Israel Medical Association Journal

Background: Lung disease in patients with famHiat dys-autonomia is caused mainly by recurrent aspiration of gastric contents, food and liquids swallowed incorrectly.

Objective: To describe chest computed tomography findings in patients with familial dyautonomia.

Methods: A retrospective analysis of chest CT findings was performed for 34 FD patients (15 females, 19 males) with a mean age of 18± 12.8 years.

Results: The CT revealed bronchial wall thickening (in 94% of the patients), atelectasis (in 73%), ground glass opacities (in 53%), focal hyperinflation (in 44%), fibrosis (in 29%) and bronchiectasis (in 26%). The extrapulmonary abnormalities were scoliosis (79%) and esophageal dilatation (35%). Silent fractures were noted in two vertebral bodies and one rib.

Conclusions: Pulmonary changes were consistent with chronic inflammation in the bronchi and interstitial tissues. Ground  glass opacities and fibrosis support the theory that these changes could be due to gastric aspiration. Bronchiectasis is less frequent. Esophageal dilatation with fluid overflow adds to aspiration. Fractures can be asymptomaflc and are often missed.
 

Background: Anti-ribosomal-P antibodies have been associated with central nervous manifestations of systemic lupus erythematosus. However, inconsistencies in their prevalence and clinical correlations have become an obstacle to their use as a diagnostic marker of the disease. This lack of consistency might stem from several factors, such as the lag period between clinical manifestations and the time blood was drawn, or the different methods used for antibodies detection.

Objectives: To evaluate three different enzyme-linked immunosorbent assay tests for the detection of anti-Rib-P Abs[1] in patients with SLE[2] and normal controls.

Methods: Sera from 50 SLE outpatients and 50 healthy subjects were tested with three ELISA[3] kits: Kit-1, which uses synthetic peptide comprising the 22 C-terminal amino-acids; Kit-2, which uses native human ribosomal proteins (P0, P1, P2); and Kit-3, which is coated with affinity-purified human ribosomal proteins. ELISA studies were performed according to the manufacturers' instructions.

Results: The prevalence of anti-Rib-P Abs in SLE patients and controls was 30% vs. 0%, 17% vs. 21%, and 30% vs. 14% in kits 1-3 respectively. Anti-Rib-P Abs detected by Kit-1 correlated with the SLEDAI score (SLE Disease Activity Index). No correlation between prior CNS[4] manifestations and anti-Rib-P Abs was observed.

Conclusions: A significant difference was documented between the ELISA kits used for the detection of anti-Rib-P Abs. A correlation was found between these antibodies (evaluated by Kit-1) and concurrent SLEDAI scores, in contrast to the lack of correlation with previous CNS manifestations. This supports the notion of "active serology" that is evaluated at the same time manifestations are present, as well as the need for standardization of laboratory assays in the future that enable a better assessment of anti-Rib-P Abs presence and clinical correlation. 

Background: The rate of cesarean section is increasing and recently exceeded 30% of all deliveries in the United States. Birth injuries during CS[1] are relatively rare. Femur fractures have a very low incidence during both vaginal delivery and CS.

Objectives: To assess our 10 year experience (2008–1997) in managing fractured femur during CS, including a typical case.

Methods: We reviewed the prevalence of femur fractures in two tertiary, academic, level one trauma center hospitals in Israel (Hadassah in Jerusalem and Soroka in Beer Sheva).

Results: During the study period 221,939 deliveries occurred in both hospitals. Of these, 17.6% were cesarean sections (33,990 CS). Of the total number of deliveries, the incidence of femur fracture was 0.082 per 1000 deliveries (17 fractures), and the incidence of femur fracture during CS was 0.308 per 1000 CS (12 fractures).

Conclusions: Cesarean section increases the risk of femur fractures (P < 0.001) with an odds ratio of 11.26 (confidence interval 3.97–31.97).

Background: The causative role of diffuse macular edema in various etiologies is often undetermined.

