BACKGROUND:The early vascular impact of metabolic dysfunction-associated steatotic liver disease (MASLD) remains unclear. This study examined associations of steatosis in childhood and young adulthood with cardiovascular risk in adulthood. METHODS:Children with severe obesity were investigated for MASLD and cardiovascular risk, with 10-year follow-up. Hepatic steatosis was measured using proton magnetic resonance spectroscopy and cardiovascular risk by ultrasound carotid intima-media thickness (cIMT). Participants were categorised into four trajectory groups based on presence of steatosis in childhood and/or adulthood: 'absent', 'diminishing', 'adult-onset' and 'persistent'. Associations between steatosis (presence and change over time) and cIMT, and between childhood metabolic factors and cIMT progression, were evaluated. RESULTS:Of 52 participants, 46% had steatosis in childhood ('diminishing' or 'persistent'), and 46% in adulthood ('adult-onset' or 'persistent'). Childhood steatosis was not associated with cIMT in adulthood, whereas adulthood steatosis was independently associated with increased cIMT (mean difference 0.035 mm, 95% CI: 0.010-0.060 mm; p-adjusted = 0.008). Mean cIMT at follow-up increased progressively across steatosis trajectory groups; (adjusted p-for-trend = 0.003). Childhood steatosis was the only metabolic factor associated with cIMT progression (β = 0.006, p = 0.030). CONCLUSIONS:Hepatic steatosis in young adulthood is independently associated with increased cardiovascular risk, and childhood steatosis relates to cIMT progression, supporting early MASLD detection and prevention.

Hypertrophic Cardiomyopathy (HCM) is an inherited myocardial disease characterised by left ventricular hypertrophy, which carries an increased risk of life-threatening arrhythmias and sudden cardiac death. The age of presentation and the underlying aetiology have a significant impact on the prognosis and quality of life of children with HCM, as childhood-onset HCM is associated with high mortality risk and poor long-term outcomes. Accurate cardiac assessment and identification of the HCM phenotype are therefore crucial to determine the diagnosis, prognostic stratification, and follow-up. Cardiac magnetic resonance (CMR) is a comprehensive evaluation tool capable of providing information on cardiac morphology and function, flow, perfusion, and tissue characterisation. CMR allows to detect subtle abnormalities in the myocardial composition and characterise the heterogeneous phenotypic expression of HCM. In particular, the detection of the degree and extent of myocardial fibrosis, using late-gadolinium enhanced sequences or parametric mapping, is unique for CMR and is of additional value in the clinical assessment and prognostic stratification of paediatric HCM patients. Additionally, childhood HCM can be progressive over time. The rate, timing, and degree of disease progression vary from one patient to the other, so close cardiac monitoring and serial follow-up throughout the life of the diagnosed patients is of paramount importance. In this review, an update of the use of CMR in childhood HCM is provided, focussing on its clinical role in diagnosis, prognosis, and serial follow-up.

Fetal cardiovascular magnetic resonance imaging (MRI) has emerged as a complementary modality for prenatal imaging in suspected congenital heart disease. Ongoing technical improvements extend the potential clinical value of fetal cardiovascular MRI. Ascertaining equivocal prenatal diagnostics obtained with ultrasonography allows for appropriate parental counseling and planning of postnatal surgery. This work summarizes current acquisition techniques and clinical applications of fetal cardiovascular MRI in the prenatal diagnosis and follow-up of fetuses with congenital heart disease. LEVEL OF EVIDENCE: 3 TECHNICAL EFFICACY: Stage 3.

