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J Hors

Publications and source records attributed to J Hors.

At least 109 records · Page 6Linked to original sources
PubMed1986

[Risk factors in insulin-dependent diabetes].

The relative risks (RR) of the immunogenetic markers of insulin-dependent diabetes mellitus (IDDM) have been calculated in a population of 235 IDDM patients compared with a control population. The highest relative risk was that of subjects heterozygous DR3/DR4 (RR = 47, P less than 0.001) which was still more increased in those who carry this combination associated with the RFLP cluster DQR4 (RR = 72). Further, a sample of 51 secondary affected siblings of IDD index cases has been compared with 265 non affected siblings (one child of each family, excluding index cases). The highest risks have been found in addition to DR3/DR4, for DR3 alone and particularly for the combination C4BQ0, DR3 (RR = 9, p less than 0.001) suggesting a role for this peculiar association in the familial penetrance. In the group of siblings HLA-identical with the index case, only two factors showed some capacity of discriminating between affected and non affected siblings: HLA-DR3 and age (less than or equal to 10 years old at onset of IDDM in the index case) (p less than 0.01). In the group of haploidentical siblings, the combination DR3/DR4 and the associations C4BQ0, DR3 and BfF1, DR3 significantly increased the susceptibility for higher familial occurrence of the disease. If confirmed by additional family series, this scale of risk factors could be helpful in predicting risk of IDDM to siblings of diabetic children.

Diabetes Mellitus, Type 1↗
PubMed1986

[HLA antigens and different clinical forms of 21-hydroxylase deficiency in the French population].

HLA associations with 21-OH deficiency were studied on respectively 109 and 60 congenital and late onset French index cases. Significant negative associations were found with antigens B8: congenital forms; B5, DR3: late onset. Significant positive associations were observed with A3, Bw47 (A3 Cw6 Bw47 DR7): congenital forms; B40: salt-wasting form; B5: simple virilizing form; Aw33, B14, DR1, DR2, DRw6 (Aw33 B14 DR1): late onset form. Among late onset patients not bearing B14 antigens significant positive associations were observed with B12 and B35.

Adrenal Hyperplasia, Congenital↗
PubMed1986

[Organ transplantation in France in 1984 (activity report of France-Transplant)].

Nine hundred and seventy four renal transplantations were performed in 1984, thus attaining a total 7927 grafts made in France at the end of 1984. The numbers of heart transplantations (77), to which 100 prospects in 1985 can be added, as well as liver grafts (13) plus 50 prospects in 1985, are showing a spectacular increase. Although the considerable improvement of heart graft and liver graft results can be attributed, essentially to the use of Cyclosporine, this trend can be dependent in the case of kidney transplantations also on transfusions before and on a better HLA-B DR compatibility which can in optimal situations represent a respective gain of 10% and 15% of graft survival.

Adult↗
PubMed1986

Effects of HLA genotype, age and birth order on empirical risk estimates for insulin-dependent diabetes in siblings of diabetic children. An actuarial evaluation.

The incidence of insulin-dependent diabetes (IDDM) was evaluated as a function of time of follow-up among the 371 siblings of 193 diabetic children using actuarial methods and comparisons were carried out according to HLA genotype, sex, age and birth order. The following parameters appeared to be relevant for empirical discrimination in terms of the risk of recurrence of the disease among siblings: The number of HLA-haplotypes shared with the first affected sibling considered as the proband. Cumulative proportions 15 yr after onset of IDD in the proband were 35%, 20% and 3% in the case of 2, 1 and 0 haplotypes in common, respectively (p less than 0.02). The birth order. Cumulative risk estimates were 26% for sibs born after the proband and 11% for sibs born before the proband (p less than 0.001). The age. Risk estimates were 24% for sibs who were aged less than 10 yr at the time of the proband's onset and 5% in sibs aged greater than 10 yr (p less than 0.001). The joint analysis of birth order or age with HLA genotypes showed significantly higher risks for both identical and haplo-identical sibs with later birth order and/or younger age. Even though these results do not reflect true incidence rates because of retrospective recruitment of part of the material, the comparison of the risk figures points out the important role of some age-related effect, probably environmental, in addition to the genetic susceptibility borne by MHC genes.

Birth Order↗
PubMed1985

A systematic study of HLA class II-beta DNA restriction fragments in insulin-dependent diabetes mellitus.

