Bulging fontanelle as presenting sign in cystic fibrosis. Vitamin A metabolism and effect on cerebrospinal fluid pressure.

A 51/2-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 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. 