Rhabdomyosarcoma, although rare, is the most frequent soft tissue sarcoma in children and adolescents. It can present as a mass at nearly any site in the body, with most common presentations in the head and neck, genitourinary tract and extremities. The optimal diagnostic approach and management of rhabdomyosarcoma require a multidisciplinary team with multimodal treatment, including chemotherapy and local therapy. Survival has improved over the last decades; however, further improvement in management is essential with current 5-year overall survival ranging from 35% to 100%, depending on disease and patient characteristics. In the full patient journey, from diagnosis, staging, management to follow-up after therapy, the paediatric radiologist and nuclear physician are essential members of the multidisciplinary team. Recently, guidelines of the European paediatric Soft tissue sarcoma Study Group, the Cooperative Weichteilsarkom Studiengruppe and the Oncology Task Force of the European Society of Paediatric Radiology (ESPR), in an ongoing collaboration with the International Soft-Tissue Sarcoma Database Consortium, provided guidance for high-quality imaging. In this educational paper, given as a lecture during the 2022 postgraduate ESPR course, the multi-disciplinary team of our national paediatric oncology centre presents the journey of two patients with rhabdomyosarcoma and discusses the impact on and considerations for the clinical (paediatric) radiologist and nuclear physician. The key learning points of the guidelines and their implementation in clinical practice are highlighted and up-to-date insights provided for all aspects from clinical suspicion of rhabdomyosarcoma and its differential diagnosis, to biopsy, staging, risk stratification, treatment response assessment and follow-up.

ABSTRACTObjectives:Disturbances in lipid metabolism play an important role in the pathogenesis of nonalcoholic fatty liver disease (NAFLD). Using lipidomics, an analytical technique that is used to broadly survey lipid metabolism, we searched for biomarkers in plasma that are correlated with the presence of hepatic steatosis in children with obesity.Methods:Lipidomics was performed in plasma samples of 21 children with obesity in whom steatosis was detected using proton magnetic resonance spectroscopy (1H‐MRS) and were compared with the lipidome of 21 samples of nonsteatotic subjects with obesity.Results:Forty‐two samples were analyzed (57% boys; median age 15 years). A total of 18 lipid classes constituting 839 different lipid species were identified. A statistically significant increase in alkyldiacylglycerol (TG[O]) and phosphatidylethanolamine (PE) species and a significant decrease in alkyl/alkenyl‐phosphatidylethanolamine (PE[O]), alkyl/alkenyl‐lysophosphatidylethanolamine (LPE[O]) and alkyl/alkenyl‐phosphatidylcholine (PC[O]) was observed in children with hepatic steatosis compared with controls. Twelve individual lipid species of 3 lipid classes were significantly increased in steatotic subjects compared with controls.Conclusions:In this pilot study, we found statistically significant alterations in 5 major lipid classes and 12 individual lipid species in children with steatosis. These might be potential biomarkers for pediatric NAFLD. Lipidomic studies in larger cohorts of children are needed to determine the diagnostic value of these lipids and determine whether results can be generalized for different age groups and ethnic backgrounds.

The last author of the original published version of the above article misses his middle name for correct identification. Timo de Haan has been presented correctly above as Timo R. de Haan.

This article originally published with all author names incorrectly listed. All author names have now been transposed and appear correctly above.

Alanine aminotransferase (ALT) and ultrasound (US) are the most commonly used tools for detecting non-alcoholic fatty liver disease (NAFLD). No direct comparison of these two modalities in children exists. We aimed to compare head-to-head the diagnostic accuracy of ALT and US and their combination for detecting NAFLD in children with obesity. Ninety-nine children with severe obesity underwent simultaneous serum-ALT and abdominal ultrasound (US steatosis score 0–3). Proton magnetic resonance spectroscopy was used as reference standard for detecting steatosis/NAFLD. ROC curve analyses were performed to determine diagnostic performance and to determine optimum screening cut-points aiming for a specificity ≥ 80%. The area under the ROC (AUROC) of ALT and US were not significantly different (0.74 and 0.70, respectively). At the optimal ALT threshold (≥40 IU/L), sensitivity was 44% and specificity was 89%. At the optimal US steatosis score (≥ 2), sensitivity was 51% and specificity was 80%. Combining ALT and US did not result in better accuracy than ALT or US alone.

This review on magnetic resonance elastography (MRE) of the breast provides an overview of available literature and describes current developments in the field of breast MRE, including new transducer technology for data acquisition and multi-frequency-derived power-law behaviour of tissue. Moreover, we discuss the future potential of breast MRE, which goes beyond its original application as an additional tool in differentiating benign from malignant breast lesions. These areas of ongoing and future research include MRE for pre-operative tumour delineation, staging, monitoring and predicting response to treatment, as well as prediction of the metastatic potential of primary tumours.

