Israel Medical Association Journal

Objectives: To investigate the reasons for non-adherence associated with cardiovascular risk factors among Bedouins.

Methods: We identified Bedouin patients with HTN, DM or LMD from medical records and randomly selected 443 high adherent and 403 low adherent patients. Using trained interviewers we conducted in-depth structured interviews regarding knowledge and attitudes to chronic illness and its treatment, health services evaluation, and socio-demographic factors.

Results: The study population included 99 high and 101 low adherent patients. More low adherent patients agreed that traditional therapy can replace prescribed medications for DM, HTN or LMD (47% vs. 26%, P < 0.01), and 10% used only traditional medications. Also, more low adherent patients believed that the side effects of prescribed drugs are actually worse than the disease itself (65% vs. 47%, P < 0.05), and 47% cited this as a reason for discontinuing drug treatment (47% vs. 31%, P < 0.05).

Conclusions: Our findings suggest that in this minority population the basis for non-adherence derives directly from patients' perceptions of chronic disease and drug treatment. A focused intervention should emphasize the importance of early evidence-based drug therapy with open patient-physician dialogue on the meaning of chronic disease and the side effects of prescribed drugs.

Objectives: To retrospectively determine the clinical response to ¹³¹I-MIBG therapy at low doses in patients with refractory neuroblastoma.

Methods: We performed a retrospective chart review of 10 patients with neuroblastoma treated with ¹³¹I-MIBG at Rambam Health Care Campus from 1994 to 2012. Clinical data, number of ¹³¹I-MIBG courses delivered, toxicities, and clinical responses were reviewed. MIBG scan was performed after each course.

Results: Twenty-one courses of ¹³¹I-MIBG were delivered to 10 patients (3 girls, 7 boys). Their mean age was 3.8 years (range 1.5–6 years). All patients received several protocols of chemotherapy including the high dose form. Three patients received three courses of ¹³¹I-MIBG with a minimum of 6 weeks between each course, five patients received two courses, and two patients received only one course. An objective response to the first course was obtained in nine patients and to the second course in six of eight, and in three children who underwent the third course the pain decreased. One patient has no evidence of disease, four are alive with disease, and five died of the disease. No unanticipated toxicities were observed.

Conclusions: Low dose ¹³¹I-MIBG is an effective and relatively non-toxic treatment in neuroblastoma disease palliation. Rapid and reproducible pain relief with ¹³¹I-MIBG was obtained in most of the children. Treatment with systemic radiotherapy in the form of low dose ¹³¹I-MIBG was easy to perform and effective in cases of disseminated neuroblastoma, demonstrating that this primary therapy can be used for palliative purposes.

Background: Transthyretin (TTR)-associated familial amyloid polyneuropathy (FAP) is an autosomal dominant multisystem disease with neurological and extra-neurological manifestations. It is caused by various mutations in the TTR gene leading to the formation of insoluble amyloid.

Objectives: To describe the clinical and genetic findings in patients with TTR-associated FAP in Israel.

Methods: We evaluated eight patients clinically and genetically during the years 2006 to 2011.

Results: At onset, all the patients exhibited sensory loss of the lower and upper limbs, five patients experienced muscle pain, and one patient had lower limb weakness. Five patients had autonomic nervous system manifestations, and four demonstrated evidence of amyloid cardiomyopathy. Nerve conduction studies showed sensorimotor axonal neuropathy in all patients. Sural nerve biopsies were obtained in five patients; only three biopsies revealed amyloid deposit. In four patients of Yemenite descent, genetic analysis of the TTR gene demonstrated ser77tyr mutation. One patient of Tunisian descent and one Ashkenazi patient harbored the val30met mutation. One patient of Iranian descent showed val32ala mutation, and another Ashkenazi patient showed phe33leu mutation.

