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Summary It is generally agreed that combined deficiency of selenium and vitamin E leads to several abnormalities including Kashin-Beck disease which is an endemic and chronic degenerative osteoarthrosis. The abnormalities can be reversed by the administration of various forms of selenium and vitamin E.The present study was designed to investigate the effects of dietary selenium and vitamin E on bone tissue and on the biomechanical properties of bone. Young rabbits of both sexes were fed with either a selenium- and vitamin E-adequate diet (control group), or a selenium- and vitamin E-deficient diet or a selenium-excess diet. The selenium-deficient diet resulted in a significant decrease in plasma selenium level and the selenium-excess diet resulted in a significant increase in the plasma selenium level with respect to the corresponding control values (p<0.05). The diets did not affect the blood cell counts considerably but erythrocyte glutathione peroxidase activity increased (decreased) relatively when the plasma selenium level increased (decreased) (p<0.05). The light microscopic investigations of the bone tissues of the two experimental groups indicate that the findings of the present work are compatible with osteomalacia. The biomechanical properties of the bones from the three groups were determined experimentally with bending tests. Both the Se-and vitamin E-deficient diet and the Se-excess diet decreased the biomechanical strength of the bones significantly while the bones belonging to the control group always had the largest modulus of elasticity (p<0.05).  相似文献   
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Although selenium, an essential trace element and a component of glutathione peroxidase, is known to protect the heart from ischemia-reperfusion (I/R)-induced injury, the mechanisms of this protection are not fully understood. For this purpose, isolated rat hearts were subjected to 30 min of global ischemia followed by 30 min of reperfusion; sodium selenite (25-1,000 nM) was added in the perfusion medium 10 min prior to ischemia, as well as during reperfusion. Selenium caused a dose-dependent improvement in cardiac performance and attenuated the decrease in the ratio of reduced glutathione to oxidized glutathione, as well as the increased level of malondialdehyde in I/R heart. Elevated ratios of nuclear factor-kappaB (NF-kappaB) in particulate and cytosolic fraction and of phosphorylated NF-kappaB and total NF-kappaB in I/R hearts were reduced by selenium. Cardiac dysfunction in hearts perfused with xanthine plus xanthine oxidase mixture, as well as hydrogen peroxide, or subjected to Ca2+ paradox was also attenuated by selenium. These data suggest that selenium protects the heart against I/R injury due to its action on the redox state and deactivation of NF-kappaB in I/R hearts.  相似文献   
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Rett syndrome is a severe neurodevelopmental disorder that arises from mutations in the X-linked MECP2 gene. It is almost exclusively seen in girls due to the predominant occurrence of the mutations on the paternal X-chromosome, and also the early postnatal lethal effect of the disease causing mutations in hemizygous boys. We identified a boy with features of classic Rett syndrome who is mosaic for the truncating MECP2 mutation R270X. Chromosome analysis showed normal karyotype. These results indicate that a MECP2 mutation associated with Rett syndrome in females could lead to a similar phenotype in males as a result of somatic mosaicism.  相似文献   
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Reperfusion of hearts with a Ca2+-containing medium after a perfusion period in Ca2+-free medium results in irreversible cell damage (calcium paradox). In this investigation we have studied coronary flow and cyclic AMP and cyclic GMP levels after several periods of Ca2+-free perfusion in isolated rat hearts. We also investigated the effects of papaverine (Pap), noradrenaline (NA), acetylcholine (ACh) and absence of inorganic phosphate during Ca2+-free perfusion on coronary flow (CF) and cyclic nucleotide levels. Inability of the heart to recover contractile activity with development of contracture during the reperfusion period was accepted as indicative of the calcium paradox. Ca2+-free perfusion alone and NA and absence of inorganic phosphate during the Ca2+-free perfusion period increased CF, whereas Pap and ACh decreased it. However, only Ca2+-free perfusion and NA elevated cyclic AMP. On the other hand, Pap and ACh increased cyclic GMP (with a transient rise of cyclic AMP in Pap infusion), and absence of inorganic phosphate decreased both cyclic AMP and cyclic GMP. Pap, ACh and absence of phosphate prevented the calcium paradox. Our study suggests that increased cyclic AMP during the Ca2+-free perfusion may contribute, with the other factors, to the occurrence of the calcium paradox.  相似文献   
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BackgroundCardiovascular complication is one of the leading causes of mortality after liver transplantation (LT). Thus, a thorough cardiac evaluation is a must before proceeding to a liver transplant surgery. Percutaneous coronary intervention (PCI) with stent and to a lesser extent coronary artery bypass grafting (CABG) are both valuable treatment options for patients with coronary artery disease.MethodsA retrospective, single-center study that included patients who underwent cardiac intervention and subsequent LT for end-stage liver disease. All patients who had PCI or CABG were included in the study.ResultsTwenty-nine adult patients out of 51 had a cardiac intervention before liver transplantation. Twenty-four patients had a diagnostic PCI, 3 patients had therapeutic PCI with stent, and 2 had failed PCI and proceeded to CABG before liver transplant. The mean age of the patients was 60.5 years. There were 24 men. All patients had cirrhosis. The 2 CABG cases were done during the same admission with a 13- and 18-day interval between the CABG and the transplantation. Both cases were live-related liver transplantation. No mortality was reported.ConclusionIn case of PCI failure, CABG may be a valuable and safe treatment option for cirrhotic patients as a preparation for liver transplantation. Live donor liver transplantation may be a good back-up for those patients in case they develop hepatic decompensation.  相似文献   
