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: Influenza vaccination of community-dwelling elderly is widely recommended. Observational studies have shown a strong association between physicians' personal vaccination status and their reported level of recommendation to patients and possibly their patients' actual vaccination. No published trials have examined whether increasing vaccination rates of primary care staff raises vaccination among their patients. Proof of a positive effect would support the notion that vaccinating health care workers benefits their patients.

Objectives: To examine whether an intervention to increase staff vaccination also increases vaccination of their patients aged 65 and over.

Methods: A trial examining an intervention aiming to raise staff immunization rates was performed in primary care community clinics in the Jerusalem area. The study population comprised the staff of 13 randomly chosen intervention clinics during the season of 2007–2008, with another 14 clinics serving as controls. The intervention resulted in a staff vaccination rate of 52.8% compared to 26.5% in the control clinics (66.1% and 32.2% among physicians). No intervention was directed at the patients. Data on patient vaccination and other patient characteristics were extracted from the health funds’ computerized databases.

Results: The percentage of patients vaccinated during the intervention season was 57.8% in both intervention and control groups, reflecting an increase of 14.4% compared to the previous season in the intervention clinics and of 13.4% in the control clinics. Logistic regression demonstrated a statistically significant association between intervention and patient vaccination with an odds ratio of 1.10 (95% confidence interval 1.03–1.18). However, analysis adjusting for clustering did not show a significant association.

Conclusions: Increasing influenza vaccination of the medical staff did not substantially increase patient vaccination. These results do not show any patient benefit from staff vaccination in primary care.
 

Background: Amyloidosis of familial Mediterranean fever (FMF) may lead to end-stage renal failure, culminating in kidney transplantation. Since amyloidosis is prompted by high serum amyloid A (SAA) levels, increased SAA is expected to persist after transplantation. However, no data are available to confirm such an assumption.

 Objectives: To determine SAA levels in kidney-transplanted FMF-amyloidosis patients and evaluate risk factors for the expected high SAA levels in this patient group.

Methods: SAA, C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) values were obtained from 16 kidney-transplanted FMF-amyloidosis patients, 18 FMF patients without amyloidosis and 20 kidney-transplanted patients with non-inflammatory underlying disease. Demographic, clinical and genetic risk factors evaluation was based on data extracted from files, interviews and examination of the patients.

Results: SAA level in FMF patients who underwent kidney transplantation due to amyloidosis was elevated with a mean of 21.1 ± 11.8 mg/L (normal ≤ 10 mg/L). It was comparable to that of transplanted patients with non-inflammatory disorders, but tended to be higher than in FMF patients without amyloidosis (7.38 ± 6.36, P = 0.08). Possible risk factors for the elevated SAA levels in kidney transplant patients that were excluded were ethnic origin, MEFV mutations, gender, age and disease duration.

Conclusions: Kidney-transplanted patients with FMF-amyloidosis and with other non-FMF causes displayed mildly elevated SAA levels, possibly resulting from exposure to foreign tissue rather than from various FMF-related factors. 

Background: Esophageal perforations and postoperative esophageal leaks are associated with substantial morbidity and mortality and pose a difficult therapeutic challenge. 

Objectives: To evaluate the outcome of removable self-expanding metallic stents (SEMS) as a treatment for postoperative leaks and perforations of the esophagus and stomach.

Methods: We conducted a retrospective study of all patients in one medical center who underwent temporary insertion of a covered plastic stent for postoperative leaks and perforations of the esophagus and stomach from June 2009 to February 2010. Data were retrieved from hospital and outpatient clinical data charts. Data included indication for insertion, post-insertion outcome including stent complications, and follow-up after stent removal.

Results: The indications for stent insertion were postoperative leak in four patients and postoperative esophagopleural fistula in one patient. Three of the patients had a leak at the gastro-esophageal junction following laparoscopic sleeve gastrectomy. In all cases the stent insertion was completed successfully. In three patients the stent migrated distally. In two of these three it was repositioned or replaced endoscopically, and in the third it was excreted in the feces. Stents were removed electively after 6 to 7 weeks. All patients recovered fully and were discharged from the hospital.

Conclusions: SEMS insertion may have an important role in the management of postoperative leaks and perforations of the esophagus and stomach and should be considered in such cases.
 

Background: Diabetic retinopathy is a leading cause of adult blindness and accounts for about 10% of cases of legal blindness in Israel. Only about half of the patients with diabetes in Israel have regular eye examinations.

Objectives: To evaluate, for the first time in southern Israel, a new service for diabetic retinopathy screening that uses a mobile non-mydriatic mobile fundus camera in primary care patients.

Methods: Diabetic members of the largest health fund in southern Israel and over 18 years old were invited for non-mydriatic fundus examination between January and October 2009. Screening was performed by a trained photographer using the Topcon TRC NW-6S non-mydriatic camera in nine primary care centers.

Results: A total of 4318 diabetic patients were screened, of whom 53% were classified as normal. The incidence of diabetic retinopathy was 15.8% (1.2% had proliferative retinopathy and 2.4% had suspected macular edema and were referred for laser treatment). Other possible sight-threatening conditions were detected in 9.3%. Fundus pictures were inadequate for assessment in 16% of cases.

Conclusions: Diabetic retinopathy screening with a mobile non-mydriatic fundus camera improved the quality of care for diabetic patients in southern Israel. This screening method identified patients requiring prompt referral to the ophthalmologist for further complete eye examination. Extending this screening program to other areas in the country should be considered.
 

