Israel Medical Association Journal

Background: Ocular hypotony is a common unexplained feature of myotonic dystrophy type 1. Spuriously low applanation tonometric readings can be caused by thin corneas, flat corneal curvature and corneal edema.

Objectives: To determine whether structure abnormalities of the cornea cause spuriously low readings in applanation tonometry.

Methods: We utilized a TMS-2N corneal topographer, a NonconRobo SP-6000 Specular microscope and a Corneo-Gage Plus 1A Pachymeter to examine seven patients with DM1[1] and eight healthy controls. Intraocular pressure, central corneal thickness, and endothelial cell density were measured, and simulated keratometry readings were made. Cornea guttata and irregularity of corneal topography patterns were also sought.

Results: The mean intraocular pressure was 9.86 ± 1.29 mmHg for all patients (intraocular operated and non‑operated eyes) and 12.88 ± 1.89 mmHg for the controls (P = 0.000021, two-tailed t-test). Central corneal thickness was 530.57 ± 35.30 micron for all patients and 535.00 ± 39.62 micron for the controls (P = 0.75, two-tailed t-test). Endothelial cell density was 3164 ± 761 cells/mm² for all patients and 3148 ± 395 cells/mm² for the controls (P = 0.94, two-tailed t-test). Simulated keratometry readings were similar in both groups when the operated eyes were excluded. Cornea guttata and irregularity of corneal topography patterns were also noted in the study group.

Conclusions: Corneal thickness, corneal curvature and corneal hydration were within normal limits and thus were not the cause for the low applanation tonometry reading in DM1. The presence of cornea guttata and irregularity of corneal topography patterns in DM1 warrants further investigation. 

Background: Multi-detector computed tomography has advanced enormously and now enables non-invasive evaluation of coronary arteries as well as cardiac anatomy, function and perfusion. However, the role of cardiac MDCT[1] is not yet determined in the medical community and, consequently, many clinically unnecessary scans are performed solely on a self-referral basis.

Objectives: To prospectively evaluate the role of a cardiologist consultation and recommendation prior to the scan, and the influence on the diagnostic yield of cardiac MDCT.

Methods: In our center, a CT service was initiated, but with the prerequisite approval of a cardiologist before performance of the CT. Each individual who wanted and was willing to pay for a cardiac CT was interviewed by an experienced cardiologist who determined whether cardiac MDCT was the most appropriate next test in the cardiovascular evaluation. Subjects were classified into three groups: a) those with a normal or no prior stress test, no typical symptoms and no significant risk factors of coronary artery disease were recommended to perform a stress test or to remain under close clinical follow-up without MDCT; b) those with an equivocal stress test, atypical symptoms and/or significant risk factors were allowed to have cardiac MDCT; and c) those with positive stress test or clinically highly suspected CAD[2] were advised to go directly to invasive coronary angiography. CT findings were categorized as normal CAD (normal calcium score and no narrowings), < 50% and > 50% CAD.

Results: A total of 254 people were interviewed, and in only 39 cases did the cardiologist approve the CT. However, 61 of the 215, despite our recommendation not to undergo CT, decided to have the scan. Assessment of the 100 cases that underwent MDCT showed a statistically significant better discrimination of significant CAD, according to the cardiologist’s recommendation: MDCT not recommended in 3/54 (6%) vs. MDCT recommended in 12/39 (31%) vs. recommended invasive coronary angiography in 4/7 (57%) (P < 0.001).

Conclusions: Detection of coronary calcification, as well as MDCT angiography can provide clinically useful information if applied to suitable patient groups. It is foreseeable that MDCT angiography will become part of the routine workup in some subsets of patients with suspected CAD. Selection of patients undergoing MDCT scans by a cardiologist improves the ability of the test to stratify patients, preventing unnecessary scans in both high and low risk patients

Background: Computed tomographic colonography, also known as virtual colonoscopy, is a rapid, non-invasive imaging technique for the detection of colorectal masses and polyps that is becoming increasingly popular.

Objectives: To evaluate the availability, technique, standards of performance and indications for CT colonography in Israel.

