{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T05:00:22Z","timestamp":1787029222328,"version":"build-2736575974"},"reference-count":25,"publisher":"MDPI AG","issue":"8","license":[{"start":{"date-parts":[[2019,8,13]],"date-time":"2019-08-13T00:00:00Z","timestamp":1565654400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nutrients"],"abstract":"<jats:p>Vitamin A is a fundamental micronutrient that regulates various cellular patterns. Vitamin A deficiency (VAT) is a worldwide problem and the primary cause of nocturnal blindness especially in low income countries. Cystic fibrosis (CF) is a known risk factor of VAD because of liposoluble vitamin malabsorption due to pancreatic insufficiency. We describe a case of a 9-year-old girl who experienced recurrent episodes of nocturnal blindness due to profound VAD. This little girl is paradigmatic for the explanation of the key role of the gut\u2013liver axis in vitamin A metabolism. She presents with meconium ileus at birth, requiring intestinal resection that led to a transient intestinal failure with parenteral nutrition need. In addition, she suffered from cholestatic liver disease due to CF and intestinal failure-associated liver disease. The interaction of pancreatic function, intestinal absorption and liver storage is fundamental for the correct metabolism of vitamin A.<\/jats:p>","DOI":"10.3390\/nu11081876","type":"journal-article","created":{"date-parts":[[2019,8,13]],"date-time":"2019-08-13T04:31:21Z","timestamp":1565670681000},"page":"1876","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Night Blindness in Cystic Fibrosis: The Key Role of Vitamin A in the Digestive System"],"prefix":"10.3390","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3322-2921","authenticated-orcid":false,"given":"Lorenzo","family":"Norsa","sequence":"first","affiliation":[{"name":"Pediatric Hepatology Gastroenterology and Transplantation, ASST Papa Giovanni XXIII, 24027 Bergamo, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Zazzeron","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Center, Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico, University of Milan, 20121 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marialaura","family":"Cuomo","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Fondazione MBBM Onlus\/Ospedale San Gerardo, University of Milano-Bicocca, 20121 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Claut","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Center, Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico, University of Milan, 20121 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna Marta Clotilde","family":"Bulfamante","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Center, Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico, University of Milan, 20121 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arianna","family":"Biffi","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Center, Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico, University of Milan, 20121 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carla","family":"Colombo","sequence":"additional","affiliation":[{"name":"Cystic Fibrosis Center, Fondazione IRCCS Ca\u2019 Granda Ospedale Maggiore Policlinico, University of Milan, 20121 Milan, Italy"},{"name":"Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Universit\u00e0 degli Studi di Milano, 20121 Milan, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2019,8,13]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1002\/mnfr.201100230","article-title":"The contribution of \u03b2-carotene to vitamin A supply of humans","volume":"56","author":"Weber","year":"2012","journal-title":"Mol. Nutr. Food Res."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1007\/978-94-024-0945-1_4","article-title":"Vitamin A Absorption, Storage and Mobilization","volume":"81","author":"Blaner","year":"2016","journal-title":"Subcell. Biochem."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1007\/s12565-017-0395-9","article-title":"The stellate cell system (vitamin A-storing cell system)","volume":"92","author":"Senoo","year":"2017","journal-title":"Anat. Sci. Int."},{"key":"ref_4","doi-asserted-by":"crossref","unstructured":"Harrison, E.H. (2019). Mechanisms of Transport and Delivery of Vitamin A and Carotenoids to the Retinal Pigment Epithelium. Mol. Nutr. Food Res., e1801046.","DOI":"10.1002\/mnfr.201801046"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"358","DOI":"10.1080\/10408363.2017.1379945","article-title":"The Canadian laboratory initiative on pediatric reference intervals: A CALIPER white paper","volume":"54","author":"Adeli","year":"2017","journal-title":"Crit. Rev. Clin. Lab. Sci."},{"key":"ref_6","doi-asserted-by":"crossref","unstructured":"Song, P., Wang, J., Wei, W., Chang, X., Wang, M., and An, L. (2017). The Prevalence of Vitamin A Deficiency in Chinese Children: A Systematic Review and Bayesian Meta-Analysis. Nutrients, 9.","DOI":"10.3390\/nu9121285"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"699","DOI":"10.1111\/jpc.13243","article-title":"Vitamin A deficiency and xerophthalmia in children of a developed country","volume":"52","author":"Chiu","year":"2016","journal-title":"J. Paediatr. Child Health"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1016\/j.nut.2004.01.014","article-title":"Nightblindness, xerophthalmia, and severe loss of visual acuity due to unnecessary dietary restriction","volume":"20","author":"Fiore","year":"2004","journal-title":"Nutrition"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"531","DOI":"10.1016\/j.bpg.2005.11.006","article-title":"Cystic fibrosis: Nutritional consequences and management","volume":"20","author":"Dodge","year":"2006","journal-title":"Best Pract. Res. Clin. Gastroenterol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1151","DOI":"10.1136\/adc.64.8.1151","article-title":"Night blindness and conjunctival xerosis caused by vitamin A deficiency in patients with cystic fibrosis","volume":"64","author":"Rayner","year":"1989","journal-title":"Arch. Dis. Child."