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Biomedical subjects

A Du Vivier

Publications and source records attributed to A Du Vivier.

At least 19 recordsLinked to original sources
PubMed2002

Congenital erythropoietic porphyria: dilemmas in present day management.

Congenital erythropoietic porphyria is a rare autosomal recessive disorder of haem biosynthesis caused by a deficiency of uroporphyrinogen III synthetase. There is resultant accumulation and hyperexcretion of porphyrinogens of the isomer I variety. These are converted by spontaneous oxidation into their corresponding photoactive porphyrins leading to photodamage. Accumulation of porphyrins results in haemolysis and extensive photosensitivity. The consequences of chronic haemolysis are splenomegaly, reactive erythroid hyperplasia, erythrodontia, bone fragility, extreme photosensitivity and photomutilation. We present a 35-year-old man who has the severe infantile form and illustrates the haematological and photodestructive complications despite attempts at treatment with hypertransfusion, oral charcoal therapy and beta-carotene. Allogenic bone marrow transplantation has been considered but because of the high associated mortality this procedure has been discounted at present in the management of our patient.

Adult↗
PubMed1995

Cancer Research Campaign health education programme to promote the early detection of cutaneous malignant melanoma. I. Work-load and referral patterns.

From 1987 to 1989 a campaign to promote the early detection of cutaneous malignant melanoma was conducted in the areas of seven health authorities in England and Scotland (total population 3.6 million). Data were collected on 17,155 patients attending pigmented lesion clinics (PLCs) in each study area during the campaign. After a dramatic rise in PLC referral rates in the first month of the campaign the average monthly referral rate among the target population in the study period settled to an average of 13 per 10(5), a twofold increase compared with the pre-campaign period. Over 85% of patients at all PLCs were seen within 4 weeks of referral from their general practitioners. The melanoma to non-melanoma detection ratio was (1:33). The organization of future early detection initiatives needs careful review and planning, in order to improve their effectiveness in all sections of the population, and to enable health services to cope with the increased work-load.

Adult↗
PubMed1995

Cancer Research Campaign health education programme to promote the early detection of cutaneous malignant melanoma. II. Characteristics and incidence of melanoma.

The effect on the detection and characteristics of melanoma, resulting from the Cancer Research Campaign's health education programme to promote the early detection of melanoma in the general population, was studied from 1987 to 1989. The seven study areas in England and Scotland yield a target population of 3.6 million. Data were collected from local clinic-based registers, pathology laboratories, and the cancer registries. The average annual incidence rates of melanoma were seven and 12 per 10(5) in males and females, respectively, age-standardized to England and Wales, 1988. These rates are similar to the national figures for Scotland, where there is a national melanoma register, but higher than those reported by the English and Welsh cancer registries. The incidence was significantly higher in females than males (P < 0.001), and increased with age. Fifty-three per cent and 65% of cases in males and females, respectively, were thin (Breslow thickness < or = 1.5 mm), similar to the national figures from Scotland. No significant decrease in the incidence of late-stage tumours was found in either sex as a result of the campaign. Because of difficulties with ascertainment of cases in England, the main evaluation will focus on future trends in mortality rates for melanoma.

Adult↗
PubMed1990

Pemphigoid gestationis and neonatal alloimmune thrombocytopenia.

A patient whose second, third and fourth pregnancies were complicated by alloimmune thrombocytopenia, developed pemphigoid gestationis after the fourth pregnancy. The infant was delivered by Caesarean section at 34 weeks gestation. The pemphigoid gestationis resolved promptly after a short course of systemic corticosteroids. A possible association between these two uncommon conditions is discussed.

Adolescent↗
PubMed1985

A case of mycosis fungoides mimicking actinic reticuloid.

A 61-year-old man presented with erythema and induration of the skin on his face and neck. Initially a photosensitive dermatosis was suspected but this was excluded on further investigation. Mycosis fungoides was diagnosed and the lesions responded to photochemotherapy (PUVA).

Diagnosis, Differential↗
PubMed1984

Erythromelalgia following pergolide administration.

Two patients are described in whom treatment of Parkinson's disease with the ergot derivative pergolide was associated with the development of erythromelalgia. The possible mechanism of pergolide-induced erythromelalgia is briefly discussed.

Aged↗
PubMed1978

Mycosis fungoides, nitrogen mustard and skin cancer.

The prevalence of epithelial cancer was determined in 202 patients with mycosis fungoides and the Sézary syndrome and was found to be 10.5%. In an attempt to establish a possible association with topically applied nitrogen mustard, it was noted that 4.0% of these patients had lesions prior to nitrogen mustard therapy (although over half subsequently developed further lesions) and 6.5% of the patients developed them de novo after therapy. In two patients in this latter group, malignancies developed in are as not usually associated with solar-induced cancer. These findings indicate that epidermal neoplasia is not uncommon in these patients and that nitrogen mustard may function as a carcinogen or a co-carcinogen.

Administration, Topical↗
PubMed1978

The effects on epidermal DNA synthesis of the butyrate esters of clobetasone and clobetasol, and the propionate ester of clobetasol.

An animal model for evaluating the effects of drugs on epidermal DNA synthesis has been used to determine the time of onset, period of maximum activity and duration of effect of two topically applied proprietary glucocorticosteroids, clobetasone butyrate and clobetasol propionate. Both drugs produced maximum inhibition of epidermal DNA synthesis by 24 h after application. Clobetasol propionate exhibited a significantly earlier onset (4 1/2 h) and longer duration (36 h) of activity than clobetasone butyrate (6 and 24 h respectively). A systemic effect was not observed at any time after the application of clobetasone butyrate but was observed for up to 24 h after the application of clobetasol propionate. Clobetasol butyrate was also assayed in this system and was found to be as potent as clobetasol propionate. It is concluded that the alcoholic, as opposed to the ketonic, grouping in the 11 positions of the steroid ring profoundly alters the potentials of this molecule for systemic activity.

Animals↗
PubMed1977

Pityriasis lichenoides--an immune complex disease.

Circulating immune complexes have been detected in patients with pityriasis lichenoides during disease activity when IgM and C3 have been observed in dermal vessels on direct immunofluorescence of fresh lesions. This implies that pityriasis lichenoides is an immune complex disorder and that deposited complexes play a part in the pathogenesis of the condition. There is a characteristic pattern of immunofluorescence which may be a diagnostic aid.

Adolescent↗
PubMed1975

Alopecia areata, autoimmunity, and Down's syndrome.

Alopecia areata occurs more often in Down's syndrome than would be expected by chance, sixty cases being found among 1000 patients with this syndrome compared with one case among 1000 subnormal controls. Because alopecia areata is associated with some organ-specific autoimmune disease and thyroid antibodies are often found in Down's syndrome sera from affected patients were examined for the presence of fluorescent autoantibodies. Antibodies against thyroid components tended to be present in female mongols with alopecia areata in comparison with females in a normal population but not in male mongols. Futhermore, eight out of 23 female mongols (35%) with alopecia areata had antibodies against thyroid components compared with two out of 23 female mongols (9%) without alopecia areata.

Adolescent↗
PubMed1974

Treatment of psoriasis with azathioprine.

Azathioprine treatment benefited 19 (66%) out of 29 patients suffering from severe psoriasis. Haematological complications were not troublesome and results of biochemical liver function tests remained normal. Minimal cholestasis was seen in two cases and portal fibrosis of a reversible degree in eight. Liver biopsies should be undertaken at regular intervals if azathioprine therapy is continued so that structural liver damage may be detected at an early and reversible stage.

Alcohol Drinking↗