Israel Medical Association Journal

Background: Fertility treatments are responsible for the rise in high order pregnancies in recent decades and their associated complications. Reducing the number of embryos returned to the uterus will reduce the rate of high order pregnancies.     

Objectives: To explore whether obstetric history and parity have a role in the clinician’s decision making regarding the number of embryos transferred to the uterus during in vitro fertilization (IVF).

Methods: In a retrospective study for the period August 2005 to March 2012, data were collected from twin deliveries > 24 weeks, including parity, mode of conception (IVF vs. spontaneous), gestational age at delivery, preeclampsia, birth weight, admission to the neonatal intensive care unit (NICU), and Apgar scores. 

Results: A total of 1651 twin deliveries > 24 weeks were recorded, of which 959 (58%) were at term (> 37 weeks). The early preterm delivery (PTD) rate (< 32 weeks) was significantly lower with increased parity (12.6%, 8.5%, and 5.6%, in women with 0, 1, and ≥ 2 previous term deliveries, respectively). Risks for PTD (< 37 weeks), preeclampsia and NICU admission were significantly higher in primiparous women compared to those who had one or more previous term deliveries. Primiparity and preeclampsia, but not IVF, were significant risk factors for PTD. 

Conclusions: The risk for PTD in twin pregnancies is significantly lower in women who had a previous term delivery and decreases further after two or more previous term deliveries. This finding should be considered when deciding on the number of embryos to be transferred in IVF.  

 

Background: Syncope is a common clinical condition spanning from benign to life-threatening diseases. There is sparse information on the outcomes of syncopal patients who received an implantable cardiac defibrillator (ICD) for primary prevention of sudden cardiac death (SCD). 

Objectives: To assess the outcomes and prognosis of patients who underwent implantable cardiac defibrillator (ICD) implantation for primary prevention of SCD and compare them to patients who presented with or without prior syncope.

Methods: We compared the medical records of 75 patients who underwent ICD implantation for primary prevention of SCD and history of syncope to those of a similar group of 80 patients without prior syncope. We assessed the episodes of ventricular tachycardia (VT), ventricular fibrillation (VF), shock, anti-tachycardia pacing (ATP) and mortality in each group during follow-up.

Results: Mean follow-up was 893 days (810–976, 95%CI) (no difference between groups). There was no significant difference in gender or age. Patients with prior syncope had a higher ejection fraction rate (35.5 ± 12.6 vs. 31.4 ± 8.76, P = 0.02), experienced more episodes of VT (21.3% vs. 3.8%, P = 0.001) and VF (8% vs. 0%, P = 0.01), and received more electric shocks (18.7% vs. 3.8%, P = 0.004) and ATP (17.3% vs. 6.2%, P = 0.031). There were no differences in inappropriate shocks (6.7% vs. 5%, P = 0.74), cardiovascular mortality (cumulative 5 year estimate 29.9% vs. 32.2%, P = 0.97) and any death (cumulative 5 year estimate 38.1% vs. 48.9%, P = 0.18).

Conclusions: Patients presenting with syncope before ICD implantation seemed to have more episodes of VT/VF and shock or ATP. No differences in mortality were observed

 

Background: Despite the increased worldwide recognition of attention deficit/hyperactivity disorder (ADHD), there is a variability in the diagnostic rate of both ADHD and its co-morbidities. These diversities are probably related to the methodology and instruments used for the diagnosis of ADHD and to awareness and cultural interpretation of its existence. 

Objectives: To identify consistent differences in the clinical profile of Arab and Jewish children with ADHD in Israel who differ in their cultural, ethnic and socioeconomic background. 

Methods: We analyzed the data of 823 children and adolescents with ADHD (516 Jews and 307 Arabs) and compared the clinical characteristics between these two ethnic groups. All patients were evaluated in two neuropediatric and child development centers in northern Israel: one in Haifa and one in Hadera. Children with autism and intellectual disabilities were excluded. 

Results: The distribution of ADHD subtypes was similar in both populations. However, learning disorders and psychiatric co-morbidities (behavioral difficulties and anxiety) were reported more frequently in the Jewish population. The most commonly reported adverse effects to psychostimulants were mood changes, anorexia, headache, insomnia and rebound effect, and were more frequently reported in the Jewish population (42.0% vs.18.0%, P < 0.05).

Conclusions: We assume that these differences are related to cultural and socioeconomic factors. We suggest that the physician take cultural background into consideration when treating patients with ADHD.

 

Background: Chronic kidney disease (CKD) is often accompanied by impairment of cardiac function that may lead to major cardiac events. Erythropoietin (EPO), a kidney-produced protein, was shown to be beneficial to heart function. It was suggested that reduced EPO secretion in CKD may play a role in the initiation of heart damage. 

Objectives: To investigate molecular changes in the EPO/erythropoietin receptor (EPO-R) axis in rat cardiomyocytes using a rat model for CKD.

Methods: We established a rat model for CKD by kidney resection. Cardiac tissue sections were stained with Masson’s trichrome to assess interstitial fibrosis indicating cardiac damage. To evaluate changes in the EPO/EPO-R signaling cascade in the myocardium we measured cardiac EPO and EPO-R as well as the phosphorylation levels of STAT-5, a downstream element in this cascade.

