Israel Medical Association Journal

Objectives: To evaluate if fetal reduction of triplets to twins improves outcome.

Methods: We analyzed the outcome of 80 triplet gestations cared for at Rambam Health Care Campus in the last decade; 34 families decided to continue the pregnancy as triplets and 46 opted for MFPR to twins.

Results: The mean gestational age at delivery was 32.3 weeks for triplets and 35.6 weeks for twins after MFPR. Severe prematurity (delivery before 32 gestational weeks) was experienced in 37.5% of triplets and in 7% of twins. Consequently, the rate of severe neonatal morbidity (respiratory distress syndrome, bronchopulmonary dysplasia, intraventricular hemorrhage) and of neonatal death was significantly higher in unreduced triplets, as was the length of hospitalization in the neonatal intensive care unit (31.4 vs. 15.7, respectively). Overall, the likelihood of a family with triplets to take home all three neonates was 80%; the likelihood to take home three healthy babies was 71.5%.

Conclusions: MFPR reduces the risk of severe prematurity and the neonatal morbidity of triplets. A secondary benefit is the reduction of cost of care per survivor. Our results indicate that MFPR should be offered in triplet gestations.

Objectives: To describe the phenotypic and genotypic features in five Bulgarian Jewish patients, from different families, with autosomal dominant OPMD.

Methods: We performed clinical follow-up, electrodiagnostic tests and mutation detection. Blood samples were obtained after informed consent and DNA was extracted; measurement of GCG repeats in both PABPN1 alleles and sequencing of OPMD mutations were performed according to standard techniques.

Results: We identified five patients (four females), aged 58 to 71 years, with bilateral ptosis, dysphagia, dysphonia (n=3) and myopathic motor units by electromyography. In all patients we noticed proximal weakness of the upper limbs with winging scapulae in three of them. All cases shared the (GCG)13-(GCG)10 PABPN1 genotype.

Conclusions: OPMD among Bulgarian Jews is produced by a (GCG)13 expansion, identical to the mutation in Uzbek Jews and French Canadians. In addition to the classical neurological and neuro-ophthalmological features, early shoulder girdle weakness is common in Bulgarian Jewish patients; this is an unusual feature during the early stages of OPMD produced by the same mutation in other populations. We suggest that besides the disease-producing GCG expansion, additional ethnicity-related genetic factors may influence the OPMD phenotype. OPMD is a rare disease, and the identification of five affected families in the rather small Bulgarian Jewish community in Israel probably represents a new cluster; future haplotype studies may elucidate whether a founder effect occurred. 

Background: Coronary slow flow phenomenon (CSFP) is a functional and structural disease that is diagnosed by coronary angiogram.    



Objectives: To evaluate the possible association between CSFP and small artery elasticity in an effort to understand the pathogenesis of CSFP.

Methods: The study population comprised 12 patients with normal coronary arteries and CSFP and 12 with normal coronary arteries without CSFP. We measured conjugated diene formation at 234 nm during low density lipoprotein (LDL) oxidation, as well as platelet aggregation. We estimated, non-invasively, arterial elasticity parameters. Mann-Whitney non-parametric test was used to compare differences between the groups. Data are presented as mean ± standard deviation.

Results: Waist circumference was 99.2 ± 8.8 cm and 114.9 ± 10.5 cm in the normal flow and CSFP groups, respectively (P = 0.003). Four patients in the CSFP group and 1 in the normal flow group had type 2 diabetes. Area under the curve in the oral glucose tolerance test was 22% higher in the CSFP than in the normal group (P = 0.04). There was no difference in systolic and diastolic blood pressure, plasma concentrations of total cholesterol, triglycerides, high density lipoprotein, LDL and platelet aggregation parameters between the groups. Lag time required until initiation of LDL oxidation in the presence of CuSO₄ was 17% longer (P = 0.02) and homocysteine fasting plasma concentration was 81% lower (P = 0.05) in the normal flow group. Large artery elasticity was the same in both groups. Small artery elasticity was 5 ± 1.5 ml/mmHgx100 in normal flow subjects and 6.1 ± 1.9 ml/mmHgx100 in the CSFP patients (P = 0.02).

Conclusions: Patients with CSFP had more metabolic derangements. Arterial stiffness was not increased in CSFP.

