Fibrogenesis imperfecta ossium (Baker’s disease): a case studied at autopsy
A man aged 40 years showed radiographic changes in the form of generalized increased bone density and patchy rarefaction. Urinary calcium was increased and serum alkaline phosphatase was elevated; serum calcium and phosphate levels were normal. Multiple fractures developed. At autopsy, all parts of...
Gespeichert in:
| Veröffentlicht in: | Bone (New York, N.Y.) N.Y.), 2000-12, Vol.27 (6), p.865-873 |
|---|---|
| 1. Verfasser: | |
| Format: | Artikel |
| Sprache: | eng |
| Schlagworte: | |
| Online-Zugang: | Volltext |
| Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
| container_end_page | 873 |
|---|---|
| container_issue | 6 |
| container_start_page | 865 |
| container_title | Bone (New York, N.Y.) |
| container_volume | 27 |
| creator | Sissons, H.A |
| description | A man aged 40 years showed radiographic changes in the form of generalized increased bone density and patchy rarefaction. Urinary calcium was increased and serum alkaline phosphatase was elevated; serum calcium and phosphate levels were normal. Multiple fractures developed. At autopsy, all parts of the skeleton showed partial replacement of bone and bone marrow by a tissue deficient in collagen fibers. Much of this tissue was unmineralized, but lesions in cortical bone showed hypermineralization on microradiographic examination. Electron microscopy showed replacement of collagen fibers by amorphous material in the affected areas. Electron probe analysis showed a normal Ca:P ratio for bone mineral in the hypermineralized areas. |
| doi_str_mv | 10.1016/S8756-3282(00)00401-4 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_72466180</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><els_id>S8756328200004014</els_id><sourcerecordid>72466180</sourcerecordid><originalsourceid>FETCH-LOGICAL-c389t-f3258d1eb8aa4130ef8923ef820eb61721f60d7c4f08fe3033f28da08817d8433</originalsourceid><addsrcrecordid>eNqFkMFOGzEQQK2KqgTaTyiyhFQlh6Vje7Pr5YIKKm0lUA-Fs-XYY2TIZoNnFyk3fqO_x5fgkAiOzMEzhzeemcfYVwFHAkT1_Z-up1WhpJZjgAlACaIoP7CR0LUqZF2pHTZ6RXbZHtEtAKimFp_YrsihVNOM2OV5nKXuBhdIkXhsl5gCut7yjigOLR-f2jtMT4__iftIaAknx9xylwtO_eAjem57boe-W9LqM_sY7Jzwyzbvs-vzn1dnv4uLv7_-nP24KJzSTV8EJafaC5xpa0uhAINupMqvBJxVopYiVOBrVwbQARUoFaT2FrQWtdelUvvs2-bfZeruB6TetJEczud2gd1AppZlVQkNGZxuQJfyQQmDWabY2rQyAszao3nxaNaSDIB58WjK3HewHTDMWvRvXVtxGTjcApacnYdkFy7SK6dVBdP1nicbCrOMh4jJkIu4cOhjypaN7-I7izwDllmOmA</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>72466180</pqid></control><display><type>article</type><title>Fibrogenesis imperfecta ossium (Baker’s disease): a case studied at autopsy</title><source>MEDLINE</source><source>ScienceDirect Journals (5 years ago - present)</source><creator>Sissons, H.A</creator><creatorcontrib>Sissons, H.A</creatorcontrib><description>A man aged 40 years showed radiographic changes in the form of generalized increased bone density and patchy rarefaction. Urinary calcium was increased and serum alkaline phosphatase was elevated; serum calcium and phosphate levels were normal. Multiple fractures developed. At autopsy, all parts of the skeleton showed partial replacement of bone and bone marrow by a tissue deficient in collagen fibers. Much of this tissue was unmineralized, but lesions in cortical bone showed hypermineralization on microradiographic examination. Electron microscopy showed replacement of collagen fibers by amorphous material in the affected areas. Electron probe analysis showed a normal Ca:P ratio for bone mineral in the hypermineralized areas.