Berg Ulla B, Németh Antal
Division of Pediatrics, Department of Clinical Science, Intervention and Technology, Karolinska Institutet, Astrid Lindgren's Children's Hospital, Karolinska University Hospital Huddinge, Stockholm, Sweden.
J Pediatr Gastroenterol Nutr. 2018 Apr;66(4):575-580. doi: 10.1097/MPG.0000000000001862.
On the basis of studies with hepatorenal syndrome, it is widely regarded that renal function is impacted in chronic liver disease (CLD). Therefore, we investigated renal function in children with CLD.
In a retrospective study of 277 children with CLD, renal function was investigated as glomerular filtration rate (GFR) and effective renal plasma flow (ERPF), measured as clearance of inulin and para-amino hippuric acid or clearance of iohexol. The data were analyzed with regard to different subgroups of liver disease and to the grade of damage.
Hyperfiltration (>+2 SD of controls) was found in the subgroups of progressive familial intrahepatic cholestasis (44%), glycogenosis (75%), and acute fulminant liver failure (60%). Patients with biliary atresia, most other patients with metabolic disease and intrahepatic cholestasis, and those with vascular anomalies and cryptogenic cirrhosis had normal renal function. Decreased renal function was found in patients with Alagille's syndrome (64% < -2 SD). Increased GFR and ERPF was found in patients with elevated transaminases, low prothrombin level, high bile acid concentration, and high aspartate-aminotransferase-to-platelet ratio.
Most children with CLD had surprisingly well preserved renal function and certain groups had even hyperfiltration. The finding that children with decompensated liver disease and ongoing liver failure had stable kidney function suggests that no prognostic markers of threatening hepatorenal syndrome were at hand. Moreover, estimation of GFR based on serum creatinine fails to reveal hyperfiltration.
基于肝肾综合征的研究,人们普遍认为慢性肝病(CLD)会影响肾功能。因此,我们对CLD患儿的肾功能进行了研究。
在一项对277例CLD患儿的回顾性研究中,通过菊粉清除率、对氨基马尿酸清除率或碘海醇清除率来测定肾功能,以肾小球滤过率(GFR)和有效肾血浆流量(ERPF)作为指标。针对不同肝病亚组和损伤程度对数据进行了分析。
在进行性家族性肝内胆汁淤积症亚组(44%)、糖原贮积症亚组(75%)和急性暴发性肝衰竭亚组(60%)中发现了超滤过(>对照组+2标准差)。患有胆道闭锁的患儿、大多数患有其他代谢性疾病和肝内胆汁淤积症的患儿,以及患有血管异常和隐源性肝硬化的患儿肾功能正常。在阿拉吉列综合征患儿中发现肾功能下降(64%<-2标准差)。转氨酶升高、凝血酶原水平低、胆汁酸浓度高和天冬氨酸转氨酶与血小板比值高的患儿GFR和ERPF升高。
大多数CLD患儿的肾功能出人意料地保存良好,某些组甚至出现超滤过。失代偿性肝病和进行性肝衰竭患儿肾功能稳定这一发现表明,尚无威胁肝肾综合征的预后标志物。此外,基于血清肌酐估算GFR无法发现超滤过情况。