Gallbladder function is altered in sickle hemoglobinopathy.
Gallbladder function is altered in sickle hemoglobinopathy.
复制标题
镰状血红蛋白病的胆囊功能发生改变。
DOI：
10.1016/s0016-5085(89)80018-6
复制
发表时间：
1989
期刊：
影响因子：
29.4
通讯作者：
Pretorius,D
中科院分区：
文献类型：
--
作者：
Everson,GT;Nemeth,A;Kourourian,S;Zogg,D;Leff,NB;Dixon,D;Githens,JH;Pretorius,D
尽管溶血率相当，但只有 50% 的镰状血红蛋白病 (SH) 患者在 20 岁时出现胆色素结石。因此，除了溶血之外的致病因素也可能导致胆结石的形成。在本研究中，我们确定患有 SH 的青少年和年轻人的胆囊功能（通过实时超声检查测量）或胆汁酸代谢（通过同位素稀释质谱法确定）是否发生改变。与健康对照相比，SH 受试者的空腹胆囊体积较大（27 ± 16 vs. 15 ± 5 ml，p < 0.02）和残余胆囊体积（8 ± 6 vs. 4 ± 2 ml，p < 0.03），但排空率相似（0.029 ± 0.016 vs. 0.034 ± 0.029 min-1）和空腹胆囊排空百分比（71% ± 13% 与 72% ± 14%）。在 SH 受试者中，患有和不患有胆结石的受试者的胆囊体积和排空情况相似。一些 SH 受试者胆囊内胆汁淤积，循环胆汁酸池与胆囊中储存的胆汁之间的同位素不平衡证明了这一点。患有胆结石的 SH 受试者往往比不患有胆结石的 SH 受试者具有更小的胆汁酸池（81 ± 11 vs. 163 ± 91 μmol/kg，p = 0.051）。我们得出的结论是，患有 SH 的青少年和年轻人胆囊增大，餐后胆汁量增加。胆囊内的胆汁滞留可能导致胆汁瘀滞并导致色素性胆结石的发病机制。
Despite comparable rates of hemolysis, only 50% of patients with sickle hemoglobinopathy (SH) develop pigment gallstones by age 20 yr. Thus, pathogenetic factors, other than hemolysis, may contribute to gallstone formation. In the present study we determined whether gallbladder function, measured by real-time ultrasonography, or bile acid metabolism, determined by isotope dilution-mass spectrometry, were altered in adolescents and young adults with SH. Compared with healthy controls, SH subjects had larger fasting (27 ± 16 vs. 15 ± 5 ml, p < 0.02), and residual (8 ± 6 vs. 4 ± 2 ml, p < 0.03) volumes of the gallbladder, but similar rates of emptying (0.029 ± 0.016 vs. 0.034 ± 0.029 min-1) and percentage of fasting volume emptied (71% ± 13% vs. 72% ± 14%). In SH subjects, the volume and emptying of the gallbladder were similar between those with and without gallstones. Some SH subjects had stasis of bile within the gallbladder, as demonstrated by isotopic disequilibrium between the circulating bile acid pool and bile stored in the gallbladder. Subjects with SH with gallstones tended to have smaller bile acid pools than SH subjects without gallstones (81 ± 11 vs. 163 ± 91 μmol/kg, p = 0.051). We conclude that adolescents and young adults with SH have enlarged gallbladders that retain an increased postprandial volume of bile. Bile retention within the gallbladder may lead to stasis and contribute to the pathogenesis of pigment gallstones.