| Ocular histopathologic studies of neonatal and childhood adrenoleukodystrophy. | |
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MedLine Citation:
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PMID: 6295171 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Histopathologic studies of the eyes of one patient (a boy who died at 14 years of age) with childhood adrenoleukodystrophy and two patients (girls who died at 24 and 31 months of age) with neonatal adrenoleukodystrophy showed the accumulation of the characteristic bileaflet inclusions in optic nerve macrophages, retinal neurons, and macrophages and loss of ganglion cell and nerve fiber layer. Additionally, in the two cases of neonatal adrenoleukodystrophy, changes resembling early retinitis pigmentosa were found, with accumulation of characteristic inclusions in the retinal pigment epithelium and pigment-laden macrophages. One of the patients with neonatal adrenoleukodystrophy also had an anterior subcapsular cataract and cystoid macular edema. |
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Authors:
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S M Cohen; W R Green; Z C de la Cruz; F R Brown; H W Moser; M W Luckenbach; D J Dove; I H Maumenee |
Publication Detail:
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Type: Case Reports; Journal Article; Research Support, U.S. Gov't, P.H.S. |
Journal Detail:
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Title: American journal of ophthalmology Volume: 95 ISSN: 0002-9394 ISO Abbreviation: Am. J. Ophthalmol. Publication Date: 1983 Jan |
Date Detail:
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Created Date: 1983-02-25 Completed Date: 1983-02-25 Revised Date: 2007-11-14 |
Medline Journal Info:
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Nlm Unique ID: 0370500 Medline TA: Am J Ophthalmol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 82-96 Citation Subset: AIM; IM |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adrenoleukodystrophy
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pathology* Atrophy Child, Preschool Diffuse Cerebral Sclerosis of Schilder / pathology* Female Humans Inclusion Bodies / ultrastructure Infant, Newborn Infant, Newborn, Diseases / pathology* Macrophages / ultrastructure Male Microscopy, Electron Nerve Fibers, Myelinated / ultrastructure Optic Nerve / pathology* Pigment Epithelium of Eye / ultrastructure Retina / pathology* Retinal Ganglion Cells / ultrastructure Retinitis Pigmentosa / pathology |
| Grant Support | |
ID/Acronym/Agency:
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EY 01773/EY/NEI NIH HHS; RR 05378-20/RR/NCRR NIH HHS |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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