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肠道手术后出现的范科尼综合征及肝炎经25-羟胆钙化醇治疗后逆转。

Fanconi syndrome following bowel surgery and hepatitis reversed by 25-hydroxycholecalciferol.

作者信息

Chesney R W, Harrison H E

出版信息

J Pediatr. 1975 Jun;86(6):857-61. doi: 10.1016/s0022-3476(75)80214-9.

Abstract

A permature male infant required intravenous alimentation for six weeks following extensive surgery for ileal and cecal necrosis. At 3 months he developed evidence of hepatitis. Subsequently osteoporosis and the Fanconi syndrome appeared. Urine phosphate clearance was 83 percent of creatinine clearance at a serum phosphate concentration of 1.6 mg/dl. Concentration of plasma immunoreactive parathyroid hormone was elevated at 550 pg/ml. 25-Hydroxycholecalciferol was given at 240 mug/day. Aminoaciduria disappeared and bone healing occurred. Serum phosphate rose to 6.5 mg/dl and phosphate clearance fell to 2 percent of creatinine clearance. Upon cessation of 25-OHCC therapy, the Fanconi syndrome recurred despite administration of vitamin D2. 25-OHCC was then administered at 40 mug/day, and the urine abnormalities were reversed. The patient probably developed hyperparathyroidism, secondary malabsorption, and hepatitis. The Fanconi syndrome was the consequence of the hyperparathyroidism. 25-OHCC therapy was more effective than vitamin D in reversing the disordered state, possibly because of impaired hepatic metabolism of vitamin D2.

摘要

一名早产男婴在接受广泛的回肠和盲肠坏死手术后需要静脉营养六周。3个月时,他出现了肝炎迹象。随后出现了骨质疏松症和范科尼综合征。在血清磷酸盐浓度为1.6mg/dl时,尿磷酸盐清除率为肌酐清除率的83%。血浆免疫反应性甲状旁腺激素浓度升高至550pg/ml。给予25-羟胆钙化醇,剂量为每日240μg。氨基酸尿消失,骨愈合发生。血清磷酸盐升至6.5mg/dl,磷酸盐清除率降至肌酐清除率的2%。停止25-OHCC治疗后,尽管给予了维生素D2,范科尼综合征仍复发。然后给予每日40μg的25-OHCC,尿液异常得到逆转。该患者可能发生了甲状旁腺功能亢进、继发性吸收不良和肝炎。范科尼综合征是甲状旁腺功能亢进的结果。25-OHCC治疗在逆转紊乱状态方面比维生素D更有效,可能是因为维生素D2的肝脏代谢受损。

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