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Published in: European Journal of Pediatrics 4/2005

01-04-2005 | Original Paper

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

Authors: Malgorzata Mrugacz, Jolanta Tobolczyk, Alina Minarowska

Published in: European Journal of Pediatrics | Issue 4/2005

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Abstract

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. Conclusion: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.
Literature
1.
2.
go back to reference Ansari EA, Sahni K, Etherington CH, Morton A (1999) Ocular signs and vitamin A status in patients with cystic fibrosis treated with daily vitamin A supplements. Br J Ophthalmol 83: 688–691PubMed Ansari EA, Sahni K, Etherington CH, Morton A (1999) Ocular signs and vitamin A status in patients with cystic fibrosis treated with daily vitamin A supplements. Br J Ophthalmol 83: 688–691PubMed
3.
go back to reference Biesalski HK, Frank J, Beck SC, Heinrich F (1999) Biochemical but not clinical vitamin A deficiency results from mutations in the gene for retinol binding protein. Am J Clin Nutr 69: 931–936PubMed Biesalski HK, Frank J, Beck SC, Heinrich F (1999) Biochemical but not clinical vitamin A deficiency results from mutations in the gene for retinol binding protein. Am J Clin Nutr 69: 931–936PubMed
4.
go back to reference Blaner WS (1989) Retinol-binding protein: the serum transport protein for vitamin A. Endocr Rev 10: 308–316PubMed Blaner WS (1989) Retinol-binding protein: the serum transport protein for vitamin A. Endocr Rev 10: 308–316PubMed
5.
go back to reference Botelho SY, Goldstein AM, Rosenlund ML (1973) Tear sodium, potassium, chloride, and calcium at various flow rates: children with cystic fibrosis and unaffected siblings with and without corneal staining. J Pediatr 83: 601–606PubMed Botelho SY, Goldstein AM, Rosenlund ML (1973) Tear sodium, potassium, chloride, and calcium at various flow rates: children with cystic fibrosis and unaffected siblings with and without corneal staining. J Pediatr 83: 601–606PubMed
6.
go back to reference Bronstein MN, Sokol RJ, Abman SH (1992) Pancreatic insufficiency, growth, and nutrition in infants identified by newborn screening as having cystic fibrosis. J Pediatr 120: 533–540PubMed Bronstein MN, Sokol RJ, Abman SH (1992) Pancreatic insufficiency, growth, and nutrition in infants identified by newborn screening as having cystic fibrosis. J Pediatr 120: 533–540PubMed
7.
go back to reference Chase HP, Long M, Lavin MH (1979) Cystic fibrosis and malnutrition. J Pediatr 95: 337–347PubMed Chase HP, Long M, Lavin MH (1979) Cystic fibrosis and malnutrition. J Pediatr 95: 337–347PubMed
8.
go back to reference Dartt DA (2002) Regulation of mucin and fluid secretion by conjunctival epithelial cells. Prog Retin Eye Res 21: 555–576CrossRefPubMed Dartt DA (2002) Regulation of mucin and fluid secretion by conjunctival epithelial cells. Prog Retin Eye Res 21: 555–576CrossRefPubMed
9.
go back to reference Di Sant’Agnese PA, Davis PB (1979) Cystic fibrosis in adults: 75 cases and a review of 232 cases in the literature. Am J Med 66: 121–32CrossRefPubMed Di Sant’Agnese PA, Davis PB (1979) Cystic fibrosis in adults: 75 cases and a review of 232 cases in the literature. Am J Med 66: 121–32CrossRefPubMed
10.
go back to reference D’Onofrio C, Colantuoni V, Cortese R (1985) Structure and cell-specific expression of a cloned human retinol-binding protein gene: the 5’-flanking region contains hepatoma specific transcriptional signals. EMBO J 4: 1981–1989PubMed D’Onofrio C, Colantuoni V, Cortese R (1985) Structure and cell-specific expression of a cloned human retinol-binding protein gene: the 5’-flanking region contains hepatoma specific transcriptional signals. EMBO J 4: 1981–1989PubMed
11.