Conclusions: TTR-associated FAP is a progressive and fatal disease that exists in the Israeli population and is unproportionally common among Yemenite Jews. This disease may be under-diagnosed and should be considered in the differential diagnosis of any patient with rapidly progressive neuropathy, especially with autonomic involvement or extra-neural features. The absence of amyloid in nerve biopsy should not rule out the diagnosis.  
 

Background: Understanding the mechanism and the main components involved in rheumatic mitral regurgitation (MR) associated with dominant pliable mitral stenosis (MS) may improve our ability to repair some mixed rheumatic mitral valve pathologies.

Objectives: To assess mitral valve structural components in pure mitral stenosis versus mitral stenosis associated with mild regurgitation

Methods: Using two-dimensional echocardiography, we performed mitral valve structural analysis in two groups of patients prior to balloon mitral valvuloplasty (BMV). The first group, consisting of 13 females and 2 males (mean age 39 ± 5 years), suffered from pure pliable mitral stenosis (PPMS), while the second group, with 22 females and 2 males (mean age 44 ± 5 years), had mixed mitral valve disease (MMVD) characterized by mild MR in the presence of dominant pliable MS. All echocardiographic measurements relating to the mechanism of MR were undertaken during the systolic phase.

Results: The mean Wilkins scores of the PPMS and MMVD groups were 7 ± 1 and 8 ± 1 respectively (P = 0.004). No significant differences were found between the MMVD group and the PPMS group regarding annular circumference (15.5 ± 1.4 cm vs. 15.4 ± 1.6 cm, P = 0.84), annular diameter (36 ± 4 mm vs. 38 ± 5 mm, P = 0.18), and chordae tendinae length directed to the anterior mitral leaflet (AML) (10 ± 2 mm vs. 11 ± 2 mm, P = 0.137). However, anterior vs. posterior mitral leaflet length during systole was significantly lower in the MMVD than in the PPMS group (2.2 ± 0.5 vs. 2.8 ± 0.4, P = 0.02), whereas the AML thickness at the co-aptation point was greater in the MMVD than in the PPMS group (7 ± 1 vs. 5 ± 1 mm, P = 0.0004).

Conclusions: In rheumatic valves, thickening and shortening of the AML are the main factors determining the appearance of mild MR in the presence of dominant pliable MS.

Background: Local recurrences after breast-conserving surgery occur mostly at the site of the primary carcinoma. The main objective of postoperative radiotherapy is sterilization of residual cancer cells. Whole-breast radiotherapy is the standard of care, but its utility has recently been challenged in favor of radiotherapy limited to the area at highest risk of recurrence. Intraoperative electron radiotherapy (IOeRT) is an innovative technique for accelerated partial breast irradiation (APBI) that is applied to selected patients affected by early breast cancer.

Objectives:  To describe our experience with IOeRT at the Rambam Health Care Campus in Haifa since we began utilizing this modality in 2006.

Methods: From April 2006 to September 2010, 31 patients affected by unifocal invasive duct breast carcinoma ≤ 2 cm diameter received wide local resection followed by intraoperative radiotherapy with electrons. Patients were evaluated for early and late complications, and other events, 1 month after surgery and every 3 months thereafter for the duration of the first 2 years.

Results: After a mean follow-up of 36 months, seven patients developed mild breast fibrosis and three suffered from mild postoperative infection. Rib fractures were observed in four patients before routine lead shielding was initiated. Additional whole-breast irradiation was given to four patients. None of the patients developed local recurrences or other ipsilateral cancers. Similarly, no contralateral cancers or distant metastases were observed.

Conclusions: Intraoperative electron radiotherapy may be an alternative to external beam radiation therapy in an appropriate selected group of early-stage breast cancer patients. However, long-term results of clinical trials are required to better evaluate the indications and utility of this technique in the management of breast cancer.
 

Background: The prognostic value of P-wave duration has been previously evaluated by signal-averaged ECG (SAECG) in patients with various arrhythmias not associated with acute myocardial infarction (AMI).

