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Symptomatic vitamin E deficiency in cystic fibrosis
Archives of Disease in Childhood
|February 1, 1985
Insights
A girl with cystic fibrosis developed neurological issues due to vitamin E deficiency. Reduced bile salts worsened the deficiency, impacting neurological function.
Area of Science:
- Neurology
- Pediatrics
- Nutritional Science
Background:
- Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs, including the pancreas.
- Pancreatic insufficiency in CF often leads to malabsorption of fat-soluble vitamins, including Vitamin E.
- Vitamin E deficiency can cause progressive neurological impairment.
Observation:
- A 10-year-old girl with a history of cystic fibrosis presented with a neurological syndrome.
- Clinical presentation suggested severe vitamin E deficiency.
- Neurological symptoms manifested early despite management of pancreatic insufficiency.
Findings:
- The patient exhibited neurological deficits likely stemming from vitamin E deficiency.
- Reduced intraluminal bile salt concentrations were identified as a contributing factor.
- This exacerbation of deficiency was linked to both pancreatic insufficiency and impaired bile salt function.
Implications:
- Highlights the critical role of vitamin E in neurological health, especially in pediatric patients with malabsorptive conditions.
- Suggests that monitoring and managing bile salt concentrations may be crucial in preventing neurological complications in cystic fibrosis.
- Underscores the need for early and comprehensive nutritional assessment in children with cystic fibrosis to prevent severe vitamin E deficiency and its neurological sequelae.
Abstract:
A girl with cystic fibrosis who developed a neurological syndrome probably secondary to vitamin E deficiency at the age of 10 years is described. The severity of the deficiency and the early development of neurological features probably result from reduced intraluminal bile salt concentrations in addition to the pancreatic insufficiency.