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Biomedical subjects

E Genth

Publications and source records attributed to E Genth.

At least 19 recordsLinked to original sources

A human monoclonal IgA rheumatoid factor using the VkIV light chain gene.

A human hybridoma stably secreting IgA rheumatoid factor (RF) was produced by cell hybridization with peripheral blood lymphocytes of a patient with rheumatoid arthritis. The RF was of the IgA1 isotype with kappa-light chains and was useful for standardization or specificity controls in class-specific RF assays. RF activity was detected only when the IgA molecular were in a polymeric state, and could be measured by enzyme linked immunosorbent assay as well as in conventional agglutination based tests. The RF had the modified Ga fine specificity described previously for several RFs and for protein A. The immunoglobulin V genes used were isolated and sequenced. The light chain was encoded by the VkIV gene rearranged to Jk2; compared to the published VkIV germ line gene there was 90% nucleotide homology. The heavy chain gene used belonged to the VHI family and was rearranged to JH4. Comparisons with published sequences revealed 90% homology with the recently characterized VH gene expressed by RF-TS3, a rheumatoid synovia RF hybridoma.

Aged

Immunogenetic associations of scleroderma-related antinuclear antibodies.

Patients selected for the presence of scleroderma-related antibodies (anti-DNA-topoisomerase I [anti-topo I; n = 43], anticentromere antibody [ACA; n = 63], or anti-Pm-Scl [n = 12]) were studied for class I and class II major histocompatibility complex antigens, as well as for Gm and Km allotypes. Anti-topo I was associated with HLA-DR5 (70% of patients versus 30.6% of controls; Pcorr = 0.0018, relative risk [RR] = 5.3). All patients with anti-Pm-Scl were positive for HLA-DR3 (versus 23.5% of controls; Pcorr less than 0.001); 6 of these patients were DR3/4 heterozygous (50% versus 3.5% of controls; Pcorr less than 0.001, RR = 27.3). Patients with ACA were frequently positive for HLA-DR1, DR4, or DRw8, with 73.7% demonstrating at least 1 of these alleles (versus 41.2% of controls; Pcorr = 0.0152, RR = 4.0). This group of ACA-positive patients who had DR1, DR4, and/or DRw8 consisted mainly of a subgroup of patients with rheumatoid arthritis. We conclude that different class II major histocompatibility complex antigens influence the formation of anti-topo I and anti-Pm-Scl. Important clinical differences between these patient groups and the immunogenetic heterogeneity support the notion of different antibody-defined scleroderma subsets.

Antibodies, Antinuclear

[Streptokinase therapy in a patient with lupus anticoagulant and thrombosis of the A. iliaca externa].

We report on a 26-year-old male who suffered from thrombocytopenia, factor V-deficiency and punctuate hemorrhages during the past. On admission the patient presented with acute left iliac artery occlusion. The walking distance was reduced to 200 m. Laboratory findings showed the existence of a lupus anticoagulant (LAC). As a therapeutical attempt a systemic lysis with high doses of streptokinase (9,250,000 I.E.) was carried out which was followed by a successful catheter lysis (250,000 I.E. streptokinase). During lysis the patient didn't show any unusual hemorrhagic events. Arterial thrombosis together with a lupus anticoagulant can be treated with high doses of streptokinase.

Adult

Antigenic determinants shared between HLA-A, -B, -C antigens and H-2 class I molecules modified by bovine beta-2 microglobulin.

The specificity of the mouse class I-specific antibody COB6-3 was examined in detail. It was found to react with the mouse class I molecules H-2Db, Kd, and Qa-2, and with human HLA-A, -B, -C antigens. The specificity pattern of COB6-3, despite its different origin, was similar to that of the monomorphic HLA class I-specific antibody W6/32. Cross-inhibition studies show that on human cells the antigenic determinants recognized by the two antibodies are situated close together and may be identical. On mouse cells, reactivity of both antibodies was generated upon replacement of mouse beta-2 microglobulin (B2m) with its bovine counterpart, but differences in specificity were observed using human B2m.

Animals

HLA-DR4 and Gm(1,3;5,21) are associated with U1-nRNP antibody positive connective tissue disease.

Patients with U1-nRNP antibodies (n = 35, 31 female, four male) were typed for HLA-A, -B, -C, and -DR antigens and IgG heavy chain allotypes G1m(1), -(2), -(3), G3m(5), and -(21). The patient group was clinically heterogeneous. Four met the American Rheumatism Association criteria for systemic lupus erythematosus, six for progressive scleroderma, and 14 for rheumatoid arthritis. Sicca syndrome was present in seven cases. Twenty three had overlapping features compatible with mixed connective tissue disease (MCTD). Healthy blood donors served as controls for HLA typing (n = 64), Gm typing (n = 228), or both (n = 56). Sixty six per cent of the patients with U1-nRNP antibodies were DR4 positive compared with 28% of the controls (relative risk = 4.9, p = 0.00053). The Gm(1,3;5,21) phenotype was found in 46% of the patients and 25% of the controls (relative risk = 2.47, p = 0.0247). Within the patient group Gm(1,3;5,21) was found only in DR4 positive individuals. The coincidence of HLA-DR4 and Gm(1,3;5,21) increases the relative risk values to 8.0 (compared with the group with neither risk factor). DR4 and Gm(1,3;5,21) primarily seem to be related to U1-nRNP antibody formation and not to disease expression. Patients with or without MCTD did not differ with respect to DR4 or Gm(1,3;5,21) frequency. Disease onset was earlier in patients with HLA-DR4/Gm(1,3;5,21) than in patients without both markers (mean 27.9 v 40.1 years; p less than 0.05).

Adolescent

[A contribution to the pseudo-LE-syndrome. Investigations of the cardiovascular system, abdominal organs, lymphatic system and drug histories (author's transl)].

13 of 30 patients suffering from pseudo-LE-syndrome showed a usually reversible enlargement of the heart during the acute stage of the disease. In two patients carditis occurring in pseudo-LE-syndrome lead to congestive heart failure and an additional patient died in the acute stage of carditis. As opposed to Systemic Lupus Erythematosus hypertension did not occur in a higher frequency than normal. In five cases cardiac catheter examinations showed slight elevation of the end-diastolic pressure in the right ventricle, in two cases an abnormal high mean pressure in the right atrium and a systolic gradient at the pulmonic valve was found. -Scintigrams showed definite enlargement of the spleen and to a lesser degree enlargement of the liver was seen. Laparascopy showed multiple concretions after peritonitis. Lymphographic changes in the retroperitoneal lymph nodes and lymphatic ducts were not observed in contrast to rheumatic diseases. Drug histories in most cases discovered intake of Venopyronum dragees prior to onset of the disease. But recurrent attacks of the disease also occurred without further intake of the drug.

Adult

E1Lymphocytopenia in pseudo-lupus-erythmetaosus syndrome.

Seven female patients with pseudo-lupus erythematosus (LE)-syndrome had markedly reduced lymphocyte counts in their peripheral blood during the active phase of the disease. One patient, we were able to study during the active phase of her disease, had a diminuation of spontaneous rosettes-forming (T-)lymphocytes, using neuraminidase-treated sheep red blood cells for the test. In this case the percentage of surface-immunoglobulin-bearing (B-)lymphocytes determined by an indirect immunofluorescence technique was augmented. In comparison with 20 normal controls 6 other patients did not show any alterations in the relation of B- and T-lymphocytes in the peripheral blood. 5 patients had circulating lymphocytotoxic antibodies in their serum. A specificity of these antibodies for T-lymphocytes could not be realized.

Acute Disease