{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,31]],"date-time":"2025-12-31T07:37:39Z","timestamp":1767166659243,"version":"build-2238731810"},"reference-count":20,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2007,12,20]],"date-time":"2007-12-20T00:00:00Z","timestamp":1198108800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Acta Ophthalmologica"],"published-print":{"date-parts":[[2008,5]]},"abstract":"<jats:title>Abstract.<\/jats:title>\n                  <jats:p>\n                    <jats:bold>Purpose:\u2002<\/jats:bold>\n                    To present long\u2010term ocular complications and electroretinographic (ERG) findings in children with long\u2010chain 3\u2010hydroxyacyl\u2010CoA dehydrogenase (LCHAD) deficiency \u2013 a life\u2010threatening metabolic disease \u2013 and the relation to age at diagnosis, treatment and other clinical parameters.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Methods:\u2002<\/jats:bold>\n                    Ten children with LCHAD deficiency underwent repeated ophthalmological evaluations including ERG.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Results:\u2002<\/jats:bold>\n                    All 10 children developed chorioretinal pathology. Regardless of age at diagnosis, initiation of treatment and age at examination, inter\u2010individual differences were present. Profound chorioretinal atrophy, severe visual impairment and progressive myopia had developed in two teenagers. Milder chorioretinopathy with or without subnormal visual acuity was present in all other children. ERG was pathological in seven children. The chorioretinopathy often started in the peripapillary or perimacular areas. In one patient, unilateral visual impairment was associated with fibrosis.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Conclusion:\u2002<\/jats:bold>\n                    Early diagnosis and adequate therapy might delay but not prevent the progression of retinal complications. Late diagnosis with severe symptoms at diagnosis, neonatal hypoglycaemia and frequent decompensations may increase the progression rate of the chorioretinopathy.\n                  <\/jats:p>\n                  <jats:p>LCHAD deficiency, a potentially lethal disease, is sometimes difficult to diagnose. Unusual chorioretinal findings should alert the ophthalmologist to the long\u2010chain 3\u2010hydroxyacyl\u2010CoA dehydrogenase deficiency, especially if there is a history of neonatal hypoglycaemia or failure to thrive.<\/jats:p>","DOI":"10.1111\/j.1600-0420.2007.01121.x","type":"journal-article","created":{"date-parts":[[2008,5,20]],"date-time":"2008-05-20T12:12:01Z","timestamp":1211285521000},"page":"329-337","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":25,"title":["Ocular characteristics in 10 children with long\u2010chain 3\u2010hydroxyacyl\u2010CoA dehydrogenase deficiency: a cross\u2010sectional study with long\u2010term follow\u2010up"],"prefix":"10.1111","volume":"86","author":[{"given":"Kristina Te\u00e4r","family":"Fahnehjelm","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gerd","family":"Holmstr\u00f6m","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Liu","family":"Ying","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Charlotte 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