Fleischer Lindsay T, Ballester Lance, Dutt Mohini, Howarth Kathryn, Poznick Laura, Darge Kassa, Furth Susan L, Hartung Erum A
Cooper Medical School of Rowan University, Camden, NJ, USA.
Biostatistics and Data Management Core, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
J Nephrol. 2023 Jan;36(1):133-145. doi: 10.1007/s40620-022-01416-8. Epub 2022 Aug 18.
Autosomal recessive polycystic kidney disease (ARPKD) causes fibrocystic kidney disease, congenital hepatic fibrosis, and portal hypertension. Serum galectin-3 (Gal-3) and intestinal fatty acid binding protein (I-FABP) are potential biomarkers of kidney fibrosis and portal hypertension, respectively. We examined whether serum Gal-3 associates with kidney disease severity and serum I-FABP associates with liver disease severity in ARPKD.
Cross-sectional study of 29 participants with ARPKD (0.2-21 years old) and presence of native kidneys (Gal-3 analyses, n = 18) and/or native livers (I-FABP analyses, n = 21). Serum Gal-3 and I-FABP were analyzed using enzyme linked immunosorbent assay. Kidney disease severity variables included estimated glomerular filtration rate (eGFR) and height-adjusted total kidney volume (htTKV). Liver disease severity was characterized using ultrasound elastography to measure liver fibrosis, and spleen length and platelet count as markers of portal hypertension. Simple and multivariable linear regression examined associations between Gal-3 and kidney disease severity (adjusted for liver disease severity) and between I-FABP and liver disease severity (adjusted for eGFR).
Serum Gal-3 was negatively associated with eGFR; 1 standard deviation (SD) lower eGFR was associated with 0.795 SD higher Gal-3 level (95% CI - 1.116, - 0.473; p < 0.001). This association remained significant when adjusted for liver disease severity. Serum Gal-3 was not associated with htTKV in adjusted analyses. Overall I-FABP levels were elevated, but there were no linear associations between I-FABP and liver disease severity in unadjusted or adjusted models.
Serum Gal-3 is associated with eGFR in ARPKD, suggesting its value as a possible novel biomarker of kidney disease severity. We found no associations between serum I-FABP and ARPKD liver disease severity despite overall elevated I-FABP levels.
常染色体隐性多囊肾病（ARPKD）可导致纤维囊性肾病、先天性肝纤维化和门静脉高压。血清半乳糖凝集素-3（Gal-3）和肠脂肪酸结合蛋白（I-FABP）分别是肾纤维化和门静脉高压的潜在生物标志物。我们研究了在ARPKD中血清Gal-3是否与肾脏疾病严重程度相关，以及血清I-FABP是否与肝脏疾病严重程度相关。
对29例患有ARPKD（年龄0.2 - 21岁）且有天然肾脏（用于Gal-3分析，n = 18）和/或天然肝脏（用于I-FABP分析，n = 21）的参与者进行横断面研究。采用酶联免疫吸附测定法分析血清Gal-3和I-FABP。肾脏疾病严重程度变量包括估计肾小球滤过率（eGFR）和身高校正后的总肾体积（htTKV）。采用超声弹性成像测量肝纤维化，并以脾长度和血小板计数作为门静脉高压的标志物来表征肝脏疾病严重程度。采用简单和多变量线性回归分析Gal-3与肾脏疾病严重程度（校正肝脏疾病严重程度）之间以及I-FABP与肝脏疾病严重程度（校正eGFR）之间的相关性。
血清Gal-3与eGFR呈负相关；eGFR每降低1个标准差（SD），Gal-3水平升高0.795个SD（95%CI - 1.116， - 0.473；p < 0.001）。校正肝脏疾病严重程度后，这种相关性仍然显著。在校正分析中，血清Gal-3与htTKV无关。总体I-FABP水平升高，但在未校正或校正模型中，I-FABP与肝脏疾病严重程度之间均无线性相关性。
在ARPKD中，血清Gal-3与eGFR相关，提示其可能作为肾脏疾病严重程度的新型生物标志物。尽管I-FABP总体水平升高，但我们未发现血清I-FABP与ARPKD肝脏疾病严重程度之间存在相关性。
