Israel Medical Association Journal

Neonatal lupus erythematosus is an uncommon transplacentally Acquired Autoimmune Disorder. The most common clinical manifestations are skin rash, congenital atrioventricular block, thrombocytopenia, leucopenia, anemia, and hepatosplenomegaly. Usually, the skin rash resembles subacute cutaneous lupus, but different forms of rash have been reported in Neonatal lupus erythematosus and some are rare forms. NLE should be suspected in babies with atypical skin lesions, even if present at birth.  

Chronic disease management has been a rapidly growing entity in the 21st century as a strategy for managing chronic illnesses in large populations. However, experience has shown that disease management programs have not been able to demonstrate their financial value. The objectives of disease management programs are to create quality benchmarks, such as principles and guidelines, and to establish a uniform set of metrics and a standardized methodology for evaluating them. In order to illuminate the essence of disease management and its components, as well as the complexity and the problematic nature of performing economic calculations of their profitability and value, we collected data from several reports that dealt with the economic intervention of disease management programs. The disease management economic evaluation is composed of a series of steps, including the following major categories: Data/information technology, information generation, assessment/recommendations, actionable customer plans, and program assessment/reassessment. We demonstrate the elements necessary for economic analysis. Disease management is one of the most innovative tools in the managed care environment and is still in the process of being defined. Therefore, objectives should include the creation of quality benchmarks, such as principles and guidelines, and the establishment of a uniform set of metrics and a standardized methodology for evaluating them.

Background: Iron deficiency is the most common single cause of anemia worldwide. Treatment consists of improved nutrition along with oral, intramuscular or intravenous iron administration.

Objectives: To describe the efficacy and adverse effects of intravenous iron sucrose therapy in a group of children with iron deficiency anemia who did not respond to oral iron therapy.

Methods: We conducted a prospective investigation of 45 children, aged 11 months to 16 years, whose oral iron therapy had failed. The children attended the Pediatric Ambulatory Care Unit where they received intravenous iron sucrose infusion.

Results: Forty-four of the 45 patients were non-compliant. Nine had Helicobacter pylori gastritis and 16 patients suffered from intestinal malabsorption from different causes. Before treatment, the blood mean hemoglobin concentration was 7.43 g/dl (range 5–10.1 g/dl). Fourteen days after treatment the mean hemoglobin concentration increased to 9.27 g/dl (SD 1.23) and 6 months later to 12.40 g/dl (SD 1.28). One patient demonstrated a severe side effect with temporary and reversible reduced blood pressure during treatment.

Conclusions: These preliminary data suggest that administration of intravenous iron in pediatric patients is well tolerated and has a good clinical result, with minimal adverse reactions.

Background: Parental fear and misconceptions about fever are widespread in western society. Ethnicity and sociodemographic factors have been suggested as contributing factors.

Objectives: To test the hypothesis that undue parental concern about fever is less in traditional than in western cultural-ethnic groups.

Methods: Bedouin (traditional society) and Jewish (western society) parents of children aged 0–5 years with fever were interviewed in a pediatric emergency unit. Interviews were conducted in the parents' most fluent language (Hebrew or Arabic). A quantitative variable (a 9 item “fever phobia” scale) was constructed.

Results: The parents of 101 Jewish and 100 Bedouin children were interviewed. More Bedouin parents were unemployed, had less formal education and had more and younger children than the Jewish parents. Parents of both groups expressed erroneous beliefs and practices about fever; quantitative but not qualitative differences in fever phobia variables were documented. Compared with their Jewish counterparts, more Bedouin parents believed that fever may cause brain damage and death, administered antipyretic medications for temperature ≤ 38ºC and at excessive doses, and consulted a physician within 24 hours even when the child had no signs of illness other than fever (all P values < 0.001). The mean fever phobia score was higher in the Bedouin than in the Jewish group (P < 0.001). By multivariate analysis, only the cultural-ethnic origin correlated with fever phobia.

Conclusions: A higher degree of fever phobia was found among parents belonging to the traditional Bedouin group as compared to western society parents.
 

Background: Roux-en-Y gastric bypass is currently considered the gold standard surgical option for the treatment of morbid obesity. Open RYGB[1] is associated with a high risk of complications. Laparoscopic RYGB has been shown to reduce perioperative morbidity and improve recovery.

Objectives: To review our experience with laparoscopic RYGB during a 19 month period.

