{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T02:08:41Z","timestamp":1789610921891,"version":"build-2803163510"},"reference-count":20,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2016,9,15]],"date-time":"2016-09-15T00:00:00Z","timestamp":1473897600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100006741","name":"Stiftelsen Frimurare Barnhuset i Stockholm","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100006741","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100006128","name":"Svenska Frimurarorden","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100006128","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Acta Paediatrica"],"published-print":{"date-parts":[[2016,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aim<\/jats:title><jats:p>There have been few studies on long\u2010term electroretinographic findings in patients with long\u2010chain 3\u2010hydroxyacyl\u2010CoA dehydrogenase deficiency (<jats:styled-content style=\"fixed-case\">LCHADD<\/jats:styled-content>). This study correlated long\u2010term electroretinographic findings with age, metabolic control and clinical symptoms.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We examined 12 Swedish patients with<jats:styled-content style=\"fixed-case\">LCHADD<\/jats:styled-content>. Visual acuity testing, fundus examinations, optical coherence tomography and electroretinography were performed. The results were correlated to age, the levels of 3\u2010hydroxyacylcarnitine and acylcarnitine and clinical metabolic control.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Blindness or moderate visual impairment was found in two patients. Retinal pigmentation, atrophy and fibrosis were present in 11, seven and one of the patients, respectively, and optical coherence tomography showed retinal thinning in three of the six patients examined. Electroretinography was performed on 11 of the 12 patients. It was pathological, with reduced rod and cone responses, in five patients, subnormal in four and was related to poor clinical metabolic control and severe neonatal symptoms. Repeated electroretinographies revealed reduced function with increasing age.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>More than 80% of the<jats:styled-content style=\"fixed-case\">LCHADD<\/jats:styled-content>patients developed pathological or subnormal retinal function. This was more pronounced in patients with neonatal symptoms, but ameliorated by strict dietary treatment. Annual ophthalmological follow\u2010ups, with electroretinography every second or third year, are recommended.<\/jats:p><\/jats:sec>","DOI":"10.1111\/apa.13536","type":"journal-article","created":{"date-parts":[[2016,7,27]],"date-time":"2016-07-27T11:27:30Z","timestamp":1469618850000},"page":"1451-1460","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":21,"title":["Most patients with long\u2010chain 3\u2010hydroxyacyl\u2010CoA dehydrogenase deficiency develop pathological or subnormal retinal function"],"prefix":"10.1111","volume":"105","author":[{"given":"Kristina Te\u00e4r","family":"Fahnehjelm","sequence":"first","affiliation":[{"name":"Department of Clinical Neuroscience Karolinska Institutet Stockholm Sweden"},{"name":"St Erik Eye Hospital Stockholm Sweden"},{"name":"Institute of Neuroscience and Physiology Sahlgrenska Akademin University of Gothenburg Gothenburg Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ying","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Clinical Neurophysiology Karolinska University Hospital Huddinge Sweden"},{"name":"Department of Ophthalmology The South Hospital Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Olsson","sequence":"additional","affiliation":[{"name":"Department of Women's and Children's Health Karolinska Institutet Stockholm Sweden"},{"name":"Department of Paediatrics Karolinska University Hospital Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Urban","family":"Amr\u00e9n","sequence":"additional","affiliation":[{"name":"Department of Clinical Neuroscience Karolinska Institutet Stockholm Sweden"},{"name":"St Erik Eye Hospital Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charlotte Bieneck","family":"Haglind","sequence":"additional","affiliation":[{"name":"Department of Women's and Children's Health Karolinska Institutet Stockholm Sweden"},{"name":"Department of Paediatrics Karolinska University Hospital Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerd","family":"Holmstr\u00f6m","sequence":"additional","affiliation":[{"name":"Department of Neuroscience\/ophthalmology Uppsala University Hospital Uppsala Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Halldin","sequence":"additional","affiliation":[{"name":"Department of Paediatric endocrinology Uppsala University Children's Hospital Uppsala Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sten","family":"Andreasson","sequence":"additional","affiliation":[{"name":"Department of Ophthalmology University of Lund Lund Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Nordenstr\u00f6m","sequence":"additional","affiliation":[{"name":"Department of Women's and Children's Health Karolinska Institutet Stockholm Sweden"},{"name":"Department of Paediatrics Karolinska University Hospital Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2016,9,15]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01799383"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(05)82826-4"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2012.02.015"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/0005-2760(94)90064-7"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00711779"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0161-6420(98)95019-9"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0420.2007.01121.x"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1136\/bjo.81.4.291"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1080\/02713689808951227"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1159\/000334874"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1005502626406"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1167\/iovs.14-14007"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-009-9000-2"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1167\/tvst.3.6.7"},{"key":"e_1_2_10_16_1","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1111\/j.1600-0420.1996.tb00376.x","article-title":"Full\u2010field electroretinograms in infants with hereditary tapetoretinal degeneration","volume":"219","author":"Andreasson S","year":"1996","journal-title":"Acta Ophthalmol Scand Suppl"},{"key":"e_1_2_10_17_1","volume-title":"Regression and Correlation. 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