Israel Medical Association Journal

Background: In southern Israel, a discrepancy between a relatively high prevalence of Group B streptococcus maternal carriage (12.3%) and a very low incidence of neonatal disease (0.1/1,000 live births) has been found despite the fact that no preventive strategy has been implemented.

Objectives: To determine the risk factors for maternal carriage in order to clarify this discrepancy and further examine the different aspects of GBS[1] in southern Israel.

Methods: Cultures for GBS were obtained from 681 healthy pregnant women and relevant demographic and obstetric data were collected. The medical records of 86 neonates born to carrier women were retrospectively examined. Statistical analysis was performed using the Pearson chi-square test.

Results: Women who were not born in Israel, particularly immigrants from the former USSR, were significantly prone to carry the pathogen compared to native Israeli women (Bedouin Arabs and Jews) (P = 0.03).

Conclusions: A high GBS transmission rate is expected among immigrants who came from areas with a high prevalence of maternal carriage to one with a low incidence of neonatal disease environment and were not subject to any preventive strategy. Clinical attention should be directed to this issue throughout Israel.

Background: Familial Mediterranean fever is an autosomal recessive disease characterized by recurrent attacks of fever and serositis. The disease is caused by mutations in the MEFV gene, presumed to act as a down-regulator of inflammation within the polymorphonuclear cells.

Objectives: To present the results of 412 FMF patients genotyped for three MEFV mutations, M694V, V726A and E148Q.

Results: The most frequent mutation, M694V, was detected in 47% of the carrier chromosomes. This mutation, especially common among North African Jewish FMF[1] patients, was not found in any of the Ashkenazi (East European origin) patients. Overall, one of the three mutations was detected in 70% of the carrier chromosomes. M694V/M694V was the most common genotype (27%), followed by M694V/V726A (16%). The full genotype could be assessed in 57% of the patients, and one disease-causing mutation in an additional 26%. Only one patient with the E148Q/E148Q genotype was detected despite a high carrier rate for this mutation in the Jewish population, a finding consistent with a low penetrance of this genotype. The M694V/M694V genotype was observed in 15 patients with amyloidosis compared to 4 amyloidosis patients with other genotypes (P < 0.0001).

Conclusions: Because of low penetrance and as yet other undetermined reasons, mutation analysis of the most common MEFV mutations supports a clinical diagnosis in only about 60% of patients with definite FMF.

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Background: The mortality rate associated with congenital heart surgery is apparently related to caseload.

Objective: To determine whether an increase in caseload over the long term at a single center affects management and outcome in children undergoing cardiac surgery.

Methods: Data were collected prospectively over a 4 year period from the computerized registry of the hospital’s pediatric intensive care unit. Five parameters were analyzed: age at surgery, type of surgery, preventive measures (open chest), surgery-related and other complications (diaphragm paralysis and acute renal failure, respectively), and mortality. The data of a single-type surgery (arterial switch) were analyzed for bypass time and mechanical ventilation on an annual basis.

Results: The age distribution changed over the years, with more children under 1 year of age (20% newborns) undergoing surgery by the fourth year of the study. The caseload increased from 216 in the first year to 330 in the fourth, with a concomitant decrease in mortality rate from 4.9% to 3.2%. The chest was left open in 3.2% of patients in the first year and in 9.2% in the fourth year. The rate of diaphragm paralysis decreased from 6% to 2.4%. Death due to acute renal failure in patients requiring dialysis decreased from more than 80% in the first 2 years to 36% in the last two. These changes show an improvement but failed to reach statistical significance. Regarding the arterial switch operation, there was a significant improvement in pump time and duration of mechanical ventilation.

Conclusions: The increase in caseload in pediatric cardiac surgery was accompanied by improved management, with a lower complications-related mortality rate. We suggest that for optimal care of children with congenital heart disorders, quality management resources should be concentrated in centers with high caseloads.
 

