Israel Medical Association Journal

The screening program in Israel for Tay-Sachs disease has proven very successful, giving Jewish couples a choice not to have affected children. The technology of carrier detection is now possible in several other severe genetic diseases that are relatively frequent among Jews. Due to the current confusion, a policy is needed to determine how the TSD screening program should be continued in the Israeli Jewish population. We propose that such a screening program include only mutations agreed by consensus as causing a disease severe enough to warrant the possibility of therapeutic abortion. We also propose that general screening include only mutations that are relatively frequent, taking into account the carrier frequencies in the Israeli Jewish population.

Background: Familial dysautonomia is a genetic disease in which there is a defect in the autonomic and sensory nervous systems. These systems have a major role in the reproductive system.

Objective: To study the inter-relationship of autonomic and sensory dysfunction and gynecological function.

Methods: The gynecological histories of 48 women with familial dysautonomia were analyzed retrospectively. Their mean age was 22.25 years (range 12-47). Thirty-three women (65%) were available for further questioning and investigation of hormonal status.

Results: Menarche had occurred in 32 of the 48 (66.7%). Their average age of menarche was significantly delayed as compared to their unaffected mothers (15.5 vs. 13.6 years respectively, P=0.002). The most prominent finding was the very high prevalence, 81.2%, of premenstrual symptoms. Seven of 26 had premenstrual syndrome symptoms of dysautonomic crisis. Blood sex hormone levels were normal in 27 of the 33 patients studied. None reached natural menopause. One patient had adenomyosis, and another, dysgerminoma. Three patients became pregnant and delivered healthy infants.

Conclusion: Menarche is delayed in women with FD, and the physiological monthly hormonal fluctuations may disturb autonomic homeostasis sufficiently to precipitate dysautonomic crisis.

Background: General pediatricians in Israel are actively involved in the initial evaluation, resuscitation and management of traumatized children. However, pediatric trauma care is not a part of pediatric specialty training in Israel, and the few Advanced Trauma Life Support^R courses per year are insufficient for most pediatricians working in accident and emergency care.

Objective: To examine the value of the course in relation to the limited resources available for such training.

Methods: A telephone survey of 115 pediatricians who had taken the course between 1990 and 1994 was conducted. The responding physicians (67%) were asked to complete a specially designed questionnaire on life-saving procedures that were taught in the course. In addition, they were asked to subjectively assess the practical utility of the course.

Results: Forty-three (56%) pediatricians reported that they routinely treated both adult and pediatric trauma cases. Of these, 81% performed 27 life-saving ATLS^R procedures. Pediatric trauma was treated by only 22 (28%), of whom 72.3% performed 18 life-saving ATLS^R procedures. These pediatricians ranked the courses as being "very high" to "high" in impact.

Conclusions: These figures indicate that an ATLS^R course designed specifically for pediatricians can markedly improve pediatric trauma care. To ensure standard education and patient care, such a course should be developed and made a mandatory component of residency training. Further studies to examine the objective impact of the courses on pediatric trauma care should be carried out.

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ATLS= Advanced Trauma Life Support

Background: Israeli physicians are very familiar with the problem of interruptions during encounters with patients. However, a thorough search of the medical literature revealed only one report of this problem from Israel, and none from other countries.

Objectives: To characterize the phenomenon of interruptions to the patient-physician encounter in a clinic in Dimona and to assess the effect of an intervention program designed to reduce the magnitude of this problem.

Methods: During an 8 day work period in March 1997 all patient-physician encounters were recorded and characterized. An intervention program was then designed and implemented to reduce the number of interruptions. Data were again collected a year after the initial data collection.

Results: During the 8 day study period prior to the intervention program there were 528 interruptions to 379 encounters (mean of 1.39 per encounter). The main causes of interruptions were entrance of uninvited patients to the examination room (31%) and telephone calls (27%). Most of the interruptions occurred during the morning hours between 8 and 10 a.m. (45%) and at the beginning of the week (Sunday 30%). After the intervention program there were 402 interruptions to 355 encounters (mean of 1.13 per appointment, P=0.21).

Conclusions: There was no statistically significant improvement in the number of interruptions following the intervention program. This finding is either the result of a local cultural phenomenon, or it indicates a national primary care health system problem that may require a long-term educational program to resolve it. Further research is needed on the magnitude, causes and consequences of interruptions in family practice and, if warranted, methods will have to devised to cope with this serious problem.

