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J Seignalet

Publications and source records attributed to J Seignalet.

At least 55 records · Page 3Linked to original sources
PubMed1979

Hereditary angio-oedema and C3 nephritic factor--HL-A study.

In one family of twenty-four members hereditary angio-oedema was present in the family for six generations. The protein C1 esterase inactivator found in nine patients proved to be non-active in a functional test. Another anomaly found in the complementary system was labelled C3 nephritic factor without any renal, or other clinical symptoms. Study of HL-A haplotypes did not show any linkage with the loci A, B and C. Hereditary angio-oedema is a disease arising from a specific defect in the inactivator of the C1 esterase (C1 INA) which is a regulating component of the complement system (Donaldson & Evans, 1963). This system is of current interest because of its interaction with other mechanisms of inflammation. Moreover, some links have been discovered recently between HL-A and hereditary defects of complement. This paper reports new findings in a family with hereditary angio-oedema.

Angioedema↗
PubMed1979

[Physiopathology of rheumatoid psoriasis].

The physiopathology of rhumatoid psoriasis (RP) is poorly known. Besides the genetic factors we study immunological disorders from a series of 51 cases. We found mainly an increase of serum IgA, a decrease of IgM, a low level of circulating immune complexes, sometimes IgG type antiglobulin factor (very seldom IgM type, which explains the sero-negativity) seldom antinuclear antibodies; we found also a diminution of T lymphocytes detected by a decrease of E rosette - forming cells and an increase of the lymphocyte subpopulation forming high avidity EA rosettes, whereas the mitogenic response is normal or slightly decreased in the presence of Con A (not significant). A increased frequency of HLA DRW5 and DRW2 was found but too few cases were studied to make possible a definite conclusion. This immunopathologic profile is compared to that of psoriasis with anthropathy. The relevance of immune disorders in the physiopathology of RP is discussed.

Adolescent↗
PubMed1978

[Mo66, a new allele of the HLA-B locus. Preliminary note (author's transl)].

Mo66 is an allele of the HLA-B locus, which is demonstrated by HLA typing of 2 000 unrealted individuals and the members of eight informative families. Mo66 is a rare antigen, with a frequency of 0.65 % in the Languedocian population. Mo66 shows a strong association desequilibrium with HLA-A3. The identification of Mo66 is difficult, without a monospecific serum, because this antigen is united by cross-reactions above all with HLA-B13, but also with HLA-Bw40, HLA-B27, HLA-B12 and HLA-B7. The presence of an anti-Mo66 antibody in some apparently anti-HLA-B27 monospecific sera can provoke some errors in the diagnosis of ankylosing spondylitis and related diseases.

Alleles↗
PubMed1978

[HLA and IgA deficiency].

The distribution of 29 HLA-A and B antigens was compared in 50 Caucasoïds with an IgA deficit and in 300 healthy controls. The patients were divided in 3 groups: 1) Partial selective IgA deficit (40); 2) Total selective Iga deficit (7); 3) IgA deficit associated with hypogammaglobulinemia (3). The patients viewed as a whole, we observed an increased frequency for the antigens HLA-Aw19, HLA-B5 and HLA-BW17. Yet, the modifications are not cleanly significant, with p less than 0.05, but p corrected not significant. We also considered the 3 groups separated and we did not remark any particular association with HLA. The data concerning HLA and congenital immune insufficiencies are reviewed. The most authors at once studied several immune defects. Only one Hungarian work was performed on IgA deficit. We do not confirm HLA-A1 and HLA-B8 increased frequencies, as it was reported, in Hungary, by Bajtai and al. There is no evident association between one HLA-A or B gene and the IgA deficit. The possible relation of IgA insufficiency with autoimmunity and allergy would justify complementary investigations, especially about HLA-D and Ia genes repartition in this disease.

Adolescent↗
PubMed1977

[HLA system and rhizomelic pseudopolyarthritis].

26 HLA antigens of the loci A and B were studied in 50 patients with pseudo-polyarthritis and in 300 control subjects without any joint disease. The arthritis was isolated and not associated with temporal arteritis. An increase in frequency of both HLA antigens was noted in the patients studied: HLA-B5 = 24% as against 13% in controls (P = 0.05 and Pc NS). HLA-Bw38 = 18% as against 5.33% in controls (p = 0.002 - Pc = o.05). The rise in frequency of HLA-B5 and HLA-Bw38 was also found in 19 subjects with polyarthritis and temporal arteritis but not in 31 patients with temporal arteritis alone. In this disease, a link with the HLA-B14 antigen was noted in 50 cases (22.9% as against 8.6% in control). These results suggest that arthritis and temporal arteritis although sometimes associated are probably distinct diseases.

