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Immunological and metabolical studies in two siblings with Papillon‐Lefevre syndrome
Author(s) -
Lyberg Torstein
Publication year - 1982
Publication title -
journal of periodontal research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.31
H-Index - 83
eISSN - 1600-0765
pISSN - 0022-3484
DOI - 10.1111/j.1600-0765.1982.tb01177.x
Subject(s) - medicine , immunology , disease , polymorphonuclear leukocyte , pathology , biology , genetics , in vitro
Two cases of typical Papillon‐Lefevre syndrome in one family were reported. Neither patient showed evidence of systemic disease as assessed by the medical history and a battery of clinical laboratory tests including a screening system for the detection of inborn errors of metabolism. A survey of the patients’ immunological status, including lymphocyte transformation tests using mitogens and polymorphonuclear leukocyte chemoiaxis studies, gave no indication of disturbances in immunological functions and host defense mechanisms. Possible pathogenetic mechanisms are discussed.