RESUMO
Polymorphonuclear leukocyte (PMN) function was investigated in two patients with glycogen storage disease type IB and neutropenia. Glycogen storage disease type IB was documented by liver biopsy and a normal amount of latent glucose-6-phosphatase activity. Patient A had stomatitis, skin infections, and septicemia; patient B had respiratory infections, periodontitis, and oral candidiasis. Absolute neutrophil counts ranged from 114 to 2580/mm3. Diminished and delayed migration of PMN into a skin "window" occurred in B. Random and directed PMN migration under agarose toward f-Met-Leu-Phe, pepstatin A, and zymosan-activated serum were severely diminished in both patients. At 10(-7) M f-Met-Leu-Phe, mean random and directed migration were 52 and 23% (A, n = 3) and 48 and 13% (B, n = 4) of controls. These results were independent of incubation time and chemoattractant concentration. Patients' PMN had diminished quantitative nitroblue tetrazolium reduction compared to controls. B had a significant defect in PMN bactericidal activity with Escherichia coli with less than 0.2 log killing at 2 h. These results further characterize the defect in PMN migration reported by Beaudet et al. (J Pediatr 97:906, 1980). The finding of other abnormalities of PMN function suggests a metabolic defect in the neutrophil which may be related to the microsomal membrane defect in hepatocytes in glycogen storage disease type IB.
Assuntos
Quimiotaxia de Leucócito , Doença de Depósito de Glicogênio Tipo I/fisiopatologia , Neutrófilos/fisiologia , Adolescente , Atividade Bactericida do Sangue , Criança , Feminino , Granulócitos/fisiologia , Humanos , MasculinoRESUMO
Two cases of severe Epstein-Barr virus (EBV) pulmonary involvement are described. Both were documented by EBV-specific serology and other causes of respiratory tract infection were excluded by culture and serology. Our first case describes fatal infectious mononucleosis (IM) with severe pneumonia in a patient without a definable immunologic disorder. Previous cases of pulmonary involvement in IM are reviewed.