Literature DB >> 22103942

A regenerative approach for bone repair in congenital pseudarthrosis of the tibia associated or not associated with type 1 neurofibromatosis: correlation between laboratory findings and clinical outcome.

Donatella Granchi1, Valentina Devescovi, Serena Rubina Baglio, Marina Magnani, Onofrio Donzelli, Nicola Baldini.   

Abstract

BACKGROUND AND AIMS: Congenital pseudarthrosis of the tibia (CPT) is a rare orthopedic disease presenting spontaneous fractures that do not heal. The treatment of CPT is characterized by repeated surgical procedures that often fail, with the inevitable outcome of severe disability and amputation. We tested the hypothesis that CPT may benefit from regenerative strategies based on mesenchymal stromal cells (MSC) combined with platelet-rich fibrin (PRF) as a source of growth factors. The aim of the study was to verify whether laboratory testing to assess the osteogenic properties of MSC and the osteo-inductive activity of PRF correlated with the clinical outcome.
METHODS: Ten patients affected by refractory CPT were treated by using MSC derived from the iliac crest (IC-MSC), PRF and lyophilized bone. In six patients, CPT was associated with type 1 neurofibromatosis (NF1). Biochemical, functional and molecular assays were performed to assess the intrinsic osteogenic potential of IC-MSC (cells cultured with fetal calf serum) and the osteo-inductive properties of PRF (cells cultured with autologous serum).
RESULTS: Bone consolidation was obtained in three patients who had CPT and NF1. In these patients, the IC-MSC exposed to autologous serum were able to form mineral nodules in vitro, while the mineralizing ability was totally abrogated in patients with a poor clinical outcome.
CONCLUSIONS: Cell therapy may be a useful tool for the treatment of refractory CPT because it increases the opportunity to achieve effective bone tissue regeneration. Our data suggest that the presence of pro-osteogenic growth factors is an essential requirement for bone healing.

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Year:  2011        PMID: 22103942     DOI: 10.3109/14653249.2011.627916

Source DB:  PubMed          Journal:  Cytotherapy        ISSN: 1465-3249            Impact factor:   5.414


  11 in total

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

Authors:  Vrisha Madhuri; Smitha Elizabeth Mathew; Karthikeyan Rajagopal; Sowmya Ramesh; B Antonisamy
Journal:  Bone Rep       Date:  2016-10-14

5.  Identification and characterization of NF1 and non-NF1 congenital pseudarthrosis of the tibia based on germline NF1 variants: genetic and clinical analysis of 75 patients.

Authors:  Guanghui Zhu; Yu Zheng; Yaoxi Liu; An Yan; Zhengmao Hu; Yongjia Yang; Shiting Xiang; Liping Li; Weijian Chen; Yu Peng; Nanbert Zhong; Haibo Mei
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9.  Evaluation of bone marrow-derived mesenchymal stem cell quality from patients with congenital pseudoarthrosis of the tibia.

Authors:  Ismail Hadisoebroto Dilogo; Fajar Mujadid; Retno Wahyu Nurhayati; Aryadi Kurniawan
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10.  Serum-derived exosomes from neurofibromatosis type 1 congenital tibial pseudarthrosis impaired bone by promoting osteoclastogenesis and inhibiting osteogenesis.

Authors:  Ge Yang; Hui Yu; Yaoxi Liu; Weihua Ye; Guanghui Zhu; An Yan; Qian Tan; Haibo Mei
Journal:  Exp Biol Med (Maywood)       Date:  2020-10-06
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