title: Infantile agranulocytosis with survival into adolescence: periodontal manifestations and laboratory findings. A case report.
abstract: A case of infantile agranulocytosis with survival into adolescence is presented. The polymorphonuclear leukocyte is considered an important source of lysosomal enzymes in gingival crevicular fluid, and evaluation of connective tissue-degrading enzymes in the fluid was performed. The activity of beta-glucuronidase, a ground substance-degrading enzyme that may serve as a marker for polymorphonuclear leukocytes, was markedly reduced in the fluid compared to samples from systemically healthy adults with periodontitis. The activities of the ground substance-degrading enzyme arylsulfatase, and collagenase, were in the low-normal range. The plaque microbiology, as characterized by dark-field microscopy and selective culturing, was consistent with advanced periodontitis. A review of the medical history revealed a series of bacterial infections since infancy. Improvement in the systemic health of the patient occurred at about the age of 15, and the intake of antibiotics to control infections was correspondingly reduced after this time. An exacerbation of the patient's periodontal disease, as evaluated by loss of alveolar bone on radiographs, occurred 1 to 2 years later. The progression of periodontal disease observed in this patient was apparently associated with the withdrawal of antibiotics administered for control of systemic (nonoral) infections.
mesh_terms: D000293:Adolescent; D000380:Agranulocytosis / Q000097:blood / Q000201:enzymology*; D001192:Arylsulfatases / Q000032:analysis; D001419:Bacteria / Q000302:isolation & purification; D005260:Female; D005883:Gingival Crevicular Fluid / Q000201:enzymology / Q000382:microbiology; D005966:Glucuronidase / Q000032:analysis; D006801:Humans; D007958:Leukocyte Count; D003012:Microbial Collagenase / Q000032:analysis; D010510:Periodontal Diseases / Q000097:blood / Q000201:enzymology* / Q000382:microbiology
chemical_list: D001192:Arylsulfatases; D005966:Glucuronidase; D003012:Microbial Collagenase