{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T12:06:14Z","timestamp":1790683574892,"version":"4.1.0"},"reference-count":29,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","license":[{"start":{"date-parts":[[2015,9,1]],"date-time":"2015-09-01T00:00:00Z","timestamp":1441065600000},"content-version":"tdm","delay-in-days":11811,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[1983,5]]},"abstract":"<jats:sec>\n                    <jats:title\/>\n                    <jats:p>Thirteen children, aged 10 months to 20 years, presenting with chronic cholestasis from the first month of life and with low serum levels of vitamins A and\/or E, have been investigated for neurological and ophthalmological symptoms. Clinical findings consisted of 4 types: peripheral neuropathy; cerebellar dysfunction; abnormalities of eye movement, and retinal degenerative changes. The results of electrophysiological and morphological studies of muscle and nerves were consistent with jieurono-axonal degeneration. Electrical abnormalities of the retina, especially a decrease of the b wave of electroretinogram, appear to be the first sign of the syndrome, allowing early detection. Evidence for vitamin deficiency (E or E+A) suggests substitutive parenteral treatment in such patients.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/hep.1840030321","type":"journal-article","created":{"date-parts":[[2010,7,5]],"date-time":"2010-07-05T09:07:01Z","timestamp":1278320821000},"page":"410-414","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":69,"title":["Nervous and Ocular Disorders in Children with Cholestasis and Vitamin A and E Deficiencies"],"prefix":"10.1097","volume":"3","author":[{"given":"Fernando","family":"Alvarez","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre","family":"Landrieu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Laget","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fr\u00e9d\u00e9rique","family":"Lemonnier","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michel","family":"Odi\u00e8vre","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Alagille","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"10.1002\/hep.1840030321-BIB1|cit1","first-page":"65","volume-title":"Textbook of pediatric nutrition","author":"Herman","year":"1981"},{"key":"10.1002\/hep.1840030321-BIB2|cit2","doi-asserted-by":"crossref","first-page":"579","DOI":"10.1136\/gut.12.7.579","article-title":"Absorption of vitamin E in children with biliary obstruction","volume":"12","author":"Harries","year":"1971","journal-title":"Gut"},{"key":"10.1002\/hep.1840030321-BIB3|cit3","doi-asserted-by":"crossref","first-page":"966","DOI":"10.1136\/gut.15.12.966","article-title":"The relative importance of the factors involved in the absorption of vitamin E in children","volume":"15","author":"Muller","year":"1974","journal-title":"Gut"},{"key":"10.1002\/hep.1840030321-BIB4|cit4","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1016\/S0300-9084(80)80058-7","article-title":"Esterification of cholesterol and lipid-soluble vitamins by human pancreatic carboxyl ester hydrolase","volume":"62","author":"Lombardo","year":"1980","journal-title":"Biochimie"},{"key":"10.1002\/hep.1840030321-BIB5|cit5","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1016\/S0022-2275(20)34754-4","article-title":"Optimization and validation of assays to estimate pancreatic esterase activity using well-characterized micellar solutions of cholesteryl oleate and toco-pheryl acetate","volume":"22","author":"Mathias","year":"1981","journal-title":"J Lipid Res"},{"key":"10.1002\/hep.1840030321-BIB6|cit6","doi-asserted-by":"crossref","first-page":"821","DOI":"10.1016\/0002-9343(64)90030-0","article-title":"Demyelinating central nervous system disease, macular atrophy and acanthocytosis (Bas-sen-Kornzweig syndrome)","volume":"37","author":"Sobrevilla","year":"1964","journal-title":"Am J Med"},{"key":"10.1002\/hep.1840030321-BIB7|cit7","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1136\/adc.52.3.209","article-title":"Long-term management of abeta-lipoproteinemia. 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