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Biomedical subjects

S Wientroub

Publications and source records attributed to S Wientroub.

At least 37 records · Page 2Linked to original sources

Functional results at the end of skeletal growth in 30 patients affected by congenital pseudoarthrosis of the tibia.

From a multicentric study on Congenital Pseudoarthrosis of the tibia (CPT) conducted on 340 patients, we evaluated the functional results on a group of thirty patients who were at the end of skeletal growth (age < or = 16 years). The prognosis of CPT is very much related to the radiologic classification. Crawford type 2 and Crawford type 4 pseudoarthrosis have a worse prognosis, with a lower percentage of fusion at the site of pseudoarthrosis. Crawford 4 patients have the worst functional results. Most of them showed a severe leg length discrepancy, needed a permanent brace, with an ankle joint function fair or poor. The presence of fibula pseudoarthrosis seems to be responsible for most of the worst functional results. The level of deformity caused by either the natural course of the disease or its treatment, is decisive in the evaluation of the functional results.

Adolescent↗

Musculoskeletal problems in Down Syndrome European Paediatric Orthopaedic Society Survey: the Israeli sample.

An Israeli sample of 475 individuals representing the full spectrum of those with Down syndrome in the Jewish population is presented. The sample is part of a European multicenter study evaluating musculoskeletal disorders. Generalized joint laxity was found to have significant relationships with a number of medical conditions (knee problems, patello-femoral instability, genu valgus, pes planus, heart defects, and gastrointestinal problems). Body mass index was also related to feet problems, vertical talus, spinal problems, genu valgum, pes planus, and heart problems. Yearly multidisciplinary clinical follow-up is recommended in persons with Down syndrome.

Adolescent↗

Tibialis anterior tendon transfer for residual dynamic supination deformity in treated club feet.

Twenty-seven previously treated club feet in 25 patients were evaluated retrospectively following tibialis anterior tendon transfer to the dorsum of the foot to correct residual dynamic supination deformity. In 11 feet, the transfer was combined with additional soft tissue and or bony procedures to treat other accompanying deformities. Electrophysiologically demonstrated peroneal weakness causing muscle imbalance contributed to the etiology of this dynamic deformity. At follow-up, none of the patients had dynamic supination deformity during ambulation. All showed active contraction of the transferred tibialis anterior tendon. There was no case of overcorrection. Functional and cosmetic results were assessed by parents and surgeons as being excellent. Tibialis anterior tendon transfer is recommended to correct residual dynamic supination deformity and to restore muscle balance after satisfactory correction of idiopathic club foot contractures.

Child↗

Serrated W/M osteotomy. Results using a new technique for the correction of infantile tibia vara.

The conventional osteotomies used to treat infantile tibia vara (Blount's disease) may require internal fixation and its subsequent removal. These techniques, which carry the risk of traction injury, and potential problems of stability and consolidation, do not always succeed in correcting the rotational deformity which accompanies the angular deformity. We have used a new surgical approach, the serrated W/M osteotomy of the proximal tibia, to correct infantile tibia vara in 15 knees of 11 patients. We present the results in 13 knees of nine patients who have been followed up for a mean of eight years. The mean angular correction achieved after operation was 18 +/- 5.8 degrees. The mean femorotibial shaft angle was corrected from 14.2 +/- 3.7 degrees of varus to 4.6 +/- 4.4 degrees of valgus. At the last follow-up, the mean angular correction had reduced to 1.3 +/- 4.9 degrees of valgus without compromising the rotational correction and the overall good clinical results. All the patients and parents were satisfied, rating the result as excellent or good. There were no major postoperative complications and no reoperations. Eight patients were free from pain and able to perform physical activities suitable for their age. One complained of occasional pain. This procedure has the advantage of allowing both angular and rotational correction with a high degree of success without the need for internal fixation.

Activities of Daily Living↗

Mutations of the GNAS1 gene, stromal cell dysfunction, and osteomalacic changes in non-McCune-Albright fibrous dysplasia of bone.

