[Abnormal proteoglycans in growing cartilage in Kniest disease].
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Biomedical subjects
Publications and source records attributed to V Stanescu.
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During rotator cuff repairs, it is recommended that the hypovascular tissue edge be resected. To investigate rotator cuff tendon histopathology, we performed immunohistochemistry on 8 surgical and 6 cadaveric specimens. Hoechst nuclear stain and standard hematoxylin-eosin were used for morphologic analysis. Antibody to human von Willebrand factor tagged with fluorescein isothiocyanate, conjugated, was used to visualize vascularity, and antibody to human procollagen type I tagged with Cy3 was used to visualize new procollagen synthesis. There were no significant differences in the vascularity of surgical specimens sectioned near the tear site (<2.5 mm from tear margin) and matched cadaveric controls. However, sections taken 2.5 to 5 mm away from the tear demonstrated more vessels than those taken from either control or surgical specimens within 2.5 mm of the tear (P <.001). There were no differences in nuclear distribution patterns or in procollagen production and distribution between surgical specimens from sites near the tear or away from the tear. On the basis of morphologic architecture, these data suggest that minimal debridement of tendon edges only is required to maximize healing of the rotator cuff tendon at the time of repair.
Fragilitas ossium, fro, is an often lethal recessive mutation that was discovered in a randombred stock of mice after treatment with the chemical mutagen tris(1-aziridinyl) phosphine-sulphine. The fro/fro mice have osteoporosis-bending and fracture of the long bones-that is associated with apparently normal cartilage growth. The roentgenological and pathological features are similar to those of the severe, often lethal recessive form of human osteogenesis imperfecta.
The lethal chondrodysplasias are a complex group of diseases of which little is known. Three disorders have been well characterized; achondrogenesis, thanatophoric dwarfism, and the lethal chondrodysplasias with polydactyly. Pathological studies of epiphyseal cartilage confirm the separateness of these diseases but further work is necessary to specify the limits of variations. In achondrogenesis, relatively minor forms differ greatly from the usual description. In the chondrodysplasias with polydactyly, skeletal involvement appears to vary from one case to another. Furthermore, we must pursue the genetic studies in thanatophoric dwarfism where the exact mode of inheritance is known.
The case of two twins with micromelic dwarfism noticed at birth are reported. The studies performed on fibroblasts, lymphocytes and epiphyseal cartilage, suggest that a disturbance is involved in the cell division mechanisms.
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