with a prefactor VIII infusion activity of 25% and a 2 h postinfusion activity of 71% after receiving 50 u kg )1 . The Bethesda titre for the inhibitor was 0.9 units at this time. The most recent factor activity has dropped to 19% at 9 months. The patient has not had any bleeds post-transplantation, however. Conclusions: 1. An inhibitor patient on long-term immune tolerance underwent successful orthotopic liver transplantation with initial normalization of factor VIII activity. 2. Beginning 2 months after OLT and continuing to the present, factor VIII activity gradually decreased associated with detection of a low titre recurrent factor VIII inhibitor. Synthesis of factor VIII by the donor liver is not sufficient for total suppression of the long-standing inhibitor.Background: Recent studies have shown that children with severe haemophilia could have moderately reduced bone density compared with gender-and age-matched control subjects. Patients with more established changes resulting from haemophilic joint disease exhibited the lowest bone mineral density. The reason for this includes factors such as lack of weight-bearing physical activity, lower height and weight reached by these patients, muscle atrophy and hepatitis C seropositivity. A lower peak bone density reached during late adolescence and young adulthood predisposes to the development of osteoporosis in the later years. Objective: While Vitamin D and calcium supplementation has been recommended by many authors for haemophiliacs, there are no studies looking at Vitamin D levels in these patients. The aim of this study was to evaluate for the prevalence of Vitamin D deficiency and the need for supplementation in the haemophiliac patients in our centre. Methods: The 25-hydroxy Vitamin D3 (25-OH D3), calcium, phosphorus and PTH levels were measured in patients between the ages of 1-18 years during their regular visits to the haemophilia centre. A 25-OH D3 level of >30ng mL )1 is considered as adequate, levels of 21-29 ng mL )1 indicates a relative insufficiency and a level <20ng mL )1 is defined by most experts as being absolutely Vitamin D deficient. Results: Of the 33 patients, 21 of them were Vitamin D deficient (63.6%). Calcium, phosphorus and PTH levels did not correlate with Vitamin D levels.Conclusions: Vitamin D deficiency is highly prevalent in patients with haemophilia in our centre, and given their propensity to develop osteopenia, Vitamin D supplementation is essential in this group of patients. As the calcium, phosphorus and PTH level do not correlate with Vitamin D levels, evaluation of Vitamin D deficiency needs the measurement of serum 25-hydroxy D3 level. The reasons for a deficiency of Vitamin D in these patients, the amount of supplementation needed and the effect of supplementation of Vitamin D on attaining the peak bone mass density of these patients need to be explored further. Vitamin D status in patients with haemophilia (25-OH D3 level)
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