Treatment of Corneal Opacification in Mucolipidosis iv with Conjunctival Transplantation
Mucolipidosis IV is a rare, inherited metabolic storage disease producing progressive psychomotor retardation and congenital corneal clouding. Histopathologic studies have shown that the corneal epithelium is strikingly involved with intracytoplasmic vacuolated storage material with relative noninvo...
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| Veröffentlicht in: | American journal of ophthalmology 1985-02, Vol.99 (2), p.137-141 |
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| container_end_page | 141 |
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| container_title | American journal of ophthalmology |
| container_volume | 99 |
| creator | Dangel, Matthew E. Bremer, Don L. Rogers, Gary L. |
| description | Mucolipidosis IV is a rare, inherited metabolic storage disease producing progressive psychomotor retardation and congenital corneal clouding. Histopathologic studies have shown that the corneal epithelium is strikingly involved with intracytoplasmic vacuolated storage material with relative noninvolvement of the corneal stroma and endothelium. Additionally, it has been noted that epithelial removal results in corneal clarity. We treated a 28-month-old patient with mucolipidosis IV with conjunctival transplantation with donor conjunctiva from an unaffected sibling. Improved corneal clarity resulted and has persisted with a follow-up of one year. |
| doi_str_mv | 10.1016/0002-9394(85)90221-1 |
| format | Article |
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Histopathologic studies have shown that the corneal epithelium is strikingly involved with intracytoplasmic vacuolated storage material with relative noninvolvement of the corneal stroma and endothelium. Additionally, it has been noted that epithelial removal results in corneal clarity. We treated a 28-month-old patient with mucolipidosis IV with conjunctival transplantation with donor conjunctiva from an unaffected sibling. Improved corneal clarity resulted and has persisted with a follow-up of one year.</description><identifier>ISSN: 0002-9394</identifier><identifier>EISSN: 1879-1891</identifier><identifier>DOI: 10.1016/0002-9394(85)90221-1</identifier><identifier>PMID: 3970116</identifier><identifier>CODEN: AJOPAA</identifier><language>eng</language><publisher>New York, NY: Elsevier Inc</publisher><subject>Adult ; Biological and medical sciences ; Conjunctiva - transplantation ; Cornea - ultrastructure ; Corneal Opacity - pathology ; Corneal Opacity - therapy ; Errors of metabolism ; Humans ; Lipids (lysosomal enzyme disorders, storage diseases) ; Male ; Medical sciences ; Metabolic diseases ; Mucolipidoses - complications</subject><ispartof>American journal of ophthalmology, 1985-02, Vol.99 (2), p.137-141</ispartof><rights>1985 Elsevier Inc.</rights><rights>1985 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c386t-10d8713706720a8ffe72071776e80b4be1e2dc07bfeae342eaa1c4496c771eba3</citedby><cites>FETCH-LOGICAL-c386t-10d8713706720a8ffe72071776e80b4be1e2dc07bfeae342eaa1c4496c771eba3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/0002-9394(85)90221-1$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3550,27924,27925,45995</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=9018124$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/3970116$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Dangel, Matthew E.</creatorcontrib><creatorcontrib>Bremer, Don L.</creatorcontrib><creatorcontrib>Rogers, Gary L.</creatorcontrib><title>Treatment of Corneal Opacification in Mucolipidosis iv with Conjunctival Transplantation</title><title>American journal of ophthalmology</title><addtitle>Am J Ophthalmol</addtitle><description>Mucolipidosis IV is a rare, inherited metabolic storage disease producing progressive psychomotor retardation and congenital corneal clouding. Histopathologic studies have shown that the corneal epithelium is strikingly involved with intracytoplasmic vacuolated storage material with relative noninvolvement of the corneal stroma and endothelium. Additionally, it has been noted that epithelial removal results in corneal clarity. We treated a 28-month-old patient with mucolipidosis IV with conjunctival transplantation with donor conjunctiva from an unaffected sibling. Improved corneal clarity resulted and has persisted with a follow-up of one year.