Israel Medical Association Journal

Acquired thrombotic thrombocytopenic purpura (TTP) is an uncommon disease in adults, characterized by fever, neurological manifestations, microangiopathic hemolytic anemia, thrombocytopenia, renal dysfunction, and the presence of antibodies against the enzyme ADAMTS13. Treatment with plasmapheresis has increased the survival from 10% to more than 90%. Still, there is a subset of patients with resistant TTP who fail to respond to plasmapheresis or remain dependent on this procedure. There is mounting evidence that rituximab may play an important role in remission induction of resistant/relapsing TTP; however, the extent of the remission is unknown. We present here four patients with chronic-relapsing TTP who responded favorably to rituximab. All four patients achieved prolonged remission of 23 to 82 months after the treatment.  One patient relapsed 6 years after the initial treatment with rituximab and re-entered remission following retreatment.

Background: As the lowest natural site on earth (-415 meters), the Dead Sea is unique for its high pressure and oxygen tension in addition to the unparalleled combination of natural resources. Furthermore, its balneotherapeutic resorts have been reported to be beneficial for patients with various chronic diseases.

Objectives: To evaluate the safety, quality of life (QoL), exercise capacity, heart failure, and arrhythmia parameters in patients with systolic congestive heart failure (SCHF) and implantable cardioverter defibrillator (ICD) following descent and stay at the Dead Sea.

Methods: The study group comprised patients with SCHF, New York Heart Association functional class II-III after ICD implantation. The following parameters were tested at sea level one week prior to the descent, during a 4 day stay at the Dead Sea, and one week after return: blood pressure, O₂ saturation, ejection fraction (echocardiography), weight, B-type natriuretic peptide (BNP), arrhythmias, heart rate, heart rate variability (HRV), and QoL assessed by the Minnesota Living with Heart Failure questionnaire.

Results: We evaluated 19 patients, age 65.3 ± 9.6 years, of whom 16 (84%) were males and 18 (95%) had ICD-cardiac resynchronization therapy. The trip to and from and the stay at the Dead Sea were uneventful and well tolerated. The QoL score improved by 11 points, and the 6 minute walk increased by 63 meters (P < 0.001). BNP levels slightly increased with no statistical significance. The HRV decreased (P = 0.018). There were no significant changes in blood pressure, weight, O₂ saturation or ejection fraction.

Conclusions: Descent to, ascent from, and stay at the Dead Sea resort are safe and might be beneficial in some aspects for patients with sCHF and an ICD.
 

Background: Obstructive sleep apnea syndrome (OSAS) is a sleep-related breathing disorder characterized by excessive daytime sleepiness, accidents and high medical expenses. The first line of treatment for OSAS is continuous positive airway pressure (CPAP).

Objectives: To examine attitudes and beliefs as well as physiological and sociodemographic factors affecting OSA patients' decision whether or not to purchase a CPAP device.

Methods: The study was divided into two stages; in the first, 83 subjects completed self-administered questionnaires prior to sleep examination (polysomnographic study). The questionnaires related to sleep habits, sleep disorders, questions organized around health belief model (HBM) concepts, sociodemographic information, health status and PSG[1] examination. In the second stage, 3 months later, 50 OSAS patients were interviewed by telephone, which included questions about their reasons for purchasing/not purchasing the CPAP device.

Results: Only 48% of the OSAS patients purchased the CPAP device. The significant factors positively affecting the decision included higher levels of physiological factors such as body mass index (coefficient 0.36, P < 0.05) and respiratory disturbance index (coefficient 0.16, P < 0.05), higher income levels (coefficient 3.26, p < 0.05), and higher levels of knowledge about OSAS (coefficient -2.98, P < 0.1).

Conclusions: Individuals who are more aware of their own health condition, are better informed about OSAS and have higher incomes are more likely to purchase the device. We suggest reducing the level of co-payment and providing patients with more information about the severe effects of OSAS.

Background: Cardiac patients express elevated levels of B-type natriuretic peptide and the amino terminal segment of its prohormone (NT-proBNP). However, there are non-cardiac causes of NT-proBNP level elevation.

Objectives: To determine the upper limit of NT-proBNP for pediatric patients with acute non-cardiac disease.

Methods: We compared NT-proBNP concentrations in healthy children and children with acute non-cardiac, mostly febrile, and acute cardiac disease. We used the Student t-test and Mann-Whitney test for group comparisons, and Pearson's and Spearman's correlation coefficients to test relationships between variables. 

Results: In 138 patients with acute non-cardiac diseases (mean age 3.7 years, 53% male), median NT-proBNP concentration was 162 pg/ml, upper limit (95% percentile) 1049 pg/ml. The level did not vary significantly by disease category; was negatively correlated with weight, weight percentile, age and hemoglobin level; and positively correlated with creatinine level. Multivariant analysis showed weight to be the only factor influencing NT-proBNP level. Levels were higher in children with acute non-cardiac diseases versus healthy children (median 88 pg/ml, P < 0.001, n= 59), and lower than levels in patients with acute cardiac disease (median 29,986 pg/ml, P < 0.001, n=29). Receiver operating characteristic analysis showed good NT-proBNP performance for differentiation between children with acute cardiac versus non-cardiac disease (area under the curve 0.958), at a cutoff of 415 pg/ml.

Conclusions: NT-proBNP levels are higher in children with acute non-cardiac diseases than in healthy children, but lower than in children with acute cardiac disease. NT-proBNP negatively correlated with weight and weight percentile.
 

Background: Surgery is considered the mainstay of treatment for esophageal carcinoma. Transhiatal esophagectomy with cervical esophagogastric anastomosis is considered relatively safe with an oncological outcome comparable to that using the transthoracic approach.