Methods: A questionnaire on CT colonography was sent to all radiology departments and private institutions that perform CTC[1] in Israel. We evaluated multiple technical parameters regarding the performance and interpretation of CTC as well as radiologists' training and experience.

Results: Fourteen institutions – 7 hospitals and 7 private clinics – participated in the study. Most of the small radiology departments and nearly all of the more peripheral radiology departments do not perform CTC studies. Since 2000 and until March 2007, a total of 15,165 CTC studies were performed but only 14% (2123 examinations) were performed at public hospitals and 86% (13,042 exams) at private clinics. CTC was performed after an incomplete colonoscopy or for various contraindications to endoscopic colonoscopy in up to a third of cases. In the various institutions patients were self-referred in 20–60% of cases, more commonly in private clinics. All CTC examinations were performed on 16–64 slice CT scanners and only a small minority was performed on 4-slice scanners in 2001. All but one center used low radiation protocols. Nearly all facilities used a 2 day bowel-cleansing protocol. All except one facility did not use stool tagging or computer-aided diagnosis. All facilities inflated the colon with room air manually. All institutions used state-of-the-art workstations, 3D and endoluminal navigation, and coronal multi-planar reconstructions routinely. There are 18 radiologists in the country who perform and interpret CTC studies; half of them trained abroad. Ten of the radiologists (56%) have read more than 500 CTC studies.

Conclusions: In Israel, CTC examinations are performed by well-trained and highly experienced radiologists using the latest CT scanners and workstations and adhering to acceptable CTC guidelines.  

Background: Since surgical repair of tetralogy of Fallot was introduced, follow-up studies have shown that the majority of patients lead actives lives and have no subjective exercise limitation.

Objectives: To examine lung function, cardiopulmonary functional capacity and echo-Doppler assessment of pulmonary pressure in adult patients 20 years after repair of TOF.

Methods: Unselected consecutive patients performed full lung function testing, progressive cardiopulmonary exercise, and echo-Doppler assessments of pulmonary pressure.

Results: Fifty consecutive patients (33 men, 17 women) aged 29 ± 11 years who underwent surgical repair of TOF at age 10.1 ± 10.9 years were enrolled in this study. Patients after TOF showed no restriction (forced expiratory vital capacity 80%, total lung capacity 91%) and had normal oxygen saturation (97%) and 6 minute walking distance (600 meters). Echocardiography showed normal pulmonary pressure and left ventricular ejection function (62%). Cardiopulmonary exercise testing showed mild limitation of exercise capacity with oxygen uptake at maximal effort of 75–78% predicted.

Conclusions: After corrections of TOF the study patients had normal lung function and pulmonary arterial pressure but mild limitation in their exercise capacity.
 

Background: Spontaneous pneumomediastinum is a rare entity that usually occurs in young males without any apparent precipitating factor. Several case series have been published focusing on clinical features, workup and prognosis. Due to the rarity of this entity, there is no consensus on the most appropriate treatment.

Objectives: To describe the clinical characteristics and course of patients with spontaneous pneumomediastinum in our institution.

Methods: This is a retrospective descriptive study based on a review of the charts of all patients discharged from our hospital with a diagnosis of SPM during the period 2000 to 2007. Thirteen patients were identified and information on their clinical presentation, course, hospital stay, investigations and outcome was gathered.

Results: In 70% of patients the presenting complaint of SPM was pleuritic chest pain, while 30% of patients developed SPM in the course of another respiratory illness. Subcutaneous emphysema was the most common clinical finding (46%). Chest X-ray was diagnostic in 12 of 13 patients, and additional tests such as esophagogram and echocardiogram were unrevealing. Leukocytosis and electrocardiographic changes in inferior leads were seen in 30% of patients. Mean hospital stay was 48 hours, treatment was supportive, and symptomatic improvement was usually noted within 24 hours. No recurrences occurred.

Conclusions: SPM is a rare entity that should be considered in patients with pleuritic chest pain. Treatment is supportive, and if no clues for esophageal rupture are present investigations other than chest X-ray are probably not warranted. It is safe to discharge the patient within 24 hours provided that symptomatic improvement is achieved.
 

