Evaluation of Fontan failure by classifying the severity of Fontan-associated liver disease: a single-centre cross-sectional study.

Objectives: Fontan-associated liver disease (FALD) is a hallmark of the failing Fontan circulation, but no general classification of FALD severity exists. In this study, we propose a scoring system to grade the severity of FALD and analyse its applicability for evaluation of Fontan failure.

Methods: From 2017 to 2019, a total of 129 successive Fontan patients received a comprehensive hepatic assessment.

Severity of Fontan-Associated Liver Disease Correlates with Fontan Hemodynamics.

Fontan-palliated patients are at risk for the development of Fontan-associated liver disease (FALD). In this study, we performed a detailed hemodynamic and hepatic assessment to analyze the incidence and spectrum of FALD and its association with patients' hemodynamics. From 2017 to 2019, 145 patients underwent a detailed, age-adjusted hepatic examination including laboratory analysis (FibroTest, n = 101), liver ultrasound (n = 117) and transient elastography (FibroScan, n = 61).

Sonographic differentiation of complicated from uncomplicated appendicitis.

Objective: This study aims to differentiate acute uncomplicated and complicated appendicitis, by investigating the correlation between sonographic findings and histological results in different types of paediatric appendicitis.

Methods: This is a retrospective study of 1017 paediatric patients (age < 18 years) who underwent ultrasound by paediatric radiologists before appendicectomy at our institution between 2006 and 2016. Histologically, uncomplicated appendicitis was primarily associated with transmural infiltration of neutrophil granulocytes, while complicated appendicitis was characterised by transmural myonecrosis.

Intramural duodenal hematoma: clinical course and imaging findings.

Background: Intramural duodenal hematoma is a rare condition. Different imaging modalities are at hand for diagnosis.

Purpose: To identify patients with intramural duodenal hematoma and report imaging findings and clinical courses.

Homozygous missense and nonsense mutations in BMPR1B cause acromesomelic chondrodysplasia-type Grebe.

Acromesomelic chondrodysplasias (ACDs) are characterized by disproportionate shortening of the appendicular skeleton, predominantly affecting the middle (forearms and forelegs) and distal segments (hands and feet). Here, we present two consanguineous families with missense (c.157T>C, p.