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Biomedical subjects

M Abbal

Publications and source records attributed to M Abbal.

At least 91 records · Page 5Linked to original sources

[HLA and Bf in idiopathic nephrotic syndrome in children: differences between corticosensitive and corticoresistant forms].

An association between HLA-DR7 and the steroid sensitive idiopathic nephrotic syndrome in the children has already been reported. Immunogenetic data in the less frequent steroid resistant form of this disease have never been published. In this study, we analyse HLA-A, B and DR typing in 99 cases of nephrotic children divided in 72 with the steroid sensitive (SS) form and 27 with the steroid resistant (SR) syndrome, in comparison with those of 207 healthy controls; Bf allotypes were determined in 53 of the patients. The results show the increased frequency of DR7 in the SS syndrome (75% vs 30%, RR = 6.9, pc less than 10(-6), while the SR one is more associated to DR3 (52% vs 27%, RR = 3, p less than 0.004). In the SS patients, atopy is associated to DR7 (p less than 0.001), which is not the case in the SR group. Furthermore, a high relative risk is associated to the phenotype DR3/DR7 (30% vs 4%; RR = 9.3; pc less than 0.0004), for the SR disease; besides, this phenotype is associated to an early onset of the disease and to lesions of focal sclerosis. Thus a heterozygous effect in the SR form of idiopathic nephrotic syndrome of children has been demonstrated; the steroid sensitive and the steroid resistant forms of the disease seem to have different immunogenetic components.

Adrenal Cortex Hormones↗

[Analysis of the cerebrospinal fluid by isoelectrofocusing on agarose in multiple sclerosis].

Intrathecal synthesis of immunoglobulins can be proved by means of two methods: quantitatively by immunoglobulins titration in CSF, the results expressed with several ratios; qualitatively by demonstration of oligoclonal distribution of gammaglobulins. IEF is the most sensitive of the qualitative methods. From a technical point of view Agarose Isoelectrofocusing seems to be a better method than polyacrylamide isoelectrofocusing and permits, when the interpretation is difficult, immunofixation into the gel. The authors report the results of a comparative study between the evaluation of the IgG Index and agarose isoelectrofocusing of 281 CSF divided into 113 CSF from patients with Multiple Sclerosis (MS) and 168 CSF from patients with other neurological diseases (OND). Sensitivity of IEF was higher than IgG index to prove intrathecal IgG synthesis: in the group of patients with MS, 91 p. 100 of CSF were abnormal instead of 72 p. 100 of IgG Index. In the group of patients with OND, abnormalities in IEF were low (5 p. 100) but the number of inflammatory diseases was poor. These results were similar with the findings of many authors using the same methods. In our opinion, IEF is the best technique which a specialized laboratory can use in routine to prove an immunoglobulin intrathecal synthesis.

Central Nervous System↗

Two subtypes of BfF by isoelectrofocusing: differential linkage to other HLA markers.

By isoelectrofocusing in agarose, the properdin factor allotype BfF could be split into two subtypes: BfFa with one major cathodic band and BfFb with the same cathodic band but in addition a major anodic band. By scanning, BfFaFb heterozygotes were distinguished from BfFbFb homozygotes by the stronger intensity of the anodic band in the latter. The two subtypes were segregated perfectly with HLA in 40 families and showed different association patterns with HLA markers. BfFa seemed to be linked to B35 while BfFb showed a strong linkage with all the components of the following haplotype: HLA-A29, Cw-, B44, BfFb, C4A3B1, DR7. The frequency of BfFa among BfFS heterozygotes was 41% (46/113) and that of BfFb 59% (67/113).

Alleles↗

Recombination between HLA-A and C and between HLA-B and complement locus C4 in the same individual.

In a French family with 2 parents and 5 children a crossing over was found in the HLA region on both of the parental haplotypes of one of the children. The following markers were studied: HLA-A, B, C,DR, DQ(MB), DP(SB), complement allotypes C4 and Bf and glyoxalase I polymorphism. In the third child, the paternal haplotype had a recombination between HLA-A and HLA-C and the maternal haplotype a recombination between HLA-B and complement locus C4. Mixed lymphocyte cultures confirmed the serological findings and non-HLA markers (blood groups and immunoglobulin allotypes) showed no evidence of extrapaternity. The family also demonstrates a probable duplication of the C4B1 gene in one of the paternal haplotypes.

Complement C4↗

[High blood triiodothyronine and the euthyroid state].

A high excess of circulating T3 was observed in an euthyroid woman. Agarose gel electrophoresis of serum preincubated with 125I-T3 revealed an abnormal T3-binding in gamma-globulin zone. This binding interfered with the hormone radioimmunoassay. Immunological characterization identified this protein as an IgG-K and IgG-lambda polyclonal antibody that bound T3 but not T4. Scatchard analysis of 125I-T3 binding to the gamma-globulin fraction isolated showed a single class of binding sites with a high affinity Ka = 0.4 X 10(9) L/M and maximal binding capacity of 5.2 X 10(-9) M.