Background: Secondhand smoke poses a serious health hazard. In Israel The recent passage of a law designed to protect people from secondhand smoke in public places was greeted with controversy. The debate is taking place without data on actual levels of pollution for secondhand smoke in public places.

Objectives: To estimate levels of small respirable suspended particles, atmospheric markers of secondhand smoke, in Israeli bars, pubs and cafes, to compare them with levels in other countries, and to analyze RSP[1] determinants.

Methods: This study was conducted in bars, pubs and cafes in Jerusalem and Tel Aviv prior to passage of the enforcement bill. Venues were randomly sampled from lists available in the local mass media.

Results: The average level of RSPs across all venues, 283 μg/m³, was nearly identical to levels in countries without enforced smoking bans. Bars and pubs had higher values than cafes (P = 0.0101). The effect of smoker concentration was borderline significant (P = 0.0540), with RSP levels increasing as smoker concentration increased. The effect of venue height was also borderline significant (P = 0.0642), with RSP levels decreasing as venue height increased.

Conclusions: Levels of indoor air pollution from secondhand smoke in Israeli bars, pubs and cafes prior to the recent passage of the enforcement bill were similar to levels in countries without enforced smoking bans, and roughly 10 times as high as countries with enforced smoking bans. Whether the new law will successfully promote clean air in Israeli bars, pubs, cafes, and other indoor places is yet to be seen.

Background: Negative-pressure therapy for the closure of wounds, a technique to accelerate secondary wound healing, is clinically available as the V.A.C.™ system (KCI Inc, San Antonio, TX, USA). Budgetary considerations in our institution precluded widespread use of the expensive V.A.C.™ system in routine cases.

Objectives: To develop a less expensive comparably effective dressing, based on the same principles.

Methods: We used our “homemade” system to treat 15 patients with appropriate complex wounds. Their hospital charts were reviewed and assessed retrospectively. Cost analysis was performed comparing our dressing with the V.A.C.™ system.

Results: Our homemade negative-pressure wound treatment system obtained results similar to what one could expect with the V.A.C.™ System in all parameters. Complications encountered were few and minor. Cost per day using our negative-pressure system for a 10 cm² wound is about US$1, as compared to US$22, utilizing the V.A.C.™ System.

Conclusions: Our homemade negative-pressure system proved to be a good cost-effective treatment for wound closure in hospitalized patients, yielding results comparable to those of the more expensive V.A.C.™ system.
 

Raynaud's phenomenon, fatigue and pain (myalgia and arthralgia) are important presenting symptoms of pediatric-onset mixed connective tissue disease. The difficulty is that many adolescent girls complain of pain along with fatigue without evidence for serious disease. However, in patients with Raynaud's phenomenon one should search for evidence of connective tissue diseases. Capillaroscopy could be helpful since capillary changes of the SD-type significantly correlate with future development of scleroderma spectrum disorders. Symptoms of MCTD[1] change in most patients during the disease course: in general the inflammatory features that are also seen in systemic lupus erythematosus and juvenile dermatomyositis have the tendency to disappear over years, but Raynaud's phenomenon is persistent and scleroderma symptoms become progressively prominent. Long-lasting remission occurs only in a minority of patients, while the majority has mild disease activity. Mortality in children with MCTD is lower than in adults. Since a change of symptoms is in the nature of the disease a thorough and frequent evaluation of children with (probable) MCTD is important to detect organ involvement which, if present, should be treated at an early (pre-symptomatic) stage. We present a diagnostic workup scheme for children and adolescents with propable MCTD.

The question of who discovered insulin is controversial. One of the scientists working on pancreas extracts was Nicolae Paulescu, the so-called Forgotten Man. In addition to his scientific research he was also active in politics. He was the father of the virulent antisemitic fascist movement “Garda de Fer” in Romania; he raved against the “Jewish Peril,” claimed in his writings that the Jews are a genetically degenerate people trying to cheat and poison the Rumanian people by alcoholism, and more. His name came up in 2003 when Romanian diabetologists initiated a move to honor him. But voices rose in protest, claiming that persons who incite hatred, support persecution and genocide and distort science as their political tools cannot be accepted or rewarded. The protesters won.

