Israel Medical Association Journal

Background: Since the identification of the MEFV gene 198 mutations have been identified, not all of which are pathologic. The screening methods used in Israel to test patients suspected of having FMF include a kit that tests for the five main mutations (M694V, V726A, M680Ic/g, M694I, E148Q), and the sequencing of MEFV exon 10 in combination with restriction analysis for detecting additional mutations.

Objectives: To determine the contribution of testing for five additional mutations – A744S, K695R, M680Ic/t, R761H and P369S – to the molecular diagnosis of patients clinically suspected of having FMF.

Methods: A total of 1637 patients were tested for FMF mutations by sequencing exon 10 and performing restriction analysis for mutations E148Q and P369S.

Results: Nearly half the patients (812, 49.6%) did not have any detectable mutations, 581 (35.5%) had one mutation, 241 (14.7%) had two mutations, of whom 122 were homozygous and 119 compound heterozygous, and 3 had three mutations. Testing for the additional five mutations enabled us to identify 46 patients who would have been missed by the molecular diagnosis kit and 22 patients who would have been found to have only one mutation. Altogether, 4.3% of the patients would not have been diagnosed correctly by using only the kit that tests for the five main mutations.

Conclusions: This study suggests that testing for the additional five mutations as well as the five main mutations in patients with a clinical presentation of FMF adds significantly to the molecular diagnosis of FMF in the Israeli population.
 

Background: Survival in T cell lymphoblastic lymphoma has improved over the past 30 years, largely due to treatment protocols derived from regimens designed for children with acute lymphoblastic leukemia.

Objectives: To assess the outcome of the NHL-BFM-95 protocol in children and adolescents hospitalized during the period 1999–2006.

Methods: We conducted a retrospective multi-institutional, non-randomized study of children and adolescents up to age 21 with T cell lymphoma admitted to pediatric departments in six hospitals in Israel, with regard to prevalence, clinical characteristics, pathological characteristics, prognostic factors, overall survival (OS) and event-free survival (EFS). All patients had a minimal follow-up of one year after diagnosis. The study was based on the NHL[1]-BFM[2]-95 protocol.

Results: At a median follow-up of 4 years (range 1–9 years), OS and EFS for all patients was 86.5% and 83.8%, respectively. OS was 86.7% and 83.3% for patients with stage III and stage IV, respectively, and EFS was 83.3% and 83.3%, respectively. EFS was 62.5% for Arab patients and 89.7% for Jewish patients (P = 0.014). Patients who did not express CD45 antigen showed superior survival (P = 0.028). Five (13.5%) patients relapsed, four of whom died of their disease. Death as a consequence of therapy toxicity was documented in one patient while on the re-induction protocol (protocol IIA).

Conclusions: Our study shows that OS and EFS for all patients was 86.5% and 83.8%, respectively.

Background: During military escalations emergency departments provide treatment both to victims of conflict-related injuries and to routine admissions. This requires special deployment by the hospitals to optimize utilization of resources.

Objectives: To evaluate “routine” visits to the ED[1] during Operation Cast Lead in Israel in 2008–2009.

Methods: We obtained data regarding routine visits to the ED at Soroka University Medical Center throughout OCL[2]. The visits one month before and after OCL and the corresponding periods one year previous served as controls.

Results: The mean number of daily visits throughout the study period (126 days) was 506 ± 80.9, which was significantly lower during OCL (443.5 ± 82) compared with the reference periods (P < 0.001). Compared to the reference periods, during OCL the relative rates were higher among Bedouins, visitors from the region closest to the Gaza Strip (< 30 km), patients transported to the ED by ambulance and patients of employment age; the rates were lower among children. No difference in the different periods was found in the rate of women patients, distance of residence from Beer Sheva, rate of patients referred to the ED by a community physician, and hour of arrival. The overall in-hospital admission rate increased during OCL, mainly in the internal medicine and the obstetric departments. There was no change in the number of in-hospital births during OCL; however, the rate of preterm labors (32–36 weeks) decreased by 41% (P = 0.013).

Conclusions: Throughout OCL the number of routine ED visits decreased significantly compared to the control periods. This finding could help to optimize the utilization of hospital resources during similar periods.