Activating missense mutations of the GNAS1 gene, encoding the alpha subunit of the stimulatory G protein (Gs), have been identified in patients with the McCune-Albright syndrome (MAS; characterized by polyostotic fibrous dysplasia, café au lait skin pigmentation, and endocrine disorders). Because fibrous dysplasia (FD) of bone also commonly occurs outside of the context of typical MAS, we asked whether the same mutations could be identified routinely in non-MAS FD lesions. We analyzed a series of 8 randomly obtained, consecutive cases of non-MAS FD and identified R201 mutations in the GNAS1 gene in all of them by sequencing cDNA generated by amplification of genomic DNA using a standard primer set and by using a novel, highly sensitive method that uses a protein nucleic acid (PNA) primer to block amplification of the normal allele. Histologic findings were not distinguishable from those observed in MAS-related FD and included subtle changes in cell shape and collagen texture putatively ascribed to excess endogenous cyclic adenosine monophosphate (cAMP). Osteomalacic changes (unmineralized osteoid) were prominent in lesional FD bone. In an in vivo transplantation assay, stromal cells isolated from FD failed to recapitulate a normal ossicle; instead, they generated a miniature replica of fibrous dysplasia. These data provide evidence that occurrence of GNAS1 mutations, previously noted in individual cases of FD, is a common and perhaps constant finding in non-MAS FD. These findings support the view that FD, MAS, and nonskeletal isolated endocrine lesions associated with GNAS1 mutations represent a spectrum of phenotypic expressions (likely reflecting different patterns of somatic mosaicism) of the same basic disorder. We conclude that mechanisms underlying the development of the FD lesions, and hopefully mechanism-targeted therapeutic approaches to be developed, must also be the same in MAS and non-MAS FD.

Adolescent↗

[Ilizarov reconstructive surgery--a solution for complex problems of the musculoskeletal system].

The Ilizarov technique is an important modality of reconstructive surgery for limb deformities, such as malunion and nonunion of fractures, persistent osteomyelitis, and bone loss following complex limb injuries, as well as in limb-lengthening procedures. It has received wide recognition in the Western world over the past decade. In MEDLINE we found 537 articles published between 1971-1995 that describe the use of this technique. Of these, only 18 were published between 1971-1975, while 261 were published between 1991-1995. The present paper describes the developments and updates in this method, as a result of the large experience gained by the authors in a number of centers in Israel using this technique. The Ilizarov apparatus is a circular frame that allows accurate control, much more than any other external fixator, during correction of limb deformities and limb-lengthening. It is minimally invasive, and open techniques and use of internal hardware and bone grafts are not needed. Based on our experience, we suggest that this method requires careful preoperative planning and meticulous surgical technique. More important, cooperation between surgeon and patient throughout the lengthy treatment is a prerequisite to ensure complete success, even in the most complicated cases.

Bone Diseases↗

Does thrombophilia play an aetiological role in Legg-Calvé-Perthes disease?

Heritable thrombophilic disorders have been proposed as one of the causes for Legg-Calvé-Perthes disease. A total of 62 patients diagnosed with this disease between 1988 and 1997 and 50 controls were screened for thrombophilia. The incidence and relationship of thrombophilia to the severity of the disease were evaluated. One patient and none of the controls had protein S deficiency. One of the control group and one of the patients had protein C deficiency with the latter child also having a combined deficiency with a mutant factor V gene. The number of children with a mutant factor V gene, protein C deficiency, who were homozygous for the C 677T polymorphism of methylenetetrahydrofolate reductase or were heterozygous for mutant G20210A prothrombin did not differ statistically in the study and the control groups. No patient had antithrombin deficiency or positive lupus anticoagulant. We found no correlation between thrombophilia and the extent of the disease. The most common risk factors for arteriovenous thromboembolism showed no statistical significance in our patients compared with the control group or with the general population. These data do not confirm an aetiological role for thrombophilia in Perthes' disease.

Child↗

Calcaneal osteochondritis: a new overuse injury.

This is a case report of osteochondritis of the medial plantar apophysis of the calcaneus presenting as medial plantar heel pain in a 15-year-old basketball player. The lesion was detected radiographically and by increased focal uptake on bone scan. Conservative treatment resulted in complete pain relief and normal calcaneal appearance with union of the osteochondral fragment. No recurrence was noted during 3 years of follow-up.

Adolescent↗

Cat-scratch disease osteomyelitis from a dog scratch.

Osteomyelitis is a rare manifestation of cat-scratch disease in patients who do not have AIDS. The clinical presentation and non-specific subacute course of the disease make diagnosis difficult. We present a child with osteomyelitis of a metacarpal following a dog scratch. Bartonella henselae was found to be the aetiological agent. The bone healed after treatment with antibiotics. Increased awareness and a comprehensive medical history are needed to identify patients with suspected Bartonella henselae osteomyelitis.

