A proposed classification for standardized reporting of liver disease in children with autosomal recessive polycystic kidney disease

Autosomal recessive polycystic kidney disease (ARPKD) is a complex disorder characterized by varying degrees of renal and hepatic involvement. It is caused by mutations in polycystic kidney and hepatic disease 1 gene on chromosome 6p12 or less commonly DAZ interacting zinc finger protein 1.1 Attempts to map out a genotype- phenotype correlation in this patient population has not met with much success due to heterogeneity in presentation and progression of disease.2 Existing classifications emphasize renal pathology and do not address the hepatic aspect. A classification for liver disease will help to tailor future investigations, treatment pathways, and help to bring uniformity in reporting of this disease. We propose to divide the liver disease into two categories, that is, clinically significant liver disease and clinically insignificant liver disease (Figure 1). These criteria were based on BAVENO VII criteria for portal hypertension as well as hepatic complications well described in ARPKD.3-6 A survey was sent to 115 pediatric hepatology/gastroenterology colleagues across the globe. We got 36 responses; 33 clinicians agreed, 2 were unsure and 1 disagreed. All respondents except one agreed with the need for such a classification. With the initial positive response received, we hope to validate this classification with larger cohort of colleagues in pediatric gastroenterology, hepatology, and nephrology worldwide and hope that professionals would use this as a standard reporting tool for future publication reports of liver disease in ARPKD children. The authors declare no conflicts of interest.