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• W2896848735 abstract "To the Editor: Thank you for the interesting and valuable reflections on our recent article (1). Renal function is an important but controversial issue in both pediatric and adult patients with liver disease, with varying, often conflicting results. The information on maintained or defect renal function is important, both because of the risk of acute kidney injury (AKI), as shown among others by Lal et al (2), and also because of the pharmacokinetics in this group. Thus, reliable information on the renal function is essential at any time during the natural course of the chronic liver disease (CLD) and we do not recommend less observance due to “false sense of security.” Instead, we think it is essential to be aware of the risk of developing AKI. We have investigated a large cohort of pediatric patients at their first work-up, that is, at the time when CLD was found, when they were under stable condition. We believe that this is also valid for the 5 children with acute liver failure, where the patients recovered spontaneously. Our aim with the study was to investigate kidney function at the onset of CLD, on naïve patients who have not received any treatment. We have investigated patients with heterogeneous diagnoses, with hepatocellular damage as the sole common denominator, that is, to study the impact of unspecific hepatocellular damage on the kidneys. We are perfectly aware of the fact that some of the diagnoses, mentioned by Lal et al can have a direct hit on the kidneys and thus we strictly applied our exclusion criteria, that is, all children with liver tumor were excluded, and those patients whose primary disease created risk of direct renal involvement, for example, patients with autosomal recessive polycystic kidney disease with liver fibrosis, and chronic viral hepatitis. It is well known that patients with Alagille syndrome often have renal involvement, which we also described and these were also the cases with the lowest glomerular filtration rate (GFR), but the extremely wide and largely unexplained variations of the Alagille syndrome phenotypes make this group interesting also in this context. None of the other groups had decreased GFR, which we think is of interest. We agree that the finding of hyperfiltration in a group of patients is of interest. We also think that it is of interest that patients with certain abnormal liver function tests showed higher GFR than those with normal liver function. In the Discussion we propose that cholestasis might increase renal blood flow, parallelly with the upregulation of the hepatic synthesis of coagulation factors described by Magnusson et al (3). We therefore think it will be of great interest to study the long-term follow-up of all patients, especially of those with hyperfiltration. The data for this study are collected and the results are being analyzed. We also agree that hypofiltration defined as <−2SD of that of the controls (ie, <94 mL/min/1.73 m2) was found in 15.2% of our patients but we did not define these cases as having AKI. Moreover our patients were studied in stable condition and neither after having developed AKI, nor with acute-on-CLD. As we mentioned in the Discussion we aim to investigate the renal function also during the natural course of the CLD on the same cohort and this work is under way. But already now we can conclude that the renal function, including GFR has to be followed regularly in children with CLD. While waiting for the perfectly reliable, noninvasive method, the local traditions have to be followed by every center." @default.
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• W2896848735 date "2019-01-01" @default.
• W2896848735 modified "2023-10-01" @default.
• W2896848735 title "Kidney Function in Children With Chronic Liver Disease" @default.
• W2896848735 cites W2013406994 @default.
• W2896848735 cites W2775012742 @default.
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• W2896848735 doi "https://doi.org/10.1097/mpg.0000000000002170" @default.
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