Aged↗

A DNA restriction fragment length polymorphism in the complement region of the human MHC shows an absolute correlation with polymorphism of complement factor B(Bf) defined by isoelectric focusing.

The gene coding for properdin factor Bf is located in the human major histocompatibility complex and is closely linked to the genes coding for the complement components C2 and C4. Recently, by Southern blotting techniques, a restriction fragment length polymorphism was identified using the endonuclease Taq I, which subdivides haplotypes carrying the F allele of factor Bf. The F allotype has also been subdivided at the protein level by isoelectric focusing into two subtypes Fa and Fb. We have investigated the DNA of 41 healthy unrelated individuals with known BfF subtypes using the 2.3 kb factor Bf cDNA probe to determine if there is any correlation between the Taq I polymorphism and F subtype. We have found that in 23 individuals who carried the Fb subtype a 6.6 kb Taq I fragment was present. The remaining 18 individuals carried the Fa subtype and showed only the 4.5 kb Taq I fragment on Southern blotting (P = 10(-12). This striking correlation (r = 1) between the Fb protein and DNA polymorphism is surprising especially as the 4.5 kb and 6.6 kb Taq I fragments overlap the Bf and C2 genes and the polymorphic Taq I site is located within the C2 gene.

Alleles↗

[Quantitative determination of FTA abs IgM in different stages of syphilis before and after treatment].

The titres of treponemal specific IgM and IgG antibodies were determined by monospecific immunofluorescence (FTA abs IgM/IgG) on 191 sera from 107 patients with treated or untreated syphilis, at various stages and from 10 neonates born from seropositive mothers. IgM antitreponemal antibody was found in all cases of untreated syphilis but two neurosyphilis. After treatment the antibody usually disappeared within one year. However it persisted in three patients treated two years ago or more. When sera from these patients were fractionated by density gradient ultracentrifugation and examined for antitreponemal antibodies of the IgM classe (FTA abs IgM 19S) they never showed detectable concentrations of IgM. False reactive results as false non reactivity due to competitive inhibition, observed with FTA abs IgM can be eliminated after separation of immunoglobulins.

Adult↗

HLA-A, B, C, DR antigens, Bf, C4 and glyoxalase I (GLO) polymorphisms in French Basques with insulin-dependent diabetes mellitus (IDDM).

The Basques were previously shown to present a high frequency of HLA-B18 and BfF1, which are known to be associated with insulin dependent diabetes mellitus (IDDM). During the VIII International Histocompatibility Workshop, we studied HLA-A, B, C, DR; Bf, C4 and GLO.I polymorphisms in 51 unrelated French Basque IDDM patients and in 50 controls. Haplotypes were established by family studies in all controls and some patients. Two haplotypes were frequently found in the controls: HLA-A1, Bw57, BfS, C4 F1S, DR7 and HLA-Aw30, Cw5, B18, Bf F1, C4Fs degree, DR3. The first one was not found in the patients. All the components of the second haplotype had increased frequencies possibly as a consequence of linkage disequilibrium with HLA-DR3: a highly significant association between IDDM and HLA-DR3 was observed (90.2% vs 24.0%, relative risk (RR) = 29.1, P less than 10(-11)). The HLA-DR4 frequency was slightly increased (37.3% vs 16.0%), and HLA-DR2 was not found. The silent allele C4s degree was particularly associated with early diagnosed IDDM (86.7% in patients with age at onset under 20 years vs 57.1% in other patients, P less than 0.02). The high relative risk for HLA-DR3/DR4 heterozygous vs that of individuals, possibly HLA-DR3 homozygous, supported the hypothesis that two HLA-DR linked genetic factors could be involved in the inheritance of IDDM susceptibility.

Adult↗

Comparison of progressive antithrombin activity and the concentration of three thrombin inhibitors in nephrotic syndrome.

In order to compare the plasmatic progressive antithrombin activity to the concentration of three thrombin inhibitors, antithrombin III (AT III), alpha 2 macroglobulin (alpha 2 M), alpha 1 anti-trypsin (alpha 1, AT) in nephrotic syndrome, a prospective study was carried out on a group of 28 children affected with the disease. A dramatic reduction of the level of AT III and of alpha 1 AT, two inhibitors of molecular weight close to that of albumin, was observed. The decreased level of AT III was counterbalanced by an increase in alpha 2 M. This phenomenon accounts for the increased progressive antithrombin activity observed in all the affected children. It is suggested that the above compensatory mechanism explains the absence of thrombotic accidents in this series and that the benefit of heparin therapy is doubtful in these conditions.

Adolescent↗

Detection of J chain in lymphomas and related disorders.