Background: Inserting a transjugular intrahepatic portosystemic shunt by means of interventional radiology has become the procedure of choice for decompression of portal hypertension. The indications and criteria for patient selection have been expanded and refined accordingly.

Objectives: To review our experience with TIPS[1] and analyze the results with emphasis on patient selection and indication (conventional vs. atypical).

Methods: In this retrospective analysis in a single center all cases were managed by a multidisciplinary team (comprising liver surgery and transplantation, hepatology, imaging, interventional radiology and intensive care).

Results: Between August 2003 and December 2009, 34 patients (mean age 51, range 27–76 years) were treated with TIPS. The cause of portal hypertension was cirrhosis (23 cases), hypercoagulabilty complicated by Budd-Chiari syndrome (n=6), and acute portal vein thrombosis (n=5). Clinical indications for TIPS included treatment or secondary prevention of variceal bleeding (10 cases), refractory ascites (n=18), mesenteric ischemia due to acute portal vein thrombosis (n=5), and acute liver failure (n=1). TIPS was urgent in 18 cases (53%) and elective in 16. Three deaths occurred following urgent TIPS. The overall related complication rate was 32%: transient encephalopathy (6 cases), ischemic hepatitis (n=2), acute renal failure (n=2) and bleeding (n=1). Long-term results of TIPS were defined as good in 25 cases (73%), fair in 4 (12%) and failure in 5 (15%). In three of five patients with mesenteric ischemia following acute portal vein thrombosis, surgery was obviated. Revision of TIPS due to stenosis or thrombosis was needed in 7 cases (20%).

Conclusions: TIPS is safe and effective. While its benefit for patients with portal hypertension is clear, the role of TIPS in treatment of portal-mesenteric venous thrombosis needs further evaluation. Patient selection, establishing the indication and performing TIPS should be done by a multidisciplinary dedicated team.

Background: The growing numbers of H1N1 "swine influenza" cases should prompt national health systems to achieve dual preparedness: preparedness of clinicians to recognize and treat cases of human H1N1 flu, and national preparedness for an influenza pandemic. This is similar to recent contingency planning for an avian flu pandemic.

Objectives: To evaluate hospital personnel's knowledge on avian flu (zoonotic, sporadic, pandemic), comparing among nurses, residents and faculty, and between those who attended lectures or other educational modalities targeted at avian flu and those who did not.

Methods: A 14 item multiple choice questionnaire was designed to test crucial points regarding preparedness for human avian flu. The directors of 26 general hospitals were instructed by the Ministry of Health to improve knowledge of and preparedness for different avian flu scenarios, and to expect an official inspection. As part of this inspection, we distributed the questionnaires to nurses, residents and senior physicians.

Results: Altogether, 589 questionnaires were collected from the 26 hospitals. Examinees who participated in training modules (course, lecture or any training provided by the hospital) did somewhat better (scoring 78 points out of 100) than those who did not attend the training (70 points) (P < 0.05). Differences in nurses’ knowledge were even more striking: 66 points for the non-attendants compared to 79 for nurses who attended the lecture (P < 0.05).  Residents had significantly lower scores compared to nurses or senior physicians: 70 compared to 77 and 78 respectively (P < 0.05).

Background: Continuous use of combined oral contraceptives is currently attracting growing interest as a means of improving menstrual related symptoms and reducing the number of bleeding days.

Objectives: To evaluate bleeding patterns, menstrual symptoms and quality of life with an extended 84/7 oral contraceptive regimen versus 21/7 cycles.

Methods: In two consecutive run-in cycles, 30 µg ethinyl estradiol and 3 mg drospirenone tablets taken on days 1–21 were followed by a tablet-free period from days 22 to 28 of each cycle and then by two 84 day cycles of pill use with a 7 day tablet-free interval. The primary outcome was the total number of bleeding/spotting days. Secondary outcomes were severity of daily symptoms, general well-being determined by the PGWBI questionnaire, and overall treatment satisfaction.

