Matches in SemOpenAlex for { <https://semopenalex.org/work/W90167701> ?p ?o ?g. }
Showing items 1 to 78 of
78
with 100 items per page.
- W90167701 endingPage "36" @default.
- W90167701 startingPage "229" @default.
- W90167701 abstract "We have examined eyes from a heterozygote (carrier) of choroideremia, an X-linked disease. Gross examination revealed irregular pigmentation at the level of the retinal pigment epithelium (RPE) except at the posterior pole, and islands of well defined depigmentation of 1-4 mm in diameter in the midperiphery. The optic nerve and retinal blood vessels appeared normal, and there was minimal pigment migration into the retina. Histopathologic examination showed normal photoreceptors in the posterior and anterior fundus, but the outer segments were short or absent in much of the equatorial region. Little gliosis was noted in areas of retinal atrophy. The RPE was abnormal, with irregular thickness and pigmentation associated with variable lipofuscin content from one RPE cell to another, as shown by fluorescence microscopy. There were areas of profound atrophy in the equatorial region, with abrupt transitions between relatively normal RPE and photoreceptors, and retina devoid of RPE and photoreceptors. Bruch's membrane was thickened to a greater extent than is common in age-related change. The choriocapillaris was normal in areas with normal photoreceptors, except for widening of the intercapillary pillars. In those regions with abnormal photoreceptors, choroidal capillaries were fewer in number, had reduced luminal diameter, and fenestrae were sparse. In some areas of intense atrophy, there were no choroidal capillaries. The findings are compatible with the primary defect residing in the RPE. The Lyon hypothesis of X-chromosome inactivation and mosaicism could explain the irregularity of change and areas of intense atrophy, but abrupt demarcation between grossly abnormal, and relatively well preserved retina also occurs in hemizygotes (affected males)." @default.
- W90167701 created "2016-06-24" @default.
- W90167701 creator A5000506767 @default.
- W90167701 creator A5008057429 @default.
- W90167701 creator A5026064711 @default.
- W90167701 creator A5026898256 @default.
- W90167701 creator A5036422156 @default.
- W90167701 date "1990-02-01" @default.
- W90167701 modified "2023-10-02" @default.
- W90167701 title "A histopathologic study of a choroideremia carrier." @default.
- W90167701 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/2303326" @default.
- W90167701 hasPublicationYear "1990" @default.
- W90167701 type Work @default.
- W90167701 sameAs 90167701 @default.
- W90167701 citedByCount "30" @default.
- W90167701 countsByYear W901677012012 @default.
- W90167701 countsByYear W901677012013 @default.
- W90167701 countsByYear W901677012014 @default.
- W90167701 countsByYear W901677012015 @default.
- W90167701 countsByYear W901677012016 @default.
- W90167701 countsByYear W901677012017 @default.
- W90167701 countsByYear W901677012018 @default.
- W90167701 countsByYear W901677012019 @default.
- W90167701 countsByYear W901677012021 @default.
- W90167701 countsByYear W901677012022 @default.
- W90167701 crossrefType "journal-article" @default.
- W90167701 hasAuthorship W90167701A5000506767 @default.
- W90167701 hasAuthorship W90167701A5008057429 @default.
- W90167701 hasAuthorship W90167701A5026064711 @default.
- W90167701 hasAuthorship W90167701A5026898256 @default.
- W90167701 hasAuthorship W90167701A5036422156 @default.
- W90167701 hasConcept C105702510 @default.
- W90167701 hasConcept C118487528 @default.
- W90167701 hasConcept C142724271 @default.
- W90167701 hasConcept C169760540 @default.
- W90167701 hasConcept C186198217 @default.
- W90167701 hasConcept C2776391266 @default.
- W90167701 hasConcept C2777093970 @default.
- W90167701 hasConcept C2778024200 @default.
- W90167701 hasConcept C2779418775 @default.
- W90167701 hasConcept C2780827179 @default.
- W90167701 hasConcept C2781172350 @default.
- W90167701 hasConcept C71924100 @default.
- W90167701 hasConcept C86803240 @default.
- W90167701 hasConceptScore W90167701C105702510 @default.
- W90167701 hasConceptScore W90167701C118487528 @default.
- W90167701 hasConceptScore W90167701C142724271 @default.
- W90167701 hasConceptScore W90167701C169760540 @default.
- W90167701 hasConceptScore W90167701C186198217 @default.
- W90167701 hasConceptScore W90167701C2776391266 @default.
- W90167701 hasConceptScore W90167701C2777093970 @default.
- W90167701 hasConceptScore W90167701C2778024200 @default.
- W90167701 hasConceptScore W90167701C2779418775 @default.
- W90167701 hasConceptScore W90167701C2780827179 @default.
- W90167701 hasConceptScore W90167701C2781172350 @default.
- W90167701 hasConceptScore W90167701C71924100 @default.
- W90167701 hasConceptScore W90167701C86803240 @default.
- W90167701 hasIssue "2" @default.
- W90167701 hasLocation W901677011 @default.
- W90167701 hasOpenAccess W90167701 @default.
- W90167701 hasPrimaryLocation W901677011 @default.
- W90167701 hasRelatedWork W1979672627 @default.
- W90167701 hasRelatedWork W1996703105 @default.
- W90167701 hasRelatedWork W2006828037 @default.
- W90167701 hasRelatedWork W2294018641 @default.
- W90167701 hasRelatedWork W2417738566 @default.
- W90167701 hasRelatedWork W2479950082 @default.
- W90167701 hasRelatedWork W3031683782 @default.
- W90167701 hasRelatedWork W3129860535 @default.
- W90167701 hasRelatedWork W315931781 @default.
- W90167701 hasRelatedWork W90167701 @default.
- W90167701 hasVolume "31" @default.
- W90167701 isParatext "false" @default.
- W90167701 isRetracted "false" @default.
- W90167701 magId "90167701" @default.
- W90167701 workType "article" @default.