Tumoral calcinosis(TC) has long been a controversial clinico-pathological entity. Its pathogenesis and genetic background have been gradually unravelled since its first description in 1943. According to the presence o...Tumoral calcinosis(TC) has long been a controversial clinico-pathological entity. Its pathogenesis and genetic background have been gradually unravelled since its first description in 1943. According to the presence or absence of an underlying calcifying disease process, TC has been divided into primary and secondary varieties. Two subtypes of the primary variety exist; a hyperphosphatemic type with familial basis represented by mutations in Gal NAc transferase 3 gene(GALNT3), KLOTHO or Fibroblast growth factor 23(FGF23) genes, and a normo-phosphatemic type with growing evidence of underlying familial base represented by mutation in SAMD9 gene. The secondary variety is mainly associated with chronic renal failure and the resulting secondary or tertiary hyperparathyroidism. Diagnosis of TC relies on typical radiographic features(on plain radiographs and computed tomography) and the biochemical profile. Magnetic resonance imaging can be done in difficult cases, and scintigraphy reflects the disease activity. Treatment is mainly surgical for the primary variety; however, a stage-oriented conservative approach using phosphate binders, phosphate restricted dietsand acetazolamide should be considered before the surgical approach is pursued due to the high rate of recurrences and complications after surgical intervention. Medical treatment is the mainstay for treatment of the secondary variety, with failure warranting subtotal or total parathyroidectomy. Surgical intervention in these patients should be kept as a last resort.展开更多
考来替兰磷酸盐结合率是临床前药物研究的一项重要内容,该文采用离子色谱法建立考来替兰对磷酸盐结合率的测定方法。采用电导检测器TSKguardcolumn Super IC-Anion HS保护柱及TSKgel Super IC-Anion HS色谱柱,淋洗液为Na_2CO_3(1.1mmol/...考来替兰磷酸盐结合率是临床前药物研究的一项重要内容,该文采用离子色谱法建立考来替兰对磷酸盐结合率的测定方法。采用电导检测器TSKguardcolumn Super IC-Anion HS保护柱及TSKgel Super IC-Anion HS色谱柱,淋洗液为Na_2CO_3(1.1mmol/L)/NaHCO_3(7.5mmol/L),流量为1.0 m L/min。结果表明,方法检出限为0. 02μg/m L,进样量在1.5～30μg/m L范围内线性关系良好(r^2=0.9994),精密度、重复性良好,测定结果的相对标准偏差为0.51%(n=6)。该方法简单方便、稳定可靠,可用于磷结合剂药物对磷酸盐结合率的测定。展开更多
文摘Tumoral calcinosis(TC) has long been a controversial clinico-pathological entity. Its pathogenesis and genetic background have been gradually unravelled since its first description in 1943. According to the presence or absence of an underlying calcifying disease process, TC has been divided into primary and secondary varieties. Two subtypes of the primary variety exist; a hyperphosphatemic type with familial basis represented by mutations in Gal NAc transferase 3 gene(GALNT3), KLOTHO or Fibroblast growth factor 23(FGF23) genes, and a normo-phosphatemic type with growing evidence of underlying familial base represented by mutation in SAMD9 gene. The secondary variety is mainly associated with chronic renal failure and the resulting secondary or tertiary hyperparathyroidism. Diagnosis of TC relies on typical radiographic features(on plain radiographs and computed tomography) and the biochemical profile. Magnetic resonance imaging can be done in difficult cases, and scintigraphy reflects the disease activity. Treatment is mainly surgical for the primary variety; however, a stage-oriented conservative approach using phosphate binders, phosphate restricted dietsand acetazolamide should be considered before the surgical approach is pursued due to the high rate of recurrences and complications after surgical intervention. Medical treatment is the mainstay for treatment of the secondary variety, with failure warranting subtotal or total parathyroidectomy. Surgical intervention in these patients should be kept as a last resort.
文摘考来替兰磷酸盐结合率是临床前药物研究的一项重要内容,该文采用离子色谱法建立考来替兰对磷酸盐结合率的测定方法。采用电导检测器TSKguardcolumn Super IC-Anion HS保护柱及TSKgel Super IC-Anion HS色谱柱,淋洗液为Na_2CO_3(1.1mmol/L)/NaHCO_3(7.5mmol/L),流量为1.0 m L/min。结果表明,方法检出限为0. 02μg/m L,进样量在1.5～30μg/m L范围内线性关系良好(r^2=0.9994),精密度、重复性良好,测定结果的相对标准偏差为0.51%(n=6)。该方法简单方便、稳定可靠,可用于磷结合剂药物对磷酸盐结合率的测定。