Lymph node specimens from 125 patients with malignant lymphomas and related disorders were studied by immunoperoxidase procedure for the presence of intracytoplasmic immunoglobulin (CIg) and J chain CIg staining was present in 22/24 cases of lymphoplasmacytic-lymphoplasmacytoid lymphomas, and in 10/10 cases of extramedullary plasmacytomas and myelomas. In the majority of these cases J chain could be demonstrated in plasmacytoid or neoplastic plasma cells. In 21/36 cases of immunoblastic lymphomas, intracytoplasmic Ig staining was present. In only two of the 36 cases were the lymphomatous cells stained positively for J chain. J chain was not detected in other lymphomas such as lymphocytic lymphomas, follicular lymphomas, lymphoblastic lymphomas or in Reed-Sternberg cells or hairy cells. J chain was demonstrated in mature plasma cells and immunoblastic cells in hyperplastic lymph nodes, and in angioimmunoblastic lymphadenopathy. These findings show that J chain is not detectable in all B cell lymphomas even in the presence of CIg synthesis, irrespective of class.

Cytoplasm↗

[Adult coeliac disease and very high IgA plasma level (author's transl)].

The authors report a case of malabsorption syndrome with jejunal atrophy in a 63 years old woman. Coeliac disease was suspected from clinical and histological features, clinical evolution with diet, and presence of HLA B8 antigen. However, this case was very particular because the infiltration of lamina propria was very dense and especially composed of plasmocytes, and because there was a very important and polyclonal rise of immunoglobulin A (32 g/L) in the serum. A review of the literature allows to consider the immunopathology of coeliac disease. Such a case was never found among the adults. Two similar observations have been described in children.

Adolescent↗

High frequency of the properdin factor Bf F1 and its linkage to HLA in French Basques.

We studied 201 unrelated French Basque individuals for HLA and Bf polymorphisms. The haplotypes of eighty-seven of them were deduced from family studies. The results show the frequency of the Bf F1 allele (0.1393) which is the highest one currently reported. They confirm the high frequencies of HLA-Aw19.2 and B18 previously reported in that population and show that a whole haplotype with strong linkage disequilibria, namely Aw19.2, Cw5, B18, Bf F1, DRw3 is frequent. On the other hand, the gene frequency of Bf S is decreased (0.5497) as compared with the other European Caucasoïd populations, while a slight increase in the Bf F gene frequency (0.2960) appears. These results point out that it is of importance to consider the genetic background choosing the population where linkage disequilibria are to be studied.

Alleles↗

Antiglobulins in normal human sera which react with baboon Ig G.

Antiglobulins of the Ig M class which react with the Ig G of baboons have been discovered in approximately half the number of normal human sera investigated by the technique of hemagglutination of red blood cells coated with baboon immune serum. The frequency of these antiglobulins is significantly higher among patients suffering from seropositive rheumatoid arthritis but there is no correlation with the presence of Milgröm-type antiglobulins. The inhibition of several sera containing these antiglobulins by the sera of baboon of different species is also reported.

Animals↗

Delayed hypersensitivity to human encephalitogenic protein as assayed by agarose leucocyte migration in multiple sclerosis patients.

Using a leucocyte migration test (Clausen's direct agarose gel migration method) hypersensitivity to human encephalitogenic protein has been examined in 50 multiple sclerosis patients (group 1), 50 healthy persons (group 2) and 25 patients with other neurological diseases (group 3). In group 1, 30 MS patients (60%) show an abnormal migration index, manifested either as inhibition or stimulation of migration; 29 controls in group 2 (58%), 11 O.N.D. patients in group 3 (44%) show an abnormal migration index. These results mean that lymphocyte hypersensitivity to myelin basic protein appears neither to be constant nor specific to multiple sclerosis. Three migration index curve types at different antigen concentration are obtained: monophasic curves within the normal index zones; monophasic curves staying in the inhibition or stimulation zone and biphasic curves with dose-effect relationship. Whatever the antigen used, this dose-effect relationship implies that the test must be carried out at different concentrations. The meaning of spontaneous sensitisation in healthy controls is discussed.

Adolescent↗

[Critical study of techniques for the detection of "light" rheumatoid factors (author's transl)].

In a number of cases of true rheumatoid arthritis, it is not possible to demonstrate the presence of 19 S "heavy" rheumatoid factors by the Waaler-Rose and globulin latex techniques. However, in some of them, 7 S rheumatoid factors are suspected. Three techniques for the detection of these "light" rheumatoid factors are described and critically evaluated. The authors report their own experience of the immuno-absorption and indirect immunofluorescence technique performed on 156 sera from RA patients. Immuno-absorption is not felt to be a reliable technique on several grounds. Indirect immunofluorescence is of value only in the diagnosis of seronegative rheumatoid arthritis where it provides information in addition to that offered by classical techniques. It is positive in 53.3% of seronegative rheumatoid arthritis sera.

Arthritis, Juvenile↗

[Von Willebrand factor activity and thrombophilic states. A review (author's transl)].

After a brief survey of some physiologic properties of Von Willebrand factor activity (VIII r WF) the numerous clinical states with an increase of this activity are described. The authors report their own results concerning sepsis, chronic arteritis of lower limbs, diabetes, toxemia of pregnancy. In most cases the increase of VIII r WF activity appears as a sign of vascular injury and/or the consequence of tissular damage.

Arteriosclerosis↗