Objectives: To investigate the clinical correlates and prognostic value of P-wave duration in patients with ST elevation AMI (STEMI).

Methods: The patients (n=89) were evaluated on the first, second and third day after admission, as well as one week and one month post-AMI. Survival was determined 2 years after the index STEMI.

Results: In comparison with the upper normal range of P-wave duration (< 120 msec), the P-wave duration in STEMI patients was significantly increased on the first day (135.31 ¡À 29.29 msec, P < 0.001), up to day 7 (127.17 ¡À 30.02 msec, P = 0.0455). The most prominent differences were observed in patients with left ventricular ejection fraction (LVEF) ¡Ü 40% (155.47 ¡À 33.8 msec), compared to LVEF > 40% (128.79 ¡À 28 msec) (P = 0.001). P-wave duration above 120 msec was significantly correlated with increased complication rate namely, sustained ventricular tachyarrhythmia (36%), congestive heart failure (41%), atrial fibrillation (11%), recurrent angina (14%), and re-infarction (8%) (P = 0.012, odds ratio 4.267, 95% confidence interval 1.37¨C13.32). P-wave duration of 126 msec on the day of admission was found to have the highest predictive value for in-hospital complications including LVEF < 40% (area under the curve 0.741, P < 0.001).  However, we did not find a significant correlation between P-wave duration and mortality after multivariate analysis.

Conclusions: P-wave duration as evaluated by SAECG correlates negatively with LVEF post- STEMI, and P-wave duration above 126 msec can be utilized as a non-invasive predictor of in-hospital complications and low LVEF following STEMI.

Background: The presence of stones in the common bile duct (CBD) may cause complications such as obstructing jaundice or ascending cholangitis, and the stones should be removed.

0bjectives: To report our results with percutaneous elimination of CBD stones from the gallbladder through the papilla.

Methods: During a 4 year period, six patients (five men and one woman, mean age 71.5 years) who had CBD stones and an existing gallbladder drain underwent percutaneous stone push into the duodenum after balloon dilatation of the papilla, with a diameter equal to that of the largest stone. Access into the CBD was from the gallbladder, using an already existing percutaneous gallbladder drain (cholecystostomy tube).

Results: Each patient had one to three CBD stones measuring 7–14 mm. Successful CBD stone elimination into the duodenum was achieved in five of the six patients. The single failure occurred in a patient with choledochal diverticulum, who was operated successfully. There were no major or minor complications during or after the procedures.

Conclusions: Trans-cholecystic CBD stone elimination is a safe and feasible percutaneous technique that utilizes existing tracts, thus obviating the need to create new percutaneous access. This procedure can replace endoscopic or surgical CBD exploration.
 

Background: Clefts of the lip and palate are the most common significant congenital birth anomaly of orofacial region. The condition may vary from a minor easily correctable cleft to a significant functional and cosmetic incapacitation. This is the first epidemiological study of orofacial clefts in the Negev region in Israel.

Objectives: To establish the frequency of cleft lip and palate in the population of the Negev, characterize the demographic features of affected individuals and find possible risk factors, compare the risk in two major population groups: Bedouin and Jewish in a well-defined geographic area, and determine whether there is a change over time in the birth of babies with facial clefts.

Methods: We conducted a retrospective survey of the Soroka Medical Center archives. The sample population comprised all 131,218 babies born at Soroka during the 11 year period 1 January 1996 to 31 December 2006. Statistical tests used Pearson's chi-square test, Student’s t-test and Spearman's correlation coefficient test according to the type of parameter tested.

Results: During the study period 140 babies were born with orofacial cleft. The overall incidence of cleft lip and palate was 1.067/1000. The incidence of facial clefts was 1.54/1000 among Bedouins and 0.48/1000 among Jews (P < 0.001). Cleft palate was significantly more frequent in female than male babies (P = 0.002). Over the study years we found a significant decrease in the incidence of facial clefts in the Bedouin population, with Spearman's correlation coefficient rank -0.9 (P < 0.01).