</description><subject>Adult</subject><subject>Biological and medical sciences</subject><subject>Conjunctiva - transplantation</subject><subject>Cornea - ultrastructure</subject><subject>Corneal Opacity - pathology</subject><subject>Corneal Opacity - therapy</subject><subject>Errors of metabolism</subject><subject>Humans</subject><subject>Lipids (lysosomal enzyme disorders, storage diseases)</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Metabolic diseases</subject><subject>Mucolipidoses - complications</subject><issn>0002-9394</issn><issn>1879-1891</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1985</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kMFq3DAQhkVJSLZp36AFH0JpD241tteSLoGyJGkgIZct9CZkeUwneCVHkrfk7aPNLnvsaRjm-4efj7FPwL8Dh_YH57wqVa2ar3L5TfGqghLesQVIoUqQCk7Y4oics_cxPuW1FY04Y2e1EhygXbA_64AmbdClwg_FygeHZiweJ2NpIGsSeVeQKx5m60eaqPeRYkHb4h-lvxl3T7OzibY5sw7GxWk0Lr2lPrDTwYwRPx7mBft9c71e_SrvH2_vVj_vS1vLNpXAeymgFrlYxY0cBsxTgBAtSt41HQJWveWiG9Bg3VRoDNimUa0VArAz9QX7sv87Bf88Y0x6Q9HimIugn6MWSyWhVlUGmz1og48x4KCnQBsTXjRwvROqd7b0zpaWS_0mVEOOfT78n7sN9sfQwWC-Xx7uJlozDtmCpXjEFAcJVZOxqz2G2cWWMOhoCZ3FngLapHtP_-_xCnyskmE</recordid><startdate>19850215</startdate><enddate>19850215</enddate><creator>Dangel, Matthew E.</creator><creator>Bremer, Don L.</creator><creator>Rogers, Gary L.</creator><general>Elsevier Inc</general><general>Elsevier</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>19850215</creationdate><title>Treatment of Corneal Opacification in Mucolipidosis iv with Conjunctival Transplantation</title><author>Dangel, Matthew E. ; Bremer, Don L. ; Rogers, Gary L.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c386t-10d8713706720a8ffe72071776e80b4be1e2dc07bfeae342eaa1c4496c771eba3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1985</creationdate><topic>Adult</topic><topic>Biological and medical sciences</topic><topic>Conjunctiva - transplantation</topic><topic>Cornea - ultrastructure</topic><topic>Corneal Opacity - pathology</topic><topic>Corneal Opacity - therapy</topic><topic>Errors of metabolism</topic><topic>Humans</topic><topic>Lipids (lysosomal enzyme disorders, storage diseases)</topic><topic>Male</topic><topic>Medical sciences</topic><topic>Metabolic diseases</topic><topic>Mucolipidoses - complications</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Dangel, Matthew E.</creatorcontrib><creatorcontrib>Bremer, Don L.</creatorcontrib><creatorcontrib>Rogers, Gary L.</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>American journal of ophthalmology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Dangel, Matthew E.</au><au>Bremer, Don L.</au><au>Rogers, Gary L.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Treatment of Corneal Opacification in Mucolipidosis iv with Conjunctival Transplantation</atitle><jtitle>American journal of ophthalmology</jtitle><addtitle>Am J Ophthalmol</addtitle><date>1985-02-15</date><risdate>1985</risdate><volume>99</volume><issue>2</issue><spage>137</spage><epage>141</epage><pages>137-141</pages><issn>0002-9394</issn><eissn>1879-1891</eissn><coden>AJOPAA</coden><abstract>Mucolipidosis IV is a rare, inherited metabolic storage disease producing progressive psychomotor retardation and congenital corneal clouding. Histopathologic studies have shown that the corneal epithelium is strikingly involved with intracytoplasmic vacuolated storage material with relative noninvolvement of the corneal stroma and endothelium. Additionally, it has been noted that epithelial removal results in corneal clarity. We treated a 28-month-old patient with mucolipidosis IV with conjunctival transplantation with donor conjunctiva from an unaffected sibling. Improved corneal clarity resulted and has persisted with a follow-up of one year.</abstract><cop>New York, NY</cop><pub>Elsevier Inc</pub><pmid>3970116</pmid><doi>10.1016/0002-9394(85)90221-1</doi><tpages>5</tpages></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0002-9394 |
| ispartof | American journal of ophthalmology, 1985-02, Vol.99 (2), p.137-141 |
| issn | 0002-9394 1879-1891 |
| language | eng |
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| source | MEDLINE; Elsevier ScienceDirect Journals Complete |
| subjects | Adult Biological and medical sciences Conjunctiva - transplantation Cornea - ultrastructure Corneal Opacity - pathology Corneal Opacity - therapy Errors of metabolism Humans Lipids (lysosomal enzyme disorders, storage diseases) Male Medical sciences Metabolic diseases Mucolipidoses - complications |
| title | Treatment of Corneal Opacification in Mucolipidosis iv with Conjunctival Transplantation |
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