Results: Of the 137 women invited to participate in the study 109 (aged 18–40 years) were enrolled. The number of bleeding days decreased by about one-third from a calculated 31.8 days of bleeding under a cyclic 21/7 regimen to an expected total of 21.8 days for the extended 84/7 regimen. The incidence of menorrhagia, intermenstrual bleeding, dysmenorrhea, abdominal bloating, breast tenderness, depressive moods and irritability – when compared at enrollment and at the end of the second extended study period – was significantly lower (P < 0.005) among women on the continuous pill regimen. The median (range) global PGWBI scores were not substantially different before and after the extended use cycles: 78.2 (39.1–96.4) and 77.3 (30.9–96.4), respectively. Body weight and skin condition also remained constant. At the completion of the study: 65.5% of the women were either highly satisfied (41.4%) or satisfied (24.1%) with the extended regimen.

Conclusions: The extended 84/7 regimen was found to be satisfactory for the majority of participants and was associated with a decrease in the number of bleeding days and an improvement in menstrual symptoms compared to 21/7 cycles. 

Background: Ritual circumcision in neonates may cause a urinary tract infection within 2 weeks of the procedure.

Objectives: To evaluate the prevalence of urinary tract infection among Jewish male circumcised neonates (¡Ü 28 days old) evaluated for fever in the emergency room.

Methods: All available medical records of neonates presenting to the pediatric emergency room for evaluation of fever over a 10 year period were reviewed. Data included gender, ethnic background, age in days on presentation to the emergency room, age in days when circumcision was performed (in males ¡Ý 8 days of age), and results of urine, blood and cerebrospinal fluid cultures. Families of males older than 8 days of age who had a UTI[1] were contacted by telephone to verify the circumcision status when the infant presented to the ER[2], to ascertain whether the circumcision was performed ritually by a mohel* or by a physician, and, when not recorded in the chart, to verify the day of life on which circumcision was performed.

Results: Among neonates older than 8 days of age, 60 (24.7%) of the 243 febrile Jewish males had a UTI, as compared to 12 (8.4%) of 143 females (P < 0.0001). In 39 of 54 male neonates (72%) for whom circumcision was performed ritually on the eighth day of life, UTI occurred within 9 days of the circumcision. For females, there was no such clustering of UTI cases in the second week of life, nor during any other time period.

Conclusions: Febrile male neonates who have undergone ritual circumcision have a high prevalence of UTI and must be evaluated and treated accordingly. 

[1] UTI = urinary tract infection

[2] ER = emergency room

* Mohel is a Jewish man trained in the practice of Brit milah (circumcision).

Background: The best therapeutic alternative for patients suffering from in-stent restenosis after drug-eluting stent implantation remains to be elucidated.

Objective: To characterize the pattern, treatment and outcomes of DES[1]-related in-stent restenosis in patients treated at our institution.

Methods: We determined the incidence and major adverse clinical events in 71 consecutive patients with DES failure among 2473 patients who were treated with 2548 drug-eluting stents between 2004 and 2007. We analyzed the clinical data, procedural parameters and clinical outcomes.

Results: The type and number of stents implanted were as follows: Cypher (n=1808), Endeavor (421) and Taxus (319) of these, 53 (2.9%), 10 (2.4%), and 8 (2.5%) patients respectively presented with restenosis. The mean time to restenosis was 11.3 ± 9.9 months. Patients’ mean age was 65 ± 11 years 75% were male, and 68% had diabetes mellitus. Unstable angina was the clinical presentation in 52 (73%). At 6 months, 3 patients had developed myocardial infarction (4.2%), repeat restenosis at follow-up was diagnosed in 8 patients (11.3%), the overall major adverse clinical events rate was 18.3% (13 patients), and 2 patients died (2.8%).

Conclusions: Drug-eluting stent-related restenosis is relatively infrequent but remains a clinical challenge. It occurs more frequently in complex lesion subsets, but the overall intermediate-term prognosis is tolerable.  

[1] DES = drug-eluting stent

Background: Falls are common events among hospital inpatients and constitute a major health problem in the rehabilitation setting. Many risk factors for falls have been identified for stroke patients, such as muscle weakness, medication side effects, hypoglycemia, hypotension, etc.

Objectives: To assess the risk factors for falls among patients hospitalized for rehabilitation following acute stroke.

