The Dkk3-derived cells represent a branch of the periosteal mesenchymal lineage that produces fibrocartilage as well as regenerating the periosteal structures. Mesenchymal progenitor cells are capable of generating a wide variety of mature cells that constitute the connective tissue system. Our Laboratory has been developing SMAA GFP reporter mice to prove to be an effective tool for identifying these cells prior to the expression of markers of differentiation characteristic of bone, fat, muscular blood vessels or fibrocartilage. Dkk3 was chosen as a candidate reporter because microarray of SMAA-sorted cells culture indicated high expression of this non-canonical anti-Wnt factor, which was not anticipated in a culture with strong osteogenic potential.Summary Statement
Introduction
We prospectively reviewed 2440 Cemented Anantomical (side specific) AGC total knee replacements performed on 2074 patients between 2002 and 2008 in our unit. The surgery was performed by a number of surgeons, both Consultant and Trainee grades. 1357 underwent PCL sacrificing surgery with implantation of Posterior Stabilised Femoral Implant (Cam and Grove) and 1083 underwent PCL retaining surgery with implantation of a Non-Stabilised Femoral Implant. The mean age at date of surgery for Posterior Stabilised prosthesis was 69 years (range 23-94) and Non-Posterior Stabilised prosthesis was 70 (range 33-97) with no significant difference p< 0.05 (C.I 95%). Follow up at present mean 4 years (range 1-6 years). 11 patients underwent secondary patella resurfaced for anterior knee pain at a mean of 1.5 years following the index procedure (range 0.5-4.1 years) with 6 patients reporting a marked improvement in anterior knee pain post operatively. 3 patients underwent removal of patella buttons for symptomatic loosening at a mean of 2.7 years follow index procedure. A total of 17 patients underwent a revision of either tibial or femoral components (0.7%). Indications for revision were infection in 11 patients, gross instability in 1 patient, periprosthetic fracture in 1 patient, persistent stiffness in 1 patient. 3 patients underwent revision for symptomatic loosening of the tibial component at a mean of 2.9 years (range 1.1-4.0). These short to medium term results for the Anatomic AGC total knee replacement agree with published results from other centres.
We report 40 cases in one family of an autosomal dominant bone dysplasia, which, though similar in some aspects to Paget's disease, seems unique in some features and in its natural history. The disease shows both general and focal skeletal changes, the latter being mainly in the limbs with an onset from the second decade. Progressive osteoclastic resorption is accompanied by medullary expansion which leads to pain, severe deformity and a tendency to pathological fracture. The serum alkaline phosphatase and urinary hydroxyproline are variably elevated, while other biochemical indices are normal. Most patients had an associated deafness of early onset and loss of dentition. No previous description of this disease has been found in the literature.