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Does pamidronate enhance the osteogenesis in mesenchymal stem cells derived from fibrous hamartoma in congenital pseudarthrosis of the tibia?

ABSTRACT: Neurofibromatosis type 1 (NF1) is a commonly occurring genetic disorder in children. Mutation in the NF1 gene has its implication in poor osteoblastic capabilities. We hypothesised that pamidronate will enhance the osteoblastic potential of the mesenchymal stem cells (MSCs) derived from lipofibromatosis tissue of children with congenital pseudarthrosis tibia (CPT) associated with NF1. In this study, bone marrow MSCs (BM MSCs) and CPT MSCs were obtained from three patients undergoing salvage surgeries/bone grafting (healthy controls) and those undergoing excision of the hamartoma and corrective surgeries respectively. The effects of pamidronate (0, 10 nM, 100 nM and 1 ?M) on cell proliferation, toxicity and differentiation potential were assessed and the outcome was measured by staining and gene expression. Our outcome showed that CPT MSCs had more proliferation rate as compared to BM MSCs. All 3 doses of pamidronate did not cause any toxicity to the cells in both the groups. The CPT MSCs showed less differentiation with pamidronate compared to the healthy control MSCs. This was quantitated by staining and gene expression analysis. Therefore, supplementation with pamidronate alone will not aid in bone formation in patients diagnosed with CPT. An additional stimulus is required to enhance bone formation.

SUBMITTER: Madhuri V

PROVIDER: S-EPMC5440779 | biostudies-other | 2016 Dec

REPOSITORIES: biostudies-other

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Does pamidronate enhance the osteogenesis in mesenchymal stem cells derived from fibrous hamartoma in congenital pseudarthrosis of the tibia?

Madhuri Vrisha V Mathew Smitha Elizabeth SE Rajagopal Karthikeyan K Ramesh Sowmya S Antonisamy B B

Bone reports 20161014

Neurofibromatosis type 1 (NF1) is a commonly occurring genetic disorder in children. Mutation in the NF1 gene has its implication in poor osteoblastic capabilities. We hypothesised that pamidronate will enhance the osteoblastic potential of the mesenchymal stem cells (MSCs) derived from lipofibromatosis tissue of children with congenital pseudarthrosis tibia (CPT) associated with NF1. In this study, bone marrow MSCs (BM MSCs) and CPT MSCs were obtained from three patients undergoing salvage surg ...[more]

PMID: 28580399

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Project description:PurposeThe goal of this study is to evaluate the treatment outcomes of anterolateral bowing and residual deformities of distal tibia in patients with CPT using circular external fixation and hydroxyapatite coated flexible intramedullary nailing without excision of affected part of tibia.Patients and methodsSix patients (4 boys and 2 girls, mean age 12.4 ± 4.1 years) were included in the study. Mean follow-up is 2.1 years. In 4 patients with early onset of disease initial surgical treatment (at age of 5-8 years) was dysplastic zone or pseudarthrosis resection with proximal metaphyseal osteotomy for bone transport. Children with unbroken bowed tibia (2 cases of type II according to Crawford classification) had no previous surgery. Neurofibromatosis type I was diagnosed in 4 cases. Surgical technique for residual deformity correction consisted of percutaneous osteotomy, application of circular external frame and composite hydroxyapatite-coated intramedullary nailing.ResultsMean external fixation time was 95.3 ± 17.5 days. All patients never get fractured after frame removal. At the present time, they are considered to be healed, in 2.1 years, in average, without fractures or deformity recurrence. Mean lower limb length discrepancy varied from 2 to 10 mm at the latest follow-up control. After realignment procedure, patients didn't require additional surgery but one. Intramedullary nails were removed in two years after deformity correction for individual reason.ConclusionCorrection of anterolateral bowing or residual deformity in children with CPT is indicated. Association of external fixation with intramedullary nailing/rodding left in situ after frame removal ensure stability and accuracy of deformity correction. Biological methods of stimulation of bone formation in dysplastic zone are obligatory to ensure bone union. Intramedullary nailing with composite hydroxyapatite-coated surface provides mechanical and biological advantages in patients with CPT.

Dataset Information

Does pamidronate enhance the osteogenesis in mesenchymal stem cells derived from fibrous hamartoma in congenital pseudarthrosis of the tibia?

Publications

Does pamidronate enhance the osteogenesis in mesenchymal stem cells derived from fibrous hamartoma in congenital pseudarthrosis of the tibia?

Similar Datasets

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