[A case of recessive dystrophic epidermolysis bullosa (localized type)].
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Biomedical subjects
Publications and source records attributed to M Manabe.
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It has been stated by McDonagh, Palma & Lightner [(1982) J. Am. Chem. Soc. 104, 6867-6871] that complexing of bilirubin with serum albumin has a marked species-dependent influence on bilirubin photoisomerization in vitro and in vivo. Therefore the kinetics for the quantitatively important reaction: (Formula: see text) of the photochemical interconversion between bilirubin and its photoisomers bound to human or rat serum albumin in aqueous solution, assayed by h.p.l.c., was used to elucidate the observed species-dependent difference. The relative rate constants for bilirubin bound to human serum albumin, except for k4, the rate of interconversion from (ZZ)-bilirubin into (EZ)-bilirubin, proved to be considerably larger than those for bilirubin bound to rat serum albumin. In accordance with these rate constants, the formation of photoisomers of bilirubin bound to human serum albumin, except for (EZ)-bilirubin, is very rapid and much greater than that for bilirubin bound to rat serum albumin.
We describe the successful management of a patient with intracaval extension of renal cell carcinoma to the level of the diaphragm. Ultrasonography delineated clearly the upper extent of the tumor thrombus and revealed no invasion of the vena caval wall at or above the entrance of the main hepatic veins. To prevent severe hypotension caused by intrapericardial control of the inferior vena cava, the aorta was clamped temporarily just above the celiac axis. The tumor was removed completely en bloc with the thrombus. Blood loss was minimal and the patient recovered promptly.
Anionic sites were demonstrated ultrastructurally in both the dermal and epidermal edges of the lamina densa using strongly cationized polyethyleneimine as a tracer. Enzyme digestion with heparitinase and pronase removed the anionic binding sites, indicating that the sites consisted largely of heparan sulfate proteoglycan.
The origin and properties of the blister formation factor in recessive dystrophic epidermolysis bullosa (RDEB) blister fluids were investigated. Organ cultures of normal human skin incubated with RDEB dermis extract or with RDEB fibroblast culture medium (FCM) produced a clear subepidermal blister with histology similar to that of a RDEB blister in vivo. The injection of RDEB dermis extract into guinea-pig skin also induced dermal-epidermal separation with similar histology to the skin lesions of RDEB patients. The blister forming activity of RDEB FCM which induces the subepidermal blister was inactivated by heat (60 degrees C for 30 min), trypsin digestion and by treating with EDTA, EGTA, alpha 2-macroglobulin, diisopropyl fluorophosphate and N-ethylmaleimide, but was not affected by dialysis. These results suggest that the RDEB fibroblast produces a blister formation factor(s), and that blister formation may be caused by a combination of a metallo-protease, serine protease and SH protease.
Extensive morphological and biochemical studies were carried out on two sibling cases of ichthyosis linearis circumflexa. The condition was found to be similar to psoriasis in the following ways: effectiveness of PUVA therapy, psoriatic changes on light and electron microscopy, high urinary polyamine levels, elevation of several enzyme activities in the scales, and a remarkable change in keratin molecules. These results may reflect an increased epidermal proliferation with a reduced epidermal transit time, as occurs in psoriasis.
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A case of X-linked ocular albinism is reported. Characteristic Masson-Fontana positive and Dopa positive giant melanin granules were found in keratinocytes, melanocytes and upper dermis. Ultrastructurally the macromelanosome was composed of a dense core and a less dense surrounding mantle.
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The use of an unmodified conventional ventilator for an anaesthesia circuit is described, whereby the dilution of the anaesthetic gas by the driving gas is avoided due to the connecting deadspace. The method was investigated in model measurements, standard ventilator settings for normoventilation are suggested, and first clinical results are reported.
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Pathogenic mechanisms involved in the blister formation of epidermolysis bullosa (EB) are not clearly understood at present. In this paper, we attempted to produce experimental blistering in vitro similar to the histologic picture of EB simplex (EBS) and recessive dystrophic EB (RDEB). It was demonstrated by light and electron microscopy that the medium containing fresh blister fluids from the patients with EBS or RDEB could produce similar histologic features in normal human skin (in vitro) to those of the skin lesions of patients (in vivo). This observation may open new avenues of approach to studying these diseases.
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