{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,5]],"date-time":"2025-11-05T20:20:44Z","timestamp":1762374044223,"version":"build-2065373602"},"reference-count":0,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[1987,6,1]],"date-time":"1987-06-01T00:00:00Z","timestamp":549504000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["AI 19031"],"award-info":[{"award-number":["AI 19031"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Periodontology"],"published-print":{"date-parts":[[1987,6]]},"abstract":"<jats:p><jats:sc>The clinical, histopathologic and functional<\/jats:sc> consequences of the genetic deficiency of leukocyte Mac\u20101, LFA\u20101 and p150,95 were assessed among (1) three affected patients, (2) heterozygotes and (3) unaffected individuals among two generations of a single kindred. Longitudinal assessments of this family afforded the unique opportunity to characterize the natural history of severe periodontal manifestations associated with this disorder. Features uniformly observed among each patient included recurrent, necrotic soft tissue infections, impaired pus formation, delayed wound healing, constant granulocytosis, severe abnormalities of adhesion\u2010dependent granulocyte functions and a profound deficiency (3%\u20136% of normal) of Mac\u20101 glycoproteins on granulocyte surfaces. Characteristic features of generalized prepubertal periodontitis including rapidly progressive alveolar bone loss affecting the primary and permanent dentitions (leading to premature tooth loss), recession, clefting and migration in association with intense gingival inflammation were uniformly observed. Biopsies of inflamed periodontal tissues in these individuals demonstrated dense infiltrates of mononuclear leukocytes but a striking absence of extravascular neutrophil granulocytes. Heterozygous family members demonstrated approximately half normal Mac\u20101 protein expression but no susceptibility to systemic infections and normal, adhesion\u2010dependent leukocyte functions. Prepubescent heterozygotes demonstrated no periodontal manifestations but a 31\u2010year\u2010old heterozygous female exhibited clinical and radiographic features typical of postjuvenile periodontitis. The profound periodontal manifestations recognized in this clinical\u2010pathologic model emphasize the physiologic importance of leukocyte adhesion reactions in defense of the periodontium and further suggest a possible pathologic role for Mac\u20101 proteins in other forms of early\u2010onset periodontitis.<\/jats:p>","DOI":"10.1902\/jop.1987.58.6.400","type":"journal-article","created":{"date-parts":[[2012,3,21]],"date-time":"2012-03-21T10:09:43Z","timestamp":1332324583000},"page":"400-416","source":"Crossref","is-referenced-by-count":118,"title":["Periodontal Manifestations of the Heritable Mac\u20101, LFA\u20101, Deficiency Syndrome"],"prefix":"10.1002","volume":"58","author":[{"given":"Thomas C.","family":"Waldrop","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Donald C.","family":"Anderson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"William W.","family":"Hallmon","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Frank C.","family":"Schmalstieg","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert L.","family":"Jacobs","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[1987,6]]},"container-title":["Journal of Periodontology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/aap.onlinelibrary.wiley.com\/doi\/pdf\/10.1902\/jop.1987.58.6.400","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,19]],"date-time":"2025-10-19T12:54:57Z","timestamp":1760878497000},"score":1,"resource":{"primary":{"URL":"https:\/\/aap.onlinelibrary.wiley.com\/doi\/10.1902\/jop.1987.58.6.400"}},"subtitle":["Clinical, Histopathologic and Molecular Characteristics"],"short-title":[],"issued":{"date-parts":[[1987,6]]},"references-count":0,"journal-issue":{"issue":"6","published-print":{"date-parts":[[1987,6]]}},"alternative-id":["10.1902\/jop.1987.58.6.400"],"URL":"https:\/\/doi.org\/10.1902\/jop.1987.58.6.400","archive":["Portico"],"relation":{},"ISSN":["0022-3492","1943-3670"],"issn-type":[{"type":"print","value":"0022-3492"},{"type":"electronic","value":"1943-3670"}],"subject":[],"published":{"date-parts":[[1987,6]]}}}