Israel Medical Association Journal

Background: Adenoviral infection in children undergoing stem cell transplantation is associated with significant morbidity and mortality. Identification of adenoviral infection by polymerase chain reaction from blood facilitates accurate and rapid diagnosis and surveillance. The incidence of adenoviral infection among children undergoing SCT[1] in Israel is not known.

Objective: To estimate the incidence of adenoviral infection in pediatric SCT patients and to characterize the morbidity associated with proven infection.

Methods: Blood samples obtained weekly from children who underwent allogeneic SCT were retrospectively tested for adenovirus using standard PCR[2]. A total of 657 samples collected from 32 patients were examined. Correlation was made between the presence of adenovirus in samples and clinical records.

Results: Of the 32 patients 4 had adenoviral infection by PCR (12.5%). Clinical disease was present in all four patients concurrent with positive PCR. Gastrointestinal complaints and abnormal hepatocellular enzymes were uniformly present. One patient died due to disseminated disease. T cell depletion was a significant risk factor for adenoviral infection (P = 0.03).

Conclusions: In the patient population studied, the incidence of adenoviral infection in children undergoing SCT was 12.5%. The combination of gastrointestinal symptoms and abnormal hepatocellular enzymes should raise the suspicion of adenoviral infection, especially when occurring during the first few months after SCT. 

Background: Patients with thick melanomas > 4 mm deep are at great risk for regional and distant metastatic disease. Historically, the appropriate management of thick melanomas has remained unclear and there is no consensus in the literature. Many have taken the nihilistic view that surgical intervention to excise regional nodal basins is not justified in light of the poor overall prognosis and risk of occult distant disease.

Objectives: To review the outcome of patients with thick node negative melanoma treated at a multidisciplinary academic center

Methods: We retrospectively reviewed a database of melanoma patients to identify patients with thick melanomas, > 4 mm, who were either clinically or sentinel node biopsy negative, staged T4N0, stage IIb or IIc. The charts of these patients were reviewed and updated, with a median follow-up of 4 years.

Results: We identified 23 patients who fit these criteria. Of these, 18 (78%) remain alive with a median follow-up of 4 years. Five patients died of metastatic disease. Of the 18 surviving patients, 14 remained with no evidence of disease after initial resection of their primary lesions. The majority of the recurrences were non-nodal.
Conclusions: The overall survival of patients in our study remains above 75%, at median follow-up of 4 years, even with thick initial index tumor depths. Most of the failures were due to hematogenous spread with lymphatic sparing. Tumor biology that may inhibit lymphatic spread could be a target of future investigation

Background: Familial dysautonomia is a genetic disease that affects the sensory and autonomic nervous systems with varying severity. The deep breath test is one of several measures used to assess the severity of autonomic diseases, but its value in familial dysautonomia has not yet been investigated.

Objectives: To determine the diagnostic value of the DBT[1] in patients with familial dysautonomia.

Methods: Eight patients with familial dysautonomia and eight healthy volunteers were examined by electrocardiography for 1 minute at rest and during forced deep breathing. The following values were recorded: maximum expiratory and minimum inspiratory heart rate and the difference between them (ΔE/I), standard deviation of the heart rate values, interbeat intervals, and E/I[2] ratio. Spectral power analysis of heart rate variability was also performed.

Results: The patients with familial dysautonomia showed a lesser change in heart rate in response to the change in breathing pattern than the controls. Mean values in the study group were significantly higher for minimal inspiratory heart rate and significantly lower for ΔE/I, heart rate standard deviation and E/I ratio, indicating a non-flexible heart response and abnormal parasympathetic function. These findings were supported by power spectral analysis.

Conclusions: Patients with familial dysautonomia have a significantly disturbed response to physiological stimuli. The DBT may serve as a reliable means to quantify autonomic dysfunction in this patient population.

Background: Although the comprehensive evaluation of the fetal heart includes echocardiography by an experienced pediatric cardiologist, economic constraints sometimes dictate the need to select patients.

Objectives: To analyze the usefulness of fetal echocardiography in the detection of congenital heart disease according to the referral indication.