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"601","DOI":"10.1016\/S0022-3476(99)70059-4","article-title":"Prospective, long-term study of fat-soluble vitamin status in children with cystic fibrosis identified by newborn screen","volume":"135","author":"Feranchak","year":"1999","journal-title":"J. Pediatr."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"605","DOI":"10.1136\/jclinpath-2013-201787","article-title":"Fat-soluble vitamin deficiency in children and adolescents with cystic fibrosis","volume":"67","author":"Rana","year":"2014","journal-title":"J. Clin. Pathol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"654","DOI":"10.1016\/j.clnu.2015.04.010","article-title":"Vitamin A intake and serum retinol levels in children and adolescents with cystic fibrosis","volume":"35","author":"Woestenenk","year":"2016","journal-title":"Clin. Nutr."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"583","DOI":"10.1136\/bjo.2003.022459","article-title":"Rapid recovery of night blindness due to obesity surgery after vitamin A repletion therapy","volume":"88","author":"Spits","year":"2004","journal-title":"Br. J. Ophthalmol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"955","DOI":"10.1136\/bjo.2006.092502","article-title":"Vitamin A deficiency in patients with a remote history of intestinal surgery","volume":"90","author":"Chae","year":"2006","journal-title":"Br. J. Ophthalmol."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"400","DOI":"10.1016\/j.nut.2008.10.003","article-title":"Prospective analysis of serum carotenoids, vitamin A, and tocopherols in adults with short bowel syndrome undergoing intestinal rehabilitation","volume":"25","author":"Luo","year":"2009","journal-title":"Nutrition"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1177\/014860710002400281","article-title":"Vitamin A status modulates intestinal adaptation after partial small bowel resection","volume":"24","author":"Rubin","year":"2000","journal-title":"JPEN J. Parenter. Enteral Nutr."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"G1559","DOI":"10.1152\/ajpgi.00567.2006","article-title":"Chronically administered retinoic acid has trophic effects in the rat small intestine and promotes adaptation in a resection model of short bowel syndrome","volume":"292","author":"Wang","year":"2007","journal-title":"Am. J. Physiol. Gastrointest. Liver Physiol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"G424","DOI":"10.1152\/ajpgi.00524.2002","article-title":"Vitamin A deficiency inhibits intestinal adaptation by modulating apoptosis, proliferation, and enterocyte migration","volume":"285","author":"Wang","year":"2003","journal-title":"Am. J. Physiol. Gastrointest. Liver Physiol."},{"key":"ref_20","first-page":"82","article-title":"Vitamin A deficiency associated with enhanced proliferation of bile duct epithelial cells in the rat","volume":"12","author":"Weiss","year":"2010","journal-title":"Isr. Med. Assoc. J. IMAJ"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"443","DOI":"10.1097\/MPG.0000000000001676","article-title":"Cystic Fibrosis-related Liver Disease: Research Challenges and Future Perspectives","volume":"65","author":"Debray","year":"2017","journal-title":"J. Pediatr. Gastroenterol. Nutr."},{"key":"ref_22","doi-asserted-by":"crossref","unstructured":"Norsa, L., Nicastro, E., Di Giorgio, A., Lacaille, F., and D\u2019Antiga, L. (2018). Prevention and Treatment of Intestinal Failure-Associated Liver Disease in Children. Nutrients, 10.","DOI":"10.3390\/nu10060664"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1016\/j.foodchem.2014.09.021","article-title":"Fat-soluble vitamin intestinal absorption: Absorption sites in the intestine and interactions for absorption","volume":"172","author":"Goncalves","year":"2015","journal-title":"Food Chem."},{"key":"ref_24","doi-asserted-by":"crossref","unstructured":"Saeed, A., Dullaart, R.P.F., Schreuder, T.C.M.A., Blokzijl, H., and Faber, K.N. (2017). Disturbed Vitamin A Metabolism in Non-Alcoholic Fatty Liver Disease (NAFLD). Nutrients, 10.","DOI":"10.3390\/nu10010029"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"3703","DOI":"10.1080\/10408398.2016.1160362","article-title":"The vicious cycle of vitamin a deficiency: A review","volume":"57","author":"Wiseman","year":"2017","journal-title":"Crit. Rev. Food Sci. Nutr."}],"container-title":["Nutrients"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2072-6643\/11\/8\/1876\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T13:10:39Z","timestamp":1760188239000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2072-6643\/11\/8\/1876"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,8,13]]},"references-count":25,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2019,8]]}},"alternative-id":["nu11081876"],"URL":"https:\/\/doi.org\/10.3390\/nu11081876","relation":{},"ISSN":["2072-6643"],"issn-type":[{"value":"2072-6643","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,8,13]]}}}