Night Blindness in Cystic Fibrosis: The Key Role of Vitamin A in the Digestive System
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 mala...
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doaj-aca59e21c93e4af9b15b059a2c86ece82020-11-25T01:15:38ZengMDPI AGNutrients2072-66432019-08-01118187610.3390/nu11081876nu11081876Night Blindness in Cystic Fibrosis: The Key Role of Vitamin A in the Digestive SystemLorenzo Norsa0Laura Zazzeron1Marialaura Cuomo2Laura Claut3Anna Marta Clotilde Bulfamante4Arianna Biffi5Carla Colombo6Pediatric Hepatology Gastroenterology and Transplantation, ASST Papa Giovanni XXIII, 24027 Bergamo, ItalyCystic Fibrosis Center, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, University of Milan, 20121 Milan, ItalyDepartment of Pediatrics, Fondazione MBBM Onlus/Ospedale San Gerardo, University of Milano-Bicocca, 20121 Milan, ItalyCystic Fibrosis Center, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, University of Milan, 20121 Milan, ItalyCystic Fibrosis Center, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, University of Milan, 20121 Milan, ItalyCystic Fibrosis Center, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, University of Milan, 20121 Milan, ItalyCystic Fibrosis Center, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, University of Milan, 20121 Milan, ItalyVitamin 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.https://www.mdpi.com/2072-6643/11/8/1876cystic fibrosisvitamin A deficiencyshort bowel syndrome |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Lorenzo Norsa Laura Zazzeron Marialaura Cuomo Laura Claut Anna Marta Clotilde Bulfamante Arianna Biffi Carla Colombo |
spellingShingle |
Lorenzo Norsa Laura Zazzeron Marialaura Cuomo Laura Claut Anna Marta Clotilde Bulfamante Arianna Biffi Carla Colombo Night Blindness in Cystic Fibrosis: The Key Role of Vitamin A in the Digestive System Nutrients cystic fibrosis vitamin A deficiency short bowel syndrome |
author_facet |
Lorenzo Norsa Laura Zazzeron Marialaura Cuomo Laura Claut Anna Marta Clotilde Bulfamante Arianna Biffi Carla Colombo |
author_sort |
Lorenzo Norsa |
title |
Night Blindness in Cystic Fibrosis: The Key Role of Vitamin A in the Digestive System |
title_short |
Night Blindness in Cystic Fibrosis: The Key Role of Vitamin A in the Digestive System |
title_full |
Night Blindness in Cystic Fibrosis: The Key Role of Vitamin A in the Digestive System |
title_fullStr |
Night Blindness in Cystic Fibrosis: The Key Role of Vitamin A in the Digestive System |
title_full_unstemmed |
Night Blindness in Cystic Fibrosis: The Key Role of Vitamin A in the Digestive System |
title_sort |
night blindness in cystic fibrosis: the key role of vitamin a in the digestive system |
publisher |
MDPI AG |
series |
Nutrients |
issn |
2072-6643 |
publishDate |
2019-08-01 |
description |
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. |
topic |
cystic fibrosis vitamin A deficiency short bowel syndrome |
url |
https://www.mdpi.com/2072-6643/11/8/1876 |
work_keys_str_mv |
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