Israel Medical Association Journal

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: Treatment of hyperlipidemia is important for secondary prevention in patients suffering from coronary heart disease. It has been proven that “young elderly” (patients aged 65–75 years) can benefit from the treatment at least as much as younger patients.

Objective: To assess the adequacy of treatment as part of secondary prevention in “young elderly” and younger patients.

Methods: In this prospective study, 389 patients discharged from the hospital with the diagnosis of coronary heart disease were divided according to age groups. Data were collected regarding lipid profile examinations, dietary and drug therapy, and results of lipid profiles.

Results: Less than one-third of the patients achieved target low density lipoprotein levels. More patients in the older age group achieved the treatment goals. The goals were achieved despite the fact that the percentage of patients treated with lipid-lowering drugs was lower in the older age group.

Conclusion: The percentage of patients treated for hyperlipidemia as part of a secondary prevention plan in Israel is similar to that in other developed countries. The fact that more “young elderly” patients achieve adequate lipid profiles compared to younger patients indicates that there is no age discrimination towards this patient group. The finding that less than one-third of the patients reach the treatment goals should prompt physicians to treat hyperlipidemia more aggressively.

Background:  After the introduction of endoscopic techniques to other surgical fields, like general surgery, gynecology and thoracic surgery, cardiac surgeons sought their own methods of using minimally invasive techniques.

Objectives:  To examine whether this approach is less invasive and yields better results, more desirable cosmetic results, and a more rapid and complete rehabilitation, maintaining safety, efficacy, and outcome equivalent to those of more established procedures, such as median sternotomy.

Methods:  From January 2000 to July 2001, 22 patients underwent video-assisted port-access mitral or aortic valve repair or replacement with the Heartport system in our department, and one underwent closure of atrial septal defect.

Results:  Intraoperative transesophageal echocardiography revealed excellent functional results. Total operating room time, perfusion time, and cross-clamp time in this technique decreased with our growing experience, and remains stable. There were no intraoperative reversals to mid-steronomy, no mortalities, and only one complication 24 hours after surgery.

Conclusions:  Thoracoscopic assisted cardiac surgery (via port access) provides all the advantages of minimally invasive surgery, accelerates recovery, decreases pain, and maintains overall surgical efficacy, while avoiding the complications and pathology of mid-sternotomy.  For appropriate patients, this is the method of choice in our department.

Background: Onchocerciasis results from infestation by the nematode Onchocerca volvulus, and is characterized clinically by troublesome itching, skin lesions and eye manifestations. Since 1992, approximately 9,000 immigrants have arrived in Israel from the Kuwara province of northwest Ethiopia where the prevalence of onchocerciasis is particularly high.

Objectives: To determine whether onchocerciasis is the cause of cutaneous and ocular symptoms among recent immigrants from the Kuwara province in Ethiopia

Methods: We examined 1,200 recent immigrants from the Kuwara province residing at the Mevasseret Zion immigration center outside Jerusalem. Among them, patients with cutaneous signs suggestive of onchocerciasis underwent a skin-snip biopsy and a thorough eye examination.

Results: In the detailed skin examination performed in 83 patients, the most common skin finding was chronic papular onchodermatitis, found in more than 46 patients (55%);depigmentation and atrophy was found in 13 (15%) and 12 (14%), respectively. In 40 patients (48%), living microfilaria were detected in their skin snips. Of the 65 patients who underwent a thorough eye examination, 45 patients (66%) had ocular complaints. Corneal abnormalities were found in 55 of the 130 eyes (42%), active anterior segment intraocular inflammation and live microfilariae were found in 4 eyes (3%) and lens changes in 16 eyes (1 %). Eleven eyes (9%) showed retinal or choroidal changes.

Conclusions: Skin and eye manifestations associated with onchocerciasis are prevalent among symptomatic Ethiopian immigrants to Israel from the Kuwara province.

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.

Diffuse idiopathic skeletal hyperostosis is often incorporated into osteoarthritis. Although DISH[1] often coexists with OA, patients affected by this disorder differ from patients with primary OA in several aspects: prevalence in the general population, gender distribution, anatomic site of primary involvement, magnitude and distribution in the spine and the peripheral joints. DISH is a distinct clinical entity. Its recognition as such should stimulate clinicians and researchers to focus on its pathogenesis, treatment and prevention.

