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PurposeRepeated gadolinium-based contrast agent (GBCA)-enhanced MRIs are crucial in the diagnosis and follow-up of oncologic and chronic disorders in pediatric patients. The aim of the study was to evaluate the frequency and severity of adverse reactions to GBCAs in children after a single vs. multiple GBCA-enhanced abdomen MRIs.Material and methodsAll pediatric patients with at least one GBCA-enhanced abdominal MRI between 2009 and 2020 were retrospectively evaluated based on adverse reactions reports, according to the classification system of the American College of Radiology and guidelines on contrast agents of the European Society of Urogenital Radiology. A Student´s t-test analysis, a spearman ρ-correlation and a Chi-square test between the reported adverse reactions and the total number of GBCA applications, and the number of each applied GBCA was calculated. A p-value ResultsOf 623 patients with 964 GBCA-enhanced abdomen MRIs, there were 464 patients with only one and 159 patients with multiple GBCA administrations. Of 964 GBCA doses administrated, two cases with urticaria (mild allergy-like adverse reaction) and one case with vomiting (mild chemotoxic adverse reaction) were recorded (3/964 = 0.31%), but all the reports were in patients with multiple GBCA administration (3/159 = 1.89%). No adverse reactions in patients with a single GBCA administration were observed. The reported adverse reactions correlated significantly with the total number of GBCAs (pConclusionGBCA-associated adverse reactions are rare and mostly mild, but initially well-tolerated GBCA could cause adverse reactions due to the increase likelihood of drug hypersensitivity upon repeated GBCA exposure.

(1) Background: Progressive familial intrahepatic cholestasis (PFIC) is a rare cause of liver failure. Surgical biliary diversion (SBD) and ileal bile salt inhibitors (IBAT) can delay or prevent liver transplantation (LTX). A comparison of the two methodologies in the literature is lacking. The combination has not been investigated. (2) Methods: We performed a literature survey on medical and surgical treatments for PFIC and reviewed the charts of our patients with PFIC of a tertiary hospital. The end points of our analysis were a decrease in serum bile acid (sBA) levels, reduction of pruritus and delay or avoidance of (LTX). (3) Results: We included 17 case series on SBD with more than 5 patients and a total of 536 patients. External or internal SBD, either conventional or minimally invasive, can reduce pruritus and sBA, but not all PFIC types are suitable for SBD. Six publications described the use of two types of IBAT in PFIC with a total of 118 patients. Treatment response was dependent on genetic type and subtype. Patients with PFIC 2 (nt-BSEP) showed the best response to treatment. Four out of eleven PFIC patients underwent SBD at our centre, with two currently receiving IBAT. (4) Conclusions: Limited data on IBAT in selected patients with PFIC show safety and effectiveness, although surgical methods should still be considered as a successful bridging procedure. Further studies to evaluate a possible combination of IBAT and SBD in PFIC are warranted and treatment decision should be discussed in an interdisciplinary board.