Gaucher disease (GD) is a lysosomal storage disorder characterized by the storage of glycosphingolipids in macrophages. Despite effective therapy, residual disease is present in varying degrees and may be associated with late complications, such as persistent bone or liver disease and increased cancer risk. Gaucher macrophages are capable of storing iron and locations of residual disease may thus be detectable with iron imaging. Forty type 1 GD (GD1) patients and 40 matched healthy controls were examined using a whole-body magnetic resonance imaging protocol consisting of standard sequences, allowing analysis of iron content per organ, expressed as R2* (Hz). Median R2* values were significantly elevated in GD1 patients as compared to healthy controls in liver [41 Hz (range 29-165) vs. 38 Hz (range 28-53), P < 0·01], femoral bone marrow [54 Hz (range 37-129) vs. 49 Hz (range 39-69), P = 0·036] and vertebral bone marrow (118 Hz (range 82-210) vs. 105 Hz (range 76-149), P < 0·01). In the spleen, primarily focal Gaucher lesions known as Gaucheroma were found to have increased R2* values. R2* values of liver, spleen and vertebral bone marrow strongly correlated with serum ferritin levels. GD1 patients with persistent hyperferritinaemia demonstrate increased iron levels in liver and bone marrow, which may carry a risk for liver fibrosis and cancer.

In Gaucher disease (GD) imaging of liver and spleen is part of routine follow-up of GD patients. Focal lesions in both liver and spleen are frequently reported at radiological examinations. These lesions often represent benign accumulations of Gaucher cells, so-called "gaucheroma", but malignancies, especially hepatocellular carcinoma, are more frequently found in GD as well. We report the imaging characteristics of all focal lesions in liver and spleen in the Dutch GD cohort. Of the 95 GD1 patients, 40% had focal splenic and/or hepatic lesions, associated with more severe GD. Lesions identified as gaucheroma have variable imaging characteristics: hyper- to hypointense on MRI, hyper- or hypoechoic on US and hypodense on computed tomography (CT). Hepatic lesions were classified as simple cysts or haemangioma based upon imaging characteristics. Focal nodular hyperplasia (FNH), gaucheroma and hepatocellular carcinoma (HCC) could not be distinguished by conventional US, CT or MRI. Growth of these lesions and/or characteristics of HCC on dynamic CT or MRI and pathology was used to identify or rule out HCC. We propose a decision-making algorithm including the use of growth and dynamic CT- or MRI-scanning to characterize lesions.

ObjectiveNonalcoholic fatty liver disease (NAFLD) has been recognized as an independent risk factor for cardiovascular disease in adults. It has not been established whether NAFLD is related to early atherosclerotic changes in children.MethodsIn a cross-sectional study, 78 non diabetic, non smoking children with severe obesity were evaluated for NAFLD. Proton magnetic resonance spectroscopy was used to detect liver steatosis and serum ALT was used as a surrogate marker for steatohepatitis. Carotid intima-media thickness (CIMT) and arterial wall stiffness were measured using ultrasound.ResultsSteatosis was present in 41 (53%) of subjects. Of these children, 26 out of 41 (63%) had elevated ALT levels. No differences in CIMT and arterial wall stiffness were observed between those without and with steatosis and those with steatosis plus elevated ALT levels [CIMT=0.47 (0.06), 0.48 (+/- 0.06) and 0.48 (+/- 0.07) mm, respectively; stiffness=2.78 (+/- 0.50), 3.00 (+/- 0.81), and 2.90 (+/- 0.78), respectively]. Steatosis and ALT were not correlated to CIMT (r=-0.02 and -0.14, respectively) or arterial wall stiffness (r=0.13 and -0.11, respectively).ConclusionsIn this study, no relationship between NAFLD and early atherosclerotic changes in children was observed. An atherogenic effect of steatohepatitis (NASH) on pediatric age and long-term atherogenic consequences of simple steatosis cannot be excluded based on this study.