Objectives: To evaluate in a randomized control trial whether uninterrupted administration of angiotensin II (AngII) blockade medications influence estimated glomerular filtration rate (eGFR) in patients undergoing non-emergent coronary angiography.

Methods: Patients receiving treatment with angiotensin-converting enzyme inhibitors/angiotensin receptor blockers (ACE-I/ARB) were recruited consecutively. The enrolled subjects were randomized into three groups at a 1:1:1 ratio: group A (ACE/ARB stopped 24 hours prior to the procedure and restarted immediately after the procedure), group B (ACE/ARB stopped 24 hours prior to the procedure and restarted 24 hours after the procedure), and group C (ACE/ARB continued throughout the study period). Plasma creatinine was measured and eGFR was calculated according to the Cockroft-Gault equation before and 48 hours after the coronary angiography. The primary endpoint was a change in eGFR at 48 hours.

Results: Groups A, B and C comprised 30, 31 and 33 patients respectively. The mean age of the study population was 65 ± 12 years and 67% were males. Fifty percent of the subjects had diabetes mellitus. The primary endpoint analysis showed that at 48 hours after the procedure there was no difference in ΔeGFR between groups A and C (4.25 ± 12.19 vs. 4.65 ± 11.76, P = 0.90) and groups B and C (3.72 ± 17.42 vs. 4.65 ± 11.76, P = 0.82). In post-hoc analysis the patients were clustered according to the following groups: medical alternation (group A and B) versus control (group C) and to baseline eGFR ≥ 60 ml/min vs. eGFR < 60 ml/min. In patients with baseline eGFR < 60 ml/min the ΔeGFR (baseline eGFR-eGFR 48 hours post-angiography) was significantly different between the intervention vs. control group (median 5.61 vs. median -2.19, P = 0.03 respectively). While in patients with baseline eGFR ≥ 60 ml/min there was no significant difference in ΔeGFR between the intervention and control groups.

Conclusions: ACE-I and ARB can safely be used before and after coronary angiography in patients with eGFR ≥ 60 ml/min. 

Background: Janus kinase-2 (JAK2) is mutated in a high proportion of patients with polycythemia vera and in a smaller number with essential thrombocythemia and primary myelofibrosis. Mutated JAK2 is an important diagnostic marker for myeloproliferative neoplasm (MPN) and may also play a major role in the pathogenesis of MPN.

Objectives: To evaluate the prevalence of mutated JAK2 (JAK2-V617F) among patients with major intraabdominal vein thrombosis who had normal blood counts at diagnosis of the initial event.

Methods: The medical records of patients who presented with a major intraabdominal venous thrombosis and normal peripheral blood counts were obtained. JAK2-V617F mutation status was determined by real-time polymerase chain reaction.

Results: Twenty-two patients were available for this analysis and 9 (41%) were found to have JAK2-V617F. Patients with positive JAK2-V617F were younger and had more frequent clinical splenomegaly than those with wild-type JAK2.

Conclusions: A high proportion of patients presenting with “idiopathic” major intraabdominal vein thrombosis and normal blood counts carry JAK2-V617F. We recommend searching for the mutation in this clinical setting to detect patients with occult MPN.

Objective: To assess the impact of multiple births on the severity of RSV infection and define risk factors for acquiring RSV infection in infants of multiple birth.

Methods: Clinical data on infants hospitalized with RSV infection between 2008 and 2010 were retrospectively collected.

Results: Twins comprised 7.6% (66/875) of hospitalized infants with RSV bronchiolitis during the study period. Infants in the twin group were younger (122.4 ± 131.7 vs. 204.5 ± 278.8 days, P = 0.014), had a lower mean gestational age (35.3 ± 2.6 vs. 38.6 ± 2.5 weeks, P < 0.001), and were more likely to have been born prematurely compared with singleton infants (65.6% vs. 13%, P < 0.001). On a multivariable logistic regression analysis, young age, early gestational age and male gender were the only variables identified as risk factors for pediatric intensive care unit admission (P < 0.001, P < 0.001 and P = 0.03, respectively). In contrast, the mere fact of a child being a twin was not found to be a significant risk factor for disease severity. In addition, if one twin is hospitalized due to RSV infection, the other has a 34% chance of also being hospitalized with bronchiolitis. Young age was a significant risk factor for hospitalization of the second twin (P < 0.001).

