1991 Fiscal Year Final Research Report Summary
Clinical and experimental study on skeletal dysplasias
| Project/Area Number |
02670672
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| Research Category |
Grant-in-Aid for General Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Research Field |
Orthopaedic surgery
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| Research Institution | Institute for Developmental Research, Aichi Prefectural Colony |
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Principal Investigator |
NOGAMI Hiroshi Institute for Developmental Research, Aichi Prefectural Colony Head, 治療学部, 部長 (30100168)
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| Project Period (FY) |
1990 – 1991
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| Keywords | Skeletal dysplasia / Osteogenesis imperfecta / Periosteum / Endotherial cell / Coffin-Lowry syndrome / Creatine kinase / S-100 protein / Center of gravity |
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| Research Abstract |
(1)Morphological examination of the periosteum from the diaphyseal bone of osteogenesis imperfecta patients revealed defective microvascular system. Hypertrophic endothelial cells of capillary vessels and arterioles, defective basement membranes and multiplication smooth muscle layers suggested existence of circulatory deficiency in the osteogenic imperfecta periosteum.
(2)Clinical and laboratory investigation of Coffin-Lowry syndrome, one of skeletal dysplasias, demonstrated that this disorder is a calcium pyrophosphate dehydrate crystal deposition disease, and it was postulated that a metabolic abnormality in collagen and proteoglycans might be also responsible for some aspects of the syndrome.
(3)Basic studies. on differentiation and growth of cartilage and bone revealed creatine kinase subunits and 5-100 protein might play a role during those processes especially in the stage of endochondral ossification.
(4)Cultured chondrocytes bound to hydroxyapatite was found to be experimentally useful material for repair of large bone defect such as congenital pseudarthrosis.
(5)Investigation of accuracy in measuring the center of gravity in standing posture of small children was performed using force plate. It is a part of our study to clarify cause of falling down tendency and abnormal standing posture in children with skeletal dysplasias.
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