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家族性腺瘤性息肉病中靠近视网膜血管的视网膜色素上皮的变化。

Changes in the retinal pigment epithelium close to retinal vessels in familial adenomatous polyposis.

作者信息

Schmidt D, Jung C E, Wolff G

机构信息

Universitäts-Augenklinik, Freiburg, Germany.

出版信息

Graefes Arch Clin Exp Ophthalmol. 1994 Feb;232(2):96-102. doi: 10.1007/BF00171670.

DOI:10.1007/BF00171670
PMID:8157181
Abstract

Congenital hypertrophy of the retinal pigment epithelium (CHRPE) is known to occur in patients with familial adenomatous polyposis. Its relation to the course of the retinal blood vessels is emphasized in this publication. A 15-year-old girl with familial polyposis coli showed a longitudinal strip of whitish change following the course of a superior nasal artery of the left eye, falsely appearing to enclose the blood vessel like a sheath. A 34-year-old woman from another family with polyposis coli also showed a longitudinally orientated area of hypo- and hyperpigmentation close to temporal retinal veins. Two further patients revealed hyperpigmentation of the RPE under retinal vessels. It is hypothesized that these changes indicate an effect of the hypo- and hyperpigmentations of the RPE on the development of retinal vessels. In four patients, multiple dotlike hyperpigmentations were found in the extreme periphery of the retina; however, the bigger patchy hyperpigmentations were predominantly located in the midperiphery of the retina.

摘要

已知先天性视网膜色素上皮肥大(CHRPE)发生于家族性腺瘤性息肉病患者中。本出版物强调了其与视网膜血管行程的关系。一名患有家族性结肠息肉病的15岁女孩,左眼鼻上象限动脉行程处有一条纵向的白色改变带,看似像鞘一样包裹着血管。另一个患有结肠息肉病家族的34岁女性,在颞侧视网膜静脉附近也有一个纵向的色素减退和色素沉着区域。另外两名患者视网膜血管下方的视网膜色素上皮出现色素沉着。据推测,这些变化表明视网膜色素上皮的色素减退和色素沉着对视网膜血管发育有影响。在4名患者中,视网膜最周边发现多个点状色素沉着;然而,较大的斑片状色素沉着主要位于视网膜中周边。

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2
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引用本文的文献

1
Fluorescein angiographic features of the congenital hypertrophy of the retinal pigment epithelium in the familial adenomatous polyposis.家族性腺瘤性息肉病中视网膜色素上皮先天性肥大的荧光素血管造影特征
Int Ophthalmol. 2005 Feb-Apr;26(1-2):59-65. doi: 10.1007/s10792-006-0007-5. Epub 2006 Jun 15.

本文引用的文献

1
Retinal vascular changes in congenital hypertrophy of the retinal pigment epithelium.视网膜色素上皮先天性肥大中的视网膜血管变化
Ophthalmology. 1993 Apr;100(4):471-4. doi: 10.1016/s0161-6420(93)31619-2.
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The Gardner syndrome. Significance of ocular features.
Ophthalmology. 1984 Aug;91(8):916-25. doi: 10.1016/s0161-6420(84)34213-0.
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Prevalence and importance of pigmented ocular fundus lesions in Gardner's syndrome.加德纳综合征中色素性眼底病变的患病率及重要性
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The gene for familial polyposis coli maps to the long arm of chromosome 5.家族性结肠息肉病基因定位于5号染色体长臂。
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Pigmented ocular fundus lesions in the inherited gastrointestinal polyposis syndromes and in hereditary nonpolyposis colorectal cancer.遗传性胃肠道息肉综合征及遗传性非息肉病性结直肠癌中的色素性眼底病变。
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Close linkage of a highly polymorphic marker (D5S37) to familial adenomatous polyposis (FAP) and confirmation of FAP localization on chromosome 5q21-q22.一个高度多态性标记(D5S37)与家族性腺瘤性息肉病(FAP)的紧密连锁以及FAP在5号染色体q21-q22区域定位的确认。
Hum Genet. 1988 Jun;79(2):183-5. doi: 10.1007/BF00280563.
10
Focal congenital anomalies of the retinal pigment epithelium.视网膜色素上皮局灶性先天性异常。
Eye (Lond). 1989;3 ( Pt 1):1-18. doi: 10.1038/eye.1989.2.