CEINGE Advanced Biotechnologies s.c.a.r.l., Naples, Italy.
Department of Molecular Medicine and Medical Biotechnology, University of Naples Federico II, Naples, Italy.
Clin Chem Lab Med. 2020 Sep 25;58(10):1725-1730. doi: 10.1515/cclm-2019-1112.
Background Patients with cystic fibrosis (CF) have a reduced intestinal absorption of cholesterol and in a preliminary study we observed differences in plasma sterol profile between patients with pancreatic sufficiency (PS) and those with pancreatic insufficiency (PI). Therefore, we hypothesized that the sterol analysis may contribute to study the digestion and absorption state of lipids in patients with CF. To this aim we evaluated plasma sterols in a significant number of adult patients with CF in relation to the pancreatic status. Methods Beside cholesterol, we measured phytosterols and lathosterol as markers of intestinal absorption and hepatic biosynthesis, respectively, by gas-chromatography in plasma of adult CF patients with pancreatic sufficiency (PS-CF, n = 57), insufficiency (PI-CF, n = 97) and healthy subjects (control group, CT, n = 71). Results PI-CF patients had cholesterol and phytosterols levels significantly lower than PS-CF and CT (p < 5 × 10-10) suggesting a reduced intestinal absorption of sterols related to PI. Instead, lathosterol was significantly higher in PI-CF patients than PS-CF and CT (p < 0.0003) indicating an enhanced cholesterol biosynthesis. In PI-CF patients, phytosterols positively correlate with vitamin E (p = 0.004). Both the classes of molecules need cholesterol esterase for the intestinal digestion, thus the reduced levels of such lipids in serum from PI-CF patients may depend on a reduced enzyme activity, despite the pancreatic enzyme supplementation in all PI-CF patients. Conclusions A plasma sterols profile may be useful to evaluate the metabolic status of lipids in adult patients with CF and could help to manage the pancreatic enzyme supplementation therapy.
囊性纤维化（CF）患者的胆固醇肠道吸收率降低，我们的初步研究观察到具有充分胰功能（PS）和胰功能不全（PI）的 CF 患者之间的血浆固醇谱存在差异。因此，我们假设固醇分析可能有助于研究 CF 患者的脂质消化和吸收状态。为此，我们评估了大量成年 CF 患者的血浆固醇，与胰腺状态有关。
除胆固醇外，我们还通过气相色谱法测量了植物固醇和羊毛固醇，分别作为肠道吸收和肝生物合成的标志物，在具有充分胰功能的 CF 患者（PS-CF，n=57）、胰功能不全的 CF 患者（PI-CF，n=97）和健康受试者（对照组，CT，n=71）的血浆中进行了测量。
PI-CF 患者的胆固醇和植物固醇水平明显低于 PS-CF 和 CT（p<5×10-10），表明与 PI 相关的固醇肠道吸收减少。相反，PI-CF 患者的羊毛固醇明显高于 PS-CF 和 CT（p<0.0003），表明胆固醇生物合成增强。在 PI-CF 患者中，植物固醇与维生素 E 呈正相关（p=0.004）。这两类分子的肠道消化都需要胆固醇酯酶，因此 PI-CF 患者血清中这些脂质的水平降低可能取决于酶活性的降低，尽管所有 PI-CF 患者都补充了胰酶。
血浆固醇谱可用于评估成年 CF 患者的脂质代谢状态，并有助于管理胰腺酶补充治疗。
