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Night Blindness in Cystic Fibrosis: The Key Role of Vitamin A in the Digestive System

Pediatric Hepatology Gastroenterology and Transplantation, ASST Papa Giovanni XXIII, 24027 Bergamo, Italy
Cystic Fibrosis Center, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, University of Milan, 20121 Milan, Italy
Department of Pediatrics, Fondazione MBBM Onlus/Ospedale San Gerardo, University of Milano-Bicocca, 20121 Milan, Italy
Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti, Università degli Studi di Milano, 20121 Milan, Italy
Author to whom correspondence should be addressed.
The authors equally contributed to the paper.
Nutrients 2019, 11(8), 1876;
Received: 29 June 2019 / Revised: 6 August 2019 / Accepted: 8 August 2019 / Published: 13 August 2019
(This article belongs to the Special Issue Vitamin A and Human Health)
PDF [1567 KB, uploaded 13 August 2019]


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–liver 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. View Full-Text
Keywords: cystic fibrosis; vitamin A deficiency; short bowel syndrome cystic fibrosis; vitamin A deficiency; short bowel syndrome

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Norsa, L.; Zazzeron, L.; Cuomo, M.; Claut, L.; Bulfamante, A.M.C.; Biffi, A.; Colombo, C. Night Blindness in Cystic Fibrosis: The Key Role of Vitamin A in the Digestive System. Nutrients 2019, 11, 1876.

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