Magnetic resonance imaging based kidney volume assessment for risk stratification in pediatric autosomal dominant polycystic kidney disease

IntroductionIn the pediatric context, most children with autosomal dominant polycystic kidney disease (ADPKD) maintain a normal glomerular filtration rate (GFR) despite underlying structural kidney damage, highlighting the critical need for early intervention and predictive markers. Due to the inverse relationship between kidney volume and kidney function, risk assessments have been presented on the basis of kidney volume. The aim of this study was to use magnetic resonance imaging (MRI)-based kidney volume assessment for risk stratification in pediatric ADPKD and to investigate clinical and genetic differences among risk groups.MethodsThis multicenter, cross-sectional, and case-control study included 75 genetically confirmed pediatric ADPKD patients (5–18 years) and 27 controls. Kidney function was assessed by eGFR calculated from serum creatinine and cystatin C using the CKiD-U25 equation. Blood pressure was assessed by both office and 24-hour ambulatory measurements. Kidney volume was calculated from MRI using the stereological method. Total kidney volume was adjusted for the height (htTKV). Patients were stratified from A to E classes according to the Leuven Imaging Classification (LIC) using MRI-derived htTKV.ResultsMedian (Q1-Q3) age of the patients was 6.0 (2.0–10.0) years, 56% were male. There were no differences in sex, age, height-SDS, or GFR between the patient and control groups. Of the patients, 89% had PKD1 and 11% had PKD2 mutations. Non-missense mutations were 73% in PKD1 and 75% in PKD2. Twenty patients (27%) had hypertension based on ABPM. Median htTKV of the patients was significantly higher than controls (141 vs. 117 ml/m, p = 0.0003). LIC stratification revealed Classes A (38.7%), B (28%), C (24%), and D + E (9.3%). All children in class D + E and 94% in class C had PKD1 variants. Class D + E patients had significantly higher blood pressure values and hypertension compared to other classes (p > 0.05 for all).DiscussionThis study distinguishes itself by using MRI-based measurements of kidney volume to stratify pediatric ADPKD patients into specific risk groups. It is important to note that PKD1 mutation and elevated blood pressure were higher in the high-risk groups stratified by age and kidney volume. Our results need to be confirmed in further studies

Global Variation of Nutritional Status in Children Undergoing Chronic Peritoneal Dialysis : A Longitudinal Study of the International Pediatric Peritoneal Dialysis Network

While children approaching end-stage kidney disease (ESKD) are considered at risk of uremic anorexia and underweight they are also exposed to the global obesity epidemic. We sought to investigate the variation of nutritional status in children undergoing chronic peritoneal dialysis (CPD) around the globe. The distribution and course of body mass index (BMI) standard deviation score over time was examined prospectively in 1001 children and adolescents from 35 countries starting CPD who were followed in the International Pediatric PD Network (IPPN) Registry. The overall prevalence of underweight, and overweight/obesity at start of CPD was 8.9% and 19.7%, respectively. Underweight was most prevalent in South and Southeast Asia (20%), Central Europe (16.7%) and Turkey (15.2%), whereas overweight and obesity were most common in the Middle East (40%) and the US (33%). BMI SDS at PD initiation was associated positively with current eGFR and gastrostomy feeding prior to PD start. Over the course of PD BMI SDS tended to increase on CPD in underweight and normal weight children, whereas it decreased in initially overweight patients. In infancy, mortality risk was amplified by obesity, whereas in older children mortality was markedly increased in association with underweight. Both underweight and overweight are prevalent in pediatric ESKD, with the prevalence varying across the globe. Late dialysis start is associated with underweight, while enteral feeding can lead to obesity. Nutritional abnormalities tend to attenuate with time on dialysis. Mortality risk appears increased with obesity in infants and with underweight in older children.Peer reviewe

Einleitung

van Norden J, Yildirim L. Einleitung. In: van Norden J, Yildirim L, eds. Historisch-politische Bildung im Diskurs. Geschichtsdidaktik theoretisch. Vol 5. 1st ed. Frankfurt am Main: Wochenschau; 2023: 5-10

Historische Erfahrung

van Norden J, Yildirim L, eds. Historische Erfahrung. Geschichtsdidaktik theoretisch. Vol 1 1st ed. Frankfurt am Main: Wochenschau; 2022

Fortschritt und Regression als Horizont emanzipativer Deutung historischer Erfahrung

McLean P. Fortschritt und Regression als Horizont emanzipativer Deutung historischer Erfahrung. In: van Norden J, Yildirim L, eds. Historische Erfahrung . Geschichtsdidaktik theoretisch. Frankfurt: Wochenschau; 2022: 170-187

Dem Historischen auf der Spur. Erfahrung bei Frank Ankersmit

Deile L. Dem Historischen auf der Spur. Erfahrung bei Frank Ankersmit. In: van Norden J, Yildirim L, eds. Historische Erfahrung. 1st ed. Frankfurt a. M: Wochenschau ; 2022

Streit um den Geschichtsunterricht: Historisch-politische Bildung und Auswahlproblematik

van Norden J. Streit um den Geschichtsunterricht: Historisch-politische Bildung und Auswahlproblematik. In: van Norden J, Yildirim L, eds. Historisch-politische Bildung im Diskurs. Perspektiven der Geschichtsdidaktik. Geschichtsdidaktik theoretisch. Vol 5. 1st ed. Frankfurt am Main: Wochenschau; 2023: 119-137

Versuche zu einer epistemologischen Historizität

van Norden J. Erfahrung und Widerfahrnis. In: van Norden J, Yildirim L, eds. Historische Erfahrung. Geschichtsdidaktik theoretisch. Vol 1. 1st ed. Frankfurt am Main: Wochenschau; 2022: 67-84