口服磷酸钙：治疗克里格勒-纳贾尔综合征的新疗法？
Oral calcium phosphate: a new therapy for Crigler-Najjar disease?
作者信息
Van der Veere C N, Jansen P L, Sinaasappel M, Van der Meer R, Van der Sijs H, Rammeloo J A, Goyens P, Van Nieuwkerk C M, Oude Elferink R P
机构信息
Department of Gastrointestinal Diseases, Academic Medical Center, Amsterdam, The Netherlands.
出版信息
Gastroenterology. 1997 Feb;112(2):455-62. doi: 10.1053/gast.1997.v112.pm9024299.
BACKGROUND & AIMS: Calcium phosphate binds unconjugated bilirubin in vitro, and dietary calcium phosphate supplementation reduces the serum bilirubin level in rats with hereditary unconjugated hyperbilirubinemia (Gunn rats). The aim of this study was to evaluate the effect of oral calcium phosphate supplementation on plasma bilirubin levels in patients with Crigler-Najjar disease.
METHODS
A placebo-controlled, double-blind, crossover design was used. Eleven patients, 2-42 years of age, participated. The group included 5 patients with type I disease who were all treated with phototherapy and 6 patients with type II disease who were primarily treated with phenobarbital. In addition to plasma bilirubin levels, dietary intake and urinary and fecal excretion of calcium and phosphate were evaluated.
RESULTS
A modest but significant decrease in serum bilirubin was observed in patients with type I disease (18% +/- 6%, P = 0.03) but not in patients with type II disease during treatment with calcium phosphate. Urinary output of calcium and phosphate did not change during the treatment period.
CONCLUSIONS
Oral calcium phosphate may be a useful adjuvant to photo-therapy in Crigler-Najjar type I disease.
背景与目的
磷酸钙在体外可结合非结合胆红素，膳食补充磷酸钙可降低遗传性非结合性高胆红素血症大鼠（冈恩大鼠）的血清胆红素水平。本研究旨在评估口服补充磷酸钙对克里格勒 - 纳贾尔病患者血浆胆红素水平的影响。
方法
采用安慰剂对照、双盲、交叉设计。11名年龄在2至42岁的患者参与了研究。该组包括5名I型疾病患者，均接受光疗；以及6名II型疾病患者，主要接受苯巴比妥治疗。除了血浆胆红素水平外，还评估了钙和磷的膳食摄入量以及尿和粪便排泄量。
结果
在磷酸钙治疗期间，I型疾病患者的血清胆红素出现适度但显著的下降（18% ± 6%，P = 0.03），而II型疾病患者未出现下降。治疗期间钙和磷的尿量未发生变化。
结论
口服磷酸钙可能是克里格勒 - 纳贾尔I型疾病光疗的有用辅助手段。
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