Combination therapy cyclosporin A--PUVA in psoriasis.
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Biomedical subjects
Publications and source records attributed to R F Hulsmans.
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In order to study the influence of fish oil on CyA-induced renal dysfunction, 13 patients with psoriasis (CyA group) received cyclosporin A (CyA) alone, and seven patients (CyA/EPA + DHA group) received a combination of cyclosporin A and fish oil (6g eicosapentaenoic acid, C20:5 omega-3, and docosahexaenoic acid, C22:6 omega-3, daily) for 3 months. The glomerular filtration rate fell by 18.0 +/- 9.6% in the CyA group compared with 8.7 +/- 6.8% in the CyA/EPA + DHA group (mean +/- SD, P less than 0.05). The effective renal plasma flow fell by 10.6 +/- 8.9% in the CyA group and did not change in the CyA/EPA + DHA group (P less than 0.05). The calculated total renal vascular resistance increased by 19.8 +/- 14.5% in the CyA group and did not change in the CyA/EPA + DHA group (P less than 0.01). The results of this pilot study suggest that fish oil can reduce CyA-associated renal dysfunction in psoriasis patients.
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A 32-year-old man suffering from linear frontoparietal scleroderma was found to have low (less than 10% normal) serum classical pathway activity although C1q, C3, C4, C5, and total alternative pathway activity was normal. Addition of purified C2 led to complete restoration of the total hemolytic activity of the classical pathway. The C2 hemolytic assays showed that the patient was not totally deficient in C2. He had about 30% of the normal C2 level. Studies on his available nucleus family members in the Netherlands also showed that the deficiency was inherited; one of the patient's brothers and one of his daughters had half of the normal C2 levels. The C2 deficiency could not be corrected by a three-week regimen of danazol. To the best of our knowledge, this is the first documented case concerning an association of linear frontoparietal scleroderma with C2 deficiency.
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In 1979 Cohen et Hayden and in 1983 Wiedemann et al. delineated a syndrome consisting of partial gigantism of the hands and/or feet, nevi, hemihypertrophy, subcutaneous tumors, macrocephaly or other skull anomalies and possible accelerated growth and visceral affections. Hitherto the literature pertaining to this syndrome consists of somewhat more than 100 cases of which some, that have been described previously or subsequently under other headings, were rediagnosed as being Proteus syndromes. Of these, more than half show vascular anomalies closely resembling those observed in the Klippel-Trenaunay syndrome, but in the Proteus syndrome appear to be more haphazardly distributed over the integument. We report 3 pediatric patients with the Proteus syndrome, all showing cutaneous angiodysplasias. These patients were initially diagnosed as suffering from "severe or atypical Klippel-Trenaunay syndrome". In one of these, cardiac tumors were observed soon after birth which subsequently showed spontaneous involution and were therefore considered to be rhabdomyomas. In the Proteus syndrome cardiac pathology is rare, and cardiac tumors have not been described previously. Moreover, we observed umbilical hernia in two of our patients, a feature which has hitherto not been reported in patients with the Proteus syndrome. In all our patients a broad thoracic cage resembling a "body-builders chest", asymmetrical and disproportional macrodactyly and broad, flat feet were conspicuous. These broad, flat feet with macrodactyly and large spaces between the first and second digits were designed by the parents of one of our patient as "chimpanzee's feet". Macrodactyly, "chimp's" feet and a broad thoracic cage are considered by us to be clinical hallmarks of the Proteus syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)
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