Israel Medical Association Journal

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Methods: The study summarizes previously published data combined with unpublished data of patients with scalp dysesthesia.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. 

Background: Janus kinase-2 (JAK2) is mutated in a high proportion of patients with polycythemia vera and in a smaller number with essential thrombocythemia and primary myelofibrosis. Mutated JAK2 is an important diagnostic marker for myeloproliferative neoplasm (MPN) and may also play a major role in the pathogenesis of MPN.

Objectives: To evaluate the prevalence of mutated JAK2 (JAK2-V617F) among patients with major intraabdominal vein thrombosis who had normal blood counts at diagnosis of the initial event.

Methods: The medical records of patients who presented with a major intraabdominal venous thrombosis and normal peripheral blood counts were obtained. JAK2-V617F mutation status was determined by real-time polymerase chain reaction.

Results: Twenty-two patients were available for this analysis and 9 (41%) were found to have JAK2-V617F. Patients with positive JAK2-V617F were younger and had more frequent clinical splenomegaly than those with wild-type JAK2.

Conclusions: A high proportion of patients presenting with “idiopathic” major intraabdominal vein thrombosis and normal blood counts carry JAK2-V617F. We recommend searching for the mutation in this clinical setting to detect patients with occult MPN.

Objectives: To examine the difference in motor function and behavioral symptoms between patients with severe dementia who wander and those who do not.

Methods: We conducted a retrospective study reviewing the medical records of 72 patients with severe dementia, all residents of a dementia special care unit. Motor and behavioral aspects were compared between "wanderers" and “non-wanderers.”

Results: No difference was found in motor performance including the occurrence of falls between the wanderers and non-wanderers. A significant difference was found in aggressiveness and sleep disturbances, which were more frequent among the wanderers. There was no preference to wandering at a certain period of the day among the patients with sleep disturbances who wander.

Conclusions: In a protected environment wandering is not a risk factor for falls. Sleep disturbances and wandering co-occur, but there is no circumstantial association between the two symptoms.

Objectives: To retrospectively analyze the results of a screening program for C-CMV performed at the Sheba Medical Center, Tel Hashomer, during a 1 year period, using real-time polymerase chain reaction (rt-PCR) from umbilical cord blood.

Methods: CMV DNA was detected by rt-PCR performed on infants’ cord blood. C-CMV was confirmed by urine culture (Shell-vial). All confirmed cases were further investigated for C-CMV manifestations by head ultrasound, complete blood count, liver enzyme measurement, ophthalmology examination and hearing investigation.

Results: During the period 1 June 2009 to 31 May 2010, 11,022 infants were born at the Sheba Medical Center, of whom 8105 (74%) were screened. Twenty-three (0.28%) were positive for CMV and 22 of them (96%) were confirmed by urine culture. Two additional infants, who had not been screened, were detected after clinical suspicion. All 24 infants were further investigated, and 3 (12.5%) had central nervous system involvement (including hearing impairment) and were offered intravenous ganciclovir for 6 weeks. Eighteen (82%) infants would not otherwise have been diagnosed.

Conclusions: The relatively low incidence of C-CMV detected in our screening program probably reflects the low sensitivity of cord blood screening. Nevertheless, this screening program reliably detected a non-negligible number of infants who could benefit from early detection. Other screening methods using saliva should be investigated further.

Objectives: To investigate whether young patients, i.e., under age 50, complain of symptoms for longer than older patients until the diagnosis of colon cancer is established.

Methods: In this retrospective cohort study, patients were divided into two groups: < 50 years old (group 1) and ≥ 50 (group 2). All had undergone surgery for left or right colon cancer during the 1 year period January 2000 through December 2009 at one medical center. Rectal and sigmoid cancers were excluded. Data collected included age, gender, quantity and quality of complaints, duration of complaints, in-hospital versus community diagnosis, pathological staging, the side of colon involved, and overall mortality. The main aim was the quality and duration of complaints. Secondary outcomes were the pathological stage at presentation and the mortality rate.

Results: The study group comprised 236 patients: 31 (13.1%) were < 50 years old and 205 (86.9%) were ≥ 50 years. No significant difference was found in the quantity and quality of complaints between the two groups. Patients in group 1 (< 50 years) complained for a longer period, 5.3 vs. 2.4 months (P = 0.002). More younger patients were diagnosed with stage IV disease (38.7% vs. 21.5%, P = 0.035) and fewer had stage I disease (3.2% vs. 15.6%, P = 0.06); the mortality rates were similar (41.9% vs. 39%). Applying a stepwise logistic regression model, the duration of complaints was found to be an independent predictor of mortality (P = 0.03, OR 1.6, 95% CI 1–3.6), independently of age (P = 0.003) and stage (P < 0.001).