Background: Smoking rates have decreased in western countries as well as in Israel during the past 20 years.

Objectives: To estimate current rates of smoking and smoking cessation, and to assess factors associated with smoking and smoking cessation in family practice.

Methods: Prospective face-to-face interviews were conducted with 1,094 subjects, aged 16 years or older, registered in a family practice.

Results: Of all subjects studied, 746 (68.2%) were non-smokers, 237 (21.7%) were current smokers, and 111 (10.1%) had stopped smoking. Overall, 31.8% of the males and 13.8% of the females were current smokers, and 20.1% males and 2.4% females had stopped smoking. Current smoking and smoking cessation rates were significantly and inversely associated with age among males and females. Smoking rates were higher among males and females who were married, had 10-12 years of education, and among males of North African origin and females of Israeli origin. The number of cigarettes smoked per day was associated with smoking and smoking cessation in males, but not in females. The highest rate of quitting occurred among males who smoked 25 cigarettes per day. In a multiple regression analysis, gender and the number of cigarettes smoked per day were the most significant factors that predicted smoking cessation. The most common reason for stopping was the appearance of new signs of illness or the development of a new chronic disease, followed by a physician's recommendation to quit smoking.

Conclusions: Female smokers and male smokers who smoke less than 25 cigarettes per day are the least likely to quit smoking. Future programs should be designed for and targeted at these groups of patients.

Background: Smoking is the most important preventable cause of chronic disease in the western world. Many smokers want to quit, but have difficulty overcoming the addictive effect of nicotine.

Objectives: To assess the quitting rate of smokers who participated in smoking cessation groups and to characterize predictors of success or failure over a 1-3 year follow-up period.

Methods: We studied 89 participants in 7 groups. Questionnaires were completed at baseline and after a follow-up period of 1 to 3 years. Smoking cessation was determined by self-report and a carbon monoxide breath test.

Results: Of the 89 participants in the support groups 76 (85%) were located. An intention-to-treat analysis was done for these participants. At follow-up 25 (33%) were non-smokers. There was a 95% agreement rate between self-report of smoking status and CO breath analysis. There were no differences between quitters and non-quitters in education level, gender, age at initiation of smoking, previous quit attempts, extent of participation in group meetings, concern about gaining weight, Fagerstrom index, or the number of close friends or relatives who smoke. Belief in one's ability to quit, satisfaction with group meetings, and spouse support were significantly associated with success (P<0.01).

Conclusions: The quit rate was 33%. Self-report is a reliable method for assessing smoking status. Smokers' belief in their ability to quit must be reinforced. Spouse participation in some group meetings may be beneficial, as may the involvement of a dietician and an expert on exercise. Follow-up "booster" meetings may also help.

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CO= carbon monoxide

* In partial fulfilment of the requirements for an MD degree.

Background: Exposure of newborn animals to high concentrations of oxygen leads to diffuse alveolar damage similar to that seen in bronchopulmonary dysplasia in human infants. Therefore, neonatal rats are a suitable practical model of hyperoxic lung damage in human infants.

Objective: To determine the involvement of tumor necrosis factor-alpha and interleukin-6 in lung injury in neonatal rats exposed to 100% O2 concentration.

Methods: A randomized controlled study was designed in which litters of term Sprague-Dawley rat pups were assigned to experimental or control groups. The pups in the experimental group were placed in 100% O2 from birth for 9 days, while the control pups were placed in room air. Twelve to 15 pups from each group were sacrificed on day 1, 3, 6, 9 and 13 after birth for bronchoalveolar lavage collection and lung histologic study. The bronchoalveolar lavage fluid was assayed for TNFα and IL-6.

Results: Newborn rats exposed to 100% O2 for the first 9 days of life showed severe pulmonary edema and hypercellularity on days 1 and 3, which then improved to nearly complete resolution on days 6 and 9. Pulmonary TNFα was produced early on O2 exposure (day 3) and pulmonary IL-6 later (days 6 and 9).

Conclusions: Hyperoxia induces sequential production of pulmonary TNFα and IL-6, which corresponds to the severity of the pathological findings and the known inflammatory and anti-inflammatory role of these cytokines.

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TNFα= tumor necrosis factor-alpha

IL-6= interleukin-6