation training and results in communication in different contextual situations or with special groups of patients. Rehearsal is important and seen as inevitable. Poorly performing students get extra training. Several didactical methods are used: the skills are demonstrated by means of videotapes and paper cases of patient stories. Skills are trained in small groups (10-15 students), with focus on role-playing with colleague students or simulated patients (SP). Videotapes of real consultations give an idea of the performance of each student. Every year the students are assessed by means of an OSCE (objective structured clinical examination). CONCLUSION: After 6 years of experience with the new curriculum, several remarks and questions need to be answered. Small group training gives a huge workload and with different trainers discrepancies between groups can appear. Choosing the most suitable trainer for communication skills is not easy; several options are available: specialists in communication like psychologists with interest in medical practice, GPs with interest in medical communication, medical specialists for communication topics concerning medical problems within their domain. As the most important didactical approach lies in practising the skills, the selection and training of simulated patients remains a challenge. PRACTICE IMPLICATION: A communication continuum during the whole curriculum seems to be worthwhile. Students with specific communicative problems are detected early, remediation is provided. Rehearsal every year seems to lead to better acquisition. The most positive point is that communication is embedded in a global patient-, student- and community-oriented curriculum and that communication skills are seen as core elements of good doctoring.     

Treatment of motoneuron degeneration by intracerebroventricular delivery of VEGF in a rat model of ALS

Neurotrophin treatment has so far failed to prolong the survival of individuals affected with amyotrophic lateral sclerosis (ALS), an incurable motoneuron degenerative disorder. Here we show that intracerebroventricular (i.c.v.) delivery of recombinant vascular endothelial growth factor (Vegf) in a SOD1(G93A) rat model of ALS delays onset of paralysis by 17 d, improves motor performance and prolongs survival by 22 d, representing the largest effects in animal models of ALS achieved by protein delivery. By protecting cervical motoneurons, i.c.v. delivery of Vegf is particularly effective in rats with the most severe form of ALS with forelimb onset. Vegf has direct neuroprotective effects on motoneurons in vivo, because neuronal expression of a transgene expressing the Vegf receptor prolongs the survival of SOD1(G93A) mice. On i.c.v. delivery, Vegf is anterogradely transported and preserves neuromuscular junctions in SOD1(G93A) rats. Our findings in preclinical rodent models of ALS may have implications for treatment of neurodegenerative disease in general.     

Identification of Burkholderia pseudomallei and related bacteria by multiple-locus sequence typing-derived PCR and real-time PCR

Close relatedness and genomic plasticity characterizing the high-threat pathogens Burkholderia pseudomallei and Burkholderia mallei render the molecular diagnosis of these species hard to guarantee with a maximal confidence level. This article describes fast molecular assays derived from compiled sequences of housekeeping genes determined in more than 1,000 strains. The assays proved to be robust and appropriate for general detection as well as species identification purposes.     

Changes in Endotoxin-Binding Proteins during Major Elective Surgery: Important Role for Soluble CD14 in Regulation of Biological Activity of Systemic Endotoxin

Assessment of circulating endotoxin during the perioperative period, which is only demonstrated by the Limulus amebocyte lysate (LAL) test, may be modulated by several endotoxin-binding proteins. Endotoxin-neutralizing capacity (ENC) and the plasma levels of soluble CD14 (sCD14), lipopolysaccharide-binding protein, and bactericidal/permeability-increasing protein (BPI) were determined in 40 patients 6 h prior to skin incision for major abdominal surgery. The bioactivity of plasma endotoxin was tested by the polymyxin B-inhibited stimulatory activity of the plasma samples on healthy monocytes as measured by the release of tumor necrosis factor alpha. Plasma endotoxin levels in almost all patients increased from 0.05 ± 0.01 to 0.23 ± 0.03 experimental units (EU) per ml (P < 0.001); more specifically, 17 of 40 samples showed endotoxin levels of greater than 0.2 EU per ml and corresponding reductions in ENC. Soluble CD14 plasma levels were decreased from 5.6 ± 0.3 to 4.6 ± 0.3 μg per ml (P < 0.05). ENC was strongly correlated with the sCD14 plasma concentration throughout the period of observation. The addition of sCD14-neutralizing monoclonal anti-sCD14 antibodies reduced ENC both pre- and postoperatively. No correlation could be established between ENC and the plasma levels of BPI, high-density lipoproteins, or low-density lipoproteins determined by measuring the concentrations of apoprotein A and apoprotein B. Biologically active endotoxin was found in only 6 of 17 samples with endotoxin levels greater than 0.2 EU per ml in the LAL test. These samples could be characterized by their perioperative loss of at least 35% of their sCD14. No change in sCD14 was detected in the remaining 11 samples. The perioperative loss of ENC is partly caused by the loss of sCD14 resulting from its consumption by endotoxin reaching the bloodstream. This study demonstrated the role of sCD14 on the bioactivity of circulating endotoxin in a human model of endotoxemia after major abdominal surgery.