Matrix extracellular phosphoglycoprotein (MEPE) correlates with serum phosphorus prior to and during octreotide treatment and following excisional surgery in hypophosphatemic linear sebaceous nevus syndrome.
Matrix extracellular phosphoglycoprotein (MEPE) correlates with serum phosphorus prior to and during octreotide treatment and following excisional surgery in hypophosphatemic linear sebaceous nevus syndrome.
复制标题
基质细胞外磷酸糖蛋白（MEPE）与低磷线性皮脂痣综合征患者奥曲肽治疗前、治疗期间以及切除手术后的血清磷相关。
DOI：
10.1002/ajmg.a.32395
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发表时间：
2008
期刊：
影响因子：
--
通讯作者：
Fedarko,NealS
中科院分区：
文献类型：
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作者：
Hoffman,WilliamH;Jain,Alka;Chen,Harold;Fedarko,NealS
遗传性和获得性低磷血症导致代谢性骨病，在儿童、青少年和成人中具有显著程度的残疾。线性皮脂腺痣综合征(LSNS)是一种罕见的散发性先天性黑素细胞瘤病，病因不明，表型多样，包括低磷血症[Carey等人，1986]。我们以前曾报道过一例LSNS并低磷血症患者，在奥曲肽和切除手术治疗前和治疗后，血浆磷酸素、成纤维细胞生长因子-23(FGF-23)与血磷呈负相关[Hoffman等人，2005]。我们现在通过报道血清磷和基质细胞外磷酸糖蛋白(MEPE)之间的正相关来扩展这一观察，MEPE是磷酸盐稳态和矿化的下游成员。
Hereditary and acquired forms of hypophosphatemia 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]. We now extend that observation by reporting on the positive correlation between serum phosphorus and matrix extracellular phosphoglycoprotein (MEPE), a downstream member of phosphate homeostasis and mineralization.