Aged↗
PubMed1977

Psoriatic arthritis and HLA antigens.

HLA groups including the characteristics of 25 antigens were determined in 108 patients suffering from psoriatic arthritis. These included 18 patients with central forms (pelvospondylitis), and 90 patients with peripheral forms (polyarthritis with or without sacroiliitis). Analysis of the results leads to the following conclusions: central psoriatic arthrisis is strongly associated with B27 and BW38, less closely with B13 and slightly with BW17. Peripheral psoriatic arthritis has the same relationship with the HLA system as has psoriasis without arthropathy.

Arthritis↗
PubMed1976

HL-A antigens in insulin-dependent diabetes mellitus.

HLA antigen typing by lymphocytotoxicity was performed for 2 groups of unrelated caucasian subjects. The first group was composed of 100 subjects with insulin-dependent diabetes mellitus. The second group was composed of 270 healthy subjects without diabetes. Our study has shown that for the inhabitants of Languedoc the BW15 antigen is not the most frequently found (11% of the diabetics, 10% of the healthy subjects) contrary to findings reported by others. The B8 antigen was the most frequently found (20% of the diabetics, 16,3% of the healthy subjects), in agreement with the findings of the same authors. The frequency of BW15 and B8 found simultaneously was increased (observed 3%, expected 1.38%). The principal findings of our study were a significantly increased frequency of Da25 and B18 (23% and 25% for the diabetic subjects, 11.26% and 12.22% for the healthy subjects), and a significantly decreased frequency of A11 and B12 (6% and 18% for the healthy subjects). The association of Da25 and B18 was observed for HLA phenotypes, suggesting a higher incidence of the Da25-B18 haplotype. For the diabetics, the types BW15, BW40 and Da25-B18 have little hereditary character, and are rapidly insulin dependent individuals.

Adult↗
PubMed1976

[HL-A antigens in dust allergy in children].

The distribution of 29 HLA antigens has been compared in 60 unrelated children presenting a dust allergy and in 300 healthy controls. We observed an increased frequency for HLA-Aw19 and HLA-B5 in patients. Yet, the differences are not very significant and there is probably no association between one HLA gene and the dust allergy.

Adolescent↗
PubMed1976

[HL-A W27 antigen and atypical rheumatic pelvispondylitis].

The authors report 26 cases of atypical inflammatory rheumatism in which the discovery of HL-A W27 antigen indicated the possibility of atypical ankylosing spondylarthritis. These patients included 17 men and 9 women with an average age of 35.6 years. The clinical symptoms included :--pelvic or vetebral signs alone in 8 cases,--pelvic or vertebral signs combined with peripheral inflammatory rheumatism, the latter being always cleaarly evident, in 9 cases,--extravertebral signs alone without any involvement of the vertebral column or of the sacroiliac joints in 9 cases (8 cases of peripheral inflammatory rheumatism, 1 case of talalgia). The vertebral radiograms were normal in 84 percent of cases. The sacroiliac joints were clear radiologically in 65 percent of cases. In the other cases the lesions, generally unilateral, were extremely discrete. In all the cases, the Waaler-Rose reaction was negative. The therapeutic test with non-hormonal anti inflammatory products were generally positive. The evolution of the condition confirmed the diagnosis of rheumatic pelvispondylitis in 2 cases. The patients have been under observation for insufficient time to be sure whether all the cases presented represent authentic cases of ankylosing spondylarthritis that were at first atypical. The authors emphasize the high percentage of female cases (38 percent) the high frequency of extra-vertebral manifestations. They also emphasize the value of looking for HL-A W27 antigen in patients with atypical inflammatory rheumatism.

Adolescent↗
PubMed1975

[Practical significance of HL-A groups in rheumatology].

Since 1972, several relations have been demonstrated between some HL-A antigens and some articular diseases. The W27 antigen frequency is highly increased in ankylosing spondylitis (88%) and in Reiter disease (78%) compared with controls (5%). In peripheral forms of psoriatic rheumatism, the W17 and HL-A13 antigens are more fréquent (24% and 15%) than in healthy subjects (4% and 5%). In central forms of psoriatic rheumatism, there is a relation with W27 (48%) and still, we do not know if this association concerns only spondylitis or also sacro-ileitis. The HL-A typing may be useful for the diagnosis of some rheumatic diseases, when they present atypical appearances. W27 possesses a considerable value for the diagnosis of ankylosing spondylitis. The relation between W27 and ankylosing spondylitis is clearly stronger than that between Waaler-Rose reaction and rheumatoid arthritis.

Arthritis, Reactive↗