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1.
Bone ; 153: 116132, 2021 12.
Article in English | MEDLINE | ID: mdl-34329814

ABSTRACT

Mechanical loading is a crucial factor in joint and bone development. Using a computational model, we investigated the role of mechanics on cartilage growth rate, ossification of the secondary center, formation of the growth plate, and overall bone shape. A computational algorithm was developed and implemented into finite element models to simulate the endochondral ossification for symmetric and asymmetric motion in a generic diarthrodial joint. Under asymmetric loading condition the secondary center ossifies asymmetrically leaning toward the external load and results in tilted growth plate. Also the mechanics seems to have greater influence in the early onset of the ossification of the secondary center rather than later progression of the center. While previous models have simulated select stages of skeletal development, our model can simulate growth and ossification during the entirety of post-natal development. Such computational models of skeletal development may provide insight into specific loading conditions that cause bone and joint deformities, and the required timing for rehabilitative repair.


Subject(s)
Models, Biological , Osteogenesis , Bone and Bones , Cartilage , Computer Simulation
2.
Pediatr Transplant ; 16(7): E296-300, 2012 Nov.
Article in English | MEDLINE | ID: mdl-22188489

ABSTRACT

Clinical and pathologic studies on adults with uremic neuropathy are numerous, but less is known about this disorder in children and adolescents. We report the clinical, electrophysiologic, and pathologic findings in an adolescent female with uremic neuropathy. Electrophysiologic findings were consistent with a primarily axonal sensorimotor polyneuropathy. Sural nerve biopsy revealed areas of focal depletion in myelin sheaths and loss of axons. Axonal degeneration with secondary myelin changes appears to be the characteristic pathology in this case, one of the youngest to our knowledge for which nerve biopsy data are available. Our patient experienced dramatic recovery after renal transplantation, similar to the reports of older patients.


Subject(s)
Glomerulosclerosis, Focal Segmental/therapy , Kidney Transplantation/methods , Polyneuropathies/therapy , Renal Insufficiency/therapy , Uremia/therapy , Adolescent , Axons/pathology , Biopsy/methods , Female , Humans , Neural Conduction , Polyneuropathies/etiology , Sural Nerve/pathology , Treatment Outcome
3.
Muscle Nerve ; 41(6): 746-50, 2010 Jun.
Article in English | MEDLINE | ID: mdl-20513101

ABSTRACT

We report a boy who received two allogeneic stem cell transplantations from umbilical cord donors to treat chronic granulomatous disease (CGD). The CGD was cured after the second transplantation, but 2.5 years later he was diagnosed with Duchenne muscular dystrophy (DMD). Examinations of his DNA, muscle tissue, and myoblast cultures derived from muscle tissue were performed to determine whether any donor dystrophin was being expressed. The boy was found to have a large-scale deletion on the X chromosome that spanned the loci for CYBB and DMD. The absence of dystrophin led to muscle histology characteristic of DMD. Analysis of myofibers demonstrated no definite donor cell engraftment. This case suggests that umbilical cord-derived hematopoietic stem cell transplantation will not be efficacious in the therapy of DMD without additional interventions that induce engraftment of donor cells in skeletal muscle.


Subject(s)
Dystrophin/deficiency , Dystrophin/genetics , Granulomatous Disease, Chronic/genetics , Granulomatous Disease, Chronic/surgery , Hematopoietic Stem Cell Transplantation/adverse effects , Muscular Dystrophy, Duchenne/genetics , Muscular Dystrophy, Duchenne/surgery , Alemtuzumab , Antibodies, Monoclonal/therapeutic use , Antibodies, Monoclonal, Humanized , Antibodies, Neoplasm/therapeutic use , Child , Chromosome Mapping , Chromosomes, Human, X , Cyclophosphamide/therapeutic use , Follow-Up Studies , Gene Deletion , Gene Expression Regulation , Humans , Male , Reoperation , Transplantation, Homologous , Treatment Outcome , Vidarabine/analogs & derivatives , Vidarabine/therapeutic use
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