Israel Medical Association Journal

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. 

Objectives: To assess the results of endovascular revascularization for lower extremity ischemia in dialysis patients.

Methods: We conducted a retrospective review of all dialysis patients who underwent endovascular treatment for critical limb ischemia (CLI) in our institution between 2007 and 2011. Data collected included comorbidities, clinical presentation, anatomic distribution of vascular lesions, amputation and survival rates.

Results: We identified 50 limbs (41 patients). Indications included: gangrene in 22%, non-healing wounds in 45%, rest pain in 31%, and debilitating claudication in 4%. Mean follow-up was 12 months (1–51 months). Nineteen patients required amputations. Freedom from amputation at 5 years was 40%. Factors associated with amputation included non-healing wounds or gangrene (68% and 36% respectively) and diabetes (P < 0.05). The survival rate was 80% after 5 years.

Conclusions:  Despite improvement in endovascular techniques for lower extremity revascularization, the incidence of limb salvage among dialysis patients remains poor, resulting in a high rate of major amputations. 

Objective: To assess the impact of multiple births on the severity of RSV infection and define risk factors for acquiring RSV infection in infants of multiple birth.

Methods: Clinical data on infants hospitalized with RSV infection between 2008 and 2010 were retrospectively collected.

Results: Twins comprised 7.6% (66/875) of hospitalized infants with RSV bronchiolitis during the study period. Infants in the twin group were younger (122.4 ± 131.7 vs. 204.5 ± 278.8 days, P = 0.014), had a lower mean gestational age (35.3 ± 2.6 vs. 38.6 ± 2.5 weeks, P < 0.001), and were more likely to have been born prematurely compared with singleton infants (65.6% vs. 13%, P < 0.001). On a multivariable logistic regression analysis, young age, early gestational age and male gender were the only variables identified as risk factors for pediatric intensive care unit admission (P < 0.001, P < 0.001 and P = 0.03, respectively). In contrast, the mere fact of a child being a twin was not found to be a significant risk factor for disease severity. In addition, if one twin is hospitalized due to RSV infection, the other has a 34% chance of also being hospitalized with bronchiolitis. Young age was a significant risk factor for hospitalization of the second twin (P < 0.001).

Conclusions: Our findings suggest that twins hospitalized with RSV bronchiolitis do not have an increased risk for severe infection as compared to singletons. However, a twin of an infant hospitalized with RSV infection has a considerable risk of also being hospitalized with bronchiolitis, thus close monitoring is recommended. 

Objectives: To evaluate health utilization patterns in two primary care clinics in southern Israel: one under continuous attacks of Qassam rockets as compared with a similar clinic not under a rocket threat.

Methods: We conducted a retrospective cross-sectional study in two primary care clinics in southern Israel, with 11,630 persons listed in the two clinics during the entire study period. The primary outcome measures were total annual number of visits per person to the clinic and for specific diagnoses, and the number of drug prescriptions issued, emergency room (ER) visits, hospitalization days, cardiac catheterizations and coronary bypass surgeries.

Results: In both clinics there was an increase over time in the mean annual number of visits per person. During the years of severe attacks there was an increase in visits with a chief complaint of depression and anxiety and an increase in the number of anxiolytic prescriptions in the study clinic compared with the control. During the same period there was a decrease in the number of ER visits in the study clinic compared with the control.

Conclusions: The population under continuous life-threatening events showed more depression and anxiety problems. Under severe bombardment, the residents prefer not to leave home, unless necessary.

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.

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: Chondrosarcoma of the larynx is a rare tumor. The most common symptom is hoarseness. Treatment is controversial.

Objectives: To describe six patients with laryngeal chondrosarcoma from a single center.

Methods: The medical records of a major tertiary hospital were reviewed for all patients with laryngeal chondrosarcoma diagnosed and treated from 1959 to 2010. Data on background, clinical treatment and outcome were collected.

Results: Six patients, all males with a mean age of 53.3 years, were identified. Partial laryngectomy was performed in three patients, and total laryngectomy, local excision, and partial cricoidectomy in one patient each. Four patients had a permanent tracheostomy after surgery. One patient required postoperative chemotherapy and one radiotherapy. Follow-up time was 12–216 months (mean 102 months). Recurrence developed in two patients 2 and 8 years after initial treatment and was treated by salvage surgery in both patients. One patient died during the follow-up from an unrelated cause. The others are currently alive.

Conclusions: This study supports earlier reports recommending initial treatment with partial or total laryngectomy for laryngeal chondrosarcoma. Long-term follow-up for recurrence is advised. We recommend preserving the larynx, if possible, even if a permanent tracheostomy is necessary.
 

Background: Myotonic dystrophy type 2 (DM2) is an autosomal dominant, multisystem disorder caused by a CCTG tetranucleotide repeat expansion located in intron 1 of the zinc finger protein 9 gene (ZNF9 gene) on chromosome 3q 21.3.

Objectives: To describe the clinical, electrophysiologic and pathologic findings in patients with myotonic dystrophy 2.

Methods: We evaluated 10 patients genetically, clinically and electrophysiologically during the years 2007 to 2008.

Results: All patients were of Jewish European ancestry. Among affected individuals, eight patients had symptoms of proximal muscle weakness, two had muscle pain, and two exhibited myotonia. On physical examination six patients had severe weakness of hip flexor muscles. Seven individuals underwent cataract surgery, and cardiac involvement was seen in one case. On the initial electromyographic (EMG) examination five patients demonstrated myotonic discharges; repeated studies showed these discharges in nine cases. Six muscle biopsies showed non-specific pathological changes. Seven patients had an affected first-degree relative with either a diagnosed or an undiagnosed muscular disorder, consistent with an autosomal dominant trait.

Conclusions: DM2 may often present with proximal muscle weakness without myotonia. EMG may initially fail to show myotonic discharges, but these discharges may eventually show in most cases on repeated EMG. Thus, DM2 may be underdiagnosed and should be included in the differential diagnosis of adult patients of Jewish European ancestry presenting with proximal lower limb weakness.
 

Background: Adjuvant/neoadjuvant chemotherapy in breast cancer patients may be associated with amenorrhea and a marked reduction in ovarian reserve.

Objectives: To assess the use of letrozole with follicle-stimulating hormone (FSH) in gonadotropin-releasing hormone (GnRH) analogue protocols, based on reported attempts to avoid the estradiol (E2) increase during controlled ovarian hyperstimulation for embryo cryopreservation in breast cancer patients using a combination of low dose FSH and aromatase inhibitor (letrozole) in a GnRH-antagonist protocol.

Methods: Twenty-four breast cancer patients were treated with recombinant FSH (150–450 U/day) and letrozole (5 mg/day) in a long GnRH-agonist (n=7) or GnRH-antagonist (n=17) protocol. After oocyte retrieval, insemination and/or intracytoplasmic sperm injection was performed. The embryos were frozen.

Results: The average interval from surgery to oocyte retrieval was 40 days. Average duration of treatment was 9.6 days and mean peak E2 level 1342 ± 1091 pmol/L, yielding 16.0 ± 16.3 oocytes (range 0–82). Mean fertilization rate was 69.5 ± 20.4% and mean number of embryos cryopreserved 10.3 ± 9.3. More oocytes were retrieved with the long GnRH protocol, but the difference was not statistically significant (24.8 ± 24.6 vs. 12.0 ± 8.8 pmol/L, P = 0.07).

Conclusions: As previously reported, ovarian stimulation with letrozole and FSH, in both the long GnRH-agonist and GnRH-antagonist protocols, is apparently effective in breast cancer patients and spares them exposure to high E2 levels.