Conclusions: Our findings suggest that twins hospitalized with RSV bronchiolitis do not have an increased risk for severe infection as compared to singletons. However, a twin of an infant hospitalized with RSV infection has a considerable risk of also being hospitalized with bronchiolitis, thus close monitoring is recommended. 

Objectives: To assess the preliminary results of transcatheter mitral valve leaflet repair (TMLR) in a single academic center.

Methods: Data were collected prospectively in the cardiology department of Rabin Medical Center in 2012. Ten consecutive patients (age 69.3 ± 15.9 years, ejection fraction 36.5 ± 9.4) who were poor surgical candidates with severe functional MR underwent general anesthesia, followed by trans-septal puncture and a TMLR procedure using the MitraClip device.

Results: All 10 patients were considered to have severe functional MR prior to TMLR treatment and were all symptomatic; the mean New York Heart Association (NYHA) class was 3.4 ± 0.5. The MR severity was 4 ± 0. There were no immediate complications or failures of the procedure. One patient died on day 5 due to massive gastrointestinal bleeding. Immediately following TMLR all 10 patients showed a profound MR reduction to a mean severity grade of 1.6 ± 0.6. At one month after the procedure, NYHA had decreased to an average of 1.7 ± 1.0 and was at least grade 2 in all but one patient. After 6 months the MR remained ≤ 2 in six of eight patients, with a NYHA average of 1.4 ± 0.5.

Conclusions: The MitraClip procedure was shown to be relatively safe, providing significant clinical benefits to a relatively sick population with severe MR. It is therefore an important alternative to surgery in these high risk patients.

Objectives: To investigate the 30 day clinical outcome of the first 300 consecutive patients treated with transfemoral TAVI at the Tel Aviv Medical Center.

Methods: The CoreValve was used in 250 patients and the Edwards-Sapien valve in 50 patients. The mean age of the patients was 83 ± 5.3 years (range 63–98 years) and the mean valve area 0.69 ± 0.18 cm² (range 0.3–0.9 cm²); 62% were women.

Results: The procedural success rate was 100%, and 30 day follow-up was done in all the patients. The average Euro-score for the cohort was 26 ± 13 (range 1.5–67). Total in-hospital mortality and 30 day mortality were both 2.3% (7 patients). Sixty-seven patients (22%) underwent permanent pacemaker implantation after the TAVI procedure, mostly due to new onset of left bundle brunch block and prolonged PR interval or to high degree atrioventricular block. The rate of stroke was 1.7% (5 patients). Forty-one patients (13.7%) had vascular complications, of whom 9 (3%) were defined as major vascular complications (according to the VARC definition).

Conclusions: The 30 day clinical outcome in the first 300 consecutive TAVI patients in our center was favorable, with a mortality rate of 2.3% and low rates of stroke (1.7%) and major vascular complications (3%).

Objectives: To evaluate the retention of CPR skills and confidence in delivering CPR by preclinical medical students.

Methods: A questionnaire and the Objective Structured Clinical Examination (OSCE) were used to assess confidence and CPR skills among preclinical, second and third-year medical students who had passed a first-aid course during their first year but were not retrained since.

Results: The study group comprised 64 students: 35 were 1 year after training and 29 were 2 years after training. The groups were demographically similar. Preparedness, recollection and confidence in delivering CPR were significantly lower in the 2 years after training group compared to those 1 year after training (P < 0.05). The mean OSCE score was 19.8 ± 5.2 (of 27) lower in those 2 years post- training than those 1 year post-training (17.8 ± 6.35 vs. 21.4 ± 3.4 respectively, P = 0.009). Only 70% passed the OSCE, considerably less in students 2 years post-training than in those 1 year post-training (52% vs. 86%, P < 0.01). Lowest retention was found in checking safety, pulse check, airway opening, rescue breathing and ventilation technique skills. A 1 year interval was chosen by 81% of the participants as the optimal interval for retraining (91% vs. 71% in the 2 years post-training group vs. the 1 year post- training group respectively, P = 0.08).

Conclusions: Confidence and CPR skills of preclinical medical students deteriorate significantly within 1 year post-training, reaching an unacceptable level 2 years post-training. We recommend refresher training at least every year.