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Biomedical subjects

M M Hulshof

Publications and source records attributed to M M Hulshof.

7 recordsLinked to original sources

Double-blind, placebo-controlled study of oral calcitriol for the treatment of localized and systemic scleroderma.

BACKGROUND: Various treatments including corticosteroids, nonsteroidal anti-inflammatory drugs, D-penicillamine, interferon gamma, cyclosporine, and cytostatic drugs have been used with limited success in both morphea and systemic sclerosis (SSc). OBJECTIVE: We investigated the effect of treatment with oral calcitriol in patients with localized or systemic scleroderma. METHODS: A randomized, double-blind, placebo-controlled study of 9 months' duration with a 6-month follow-up was performed at the Department of Dermatology. A total of 27 patients (7 patients with SSc and 20 with morphea) were selected on a minimal skin score of 3 for patients with morphea and 12 for those with SSc. Each patient received calcitriol (0.75 microg/day for 6 months plus 1.25 microg/day for 3 months) or placebo for 9 months. Efficacy parameters included skin score, measurement of serum markers of collagen synthesis and degradation and, additional for the patients with SSc, oral aperture measurements, lung function studies, and esophagus motility. RESULTS: The skin score in patients with morphea after 9 months' treatment showed no significant difference between the placebo and calcitriol groups (mean percentage reduction [SD] in skin score in the placebo group was -29.3 [57.9]; in the calcitriol group it was -19.4 [46.6]). The small group of patients with SSc was inadequate to allow us to draw any conclusions regarding efficacy. No significant change was found in the serum markers of collagen metabolism. CONCLUSION: In this study calcitriol was not more effective than placebo in patients with morphea. Because of the small group of patients with SSc treated, no conclusions regarding efficacy in SSc can be drawn.

Administration, Oral↗

Multiple agminate Spitz naevi.

We report two patients with multiple agminate Spitz naevi (MASM), a rare disorder. In case 1, a 16-year-old girl, the results of chromosomal investigation of fibroblasts from the affected area (translocation 45, X, t(4; 7) (p14; p22) in a mosaic pattern) suggest that early during embryogenesis a de novo reciprocal translocation has occurred between chromosomes 4 and 7. This resulted in the skin lesions as described on the right shoulder, arm and hand. The melanoma risk in MASM is also discussed.

Adolescent↗

Long-term prognosis in patients treated for erythema chronicum migrans and acrodermatitis chronica atrophicans.

OBJECTIVE: To determine whether Lyme borreliosis persisted or had recurred in patients treated for erythema chronicum migrans and acrodermatitis chronica atrophicans. DESIGN: Retrospective follow-up study. Mean time between treatment and follow-up study was 8.8 years (SD, 66.6 years). SETTING: Department of dermatology. PATIENTS: Patients (N = 52) treated from July 1964 to October 1992 for erythema chronicum migrans (ECM; n = 44) or acrodermatitis chronica atrophicans (ACA; n = 8). Fifty-two of the 56 successfully contacted and registered patients agreed to participate, for a response rate of 93%. MAIN OUTCOME MEASURES: Signs and symptoms of Lyme borreliosis; serum antibodies to Borrelia burgdorferi. RESULTS: The interval from the time of diagnosis to study entry was 0.8 to 28.7 years (mean, 8.2 years). No symptoms or signs of active Lyme borreliosis were observed in the 52 patients. Antibodies to B burgdorferi were found in the ECM group in 1 of the 23 patients who received a recommended treatment and 2 of the 21 patients who received other treatments; antibodies were found in the ACA group in all 5 adequately treated patients who received a recommended treatment and in 1 of the 3 patients who received other treatments. CONCLUSIONS: There was no association between serologic results and type of treatment or between serologic results and complaints or symptoms at the time of the study in either of the patient groups. The prognosis in most patients with Lyme borreliosis is excellent.

Acrodermatitis↗

Oral calcitriol as a new therapeutic modality for generalized morphea.

BACKGROUND: None of the commonly used drugs for the treatment of scleroderma appears to significantly influence the fibrotic stage of this disorder. Recently, a beneficial effect of the treatment with oral calcitriol (1,25 dihydroxyvitamin D3) in 10 patients with systemic sclerosis and four patients with morphea was described. This fact could be ascribed to the immunoregulatory effects of calcitriol observed in vitro and to inhibition of fibroblast growth. We treated three patients with extensive morphea with remarkable results. OBSERVATION: Three patients with generalized morphea were treated with calcitriol in an oral daily dose of 0.50 to 0.75 microgram. After 3 to 7 months of treatment, the mobility of the joints improved and the skin extensibility increased. No adverse effects were observed. The improvement persisted after discontinuation of therapy during a follow-up period of 1 to 2 years. CONCLUSION: Calcitriol showed a beneficial effect in generalized morphea during an open study. Double-blind, placebo-controlled trials are needed to assess its therapeutic value.

Administration, Oral↗

Granulomatous hypersensitivity to protamine as a complication of insulin therapy.

A patient is described who developed a granulomatous skin reaction to injections of protamine-insulin for the treatment of her diabetes mellitus. By means of skin testing it was shown that this reaction was caused by protamine. No adverse effects were seen when she was injected with insulin which did not contain protamine.

Aged↗

Epitopes of apolipoprotein B-100 and B-48 in both liver and intestine. Expression and evidence for local synthesis in recessive abetalipoproteinemia.

The presence of apolipoprotein (apo) B in liver and intestine from a patient with abetalipoproteinemia was evaluated by immunohistochemistry with a polyclonal and six monoclonal antibodies to different apo B-48 and B-100 epitopes. In normal liver, apo B was present inside and outside hepatocytes. The patients liver exhibited staining in the cytoplasm with the polyclonal and three monoclonal antibodies. By immunoelectron-microscopy, apo B was found to be present in the smooth endoplasmatic reticulum and the Golgi complex. Normal intestinal epithelium was labeled with polyclonal and all monoclonal antibodies, including those specific for apo B-100. The patients epithelium stained with polyclonal and six monoclonals, and apo B was present in the Golgi complex. Thus, normal intestinal mucosa expressed apo B-48 and B-100 epitopes, which indicates apo B-100 synthesis in the gut. The synthesis of the apo B molecule in the patient seems to be retained in both liver and gut, which suggests a posttranslational defect.

Abetalipoproteinemia↗