{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T14:10:48Z","timestamp":1770819048975,"version":"3.50.1"},"reference-count":16,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2005,6,3]],"date-time":"2005-06-03T00:00:00Z","timestamp":1117756800000},"content-version":"vor","delay-in-days":5754,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Am. J. Med. Genet."],"published-print":{"date-parts":[[1989,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Certain disorders of the immune system seem to be associated with skeletal defects. The association was first recorded by McKusick et al. (<jats:italic>Bulletin of the Johns Hopkins Hospital<\/jats:italic> 116:285\u2013326, 1964). A number of relationships between lymphocytes and osteocytes can be proposed. These include a common environment for development, common metabolic needs and effects upon osteocytes by products (cytokines) elaborated from lymphocytes or monocytes during immune responses. Thus, bony defects of varying degrees of severity are seen in short\u2010limb dwarfs, cartilage\u2010hair hypoplasia, and adenosine deaminase (ADA) deficiency. Cytokine activation of osteoclasts accounts for the lytic lesions seen in malignancies and the excessive bone resorption which accompanies autoimmune disorders such as rheumatoid arthritis.<\/jats:p><jats:p>Correction of primary immune deficiency is accomplished by bone marrow transplantation. If the bony abnormality is subtle (as in some cases of ADA deficiency) the skeletal problem is resolved; if the bone defect is major as in short\u2010limb dwarfism, no improvement is seen.<\/jats:p>","DOI":"10.1002\/ajmg.1320340111","type":"journal-article","created":{"date-parts":[[2005,6,11]],"date-time":"2005-06-11T13:27:54Z","timestamp":1118496474000},"page":"55-59","source":"Crossref","is-referenced-by-count":23,"title":["Associations of the skeletal and immune systems"],"prefix":"10.1002","volume":"34","author":[{"given":"Richard","family":"Hong","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2005,6,3]]},"reference":[{"key":"e_1_2_1_2_1","first-page":"389","article-title":"Unusual bone changes in thymic alymphoplasia","volume":"11","author":"Alexander WJ","year":"1968","journal-title":"Ann Radiol"},{"key":"e_1_2_1_3_1","first-page":"286","volume-title":"Immunologic Disorders in Infants and Children","author":"Ammann AJ","year":"1980"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/319516a0"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(76)80793-7"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.2.5526.1371"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198708273170902"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(72)92345-8"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1038\/306378a0"},{"key":"e_1_2_1_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02009053"},{"key":"e_1_2_1_11_1","first-page":"285","article-title":"Dwarfism in the Amish. II. Cartilage\u2010hair hypoplasia","volume":"116","author":"McKusick VA","year":"1964","journal-title":"Bull Johns Hopkins Hosp"},{"key":"e_1_2_1_12_1","doi-asserted-by":"crossref","first-page":"664","DOI":"10.1182\/blood.V70.3.664.664","article-title":"Immunologic reconstitution after haploidentical bone marrow transplantation for immune deficiency disorders: Treatment of bone marrow cells with monoclonal antibody CT\u20102 and complement","volume":"70","author":"Moen RC","year":"1987","journal-title":"Blood"},{"key":"e_1_2_1_13_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood.V62.5.941.941"},{"key":"e_1_2_1_14_1","first-page":"621","article-title":"Lymphocyte dysfunction in cartilage hair hypoplasia. II. Evidence for a cell cycle specific defect in T cell growth","volume":"50","author":"Pierce GF","year":"1982","journal-title":"Clin Exp Immunol"},{"key":"e_1_2_1_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(87)90491-8"},{"key":"e_1_2_1_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(84)80014-1"},{"key":"e_1_2_1_17_1","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1080\/21548331.1987.11707674","article-title":"Bone marrow transplantation in hematologic malignancies","volume":"22","author":"Thomas ED","year":"1987","journal-title":"Hosp Pract [Off]"}],"container-title":["American Journal of Medical Genetics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fajmg.1320340111","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/ajmg.1320340111","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,22]],"date-time":"2023-10-22T12:22:17Z","timestamp":1697977337000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/ajmg.1320340111"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1989,9]]},"references-count":16,"journal-issue":{"issue":"1","published-print":{"date-parts":[[1989,9]]}},"alternative-id":["10.1002\/ajmg.1320340111"],"URL":"https:\/\/doi.org\/10.1002\/ajmg.1320340111","archive":["Portico"],"relation":{},"ISSN":["0148-7299","1096-8628"],"issn-type":[{"value":"0148-7299","type":"print"},{"value":"1096-8628","type":"electronic"}],"subject":[],"published":{"date-parts":[[1989,9]]}}}