Conclusions: Younger patients are more often diagnosed with colon cancer later, at a more advanced stage. Alertness to patients’ complaints, together with evaluation regardless of age but according to symptoms and clinical presentation are crucial. Large-scale population-based studies are needed to confirm this trend. 

Objectives: To describe the exposures experienced by emergency responders to smoke, fire retardants and stress; the utilization of protective equipment; and the frequency of corresponding symptoms during and following the Carmel Forest fire.

Methods: A cohort of 204 firefighters and 68 police who took part in rescue and fire-abating activities during the Carmel Forest fire were recruited from a representative sample of participating stations throughout the country and interviewed regarding their activities during the fire and their coinciding symptoms. Unpaired two-sample t-test compared mean exposures and symptom frequency for firefighters and police. Chi-square estimates of OR and 95% CI are provided for odds of reporting symptoms, incurring injury or being hospitalized for various risk factors.

Results: Of the study participants, 87% reported having at least one symptom during rescue work at the Carmel Forest fire, with eye irritation (77%) and fatigue (71%) being the most common. Occupational stress was extremely high during the fire; the average length of time working without rest was 18.4 hours among firefighters.

Conclusions: Firefighters and police were exposed to smoke and occupational stress for prolonged periods during the fire. Further research is needed on the residual health effects from exposure to forest fires among emergency responders, and to identify areas for improvement in health preparedness.  

Background: The management of sexual assault victims comprises complex medical, psychological, social and judicial care that was previously provided by various disciplines at several distant locations. This novel concept is the delivery of comprehensive care to victims of sexual assault at one location 24 hours a day.

Objectives: To describe the characteristics of sexual assault victims, their assailants, the assault and the treatment, and provide descriptive data on the evidentiary examination.

Methods: We performed a retrospective analysis of the charts of all sexual assault victims presenting to the Regional Israeli Center for Care of Sexual Assault Victims between October 2000 and July 2010. The center, the first in Israel, provides comprehensive care to victims of sexual assault in one location 24 hours a day using a multidisciplinary approach.

Results: The study group comprised 1992 subjects; 91.5% of the victims were females and 8.5% were males, and the age ranged from 1 to 88 years (mean age 22.3 years). Of the 1992 victims, 1635 were single (82.2%), 195 were divorced (9.8%), 141 were married (7.1%), 18 were widowed (0.9%) and 3 were unspecified. The assailant was a stranger in 794 (39.8%) of the cases, someone familiar to the victim in 786 cases (39.0%), a partner in 127 cases (6.4%), a family member in 117 cases (5.9%), someone met via the internet in 53 cases (2.7%), an authority figure in 39 cases (2.0%), and unspecified in 76 (3.9%). In the majority of cases the attack occurred either in the evening or at night (71.7%).

Conclusions: We identified several risk factors for sexual assault that can be used in prevention programs. The sexual assault victim in our study tended to be a young, single woman who was attacked by a familiar assailant in the evening or at night. Our center provides comprehensive care to victims 24 hours a day at one location and includes a team of forensic, psychological, physical and legal specialists.
 

Background: Recently, cognitive assessments of patients with Fabry disease highlighted neurocognitive impairment using test batteries that are time and labor intensive.

Objectives: To introduce a user-friendly self-administered tool for cognitive testing in patients with Fabry disease.

Methods: We used a computerized system requiring about 1 hour for patient follow-up. All patients with enzymatic and/or molecular diagnosis of Fabry disease seen in our clinic underwent assessment with the Fabry-specific Mainz Severity Score Index (MSSI) with subscores (neurological, renal, cardiac, and general) and a Mindstreams neurocognitive battery for mild impairment, evaluating memory, executive function, attention, information processing, visual spatial processing, verbal function, and motor skills. A Global Cognitive Score (GCS) was also computed.

Results: Ten patients (3 males, 7 females) were tested (mean age 41.5, range 25–56 years). Males were younger, had moderate nephropathy and no cerebrovascular accident (CVA); their Mindstreams GCS was 85.6–107 points. Three females had mild-moderate (8,10,15 points) neurological MSSI subscores (two CVA); all females had Mindstreams GCS of 59–107.7 points. Below-average performance was prevalent, particularly in information processing and motor skills consistent with mild impairment. Average GCS in females (90.3 points) was lower than in males (98.2 points). For individual patients, performance was poorest in information processing (n=4), attention (n=2), motor skills (n=2), verbal function (n=1), and visual spatial processing (n=1).

Conclusions: MindStreams may simplify cognitive assessment monitoring in Fabry disease.