Methods:  In a retrospective study of 56 falls over a period of 5 years in 41 stroke patients hospitalized for rehabilitation we surveyed the nurses’ safety risk assessment of the fall. Thirty patients fell once, 9 patients twice and 2 patients four times. The data were obtained from the medical and nursing records. Safety precautions were taken by the nurses for the entire group of patients.

Results: Most of the falls occurred among male patients who had reduced muscular tone (70%), paralysis (54%) and/or hypoesthesia in the involved side of the body. Patients who suffered from hemiplegia fell more often than those with hemiparesis (Wilcoxon rank sum test, P = 0.04, one-sided). Forty-eight percent of the falls occurred during the first month after the last stroke onset, 70% during the morning or the afternoon, and 62% occurred close to the patient’s bed. In 89% of falls the patients used hypoglycemic, antihypertensive, tranquilizing or neuroleptic drugs. Communication disorders (29%), hemianopia or blindness (21%) and visuospatial agnosia (18%) were incremental risk factors for falls. Fifty percent of the falls were caused by either an intrinsic or extrinsic mechanism.

Conclusions: These data suggest that the group of stroke patients at risk for falls in a rehabilitation department can be identified by a variety of impairment and functional assessments. The information may be potentially useful for designing interventions directed at reducing fall frequency among stroke survivors. 

Background: Tubeless percutaneous nephrolithotomy is defined as PCNL[1] without postoperative nephrostomy tubes. It is reported to reduce postoperative pain, hospital stay and recovery time. To date the procedure has been reserved for selected patients.

Objectives: To assess our initial experience in extending the implementation of tubeless PCNL without preoperative patient selection.

Methods: All consecutive PCNLs performed during 2004–2008 were evaluated. Tubeless PCNL was performed when residual stones, bleeding and extravasation were excluded intraoperatively. Staghorn stones, stone burden, supracostal and multiple accesses, anatomic anomalies, solitary kidneys and operative time were not considered contraindications. We analyzed the clinical data and the choice of tubeless PCNL over time.

Results: Of 281 PCNLs performed during the study period, 200 (71%) were tubeless. The patients' average age was 53 years (range 28–82 years), the stone burden was 924 mm² (400–3150 mm²), operative time was 99 minutes (45–210 min), complication rate was 14% and immediate stone-free rate 91%. There were 81 conversions to standard PCNL (29%) due to expected second-look (n=47, 58%), impression of bleeding (n=21, 26%), suspected hydrothorax (n=7, 9%) and extravasation (n=6, 7%). The transfusion rate was 1%. The median hospital stay was 1 day (1–15 days) and recovery time 7 days (5–20 days). The rate of implementing the tubeless procedure increased steadily along time from 46% to 83% (P = 0.0001). 

Conclusions: Tubeless PCNL can be safely and effectively performed based on intraoperative decisions, without preoperative contraindications. They are easily accommodated by experienced endourologists and provide real advantages.

Background: While the burden of rotavirus infection with regard to hospitalizations has been extensively investigated, there are sparse data regarding the impact and the cost of this infection on the ambulatory part of the health system in Israel.

Objectives: To investigate the burden of rotavirus infection on the ambulatory system in Israel.

Methods: Infants younger than 3 years old examined for acute gastrointestinal symptoms in four pediatric clinics had their stool tested for rotavirus. The parents were contacted 7–10 days later and questioned about the symptoms of illness, medications given, use of diapers, consumption of formula, and any loss of parents' workdays.

Results: Rotavirus was detected in 71 of the 145 stool samples tested (49%). A total of 51 parents responded to the telephonic survey. Patients' mean age was 15.4 months. Three patients were hospitalized due to the illness. The mean duration of fever was 1.7 days. Infants with rotavirus gastroenteritis had on average 2.25 days of vomiting and 7.5 days of diarrhea. The average number of workdays lost was 2.65 days per RVGE[1] case. The cost of the average case of RVGE in Israel is 257 euros; 69.64% of this cost (179 euros) is due to parental work loss.

Conclusions: Our results indicate that the economic impact of the ambulatory cases in Israel is quite significant.