Conclusions: A significant decrease occurred in the incidence of facial clefts among Bedouin. This change may be attributed to prenatal care in the Bedouin Negev population as part of social and health-related behavior changes. The reduction in rates of congenital malformations, however, does not mean a reduction in the number of cases in a growing population. Also, with a modern western lifestyle, the expectancy and demand for reconstructive facial surgery and comprehensive care for these children are on the rise.

Background: The treatment of rectal cancer has changed significantly over the last few decades. Advanced surgical techniques have led to an increase in the rate of sphincter-preserving operations, even for low rectal tumors. This was facilitated by preoperative oncologic treatment and the use of chemoradiation to downstage the tumor before resection. The introduction of total mesorectal excision further improved the oncologic outcome and became the standard of care. The use of laparoscopy for rectal resection is the most recent addition to this series of improvements, but in contrast to the use of laparoscopy in colon cancer its role is not yet well defined.

Objectives: To present our experience with laparoscopic surgery for upper and lower rectal tumors.

Methods: A database was used to prospectively collect all data on laparoscopic rectal surgery in our department since we started performing these procedures in 1997. Follow-up data were collected from outpatient clinic visits, oncology files and telephone interviews. Updated survival data were retrieved from the national census.

Results: Of 750 laparoscopic colorectal procedures performed over a 13 year period, 67 were for rectal cancer. Of these, 29 were resections for tumors in the upper rectum (11–15 cm from the anal verge) and 38 for tumors at 10 cm or below. Surgery was performed in 24 patients after neoadjuvant chemoradiation. There were 54 sphincter-preserving operations and 13 abdominoperineal resections. The mean operative time was 283 minutes. Conversion to an open procedure was required in 22% of the cases. Anastomotic leaks occurred in 17% of cases. Postoperative mortality was 4.5%. Long-term follow-up was available for 77% of the group, for a mean period of 42 months. Local recurrence was diagnosed in 4.5% of the patients and overall 5 year survival was 68%.

Conclusions: Laparoscopic rectal resection is a demanding procedure. However, laparoscopy may become the preferred approach since it is a minimally invasive procedure and has an acceptable oncologic outcome that is comparable to the open approach. This conclusion, however, needs further validation.
 

Background: Transjugular kidney biopsy (TJKB) was first described in 1990. Indications for TJKB include uncorrectable bleeding disorders and conditions precluding the prone position. Objectives: To describe our initial experience with TJKB.

Methods: Between February 2008 and December 2009 all patients in whom percutaneous biopsy was contraindicated or unsuccessful underwent image-guided TJKB using a standard set with a 19 gauge core biopsy needle. Prospectively collected data included indication, number of needle passes, contrast dose, tissue yield, and complications.

Results: Twelve patients, age range 15–76 years (mean 55), underwent 14 TJKB procedures. Indications for the transjugular route included bleeding diathesis, dyspnea, ventral hernia, ascites, marked obesity, need for concomitant liver biopsy or concomitant insertion of tunneled dialysis catheter, discrepant kidney size, and failed percutaneous attempt. Thirteen biopsies were performed in 11 patients; in one patient TJKB was abandoned due to unfavorable renal vein anatomy. Four patients were premedicated with desmopressin and one with platelet transfusion, due to prolonged bleeding time. Three to six passes (mean 3.8) were made per biopsy, with an overall yield of 9.6 ± 8.2 glomeruli, providing a definite diagnosis in nine patients and a probable diagnosis in two. In two patients the first biopsy attempt yielded insufficient tissue, necessitating a repeat procedure. There were two minor bleeding episodes not requiring intervention. Serum creatinine was unchanged after the procedure and hemoglobin levels asymptomatically dropped by 0.3 ± 1.0 g/dl within 48 hours, requiring no treatment.

Conclusions: TJKB appears to safely allow adequate tissue diagnosis in patients at increased risk for complications from or contraindications to percutaneous renal biopsy.