Results: At 11 weeks after resection, animals presented severe renal failure reflected by reduced creatinine clearance, elevated blood urea nitrogen and presence of anemia. Histological analysis revealed enhanced fibrosis in cardiac sections of CKD animals compared to the sham controls. Parallel to these changes, we found that although cardiac EPO levels were similar in both groups, the expression of EPO-R and the activated form of its downstream protein STAT-5 were significantly lower in CKD animals.

Conclusions: CKD results in molecular changes in the EPO/EPO-R axis. These changes may play a role in early cardiac damage observed in the cardiorenal syndrome.

 

Background: Children dependent on gastrostomy tube feeding and those with extremely selective eating comprise the most challenging groups of early childhood eating disorders. We established, for the first time in Israel, a 3 week intensive weaning and treatment program for these patients based on the "Graz model."

Objectives: To investigate the Graz model for tube weaning and for treating severe selective eating disorders in one center in Israel. 

Methods: Pre-program assessment of patients’ suitability to participate was performed 3 months prior to the study, and a treatment goal was set for each patient. The program included a multidisciplinary outpatient or inpatient 3 week treatment course. The major outcome measures were achievement of the target goal of complete or partial tube weaning for those with tube dependency, and expansion of the child's nutritional diversity for those with selective eating. 

Results: Thirty-four children, 28 with tube dependency and 6 with selective eating, participated in four programs conducted over 24 months. Their mean age was 4.3 ± 0.37 years. Of all patients, 29 (85%) achieved the target goal (24 who were tube-dependent and 5 selective eaters). One patient was excluded due to aspiration pneumonia. After 6 months follow-up, 24 of 26 available patients (92%) maintained their target or improved. 

Conclusions: This intensive 3 week program was highly effective in weaning children with gastrostomy tube dependency and ameliorating severe selective eating. Preliminary evaluation of the family is necessary for completion of the program and achieving the child’s personal goal, as are an experienced multidisciplinary team and the appropriate hospital setup, i.e., inpatient or outpatient. 

 

Background: The incidence of post-infectious glomerulonephritis (PIGN) has decreased over the last decades. As a result, recent epidemiological data from industrialized countries are scarce. 

Objectives: To evaluate patterns of PIGN in children and detect possible predictors of disease severity.

Methods: We collected clinical and laboratory data of patients with PIGN admitted to Schneider Children's Medical Center during 1994–2011. Diagnostic criteria included presence of hematuria with/without other features of nephritic syndrome along with hypocomplementemia and/or microbiological/serological evidence of streptococcal infection. Patients with other diseases (systemic lupus erythematosus, vasculitis, etc.) were excluded from the study. 

Results: A total of 125 patients with a mean age of 5.8 ± 3.3 years (range 1.5–17.6), of whom 16% were < 3 years, matched the study criteria. Presenting features included hypertension in 103 (82.4%) patients, azotemia in 87 (70.2%), fever in 49 (40%), and elevated C-reactive protein in 75 (81.5%). Isolated macrohematuria was found in 21 (16%). Full-blown nephritic syndrome was diagnosed in 51 patients (41.1%) and 28 (22.9%) had nephritic syndrome with nephrotic-range proteinuria. Depressed C3 complement levels were associated with the presence of nephritic syndrome (OR 0.73, 95%CI 0.60–0.88, P = 0.001) as well as older age (OR1.24, CI 1.08–1.43, P = 0.001). At last follow-up (mean 42 months) all examined patients (100 of 125) had normal renal function, 6 had hypertension, and 1 had proteinuria.

Conclusions: PIGN remains an important cause of glomerular disease in children and may affect very young patients. Nephrotic-range proteinuria with hypoalbuminemia seems to be more frequent than previously reported. Hypocomplementemia is associated with a more severe disease course, namely, azotemia and nephritic syndrome. 

 

Background: Neurolathyrism is a toxic nutritional disorder caused by consumption of the grass pea, Lathyrus sativus. The disease, which manifests as an acute or insidiously evolving spastic paraparesis, continues to occur throughout Africa and Asia. Research on this disease is limited, and to our knowledge no imaging studies of patients with neurolathyrism have been published. 

Objectives: To better localize the site of damage in neurolathyrism using advanced imaging methods. 

Methods: Three male patients, immigrants from Ethiopia, were included in the study. All had a history of arrested spastic paraparesis that had evolved before their emigration from Ethiopia, and a past history of exposure to grass pea without any other cause. Functional magnetic resonance imaging (fMRI) included simple motor tasks to evaluate cortical motor areas. Anatomic scans included diffusion tensor imaging (DTI) to evaluate the corticospinal tracts.

Results: In all patients clear activation was found in motor regions and the patients’ activity pattern was qualitatively similar to that in control subjects. In one patient in whom clinical symptoms were asymmetric, an asymmetric activity pattern in M1 was identified. DTI analysis identified intact corticospinal tracts connecting the pons and the primary motor regions, similar to control subjects. 

