Abstract
A 25-yr-old black man with cystic fibrosis and cirrhosis developed symptoms of osteomalacia and hypocalcemia, hypophosphatemia, secondary hyperparathyroidism, and low circulating 25-hydroxyvitamin D (25-OHD). Serum 1,25-dihydroxyvitamin D (1,25-[OH]2D) was within the normal range. Iliac crest bone biopsy confirmed the diagnosis of osteomalacia. Oral administration of 50,000 IU of vitamin D2 failed to relieve symptoms or raise serum 25-OHD levels to normal. Intramuscular vitamin D2, 10,000 IU every 8-12 week, improved symptoms, raised serum 25-OHD to normal, and increased circulating 1,25-[OH]2D to values five times normal. Over the next 10 mo circulating 1,25-[OH]2D remained elevated despite normalization of serum calcium, phosphorus, and parathyroid hormone. Repeat bone biopsy 1 yr after parenteral vitamin D showed healing of the osteomalacia. Malabsorption of vitamin D appears secondary to profound steatorrhea due to pancreatic insufficiency and secondary biliary cirrhosis. Although extensive hepatocellular disease was present, hepatic conversion of vitamin D to 25-OHD was intact. Both high and low circulating 1,25-[OH]2D levels during active osteomalacia have been reported; initially, the level was in the normal range and higher values in this patient occurred with repletion of 25-OHD substrate. This study shows that symptomatic osteomalacia may be a major manifestation of cystic fibrosis in those patients surviving into adulthood. Measurements of serum 25-OHD in cystic fibrosis patients may identify those who should receive supplemental vitamin D.
Original language | English (US) |
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Pages (from-to) | 808-813 |
Number of pages | 6 |
Journal | Gastroenterology |
Volume | 88 |
Issue number | 3 |
DOIs | |
State | Published - Mar 1985 |
Funding
Received January 25, 1984. Accepted September 25, 1984. Address requests for reprints to: Craig B. Langman, M.D., Department of Pediatrics, Michael Reese Hospital and Medical Center, 31st at Lake Shore Drive, Chicago, Illinois 60616. This work was supported in part by grants from the National Institutes of Health (AM 20585) and the Michael Reese Research Institute. The authors thank Vrshali Tembe and Peggy Imanaka for expert technical assistance. 0 1985 by the American Gastroenterological Association 0016-5085/85/$3,30
ASJC Scopus subject areas
- Gastroenterology
- Hepatology