Background: Renal tubular dysgenesis is a rare lethal kidney abnormality clinically manifested by olighydramnios, anuria and respiratory distress. Most of the information on this entity is provided by case reports and short series.
Objectives: To evaluate the incidence and comparative frequency of clinical manifestations in different etiologic-pathogenic variants of RTD in Israel and in summarized published data.
Methods: Stillborn and neonatal autopsy material from nine medical centers in northern and central Israel was studied. Information concerning pregnancy, labor and postnatal status and autopsy findings of cases with histologically, histochemically and immunohistochemically confirmed RTD were obtained from corresponding reports and from published material.
Results: From the 1538 autopsies of fetuses (≥ 20 weeks gestation) and neonates that were performed between 1976 and 2007 we identified 12 cases of RTD (0.78%). Abnormality occurred more often (1.4%) in the Upper and Western Galilee than in Israel as a whole.
Conclusions: Our study and a review of the literature showed that the autosomal recessive variant of RTD was more frequent than twin-twin transfusion induced. Most symptoms were similar in all variants of RTD, but their frequency was different in each of them.
R. Michelis, S. Sela, W. Sbeit, H.I. Cohen and R. Reshef
Background: The trefoils factor family is a relatively new family of peptides. Their abundant expression in the epithelial cells of the gastrointestinal tract in the normal physiological state and in various ulcerative conditions suggests an important role in mucosal defense and repair. Infection with Helicobacter pylori interferes with normal mucosal activity.
Objectives: To investigate whether H. pylori infection alters the expression of trefoils TFF1 and TFF2 in the gastric mucosa of patients with H. pylori-associated chronic active gastritis, positive or negative for the CagA strain.
Methods: During investigation for dyspepsia, gastric biopsies and blood samples were obtained from patients who underwent upper gastrointestinal endoscopy. Rapid urease testing, histology for determination of H. pylori-associated CAG and Western analysis for TFF1 and TFF2 expression with antisera were performed. CagA state was determined using a commercial kit.
Results: TFF2 expression was significantly reduced in both groups of patients with H. pylori-associated CAG compared to healthy patients without H. pylori infection, particularly in CagA-positive patients. TFF1 expression showed a tendency of reduction (not significant) in this group only.
Conclusions: These results suggest that H. pylori-associated CAG has a deleterious effect on the expression of TFF2 in the gastric antrum. This reduced expression may contribute to the damage induced to the gastric mucosa by H. pylori.
 CAG = chronic active gastritis
A. Dortort Lazar, O. Shpilberg, M. Shaklai and O. Bairey
Background: There is currently no standard salvage chemotherapy for the 40–50% of patients with non-Hodgkin’s lymphoma who fail first-line treatment.
Objectives: To review the experience of a major tertiary medical center with DVIP (dexamethasone, etoposide, ifosfamide and cisplatin) salvage therapy for primary refractory/relapsing NHL.
Methods: We reviewed the records of all patients with NHL who received DVIP salvage therapy during the period 1993 to 2005.
Results: We identified 37 adult patients (mean age 56.3 years): 29 with aggressive lymphoma and 8 with indolent lymphoma. Mean event-free survival was 13.5 months (range 0–82 months), mean time between diagnosis and DVIP treatment 18.5 months (range 2–101), and mean number of DVIP cycles 1.9. Four patients (11%) achieved a complete response and 9 (24%) a partial response (overall response 35%). Consolidation with stem cell transplantation was used in 14 patients with aggressive lymphoma and 4 with indolent lymphoma; 14 patients, all with aggressive lymphoma, responded (12 complete, 2 partial). Of the 10 patients who underwent SCT despite no response to salvage DVIP, 6 achieved a complete response. Five year overall survival from diagnosis for the whole sample was 39.4 ± 8.7%, and 5 year post-DVIP overall survival 37.6 ± 8.0%. On multivariate analysis, SCT was the strongest predictor of survival (relative risk 0.73, P < 0.0001) followed by a high score on the International Prognostic Index (RR 3.71, P = 0.032).
Conclusions: DVIP salvage therapy for NHL was associated with a low response rate of 35% but a 5 year post-DVIP survival rate of 37.6%. Patients who are refractory to salvage treatment with DVIP might still be salvaged with SCT.
H. Gilutz, L. Novack, P. Shvartzman, J. Zelingher, D.Y. Bonneh, Y. Henkin, M. Maislos, R. Peleg, Z. Liss, G. Rabinowitz, D. Vardy, D. Zahger, R. Ilia,
N. Leibermann and A. Porath
Background: Dyslipidemia remains underdiagnosed and undertreated in patients with coronary artery disease. The Computer-based Clinical Decision Support System provides an opportunity to close these gaps.
