Mixed-sclerosing-bone-dystrophy: 42-year follow-up of a case reported as osteopetrosis
Identifieur interne : 00C783 ( Main/Exploration ); précédent : 00C782; suivant : 00C784Mixed-sclerosing-bone-dystrophy: 42-year follow-up of a case reported as osteopetrosis
Auteurs : Roberto Pacifici [États-Unis] ; William A. Murphy [États-Unis] ; Steven L. Teitelbaum [États-Unis] ; Michael P. Whyte [États-Unis]Source :
- Calcified Tissue International [ 0171-967X ] ; 1986-05-01.
English descriptors
- KwdEn :
- Abnormality, Alkaline phosphatase activity, Biopsy, Bone disease, Bone formation, Bone mass, Bone resorption, Case report, Cerebellar tonsils, Chronic hypocalcemia, Coronal images, Cortical abnormalities, Dense metaphyseal spots, Diffuse osteosclerosis, Distal aspects, Distal femora, Exposure factors, Foramen magnum, Histopathological studies, Hypophosphatemia, Iliac, Iliac crest, Iliac crest biopsy, Inorganic phosphate, Ischial tuberosities, Jewish hospital, Long bones, Mallinckrodt institute, Medullary space, Melorheostosis, Mineral metabolism, Msbd, Natural history, Nutritional status, Osteoid seams, Osteopathia, Osteopathia striata, Osteopathia striata pattern, Osteopetrosis, Osteopoikilosis, Osteosclerosis, Pacifici, Paranasal sinuses, Poor nutrition, Quiescent bone formation, Radial striation pattern, Radiological, Radiological abnormalities, Radiological features, Rare occurrence, Research center, Residual marrow, Sclerosing bone dystrophy, Secondary hyperparathyroidism, Single subject, Skeletal disease, Skeletal mass, Spinal cord, Striata, Striata pattern, Striation, Term msbd, Tetracycline, Tetracycline labels, Transverse image, Vertebral bodies, Washington university.
- Teeft :
- Abnormality, Alkaline phosphatase activity, Biopsy, Bone disease, Bone formation, Bone mass, Bone resorption, Case report, Cerebellar tonsils, Chronic hypocalcemia, Coronal images, Cortical abnormalities, Dense metaphyseal spots, Diffuse osteosclerosis, Distal aspects, Distal femora, Exposure factors, Foramen magnum, Histopathological studies, Hypophosphatemia, Iliac, Iliac crest, Iliac crest biopsy, Inorganic phosphate, Ischial tuberosities, Jewish hospital, Long bones, Mallinckrodt institute, Medullary space, Melorheostosis, Mineral metabolism, Msbd, Natural history, Nutritional status, Osteoid seams, Osteopathia, Osteopathia striata, Osteopathia striata pattern, Osteopetrosis, Osteopoikilosis, Osteosclerosis, Pacifici, Paranasal sinuses, Poor nutrition, Quiescent bone formation, Radial striation pattern, Radiological, Radiological abnormalities, Radiological features, Rare occurrence, Research center, Residual marrow, Sclerosing bone dystrophy, Secondary hyperparathyroidism, Single subject, Skeletal disease, Skeletal mass, Spinal cord, Striata, Striata pattern, Striation, Term msbd, Tetracycline, Tetracycline labels, Transverse image, Vertebral bodies, Washington university.
Abstract
Summary: We present a detailed metabolic investigation and 42-year radiological follow-up of a 52-year-old man with mixed-sclerosing-bone-dystrophy, the rare occurrence of two or more distinct patterns of sclerosing-bone-dysplasia (e.g., osteopathia striata, osteopoikilosis, melorheostosis) in a single subject. Review of radiographs from 1942, when he was reported to have osteopetrosis, demonstrated diffuse osteosclerosis, osteopathia striata, osteopoikilosis, and focal cortical hyperostosis. Forty-two years later, there had been significant progression and evolution of his skeletal disease with the appearance of new areas of osteopathia striata and osteopoikilosis and a generalized increase in skeletal mass as assessed radiographically. Presence of subperiosteal bone apposition on biopsy of the iliac crest together with chronic mild hypocalcemia, secondary hyperparathyroidism, and hypophosphatemia suggested that enhanced bone formation, perhaps with defective skeletal resorption, is a fundamental abnormality which accounts for the increased bone mass of this patient.