Background: A polymeric diet rich in transforming growth factor-beta 2 used as a single nutrient has been shown to induce remission in 79% of children with Crohn's disease.

Objectives: To summarize the experience of several pediatric gastroenterology units in Israel using a TGFβ2[1]-enriched polymeric diet (Modulen IBD) supplementation in children and adolescents with Crohn's disease.

Methods: In a retrospective study we reviewed the charts of 28 children with Crohn's disease (10 girls, 18 boys) who received, in addition to conventional treatment, Modulen IBD™ as a supplement to their regular nutrition. These children were compared with 18 children supplemented with standard polymeric formula (Ensure Plus®) and 18 children without formula supplementation. We recorded clinical manifestations, growth, and the Pediatric Crohn's Disease Activity Index before and after initiation of the polymeric diet.

Results: The Modulen-treated children showed a significant decrease in PCDAI[2] from 34.3 to 15.7 (P < 0.0001). A significant decrease in PCDAI was recorded also in the Ensure Plus group, from 35 to 22 (P = 0.02) but not in the non-supplemented group. Significant improvements in body mass index (P = 0.01) and erythrocyte sedimentation rate (P = 0.03) were recorded at follow-up (median 3.4 months) only in the Modulen IBD group.

Conclusions: In this cohort of children with Crohn's disease, supplementation of the diet with Modulen IBD as well as supplementation with Ensure Plus was associated with a decrease in PCDAI. The children supplemented with Modulen IBD also showed improvement in BMI[3], suggesting an additional advantage of nutritional therapy in children with this disease.

Background: It has been suggested that increased calcium intake plays a role in preventing obesity and promoting weight loss.

Objectives: To assess the association between calcium intake, body mass index and waist circumference in Israel.

Methods: MABAT is a cross-sectional survey based on a random sample of 3246 Israelis aged 25 to 64. Of the 3246 survey participants, height and weight measurements were recorded for 2782 (1371 men and 1411 women). These were divided into three groups according to their BMI[1] (group A ≤ 24.9, group B 25–29.9, and group C ≥ 30) and given a 24 hour food consumption recall questionnaire. Waist circumference was measured in 2601 participants (1760 men and 841 women) and was considered to be excessive if ≥102 cm for men or 88 cm for women.

Results: The mean calcium intake was 511.5 ± 301.8 mg for group A, 499.4 ± 283.7 mg for group B, and 464.7 ± 280.1 mg for group C (group A significantly differed from group C, P < 0.002). The mean daily milk consumption in group A was higher than in groups B and C (103.4 ± 147.5, 85.7 ± 122.25, and 84.5 ± 135.1 g, respectively; P < 0.01). There was no correlation between daily dietary calcium intake and waist circumference for men but women with a waist circumference below 88 cm consumed significantly more dietary calcium than those with a waist circumference ≥ 88 cm (P < 0.03).

Conclusions: The study confirms the inverse relationship between daily dietary calcium intake and obesity. This linkage relates to the intake of milk, but not to other dairy products.

Background: Long-term therapy with botulinum toxin is sometimes associated with therapy failure following repeated injections of the neurotoxin, presumably due to specific antibody production. Primary therapy failure with botulinum toxin is less common and poorly understood.

Objectives: To examine the effectiveness of the botulinum neurotoxin Dysport® in patients with blepharospasm and hemifacial spasm after primary or secondary failure with Botox® treatments. .

Methods: In this case series study, eight patients with blepharospasm and hemifacial spasm who experienced primary or secondary therapy failure with Botox were treated with Dysport. In order to render an equivalent Dysport dose, a conversion ratio of 1:3 to 1:4 Botox /Dysport was used.

Results: Two patients, one with blepharospasm and the other with hemifacial spasm, who showed primary therapeutic failure with Botox showed good response to Dysport treatments. One patient with tardive blepharospasm did not respond to either drug. Two patients with blepharospasm and three patients with hemifacial spasm who experienced Botox secondary therapy failure responded well to Dysport treatments.

Conclusions: Botox and Dysport are both serotype A botulinum toxins but carry different characteristics of biological activity. These differences possibly account for the favorable therapeutic response to Dysport in patients with hemifacial spasm or blepharospasm following failure with Botox treatments.