Background/Aims: Lifestyle intervention is the only established therapy for non-alcoholic fatty liver disease (NAFLD). The optimal treatment schedule and predictors of response of this treatment have not been established in children. We aimed to evaluate the 2-year efficacy of an inpatient versus ambulatory intensive lifestyle intervention for treating NAFLD in children with severe obesity. Methods: A cohort study of 51 severely obese non-diabetic children (mean age 14.7 (±2.4) years; BMI- z -score 3.5 (±0.5)) with liver steatosis were non-randomly allocated to inpatient treatment (2 or 6 months), ambulatory treatment or usual care. Proton Magnetic Resonance Spectroscopy determined liver steatosis and serum alanine aminotransferase (ALT) at 6 months were the primary outcome measures. Baseline variables were evaluated as predictors of treatment response. Results: Liver steatosis had disappeared in 43, 29 and 22% and serum ALT normalized in 41, 33 and 6% at the end of 6 months in the inpatient, ambulatory or usual care treatment groups, respectively. Only the proportions of ALT normalization in inpatient and ambulatory treatment compared with usual care were significantly higher. Treatment effects of inpatient and ambulatory treatment were sustained at 1.5 years follow-up. No baseline characteristic, including PNPLA3 polymorphism or leptin, was consistently predictive for treatment response. Conclusions: A 6-month intensive inpatient and ambulatory lifestyle treatment in children with severe obesity reverses NAFLD in a minority of patients. This study suggests that inpatient compared with ambulatory intensive treatment does not importantly increase treatment success. Further efforts to optimize and individualize lifestyle interventions and additional treatments options are needed particular for children with severe obesity resistant to conventional lifestyle interventions.

Disclaimer/Complaints regulations If you believe that digital publication of certain material infringes any of your rights or (privacy) interests, please let the Library know, stating your reasons. In case of a legitimate complaint, the Library will make the material inaccessible and/or remove it from the website. Please Ask the Library: http://uba.uva.nl/en/contact, or a letter to: Library of the University of Amsterdam, Secretariat, Singel 425, 1012 WP Amsterdam, The Netherlands. You will be contacted as soon as possible.

MR elastography (MRE) can serve as an accurate surrogate marker of liver fibrosis. For any diagnostic test that is to replace the current reference standard, interobserver agreement should be at least as good and preferably better. The objective of this study was to perform a head-to-head comparison of the interobserver agreements of MRE and liver fibrosis staging on biopsy in a single cohort of hepatitis patients.

To compare the diagnostic accuracy of TE and MRE and establish cutoff levels and diagnostic strategies for both techniques, enabling selection of patients for liver biopsy.

SummaryWhat is already known about this subject Visceral fat accumulation is a risk factor for obesity‐related complications. Waist circumference is used in clinical practice to assess visceral adiposity. What this study adds Ultrasound is not superior to waist circumference for assessing visceral obesity in obese children. The optimal site for measuring waist circumference in obese children is at the smallest body circumference between xiphisternum and umbilicus. ObjectiveVisceral fat accumulation is a well‐established risk factor for obesity‐related complications. In children, it has not been determined whether ultrasonography is superior to waist measurement for assessing visceral fat. Moreover, the optimal site for waist measurement has not been determined.DesignIn a prospective cohort of 92 severely obese children and adolescents (age 13.9 ± 2.2 years, body mass index z‐score 3.29 ± 0.33), we evaluated the performance of ultrasonography and two different methods of waist circumference measurement, using magnetic resonance imaging as the reference standard.ResultsWaist circumference, defined as the smallest body circumference between xiphisternum and umbilicus had the strongest correlation with visceral fat quantity (r = 0.69 all, r = 0.68 girls, r = 0.64 boys). It was not outperformed by ultrasonography (r = 0.60 all, r = 0.62 girls, r = 0.50 boys) and correlated significantly better than the World Health Organization standard for waist measurement, midway between lower margin of the last rib and the crest of the ilium, (r = 0.51 all, r = 0.39 girls, r = 0.46 boys).ConclusionsWaist circumference measurement, defined as the smallest body circumference between xiphisternum and umbilicus, is the preferred non‐invasive technique for daily clinical practice to assess visceral fat accumulation in severely obese children and adolescents. There is no place for ultrasonography for the quantification of visceral fat in this group.