Url:
DOI: 10.1007/BF02556878
Affiliations:
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Le document en format XML
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<term>Biopsy</term>
<term>Bone disease</term>
<term>Bone formation</term>
<term>Bone mass</term>
<term>Bone resorption</term>
<term>Case report</term>
<term>Cerebellar tonsils</term>
<term>Chronic hypocalcemia</term>
<term>Coronal images</term>
<term>Cortical abnormalities</term>
<term>Dense metaphyseal spots</term>
<term>Diffuse osteosclerosis</term>
<term>Distal aspects</term>
<term>Distal femora</term>
<term>Exposure factors</term>
<term>Foramen magnum</term>
<term>Histopathological studies</term>
<term>Hypophosphatemia</term>
<term>Iliac</term>
<term>Iliac crest</term>
<term>Iliac crest biopsy</term>
<term>Inorganic phosphate</term>
<term>Ischial tuberosities</term>
<term>Jewish hospital</term>
<term>Long bones</term>
<term>Mallinckrodt institute</term>
<term>Medullary space</term>
<term>Melorheostosis</term>
<term>Mineral metabolism</term>
<term>Msbd</term>
<term>Natural history</term>
<term>Nutritional status</term>
<term>Osteoid seams</term>
<term>Osteopathia</term>
<term>Osteopathia striata</term>
<term>Osteopathia striata pattern</term>
<term>Osteopetrosis</term>
<term>Osteopoikilosis</term>
<term>Osteosclerosis</term>
<term>Pacifici</term>
<term>Paranasal sinuses</term>
<term>Poor nutrition</term>
<term>Quiescent bone formation</term>
<term>Radial striation pattern</term>
<term>Radiological</term>
<term>Radiological abnormalities</term>
<term>Radiological features</term>
<term>Rare occurrence</term>
<term>Research center</term>
<term>Residual marrow</term>
<term>Sclerosing bone dystrophy</term>
<term>Secondary hyperparathyroidism</term>
<term>Single subject</term>
<term>Skeletal disease</term>
<term>Skeletal mass</term>
<term>Spinal cord</term>
<term>Striata</term>
<term>Striata pattern</term>
<term>Striation</term>
<term>Term msbd</term>
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<term>Vertebral bodies</term>
<term>Washington university</term>
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<term>Biopsy</term>
<term>Bone disease</term>
<term>Bone formation</term>
<term>Bone mass</term>
<term>Bone resorption</term>
<term>Case report</term>
<term>Cerebellar tonsils</term>
<term>Chronic hypocalcemia</term>
<term>Coronal images</term>
<term>Cortical abnormalities</term>
<term>Dense metaphyseal spots</term>
<term>Diffuse osteosclerosis</term>
<term>Distal aspects</term>
<term>Distal femora</term>
<term>Exposure factors</term>
<term>Foramen magnum</term>
<term>Histopathological studies</term>
<term>Hypophosphatemia</term>
<term>Iliac</term>
<term>Iliac crest</term>
<term>Iliac crest biopsy</term>
<term>Inorganic phosphate</term>
<term>Ischial tuberosities</term>
<term>Jewish hospital</term>
<term>Long bones</term>
<term>Mallinckrodt institute</term>
<term>Medullary space</term>
<term>Melorheostosis</term>
<term>Mineral metabolism</term>
<term>Msbd</term>
<term>Natural history</term>
<term>Nutritional status</term>
<term>Osteoid seams</term>
<term>Osteopathia</term>
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<term>Osteopathia striata pattern</term>
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<term>Osteopoikilosis</term>
<term>Osteosclerosis</term>
<term>Pacifici</term>
<term>Paranasal sinuses</term>
<term>Poor nutrition</term>
<term>Quiescent bone formation</term>
<term>Radial striation pattern</term>
<term>Radiological</term>
<term>Radiological abnormalities</term>
<term>Radiological features</term>
<term>Rare occurrence</term>
<term>Research center</term>
<term>Residual marrow</term>
<term>Sclerosing bone dystrophy</term>
<term>Secondary hyperparathyroidism</term>
<term>Single subject</term>
<term>Skeletal disease</term>
<term>Skeletal mass</term>
<term>Spinal cord</term>
<term>Striata</term>
<term>Striata pattern</term>
<term>Striation</term>
<term>Term msbd</term>
<term>Tetracycline</term>
<term>Tetracycline labels</term>
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<front><div type="abstract" xml:lang="en">Summary: We present a detailed metabolic investigation and 42-year radiological follow-up of a 52-year-old man with mixed-sclerosing-bone-dystrophy, the rare occurrence of two or more distinct patterns of sclerosing-bone-dysplasia (e.g., osteopathia striata, osteopoikilosis, melorheostosis) in a single subject. Review of radiographs from 1942, when he was reported to have osteopetrosis, demonstrated diffuse osteosclerosis, osteopathia striata, osteopoikilosis, and focal cortical hyperostosis. Forty-two years later, there had been significant progression and evolution of his skeletal disease with the appearance of new areas of osteopathia striata and osteopoikilosis and a generalized increase in skeletal mass as assessed radiographically. Presence of subperiosteal bone apposition on biopsy of the iliac crest together with chronic mild hypocalcemia, secondary hyperparathyroidism, and hypophosphatemia suggested that enhanced bone formation, perhaps with defective skeletal resorption, is a fundamental abnormality which accounts for the increased bone mass of this patient.</div>
</front>
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<name sortKey="Murphy, William A" sort="Murphy, William A" uniqKey="Murphy W" first="William A." last="Murphy">William A. Murphy</name>
<name sortKey="Teitelbaum, Steven L" sort="Teitelbaum, Steven L" uniqKey="Teitelbaum S" first="Steven L." last="Teitelbaum">Steven L. Teitelbaum</name>
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