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