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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Main Authors: Lorenzo Norsa, Laura Zazzeron, Marialaura Cuomo, Laura Claut, Anna Marta Clotilde Bulfamante, Arianna Biffi, Carla Colombo
Format: Article
Language:English
Published: MDPI AG 2019-08-01
Series:Nutrients
Subjects:
Online Access:https://www.mdpi.com/2072-6643/11/8/1876
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spelling 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
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