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Biomedical subjects

D Atherton

Publications and source records attributed to D Atherton.

24 records · Page 2Linked to original sources

Quantitative and functional deficit of suppressor T cells in children with atopic eczema.

Helper (OKT4+) and suppressor (OKT8+) T cells were enumerated in 16 children with severe atopic eczema. Compared to controls (median 1 . 8) the ratio of OKT4+/OKT8+ cells in the patients was significantly higher (median 2 . 65, P less than 0 . 002). Functional suppressor activity in these patients was assessed by concanavalin A (Con A) activation and suppression of pokeweed mitogen (PWM) induced immunoglobulin production by plasma cells and Con A proliferation of T cells. In both assays a lack of suppression was shown (Con A/PWM, P less than 0 . 02; Con A/Con A, P less than 0 . 05). There was a significant inverse correlation between the helper/suppressor ratio and functional suppressor activity (P less than 0 . 01). These results indicate that a defect of T cell regulation does exist in atopic eczema and if it is of primary pathogenic importance, immunotherapy to restore the balance may prove useful.

Adolescent↗

Spontaneous remission of congenital leukemia: a case for conservative treatment.

A newborn infant with spontaneous remission of congenital leukemia cutis is described and a literature review of this uncommon phenomenon is provided. In view of the unusual and unpredictable behavior of this disease, chemotherapy should be withheld unless there is evidence of an 11q23 translocation or progressive disease. Otherwise, overall survival does not appear to be affected by adopting a conservative approach. Because of occasional late relapses, long-term follow-up is recommended. The biologic basis underlying spontaneous remission of congenital leukemia is unknown; therefore, molecular or molecular cytogenetic analysis of DNA obtained from a skin biopsy is recommended.

Biomarkers, Tumor↗

Practical and psychological problems for parents of children with epidermolysis bullosa.

Parents of children with epidermolysis bullosa were sent a questionnaire seeking information on problems associated with the condition; 45% replied and their responses are discussed in terms of reported needs. Epidermolysis bullosa (EB) is the name given to a group of distinct, inherited disorders which are manifest as an exceptional liability of the skin to blister and ulcerate following mechanical trauma. There are three principal groups: EB simplex, dystrophic EB and junctional EB. All forms of EB simplex are inherited as autosomal dominant traits. These diseases are generally relatively mild but some patients are sufficiently disabled to prevent their walking more than 200 yards at a time. Dystrophic EB is of variable severity; not unknown are problems such as fusion of the fingers, fixation of the tongue, shrinkage of the mouth and oesophageal stenosis. When severe this is an exceptionally disabling disorder associated with a significant reduction in life expectancy. Junctional EB is always serious and death within the first few years of life, often within the first few weeks, is usual. DEBRA, the Dystrophic Epidermolysis Bullosa Research Association, is a self-help group of sufferers and their families. A questionnaire aimed at eliciting problems associated with this condition was sent to 138 members and their replies form the basis of this paper.

Adolescent↗

Constipation in epidermolysis bullosa: successful treatment with a liquid fiber-containing formula.

In epidermolysis bullosa (EB), chronic constipation, painful defecation, and fecal impaction frequently contribute to malnutrition and growth failure. Standard treatments for constipation, such as increased intake of conventional dietary fiber and fluids and/or the use of laxatives and stool softeners, are largely unsuccessful. We evaluated by questionnaire the use of a fiber-containing liquid formula (Enrich) in 20 chronically constipated children with dystrophic EB. All derived substantial improvement in constipation when taking 250 to 750 ml Enrich per day. We recommend that such a fiber-containing food be prescribed for chronic constipation in EB. In cases of fecal impaction, this should be preceded by bowel cleansing.

Adolescent↗