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Bulging Fontanelle as Presenting Sign in Cystic Fibrosis: Vitamin A Metabolism and Effect on Cerebrospinal Fluid Pressure

Bulging Fontanelle as Presenting Sign in Cystic Fibrosis: Vitamin A Metabolism and Effect on... Abstract • A 5½-month-old infant had the single problem of a bulging fontanelle. A diagnosis of cystic fibrosis with secondary hypovitaminosis A was made by the findings of high sweat chloride values and a low serum carotene level. A greatly accelerated rate of weight gain following the addition of pancreatic enzyme supplements confirmed the presence of malabsorption. The infant developed characteristic cystic fibrosis pulmonary disease at 20 months of age. Animal studies have shown vitamin A deficiency to be associated with increased cerebrospinal fluid (CSF) pressure, diminished absorption of CSF, and pathological findings of thickening and infiltration with mucopolysaccharides of the dura mater around the arachnoid villi. (Am J Dis Child 130:1360-1362, 1976) References 1. Keating JP, Feigin RD: Increased intracranial pressure associated with probable vitamin A deficiency in cystic fibrosis . Pediatrics 46:41-46, 1970. 2. Cornfeld D, Cooke RE: Vitamin A deficiency: Case report: Unusual manifestations in a 5½ month old baby . Pediatrics 10:33-39, 1952. 3. Bass MH, Caplan J: Vitamin A deficiency in infancy . J Pediatr 47:690-695, 1955.Crossref 4. Buchheit WA, Burton C, Haag B, et al: Papilledema and idiopathic intracranial hypertension: Report of a familial occurrence . N Engl J Med 280:938-942, 1969.Crossref 5. Moore LA, Sykes JF: Cerebrospinal fluid pressure and vitamin A deficiency . Am J Physiol 130:684-689, 1940. 6. Millen JW, Woolam DHM, Lamming GE: Congenital hydrocephalus due to experimental hypovitaminosis A . Lancet 2:679-683, 1954.Crossref 7. Eaton HD: Chronic bovine hypo- and hypervitaminosis A and cerebrospinal fluid pressure . Am J Clin Nutr 22:1070-1080, 1969. 8. Calhoun MC, Hurt HD, Eaton HD, et al: Rates of formation and absorption of cerebrospinal fluid in bovine hypovitaminosis A . J Dairy Sci 50:1486-1494, 1967.Crossref 9. Hayes KC, Nielsen SW, Eaton HD: Pathogenesis of the optic nerve lesion in vitamin A-deficient calves . Arch Ophthalmol 80:777-787, 1968.Crossref 10. Cousins RJ, Eaton HD, Rousseau JE Jr, et al: Biochemical constituents of the dura mater in vitamin A deficiency . J Nutr 97:409-418, 1969. 11. Danes BS, Bearn AG: Hurler's syndrome: Effect of retinol (vitamin A alcohol) on cellular mucopolysaccharides in cultured human skin fibroblasts . J Exp Med 124:1181-1198, 1966.Crossref 12. Hurt HD, Eaton HD, Rousseau JE Jr, et al: Rates of formation and absorption of cerebrospinal fluid in chronic bovine hypervitaminosis A . J Dairy Sci 50:1941-1947, 1967.Crossref 13. Maddux GW, Foltz FM, Nelson SR: Effect of vitamin A intoxication on intracranial pressure and brain water in rats . J Nutr 104:478-482, 1974. 14. Gorgacz EJ, Rousseau JE Jr, Frier HI, et al: Composition of the dura mater in chronic bovine hypervitaminosis A . J Nutr 101:1541-1546, 1971. 15. Roels OA: Vitamin A physiology . JAMA 214:1097-1102, 1970.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Bulging Fontanelle as Presenting Sign in Cystic Fibrosis: Vitamin A Metabolism and Effect on Cerebrospinal Fluid Pressure

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Publisher
American Medical Association
Copyright
Copyright © 1976 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1976.02120130066013
Publisher site
See Article on Publisher Site

Abstract

Abstract • A 5½-month-old infant had the single problem of a bulging fontanelle. A diagnosis of cystic fibrosis with secondary hypovitaminosis A was made by the findings of high sweat chloride values and a low serum carotene level. A greatly accelerated rate of weight gain following the addition of pancreatic enzyme supplements confirmed the presence of malabsorption. The infant developed characteristic cystic fibrosis pulmonary disease at 20 months of age. Animal studies have shown vitamin A deficiency to be associated with increased cerebrospinal fluid (CSF) pressure, diminished absorption of CSF, and pathological findings of thickening and infiltration with mucopolysaccharides of the dura mater around the arachnoid villi. (Am J Dis Child 130:1360-1362, 1976) References 1. Keating JP, Feigin RD: Increased intracranial pressure associated with probable vitamin A deficiency in cystic fibrosis . Pediatrics 46:41-46, 1970. 2. Cornfeld D, Cooke RE: Vitamin A deficiency: Case report: Unusual manifestations in a 5½ month old baby . Pediatrics 10:33-39, 1952. 3. Bass MH, Caplan J: Vitamin A deficiency in infancy . J Pediatr 47:690-695, 1955.Crossref 4. Buchheit WA, Burton C, Haag B, et al: Papilledema and idiopathic intracranial hypertension: Report of a familial occurrence . N Engl J Med 280:938-942, 1969.Crossref 5. Moore LA, Sykes JF: Cerebrospinal fluid pressure and vitamin A deficiency . Am J Physiol 130:684-689, 1940. 6. Millen JW, Woolam DHM, Lamming GE: Congenital hydrocephalus due to experimental hypovitaminosis A . Lancet 2:679-683, 1954.Crossref 7. Eaton HD: Chronic bovine hypo- and hypervitaminosis A and cerebrospinal fluid pressure . Am J Clin Nutr 22:1070-1080, 1969. 8. Calhoun MC, Hurt HD, Eaton HD, et al: Rates of formation and absorption of cerebrospinal fluid in bovine hypovitaminosis A . J Dairy Sci 50:1486-1494, 1967.Crossref 9. Hayes KC, Nielsen SW, Eaton HD: Pathogenesis of the optic nerve lesion in vitamin A-deficient calves . Arch Ophthalmol 80:777-787, 1968.Crossref 10. Cousins RJ, Eaton HD, Rousseau JE Jr, et al: Biochemical constituents of the dura mater in vitamin A deficiency . J Nutr 97:409-418, 1969. 11. Danes BS, Bearn AG: Hurler's syndrome: Effect of retinol (vitamin A alcohol) on cellular mucopolysaccharides in cultured human skin fibroblasts . J Exp Med 124:1181-1198, 1966.Crossref 12. Hurt HD, Eaton HD, Rousseau JE Jr, et al: Rates of formation and absorption of cerebrospinal fluid in chronic bovine hypervitaminosis A . J Dairy Sci 50:1941-1947, 1967.Crossref 13. Maddux GW, Foltz FM, Nelson SR: Effect of vitamin A intoxication on intracranial pressure and brain water in rats . J Nutr 104:478-482, 1974. 14. Gorgacz EJ, Rousseau JE Jr, Frier HI, et al: Composition of the dura mater in chronic bovine hypervitaminosis A . J Nutr 101:1541-1546, 1971. 15. Roels OA: Vitamin A physiology . JAMA 214:1097-1102, 1970.Crossref

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: Dec 1, 1976

References