Objectives: To describe an association between extrafoveal vitreous traction and non-diabetic diffuse macular edema secondary to various ocular entities.

Methods: In a retrospective study of eyes with non-diabetic diffuse macular edema, charts and optical coherence tomography scans demonstrating extrafoveal vitreous traction were analyzed. Excluded were diabetic patients and eyes that had vitreofoveal traction. A control group (n=12) allowed for mapping of normal macular thickness.

Results: Five eyes with macular edema were associated with extrafoveal traction, each secondary to and representing a different etiology. The causes were penetrating injury, cataract extraction, branch retinal vein occlusion, central retinal vein occlusion, and idiopathic. Vitreous traction was detected either at the papillomacular bundle (n=3), superonasally to the fovea (n=1), and at the optic nerve head (n=1). The associated retinal edema (all eyes) and serous retinal detachment (four eyes) at the traction sites were in continuum with the foveal edema in each eye, manifesting as diffuse macular edema. Of the two modalities, the OCT[1]-Line group program and the OCT-Automatic central program, only the former enabled detection of extrafoveal traction in each.

Conclusions: Diffuse macular edema secondary to various ocular diseases may be associated with extrafoveal vitreous traction. The OCT-Automatic central program may omit some of these extrafoveal traction sites. Further studies are required to validate these findings and to assess whether early vitrectomy may improve visual prognosis in these eyes.

Head and neck cancer is the sixth most common cancer worldwide. HNCs[1] can originate in the skin or soft tissue, in the upper aerodigestive tracts (oral cavity, oropharynx, hypopharynx, larynx, nasopharynx, paranasal sinuses, salivary glands), or in the thyroid. In each of these sites, tumors vary not only by the primary site but also by pathophysiology, biological behavior and sensitivity to radiotherapy or chemotherapy. Management should be planned according to the tumor's characteristics, patient factors and expertise of the medical team. The main goals of therapy are ablation of the cancer while minimizing morbidity and preserving function and cosmesis. A multidisciplinary team is needed to achieve these goals. Early-stage HNC (stage I and II) should be managed with a single modality, and advanced tumors (stage III and IV) with multimodality therapy. Treatment should be directed to the primary tumor and the area of its lymphatic drainage – the neck lymph nodes. Evidence of metastases in the neck necessitates comprehensive clearance of regional lymphatic basins. However, even if there is no evidence of lymph nodes metastases, when the risk for positive neck lymph nodes exceeds 15–20% elective neck dissection is indicated. Advances in minimally invasive techniques now enable reliable microscopic and endoscopic procedures that mimic the open approaches. Development of contemporary surgical techniques and reconstructive means will help improve the quality of life of patients and prolong survival.

There are isolated cases of physicians who murdered their patients. However, never had a single physician personally supervised the mass murder of hundreds of thousands of individuals, until Dr. Irmfried Eberl. Commander of the Nazi death camp Treblinka, he killed both the ill and those he considered "a disease to his nation." At age 32 Dr. Eberl established Treblinka, where he was responsible for the killing of approximately 280,000 individuals within a few weeks. The position of camp commandant was earned following his success as head of two psychiatric hospitals in Germany where he coordinated the murder of thousands of mentally ill Jews and non-Jews within the context of the euthanasia program. However, few in medicine have heard of him or the harm he caused to the ethical practice of the profession and to human rights.

Background: Fracture of the scaphoid is the most common fracture of a carpal bone. Nevertheless, the diagnosis of SF[1] might be challenging. Plain X-rays that fail to demonstrate a fracture line while clinical findings suggest the existence of such a fracture is not uncommon. Currently there is no consensus in the literature as to how a clinically suspected SF should be diagnosed, immobilized and treated.

Objectives: To assess the current status of diagnosis and treatment of clinically suspected scaphoid fractures in Israeli emergency departments

Methods: We conducted a telephonic survey among orthopedic surgeons working in Israeli EDs[2] as to their approach to the diagnosis and treatment of occult SF.

