Background & Aims: Although transient elastography (TE) is used to determine liver stiffness as a surrogate to hepatic fibrosis, the normal range in children is not well defined. We performed a systematic review and individual participant data (IPD) | 2603 LI et aL. Funding Information None.

Currently, liver histology is the gold standard for the detection of liver fibrosis. In recent years, new methods such as transient elastography (TE) have been introduced into clinical practice, which allow a non-invasive assessment of liver fibrosis. The aim of the present study was to investigate the predictive value of TE for higher grade fibrosis and whether there is any relevance which histologic score is used for matching. For this purpose, we compared TE with 4 different histologic scores in pediatric patients with hepatopathies. Furthermore, we also determined the aspartate aminotransferase-to-platelet ratio (APRI) score, another non-invasive method, to investigate whether it is equally informative. Therefore, liver fibrosis in 75 children was evaluated by liver biopsy, TE and laboratory values. Liver biopsies were evaluated using four common histological scoring systems (Desmet, Metavir, Ishak and Chevalier’s semi-quantitative scoring system). The median age of the patients was 12.3 years. TE showed a good correlation to the degree of fibrosis severity independent of the histological scoring system used. The accuracy of the TE to distinguish between no/minimal fibrosis and severe fibrosis/cirrhosis was good (p = 0.001, AUC-ROCs > 0.81). The optimal cut-off value for the prediction of severe fibrosis was 10.6 kPa. In contrast, the APRI score in our collective showed no correlation to fibrosis.Conclusion: TE shows a good correlation to the histological findings in children with hepatopathy, independent of the used histological scoring system. What is Known:• The current gold standard for detecting liver fibrosis is liver biopsy. Novel non-invasive ultrasound-based methods are introduced to clinical diagnostics.• Most histological scores have been developed and evaluated in adult populations and for only one specific liver disease.What is New:• Transient elastography (TE) in children showed a good correlation to fibrosis severity irrespective of the utilized histological scoring system.• The aspartate aminotransferase-to-platelet ratio (APRI) showed no correlation with different stages of liver fibrosis in children.

Wilson’s disease (WD) is an autosomal recessive inherited disease in which a pathological storage of copper in various organs is the mean pathophysiological mechanism. The therapy consists of drug therapy with chelating agents or zinc. For patients, nutrition is always an important issue. The aim of this review was to determine whether there are clear recommendations for a low copper diet for WD patients, or whether the essential trace element zinc plays a role? We were able to show that some of the foods with high copper content would have to be consumed in such large quantities that this is regularly not the case. Furthermore, there are also different absorption rates depending on the copper content. A lower copper intake only prevents the re-accumulation of copper. In summary, consistent adherence to drug therapy is more important than a strict diet. Only two foods should be consistently avoided: Liver and Shellfish.

Objectives: The aim of the study was to investigate the efficacy and safety of trientine-dihydrochloride (TD) in pediatric patients with Wilson disease (WD) and the effect of different weight-based dosages on their clinical and biochemical outcome. Methods: We retrospectively reviewed the clinical data of 31 children with WD receiving TD therapy ages under 18 years at the time of diagnosis. Outcome measures included parameters of copper metabolism and liver function tests. To examine the impact of different weight-based dosages, 2 dosage subgroups were analyzed. Group 1 received less than 20 mg/kg TD per day, group 2 more than 20 mg · kg−1 · day−1. Results: Median follow-up was 60 (5–60) months in the total study group. During TD therapy, nonceruloplasmin-bound copper was reduced from mean 1.53 (0.01–6.95) at baseline to 0.62 (0.01–4.57) μmol/l. 24h-urinary copper excretion diminished to 1.85 (0.8–9.6) μmol/day approximating the therapeutic goal of 1.6 μmol/day. Seven of 31 patients (22.6%) required discontinuation of TD treatment, in 4 cases it was because of adverse events (ulcerative colitis, gingival and breast hypertrophy, hirsutism, elevation of transaminases). Investigations about weight-based dosage showed no significant difference of any laboratory parameter between the 2 cohorts. But in terms of clinical safety, adverse effects because of TD were only found in 6.7% of children in group 1 (<20 mg · kg−1 · day−1, median follow-up 60 [9–60] months), whereas in group 2 (>20 mg · kg−1 · day−1, median follow-up 60 [14–60] months), it was 63.6%. Conclusions: TD proves to be an efficacious alternative chelating agent for children with WD. Weight-based dosages above the recommended 20 mg · kg−1 · day−1 may increase the rate of adverse effects in pediatric patients.

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Continuous glucose monitoring (CGM) systems are increasingly implemented in patients with diabetes and other disorders with disturbed glucose homeostasis to promote good metabolic control.However, shortly after their appearance on the market, the first concerns about adverse skin reactions were raised. 1 CGM devices are usually combined with a subcutaneous catheter and a transmitter for blood glucose sensors applied to the skin by an adhesive patch for a period of 3-14 days. 2 Dermatological complications are especially common in children and adolescents using GCM devices. 3 Herman et al have recently identified isobornyl acrylate as the main culprit allergen to cause allergic contact dermatitis (ACD) in patients using the FreeStyle ® Libre device. 2 Interestingly, isobornyl acrylate was the American Contact Dermatitis Society Allergen of the Year in 2020. 4 We herein report on a girl with glycogen storage disease (GSD) type 9b who developed severe ACD to both FreeStyle ® Libre and Dexcom ® G6 devices. Our case highlights the severe consequences of sensitization for pediatric patients and the urgent need for open labeling as well as the use of less allergenic adhesives and components in medical devices.

Background Glycogen storage disease type Ib (GSD Ib) due to biallelic mutations in SLC37A4 is often associated with inflammatory bowel disease. Peritoneal inclusion cysts (PICs) are variable sized, fluid-filled, mesothelial-lined cysts that usually occur in premenopausal woman. Risk factors for the development of PICS comprise prior abdominal surgeries and inflammatory diseases. Results We herein report on a female patient with GSD Ib and Crohn’s like disease who underwent recurrent abdominal surgery due to suspected intraabdominal abscesses. At the age of 36, abdominal pain and an increase in the abdominal girth was observed. MRI revealed a large PIC. Due to multiple previous complications conservative management was chosen, and the cyst remained stable in size during a 1-year follow-up. Conclusions PIC should be included in the differential diagnosis of abdominal cystic masses especially in premenopausal women with either IBD or previous abdominal surgery. Radiologist, gynecologist, and gastroenterologist should be aware of this rare condition to prevent inappropriate and aggressive treatments.