Methods: The data were collected prospectively. The study group comprised all patients who underwent laparoscopic RYGB for treatment of morbid obesity as their primary operation between February 2006 and July 2007. The reported outcome included surgical results, weight loss, and improved status of co-morbidities, with follow-up of up to 19 months.

Results: The mean age of the 50 patients was 36.7 years. Mean body mass index was 44.7 kg/m² (range 35–76 kg/m²); mean duration of surgery was 171 minutes. There was no conversion to open surgery. The mean length of stay was 4 days (range 2–7 days). Five patients (10%) developed a complication, but none of them required early reoperation and there were no deaths. Mean follow-up was 7 months (range 40 days–19 months). The excess body weight loss was 55% and 61% at 6 and 12 months respectively. Diabetes resolved completely or significantly improved in all five patients with this condition, as did hypertension in eight patients out of nine.

Conclusions: Laparoscopic RYGB is feasible and safe. The results in terms of weight loss and correction of co-morbidities are comparable to other previously published studies. However, only surgeons with experience in advanced laparoscopic as well as bariatric surgery should attempt this procedure.

Background: The new syndrome, known as PFAPA, of periodic fever characterized by abrupt onset of fever, malaise, aphthous stomatitis, tonsillitis, pharyngitis and cervical adenopathy  has been described only in pediatric patients. It usually begins before the age of 5 years and in most cases resolves spontaneously before age 10. 

Objectives: To describe a series of adults with PFAPA syndrome.

Methods: This 6 year retrospective descriptive study includes all newly diagnosed incident adult cases aged 18 years and over referred to our center with symptomatology suggestive of PFAPA syndrome. Patients’ medical records were reviewed for past history of the disease, demographic characteristics, symptoms and signs, course of the disease, laboratory findings, and outcome following corticosteroid therapy. The comparison group included our pediatric cohort children (N=320, age 0–18 years) followed for the last 14 years (1994–2008).

Results: Fifteen adult patients were diagnosed with PFAPA syndrome. Episodes of fever occurred at 4.6 ± 1.3 week intervals, beginning at the age of 20.9 ± 7.5.  All patients had monthly attacks at the peak of the disease, with attacks recurring at 4–8 week intervals over the years. Between episodes the patients were apparently healthy, without any accompanying diseases. Attacks were aborted by a single 60 mg dose of oral prednisone in all patients.

Conclusions: This study reports the presence of PFAPA syndrome in adult patients. Although the disease is rare, an increased awareness by both patients and family physicians of this clinical syndrome has resulted in more frequent diagnosis in adult patients.
 

Background: In both adjuvant arthritis and rheumatoid arthritis edema and inflammation appear in synovial joints. Edema or effusion reflects an imbalance in lymph dynamics. Purified micronized flavonoid fraction is mainly used in the treatment of chronic venous insufficiency. This compound improves lymphatic drainage with a signicant increase in lymphatic flow and lymphatic pulsality. It is suggested that the beneficial effect of purified micronized flavonoid fraction may be involved in the treatment of adjuvant arthritis in rats.

Objectives: To evaluate the effect of Detralex on methotrexate prophylactic treatment of adjuvant arthritis in rats.

Methods: Groups of rats with adjuvant arthritis were treated with methotrexate (0.6 mg/kg/week), Detralex (20 mg/kg/day) and their combination for 50 days from adjuvant application. Hind paw swelling, arthrogram scores, serum albumin level, serum nitrite/nitrate concentrations, whole body mineral density and X-ray scans of synovial joints were evaluated as markers of inflammation and destructive changes associated with arthritis.

Results: Long-term prophylactic treatment with low dose methotrexate significantly inhibited the markers of both inflammation and arthritis. Detralex administered alone slightly decreased both the hind paw swelling and the arthritic score. Other inflammatory and arthritic markers were not significantly influenced. However, detralex combined with methotrexate markedly potentiated the beneficial effects of methotrexate, which resulted in a more significant reduction in hind paw swelling, arthritic scores, and serum concentrations of nitrite/nitrate. Interestingly, the arthritis-induced decrease of BMD[1] in AA[2] rats was significantly lower only in the group treated with the combination of Detralex+methotrexate.

Conclusion: Detralex increased the therapeutic efficacy of methotrexate basal treatment in AA. We suggest that this may be related to the beneficial effect of Detralex on microcirculation, especially on venules and lymphatic vessels.