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Biomedical subjects

K Welsh

Publications and source records attributed to K Welsh.

At least 91 records · Page 5Linked to original sources
PubMed1987

Autoantibodies and immunogenetics in 30 patients with systemic sclerosis and their families.

Clinical and serological evidence of connective tissue disease was found in a high proportion of 132 family members of 30 patients with systemic sclerosis. In 20 probands with the milder CREST form of the disease, 10 had HLA-DR5 and 12 had null alleles at the C4 loci. None of 11 probands with more severe systemic sclerosis had HLA-DR5; all 11 had null alleles at the C4 loci. All but two of the probands had either HLA-DR5 or a C4 null allele, and this was also the case for the majority of the relatives with autoantibodies. Genetic markers of the major histocompatibility complex, including HLA-DR5 and C4 null alleles, appear to be closely associated with markers of disease in these probands and their families.

Antibodies, Antinuclear↗
PubMed1986

A strong association between null alleles at the C4A locus in the major histocompatibility complex and systemic sclerosis.

Allotyping of the major histocompatibility complex (MHC)-linked complement component C4 has revealed a strong association of the null allele, C4A*Q0, with systemic sclerosis (SSc). Sixty-four percent of patients with SSc carried the C4A*Q0 allele, compared with 17% of the control group. Twenty-five patients and their families were typed for HLA antigens (A, B, Cw, and DR) and the MHC-linked complement components C4 and factor B to identify haplotypes in the MHC linkage group and C4 null alleles. Strong allelic association of HLA-B8 and DR3 with C4A*Q0 probably explains the previously reported association of SSc with the extended haplotype carrying HLA-B8 and DR3. Ninety-two percent of the patients had either C4A*Q0 or DR5; 31% of the controls had either C4A*Q0 or DR5.

Alleles↗
PubMed1986

Transplantation of fetal fibroblasts and correction of enzymatic deficiencies in patients with Hunter's or Hurler's disorders.

An attempt was made at correcting the specific lysosomal enzyme deficiencies in 7 children with Hunter's or Hurler's diseases by transplantation of fetal fibroblasts. In spite of pretreating the young patients with stored blood, following a procedure employed successfully to avoid rejection of kidneys from incompatible donors, the use of serum-free media for culturing the cells before being harvested and incubation of the cells with chorionic gonadotrophin, the transplantation of fetal fibroblasts was not associated with biochemical or clinical changes. None of the seven patients showed immune reactions against the transplanted cells, HLA antigens, or the missing enzymes.

Animals↗
PubMed1986

Extended haplotypes in rheumatoid arthritis and preliminary evidence for an interaction with immunoglobulin genes.

The incidence of extended haplotypes of the Major Histocompatibility Complex was compared between 20 probands with RA, their unaffected family members, and 42 controls. One haplotype only, HLA-Bw62 BfS C4A*3 C4B*3 DR4 GLO2, was significantly increased in the patient group, whereas HLA-B7 BfS C4A*3 C4B*1 DR2 GLO1, which was the most common haplotype in the control groups, was absent. The immunoglobulin allotype Glm(2) was significantly increased in frequency in the RA patients, and analysis showed that of the seven patients carrying Bw62-DR4, five were G1m(2) positive. Further, the increase in frequency of the phenotype Gm(1,2,17,21,3,5,23) was also significant and was carried by two of four probands with the extended haplotype HLA-Bw62 BfS C4A*3 C4B*3 DR4 GLO2 and by one proband also bearing this haplotype but with a null allele at the C4A locus. The striking association of G1m(2) and Bw62 with DR4 in our patients suggests that in interaction of immunoglobulin genes with DR4 is stronger when DR4 is associated with particular haplotypes rather than with DR4 in general.

Arthritis, Rheumatoid↗
PubMed1986

Immune factors in narcolepsy.

Most but not all subjects with the narcoleptic syndrome have the human leukocyte antigen (HLA) DR2 (and DQ1). The narcolepsy-DR2 association is the highest disease-HLA linkage known, and occurs in nonfamilial as well as familial cases of the narcoleptic syndrome. In other forms of daytime drowsiness, there is no relationship with a specific HLA, although some subjects considered to have "essential" hypersomnolence probably have the narcoleptic syndrome. The cause of the narcoleptic syndrome remains unknown, although in a few instances the condition follows infection. There is no evidence for a circulating sleep factor in the blood or in the cerebrospinal fluid of narcoleptic subjects, and no unequivocal marker of cellular immunity has yet been found. However, a few subjects with the narcoleptic syndrome have oligoclonal bands or raised immunoglobulin concentration in the cerebrospinal fluid. It is highly likely that the narcoleptic syndrome is an immune-mediated disorder, occurring in a genetically susceptible (DR2/DQ1-positive) subject.

