title: Defective interleukin-1 production in a familial monocyte disorder with a combined abnormality of mobility and phagocytosis-killing.
abstract: Monocytes in a familial monocyte disorder, a recently recognized primary immunodeficiency syndrome, with impaired phagocytic functions were studied for their ability to produce interleukin 1 (IL-1) as well as the surface property. Monocytes from two children (siblings) with the disorder possessed CD11b, CD13, CD14, CD33, Ia and LFA-1/Mac-1/p150,95 beta subunit antigens as determined by flow cytometry. Electron microscopic cytochemistry showed that the monocytes had surface glycoproteins reactive with four representative lectins. The IL-1 production by monocytes was assayed in the two patients and compared with that in six children with primary immunodeficiency syndromes and some monocyte abnormalities; three had congenital neutropenia, two had hyper-IgE syndrome, and one had defective monocyte chemotaxis. Monocyte culture supernatants were prepared with stimulation by lipopolysaccharide or silica, and their IL-1 activity was measured by the mouse thymocyte-proliferation assay. The patients' monocytes were defective in IL-1 production: the values were less than 1.0% of the control monocyte values (n = 12) and were in contrast with those of congenital neutropenia monocytes of 186.2% to 204.3%. These results demonstrate a familial monocyte disorder which is characteristic among the immunodeficiency syndromes with regard to the defective IL-1 production and the impaired phagocytic functions.
authors.affiliation: Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan.
authors.forename: A
authors.initials: A
authors.lastname: Komiyama
authors.forename: M
authors.initials: M
authors.lastname: Ichikawa
authors.forename: H
authors.initials: H
authors.lastname: Kanda
authors.forename: K
authors.initials: K
authors.lastname: Aoyama
authors.forename: K
authors.initials: K
authors.lastname: Yasui
authors.forename: M
authors.initials: M
authors.lastname: Yamazaki
authors.forename: H
authors.initials: H
authors.lastname: Kawai
authors.forename: Y
authors.initials: Y
authors.lastname: Miyagawa
authors.forename: T
authors.initials: T
authors.lastname: Akabane
mesh_terms: D000293:Adolescent; D000954:Antigens, Surface / Q000032:analysis; D002634:Chemotaxis, Leukocyte; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000235:genetics / Q000276:immunology*; D007375:Interleukin-1 / Q000096:biosynthesis*; D007958:Leukocyte Count; D009000:Monocytes / Q000276:immunology*; D010587:Phagocytosis
chemical_list: D000954:Antigens, Surface; D007375:Interleukin-1