[Incontinentia pigmenti (Bloch-Sulzberger) and pseudoglioma].
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Biomedical subjects
Publications and source records attributed to H Mensing.
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Certain clinical symptoms such as hernias or joint contracture in patients with mucopolysaccharidoses (MPS) cannot be explained as direct consequences of the disturbed glycosaminoglycan metabolism. They may be related to secondary changes of connective tissue components. The glycoprotein fibronectin is a constituent of connective tissue with a high affinity to polyanions such as heparan sulfate or heparin. Fibronectin in addition is a potent stimulus for fibroblasts to migrate chemotactically. We studied this cell property in MPS fibroblasts. The chemotactic activity of all MPS types was diminished. MPS II fibroblasts were chemotactically inactive. When the cells were corrected for the lacking enzyme by adding conditioned medium from control fibroblasts, the chemotactic migration increased except for MPS type II cells. The known enzyme defect in the degradation process of glycosaminoglycans in MPS results in lysosomal storage of degradation products and in addition causes changes of other cell properties.
Chemotaxis of human embryo fibroblasts and rhabdomyosarcoma cells was studied in a blind well Boyden chamber using fibronectin as a chemoattractant. The cell strains studied show a differential response to fibronectin, a fact which may mirror the origin of the cells, that means normal skin or tumor associated tissue, respectively. Furthermore, we detected another chemoattractive fraction synthesized and secreted by fibroblasts in addition to fibronectin and collagen derived fragments. Initial experiments demonstrated the proteinous nature of the component(s) and provided some information on the biochemical features.
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14 patients with advanced malignant melanoma were treated in 36 therapy cycles with cisplatin. 8 patients had been pretreated with dacarbazine and 6 had received additional BCG immunotherapy. 4 patients had been irradiated after surgical removal of lymph node metastases. All patients showed significant tumor progression. 4 patients were treated showing ultimately disseminated melanoma with widespread visceral involvement. 5 patients with lymph node metastases had been operated radically and were treated postoperatively. Cisplatin was administered as a 24-h high-dose therapy (200 mg or 120-200 mg/m2) under forced mannitol diuresis, treatment cycles were repeated monthly. Of all the patients, 1 showed complete remission of supraclavicular metastases lasting for 6 months until now. 1 patient showed an initial minor response with subsequent stabilization without appearance of additional metastases for 1 year. 2 patients who had received cisplatin postoperatively showed no reappearance of tumor growth for 8 months up until now. 11 patients showed no change or progression of disease, 6 of them had received only one therapy cycle. Under clinical conditions, side effects of cisplatin treatment can be managed satisfactorily, no irreversible kidney damage could be observed under forced diuresis. As far as the above-mentioned results are concerned, antineoplastic activity of cisplatin as to advanced malignant melanoma must be considered to be of limited benefit using cisplatin as a single-agent treatment. Improvement of results might be obtained using cisplatin in a combination therapy together with other antineoplastic agents, which at the present time are being investigated in several prospective trials.
A report is given on a progeroid disease affecting three brothers, 11, 13, and 14 years old. The difficult differential diagnosis of such progeroid syndromes is discussed. The clinical signs were mostly consistent with Werner's syndrome. Since some of the symptoms of this disease were not present, probably because of the young age of the patients, this progeroid syndrome was classified as Werner syndrome-like. Electronmicroscopic findings of the patients skin showed changes of the blood vessels and nerves, which were until now not described in Werner's syndrome or other progerias. The latter findings may give some hints with regard to the pathogenesis of this disease.
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A rare case of plasma-cell vulvitis (Zoon) is reported in a 53 years old female. The painful erythematous plaque affected parts of the vulva. After disappointing local treatment for three years repeated intralesional injections of triamcinolone improved the condition satisfactory.
Cutis laxa is an extremely rare genetic disorder of connective tissue. The striking dermatologic features are loose skin folds and skin laxity. Due to these folds the patients appear to be prematurely aged. Plastic surgery can improve these changes. This treatment is especially indicated in patients with the autosomal dominant type of cutis laxa where internal manifestations do not occur. We report the case of a seventeen years old boy with cutis laxa and surgical therapy.
Harmartomas are tumor-like local deformations of tissue-parts. Thus tumors of epidermal appendages must be classified in this sense. Until now these deformations were also called "organoid nevus". In the present paper we report studies on the nevus sebaceus of Jadassohn and the syringocystadenoma papilliferum, especially with regard to the coincidence with other dermoepidermal changes. From 1955 to 1979 a total of 164 histological specimens were diagnosed as nevus sebaceus Jadassohn (NSJ) or syringocystadenoma papilliferum (SCAP) in the department of dermatology, University Hospital Hamburg. 134 were NSJ, 11 were SCAP and 19 were combinations of both tumors. The percentage of multiple deformations of all 164 organoid nevi were 24.
A 75 year old patient had atypical granulomata annularia in several regions of his body. Besides he presented a poorly controlled diabetes mellitus. Morphologically it was difficult to classify the disease. Clinically cutaneous lymphoma were considered.
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