Low skeletal muscle mass and liver fibrosis in children with cerebral palsy.

The purpose of the study was to conduct a nutritional and metabolic assessment of children with cerebral palsy, including an investigation of liver status, body composition, and bone mineral density. In this cross-sectional study we included 22 children with cerebral palsy. By using ultrasound, transient elastography, dual x-ray absorptiometry (DXA) scan, blood samples, anthropometric measurements, and a three-day diet registration, the nutritional and metabolic status was evaluated. Liver fibrosis and steatosis were found in four patients (18.2%), all with severe motor impairments, low skeletal muscle mass, and epilepsy. All patients with liver involvement had normal liver-related blood samples. Decreased bone mineral density was found in 26.3%, and 91.0% had low skeletal muscle mass. Fat mass and muscle mass were significantly lower in the patients with severe motor impairments compared to the patients with less severe motor impairments. Within the children classified as 'underweight' or 'normal' according to body mass index, body fat determined by DXA scan was normal or high in 50% of these patients. CONCLUSIONS: This study is the first to report liver fibrosis and steatosis in children with cerebral palsy. Possible causes of liver fibrosis and/or steatosis are altered body composition with low skeletal muscle mass, decreased mobility and medical drug intake. Further investigations of liver involvement and risk factors are needed. WHAT IS KNOWN: • Children and adolescents with cerebral palsy are at risk of malnutrition and altered body composition, both of which can lead to fatty liver disease. • It is unknown whether children with cerebral palsy are at increased risk of metabolic disturbances such as fatty liver disease. WHAT IS NEW: • Altered body composition and low skeletal muscle mass, regardless of ambulation is present in 91% of the children with cerebral palsy. • Liver fibrosis and/or steatosis were found in 18.2% of the patients. Possible causes are altered body composition, decreased mobility and medical drug intake.

Metabolic assessment in children with neuromuscular disorders shows risk of liver enlargement, steatosis and fibrosis.

AIM: The aim of this study was to conduct a metabolic and nutritional assessment of children with neuromuscular disorders, including the investigation of the liver and bone mineral density. METHODS: In this observational study, we included 44 children with neuromuscular disorders. The nutritional status, bone health and liver were assessed by ultrasound, transient elastography, dual X-ray absorptiometry scan, blood samples, anthropometric measurements and 3-day diet registration. RESULTS: Liver involvement was found in 31.0%: liver enlargement in 7.1%, steatosis in 4.8%, fibrosis in 14.3% and liver enlargement together with steatosis or fibrosis was found in 4.8%. These changes were found in 9/23 patients with Duchenne muscular dystrophy, 4/9 patients with spinal muscular atrophy type II and 0/12 patients with other neuromuscular diagnoses. Low bone mineral density was found in 44.0% of the patients, though the majority used daily vitamin D and calcium supplements. Vitamin D insufficiency or deficiency was found in 22.6%. CONCLUSION: The metabolic assessment in children with neuromuscular disorders shows an increased risk of liver enlargement, steatosis and fibrosis. Possible causes are obesity, decreased mobility, low skeletal muscle mass and for a subgroup the use of glucocorticoids. The findings suggest that monitoring liver function should be part of the nutritional assessment in patients with neuromuscular disorders.

Inter- and Intra-Observer Agreement When Using a Diagnostic Labeling Scheme for Annotating Findings on Chest X-rays-An Early Step in the Development of a Deep Learning-Based Decision Support System.

Consistent annotation of data is a prerequisite for the successful training and testing of artificial intelligence-based decision support systems in radiology. This can be obtained by standardizing terminology when annotating diagnostic images. The purpose of this study was to evaluate the annotation consistency among radiologists when using a novel diagnostic labeling scheme for chest X-rays. Six radiologists with experience ranging from one to sixteen years, annotated a set of 100 fully anonymized chest X-rays. The blinded radiologists annotated on two separate occasions. Statistical analyses were done using Randolph's kappa and PABAK, and the proportions of specific agreements were calculated. Fair-to-excellent agreement was found for all labels among the annotators (Randolph's Kappa, 0.40-0.99). The PABAK ranged from 0.12 to 1 for the two-reader inter-rater agreement and 0.26 to 1 for the intra-rater agreement. Descriptive and broad labels achieved the highest proportion of positive agreement in both the inter- and intra-reader analyses. Annotating findings with specific, interpretive labels were found to be difficult for less experienced radiologists. Annotating images with descriptive labels may increase agreement between radiologists with different experience levels compared to annotation with interpretive labels.

