[Absence of a high level of skin Langerhans cells during Fabry's disease].
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Biomedical subjects
Publications and source records attributed to J Guillemette.
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Among 500 patients on maintenance hemodialysis, 6 patients (5 young women and a 49-year-old man) developed bullous dermatosis, 2-54 months after initiating dialysis treatment. The skin lesions occurred mainly in sunlight-exposed areas, and 4 out of the 6 patients showed increased cutaneous fragility in response to trauma. Skin biopsy revealed subepidermal blisters for all of them, and skin immunofluorescence studies were negative for 2 patients. No increase in fecal or red cell coproporhyrin and protoporphyrin levels was found in any of the 6 patients. The syndrome was clinically and histologically indistinguishable from porphyria cutanea tarda.
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Generalized lymphangiomatosis is an extremely rare condition with clinical features depending on the extent of involvement. We report a newborn infant with chylothorax and cutaneous lymphangiomas of unique clinical presentation. The baby required artificial ventilation, pleural drainage, low triglyceride infusions, and diet. The course was favorable, with a complete regression of the cutaneous lymphangiomas.
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Hepatocytes in culture represent a useful model for investigating the effects of toxic agents on liver cells. However, further development of this model is hampered by the difficulty in promoting cell proliferation over prolonged periods and the lack of knowledge about the biochemical status of the cells relevant to the toxic response under proliferation conditions. In an effort to overcome these limitations, this work focused on the establishment of conditions to ameliorate the promotion of hepatocyte proliferation in vitro. It also examined the effects of growth stimuli on the levels of glutathione (GSH), a highly significant parameter influencing the resistance against toxic agents. In addition, albumin secretion was monitored as an indicator of liver-specific functions. Two modified L-15 media were developed: medium A for supporting cell differentiation, and medium B for promotion of proliferation. Collagen and Matrigel were used as substrata. In medium A, the time course of GSH levels was comparable for both substrata, with an initial increase followed by a plateau and then by a progressive decrease from the second to the fourth week. Hepatocytes cultured on collagen and sequentially exposed to medium B (containing epidermal growth factor +/- norepinephrine) and medium A, showed repeated responsiveness to stimulation of DNA synthesis. Moreover, for cultures on collagen, a higher GSH content was observed in parallel with DNA synthesis stimulation, while albumin secretion was diminished. Although cells on Matrigel were refractory to DNA synthesis stimulation, GSH levels were still increased upon exposure to the growth factors, while under these conditions, albumin synthesis remained unaltered.(ABSTRACT TRUNCATED AT 250 WORDS)
We report a case of multiform granuloma observed in a young woman of 28 years from Brazzaville, Congo. Extended lesions on the arms, back, face, legs and feet appeared over one month. They consisted of slightly squamous erythemato-oedematous plaques, with polycyclical margins and raised papular borders. The diagnosis was made histologically and recovery was spontaneous over a period of two months. We discuss clinical and histopathological aspects, which can sometimes lead to confusion with Hansen's disease.