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首页> 外文期刊>Journal of dermatological science >Dermal elastic fibres in the inherited hypermobile disorders.
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Dermal elastic fibres in the inherited hypermobile disorders.

机译：遗传性运动亢进性疾病中的皮肤弹性纤维。

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BACKGROUND: Elastic fibres in the inherited hypermobile disorders are probably abnormal on the inherited grounds. The abnormality may contribute for diagnosis and investigation of connective tissue biology. OBJECTIVE: The abnormality will be specific for every disorders and disclosed in the age-dependent change when exposure-dependent change was excluded. MATERIALS AND METHODS: Skin specimens from Ehlers-Danlos syndrome and hypermobile syndrome, Marfan syndrome, Osteogenesis imperfecta type I, homocysteinuria and normal controls are studied by routine electron microscopy. Age-dependent ultrastructural change of broad elastic fibres was evaluated in reticular dermis. RESULTS: Age-dependent change was categorized in infantile, adolescent, adult and senile pattern. Infantile pattern showed normal ultrastructure. Degeneration was first found in adolescent pattern by disarrayed microfibrils and degenerate matrix. Degeneration proceeded in adult pattern and ended in senile pattern. Typical abnormality developed in adult pattern. Ehlers-Danlos syndrome and hypermobile syndrome showed no specific abnormality but the degeneration began earlier and was severer than the control. Marfan syndrome showed defects of microfibrils and matrix surface. Moth-eaten figure was characteristic. Osteogenesis imperfecta showed abnormal matrix and revealed homogenous bulges of matrix. Homocysteinuria was specified by numerous microfibrils on the matrix surface in infantile pattern. CONCLUSION: Abnormality of elastic fibre was recognized in the reticular dermis of elbow. Ehlers-Danlos syndrome and hypermobile syndrome showed no specific abnormality but the degeneration was more intensive than the control. Abnormality of Marfan syndrome was degeneration of elastic microfibrils and matrix surface, Osteogenesis imperfecta was characterized by excess amount of matrix. Homocysteinuria revealed numerous microfibrils. Ultrastructural abnormality provided grounds for studies on histopathology and biology of elastic fibre.
机译：背景：遗传性超活动性疾病中的弹性纤维可能是遗传性异常。异常可能有助于结缔组织生物学的诊断和研究。目的：该异常将针对每种疾病，并且在排除暴露相关变化的情况下，会在年龄相关变化中披露。材料与方法：通过常规电子显微镜研究了Ehlers-Danlos综合征和高活动性综合征，Marfan综合征，I型成骨不全症，高半胱氨酸尿症和正常对照的皮肤标本。在网状真皮中评估了年龄依赖性的宽弹性纤维的超微结构变化。结果：年龄依赖性变化分为婴儿，青少年，成人和老年模式。婴儿模式显示正常的超微结构。变性首先是由杂乱的微纤维和变性基质以青春期模式发现的。变性以成年模式进行，并以老年模式结束。在成人模式中发展出典型的异常。 Ehlers-Danlos综合征和高活动性综合征没有表现出特定的异常，但变性较早开始，并且比对照组更严重。马凡氏综合症显示出微纤维和基质表面的缺陷。吃蛾子的人物是特征。成骨不全症表现出异常的基质并显示出基质的均匀凸起。同型半胱氨酸尿症是由婴儿表面的基质表面上的许多微纤维指明的。结论：在肘部网状真皮中发现弹性纤维异常。 Ehlers-Danlos综合征和高活动性综合征没有表现出特定的异常，但变性比对照组更严重。马凡氏综合征的异常是弹性微纤维和基质表面的变性，成骨不全的特征是基质过量。高半胱氨酸尿症揭示了许多微纤维。超微结构异常为弹性纤维的组织病理学和生物学研究提供了依据。

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