go back to reference Eid NS, Shoemaker LR, Samiec TD (1990) Vitamin A in cystic fibrosis: case report and review of the literature. J Pediatr Gastroenterol Nutr 10: 265–269PubMed Eid NS, Shoemaker LR, Samiec TD (1990) Vitamin A in cystic fibrosis: case report and review of the literature. J Pediatr Gastroenterol Nutr 10: 265–269PubMed
12.
go back to reference Fiore P, De Marco R, Sacco O, Priolo E (2004) Night blindness, xerophthalmia, and severe loss of visual acuity due to unnecessary dietary restriction. J Nutr 20: 477CrossRef Fiore P, De Marco R, Sacco O, Priolo E (2004) Night blindness, xerophthalmia, and severe loss of visual acuity due to unnecessary dietary restriction. J Nutr 20: 477CrossRef
13.
go back to reference Fujikawa A, Gong H, Amemiya T (2003) Vitamin E prevents changes in the cornea and conjunctiva due to vitamin A deficiency. Graefes Arch Clin Exp Ophthalmol 241: 287–297PubMed Fujikawa A, Gong H, Amemiya T (2003) Vitamin E prevents changes in the cornea and conjunctiva due to vitamin A deficiency. Graefes Arch Clin Exp Ophthalmol 241: 287–297PubMed
14.
go back to reference Greer RM, Buntain HM, Lewindon PJ, Wainwright CE, Potter JM, Wong JC, Francis PW, Batch JA, Bell SC (2004) Vitamin A levels in patients with CF are influenced by the inflammatory response. J Cyst Fibrosis 3: 143–149CrossRef Greer RM, Buntain HM, Lewindon PJ, Wainwright CE, Potter JM, Wong JC, Francis PW, Batch JA, Bell SC (2004) Vitamin A levels in patients with CF are influenced by the inflammatory response. J Cyst Fibrosis 3: 143–149CrossRef
15.
go back to reference Hasko G, Deitch EA, Nemeth ZH, Kuhel DG (2002) Inhibitors of ATP-binding cassette transporters suppress interleukin-12 p40 production and major histocompatibility complex II up-regulation in macrophages. J Pharmacol Exp Ther 301: 103–110CrossRefPubMed Hasko G, Deitch EA, Nemeth ZH, Kuhel DG (2002) Inhibitors of ATP-binding cassette transporters suppress interleukin-12 p40 production and major histocompatibility complex II up-regulation in macrophages. J Pharmacol Exp Ther 301: 103–110CrossRefPubMed
17.
go back to reference Huet F, Semams D, Maingueneau C, Charavel A, Nivelon J-L (1997) Vitamin A deficiency and nocturnal vision in teenagers with cystic fibrosis. Eur J Pediatr 156: 949–951CrossRefPubMed Huet F, Semams D, Maingueneau C, Charavel A, Nivelon J-L (1997) Vitamin A deficiency and nocturnal vision in teenagers with cystic fibrosis. Eur J Pediatr 156: 949–951CrossRefPubMed
18.
go back to reference Junko H (1998) AD classification of fluorescein staining. In: Tano Y, Watanabe N (eds) Practical ophthalmology. Bunkodo Publishers, Tokyo pp 61 Junko H (1998) AD classification of fluorescein staining. In: Tano Y, Watanabe N (eds) Practical ophthalmology. Bunkodo Publishers, Tokyo pp 61
19.
go back to reference Kalayci D, Kiper N, Ozcelik U, Gocmen A, Hasiripi H (1996) Clinical status, ocular surface changes and tear ferning in patients with cystic fibrosis. Acta Ophthalmol Scand 74: 563–565PubMed Kalayci D, Kiper N, Ozcelik U, Gocmen A, Hasiripi H (1996) Clinical status, ocular surface changes and tear ferning in patients with cystic fibrosis. Acta Ophthalmol Scand 74: 563–565PubMed
20.
go back to reference Kasarskis EJ, Bass NH (1982) Benign intracranial hypertension induced by deficiency of vitamin A during infancy. Neurology 32: 1292–1295PubMed Kasarskis EJ, Bass NH (1982) Benign intracranial hypertension induced by deficiency of vitamin A during infancy. Neurology 32: 1292–1295PubMed
21.
go back to reference Knorre KS, Wagner M, Schaefer HE, Colledge WH, Pahl HL (2002) Delta F508-CFTR causes constitutive NF-kappa B activation through an ER-overload response in cystic fibrosis lungs. J Biol Chem 383: 2710–2712 Knorre KS, Wagner M, Schaefer HE, Colledge WH, Pahl HL (2002) Delta F508-CFTR causes constitutive NF-kappa B activation through an ER-overload response in cystic fibrosis lungs. J Biol Chem 383: 2710–2712
22.
