Retinol binding protein status in relation to ocular surface changes in patients with cystic fibrosis treated with daily vitamin A supplements

• Published in: European journal of pediatrics Vol. 164; no. 4; pp. 202 - 206
• Main Authors: MRUGACZ, Malgorzata, TOBOLCZYK, Jolanta, MINAROWSKA, Alina
• Format: Journal Article
• Language: English
• Published: Heidelberg Springer 01.04.2005; Berlin Springer Nature B.V
• Subjects: Adolescent; Adult; Biological and medical sciences; Child; Cystic Fibrosis - blood; Cystic Fibrosis - drug therapy; Cystic Fibrosis - metabolism; Female; Gastroenterology. Liver. Pancreas. Abdomen; General aspects; Humans; Liver. Biliary tract. Portal circulation. Exocrine pancreas; Lung Volume Measurements; Male; Medical sciences; Nutritional Status; Other diseases. Semiology; Retinol-Binding Proteins - metabolism; Retinol-Binding Proteins, Plasma; Vitamin A - therapeutic use; Xerophthalmia - drug therapy; Xerophthalmia - metabolism; Human; Pediatrics; Respiratory disease; Vitamin; Metabolic diseases; Patient; Cystic fibrosis; Change; Genetic disease; Retinol; Surface; Eye; Visual system; Binding protein; Daily; Relation; Retinol binding protein; Treatment; Digestive diseases; Pancreatic disease; Food supplement
• ISSN: 0340-6199; 1432-1076
• DOI: 10.1007/s00431-004-1587-6

Cystic fibrosis (CF) is an autosomal recessive disease characterised by increased viscosity of mucus secretions and high chloride concentration in exocrine secretions. Clinically, the patients suffer from chronic pulmonary changes, chronic pancreatic deficiency, and an obstruction of the gastrointestinal tract. The disease affects all secretory epithelia including the eye. The influence of nutritional status on long-term survival and quality of life of CF patients is well documented. Steatorrhea, a consequence of decreased fat digestion and absorption may be associated with vitamin deficiences, including vitamin A. The aim of this study was to document plasma retinol binding protein (RBP) status, a specific plasma transport protein for vitamin A, and ocular surface changes in children and adolescents with CF. The patients were recruited at the 3rd Department of Paediatric Diseases, Medical University of Bialystok, Poland. All patients were regularly seen by a CF specialist dietitian. A group of 15 patients had the following investigations: plasma RBP, visual acuity, physical examination, tear film break-up time, fluorescein staining and Schirmer tear test. A group of 15 age- and sex-matched controls without CF or ocular pathology were also recruited. Plasma RBP concentrations were significantly lower in patients with CF than in the control group. CF patients showed a statistically significant increase in the incidence of clinical blepharitis. Five of the CF patients had clinical evidence of dry eyes. Low plasma retinol binding protein levels frequently occur in clinically stable and retinol supplemented cystic fibrosis patients, of whom five had dry eyes. We recommend monitoring of plasma retinol binding protein levels and evaluation of ocular surface changes, especially those with dry eye symptoms in all cystic fibrosis patients.