Portal hypertension syndrome in children with autosomal recessive polycystic kidney disease with liver cysts and hepatic fibrosis

Hepatic fibrosis, liver cysts, and portal hypertension are extrarenal manifestations that determine the prognosis of autosomal recessive polycystic kidney disease in children.Purpose. To assess the features of the manifestation and course of liver cystic disease and fibrosis, the development of portal hypertension in the follow-up medical history of children with autosomal recessive polycystic kidney disease.Material and methods. We studied 27 children with autosomal recessive polycystic kidney disease, with two children with a fatal outcome in the neonatal period excluded. 25 children 1–17 years old with autosomal recessive polycystic kidney disease were divided into 2 groups depending on the presence of portal hypertension syndrome. In the long-term follow-up 10 (40%) of 25 children with autosomal recessive polycystic kidney disease had no signs of portal hypertension (group 1), 15 (60%) children had portal hypertension syndrome (group 2). The long-term follow-up, clinical, genealogical, laboratory and instrumental (US, MRI/CT of kidney and abdominal cavity, liver elastography) research methods were used in the study. The diagnosis was confirmed by autopsy for 3 children.Results. There were no cases of changes in the liver and bile ducts characteristic of autosomal recessive polycystic kidney disease by prenatal ultrasound examination. From 27 children with autosomal recessive polycystic kidney disease, in 10 (37%) were diagnosed the liver fibrosis in the long-term follow-up, 22 (81%) had cystic enlargement of intrahepatic veins, of which 15 (68%) had polycystic liver disease, 3 (14%) had Caroli disease. All children with autosomal recessive polycystic kidney disease and portal hypertension syndrome had varicose veins of the esophagus and stomach according to esophagoduodenoscopy, 53% with indications for ligation of phlebectasia, 47% were diagnosed with thrombocytopenia, 67% with anemia, 100% with splenomegaly, 13% with esophageal-gastric bleeding.Conclusion. The differences in the initial manifestations of autosomal recessive polycystic kidney disease were revealed, while no differences in the incidence of hepatic fibrosis and liver cysts were found in 2 compared groups of children.