Results: A total of 42 orthopedic surgeons in 6 hospital EDs participated in the survey. They reported performing a mean of 2.45 ± 0.85 clinical tests, with tenderness over the snuffbox area being the sign most commonly used.  A mean of 4.38 ± 0.76 X-ray views were ordered for patients with a clinically suspected SF. The most common combination included posterior-anterior, lateral, semipronated and semisupinated oblique views. All participating surgeons reported immobilizing the wrists of patients with occult fractures in a thumb spica cast based on their clinical findings. Upon discharge from the ED patients were advised to have another diagnostic examination as follows: 29 (69%) repeated X-rays series, 18 (43%) were referred to bone scintigraphy and 2 (5%) to computed tomography; none were referred to magnetic resonance imaging.

Conclusions: No consensus was found among Israeli orthopedic surgeons working in EDs regarding the right algorithm for assessment of clinically suspected SF. There is a need for better guidelines to uniformly dictate the order and set of tests to be used in the assessment of occult fractures.

Background: The forced oscillation technique is a non-invasive and effort-independent technique and is well suited for lung function measurement in young children. FOT[1] employs small-amplitude pressure oscillations superimposed on normal breathing. Therefore, it has the advantage over conventional lung function techniques in that it does not require patient cooperation for conducting respiratory maneuvers.

Objectives: To test the feasibility of the FOT test in preschool children and to compare the results to the commonly used spirometry before and after the administration of bronchodilator therapy.

Methods: Forty-six children (median age 4.9 years, range 1.8–18.3) attending the Pulmonary Clinic at Schneider Children's Medical Center tried to perform FOT and routine spirometry. Results were retrospectively analyzed. 

Results: Of the 46 children 40 succeeded in performing FOT and only 29 succeeded in performing simple spirometry. All but one of the 32 children aged 4 years and above (97%) could perform both tests. Nine of 14 children (64%) aged 4 and less could perform the FOT but only 3 (21%) could perform spirometry. Baseline values of respiratory resistance measured at 6 Hz (R6) negatively correlated with body length (r² = 0.68, P < 0.005). Twenty-four children performed both tests before and after bronchodilator therapy. A significant concordance was found between the measured responses to bronchodilators by FOT and spirometry (P < 0.01). Only one child had a negative response by FOT but a positive response by spirometry.

Conclusions: The FOT is a simple, non-invasive technique that does not require subject cooperation and thus can be utilized for measuring lung function in children as young as 2 years of age. Furthermore, the FOT was shown to reliably measure response to bronchodilator therapy.

Background: The TANTALUS^™ System (MetaCure Ltd.) is a minimally invasive implantable device for the treatment of type 2 diabetes. The system detects food intake by sensing gastric electrical variations and applies electrical stimulation to the gut synchronized to natural gastric activity. The system is commercially available in Europe and Israel and is in clinical trials in the United States. It has been tested in 132 patients worldwide to date.

Objectives: To re-analyze previously reported data from different studies. This retrospective analysis of the type 2 diabetes subpopulation analyzed the expected benefit and characterize the significance of baseline A1c in the determination of the expected clinical outcome.

Methods: From the total cohort of 132 patients implanted with the TANTALUS device in 10 different centers in Europe and the U.S., 50 subjects (27 females, 23 males) who were obese with uncontrolled T2DM[1] on a stable regime of oral medication for 3 months prior to implant were identified. This population had similar inclusion/exclusion criteria as well as treatment protocols and were all treated for at least 24 weeks. The analysis was based on the A1c change compared to baseline.

Results: Data after 24 weeks demonstrate a reduction in A1c in 80% of the patients with average drop in A1c of 1.1 ± 0.1%. The average weight loss was 5.5 ± 0.7 kg.

Conclusions: The results suggest that the TANTALUS stimulation regime can improve glucose levels and induce moderate weight loss in obese T2DM patients.