EFFECT OF ORTHOTOPIC LIVER-TRANSPLANTATION ON BONE-MINERAL CONTENT AND SERUM VITAMIN-D METABOLITES IN INFANTS AND CHILDREN WITH CHRONIC CHOLESTASIS

Almost all infants and children with chronic cholestasis have osteopen ia. We evaluated the effect of orthotopic liver transplantation on bon e mineral content and serum 25(OH)-vitamin D-[25(OH)D]-of nine infants and children (five girls; age, 6 to 21 mo at the time of orthotopic l iver transplantation) with end-stage liver disease resulting from chro nic cholestasis. We hypothesized that after orthotopic liver transplan tation, decreased bone mineral content will recover and the serum 25(O H)D level will either normalize or remain normal in those who were pre viously vitamin D deficient or sufficient, respectively. All had subno rmal bone mineral content before transplant. On long-term follow-up (> 4 mo) of seven patients, bone mineral content normalized in all betwee n 6.5 and 19 mo after transplant, with a mean of 11.2 +/- 4.5 mo. In s ix patients with normal serum 25(OH)D levels before orthotopic liver t ransplantation, the serum 25(OH)D levels had declined markedly 1 to 2 mo after transplant, followed by return to normal by 3 to 6 mo. Low se rum 25(OH)D levels (<15 ng/ml) in three patients before orthotopic liv er transplantation normalized after transplant. Although there was no correlation between bone mineral content and serum 25(OH)D level befor e transplant, sustained normal serum 25(OH)D and 1,25(OH),D levels pre ceded or accompanied normalization of bone mineral content in the seve n patients available for long-term follow-up. We conclude that (a) in infants and children younger than 2 yr with chronic cholestasis, bone mineral content normalizes approximately 11 mo after orthotopic liver transplantation. This normalization is preceded by a sustained period of normal serum 25(OH)D levels. (b) Normal baseline serum 25(OH)D leve ls decrease 1 to 2 mo after orthotopic liver transplantation, with sub sequent normalization, whereas initially low serum 25(OH)D levels grad ually normalize within 2 to 4 mo. We speculate that maintenance of nor mal serum 25(OH)D levels with oral vitamin D supplementation may enhan ce recovery of bone disease after orthotopic liver transplantation.