Ensuring competence in ultrasound-guided procedures-a validity study of a newly developed assessment tool.

OBJECTIVES: To investigate the validity of the Interventional Ultrasound Skills Evaluation (IUSE) tool for assessment of procedural competence in ultrasound-guided procedures in a clinical environment, including a pass/fail score. METHODS: Novices and experienced radiologists were recruited from four hospitals and were observed and assessed while performing ultrasound-guided procedures. Performances were assessed using the IUSE tool by two independent raters. Validity evidence was gathered in accordance with Messick's framework: response process was ensured by standardisation of written rater instructions. Internal structure was explored using Cronbach's alpha for internal consistency reliability; inter-rater reliability was calculated as Pearson's r independently across all ratings, and test-retest reliability was reported using Cronbach's alpha. Relationship to other variables was investigated by comparing performances of the participants in each group. Consequences evidence was explored by calculating a pass/fail standard using the contrasting groups method. RESULTS: Six novices and twelve experienced radiologists were enrolled. The IUSE tool had high internal consistency (Cronbach's alpha = 0.96, high inter-rater reliability (Pearson's r = 0.95), and high test-retest reliability (Cronbach's alpha = 0.98), and the mean score was 33.28 for novices and 59.25 for experienced with a highly significant difference (p value < 0.001). The pass/fail score was set at 55 resulting in no false positives or false negatives. CONCLUSIONS: Validity evidence from multiple sources supports the use of the IUSE tool for assessment of competence in ultrasound-guided procedures in a clinical environment and its use in high-stakes assessment such as certification. A credible pass/fail criterion was established to inform decision-making. KEY POINTS: • A multi-site validity investigation established that the Interventional Ultrasound Skills Evaluation (IUSE) tool can be used to assess procedural competence in ultrasound-guided procedures. • Validity evidence was gathered according to Messick's framework validity from the following sources: response process, internal structure, relationship to other variables, and consequences evidence. • The IUSE tool can be used for both formative and summative assessment, and a credible pass/fail score was established to help inform decision-making such as certification.

Rapidly progressing HELLP syndrome and massive liver destruction in a 22-year-old female pregnant with monochorionic diamniotic gemelli.

Haemolysis Elevated Liver parameters and Low Platelets (HELLP) syndrome can present with abdominal pain as the only symptom. It can be rapid with failure of multiple organs and is a cause of maternal death. A 22-year-old female pregnant with twins contacted her local hospital due to abdominal pain. Within 20 hours of the debut of the abdominal pain, a caesarean section was performed, the patient was re-operated due to the suspicion of abdominal bleeding. Bescause she was hypotensive and blood tests showed signs of liver destruction and acidosis, she was transferred to the intensive care unit (ICU). During an admission of 40 days, the patient received 5 plasmapheresis treatments and 10 courses of haemodialysis. CT scans showed massive liver haematomas intraparenchymal and subcapsular. The CT scan images are very interesting and included in the case report.This case presents a good prognosis after a rapid progression of HELLP in spite of a dramatic clinical presentation. This topic is of great interest to a wide spectre of clinicians, as an early multidisciplinary approach is necessary.

Role of CT angiography in bilateral pulmonary sequestration: a case report.

Bilateral pulmonary sequestration (PS) is a very rare congenital malformation. We describe a case of bilateral intralobar pulmonary sequestration (ILS) in a newborn. Both sequestrations received arterial supply from separate branches of the descending aorta and venous drainage was into ipsilateral inferior pulmonary veins. Prenatal ultrasonography showed cystic changes in the lungs. Computed tomography angiography (CTA) with supplemental two-dimensional (2D) and three-dimensional (3D) images was performed to clearly define the pathology and revealed bilateral intralobar pulmonary sequestration with aberrant blood supply. The child underwent successful video-assisted thoracoscopic surgical (VATS) lobectomy on the left side and thoracoscopic wedge resection on the right side. There were no complications. CTA with supplemental 2D and 3D images plays a vital role in revealing the exact pathology in congenital pulmonary malformations associated with anomalous vasculature.