Methods: This retrospective survey relates to all 3965 FE studies performed in our center from January 2000 to December 2004. The diagnosed cardiac anomalies were classified as significant and non-significant malformations. All FE[1] studies were done by a single operator (A.L.) at Meir Medical Center, a referral center for a population of about 400,000. The 3965 FE studies were performed for the following indications: abnormal obstetric ultrasound scans, maternal and family history of cardiac malformations, medication use during the pregnancy, and maternal request. The relative risk of detecting CHD[2] was calculated according to the various referral indications.

Results: Overall, 228 (5.8%) cases of CHD were found. The most common indication for referral was suspicion of CHD during a four-chamber view scan in a basic system survey or during a level II ultrasound survey. No correlation was found between maternal age and gestational age at the time of scanning and the likelihood of finding CHD.

Conclusions: Our data suggest that a suspicious level-II ultrasound or the presence of polyhydramnios is an important indication for FE in the detection of significant CHD.

Background: Gunshot wounds impose a continuous burden on community and hospital resources. Gunshot injuries to the extremities might involve complex soft tissue, bone, vascular, musculotendinous, and nerve injuries. A precise knowledge of anatomy is needed to evaluate and treat those injuries.

Objectives: To review our experience with gunshot wounds to the extremities.

Methods: We retrospectively reviewed all cases of gunshot wounds to the limbs in a civilian setting treated in our institution during 2003–2005. Altogether, we evaluated 60 patients with 77 injuries.

Results: Of the 60 patients 36 had fractures, 75% of them in the lower extremity and 81% in long bones. The most common fixation modality used was external fixation (33%), followed by intramedullary nailing (25%). This relatively high percentage of fracture treated with external fixation may be attributed to the comminuted pattern of the fractures, the general status of the patient, or the local soft tissue problems encountered in gunshot wounds. About one-fifth of the fractures were treated by debridement only without hardware fixation. We treated 10 vascular injuries in 8 patients; 6 of them were injuries to the popliteal vessels. Fractures around the knee comprised the highest risk factor for vascular injuries, since 5 of the 12 fractures around the knee were associated with vascular injury requiring repair or reconstruction. There were 13 nerve injuries (16.8%), most of them of the deep peroneal nerve (38%). Only three patients had concomitant nerve and vascular injuries. The overall direct complication rate in our series was 20%.

Conclusions: Treating complex gunshot injuries requires a team approach, necessary for a favorable outcome. This team should be led by an orthopedic surgeon knowledgeable in the functional anatomy of the limbs.
 

Background: Double balloon enteroscopy is a new technique that enables deep intubation of the endoscope into the small bowel lumen. Through a channel in the endoscope, invasive procedures such as biopsy, polypectomy and hemostasis can be performed, avoiding the need for surgery.

Objectives: To prospectively analyze our results of the first 124 DBEs[1] performed since February 2007.

Methods: The study group comprised all patients who underwent DBE at the Sheba Medical Center between February 2007 and February 2009. Recorded were the patients' demographic data, comorbidities, indications for the examination, results of previous non-invasive small bowel imaging (computed tomography enterography, capsule endoscopy, etc), investigation time, and results of the procedure including findings, endoscopic interventions, complications and pathological report.

Results: A total of 124 procedures were performed in 109 patients. Of the 124 examinations, 57 (46%) were normal and 67 (54%) showed pathology. The main pathologies detected on DBE were polyps (14%), vascular lesions (17.6%) and inflammation (12%). Endoscopic biopsies and therapeutic interventions were required in 58 examinations (46%). A new diagnosis was established in 15% of patients, diagnosis was confirmed in 29% and excluded or corrected in 12%. One complication was observed: a post-polypectomy syndrome that was treated conservatively.

Conclusions: DBE is a safe procedure and has a high diagnostic and therapeutic yield. Most of the examinations were performed under conscious sedation, and only a minority of patients required deeper sedation. 

Background: Unicompartmental knee replacement has become a surgical alternative for treating isolated medial or lateral osteoarthritis of the knee or avascular necrosis of the femoral condyls.

Objectives: To evaluate the short-term results of the Oxford Phase 3^® unicompartmental knee replacement for unicompartmental knee arthrosis or avascular necrosis of the medial

femoral condyle.

