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Biomedical subjects

Ehud Lebel

Publications and source records attributed to Ehud Lebel.

5 recordsLinked to original sources
PubMed2006

Kingella kingae infections in children.

Kingella kingae is a beta-hemolytic gram-negative bacillus. It was first described in the 1960's by EO King and has been reported as a cause of osteo-articular pediatric infections since the early 1980's. We performed a retrospective review of all pediatric cases of invasive K. kingae infection between 1997 and 2002, in order to define the incidence, clinical presentation and outcome of invasive K. kingae infections in a pediatric population. During the study period, a total of 24 pediatric patients with K. kingae infection were identified. There were 15 blood culture isolates of K. kingae, out of a total of 1151 (1.3%) positive blood cultures, and 9 synovial fluid culture isolates out of a total of 76 (11.8%) positive synovial fluids. Fifteen patients had osteo-articular infections and 9 had primary bacteremia without osteo-articular infection. Outcome was favorable in all cases and only in 2 patients with knee joint infection was surgical intervention performed, by means of formal knee arthrotomy. All patients recovered uneventfully, in 7 cases without any intervention and in the others with intravenous or oral antibiotic. In conclusion, invasive K. kingae infection is not uncommon in Israel. It usually has a mild course and thus is not always detected and treated. As K. kingae grows best in blood culture broth, blood and joint fluid should always be inoculated into blood culture bottles in suspected cases. This bacterium is highly sensitive to betalactame antibiotics and infection resolves quickly with antibiotic treatment. Surgical intervention for osteo-articular infection is seldom indicated.

Adolescent↗
PubMed2006

Serum levels of osteoprotegerin and osteoprotegerin polymorphisms in Gaucher disease.

Bone involvement in Gaucher disease causes disability and reduced quality of life; loss of function and pain are important indications for enzyme replacement therapy. The purpose of this study was to ascertain whether osteoprotegerin (OPG), which decreases osteoclast activity, is indicative of incipient bone involvement by comparing OPG serum levels to Gaucher disease severity (SSI) and bone mineral density (BMD), and to correlate bone and disease markers to OPG polymorphisms: OPG1-2(A163G), OPG3-4(T129C) and OPG5-6(C1217T). Of 554 patients, 173 Ashkenazi Jewish patients with non-neuronopathic Gaucher disease were enrolled and 32 healthy Ashkenazi Jews served as controls. Serum OPG levels were detected by enzyme-linked immunosorbent assay and BMD was obtained by dual X-ray absorptiometry. OPG polymorphisms were determined in 63 randomly chosen patients. Serum OPG values for patients were not greater than in controls, but showed a statistically significant trend to increase with age (P = 0.057). No correlation existed between OPG levels and BMD or with genotype or other disease markers. A significant correlation was noted between OPG5-6 genotype and SSI. A significant difference was found between the allele distributions of each OPG polymorphism when compared with Caucasians and Ashkenazi Jews. OPG levels probably do not predict BMD in Gaucher disease and hence are not indicative of osteoporosis in Gaucher disease.

Adolescent↗
PubMed2004

Bone density changes with enzyme therapy for Gaucher disease.

Gaucher disease is the most common lysosomal storage disease. Enzyme replacement therapy engenders improvement in hematological and visceral parameters; however, improvement in bone density (BMD) with treatment has not been confirmed. This study presents follow-up of BMD in the first ten patients in Israel treated with low-dose recombinant enzyme for up to 108 months. BMD at femoral neck and lumbar spine was determined by dual-energy X-ray absorptiometry (DEXA) at the start of the trial, after 3-6 months, after 18-24 months, and at the most recent follow-up. BMD in all patients was very low at onset and never normalized. There was a decrease in BMD in all patients at 3-6 months. Older patients (four women, two men; >30 years of age) showed some improvement in BMD during treatment. Younger patients (four females; 18-23 years of age) did not show a statistically significant improvement. These findings might reflect the failure of patients with Gaucher disease to achieve expected peak bone density at appropriate chronological milestones despite treatment. Nonetheless, the z-scores of the older patients were better than those of the younger patients, implying some catch-up period. Yet, some patients with Gaucher disease evince rapid onset of osteoporosis in early adulthood. Enzyme treatment per se, as well as attendant improved well-being and increased physical activity, may induce amelioration in BMD at this later stage. One may consider adding anti-osteoporosis therapy in young adults to induce earlier "catch up" to peak bone mass, and then enzyme replacement in later adulthood to prevent decrements in bone mass related to Gaucher cell infiltration.

Adolescent↗
PubMed2004

Orthopedic considerations in Gaucher disease since the advent of enzyme replacement therapy.

Gaucher disease, the most prevalent lysosomal storage disorder, is characterized by hepatosplenomegaly, hypersplenism, and rarely, neurological involvement. The most variable symptoms relate to skeletal disease, and both onset and progression are difficult to predict on the basis of genotype. This review describes findings from a large referral clinic (> 500 patients) and from the literature in the decade since the advent of specific enzyme replacement therapy. Such therapy is effective in reducing visceral and hematological involvement, but its greatest advantage as regards the skeleton is prevention of irreversible damage. Avascular necrosis of the joints-particularly the hips but also the knees and shoulders-and pathological fractures of the long bones including the ribs, as well as episodic "crises' of bone pain in children and young adults, are common manifestations. Various imaging modalities should be performed at baseline for life-long monitoring, and then as required because of specific complaints. Surgical interventions such as joint arthroplasties are important adjuvant treatments in this population; presurgical hematological profiling plus antibiotic cover and postoperative pain control are equally critical. Opportunities for orthopedic consultations with senior surgeons are not abused by our patients. These reflect disease-related morbidity, with greater numbers of requests being made by patients requiring enzyme therapy, who by definition have more severe disease characteristics.

Adolescent↗