DNA restriction fragments of the genes encoding HLA class II-beta antigens were compared in 34 patients with insulin-dependent diabetes mellitus and 34 HLA-DR-matched healthy individuals. Ninety-three fragments, determined by six restriction enzymes (EcoRI, EcoRV, HindIII, BamHI, Pvu II, and Taq I), were analyzed: (i) A DR Taq I 12.7-kilobase-pair fragment might be a marker for the extended haplotype HLA-B8, DR3. (ii) In controls, DR4 haplotypes are associated with two distinct clusters of DQ restriction fragments (DQR4 and DQR5). Almost all (94%) DR4 patients belong to the DQR4 and not to the DQR5 cluster. This suggests that, among HLA-DR4 haplotypes, only DQR4 haplotypes are involved in susceptibility to insulin-dependent diabetes mellitus. (iii) A DR Taq I 14.5-kilobase-pair fragment was found to be strongly associated with DQR4, mainly in DR3/DR4 heterozygous patients (P = 5 X 10(-4). However, these results must be interpreted with caution, taking into account the high number of statistical tests performed.

DNA Restriction Enzymes↗
PubMed1985

[Interaction between HLA and Gm for susceptibility to insulin-dependent diabetes].

It is now well established that HLA system is involved in the susceptibility to Type 1 diabetes mellitus. In this study we look for a possible effect of the Gm system. A first study (cases-controls) suggests that among individuals who had HLA-DR3 but not HLA-DR4 or HLA-DR4 without HLA-DR3, there is a possible effect of the phenotype Gm3,23,5 or Gm3,-5 in the susceptibility to IDDM. Moreover, we have tested by the sibpair method whether HLA and Gm are transmitted independently from IDDM: an unaffected sib, sharing the same HLA haplotypes than an affected individual, seems to be more often phenotypically different at the Gm loci.

Diabetes Mellitus, Type 1↗
PubMed1985

Long-term complete autologous reconstitution following cyclophosphamide and allogeneic marrow infusion in a case of severe aplastic anemia.

A 23 year old man with severe idiopathic aplasia, who had previously been unsuccessfully treated with oxymetholone for a period of 6 months, received two intravenous (IV) infusions of bone marrow cells from his HLA identical brother. He was given 200 mg/kg anti-lymphocyte globulins (ALG) IV, followed by a first infusion of 13 X 10(9) nucleated cells IV, but no improvement was observed. Three months later, he was treated with cyclophosphamide (25 mg/kg over 4 days) followed by a second infusion of 18 X 10(9) nucleated cells IV of the same donor. Within a four month period there was evidence of complete marrow reconstitution, and the patient has experienced no relapse of the disease during the subsequent 13 years follow up period. The mechanism of the recovery is unclear: no evidence of graft acceptance could be established. The respective roles of ALG, cyclophosphamide and/or bone marrow infusions are discussed.

Adult↗
PubMed1985

[Value of genetic studies and immunologic markers in diabetes. Prediction of insulin dependence].

A new classification based on physiopathological criteria distinguishes Type I diabetes, observed in patients with stigmata of anti-islet of Langerhans auto-immunity, from Type II diabetes without these autoimmune changes. Type I diabetes is sub-divided into Classes Ia and Ib, Class Ib comprising those cases associated with other auto-immune diseases. Serological analysis of 76 patients with clinical type Ia diabetes and 215 healthy, first degree relatives showed that the distinction between Classes Ia and Ib was not clear-cut and that patients classified clinically Ia were in fact infraclinical Ib subjects. Forty-one per cent of patients with Class Ia diabetes had anti-gastric, anti-adrenal or anti-thyroid antibodies, and 28 p. 100 of their healthy relatives also had the same types of antibodies. Several prospective studies of the families of patients with Type I diabetes have shown that anti-islet of Langerhans antibodies were associated with a high risk of diabetes. Similarly, the presence of these antibodies in patients apparently with Type II diabetes (non-insulin dependent) was associated with an increased risk of developing insulin dependence. These results illustrate the value of immunological investigations in diabetic patients. They may influence the choice of treatment in the future.

Animals↗
PubMed1984

Detection of islet cell antibodies by a microcytotoxicity method.