Conclusions: Advanced neuroimaging clearly identified well-functioning motor regions and tracts in neurolathyrism patients, suggesting a spinal etiology.

 

Background: Prophylaxis for hospitalized venous-thromboembolic events (VTEs) is frequently underutilized, in part due to lack of a simple risk assessment model (RAM). 

Objectives: To compare patient selection and administration of VTE prophylaxis according to the American College of Chest Physicians (ACCP) 2008 guidelines versus the newer 2012 guidelines, and assess the feasibility of developing simpler local RAMs.

Methods: We conducted a prospective assessment of VTE risk among 300 unselected consecutive patients admitted to a medical hospital ward, using the 2008 and 2012 ACCP guidelines. The frequency and relative weight of each risk factor in the 2012 ACCP guidelines were used to develop a local VTE RAM.

Results: VTE prophylaxis was indicated by the 2008 and 2012 ACCP guidelines in 40% and 42% of the cohort respectively, and was administered in 28% and 26% of eligible patients, respectively. Contraindication to VTE prophylaxis was found in 29% of patients according to both guidelines. In comparison to the 2008 guidelines, sensitivity and specificity of the 2012 guidelines were 96% and 88%, respectively. A local RAM based on the following concise score, comprising age, malignancy and immobility, correctly identified 99% of at-risk patients based on the 2012 guidelines, with a sensitivity and specificity of 98% and 95%, respectively.

Conclusions: Both guidelines performed to a similar degree and were poorly implemented in daily practice. A simplified RAM accurately identified the vast majority of these eligible patients. The development of local RAMs is feasible and may result in higher utilization rates.

 

Background: Fine-needle aspiration cytology (FNAC) is used to provide rapid diagnostic information regarding masses of the head and neck. To achieve good results, adequate training is essential.

Objectives: To evaluate the efficacy of FNAC in the diagnosis of head and neck masses performed by residents and attending physicians.

Methods: Palpable guided FNA biopsies from 166 consecutive patients with head and neck masses, excluding thyroid, who were treated in our department between 2008 and 2010 were retrospectively reviewed. Accuracy, sensitivity, specificity, and positive and negative predictive values were calculated.

Results: A total of 193 FNACs were performed in 161 patients (5 patients were excluded due to age under 18). Mean age was 57.3 years; female to male ratio was approximately 5:4. Most FNACs were performed in masses in the parotid gland (37.3%), 14.5% in the posterior neck, 19.1% in the lateral neck, 15% at level 1, and 9.3% at level 6. The median size of the masses aspirated was 2 cm. Most FNACs were performed by an experienced physician (2.5:1). About 25% of the patients required a second FNAC. Almost 70% of FNACs were diagnostic. Of these, 71.2% were of benign processes and 28.8% of malignancies.

Conclusions: An FNAC of a palpable mass in all sites of the neck, excluding the thyroid, can be done as an office procedure with reasonable results without imaging guidance. About 25% of patients will require another biopsy. The procedure is not difficult to master, as evident by the fact that there were no differences in the results of FNACs performed by an attending otolaryngologist or a resident.

 

The progression from standard celluloid films to digitalized technology led to the development of new software programs to fulfill the needs of preoperative planning. We describe here preoperative digitalized programs and the variety of conditions for which those programs can be used to facilitate preparation for surgery. A PubMed search using the keywords “digitalized software programs”, “preoperative planning” and “total joint arthroplasty” was performed for all studies regarding preoperative planning of orthopedic procedures that were published from 1989 to 2014 in English. Digitalized software programs are enabled to import and export all picture archiving communication system (PACS) files (i.e., X-rays, computerized tomograms, magnetic resonance images) from either the local working station or from any remote PACS. Two-dimension (2D) and 3D CT scans were found to be reliable tools with a high preoperative predicting accuracy for implants.  The short learning curve, user-friendly features, accurate prediction of implant size, decreased implant stocks and low-cost maintenance makes digitalized software programs an attractive tool in preoperative planning of total joint replacement, fracture fixation, limb deformity repair and pediatric skeletal disorders.

The European Union defines rare diseases (RDs) as life-threatening or chronically debilitating conditions whose prevalence is less than 5 per 10,000. Moreover, for many RDs, including those of genetic origin, combined efforts are required to reduce morbidity or perinatal or early mortality, and address the considerable decline in an individual's quality of life and socioeconomic potential. Their specificities, i.e., a limited number of patients and scarcity of relevant knowledge and expertise, make RDs a unique condition which requires wide cooperation at a supranational level. Many steps were therefore taken to develop a network of European Reference Centers and to improve RDs coding and classification. In Italy, the RDs issue was addressed in 2001 with the development of a national network and a national registry coordinated by the National Center for RDs of the Italian National Institute of Health. Registries are an important resource for the development of appropriate public health policies and research on specific RDs. Research on RDs is essential for the development of novel therapeutic approaches and requires the involvement of scientific societies and patient organizations. Nevertheless, the management of patients with chronic RDs requires a qualified care network. The network for RDs of Piedmont and Aosta Valley (North-West Italy) represents an example of health care organization based on the availability of advanced therapies close to the patient’s home.