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[舍格伦-拉松综合征]

[Sjögren-Larsson syndrome].

作者信息

Fehlow P, Tennstedt A

出版信息

Psychiatr Neurol Med Psychol (Leipz). 1975 Jun;27(6):341-7.

PMID:1208705
Abstract

The observations made on a 27-year-old patient with ichthyosis, debility, psychoinfantilism, and Little's syndrome are reported. Clinically, the condition of the skin is similar to that associated with ichthyosis vulgaris. Histologically, however, the picture is largely that of ichthyosis congenita, the condition being even more pronounced than that of ichthyosis congenita mitis. This particular case may be placed within the class of transitional forms of hereditary ichthyoses, and it may thus be proved that the Sjögren-Larsson syndrome also has its 'formes de passage'. In the etiological determination of oligophrenias and infantile cerebral motor disorders it is always necessary to give attention to the probable presence of ichthyosiform conditions of the skin.

摘要

报告了对一名患有鱼鳞病、虚弱、精神幼稚症和利特尔综合征的27岁患者的观察结果。临床上,皮肤状况与寻常型鱼鳞病相关的状况相似。然而,组织学上,其表现主要是先天性鱼鳞病的表现,这种情况比重症先天性鱼鳞病更为明显。这个特殊病例可能属于遗传性鱼鳞病的过渡形式类别,因此可以证明舍格伦-拉尔松综合征也有其“过渡形式”。在智力发育迟缓及婴儿期脑性运动障碍的病因判定中,始终有必要关注皮肤鱼鳞病样状况可能的存在。

相似文献

1
[Sjögren-Larsson syndrome].[舍格伦-拉松综合征]
Psychiatr Neurol Med Psychol (Leipz). 1975 Jun;27(6):341-7.
2
[Dermatoglyphic characteristics in hereditary ichthyosis].[遗传性鱼鳞病的皮纹特征]
Vestn Dermatol Venerol. 1989(1):18-20.
3
Laboratory tests for ichthyosis.
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[Generalized ichthyosis associated with a spastic syndrome of the lower limbs, a variant of Sjögren-Larsson syndrome? Light and electron microscopy study of ichthyosis and its evolution with oral etretinate treatment].[与下肢痉挛综合征相关的全身性鱼鳞病,舍格伦-拉尔松综合征的一种变异型?鱼鳞病的光镜和电镜研究及其口服依曲替酯治疗的演变]
Ann Dermatol Venereol. 1984;111(10):885-92.
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[Sjogren-Larsson syndrome. Apropos of a case].
Ann Dermatol Venereol. 1987;114(8):947-55.
6
The expression of the 27-kd heat shock protein in keratinization disorders: an immunohistological study.27-kD热休克蛋白在角化异常性疾病中的表达:一项免疫组织学研究。
Hum Pathol. 2005 Jun;36(6):686-93. doi: 10.1016/j.humpath.2005.04.009.
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[Rings of white dots in the fundus oculi in patients with Sjögren-Larsson syndrome].[干燥综合征-拉松综合征患者眼底的白色点状环]
Lakartidningen. 1982 Jun 9;79(23):2308-10.
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Ichthyoses: differential diagnosis and molecular genetics.鱼鳞病:鉴别诊断与分子遗传学
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[Contribution to the Sjögren-Larsson syndrome (author's transl)].对舍格伦-拉尔松综合征的贡献(作者译)
MMW Munch Med Wochenschr. 1974 Mar 15;116(11):577-80.
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A syndrome of congenital ichthyosis, hypogonadism, small stature, facial dysmorphism, scoliosis and myogenic dystrophy.一种先天性鱼鳞病、性腺功能减退、身材矮小、面部畸形、脊柱侧弯和肌源性营养不良综合征。
Ann Genet. 1999;42(1):45-50.

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Neuroradiology. 1981 Mar;21(2):101-5. doi: 10.1007/BF00342989.