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Coloboma of the lens is characterized by notching of the equator of the lens. The cause of the condition is faulty development of the zonule. The lens deprived of its normal pull in the defective region is thicker and more spherical as if there were no loss of its substance. We report five cases of lens coloboma and localized zonular defect without any associated systemic anomalies. One case included retinal detachment; another included an iris coloboma; and a third included iris, choroid and optic disk colobomas and retinal detachment with lens coloboma. The remaining two cases were without associated ocular anomalies. The pathogenic mechanisms and relationship of this defect with the differentiation of the tertiary vitreous and development of the zonule is discussed. The defect most likely occurs in the third to fourth months of gestation during differentiation of the tertiary vitreous in response to toxic, inflammatory or genetic factors.  相似文献   
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From November 3, 1975 to November 3, 1990, 874 kidney transplants were performed at our centers. Of these, 675 (77.2%) were from living donors and 199 (22.8%) were from cadaver donors. Five hundred eighty (66.4%) of the living donors were first degree related while 99 (11.3%) were unrelated or second degree related donors, 29 of which were spouses. All donor recipient pairs were ABO-compatible, with the exception of one pair. Donor recipient relations were wife to husband in 25 cases and husband to wife in 4 cases. All were first grafts and started functioning during surgery. In this series, the follow-up for the recipients was 4 to 64 months (mean 33.5 ± 4.5 months). One-year patient survival and graft survival rates were 92.4% and 81.9%, respectively. Two-year patient survival and graft survival rates were 92.4% and 78.2%, respectively. The single ABO-incompatible case is also doing well, 21 months postoperatively. This study demonstrates that the interspouse kidney transplantation may be used when cadaver organ shortage is a problem. While providing the couple with a better quality of life, interspouse kidney transplantation also enables the couple to share the joy of giving and receiving the gift of life from one another.
Resumen En nuestro centro se efectuaron 874 trasplantes renales entre noviembre 3 de 1975 y noviembre 3 de 1990; 675 (77.2%) fueron de donantes vivos y 199 (22.8%) de donantes cadavéricos; 580 (66.4%) de los donantes vivos fueron familiares de primer grado y 99 (11.3%) fueron donantes no relacionados familiarmente o familiares de segundo grado, de los cuales 29 eran cónyuges. Todas las parejas donante-recipiente exhibieron compatibilidad ABO, con excepción de una. La relación donante-recipiente fue esposa a esposo en 25 casos y esposo a esposa en 4 casos. Todos los injertos eran de primera vez y todos comenzaron a funcionar en la mesa de cirugía. El seguimiento osciló entre 4 y 64 meses (33.5 ± 4.5). Las tasas de sobrevida a un año del paciente y del injerto fueron 92.4% y 81.9% respectivamente; las tasas a dos años fueron 92.4% y 78.2% respectivamente. El único caso ABO no compatible también se encuentra bien, a 21 meses en la actualidad. El presente estudio demuestra que el trasplante renal entre esposos puede ser utilizado cuando haya escases de órganos cadavéricos. Al tiempo que permite una mejor calidad de vida, el procedimiento da a la pareja la oportunidad de gozar el hecho de otorgar y de recibir el regalo de la vida entre uno y otro.

Résumé Nous avons effectué 874 transplantations rénales dans nos centres de transplantation entre le 3 Nov, 1975 et le 3 Nov, 1990. Parmi celles-ci, 675 (77.2%) provenaient de donneurs vivants et 199 (22.8%) des reins provenaient de cadavres. Cinq cent quatre vingt des donneurs vivants (66.4%) étaient parents au premier degré alors que 99 (11.3%) étaient parents au 2è degré ou n'étaient pas parents, parmi lesquels 29 étaient des époux. Tous les couples donneur/receveur, sauf un, étaient compatibles dans le système ABO. Le couple donneur/receveur était femme à mari dans 25 cas et mari à femme dans quatre. Il s'agissait dans tous les cas d'une première greffe et qui a commencé à bien fonctionner sur la table d'opération. Dans cette série, le suivi des receveurs allait de 4 à 64 (33.5 ± 4.5) mois. Les taux de survie des malades et des greffes à un an étaient respectivement de 92.4% et 81.9%. Les taux de survie des malades et des greffes à deux ans étaient respectivement de 92.4% et 78.2%. Le seul cas avec incompatabilité ABO va très bien avec un recul de 21 mois. Cette étude montre que la transplantation entre époux est une solution valable en cas de manque de reins. En plus d'améliorer la qualité de survie du receveur et par là même du couple, cette variété de transplantation donne également au couple la possibilité d'avoir la joie de donner et de recevoir un cadeau de vie de leur époux.
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