go back to reference Lobeck CC (1972) Cystic fibrosis. In: Standbury JB, Wyngaarden JB, Fredrickson DS (eds) The metabolic basis of inherited Disease, 3rd edn. McGraw-Hill, New York, pp 1605–1626 Lobeck CC (1972) Cystic fibrosis. In: Standbury JB, Wyngaarden JB, Fredrickson DS (eds) The metabolic basis of inherited Disease, 3rd edn. McGraw-Hill, New York, pp 1605–1626
23.
go back to reference Manthorpe R, Oxholm P, Prause JU, Schiodt M (1986) The Copenhagen criteria of Sjogren’s syndrome. Scand J Rheumatol [Suppl] 61: 19–21 Manthorpe R, Oxholm P, Prause JU, Schiodt M (1986) The Copenhagen criteria of Sjogren’s syndrome. Scand J Rheumatol [Suppl] 61: 19–21
24.
go back to reference Morkeberg JC, Edmund C, Prause JU, Lanng S (1995) Ocular findings in cystic fibrosis patients receiving vitamin A supplementation. Graefes Arch Clin Exp Ophthalmol 233: 709–713CrossRefPubMed Morkeberg JC, Edmund C, Prause JU, Lanng S (1995) Ocular findings in cystic fibrosis patients receiving vitamin A supplementation. Graefes Arch Clin Exp Ophthalmol 233: 709–713CrossRefPubMed
25.
go back to reference Muto Y, Smith JE, Milch PO, Goodman DS (1979) Regulation of retinol-binding protein metabolism by vitamin A status in the rat. J Biol Chem 247: 2542–2550 Muto Y, Smith JE, Milch PO, Goodman DS (1979) Regulation of retinol-binding protein metabolism by vitamin A status in the rat. J Biol Chem 247: 2542–2550
26.
go back to reference Ocendy D, McMorran BJ, Smith SN, Schreiber R (2002) Gene complementation of airway epithelium in the cystic fibrosis mouse in necessary and sufficient to correct the pathogen clearance and inflammatory abnormalities. Hum Mol Genet 11: 1059–1067CrossRefPubMed Ocendy D, McMorran BJ, Smith SN, Schreiber R (2002) Gene complementation of airway epithelium in the cystic fibrosis mouse in necessary and sufficient to correct the pathogen clearance and inflammatory abnormalities. Hum Mol Genet 11: 1059–1067CrossRefPubMed
27.
go back to reference Olson JA (1978) Evaluation of vitamin A status in children. Nutr Diet 31: 130–134 Olson JA (1978) Evaluation of vitamin A status in children. Nutr Diet 31: 130–134
28.
go back to reference Quadro L, Blaner WS, Hamberger L, Van Gelder N (2002) Muscle expression of human retinol-binding protein (RBP). J Biol Chem 277: 30191–30197CrossRefPubMed Quadro L, Blaner WS, Hamberger L, Van Gelder N (2002) Muscle expression of human retinol-binding protein (RBP). J Biol Chem 277: 30191–30197CrossRefPubMed
29.
go back to reference Rayner RJ, Tyrrell JC, Hiller EJ (1989) Night blindness and conjunctival xerosis caused by vitamin A deficiency in patients with cystic fibrosis. Arch Dis Child 64: 1151–1156PubMed Rayner RJ, Tyrrell JC, Hiller EJ (1989) Night blindness and conjunctival xerosis caused by vitamin A deficiency in patients with cystic fibrosis. Arch Dis Child 64: 1151–1156PubMed
30.
go back to reference Riodan JR, Rommens JM, Kerem B, Alon N, Rozmahel R, Grzelczak Z, Zielenski J, Lok S, Plavsic N, Chou (1989) Identification of the cystic fibrosis gene: cloning and characterisation of complementary DNA. Science 245: 1066–1073PubMed Riodan JR, Rommens JM, Kerem B, Alon N, Rozmahel R, Grzelczak Z, Zielenski J, Lok S, Plavsic N, Chou (1989) Identification of the cystic fibrosis gene: cloning and characterisation of complementary DNA. Science 245: 1066–1073PubMed
31.
go back to reference Rosales FJ, Ritter SJ, Zolfaghari R (1996) Effects of acute inflammation on plasma retinol, retinol-binding-protein, and its messenger RNA in the liver and kidneys of vitamin A sufficient rats. J Lipid Res 37: 962–971PubMed Rosales FJ, Ritter SJ, Zolfaghari R (1996) Effects of acute inflammation on plasma retinol, retinol-binding-protein, and its messenger RNA in the liver and kidneys of vitamin A sufficient rats. J Lipid Res 37: 962–971PubMed
32.
