Department of Adaptive Biotechnologies, Global Change Research Institute CAS, Brno, Czech Republic.
Unit of Molecular Genetics of Complex Phenotypes, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Hepatol Commun. 2022 Dec;6(12):3311-3323. doi: 10.1002/hep4.2082. Epub 2022 Oct 20.
Nonalcoholic fatty liver disease (NAFLD) has become the most common chronic liver disease in children and adolescents, increasing the risk of its progression toward nonalcoholic steatohepatitis (NASH), cirrhosis, and cancer. There is an urgent need for noninvasive early diagnostic and prognostic tools such as epigenetic marks (epimarks), which would replace liver biopsy in the future. We used plasma samples from 67 children with biopsy-proven NAFLD, and as controls we used samples from 20 children negative for steatosis by ultrasound. All patients were genotyped for patatin-like phospholipase domain containing 3 (PNPLA3), transmembrane 6 superfamily member 2 (TM6SF2), membrane bound O-acyltransferase domain containing 7 (MBOAT7), and klotho-β (KLB) gene variants, and data on anthropometric and biochemical parameters were collected. Furthermore, plasma cell-free DNA (cfDNA) methylation was quantified using a commercially available kit, and ImageStream(X) was used for the detection of free circulating histone complexes and variants. We found a significant enrichment of the levels of histone macroH2A1.2 in the plasma of children with NAFLD compared to controls, and a strong correlation between cfDNA methylation levels and NASH. Receiver operating characteristic curve analysis demonstrated that combination of cfDNA methylation, PNPLA3 rs738409 variant, coupled with either high-density lipoprotein cholesterol or alanine aminotransferase levels can strongly predict the progression of pediatric NAFLD to NASH with area under the curve >0.87. Conclusion: Our pilot study combined epimarks and genetic and metabolic markers for a robust risk assessment of NAFLD development and progression in children, offering a promising noninvasive tool for the consistent diagnosis and prognosis of pediatric NAFLD. Further studies are necessary to identify their pathogenic origin and function.
非酒精性脂肪性肝病(NAFLD)已成为儿童和青少年中最常见的慢性肝病,增加了向非酒精性脂肪性肝炎(NASH)、肝硬化和癌症进展的风险。迫切需要非侵入性的早期诊断和预后工具,如表观遗传标记(epimarks),这些工具将来可能会取代肝活检。我们使用了 67 名经活检证实为 NAFLD 的儿童的血浆样本,并将 20 名经超声检查无脂肪变性的儿童的样本作为对照。所有患者均进行 patatin-like phospholipase domain containing 3 (PNPLA3)、transmembrane 6 superfamily member 2 (TM6SF2)、membrane bound O-acyltransferase domain containing 7 (MBOAT7) 和 klotho-β (KLB) 基因变异的基因分型,并收集了人体测量和生化参数的数据。此外,使用商业上可获得的试剂盒定量了血浆无细胞游离 DNA (cfDNA) 甲基化,并使用 ImageStream(X) 检测游离循环组蛋白复合物和变体。我们发现与对照组相比,NAFLD 儿童的血浆中组蛋白 macroH2A1.2 的水平显著富集,并且 cfDNA 甲基化水平与 NASH 之间存在很强的相关性。接受者操作特征曲线分析表明,cfDNA 甲基化、PNPLA3 rs738409 变体与高密度脂蛋白胆固醇或丙氨酸氨基转移酶水平的结合可以强烈预测儿科 NAFLD 向 NASH 的进展,曲线下面积>0.87。结论:我们的初步研究将表观遗传标记与遗传和代谢标志物相结合,用于儿童 NAFLD 发展和进展的稳健风险评估,为儿科 NAFLD 的一致诊断和预后提供了一种很有前途的非侵入性工具。需要进一步的研究来确定它们的发病机制和功能。