Israel Medical Association Journal

Background: Severe hearing impairment can have devastating effects on social integration and vocational opportunities.

Objectives: To investigate how well – or poorly – individuals who underwent cochlear implantation as children integrated into the general Israeli hearing community.

Method: We sent a questionnaire to the 30 subjects ≥ 18 years old who underwent cochlear implants our department from 1990 to 2004 when they were < 18 years of age and had used their device for at least 3 years before replying.

Results: Eighteen implant users responded (14 males), yielding a 60% response rate. Their mean age was 13.3 ± 7.0 years (range 6–17) at implantation and 21.1 ± 3.6 years (range 18–34) when they filled in the questionnaire. Five were attending rabbinical school (yeshiva students), four were in regular military service, five were university students (three also held jobs), two were attending high school, one was employed (and had a university degree), and one had left the yeshiva and was unemployed when he returned the questionnaire. Fourteen respondents use the oral communication mode for conversation and the other 4 use both oral and sign languages. Longer daily implant use was significantly associated with coping with the difficulties in the setting in which they were currently active, with a higher level of satisfaction with their current lifestyle and with recognition of the implant’s contribution to this satisfaction (P = 0.037, P = 0.019 and P = 0.001, respectively).

Conclusions: Advances in cochlear implant technologies enable profoundly deaf implanted children to integrate well into the Israeli hearing society, albeit with a large inter-subject variability.

Background: Rectal intussusception, rectocele and rectal prolapse are anatomic disorders in obstructed defecation syndrome. A relatively new surgical approach, Stapled Transanal Rectal Resection, was designed to treat these anomalies.

Objectives: To present our preliminary results with this technique.

Methods: Thirty patients with ODS[1] not responding to medical treatment or biofeedback were operated on with the STARR[2] technique. All the patients underwent a complete workup in the Pelvic Floor Unit. The operation was performed according to the technique described elsewhere.

Results: The patients' mean age was 67.1 years, and the median duration of symptoms was 7 years. The mean operating time was 40 minutes (range 35–80 min) and the mean hospital stay was 2 days (range 1–4 days). The mean follow-up was 26 months (range 6–48 months). ODS symptoms were ameliorated in 27 patients (90%), decreased significantly in 18, and in 9 patients the symptoms disappeared. The procedure failed in 3 patients (10%). Complications included minor bleeding that required homeostasis in eight patients during the operation. Three patients had transient tenesmus and five patients had anal pain. There were no cases of mortality or pelvic sepsis.

Conclusions: STARR is an effective and safe procedure for the treatment of obstructed defecation syndrome due to rectal intussusception, rectocele and small rectal prolapse.

Background: Vitamin A and its derivative retinoic acid regulate various aspects of cell behavior as growth, differentiation, and proliferation. Retinoic acid derivatives have been suggested to play a role in processes such as hepatic regeneration and fibrosis.

Objectives: To evaluate the influence of vitamin A on rat liver epithelial cell proliferation.

Methods: We performed common bile duct ligation in rats that had been subjected to differing vitamin A diets and compared their livers to control rats. Proliferation, apoptosis, and retinoic acid receptors were evaluated by histology and immunohistochemistry in bile duct cells and hepatocytes.

Results: Vitamin A deficiency was found to be associated with enhanced proliferation of bile duct epithelial cells following CBD[1] ligation. The proliferation was manifested by increased numbers of ducts, by aberrant extended ductal morphology, and by elevated numbers of nuclei expressing the proliferation marker Ki67. The amount of vitamin A in the rat diet did not affect detectably ductal cell apoptosis. We observed up-regulated expression of the retinoid X receptor-alpha in the biliary epithelium of vitamin A-deficient rats that had undergone CBD ligation, but not in vitamin A-sufficient rats.

Conclusions: We speculate that the mechanism underlying the ductal proliferation response involves differential expression of RXR[2]-alpha. Our observations suggest that deficiency of vitamin A may exacerbate cholestasis, due to excessive intrahepatic bile duct proliferation.

Background: There are several treatment options for simple bone cysts, with treatment depending mainly on the experience and preference of the surgeon and the extension and location of the cyst.

Objectives: To assess our experience with the surgical treatment of bone cyst lesions in pediatric patients at one institution by the same group of surgeons.

