[Primary immunodefects in Denmark. Report from the Danish registry of primary immunodefects].
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Biomedical subjects
Publications and source records attributed to V Faber.
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Serial assessments of some blood coagulation factors, antithrombin III (AT III), and complement were made in 18 patients with meningococcal (mgc) infection. All patients displayed laboratory evidence of activation of the blood clotting system. Two patients showed clinical signs of disseminated intravascular coagulation. Only AT III differed significantly between patients with and without complications. There was no correlation between changes in blood clotting, activation of the complement system and the course or duration of the disease. These results do not enable one t identify patients who need specific prophylactic therapy. Controlled clinical trials, including administration of heparin, dextran, aprotinin, and others, are still required to ensure optimal treatment in fulminant mgc infections.
Among 130 male patients admitted during a 30-month period with acute hepatitis in the Copenhagen Clinic for Infectious Diseases, 31 were homosexuals. Hepatitis B virus occurred in an endemic fashion in the homosexual subpopulation while hepatitis A infection caused a small epidemic among homosexuals during the winter 1977-78. The course of these infections showed no serological or clinical differences from the findings in 38 heterosexual male patients seen during the same period.
In the present survey 3 groups of patients were studied: 95 homosexual males, 117 heterosexual males and 68 females attending clinics for venereal diseases in Copenhagen. 10 cases of hepatitis B infection were found, all in homosexual males. Chronic carriership of hepatitis B surface antigen (HBsAg) and presence of hepatitis B e-antigen (HBeAg) was demonstrated in 8 and 5 cases, respectively and liver biopsy showed chronic liver disease in 5 out of the 8 former. The prevalence rate of hepatitis B antibody was significantly higher in all patient groups than in the general population in the area. Two patients had non-B hepatitis, 1 homosexual male and 1 female, but the prevalence rate of hepatitis A antibody (anti-HAV) was not increased in the venereal clinic population. The serological tests identified certain high risk groups regarding hepatitis B infection: (1) homosexual males > 25 years with anti-HAV and a high promiscuity; and (2) heterosexual males and females > 25 years with a past history of venereal disease and clinical hepatitis. The data are compatible with the hypothesis that sexual transmission plays an important role in the spread of hepatitis B infection. It is recommended that routine serological tests for hepatitis be taken in the high risk groups of venereal clinic populations.
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Ten cases of hepatitis B virus infection were identified among asymptomatic male homosexuals. These patients shared a number of characteristics: A subclinical origin and course of infection; Persistence of HGsAg for periods exceeding six to 25 months; Persistent GPT elevation of two to five times upper normal limit; Morphological changes in the liver with portal and parenchymal inflammation (chronic persistent hepatitis, six cases; non-specific reactive hepatitis, 2 cases; cirrhosis and acute hepatitis with signs of chronicity, one case each). HBeAg was found in six cases, anti-HBe in none. These results indicate that screening for hepatitis B should be performed whenever these individuals come under medical attention in order to detect asymptomatic chronic liver diseases and to detect these silent vectors of an infection that presently shows an increased frequency among homosexuals.
Severe combined immunodeficiency (SCID) was diagnosed in a girl immediately after birth; her older brother had SCID and was successfully reconstituted by bone marrow transplantation from his uncle. She was isolated in a laminar air flow bench and decontaminated. The father differed by one HLA-A antigen but was HLA-Dw2 homozygous like the patient; his lymphocytes showed a slight response to the patient's cells in mixed lymphocyte culture (MLC). At the age of 2 1/2 months and again at 5 months, she was given a bone marrow transplant from the father. During the entire course the patient had no infections, and apart from a transient eosinophilia she had no signs of graft-versus-host reaction. Immunological reconstitution was nearly complete at 9 months of age, when she was recontaminated. One year later plasma immunoglobulin concentrations are in the low normal range (IgG and IgM) or decreased (IgA); tests of cell-mediated immunity are normal. Apart from slight upper respiratory infections, the patient has been healthy. Physical and psychological development have been normal.
Consecutive serum and plasma samples, from a patient receiving 100 ml polyvalent horse anti-venom globulin after a rattlesnake bite, were analysed for circulating immune complexes (IC) and activation of complement factors. IC were determined by two independent methods, a complement consumption assay and a Clq-binding assay. Rapidly rising levels of complement-fixing circulating IC were detected as early as 4--5 days after the serum treatment and distinct IC-activity was recorded in both assays on day 8 when clinical symptoms of serum sickness were observed. The IC remained in circulation for at least 5 weeks. Signs of intravascular C-activation in the form of low C3, C4 and C5 values was noted on day 1 after treatment. Factor B was demonstrable 3--4 days after the snake bite and this factor and C3c attained a peak around day 8, just before maximal suppression of native C3 and C4. 14 days after the globulin treatment C3c and B were declining rapidly while C3 and C4 approached normal values first 36 days after treatment. An increase in heterophilic antibodies to sheep erythrocytes was observed after treatment with anti-venom globulin.
Antibodies to tissue components were studied by the indirect immunofluorescent method in 24 patients with infectious mononucleosis (IM). Smooth-muscle antibodies (SMA) were found in 70.8% of the patients, and in only one (3.8%) of 26 controls. SMA in patients were of the IgG (54.2%), the IgA (12.5%) and the IgM (41.6%) class. IgM-SMA occurred in the early phase of the disease, while IgG-SMA were found both early and late in its course. It was not possible to demonstrate that the development of SMA was due to liver involvement. Antinuclear antibodies, parietal-cell antibodies and antibodies to the cytoplasm of renal tubular cells were not detected significantly more often in patients than in controls. The serum concentrations of IgG, IgA and IgM were elevated in the acute phase of IM, and it was found that the Ig values were higher in patients with SMA than in those without.
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