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[门克斯综合征(毛发硫营养不良)中早幼粒细胞的单系空泡化(作者译)]

[Monophylic vacuolisation of promyelocytes in Menke's-syndrome (trichopoliodystrophy) (author's transl)].

作者信息

Heyne K, Dörner K, Graucob E, Wiedemann H R

出版信息

Klin Padiatr. 1978 Nov;190(6):576-9.

PMID:568684
Abstract

In one case of Menkes' Syndrome (Trichopoliodystrophy), a monophylic vacuolisation of myeloic cells (promyelocytes) of the bone marrow was observed. This finding correlates with an identical observation reported in medical literature, as well as with vacuolisations of metabolic active cells of the brain, musculature and skin observed in patients suffering from this disease. In the present paper, this finding is interpreted as an expression of the underlying disease and of the deficiency of oxidative cell ferment systems, and is discussed as a possible diagnostic and therapeutic criterion.

摘要

在一例门克斯综合征(毛发硫营养不良)中,观察到骨髓中髓样细胞(早幼粒细胞)出现单系空泡化。这一发现与医学文献中报道的相同观察结果相关,也与该疾病患者大脑、肌肉组织和皮肤中代谢活跃细胞的空泡化相关。在本文中,这一发现被解释为潜在疾病和氧化细胞发酵系统缺陷的一种表现,并作为一种可能的诊断和治疗标准进行了讨论。

相似文献

1
[Monophylic vacuolisation of promyelocytes in Menke's-syndrome (trichopoliodystrophy) (author's transl)].[门克斯综合征(毛发硫营养不良)中早幼粒细胞的单系空泡化(作者译)]
Klin Padiatr. 1978 Nov;190(6):576-9.
2
[Menkes' disease (new skin and hair ultrastructural abnormalities) (author's transl)].门克斯病(新的皮肤和毛发超微结构异常)(作者译)
Ann Dermatol Venereol. 1978 May;105(5):493-8.
3
[X - linked copper malabsorption (trichopoliodystrophy, Menkes' disease). Anatomical and neurochemical study of a case (author's transl)].
Ann Pathol. 1981;1(1):69-76.
4
Trichopoliodystrophy: report of a case with ultrastructural study.毛发硫营养不良:1例超微结构研究报告
J Med Assoc Thai. 1982 Mar;65(3):158-66.
5
[Menke's disease. A case report (author's transl)].[门克斯病。病例报告(作者译)]
An Esp Pediatr. 1977 Feb;10(2):205-14.
6
[Autopsy case of Menkes kinky hair disease].[门克斯卷发综合征尸检病例]
Nihon Rinsho. 1979 Mar 10;37(3):655-7.
7
[Menkes disease: anatomoclinical study].[门克斯病:解剖临床研究]
Med Cutan Ibero Lat Am. 1980;8(1-3):23-32.
8
[Neuropathological study of Menkes' kinky hair disease: on the mechanism of development of cerebrovascular lesions (author's transl)].
No To Shinkei. 1982 Apr;34(4):311-9.
9
[Ultrastructural study of the hair in Menkes' syndrome].
G Ital Dermatol Venereol. 1982 Sep-Oct;117(5):269-72.
10
[Macular dystrophy in menkes disease. Histological ocular study (author's transl)].门克斯病中的黄斑营养不良。眼部组织学研究(作者译)
J Fr Ophtalmol. 1978 Jun-Jul;1(6-7):457-60.

引用本文的文献

1
ATP7A-Regulated Enzyme Metalation and Trafficking in the Menkes Disease Puzzle.ATP7A调控的酶金属化与门克斯病谜题中的转运
Biomedicines. 2021 Apr 6;9(4):391. doi: 10.3390/biomedicines9040391.
2
A case of Menkes syndrome with cataracts.一例伴有白内障的门克斯综合征。
Eur J Pediatr. 1982 Jul;138(4):357-8. doi: 10.1007/BF00442519.