Sepsis is an infection-induced inflammatory syndrome that results in a complex network of adaptive and maladaptive alterations in homeostatic mechanisms. Severe sepsis, defined as sepsis associated with acute organ failure, is a serious disease with a mortality rate of 30–50%. The coagulation system, through complex interactions, has an important role in the final outcome of the sepsis-induced inflammatory cascade. A fine and delicate balance that normally exists between anticoagulant mechanisms and the procoagulant response is altered in sepsis. Activated protein C, an endogenous vitamin K-dependent anticoagulant, plays a major role in the down-regulation of the procoagulant arm. It also possesses anti-inflammatory properties. Endothelial damage during sepsis impairs the endothelium-dependent activation of protein C, thus shifting the balance towards thrombosis. This shift may contribute to the development of sepsis-related multi-organ failure. Evidence suggesting that activation of the coagulation system may contribute to sepsis-related morbidity and mortality has led to extensive research attempting to correct the hemostatic defects seen in septic patients. Indeed, a recent randomized controlled trial demonstrated a reduction in overall mortality in patients with severe sepsis treated with APC[1]. In this review we discuss the pathogenesis of the coagulopathy of sepsis, as well as the new therapeutic approaches aimed at correcting the defects in the coagulation system.

The induced sputum technique allows sampling of the airways in a non-invasive manner and thus offers a unique opportunity to identify biomarkers of potential clinical utility in respiratory medicine. Sputum cells were originally examined in stained smears and the procedure was applied in both research and clinical settings from the 1950s through the 1970s. The cells, recovered from spontaneous coughing, were used to study lung cancer and respiratory infections and, later on, to diagnose Pneumocystis carinii pneumonia in patients infected with human immunodeficiency virus. The method was largely improved by the induction of sputum with aerosol of hypertonic saline and was extended to become part of the assessment of airway inflammation in bronchial asthma and chronic obstructive pulmonary disease. It was recently shown that induced sputum can be used to study interstitial lung diseases and, more specifically, sarcoidosis, non-granulomatous ILD[1], occupational lung diseases and other systemic diseases with lung involvement.

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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: Technetium-99m sestamibi scintigraphy has become one of the most popular techniques for localization of the parathyroid gland after failure of primary neck exploration.

Objective: To examine the efficacy of sestamibi with the hand-held gamma ray detecting probe for the identification of parathyroid adenomas during revision parathyroidectomy.

Methods: We reviewed six cases of probe-assisted neck exploration for parathyroid lesions following unsuccessful primary exploration.

Results: In all cases, the pathologic glands were successfully detected and removed.

Conclusions: With careful planning, a gamma ray detecting probe can be used optimally at 2–3 hours after technetium-99m sestamibi injection. The probe is efficient, easy and convenient to use.
 

Background: The osteoporosis-pseudoglioma syndrome is a rare autosomal recessive disorder characterized by severe juvenile-onset osteoporosis and congenital or early-onset blindness. Other manifestations include muscular hypotonia, ligamentous laxity, mild mental retardation and seizures. The gene responsible was recently identified to be the low density lipoprotein receptor-related family member LRP5 on chromosome 11q11-12.

Objective: To measure bone density in two siblings with the OPPG[1] syndrome as well as in their family members (parents and siblings).

Methods: Bone mineral density was determined in the lumbar spine (antero-posterior), femoral neck, two-thirds distal forearm (>95% cortical bone) and ultradistal forearm (predominantly trabecular bone) by dual-energy X-ray absorptiometry.

Results: The studies revealed osteoporotic changes both in the patients and the carriers.

Conclusion: The findings demonstrate that OPPG carriers have reduced bone mass, which is a risk factor for development of early osteoporotic changes.

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Background: Cystinuria is an autosomal recessive disease that is manifested by the development of kidney stones. Mutations in SLC3A1 cause type I disease, while mutations in SLC7A9 are associated with non-type I disease. In Israel cystinuria is especially common among Libyan Jews who suffer from non-type I disease.

Objectives: To compare clinical manifestations of patients with mutations in SLC3A1 to those with mutations in SLC7A9, and to assess the carrier rate among unaffected Libyan Jewish controls.

Methods: Clinical manifestations were evaluated in patients with mutations in SLC3A1 and in patients with mutations in SLC7A9. Carrier rates for two SLC7A9 mutations were assessed in 287 unaffected Libyan Jewish controls.

Results: Twelve patients with mutations in SLC3A1 were compared to 15 patients with mutations in SLC7A9. No differences were detected between the patients with mutations in SLC3A1 and those with mutations in SLC7A9 in relation to the age of disease onset, the estimated number of stones, the number of invasive procedures, the number of patients receiving drug therapy, or the patients’ urinary pH. Eleven of the unaffected Libyan Jewish controls were found heterozygotes for the V170M mutation, establishing a carrier rate of 1:25. The 1584+3 del AAGT mutation was not found in any of the Libyan Jewish controls.

Conclusion: Mutations in SLC3A1 and SLC7A9 cystinuria patients result in indistinguishable disease manifestations. The high carrier rate among Libyan Jews is a result of a single missense mutation, V170M.