Objectives: To review the results of the first 100 transhiatal esophagectomies performed in a single Israeli center.

Methods: The records of all patients who had undergone transhiatal esophagectomy during the period 2003–2009 were reviewed. The study group comprised the first 100 patients. All patients who had undergone colon or small bowel transposition were excluded. Indications for surgery included esophageal cancer, caustic injury and achalasia.

Results: The median follow-up period was 19.5 months. The anastomotic leakage rate was 15% and all were managed successfully with local wound care. The benign stricture rate was 10% and all were managed successfully with endoscopic balloon dilation. Anastomotic leakage was found to be a risk factor for stricture formation. Overall survival was 54%. Response to neoadjuvant therapy was associated with a favorable prognosis.

Conclusions: Transhiatal esophagectomy is a relatively safe approach with adequate oncological results, as long as it is performed in a high volume center.
 

 Background: The prevalence of vertebral fractures in systemic lupus erythematosus (SLE) ranges between 20% and 21.4%, and patients with these fractures have impaired walking and activities of daily living. Moreover, clinical and radiological vertebral fractures have been associated with increased mortality.
 Objectives: To compare the quality of life of patients with SLE[1] with and without vertebral fractures.

Methods: The study group comprised 140 women with SLE undergoing screening for vertebral fractures using a standardized method. SLE disease activity and organ damage were measured by the Mexican Systemic Lupus Erythematosus Disease Activity Index (MEX-SLEDAI) and Systemic International Collaborating Clinics/American College of Rheumatology damage index (SLICC), respectively. The QUALEFFO and Center for Epidemiologic Studies Depression Scale were used to measure health-related quality of life and depression, respectively.

Results: The median age of the 140 patients was 43 years (range 18–76); disease duration was 72 months (range 6–432); 49.7% were menopausal. Thirty-four patients (24.8%) had vertebral fractures (≥ 1), mostly in the thoracic spine. Patients with vertebral fractures had a higher mean age (49.5 ± 13.4 vs. 41 ± 13.2 years, P = 0.001) and disease damage (57.1% vs. 34.4%, P = 0.001). The global QUALEFFO score was not different between the vertebral fractures group and the non-vertebral group. The only significant difference in the QUALEFFO items was in physical function (P = 0.04). A significant correlation was found between the severity of vertebral fractures and the QUALEFFO pain (r = 0.27, P = 0.001) and physical function (r = 0.37, P = 0.02) scores. The number of vertebral fractures correlated only with physical function (r = 0.01).

Conclusions: The HRQOL of women with SLE is low, regardless of whether they have vertebral fractures or not, but patients with vertebral fractures have worse physical function compared to those without. Strategies to improve the HRQOL of patients with SLE with or without vertebral fractures are necessary.

Background: The rate of infection with Clostridium difficile colitis and its associated mortality have been increasing in the last decade. The molecular epidemiology of C. difficile in Israel has as yet not been studied.

Objectives: To screen for the existence of the 027 and 078 ribotypes and determine the longitudinal molecular epidemiology of the circulating clinical C. difficile isolates in a large hospital in central Israel.

Methods: Polymerase chain reaction (PCR) ribotyping was performed on C. difficile isolates obtained from hospitalized patients from November 2003 to May 2004 (first study period) and September 2009 (second study period). Isolates with PCR[1] ribotype patterns, unlike those of the available reference strains (078 and 027), were labeled with letters. Forty-six isolates from the first study period and 20 from the second were analyzed.

Results: PCR strain typing of C. difficile isolates yielded approximately 26 unique ribotypes. During the first study period, ribotype A and B accounted for 30% and 28%, respectively, whereas ribotype E and K accounted for 6.5% for each. During the second study period, ribotypes A, E and K disappeared, and the incidence of ribotype B decreased from 28% to 15%. One isolate (1/20, 5%) emerged during the second period and was identified as ribotype 027. Moxifloxacin resistance was found in 93% of ribotype A isolates, 81% of the ribotype B group, and in 44% of other ribotypes.

Conclusions: The predominant ribotypes circulating in our institution were diverse and changing. This is the first report on the emergence of the 027 ribotype in Israel.

Background: The incidence and prevalence of coronary heart disease (CHD) among Bedouins living in the Negev region was very low until the 1960s. During the past 50 years this pattern has changed: in parallel to the changes in lifestyle and nutrition in the Bedouin population, a rapid increase in incidence and mortality from CHD occurred. The relationship between the rise in CHD incidence and the degree of urbanization in this population has not been investigated to date. The study hypothesis was that the prevalence of risk factors and the outcome of myocardial infarction in Bedouins differ between those settled in permanent villages and those remaining in unrecognized villages.

Objectives: To compare the prevalence of cardiovascular risk factors, clinical characteristics, and in-hospital management of a first acute myocardial infarction (AMI) in two Bedouin groups: those residing in permanent villages versus those residing in unrecognized villages.

Methods: We conducted a retrospective analysis of in-hospital data of 352 patients admitted with a first AMI during the period 1997–2003 to Soroka Medical Center, the only medical facility in the region.

Results: There were no differences between the two groups regarding the major cardiovascular risk factors and outcome. A relatively greater number of patients from urban areas underwent catheterization of any sort during their hospitalization (primary, rescue, and risk stratification; P = 0.038). No significant difference was found between the two groups in the type of catheterization performed (P = 0.279).

Conclusions: We found no differences in the clinical characteristics and in-hospital management of patients with AMI between Bedouins residing in permanent villages versus unrecognized villages.