Israel Medical Association Journal

Objectives: To assess the role of electrocardiography-gated CT-angiography (ECG-CTA) in the routine evaluation of CHD in neonates and infants particularly for the assessment of extracardiac findings.

Methods: The study cohort comprised 40 consecutive patients who underwent trans-thoracic echocardiography (TTE) and ECG-CTA. TTE and ECG-gated CTA findings regarding extracardiac vascular structures, coronary arteries and airways were compared with surgical or cardiac catheterization findings. Scans were evaluated for image quality using a subjective visual scale (from 1 to 4). Effective radiation dose was calculated for each scan.

Results: Median age was 28 ± 88 days and mean weight 3.7 ± 1.5 kg. Diagnostic quality was good or excellent (visual image score 3–4) in 39 of 40 scans (97.5%). ECG-CTA provided important additional information regarding extracardiac vascular structures and airway anatomy, complementing TTE in 75.6% of scans. Overall sensitivity of ECG-gated CTA for detecting extracardiac findings as compared with operative and cardiac catheterization findings was 97.6%. The calculated mean effective radiation dose was 1.4 ± 0.07 mSv (range 1.014–2.3 mSv).

Conclusions: ECG-CTA is an accurate modality for demonstrating extracardiac structures in complex CHD. It provides important complementary information to TTE regarding extracardiac vascular structures and coronary artery anatomy. This modality may obviate the need for invasive cardiac catheterization, thus exposing the patient to a much lower radiation dose. 

Objectives: To investigate the prevalence of hypertension and its relation to body mass index (BMI) in obese children aged 9–17 years in Israel.

Methods: Weight, height, BMI, and systolic and diastolic blood pressure (BP) (twice) were measured in children attending general and pediatric endocrine clinics. Obesity was defined as BMI ≥ 95th percentile and overweight as BMI ≥ 85th percentile. Pre-hypertension and hypertension were defined as systolic and/or diastolic BP ≥ 90th percentile for age, gender and height and BP ≥ 95th percentile respectively. In children with pre-hypertension or hypertension, repeated measurements were performed.

Results: We evaluated 264 children of whom 152 had BMI ≥ 85th percentile (study group). Their mean age was 12.5 years. The prevalence of elevated BP (both pre-hypertension and hypertension) in the study group was 44.1% and 31% at the first and second measurements respectively, compared to 11.6% and 1.9% in the normal-weight group. Hypertension was documented in 17.2% of the study group at the second measurement.

Conclusions: Elevated BP was diagnosed in 31% of overweight and obese children and adolescents. Increased awareness and early diagnosis and treatment are essential.

Objectives: To describe the results of nerve conduction studies in patients with anogenital pruritus, brachioradial pruritus and scalp dysesthesia, and compare these sites to typical sites of lichen simplex chronicus (LSC).

Methods: The study summarizes previously published data combined with unpublished data of patients with scalp dysesthesia. Nerve conduction studies included measurements of distal sensory and motor latency, conduction velocity and F-responses.

Results: A neuropathy was demonstrated in 29 of 36 patients with anogenital pruritus (80.5%), 8/14 with brachioradial pruritus (57.1%) and 4/9 with scalp dysesthesia (44.4%). The typical sites overlapped with some but not all LSC sites.

Conclusions: A considerable proportion of patients with brachioradial pruritus, anogenital pruritus and scalp dysesthesia have abnormal nerve conduction findings, suggesting a neuropathic origin. The skin sites overlap with some common LSC sites, suggesting that in some cases of LSC a local neuropathy could be a possible cause.

Objectives: To describe a single-center experience with fenestrated and branched endografts for the treatment of complex aortic aneurysms.

Methods: We reviewed all cases of complex aortic aneurysms treated with branched or fenestrated devices in our center. Data collected included device specifics, perioperative morbidity and mortality, re-intervention rates and mid-term results.

Results: Between 2007 and 2012 nine patients were treated with branched and fenestrated stent grafts. Mean age was 73 years. Mean aneurysm size was 63 mm. Perioperative mortality was 22% (2/9). During the follow-up, re-interventions were required in 3 patients (33%). Of 34 visceral artery branches 33 remained patent, resulting in a patency rate of 97%. Sac expansion was seen in a single patient due to a large endoleak. No late aneurysm- related deaths occurred.

Conclusions: Branched and fenestrated stent grafts are feasible and relatively safe alternatives for the treatment of complex aortic aneurysms involving the visceral segment. Further research is needed to determine the long-term durability of this new technology. 

Objectives: To examine the quality and accuracy of diagnoses recorded in the ED in order to appraise its potential utility as a guage of the population's medical needs.

Methods: Using the Delphi process, a preliminary list of health indicators generated by an expert focus group was transformed into a query to the Ministry of Health's database. In parallel, medical charts were reviewed in four hospitals to compare the handwritten diagnosis in the medical record with that recorded on the standard diagnosis "pick list" coding sheet. Quantity and quality of coding were assessed using explicit criteria.

Results: During 2010 a total of 17,761 charts were reviewed; diagnoses were not coded in 42%. The accuracy of existing coding was excellent (mismatch 1%–5%). Database query (2,670,300 visits to 28 hospitals in 2009) demonstrated potential benefits of these data as indicators of regional health needs.

Conclusions: The findings suggest that an increase in the provision of community care may reduce ED attendance. Information on ED visits can be used to support health care planning. A "pick list" form with common diagnoses can facilitate quality recording of diagnoses in a busy ED, profiling the population’s health needs in order to optimize care. Better compliance with the directive to code diagnosis is desired.

Objectives: To assess the results of endovascular revascularization for lower extremity ischemia in dialysis patients.

Methods: We conducted a retrospective review of all dialysis patients who underwent endovascular treatment for critical limb ischemia (CLI) in our institution between 2007 and 2011. Data collected included comorbidities, clinical presentation, anatomic distribution of vascular lesions, amputation and survival rates.

Results: We identified 50 limbs (41 patients). Indications included: gangrene in 22%, non-healing wounds in 45%, rest pain in 31%, and debilitating claudication in 4%. Mean follow-up was 12 months (1–51 months). Nineteen patients required amputations. Freedom from amputation at 5 years was 40%. Factors associated with amputation included non-healing wounds or gangrene (68% and 36% respectively) and diabetes (P < 0.05). The survival rate was 80% after 5 years.

Conclusions:  Despite improvement in endovascular techniques for lower extremity revascularization, the incidence of limb salvage among dialysis patients remains poor, resulting in a high rate of major amputations. 

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 the safety and applicability of the Elekta Synergy-S radiation unit for the treatment of spinal tumors.

Methods: We retrospectively reviewed all patients treated with stereotactic radiosurgery to spinal tumors between November 2007 and June 2011.

Results: Thirty-four patients were treated for 41 lesions. Treatment indications were local tumor control and pain palliation. The mean follow-up was 10.8 ± 11.6 months (range 0.5–38 months). No acute radiation toxicity or new neurological deficits occurred during the follow-up period. Local tumor control was achieved in 21 of the 24 lesions (87.5%) available for radiological follow-up at a median of 9.8 months (range 3–32 months). Good analgesia was achieved in 24/30 lesions (80%) that presented with intractable pain.

Conclusions: The safety and feasibility of delivering single and multiple-fraction stereotactic spinal irradiation was demonstrated and became a standard treatment option in our institution.