Animals↗

Simple bone cyst: etiology, classification, pathology, and treatment modalities.

Simple bone cyst (SBC) is an enigma to the radiologist, pathologist and the orthopaedic surgeon. The etiology of this asymptomatic lesion that frequently causes pathological fracture is still unknown. It is probably self limited in nature, seen in children but rare among adults. The biological behavior is unpredictable as is the clinical course in various anatomical sites. This reflects on the high recurrence rate that has been associated with various treatment modalities. The clinical, radiological and biological features are discussed together with comparative review of treatment options from resection, curettage, and bone grafting to steroid injection and the latest experience of the use of percutaneous autologous marrow grafting in SBC.

Adolescent↗

Osteocalcin (BGP), gene expression, and protein production by marrow stromal adipocytes.

This study was designed to demonstrate the expression and production of osteocalcin, a bone Gla-protein (BGP), by marrow stromal cells. We were able to accomplish this by using a series of marrow stromal cell lines (MBA cells). A unique expression of the osteocalcin was detected by the adipocyte 14F1.1 cells. This was at the mRNA level by Northern blot and by RT-PCR analysis. The secreted protein was quantitated by radioimmunoassay (RIA), in conditioned medium (CM) harvested from these cultured cells. These findings offer the first evidence that marrow adipocyte 14F1.1 derived cells express mRNA for osteocalcin and produce the protein.

Adipocytes↗

Primary subacute haematogenous osteomyelitis of the tarsal bones in children.

Primary subacute haematogenous osteomyelitis (PSHO) of the small bones of the foot is a rare and infrequently considered cause of a limp in children. We describe 11 patients with PSHO, of whom nine were under three years of age, who had a limp with few symptoms. The talus was involved in 36%. Bone scans were positive in all patients and led to localisation of the lesion in two. The radiological features included soft-tissue swelling, an osteolytic lesion in the talus and the calcaneus and a sclerotic appearance of the cuboid and the navicular bones. All patients except one were cured with antibiotics.

Acute Disease↗

Phenotypic expression of marrow cells when grown on various substrata.

Our aim was to study the role of various extracellular matrices (ECM) on growth and differentiation of marrow stromal cells in vitro. Morphology changes, gene expression, and enzymatic activities were monitored in stromal osteoblastic MBA-15 and adipocytic 14F1.1 cells. These stromal cells were plated on dishes precoated with different substrata, such as matrigel (basement membrane), collagen type I, and endothelial ECM, and compared with cells plated on protein-free dishes. Striking morphological differences were observed when the cells grew on these different substrata. Changes in cell shape and growth also led to differential mRNA expression and enzymatic activities. When MBA-15 cells were plated on collagen, there was a decrease in mRNA for alkaline phosphatase (ALK-P), osteopontin (OP), and osteonectin (ON), and an increase in mRNA for procollagen (I). A differential effect was noted on 14F1.1 cells, the mRNA for ALK-P increased, the expressions of OP and ON lowered, and no expression for procollagen (I) was monitored. MBA-15 cells cultured on matrigel had decreased mRNA for ALK-P and OP, while they had increased ON mRNA expression and remained unchanged for procollagen I. No change in mRNA expression by 14F1.1 cells was monitored when cultured on matrigel. Functional enzymatic activities of ALK-P markedly decreased in MBA-15 cells cultured on various substrata, and increased or were unchanged in 14F1.1 cells. An additional enzyme, neutral endopeptidase (CD10/NEP), altered differentially in both cell types; this enzymatic activity increased or was unchanged when cells were cultured on these matrices. The results indicate a specific role for different ECM on various stromal cell types and their function.

Adipocytes↗

Simple bone cysts treated by percutaneous autologous marrow grafting. A preliminary report.

We prospectively evaluated the percutaneous injection of autogenous bone marrow for the treatment of active simple bone cysts in ten consecutive children with cysts in the proximal humerus, proximal femur or tibia. The treatment included percutaneous biopsy, aspiration of fluid and the injection of autogenous bone marrow aspirated from the iliac crest. All the patients became painfree after a mean of two weeks and resumed full activities within six weeks. All ten cysts consolidated radiologically and showed remarkable remodelling within four months. Review at 12 to 48 months showed satisfactory healing without complications. Percutaneous injection of autologous bone marrow appears to be an effective treatment for active simple bone cysts.

Adolescent↗