Objectives: To study the impact of computerized intervention on secondary prevention of CAD.
Methods: The CDSS was programmed to automatically detect patients with CAD and to evaluate the availability of an updated lipoprotein profile and treatment with lipid-lowering drugs. The program produced automatic computer-generated monitoring and treatment recommendations. Adjusted primary clinics were randomly assigned to intervention (n=56) or standard care arms (n=56). Reminders were mailed to the primary medical teams in the intervention arm every 4 months updating them with current lipid levels and recommendations for further treatment. Compliance and lipid levels were monitored. The study group comprised all patients with CAD who were alive at least 3 months after hospitalization.
Results: Follow-up was available for 7448 patients with CAD (median 19.8 months, range 6–36 months). Overall, 51.7% of patients were adequately screened, and 55.7% of patients were compliant with treatment recommended to lower lipid level. A significant decrease in low density lipoprotein levels was observed in both arms, but was more pronounced in the intervention arm: 121.9 ± 34.2 vs. 124.3 ± 34.6 mg/dl (P < 0.02). A significantly lower rate of cardiac rehospitalizations was documented in patients who were adequately treated with lipid-lowering drugs, 37% vs. 40.9% (P < 0.001).
Conclusions: This initial assessment of our data represent a real-world snapshot where physicians and CAD patients often do not adhere to clinical guidelines, presenting a major obstacle to implementing effective secondary prevention. Our automatic computerized reminders system substantially facilitates adherence to guidelines and supports wide-range implementation.
Y. Senecky, G.Chodick, G. Diamond, D. Lobel, R. Drachman and D. Inbar
Background: Studies from many countries have reported an increasing prevalence of autistic spectrum disorder in childhood. No comprehensive epidemiological studies of ASD have been performed in Israel.
Objectives: To describe time trends in the reported number of patients with ASD in Israel and to characterize the demographic features of the reported patients.
Methods: We reviewed the charts of the National Insurance Institute of Israel from 1972 to 2004 for all children with a diagnosis of ASD receiving disability benefits.
Results: A total of 3509 children met the study criteria. Eighty percent were boys and 98% were Jewish. The incidence data showed an increase in the number of cases from zero in 1982–84 and 2 (1.2 per million capita under 18 years) in 1985 to a high of 428 cases in 2004 (190 per million).
Conclusions: This is the first comprehensive study of the incidence of ASD in Israel. According to data derived from official health records, the rate of occurrence of ASD has substantially increased in the last 20 years. Further studies are needed to determine if this is a true increase or if the findings were confounded by external factors, such as recent improvements in diagnostic measures and social stigmas.
I.R. Makhoul, H. Sprecher, R. Sawaid, P. Jakobi, T. Smolkin, P. Sujov, I. Kassis and S. Blazer
Background: According to the U.S. Centers for Disease Control guidelines, prolonged rupture of membranes mandates intrapartum antimicrobial prophylaxis for group B Streptococcus whenever maternal GBS status is unknown.
Objectives: To evaluate the local incidence, early detection and outcome of early-onset GBS sepsis in 35–42 week old neonates born after PROM to women with unknown GBS status who were not given intrapartum antimicrobial prophylaxis.
Methods: During a 1 year period, we studied all neonates born beyond 35 weeks gestation with maternal PROM ≥ 18 hours, unknown maternal GBS status and without prior administration of IAP. Complete blood count, C-reactive protein, blood culture and polymerase chain reaction amplification of bacterial 16S rRNA gene were performed in blood samples collected immediately after birth. Unfavorable outcome was defined by one or more of the following: GBS bacteremia, clinical signs of sepsis, or positive PCR.
Results: Of the 3616 liveborns 212 (5.9%) met the inclusion criteria. Only 12 (5.7%) of these neonates presented signs suggestive of sepsis. PCR was negative in all cases. Fifty-eight neonates (27.4%) had CRP > 1.0 mg/dl and/or complete blood count abnormalities, but these were not significantly associated with unfavorable outcome. Early-onset GBS sepsis occurred in one neonate in this high risk group (1/212 = 0.47%, 95% CI 0.012–2.6).
Conclusions: In this single-institution study, the incidence of early-onset GBS sepsis in neonates born after PROM of ≥ 18 hours, unknown maternal GBS status and no intrapartum antimicrobial prophylaxis was 0.47%.