Background: Decreased elasticity of the aorta is associated with aging and several risk factors of atherosclerosis. The data regarding this phenomenon in patients with familial hypercholesterolemia are rather sparse.

Objectives: To evaluate non-invasively the elasticity of the proximal ascending aorta of 51 heterozygous FH[1] patients compared to 42 normal age and gender-matched controls.

Methods: Aortic elasticity was estimated by transthoracic echocardiography using the “pressure-strain” elastic modulus and aortic strain formulas.

Results: The elastic modulus score was higher in the FH group than in the controls (1.12 ± 0.91 10⁶ dynes/cm² vs. 0.65 ± 0.46 10⁶ dynes/cm² respectively, P = 0.01). This was consistent in both the pediatric (0.5 ± 0.2 10⁶ dynes/cm² vs. 0.4 ± 0.1 10⁶ dynes/cm² respectively, P = 0.009) and adult subgroups (1.3 ± 1.0 10⁶ dynes/cm² vs. 0.8 ± 0.5 10⁶ dynes/cm² respectively, P = 0.0004). Aortic strain was significantly lower in patients with FH than in controls (6 ± 4% vs. 9 ± 5% respectively, P = 0.0002). These findings reflected decreased elasticity of the proximal ascending aorta in the FH patients. In multivariate analysis, age, serum cholesterol level and serum triglycerides level were the independent predictors of the elastic modulus score, whereas age was the predictor of aortic strain.

Conclusions: The elasticity of the proximal ascending aorta is decreased in heterozygous FH patients.

Background: Multiple sclerosis is a chronic demyelinating disease of the central nervous system that presents with variable signs and symptoms. This variability in the clinical presentation may result in misdiagnosis, unnecessary referrals and misleading information to the patients.

Objectives: To identify the types of misdiagnoses made on the presentation of MS.

Methods: Fifty consecutive MS patients were questioned on their early symptoms, their mental status, the disease course until the diagnosis was confirmed, and the different diagnoses they received.

Results: The patients had been referred to 2.2 ± 1.3 specialists before seeing a neurologist, and learned about their disease 3.5 years after the onset of symptoms. Twenty-nine patients (58%) were initially given 41 wrong diagnoses. While the majority of women were misdiagnosed mentally, orthopedic work-up was offered to the men. Misdiagnosis of MS occurred most often in patients who presented with non-specific sensory symptoms that did not conform to a specific neurologic syndrome. The patients emphasized the fact that not knowing worsened their anxiety, whereas receiving the diagnosis enabled them to begin coping with their disease.

Conclusions: MS is often overlooked when patients present with non-specific sensory complaints. The difference in type of misdiagnosis between men and women may reflect a gender-dependent bias in the way physicians interpret sensory complaints.

Background: Radiofrequency ablation has recently become a viable treatment option for unresectable primary or secondary lesions confined to the liver.

Objective: To study the local therapeutic efficacy, side effects and complications of radiofrequency ablation for the treatment of hepatocellular carcinoma and liver metastases This is the first reported experience of radiofrequency ablation for treating malignant hepatic tumors in Israel.

Methods: Fifteen consecutive patients, aged 53–73 years, with 23 lesions (8 patients with HCC[1] and 7 with secondary liver tumors) underwent radiofrequency ablation under general anesthesia. RITA nine-array 5 cm thermal ablation catheter and the model 1500 generator were used. The mean diameter of all tumors was 4.28 cm (range 1–10 cm). Three lesions were 1–3 cm in diameter (small), 17 lesions measured 3.1–5 cm (medium), and 3 measured 5.1–10 cm (large).

Results: Complete necrosis was found in 8 (66%) of 12 HCCs by computed tomography scan. Of the remainder, diffuse tumor recurrence was demonstrated in three lesions (25%) after lipiodol injection and there was one local tumor recurrence. In the metastases group complete necrosis was found in 5 of 11 lesions (45%). One major complication (peritonitis) was treated with antibiotics and four (26%) minor complications (right pleural effusion, small subcapsular hematoma) were monitored.