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: Assessment of small intestinal disease remains a challenge for both clinicians and radiologists. Modern magnetic resonance enterography (MRE) is a non-radiation modality that can demonstrate both intestinal wall pathologies and extraluminal lesions.

Objectives: To analyze the results of 213 MRE scans performed since 2005.

Methods: Consecutive MRE[1] scans performed in our academic medical center between December 2005 and November 2009 were reviewed for patients' demographic data, indications for the examination, and main imaging findings. The imaging findings recorded were mural changes and intraluminal filling defects; there were also mesenteric findings and extraintestinal inflammatory findings.

Results: During the study period 213 MRE scans were performed; 70% of them for proven or suspected Crohn's disease (CD) of the small bowel. Another indication was small bowel neoplasm (6% of the scans). Bowel wall thickening and enhancement were seen in 60% and 53% of MRE scans, respectively. Mesenteric involvement was found in 52% of the patients. Incidental extraintestinal findings were detected in 17% of the scans. In 22% of the scans there was no pathological finding.

Conclusions: Our 4-year clinical experience with MRE shows that this non-invasive and non-radiating modality is a powerful technique for evaluation and long-term follow-up of small bowel pathologies. The most common clinical indication was the evaluation of Crohn’s disease. With physicians’ increased awareness, the future use of MRE in the evaluation of other small bowel pathologies such as neoplasm and celiac disease will increase.

Background: The diagnostic and therapeutic approach to hilar cholangiocarcinoma and thus the prognosis have changed significantly over the last two decades. Nonetheless, hilar  cholangiocarcinoma  presents a complex surgical challenge.

Objectives: To assess the outcome of the radical approach for the management of types III and IV hilar  cholangiocarcinoma.

Methods: We conducted a retrospective single-center study. Preoperative diagnosis was based on ultrasound, computed tomography and selective percutaneous cholangiography without tissue diagnosis. Surgery was radical and included en-bloc liver, extrahepatic biliary tree and hilar lymph nodes resection, followed by biliary reconstruction with hepatico-jejunostomy.

Results: Fifteen patients (mean age 49 years, range 24–72) were managed accordingly. Anatomic classification of the biliary involvement was Bismuth-Corlette type IIIA (n=4), type IIIB (n=3) and type IV (n=8). The surgical procedures performed included four right hepatic lobectomies, five left hepatic lobectomies and six trisegmentectomies (all extended to the caudate lobe). Complete negative resection margins (R0) were accomplished in 12 cases (80%). Regional lymph node metastases were detected in five cases. There were two perioperative mortalities. Long-term follow-up (mean 30 months, range 6–72) revealed local recurrences in two cases, distant metastases in three, and both local and distant in two cases. Eleven patients are alive and 6 are without evidence of disease. The overall 2- and 5-year survival is 78% and 38% respectively.

Conclusions: In selected patients, the aggressive surgical approach to hilar cholangiocarcinoma is justified and can result in long-term survival.
 

Background: The rate of brain abnormalities in asymptomatic term neonates varies substantially in previous studies. Some of these rates may justify general screening of healthy newborns by head ultrasound.

Objectives: To assess the incidence of intracranial abnormalities among asymptomatic term newborns with HUS[1] and to detect high-risk populations that might need such screening.

Methods: This was a prospective study in 493 term newborns who underwent HUS and a neurological evaluation during the first 3 days of life. The neurological examination results were unknown to the sonographist and the examiner was blinded to the HUS findings. The abnormal HUS findings were classified as significant or non-significant according to the current literature.

Results: Abnormal HUS was found in 11.2% of the neonates. Significant findings were noted in 3.8% of the infants. There was no association between non-structural HUS findings (hemorrhage or echogenicity) and mode of delivery. There was no relationship between any HUS abnormality and birth weight, head circumference and maternal age, ethnicity, education or morbidity. The rate of abnormal neurological, hearing or vision evaluation in infants with a significant abnormal HUS (5.2%) was comparable to the rate in infants with normal or non-significant findings on HUS (3.1%).

Conclusions: There is no indication for routine HUS screening in apparently healthy term neonates due to the relatively low incidence of significant brain abnormalities in these infants in our population.

Background: The term chronic multi-symptom illness refers to a spectrum of pain disorders, such as fibromyalgia and chronic fatigue syndrome, that are characterized by unexplained chronic pain, fatigue, and cognitive and mood complaints

Objectives: To examine the hypothesis that exercise cessation is associated with symptoms similar to CMI[1] in well-trained amateur athletes.

Methods: The study, conducted in running and triathlon clubs in Israel, involved 26 asymptomatic healthy athletes who regularly exercise 6.75 ± 3.65 hours a week. All athletes were instructed to refrain from physical activity for 7 days. All underwent a complete physical exam, rheumatological assessment including non-articular tenderness threshold (using dolorimeter) and tender points. In addition they completed the SF-36 quality of life questionnaire. Assessments were conducted before exercise cessation and 7 days later.

Results: Seven days after sports deprivation all subjects were significantly more tender by all tender measures (P < 0.001) (dolorimeter thresholds and tender point count). There was also a significant reduction in the scores for physical role function (P < 0.001), emotional role function (P < 0.001) and summary subscales of the SF-36 questionnaire after exercise cessation.

Conclusions: Exercise deprivation is associated with change in non-articular tenderness threshold and reduction in quality of life scores. This may be associated with the development of chronic multi-symptom illness.