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Fanconi syndrome following bowel surgery and hepatitis reversed by 25-hydroxycholecalciferol
Insights
This study details a premature infant with Fanconi syndrome, osteoporosis, and hepatitis. 25-hydroxycholecalciferol effectively treated these conditions, unlike vitamin D2, suggesting impaired liver metabolism.
Area of Science:
- Pediatric Endocrinology
- Gastroenterology
- Nephrology
Background:
- Premature infant developed complications including hepatitis, osteoporosis, and Fanconi syndrome after extensive surgery for ileal and cecal necrosis.
- Elevated parathyroid hormone and impaired phosphate reabsorption indicated severe metabolic derangement.
Abstract:
A permature male infant required intravenous alimentation for six weeks following extensive surgery for ileal and cecal necrosis. At 3 months he developed evidence of hepatitis. Subsequently osteoporosis and the Fanconi syndrome appeared. Urine phosphate clearance was 83 percent of creatinine clearance at a serum phosphate concentration of 1.6 mg/dl. Concentration of plasma immunoreactive parathyroid hormone was elevated at 550 pg/ml. 25-Hydroxycholecalciferol was given at 240 mug/day. Aminoaciduria disappeared and bone healing occurred. Serum phosphate rose to 6.5 mg/dl and phosphate clearance fell to 2 percent of creatinine clearance. Upon cessation of 25-OHCC therapy, the Fanconi syndrome recurred despite administration of vitamin D2. 25-OHCC was then administered at 40 mug/day, and the urine abnormalities were reversed. The patient probably developed hyperparathyroidism, secondary malabsorption, and hepatitis. The Fanconi syndrome was the consequence of the hyperparathyroidism. 25-OHCC therapy was more effective than vitamin D in reversing the disordered state, possibly because of impaired hepatic metabolism of vitamin D2.