title: Benign intracranial hypertension in an older child with cystic fibrosis.
abstract: Despite having normal height and weight, a 6-year-old girl had frequent bowel movements and slight recurrent chest infections since the age of 4 years and headache for 1 year. The patient appeared healthy, but examination of the ocular fundus revealed papilledema. Cranial computed tomography appeared normal. Lumbar puncture disclosed an elevated opening cerebrospinal fluid pressure, with normal biochemical, cellular, and bacteriologic findings. Laboratory investigations indicated pathologic steatorrhea, elevated electrolytes in 3 sweat tests, and low serum levels of vitamins A and E. The diagnosis of pseudotumor cerebri in a patient with cystic fibrosis was made. After treatment with prednisone (1 mg/kg/day), pancreatic extracts, and vitamin supplements, headache and papilledema resolved and serum vitamin A and E levels subsequently became normal. Older children with cystic fibrosis rarely have benign intracranial hypertension, but when present it is often due to hypervitaminosis during correction of malnutrition. In this child, pseudotumor cerebri and associated hypovitaminosis improved after combined corticosteroid and vitamin treatment.
authors.affiliation: Department of Gastroenterology, Hospital Bambino Gesù, Rome, Italy.
authors.forename: V
authors.initials: V
authors.lastname: Lucidi
authors.forename: M
authors.initials: M
authors.lastname: Di Capua
authors.forename: P
authors.initials: P
authors.lastname: Rosati
authors.forename: B
authors.initials: B
authors.lastname: Papadatou
authors.forename: M
authors.initials: M
authors.lastname: Castro
mesh_terms: D002648:Child; D003550:Cystic Fibrosis / Q000150:complications* / Q000188:drug therapy; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D009460:Neurologic Examination / Q000187:drug effects; D010184:Pancreatic Extracts / Q000008:administration & dosage; D011241:Prednisone / Q000008:administration & dosage; D011559:Pseudotumor Cerebri / Q000188:drug therapy / Q000209:etiology*; D014802:Vitamin A Deficiency / Q000150:complications / Q000188:drug therapy; D014811:Vitamin E Deficiency / Q000150:complications / Q000188:drug therapy; D014815:Vitamins / Q000008:administration & dosage
chemical_list: D010184:Pancreatic Extracts; D014815:Vitamins; D011241:Prednisone