Methods: During the period 2003–2005 a total of 59 patients (59 knees) underwent medial Oxford Phase 3^® unicompartmental knee replacement in our unit. The patients were interviewed and examined, and standing anteroposterior and lateral X-rays were taken. All patients completed the Western Ontario and McMaster Universities Index of Osteoarthritis and the Short Form 12 questionnaire, and the International Knee Society score was evaluated. The data were collected and statistical analysis was performed.

Results: X-rays were performed and scores for the WOMAC[1] and IKS[2] were assessed for 42 patients (31 females, 11 males). At an average of 32 months after surgery, the total WOMAC score was 30. The mean SF[3]12 physical score was 38 and the mean SF12 mental score was 51. The mean IKS score was 166. Ninety-one percent of the patients had active flexion of 120 degrees or more. Of 59 knees 7 were converted to total knee arthroplasty – all of them within the first 2 years of starting the procedure and all of them in relatively young patients.

Conclusions: Despite the higher revision rate to TKR[4] in this study, our findings confirm the short-term results reported in other studies of the Oxford medial unicompartmental knee and our early failure rate could be explained by a performance learning curve. This study confirms that this bone-preserving procedure should be considered in end-stage isolated unicompartmental osteoarthritis or avascular necrosis by surgeons who have the adequate training and experience.

Background: Monitoring the rate of infections in individual centers that treat patients with hematological malignancies is of major importance. However, there are no uniform guidelines for infection surveillance.

Objectives: To describe the epidemiology of bacterial and fungal infections in a single hematology ward and to compare methods for reporting surveillance and infection rates in other centers in Israel.

Methods: We conducted a prospective surveillance of all patients admitted to our hematology ward, applying standard definitions for invasive fungal infections and adapting definitions for non-fungal infections. Incidence rates were calculated using patients, admissions, hospital days and neutropenia days. We performed a search for other reported surveillance studies in Israel.

Results: We detected 79 infectious episodes among 159 patients admitted to the hematology ward during 1 year. Using neutropenia days as the denominator for calculation of incidence discriminated best between patients at high and low risk for infection. The incidence of invasive fungal infections was 7, 10 and 18 per 1000 neutropenia days, among all patients, those with acute leukemia and those with acute leukemia undergoing induction therapy, respectively. Only 10 reports from Israel were identified, 6 of which were prospective. Our data could not be compared to these reports because of the varying definitions and denominators used.

Conclusions: Hematology centers should monitor infection rates and report them in a uniform methodology.
 

Background: West Nile virus, the etiologic agent of West Nile fever, is an emerging mosquito-borne disease. WNV[1] was recognized as a cause of severe human meningo-encephalitis in elderly patients during outbreaks in various parts of the world.

Objectives: To analyze WNV encephalitis therapy and its outcome after prescribing hyperimmune gammaglobulin therapy.

Methods: Eight subjects with WNV encephalitis were treated with supportive therapy and 5 days of IVIG[2] 0.4 g/kg/day containing high WNV antibodies obtained from healthy blood donors.

Results: Patients who were treated with IVIG as soon as possible exhibited an improvement in their symptoms. All subjects presented with high fever, progressive confusion and headaches, nausea and vomining. The Glasgow Coma Screen for six patients ranged between 8 and 13 and all were discharged with a score of 15. The remaining two subjects died during their hospitalization.

Conclusions: In severe WNV infection, where the disease affects the central and/or peripheral nervous system, early intervention with IVIG together with supportive treatment is recommended.

Background: Elastofibroma is a rare type of lesion consisting of elastic fibers within a stroma of collagen and fatty tissue. It is usually located on the lower scapular region attached firmly to the thoracic cage, often causing debilitating pain. Its clinical presentation mimics a soft tissue tumor.

Objectives: To evaluate the diagnosis and treatment results of elastofibroma.

Methods: Clinical and radiographic evaluations were performed in 11 patients with thoracic wall mass. In five of them a biopsy was taken before surgery. All patients were operated and the diagnosis of elastofibroma was confirmed by histology. 

Results: Two patients had a postoperative seroma that resolved spontaneously within a few days. All patients resumed their preoperative activities, including sports.

Conclusions: Considering the slow-growing nature of this tumor and its typical presentation, we believe that when this diagnosis is suspected, investigation does not necessitate staging (as in sarcomas). Also, marginal surgical excision is sufficient. Observation is an acceptable alternative to surgery.