An inherited syndrome characterized by recurrent or progressive necrotic soft-tissue infections, diminished pus formation, impaired wound healing, granulocytosis, and/or delayed umbilical cord severance was recognized in four male and four female patients. As shown with subunit-specific monoclonal antibodies in immunofluorescence flow cytometry and 125I immunoprecipitation techniques, in addition to a NaB3H4-galactose oxidase labeling assay, granulocytes, monocytes, or lymphocytes from these individuals had a "moderate" or "severe" deficiency of Mac-1, LFA-1, or p150,95 (or a combination)--three structurally related "adhesive" surface glycoproteins. Two distinct phenotypes were defined on the basis of the quantity of antigen expressed. Three patients with severe deficiency and four patients with moderate deficiency expressed less than 0.3% and 2.5%-31% of normal amounts of these molecules on granulocyte surfaces, respectively. The severity of clinical infectious complications among these patients was directly related to the degree of glycoprotein deficiency. More profound abnormalities of tissue leukocyte mobilization, granulocyte-directed migration, hyperadherence, phagocytosis of iC3b-opsonized particles, and complement- or antibody-dependent cytotoxicity were found in individuals with severe, as compared with moderate, deficiency. It is proposed that in vivo abnormalities of leukocyte mobilization reflect the critical roles of Mac-1 glycoproteins in adhesive events required for endothelial margination and tissue exudation. The recognition of phenotypic variation among patients with Mac-1, LFA-1 deficiency may be important with respect to therapeutic strategies.

Abs sbtract. Investigations ofpolymorphonuclear leukocyte (PMN) function were performed in a 5-yr-old white female with delayed umbilical cord separation, impaired pus formation, and a severe defect of PMN chemotaxis. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis demonstrated an almost total deficiency of a high molecular weight glycoprotein(s) (GP1 38)

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The role of antiviral antibodies in protection against neonatal herpes simplex virus (HSV) infection remains controversial. The relationship between neonatal and maternal anti-HSV antibodies and disease presentation was analyzed in 47 babies. Of the neonates, 77% had localized and 23% had disseminated HSV infection. Antibody-dependent cellular cytotoxic (ADCC) antibodies were evaluated in comparison with HSV neutralizing antibodies. High maternal (greater than 1:10(4)) or neonatal (greater than 1:10(3)) anti-HSV ADCC antibody levels or high neonatal antiviral neutralizing levels (greater than 1:20) were independently associated with an absence of disseminated HSV infection. Cochran-Mantel-Haenszel analysis demonstrated that ADCC levels were associated with disease status (P less than .02) while controlling for the level of neutralizing antibody.

Rabbit antisera to autologous T and R lvmphohlast cell lines HSB-2 and SR, after reciprocal absorption, were found to detect human T and R lymphocyte antigens (HTLA .'nd HBLA antigens). When tested by indirect imnunofluorescence and C' dependent cytotoxicity, the anti-T cell line serum reacted with T cells but not B cells whereas the anti-B cell line serum reacted with B cells but not T cells. These two antisera also reacted with peripheral blood null cells prepared hy nylon column filtration and E rosette depletion. The sum of the percentages of null cells reacting with each antiserum approximated 100%. Null cell suspensions depleted of cells reacting with one antiserum showed a reciprocal increase in cells reacting with the opposite antiserum.These The suppression of CMI during some acute viral infections, including CMV infections, has been postulated to account for the persistence of these viruses in humans and experimental animals. We have developed an experimental animal model in C3H mice to study CHI during this viral infection. The response of splenic lymphocytes from murine CMV(MCMV) infected mice to phytohemagglutinin(PHA) and lipopolysaccharide(LPS) was suppressed during the acute phase of the infection with maximal suppression on day 4 (25% of control for PHA and 28% of control for LPS) and returned to normal by day 15 The mixed lymphocyte reaction(MLR) of splenic lymphocytes from infected animals was suppressed to 38% of control on day 2, to 9% on day 4, and returned to normal by day 15In contrast, lymphocytes responded to MCMV-infected ayngeneic mo se fibroblasts(MEF) by day 2 incorporating 52.183? Y 9866 CPM of H-thymidine compared to 23.58723909 CPM with uninfected MEF. The response to infected MEF increased to 132,560t 22.739 CPM by day LO These data show that lymphocytes responsive to MCMV antigen are generated at the same time that suppression of the response to mitogens and the MLR is observed. These data suggest that the use of non-specific lymphocyte reactions as an index of CMI may provide misleading information and that the suppressions of such reactions may be a result of the regulation of the specific immune response of the host to the viral infection rather than to direct imnunosuppressive effects of the virus itself. NEONATAL ANDROGEN LEVELS AND ANTIBODY PRODUCTION.705 Jean F. Kenny and Pamela C. Pangburn. Univ. of Pittsburgh Sch. of Med., Dept. of Ped., Pittsburgh. Pa. While data suggest that the effects of sex hormones mainly estrogen(E). may be the basis for sex differences in immunity in later 1ife.the reason for the differences in infancy are obscure. Though serum levels of E in male and female babies arelowand not significant,recent data show marked sex differences in serum concentrations(C)of testosterone(T)with levels in males of 0-3months ranging from 350pg/ml to 2000pg/ml and those in females from 50-100pg/ml. To determine what effects these C of T might have on numbers of anti-bacterial antibody producing cells (APC)spleen cells from outbred mcle Swiss mice injected with 3xl...