</description><identifier>ISSN: 8756-3282</identifier><identifier>EISSN: 1873-2763</identifier><identifier>DOI: 10.1016/S8756-3282(00)00401-4</identifier><identifier>PMID: 11113399</identifier><language>eng</language><publisher>New York, NY: Elsevier Inc</publisher><subject>Adult ; Biological and medical sciences ; Bone ; Bone and Bones - pathology ; Bone Diseases - diagnostic imaging ; Bone Diseases - pathology ; Collagen ; Collagen Diseases - diagnostic imaging ; Collagen Diseases - pathology ; Diseases of the osteoarticular system ; Fatal Outcome ; Femur - pathology ; Humans ; Male ; Medical sciences ; Osteoporosis. Osteomalacia. Paget disease ; Osteosclerosis ; Radiography ; Ribs - pathology ; Spine - pathology ; Tibia - pathology</subject><ispartof>Bone (New York, N.Y.), 2000-12, Vol.27 (6), p.865-873</ispartof><rights>2000 Elsevier Science Inc.</rights><rights>2001 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c389t-f3258d1eb8aa4130ef8923ef820eb61721f60d7c4f08fe3033f28da08817d8433</citedby><cites>FETCH-LOGICAL-c389t-f3258d1eb8aa4130ef8923ef820eb61721f60d7c4f08fe3033f28da08817d8433</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/S8756-3282(00)00401-4$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3550,27924,27925,45995</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=836053$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/11113399$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Sissons, H.A</creatorcontrib><title>Fibrogenesis imperfecta ossium (Baker’s disease): a case studied at autopsy</title><title>Bone (New York, N.Y.)</title><addtitle>Bone</addtitle><description>A man aged 40 years showed radiographic changes in the form of generalized increased bone density and patchy rarefaction. Urinary calcium was increased and serum alkaline phosphatase was elevated; serum calcium and phosphate levels were normal. Multiple fractures developed. At autopsy, all parts of the skeleton showed partial replacement of bone and bone marrow by a tissue deficient in collagen fibers. Much of this tissue was unmineralized, but lesions in cortical bone showed hypermineralization on microradiographic examination. Electron microscopy showed replacement of collagen fibers by amorphous material in the affected areas. Electron probe analysis showed a normal Ca:P ratio for bone mineral in the hypermineralized areas.</description><subject>Adult</subject><subject>Biological and medical sciences</subject><subject>Bone</subject><subject>Bone and Bones - pathology</subject><subject>Bone Diseases - diagnostic imaging</subject><subject>Bone Diseases - pathology</subject><subject>Collagen</subject><subject>Collagen Diseases - diagnostic imaging</subject><subject>Collagen Diseases - pathology</subject><subject>Diseases of the osteoarticular system</subject><subject>Fatal Outcome</subject><subject>Femur - pathology</subject><subject>Humans</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Osteoporosis. Osteomalacia. Paget disease</subject><subject>Osteosclerosis</subject><subject>Radiography</subject><subject>Ribs - pathology</subject><subject>Spine - pathology</subject><subject>Tibia - pathology</subject><issn>8756-3282</issn><issn>1873-2763</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2000</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkMFOGzEQQK2KqgTaTyiyhFQlh6Vje7Pr5YIKKm0lUA-Fs-XYY2TIZoNnFyk3fqO_x5fgkAiOzMEzhzeemcfYVwFHAkT1_Z-up1WhpJZjgAlACaIoP7CR0LUqZF2pHTZ6RXbZHtEtAKimFp_YrsihVNOM2OV5nKXuBhdIkXhsl5gCut7yjigOLR-f2jtMT4__iftIaAknx9xylwtO_eAjem57boe-W9LqM_sY7Jzwyzbvs-vzn1dnv4uLv7_-nP24KJzSTV8EJafaC5xpa0uhAINupMqvBJxVopYiVOBrVwbQARUoFaT2FrQWtdelUvvs2-bfZeruB6TetJEczud2gd1AppZlVQkNGZxuQJfyQQmDWabY2rQyAszao3nxaNaSDIB58WjK3HewHTDMWvRvXVtxGTjcApacnYdkFy7SK6dVBdP1nicbCrOMh4jJkIu4cOhjypaN7-I7izwDllmOmA</recordid><startdate>20001201</startdate><enddate>20001201</enddate><creator>Sissons, H.A</creator><general>Elsevier Inc</general><general>Elsevier Science</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20001201</creationdate><title>Fibrogenesis imperfecta ossium (Baker’s disease): a case studied at