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黏多糖贮积症患者皮肤的超微结构。血浆输注治疗前后的研究。

Ultrastructure of the skin in mucopolysaccharidoses. Studies performed before and after plasma infusion therapy.

作者信息

Lasser A, Carter D M, Mahoney M J

出版信息

Arch Pathol. 1975 Mar;99(3):173-6.

PMID:804305
Abstract

Skin biopsy specimens of two siblings with Hunter syndrome and two siblings with type B Sanfilippo syndrome were examined by electron microscopy, at six-month intervals, before and after plasma infusion therapy. Electron-lucent, membrane-bound vacuoles were seen in fibroblasts, Schwann cells, and in mononuclear cells both before and after the infusions. Based on the ultrastructural, clinical, and biochemical findings, it was concluded that there was no therapeutic benefit from these plasma infusions.

摘要

对两名患有亨特综合征的兄弟姐妹以及两名患有B型桑菲利普综合征的兄弟姐妹的皮肤活检标本,在血浆输注治疗前后每隔六个月进行一次电子显微镜检查。在输注前后,在成纤维细胞、施万细胞和单核细胞中均可见电子透明的膜结合空泡。根据超微结构、临床和生化检查结果,得出结论:这些血浆输注没有治疗益处。

相似文献

1
Ultrastructure of the skin in mucopolysaccharidoses. Studies performed before and after plasma infusion therapy.黏多糖贮积症患者皮肤的超微结构。血浆输注治疗前后的研究。
Arch Pathol. 1975 Mar;99(3):173-6.
2
The ultrastructure of the skin in patients with mucopolysaccharidoses.黏多糖贮积症患者皮肤的超微结构
Lab Invest. 1974 Nov;31(5):488-502.
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[Cytochemical and electron microscopic research on the peripheral blood leukocytes and skin fibroblasts in mucopolysaccharidoses].黏多糖贮积症患者外周血白细胞及皮肤成纤维细胞的细胞化学与电子显微镜研究
Tsitol Genet. 1986 Sep-Oct;20(5):330-4.
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[Ultrastructure of skin fibroblasts in storage diseases (mucopolysaccharidosis types IV and VI)].[贮积病(IV型和VI型黏多糖贮积症)中皮肤成纤维细胞的超微结构]
Tsitol Genet. 1980 Sep-Oct;14(5):21-6.
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Ultrastructure of the skin in the genetic mucopolysaccharidoses.遗传性黏多糖贮积症患者皮肤的超微结构
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Mucopolysaccharidoses: ultrastructure of leukocyte inclusions.黏多糖贮积症:白细胞包涵体的超微结构
Ann Neurol. 1980 Sep;8(3):332-6. doi: 10.1002/ana.410080320.
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[Comparative study of the ultrastructure of the fibroblasts of tissue culture and skin biopsy in mucopolysacchariodoses].[黏多糖贮积症中组织培养成纤维细胞与皮肤活检成纤维细胞超微结构的比较研究]
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引用本文的文献

1
Mucopolysacccharidoses: From understanding to treatment, a century of discoveries.黏多糖贮积症:从认识到治疗,一个世纪的探索。
Genet Mol Biol. 2012 Dec;35(4 (suppl)):924-31. doi: 10.1590/s1415-47572012000600006. Epub 2012 Dec 18.
2
Morphological study of skin biopsy specimens: a contribution to the diagnosis of metabolic disorders with involvement of the nervous system.皮肤活检标本的形态学研究:对伴有神经系统受累的代谢紊乱诊断的贡献。
J Neurol Neurosurg Psychiatry. 1978 Mar;41(3):232-48. doi: 10.1136/jnnp.41.3.232.