A microcytotoxicity test for antibodies against islet cells (ICA) is described. Sera from patients with insulin-dependent diabetes, their healthy first degree relatives, and normal controls, genotyped for HLA-A, -B and -DR, were tested by 4 different methods. Cytoplasmic ICA and complement fixing ICA were detected by indirect immunofluorescence with human pancreas sections, and cytotoxic complement dependent ICA and surface ICA were tested against murine beta cell suspensions. Strong correlation was found between cytotoxic and surface antibodies (P less than 10(-7). The technique described is appropriate for use in the screening of large numbers of sera.

Animals↗
PubMed1984

Segregation of HLA-DR2 among affected and non-affected offspring of 66 families with type 1 (insulin-dependent) diabetes.

Segregation of HLA-DR2 among affected and unaffected offspring was studied in 66 HLA-genotypes families with Type 1 diabetes in whom at least one parent carried DR2. The frequency of DR2-positive parents (21%) was not different from that of control families (29%). Among the diabetic probands, the gene frequency of DR2 was significantly decreased compared with control subjects (0.05 versus 0.17, p less than 0.001) as were DR5 (0.07 versus 0.17, p less than 0.01) and DR7 (0.06 versus 0.13, p less than 0.003). Twenty probands carried DR2, in 11 or whom (55%) it was found in combination with either DR3 or DR4. The nine cases who carried another DR allele included one who was DR2 homozygous. Transmission of DR2 was reduced in affected offspring, and random in unaffected siblings, compared with the expected ratio. However, when the DR2 transmission was analysed separately for parents bearing DR2 with DR3, DR4 or another DR allele, it appeared that DR2 transmission to affected offspring was random when the parents carried neither DR3 or DR4, the transmission deficit being due to over-transmission of DR3 and DR4. The haplotype analysis showed that the haplotype A3, Cw7, B7, GfS, DR2, found in 19% of "non-diabetic" DR2 haplotypes was practically absent among "diabetic" DR2-haplotypes (4%). In conclusion, population and segregation analysis could not demonstrate a specific protective effect of DR2.

Diabetes Mellitus, Type 1↗
PubMed1984

Genetic polymorphism of the fourth component of complement and type 1 (insulin-dependent) diabetes.

The complement proteins, Bf, C2, C4A and C4B, are closely linked to HLA. In 74 propositi and their families, and 97 controls genotyped for HLA-A, -B, -C, DR, -Bf, a high incidence of the C4BQ0 variant was detected in the patient group (33% versus 12%, p less than 0.00001); C4BQ0 was more frequent in propositi than in non-affected siblings (40 out of 74 versus 36 out of 92, p less than 0.05). When comparing the distribution of the phenotype C4BQ0 in Type 1 diabetic patients and normal control subjects, the difference was significant in patients bearing DR3 or DR4 (56% and 25%, respectively, p less than 0.003). The main linkage disequilibria were observed among the 74 propositi: B18, BfF1, C4, A3, BQ0, DR3; B12, BfS, C4, A3, BQ0, DR4. The existence of a silent allele at the C4 B locus is known to be associated with a defective immune response.

Complement C4↗
PubMed1984

Islet-cell antibodies (ICA-CFICA) and HLA genotypes in 107 IDD patients and their first-degree relatives.

The prevalence of ICA and CFICA in relation to HLA-DR genotypes was analyzed in 107 insulin-dependent diabetic (IDD) patients with a duration of IDD ranging from onset to 30 years, and 247 nondiabetic first-degree relatives. In 46 patients tested at onset of IDD, the prevalence of ICA and of CFICA was 61 and 50%, respectively; with the longer duration of IDD, the prevalence of CFICA decreased more rapidly than that of ICA. No significant association was observed between ICA and any HLA allele in patients tested from onset till 2 years after onset. However, a higher prevalence of ICA was found in DR3 positive patients with a duration of IDD of more than 2 years (p less than 0.02). Among the healthy relatives, the prevalence of ICA was 7% in parents and 13% in siblings; one out of 101 siblings had CFICA. No association was found between ICA and any HLA marker. The presence of ICA in sibs was independent of the number of haplotypes they shared with the IDD proband: among the 13 ICA-positive healthy sibs, one shared 2 haplotypes, nine shared 1 haplotype and three shared no haplotype with the proband, which is not different from the random distribution.

Adolescent↗