Search

Introduction: Juvenile Idiopathic Arthritis (JIA) includes a group of systemic inflammatory disorders of unknown etiology for which no curative treatment currently exists. The traditional treatment for these disorders which includes the use of NSAIDs and disease modifying anti-rheumatic drugs (DMRADs) is only effective in a subset of patients. Recent advances in understanding the pathophysiology of the inflammatory response, have led to the development of a new class of medications, termed biologic agents which are capable of selectively inhibiting the principal mediators of inflammation. The introduction of biologic drugs has opened a new era in the treatment of rheumatic disorders in children. In this review we describe the characteristics of the currently available biologic agents in Israel, and the updated information regarding indications, efficacy and the safety of these drugs in pediatric patients. Disclosure: Consultant and participating in clinical trials for Novartis com.

Introduction: Stem cell transplantation is indicated in hematological malignancies as well as some solid tumors and congenital abnormalities. Autologous transplantation allows the administration of high dose chemotherapy without prolonged bone marrow aplasia. Allogeneic transplantation allows us to give the patient a new immune system that can locate and destroy remaining tumor cells. First attempts in patients began in 1939. Improved outcomes occurred after discovering the human leukocyte antigen system which allowed for matching the donor to the patient. Immunosuppression therapy to prevent graft versus host disease also improved the outcomes. Since the 1970's, more and more centers in North America and Europe opened stem cell transplantation programs. Today it is performed worldwide and on December 2012, the one million milestone transplant worldwide was achieved. The bone marrow transplantation program started at Rambam Health Care campus on September 1995. Since then 2000 transplantations were performed at Rambam. A third of these procedures were allogeneic and two thirds were autologous. In the last decade patient survival has improved significantly due to better supportive care and the use of reduced intensity conditioning relying on the graft versus tumor effect (GVT). New ways to reduce graft versus host disease (GVHD), while improving GVT effect are based on manipulating T cells in the graft and on genetically engineered T cell with enhanced antitumor cytotoxicity. In the future, allogeneic transplantation will become more complex, more individualized and more efficient.

Introduction: Atherosclerosis is emerging as one of the most important causes of morbidity and mortality among patients with different rheumatologic disease. Endothelial dysfunction may be an early sign of atherosclerosis., Objectives: To evaluate the occurrence of endothelial dysfunction in children with autoimmune diseases, including juvenile idiopathic arthritis (JIA), systemic lupus erythematosus (SLE) and dermatomyositis, using a novel noninvasive technique., Methods: The study group consisted of 24 children with autoimmune diseases, and was compared to a control group of 17 healthy, age- and BMI-matched controls. Endothelial function was assessed by a noninvasive technology that captures a beat-to-beat plethysmographic recording of the finger arterial pulse-wave amplitude with pneumatic probes, utilizing a Peripheral Arterial Tonometry (PAT) device., Results: In the study group, 7 out of the 24 (29%) patients had evidence of impaired endothelial function, compared to 1 out of 17 (6%) children in the control group (p <0.05). Thirty-three per cent of our patients with SLE and 23% of patients with JIA had impaired endothelial function. There were no differences between the two groups of patients with and without endothelial dysfunction as to age, body mass index, fasting glucose level, triglycerides, cholesterol, and dose and duration of steroid treatment. The patients with normal endothelial function had higher systolic blood pressure compared with the group with impaired endothelial function (112.82 ± 7.65 vs13.88 ± 104.85 respectively, p=0.04)., Conclusions: Children with autoimmune diseases may have a high tendency to develop endothelial dysfunction. Larger studies are needed to confirm our findings and to explore the influence of endothelial dysfunction on the development of atherosclerosis in young children.

Background: In recent years, faculty development has turned into a central component of medical education and a primary instrument in qualifying physicians to be teachers and educators. The faculty development program at the Ruth & Bruce Rappaport Faculty of Medicine ("Summit" program) was established in order to improve teaching of the clinical professions, to create a community of medical teachers and educators and to develop leadership in medical education within the Faculty of Medicine., Objective: This article aims to describe the design, implementation and evaluation of the faculty development program in the Technion's Faculty of Medicine., Methods: The program was designed for a group of 20 clinical teachers, of various clinical professions, who had gained at least one year of undergraduate teaching experience and wished to develop a career in medical education. The program included seven monthly, eight-hour meetings throughout the academic year. Learning was based on small group discussions, interactive exercises, role-plays and simulations, self-directed reading and reflective writing. At the end of the final meeting, participants completed an evaluation form., Results: Seventeen of the 20 participants (85%) graduated and received certificates. Learners' overall satisfaction was high. Graduates expressed high motivation to practice medical education within the Faculty of Medicine and reported that they gained new knowledge in medical education and skills regarding various aspects of teaching and learning, such as formulation of learning objectives, designing role plays, and providing effective feedback., Conclusions: The "Summit" program is an innovative initiative in the field of medical education in Israel. The program had a significant impact on participants' knowledge, teaching skills and attitudes. In order to ensure implementation of the acquired tools and skills, its shortterm and long-term effects on teaching behavior and the learning climate have yet to be demonstrated. In addition, it is necessary to check if the program affected the faculty as an organization, promoted changes in curricula, teaching and evaluation methods.

KLeine-Levin Syndrome (KLS) is a rare disease characterized by recurrent episodes of hypersomnia associated with cognitive and behavioral disturbances, compulsive eating behavior and hypersexuality. Episodes are separated by weeks or months of normal sleep and behavior. The disease predominantly affects adolescent males. The median duration of the disease is eight years. Fifteen percent of the KLS population is of Jewish origin and the incidence reported in Israel is unproportionately high. The etiology and pathophysiology are unknown. The current concept is that the disease is caused by genetic predisposition combined with environmental factors. Autoimmune etiology has also been suggested. KLS poses diagnostic and therapeutic challenges. Diagnosis is usually based on clinical manifestations. Physical examination including neurological evaluation is usually normal. EEG, brain imaging and CSF examination are normal. Stimulants are partially effective on sleepiness. Lithium was reported to induce positive effects in preventing or delaying recurrences. Increased awareness to KLS among physicians in Israel is important due to the relatively higher incidence of KLS among Jewish and IsraeLi patients.

The obstructive sleep apnea syndrome (OSAS) is a common and potentially serious problem during childhood. Airway obstruction during sleep may occur due to narrowed bony passages in conditions such as congenital craniofacial anomalies or in the collapsible segments of the upper airways (naso/hypo pharynx). Adenotonsillar hypertrophy has been considered as the main and most important causative factor for OSAS in the pediatric population. However, adenotonsillar hypertrophy is not the sole cause for this syndrome. Normal infants and children have relatively narrowed upper airways. They maintain airway patency during sleep by increasing the upper airway neuromotor tone and adjusting central ventilatory drive. Recent studies suggest that childhood OSAS is a dynamic process resulting from a combination of structural and neuromotor abnormalities, rather than from structural abnormalities alone. Genetic factors have also been suggested. This review delineates the current concepts of the pathophysiology of OSAS in infants and children.