ABSTRACT. Polymorphonuclear leukocyte (PMN) func-GSD type IB is characterized by the clinical features of glucosetion was investigated in two patients with glycogen storage 6-phosphatase deficiency (GSD type IA), including hepatomegdisease type IB and neutropenia. Glycogen storage disease aly, growth retardation, fasting hypoglycemia, and lactic acidosis, type IB was documented by liver biopsy and a normal but normal latent enzymatic activity in the liver (l,2). Biochemamount of latent glucose-6-phosphatase activity. Patient A ical studies have demonstrated a defect in the microsomal transhad stomatitis, skin infections, and septicemia; patient B port system for G6P in GSD type IB (2, 3). Recurrent infection had respiratory infections, periodontitis, and oral candidi-occurs more prominently in type IB, and neutropenia is another asis. Absolute neutrophil counts ranged from 114 to 25801 distinguishing feature of this type (4, 5). Studies of PMN migramm3. Diminished and delayed migration of PMN into a tion in a patient with GSD type IB and neutropenia indicated skin "window" occurred in B. Random and directed PMN impaired PMN mobilization in vivo and impaired random and migration under agarose toward f-Met-Leu-Phe, pepstatin directed PMN migration in vivo (I). We have further investigated A, and zymosan-activated serum were severely diminished PMN function in two patients and demonstrated abnormalities in both patients. At lo-' M f-Met-Leu-Phe, mean random in metabolic and bactericidal functions as well as motility.and directed migration were 52 and 23% (A, n = 3) and 48 and 13% (B, n = 4) of controls. These results were inde-CASE REPORTS

These results suggest that IgG and IgM anti-LPS mAbs exert protective capacity by extracellular neutralization of LPS, while IgG Fc receptor-mediated cellular uptake also may serve to bypass macrophage activation and TNF-alpha secretion by promoting internalization and intracellular neutralization.

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