Ling S C, Amarri S, Slater C, Hollman A S, Preston T, Weaver L T
Department of Child Health, University of Glasgow, United Kingdom.
J Pediatr Gastroenterol Nutr. 2000 Apr;30(4):368-72. doi: 10.1097/00005176-200004000-00004.
Liver disease associated with cystic fibrosis may not only limit the solubilisation and absorption of the products of fat digestion, but also may depress the activity of pancreatic lipase. The purpose of this study was to measure the effect of liver disease on triacylglycerol lipolysis using the 13C-mixed triacylglycerol breath test.
Forty children with cystic fibrosis took 13C-mixed triacylglycerol with a standard breakfast and the child's normal pancreatic enzyme replacement therapy. Breath samples were collected before and every 30 minutes after ingestion for 6 hours. The cumulative percentage dose of 13C recovered at 6 hours was calculated from sequential measurements of 13C enrichment of breath CO2, measured by isotope ratio mass spectrometry. Liver abnormalities and portal hypertension were defined by ultrasound scan and clinical examination.
Twenty-four children had liver abnormalities, including 5 with portal hypertension. No difference was found between cumulative percentage dose of 13C recovered at 6 hours in 16 children with no liver abnormality (mean, 21.4%+/-11.1%), 19 children with liver abnormalities (22.2%+/-10.0%) and 5 children with portal hypertension (20.9%+/-7.1%).
Intestinal lipolysis is not reduced in cystic fibrosis liver disease when measured using the 13C mixed triacylglycerol breath test. These findings affirm the test's use as an indirect measure of fat digestion that is not affected by inadequate intraluminal bile salts or liver disease.
与囊性纤维化相关的肝脏疾病不仅可能限制脂肪消化产物的溶解和吸收，还可能抑制胰脂肪酶的活性。本研究的目的是使用13C混合甘油三酯呼气试验来测量肝脏疾病对甘油三酯脂解的影响。
40名囊性纤维化患儿在早餐时服用13C混合甘油三酯并接受其常规的胰酶替代疗法。在摄入前以及摄入后每30分钟采集呼气样本，持续6小时。通过同位素比率质谱法测量呼气中二氧化碳的13C富集度，并据此计算6小时时回收的13C累积剂量百分比。通过超声扫描和临床检查来定义肝脏异常和门静脉高压。
24名儿童存在肝脏异常，其中5名患有门静脉高压。在16名无肝脏异常的儿童（平均21.4%±11.1%）、19名有肝脏异常的儿童（22.2%±10.0%）和5名患有门静脉高压的儿童（20.9%±7.1%）中，6小时时回收的13C累积剂量百分比未发现差异。
使用13C混合甘油三酯呼气试验测量时，囊性纤维化肝脏疾病患儿的肠道脂解并未降低。这些发现证实了该试验可作为一种不受肠腔内胆汁盐不足或肝脏疾病影响的脂肪消化间接测量方法。