autopsy</title><author>Sissons, H.A</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c389t-f3258d1eb8aa4130ef8923ef820eb61721f60d7c4f08fe3033f28da08817d8433</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2000</creationdate><topic>Adult</topic><topic>Biological and medical sciences</topic><topic>Bone</topic><topic>Bone and Bones - pathology</topic><topic>Bone Diseases - diagnostic imaging</topic><topic>Bone Diseases - pathology</topic><topic>Collagen</topic><topic>Collagen Diseases - diagnostic imaging</topic><topic>Collagen Diseases - pathology</topic><topic>Diseases of the osteoarticular system</topic><topic>Fatal Outcome</topic><topic>Femur - pathology</topic><topic>Humans</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Osteoporosis. Osteomalacia. Paget disease</topic><topic>Osteosclerosis</topic><topic>Radiography</topic><topic>Ribs - pathology</topic><topic>Spine - pathology</topic><topic>Tibia - pathology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Sissons, H.A</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Bone (New York, N.Y.)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Sissons, H.A</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Fibrogenesis imperfecta ossium (Baker’s disease): a case studied at autopsy</atitle><jtitle>Bone (New York, N.Y.)</jtitle><addtitle>Bone</addtitle><date>2000-12-01</date><risdate>2000</risdate><volume>27</volume><issue>6</issue><spage>865</spage><epage>873</epage><pages>865-873</pages><issn>8756-3282</issn><eissn>1873-2763</eissn><abstract>A man aged 40 years showed radiographic changes in the form of generalized increased bone density and patchy rarefaction. Urinary calcium was increased and serum alkaline phosphatase was elevated; serum calcium and phosphate levels were normal. Multiple fractures developed. At autopsy, all parts of the skeleton showed partial replacement of bone and bone marrow by a tissue deficient in collagen fibers. Much of this tissue was unmineralized, but lesions in cortical bone showed hypermineralization on microradiographic examination. Electron microscopy showed replacement of collagen fibers by amorphous material in the affected areas. Electron probe analysis showed a normal Ca:P ratio for bone mineral in the hypermineralized areas.</abstract><cop>New York, NY</cop><pub>Elsevier Inc</pub><pmid>11113399</pmid><doi>10.1016/S8756-3282(00)00401-4</doi><tpages>9</tpages></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 8756-3282 |
| ispartof | Bone (New York, N.Y.), 2000-12, Vol.27 (6), p.865-873 |
| issn | 8756-3282 1873-2763 |
| language | eng |
| recordid | cdi_proquest_miscellaneous_72466180 |
| source | MEDLINE; ScienceDirect Journals (5 years ago - present) |
| subjects | Adult Biological and medical sciences Bone Bone and Bones - pathology Bone Diseases - diagnostic imaging Bone Diseases - pathology Collagen Collagen Diseases - diagnostic imaging Collagen Diseases - pathology Diseases of the osteoarticular system Fatal Outcome Femur - pathology Humans Male Medical sciences Osteoporosis. Osteomalacia. Paget disease Osteosclerosis Radiography Ribs - pathology Spine - pathology Tibia - pathology |
| title | Fibrogenesis imperfecta ossium (Baker’s disease): a case studied at autopsy |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-06T20%3A35%3A08IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_cross&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Fibrogenesis%20imperfecta%20ossium%20(Baker%E2%80%99s%20disease):%20a%20case%20studied%20at%20autopsy&rft.jtitle=Bone%20(New%20York,%20N.Y.)&rft.au=Sissons,%20H.A&rft.date=2000-12-01&rft.volume=27&rft.issue=6&rft.spage=865&rft.epage=873&rft.pages=865-873&rft.issn=8756-3282&rft.eissn=1873-2763&rft_id=info:doi/10.1016/S8756-3282(00)00401-4&rft_dat=%3Cproquest_cross%3E72466180%3C/proquest_cross%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=72466180&rft_id=info:pmid/11113399&rft_els_id=S8756328200004014&rfr_iscdi=true |