Conclusions: Radiofrequency ablation appears to be an effective, safe and relatively simple procedure for the treatment of liver tumors.

Background: Prior studies have suggested that women are at higher risk for morbidity and mortality during coronary angioplasty, although long-term prognosis is similar after successful procedures.

Objectives: To examine the role of gender in coronary stenting, including immediate procedural success as well as early and late outcomes.

Methods: The study group comprised 560 consecutive patients (119 women and 441 men) who had undergone stenting over a 3 year period.

Results: The indications for coronary stenting were similar among women and men, and stents were successfully deployed at similar rates without complications (92 vs. 90% respectively). Cardiac death or myocardial infarction within 30 days of the procedure was observed in 5% of women and men, whereas none of the women, compared to 1.4% of men, had early revascularization. Bleeding complications occurred in 4% of women and 2% of men. During 10 ± 2.8 months of follow-up, 58% of women and men underwent repeat cardiac catheterization, revealing similar rates of restenosis, 36 vs. 32% respectively. During the study period, 3.3% of women as compared to 0.9% of men had a cardiac death (not significant). Cardiac death or myocardial infarction was observed in 7% of women and 8% of men, and the combined endpoint of death, myocardial infarction or revascularization, was noted in 24% and 26% respectively. Multivariate Cox analyses of the clinical, angiographic and procedural characteristics revealed that multiple stent deployment was the only predictor of major adverse cardiac event among men, whereas none of these characteristics predicted outcome in women.

Conclusion: Coronary stenting is performed with similar success rates among women and men, with similar restenosis rates, as well as early and late major adverse cardiac events.
 

Background: Infant mortality in Israel is twofold higher among non-Jews than Jews.

Objectives: To determine the impact of congenital malformations and Mendelian diseases on infant mortality.

Methods: We compared the causes of infant mortality in a 4 year period among Jewish and non-Jewish Israeli citizens. Classification was done by analyzing all the death reports according to whether or not the child had any known major malformation, Mendelian disease and/or a syndrome, irrespective of the immediate cause of death.

Results: The infant mortality among non-Jews was double that among Jews (9 versus 4.4 per 1,000 live births). The rate of children with malformations/genetic syndromes was 3.1 times higher among non-Jews than among Jews (2.94 vs. 1.25 per 1,000 live births). The most significant difference was in the rate of Mendelian diseases, which were 8.3 times more frequent in non-Jewish children (0.16 vs. 1.33 per 1,000 live births respectively). A Mendelian disease was diagnosed in almost 15% of the non-Jewish infants and in less than 5% of the Jewish infants.

Conclusions: The most striking difference between the Jewish and non-Jewish infants was the incidence of congenital malformations and Mendelian diseases parallel to the differences in the consanguinity rates between the two populations.
 

Background: Primary hyperparathyroidism in elderly patients is usually associated with additional co-morbidity that increases operative risk, and thus many geriatric patients are denied the benefit of surgery for a single parathyroid adenoma.

Objectives:  To evaluate the safety and efficacy of accurate single photon emission computed tomography sestamibi scintigraphy, enabling precise localization of a single adenoma, in the geriatric population

Methods: Twenty-two patients aged 70 years and over with biochemically proven PHPT[1] and with a single parathyroid adenoma identified by localization studies (sestamibi SPECT[2] scan and ultrasonography) underwent 23 operations over 29 months (out of a total of 140 patients operated upon during the same period). Immediate preoperative sestamibi scintigraphy and marking of focal adenoma uptake followed by intraoperative hand-held gamma probe were used for the removal of the parathyroid adenoma by unilateral minimal access surgery. Associated major co-morbid conditions and pre- and postoperative calcium, phosphorus and parathormone levels were recorded. Indications for surgery were listed and operative and postoperative complications were noted. The patients were followed for a mean period of 17.7 months using the same parameters.

