Biomedical subjects
Publications and source records attributed to C Jeandel.
Nephroblastoma is a rare tumor in adult. We report here one case in which all the radiological examination were performed. The diagnosis was made by histological study. As a rule, except if some signs are present, it's impossible to suggest diagnosis on X-rays.
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The authors report the case of a patient with a stable IgA kappa gammapathy over several years which finally evolved to an IgA lambda myeloma during which the initial gammapathy regressed. As the two gammapathies probably arose from two different cellular populations, the regression of the IgA kappa dysglobulinaemia is an additional argument in favour of the hypothesis that benign monoclonal gammapathies are the result of cellular hyperplasia.
The exact nature of the genetic defect of hyalinosis cutis et mucosae or d'Urbach-Whiete syndrome is still matter of controversy. The present article reports on three new cases in which several different ultrastructural and biochemical investigations add more arguments to support an anomaly of the glycosaminoglycans degradation in the dermal fibroblasts. Cationic dyes as polyethyleneimine and alcian blue show an intense ultrastructural staining of the abnormal basal laminae and the intracellular lysosomal bodies in cultured fibroblasts. These are related to the accumulation of anionic charged proteoglycans. The primary defect of hyalinosis cutis et mucosae is likely due to a lysosomal defect so far not biochemically defined.
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Authors report an original case of mental neuropathy which reveal prolymphocytoid transformation of Chronic lymphocytic leukaemia. They discuss various pathogenic hypotheses which can join these two events; these are evolutive markers of bad prognosis concerning this hemopathy. Clinical, etiopathological and prognostic features of mental neuropathy associated with hemopathies are studied through this case.
Pulmonary arterial hypertension may develop in patients with systemic lupus erythematosus (SLE) in the absence of lung tissue lesion or embolism in the pulmonary circulation. Its mechanisms and prognosis are imperfectly known, although various suggestions have been made concerning the possible role of pulmonary arterial spasm, immune complex arteritis or arterial wall fibrosis. We report two cases of SLE in female patients who presented with clinical signs of pulmonary arterial hypertension. The fact that pulmonary arterial hypertension regressed completely in one patient and resulted in death in the other points to different pathogenic mechanisms. In the first patient the dramatic therapeutic effectiveness of a calcium inhibitor suggests that an arterial spasm was involved, whereas the anatomical lesions found in the second patient are in favour of a fibrotic inflammatory arteritis. This pathogenic heterogeneity of pulmonary arterial hypertension in SLE, which may correspond to different evolutive stages of the disease, is documented by a review of the literature with special attention to the frequency and to the clinical biochemical, haemodynamic and histological aspects of this complication of SLE.
Virilization of the external genitalia in the male fetus requires testosterone and dihydrotestosterone (DHT), which is formed from testosterone by the action of the enzyme, 5alpha-reductase type 2 (5alphaR-2). Mediation of the effects of both testosterone and DHT requires a functional androgen receptor (AR) located in the cytoplasmic compartment of target cells. DHT (or testosterone) binding induces a conformational change which facilitates AR nuclear transport, phosphorylation and dimerization, ultimately regulating of the rate of transcription of androgen-dependent genes. Any event which impairs DHT formation (mutation within the 5alphaR-2 gene or 5alphaR-2 inhibitors) or normal function of the AR (mutation in the AR gene, antiandrogens) may result in insufficient androgen action in the male fetus and in subsequent undervirilization in the newborn. Hypospadias may be due to a defect in androgen action due to mutation of the 5alphaR-2 or of the AR gene. Mutation of unidentified genes is likely to underlie this displacement of the urethral meatus from the tip to the ventral side of the phallus. An aetiological role for environmental chemical products has been postulated, since ethnic as well as geographical differences in the incidence of hypospadias have been noted. Increasing evidence has been gathered indicating that widely used industrial and agricultural chemicals have deleterious effects on normal male sexual differentiation. Cryptorchidism and micropenis may represent an intersex phenotype, even if they are isolated. Aetiological factors include 5alphaR-2 gene mutation, AR gene mutation or environmental hormonal disruptors. In conclusion, several phenotypes have been attributed to insufficient androgen action during fetal life. Whereas mutations in the 5alphaR-2 gene and AR gene are natural, attention should be focused on environmental endocrine disruptors that are able to mimic steroid 5alpha-reductase deficiency or partial androgen insensitivity syndrome.
Although weight, lean mass, fat mass and muscular strength are often found to be intercorrelated, the respective role of each parameter in bone mineral density (BMD) remains unknown in older women. The aim of the present study was to investigate the relationship between body weight and composition and quadriceps strength on femoral neck and lumbar spine BMD in healthy postmenopausal women. The relationship between isokinetic quadriceps strength measured by Biodex and BMD measured by dual energy X-ray absorptiometry was studied in 56 women aged 60-81 (70.5 +/- 6.2) years in multiple regression models adjusted for age, body composition and menopausal treatment. Weight and age were associated with femoral neck BMD (33 and 10% of variance accounted for, respectively) and lumbar spine BMD (23 and 8% of its variance). When body weight and quadriceps strength were excluded from the model, lean mass and age were associated with femoral neck BMD (29 and 14% of variance explained, respectively) and lumbar spine BMD (28 and 11% of variance explained, respectively). When quadriceps strength was entered into the model, it was strongly associated with femoral neck BMD (30% of variance accounted for), in addition to lean mass (9%) and age (7%), whereas it was not associated with lumbar spine BMD. In conclusion, lean mass explains a great part of the strong association between body weight and femoral neck and lumbar spine BMD. Quadriceps strength explains a great part of the association between lean mass and BMD at the femoral neck site but not at the lumbar spine site. These results suggest a site-specific effect of muscular strength on bone and a potential role of the age-related decline of muscle strength in age-related bone loss in postmenopausal women.
BACKGROUND: Ageing is associated with a reduction in balance, in particular through dysfunction of each level of postural control, which results in an increased risk of falling. Conversely, the practice of physical activities has been shown to modulate postural control in elderly people. OBJECTIVE: This study examined the potential positive effects of two types of regular physical and sporting activities on vestibular information and their relation to posture. METHODS: Gaze and postural stabilisation was evaluated by caloric and rotational vestibular tests on 18 healthy subjects over the age of 60 who regularly practised low-energy or bioenergetic physical activities and on 18 controls of a similar age who only walked on a regular basis. These subjects were also submitted to static and dynamic posturographic tests. RESULTS: The control group displayed less balance control, with a lower vestibular sensitivity and a relatively high dependency on vision compared to the group practising low-energy physical activities, which had better postural control with good vestibular sensitivity and less dependency on vision. The postural control and vestibular sensitivity of subjects practising bioenergetic activities was average, and required higher visual afferent contribution. CONCLUSION: Low-energy exercises, already shown to have the most positive impact on balance control by relying more on proprioception, also appear to develop or maintain a high level of vestibular sensitivity allowing elderly people practising such exercises to reduce the weight of vision.
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Explore the source record for details and available documents.
Explore the source record for details and available documents.
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