Matrix extracellular phosphoglycoprotein (MEPE) correlates with serum phosphorus prior to and during octreotide treatment and following excisional surgery in hypophosphatemic linear sebaceous nevus syndrome.

摘要

Hereditary and acquired forms of hypophospha-temia result in metabolic bone disease with significant degrees of disability in children, adolescents and adults. Linear sebaceous nevus syndrome (LSNS) is a rare sporadic congenital phakomatosis of unknown etiology with a variable phenotype that includes hypophosphatemia [Carey et al., 1986]. We have previously reported on a patient with LSNS and hypophosphatemia where the plasma phosphatonin, fibroblast growth factor-23 (FGF-23), was inversely related to the serum phosphorous prior to and after treatment with octreotide and excision surgery [Hoffman et al., 2005].