Results: The 22 patients with PHPT had a mean age of 76.3 ± 5.9 years (range 70–88 years)  and a female to male ratio of 13:9. Associated co-morbidity included ischemic heart disease (n=15), hypertension (n=22), non-insulin-dependent diabetes mellitus (n=9), chronic obstructive pulmonary disease (n=3), and previous neck surgery (n=3). Mean preoperative serum calcium, phosphorous and PTH[3] were 11.7 ± 1.3 mg/dl, 2.5 ± 0.5 mg/dl and 160.9 ± 75.4 pg/ml respectively. In 20 of the 22 patients, surgery was successful in curing PHPT (91%). One patient had persistent hypercalcemia due to a missed adenoma, and repeat operation (by focused minimal access surgery) was successfully performed 2 weeks later. There were no complications and no morbidity postoperatively. Mean postoperative serum calcium, phosphorous and PTH were 9.6 ± 1.2 mg/dl, 3.0 ± 0.5 mg/dl and 35.2 ± 24 pg/ml respectively. In all patients, serum calcium levels remained normal (9.7 ± 1.3 mg/ml) after long-term follow-up (mean 17.7 ± 9.6 months).

Conclusions: Minimally invasive, radio-guided focused parathyroidectomy for a single adenoma is a safe and effective method to cure hyperparathyroidism in the elderly. Success of surgery is directly related to the surgeon's experience and to the precise localization marking provided by sestamibi scintigraphic SPECT localization and concurrent sonographic findings.

Background: While age at death is on the rise, the number of postmortem examinations is declining and is disproportionately low among the elderly population. Research on the subject of gender-associated pathology in the elderly is also scarce.

Objective: To seek eventual gender-related differences in autopsies of elderly patients.

Methods: We analyzed the data extracted from a published report on 93 PMEs[1] performed at a geriatric hospital during the past 20 years.

Results: Ninety-three autopsies, representing 1.2% of the 8,101 deaths during these 20 years, were performed. Forty-five of the deceased were women and 48 were men. The incidence of pulmonary embolism was significantly higher in women (28%) than in men (10%) (P< 0.02). There was no significant difference in the gender distribution of the other diagnoses.

Conclusion: Gender distribution of PME-based causes of death in elderly patients revealed a significant rate of pulmonary embolism in women. A thorough search of the medical literature revealed two previous studies with similar findings. Further research will determine whether pulmonary embolism is more frequent or whether it has a worse prognosis in frail elderly women.

Background: Although the preferred management of a patient presenting with an acute myocardial infarction is in a coronary care unit, data based on discharge diagnoses in Israel indicate that many of these patients are treated outside such units.

Objectives: To compare the demographic and clinical characteristics, treatment and mortality of AMI[1] patients treated inside and outside a CCU[2].

Methods: We compiled a registry of all patients admitted to three general hospitals in Haifa, Israel during January, March, May, July, September and November 1996.

Results: The non-CCU admission rate was 22%. CCU patients were younger (61.6 vs. 65.5 years), less likely to report a past AMI (18% vs. 34%), and arrived earlier at the emergency room. Non-CCU patients were more likely to present with severe heart failure (30 vs. 11%). Non-CCU patients received less aspirin (81 vs. 95%) and beta-blockers (62 vs. 80%). Upon discharge, these patients were less frequently prescribed beta-blockers and cardiac rehabilitation programs. CCU-treated patients had lower unadjusted mortality rates at both 30 days (odds ratio=0.35) and in the long term (hazards ratio=0.57). These ratios were attenuated after controlling for gender, age, type of AMI, and degree of heart failure (OR[3]=0.91 and HR[4]=0.78, respectively).

Conclusions: A relatively high proportion of AMI patients were treated outside a CCU, with older and sicker patients being denied admission to a CCU. The process of evidence-based care by cardiologists was preferable to that of internists both during the hospital stay and at discharge. In Israel a significant proportion of all AMI admissions are initially treated outside a CCU. Emphasis on increasing awareness in internal medicine departments to evidence-based care of AMI is indicated.