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Biomedical subjects

R S Wells

Publications and source records attributed to R S Wells.

At least 19 recordsLinked to original sources

Enzymatic basis of typical X-linked icthyosis.

Steroid sulphatase activity was determined in cultured fibroblasts from 25 individuals with X-linked ichthyosis from four countries. All those with X-linked disease had markedly reduced enzyme levels compared with controls and patients with other types of ichthyosis. X-linked ichthyosis seems to be the result of a common mutation affecting the expression of steroid-sulphatase activity.

Adult

A double-blind controlled crossover trial of an antigen-avoidance diet in atopic eczema.

20 out of 36 children (aged two to eight years) with atopic eczema completed a twelve-week, double-blind, controlled, crossover trial of an egg and cows' milk exclusion diet. During the first and third four-week periods, patients on an egg and cows' milk exclusion diet received a soya-based milk substitute (trial period) or an egg and cows' milk preparation (control period). Response was assessed in terms of eczema activity, number of areas affected, pruritus, sleeplessness, and antihistamine usage while on the two diets. During the middle period patients resumed their normal diet to minimise any carry-over effect. 14 patients responded more favourably to the antigen-avoidance diet than to the control diet, whereas only 1 responded more favourably to the control diet than the trial diet. Patients experienced more benefit during the first diet period than the second, whatever the nature of the diet. There was no correlation between a positive prick test to egg and cows' milk antigen and response to the trial diet.

Allergens

Defective yeast opsonization and C2 deficiency in atopic patients.

Twenty-seven per cent of atopic patients initially presenting with infantile eczema or hay fever were defective for yeast opsonization and 18% had low levels of C2; these deficiencies were mutually exclusive, suggesting that they are primary. Both defects were associated with each of four different atopic syndromes, some of which were related to certain HLA haplotypes.

Adolescent

HLA in eczema and hay fever.

The presumed HLA haplotype A1:B8 was more frequent and the combination of A3 and B7 was less frequent in allergic subjects presenting with eczema, than in those presenting with hayfever. A1:B8 was most frequent (36%) in eczema complicated by asthma and/or hay fever, and least frequent (5%) in hay fever alone, considerably above and below the frequency in the general population (17%).

Adolescent

Genetics and dermatology: a review.

The development of genetic studies over the last hundred years, in particular as they have affected dermatology, has been reviewed. The clinical application of knowledge derived from other fields has been emphasized, areas of future research have been indicated, and suggestions for cooperation with other disciplines have been offered.

Animals

Atypical ichthyosiform erythrodernam deafness and keratitis. A report of two cases.

Two patients with ichthyosiform erythroderma of the same unusual but characteristic distribution are described. Both patients were born with perceptive deafness and developed severe vascularizing keratitis in early childhood. There is no family history of the disorder in either case. This syndrome is discussed in relation to previous reports of atypical ichthyosiform erythroderma associated with deafness.

Adult

The syndrome of ankyloblepharon, ectodermal defects and cleft lip and palate: an autosomal dominant condition.

Seven patients from four families are reported who had an inherited condition of which the main features were ankyloblepharon, ectodermal defects and cleft lip and palate. The ectodermal defects were partial or complete hair loss, absent or dystrophic nails, pointed widely spaced teeth and partial anhidrosis. Associated anomalies included lacrimal duct atresia, supernumerary nipples, syndactyly and auricular deformities. The inheritance of this abnormality was consistent with that of an autosomal dominant trait. The relationship between this and similar syndromes is discussed.

Adult