go back to reference Sade de Paiva C, Pflugfelder SC (2004) Corneal epitheliopathy of dry eye induces hyperesthesia to mechanical air jet stimulation. Am J Ophthalmol 137: 109–115CrossRefPubMed Sade de Paiva C, Pflugfelder SC (2004) Corneal epitheliopathy of dry eye induces hyperesthesia to mechanical air jet stimulation. Am J Ophthalmol 137: 109–115CrossRefPubMed
33.
go back to reference Schemann J, Banou A, Malvy D, Guindo A, Traore L, Momo G (2003) National immunisation days and vitamin A distribution in Mali: has the vitamin A status of pre-school children improved? Public Health Nutr 6: 233–244CrossRefPubMed Schemann J, Banou A, Malvy D, Guindo A, Traore L, Momo G (2003) National immunisation days and vitamin A distribution in Mali: has the vitamin A status of pre-school children improved? Public Health Nutr 6: 233–244CrossRefPubMed
34.
go back to reference Sheppard JD, Orenstein DM, Chao C-C, Butala S, RP Kowalski (1989) The ocular surface in cystic fibrosis. Ophthalmology 96: 1624–1630PubMed Sheppard JD, Orenstein DM, Chao C-C, Butala S, RP Kowalski (1989) The ocular surface in cystic fibrosis. Ophthalmology 96: 1624–1630PubMed
35.
go back to reference Smith FR, Underwood BA, Denning CR (1972) Depressed plasma retinol-binding protein levels in cystic fibrosis. J Lab Clin Med 80: 423–433PubMed Smith FR, Underwood BA, Denning CR (1972) Depressed plasma retinol-binding protein levels in cystic fibrosis. J Lab Clin Med 80: 423–433PubMed
36.
go back to reference Soprano DR, Smith JE, Goodman DS (1982) Effect of retinol status on retinol-binding protein biosynthesis rate and translatable messenger RNA level in rat liver. J Biol Chem 257: 7693–7697PubMed Soprano DR, Smith JE, Goodman DS (1982) Effect of retinol status on retinol-binding protein biosynthesis rate and translatable messenger RNA level in rat liver. J Biol Chem 257: 7693–7697PubMed
37.
go back to reference Thurnham DI, Northrop-Clewes CA (1999) Optimal nutrition: vitamin A and the carotenoids. Proc Nutr Soc 58: 449–457PubMed Thurnham DI, Northrop-Clewes CA (1999) Optimal nutrition: vitamin A and the carotenoids. Proc Nutr Soc 58: 449–457PubMed
38.
go back to reference Yoshimura K, Chu CS, Crystal RG (1993) Alternative splicing of intron 23 of the human cystic fibrosis transmembrane conductance regulator gene resulting in a novel exon and transcript coding for a shortened intracytoplasmic C terminus. J Biol Chem 268: 686–690PubMed Yoshimura K, Chu CS, Crystal RG (1993) Alternative splicing of intron 23 of the human cystic fibrosis transmembrane conductance regulator gene resulting in a novel exon and transcript coding for a shortened intracytoplasmic C terminus. J Biol Chem 268: 686–690PubMed
39.
go back to reference Watson NJ, Hutchinson CH, Atta HR (1995) Vitamin A deficiency and xerophthalmia in the United Kingdom. BMJ 310: 1050–1051PubMed Watson NJ, Hutchinson CH, Atta HR (1995) Vitamin A deficiency and xerophthalmia in the United Kingdom. BMJ 310: 1050–1051PubMed
40.
go back to reference Wood LG, Fitzgerald DA, Lee AK, Garg ML (2003) Improved antioxidant and fatty acid status of patients with cystic fibrosis after antioxidant supplementation is linked to improved lung function. Am J Clin Nutr 77: 150–159PubMed Wood LG, Fitzgerald DA, Lee AK, Garg ML (2003) Improved antioxidant and fatty acid status of patients with cystic fibrosis after antioxidant supplementation is linked to improved lung function. Am J Clin Nutr 77: 150–159PubMed
Metadata
Title
Retinol binding protein status in relation to ocular surface changes in patients with cystic fibrosis treated with daily vitamin A supplements
Authors
Malgorzata Mrugacz
Jolanta Tobolczyk
Alina Minarowska
Publication date
01-04-2005
Publisher
Springer-Verlag
Published in
European Journal of Pediatrics / Issue 4/2005
Print ISSN: 0340-6199
Electronic ISSN: 1432-1076
DOI
https://doi.org/10.1007/s00431-004-1587-6

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