Female↗
PubMed1986

Genetic susceptibility to scleroderma-like syndrome in symptomatic and asymptomatic workers exposed to vinyl chloride.

We have previously reported a genetic susceptibility to a scleroderma-like disorder in a group of workers exposed to polyvinyl chloride and this susceptibility could be mapped to the major histocompatibility complex area of the 6th chromosome. To date no genetic studies have been reported comparing affected with unaffected workers. We report such data and discuss it in the light of our previous findings in vinyl chloride exposed patients and in patients with classical scleroderma. Our results suggest that susceptibility to this disorder is increased in the presence of HLA-DR5 or of a gene in linkage disequilibrium with it and an antigen associated with the haplotype A1 B8, while DR3 favours progression of the disease.

Chemical Industry↗
PubMed1985

Exercise blood pressure and baroreflex function in borderline hypertensive and normotensive young men.

1. Resting carotid baroreflex sensitivity and blood pressure responses to standardized conditions of rest and exercise were measured in 17 borderline hypertensive males and 12 normotensive males. 2. The borderline hypertensive group had significantly higher systolic and diastolic blood pressures during orthostatic rest and isometric handgrip exercise and higher systolic blood pressure during supine rest and submaximum and maximum treadmill exercise. 3. The borderline hypertensive group had an attenuation of baroreflex sensitivity compared with the normotensive group. Resting baroreflex sensitivity was significantly correlated with absolute systolic blood pressure during supine rest, orthostatic rest, isometric handgrip exercise and submaximum treadmill exercise. 4. The results indicate that blood pressure is regulated at a significantly higher level during rest and exercise in borderline hypertension and is associated with reduced baroreflex sensitivity measured at supine rest.

Adult↗
PubMed1985

Glomerular C3b receptor loss in renal allografts.

There is almost no data from the glomeruli of allografted kidneys with respect to changes in the CR-1 (C3b) receptor expressed on glomerular podocytes. We studied 22 renal graft biopsies from rejecting and stable allografts, using a panel of monoclonal antibodies. We found that the CR-1 expression was decreased in a focal and segmental fashion in some biopsies, particularly in rejecting kidneys. These changes correlated with the intensity of glomerular mononuclear cell infiltration, but in contrast no correlation was seen with peripheral capillary wall deposition of complement (C3). Thus, some active process is occurring in the glomeruli of rejecting grafts which affects the expression of the CR-1 receptor.

Graft Rejection↗
PubMed1985

Transplantation of amniotic epithelial membranes in patients with mucopolysaccharidoses.

This paper reports the biochemical results of transplanting human amniotic epithelial cells in 3 children with Hunter's and 2 with Hurler's disease. A transient and modest increase of alpha-L-idurono-2-sulphate sulphatase or alpha-iduronidase was observed in the white cells collected from 1 patient with Hunter's and 1 with Hurler's disease. No variation in the excretion of glycosaminoglycans or oligosaccharides was detected in all 5 patients. There was no evidence of immune response towards the transplanted cells or the specifically deficient enzyme. Thus, in spite of the absence of the major histocompatibility antigens, HLA A, B, C and DR, on the surface of the amniotic epithelial cells, no long-term correction of lysosomal enzyme deficiencies was achieved by transplanting amniotic epithelial membranes collected at the end of the gestational period.

Amnion↗
PubMed1984

Human renal allograft and peripheral blood T lymphocyte subpopulations during the onset and treatment of rejection.

In order to study the changes in T lymphocyte subpopulations in both allografts and peripheral blood of patients following renal transplantation, we have examined 72 fine needle allograft aspirates and 56 peripheral blood samples from 24 patients during the first month following transplantation. The patients were studied before, during and after rejection episodes. Monoclonal antibodies directed against T helper (TH) and T cytotoxic/suppressor (TCS) cells were used to identify lymphocyte subpopulations in the allograft aspirates and peripheral blood. The ratio of TH:TCS cells in both the allograft aspirates and peripheral blood decreased significantly (p less than 0.01) during the three days before rejection became clinically manifest. During rejection, the aspirate TH:TCS ratios, but not the peripheral blood TH:TCS ratios, remained depressed. Following successful treatment for rejection, the aspirate TH:TCS ratios returned to levels found in non-rejecting allografts. However, in the allografts which were lost as a result of rejection or had persistent rejection despite treatment, the aspirate TH:TCS ratios, but not peripheral blood TH:TCS ratios, remained significantly low (p less than 0.01) when compared with the successfully treated allografts. Our study indicates that TH:TCS ratios, particularly in allograft aspirates, may be of value in predicting the onset and outcome of renal allograft rejection.

Adolescent↗