Methods: The study group comprised 60 patients (43 boys, 17 girls) treated surgically for monostatic lesions between January 2002 and July 2007. The mean age at surgery was 11.8 years (range 4–17 years). Mean follow-up was 4.2 years. Most of the lesions were located at the proximal humerus. Patients were divided into five groups according to treatment method: a) corticosteroids (methylprednisolone 40-80 mg) (n=26); b) curettage and bone grafting (fibula or iliac crest) (n=16); c) aspiration of the bone cavity and subsequent bone marrow transplantation (n=10); d) internal preventive fixation using an elastic stable intramedullary nail (n=5); and e) curettage and implantation of a synthetic cancellous bone substitute (pure beta-tricalcium phosphate substitute, ChronOS®, Synthes, Switzerland) (n=3).

Results: Treatment success was evaluated by the Capanna criteria. Successful results were observed in 68% (18 complete healing, 23 healing with residual radiolucent areas), 30% recurrence rate, and no response to treatment in one patient (2%). We recorded recurrence in 50% of the children treated by corticosteroid injection, and one child did not respond to treatment.

Conclusions: The best results were achieved in children treated by curettage and the subsequent use of an osteoconductive material, and in children treated with elastic intramedullary nail fixation. Despite our limited experience with calcium-triphosphate bone substitute, the treatment was mostly successful. Because of the short follow-up, further observation and evaluation are necessary.

Background: Autoimmune neutropenia of infancy is caused by neutrophil-specific autoantibodies. Primary AIN[1] is characterized by neutrophil count < 500 ml and a benign self-limiting course. Detecting specific antibodies against the polymorphic human neutrophil antigen usually confirms the diagnosis. Current available tests, however, are expensive and inapplicable in many laboratories as they require the use of isolated and fixed granulocytes obtained from donors pretyped for their distinct HNA[2] alloform.

Objectives: To assess the performance of a modified test to identify by FACS-analysis granulocyte-specific antibodies in the sera of neutropenic children.

Methods: We evaluated 120 children with a clinical suspicion of AIN, whose sera were analyzed by flow cytometry for the presence of autoantibodies using the indirect granulocyte immunofluorescence test. In contrast to the traditional tests, the sera were tested against randomly selected untyped neutrophils derived from a batch of 10 anonymous healthy subjects, presumably including the common HNA alloforms. Control sera samples were from patients with chemotherapy-induced, familial or congenital neutropenias. To further assure the quality of the new test, we retested six samples previously tested by the gold standard method. All medical files were screened and clinical outcomes were recorded.

Results: Our method showed specificity of 85%, sensitivity of 62.5%, and a positive predictive value of 91.8%, values quite similar to those obtained by more traditional methods.

Conclusions: The new method showed high specificity for detection of anti-neutrophil antibodies in the appropriate clinical setting and could be an effective aiding tool for clinical decision making.

Background: Diabetes mellitus is a multi-organ disorder affecting many types of connective tissues, including bone and cartilage. Certain skeletal changes are more prevalent in diabetic patients than in non-diabetic individuals. A possible association of diabetes mellitus and lumbar spinal stenosis has been raised.

Objectives: To compare the prevalence of diabetes mellitus in patients with spinal stenosis, degenerative disk disease or osteoporotic vertebral fractures.

Methods: A cross-sectional analysis was performed of 395 consecutive patients diagnosed with spinal stenosis, degenerative disk disease or osteoporotic vertebral fractures. All the patients were examined by one senior author in the outpatient orthopedic clinic of a large general hospital between June 2004 and January 2006 and diagnosed as having either lumbar spinal stenosis (n=225), degenerative disk disease (n=124) or osteoporotic vertebral fractures (n=46).

Results: The prevalence of diabetes mellitus in the three groups (spinal stenosis, osteoporotic fracture, degenerative disk disease) was 28%, 6.5% and 12.1%, respectively, revealing a significantly higher prevalence in the spinal stenosis group compared with the others (P = 0.001). The higher prevalence of diabetes in the stenotic patients was unrelated to the presence of degenerative spondylolisthesis.

Conclusions: There is an association between diabetes and lumbar spinal stenosis. Diabetes mellitus may be a predisposing factor for the development of lumbar spinal stenosis.