Alagille's syndrome is an inherited disorder affecting multiple organ systems. Most characteristic is the paucity of the interlobular bile ducts with cholestasis. The anaesthetic plan for these patients should be based on careful preoperative preparation and attention to the issues involving hepatobiliary, cardiac, neurodevelopmental, nutritional, haematological, ocular and facial abnormalities. We report the case of a five-and-a-half-year old patient with Alagille's syndrome who sustained a pathological fracture of the femur and was scheduled for closed reduction and application of a hip spica cast. Relevant anaesthetic issues are discussed.
Secondary hyperparathyroidism in patients with end-stage renal disease is characterised by elevated circulating levels of parathyroid hormone, due to inadequate synthesis of calcitriol, the active metabolite of vitamin D. Recent studies suggest that administration of calctriol may directly suppress parathyroid (PTH) secretion independent of changes in serum calcium. We have studied the effect of intravenous calcitriol administration on the PTH level in 14 patients on maintenance haemodialysis with serum PTH levels above 2,000 pmol/l over a 16-week period. There was a significant reduction in the PTH level (65%) and a rise of serum calcium to the normal range. There was a significant reduction in serum PTH levels before the serum calcium concentrations increased, suggesting that calcitriol directly inhibits PTH release. In conclusion, intravenous treatment with calcitriol is of clinical importance, because it suppresses hypersecretion of PTH in uraemic patients, with minimal side effects.
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