Edvardsenherman2369

The aim of the study was to evaluate the role of cardiac magnetic resonance (CMR) mapping and strain analysis in the identification of cardiac involvement in idiopathic inflammatory myopathy (IIM) patients with preserved left ventricular ejection fraction.

In all, 38 IIM patients who underwent CMR examination at our institution were retrospectively included. Twenty-three age-matched healthy individuals served as controls. Mapping parameters including native T1, extracellular volume (ECV), and T2 mapping and strain parameters including global radial strain, global circumferential strain, and global longitudinal strain were measured semiautomatically using a dedicated processing software. All the mapping and strain values were compared between patients and controls.

Late gadolinium enhancement was only present in IIM patients (n=17, 44.7%). IIM patients showed higher native T1 (1346 vs. 1269 ms, P<0.001), ECV (31.1% vs. 27.4%, P<0.01), and higher T2 (44.4 vs. 39.2 ms, P<0.001) values compared with controls. The global radial strain (36.7% vs. 46.9%, P<0.001), global circumferential strain (-21.2% vs. -24.1%, P<0.01), and global longitudinal strain (-13.6% vs. -15.6%, P<0.05) values were significantly reduced compared with controls.

Native T1, ECV, T2 values, and global strain values may hold promise for the detection of subclinical myocardial involvement in IIM patients with preserved left ventricular ejection fraction.

Native T1, ECV, T2 values, and global strain values may hold promise for the detection of subclinical myocardial involvement in IIM patients with preserved left ventricular ejection fraction.

The relevance of methane measurement in breath tests for the detection of carbohydrate malabsorption in children is controversial. The need for correction for poor sample collection is disputed. We evaluated the relevance of methane/CO2 measurements for the diagnosis of paediatric carbohydrate malabsorption.

A total of 132 breath tests (fructose n = 54; lactose n = 78) were performed in 91 children/adolescents with functional abdominal complaints. Breath samples were collected and analysed for hydrogen, methane, and CO2. Malabsorption was defined by a net increase over baseline of ≥20 parts per million (ppm) for hydrogen, ≥5 to ≥12 ppm for methane, and ≥10 to ≥15 ppm for hydrogen-plus-methane. The diagnosis was made before and after the use of a CO2-based correction factor (5.5% as the numerator). Hydrogen-based test results were compared with results obtained with other cut-off values.

Fifty-eight positive tests were obtained by hydrogen measurement (without CO2 correction). The addition of methane meatients but did not significantly alter overall test results.

Caregivers of children with intestinal failure (IF) face difficult decisions without a clear best alternative. Providers assist in decision-making but often lack knowledge of caregiver perspectives. Using decision-making around anemia treatment as a focal point, we explored how caregivers of children with IF prefer to make decisions. Our goal was to offer insight to guide providers as they assist in decision-making.

We conducted 12 half-hour semistructured interviews with parents of children with IF. Interview questions addressed general decision-making and specifics of iron supplementation, including key factors and stakeholders in decision-making. Interviews were transcribed verbatim. Two investigators coded the transcripts and inductively derived themes.

Four themes were identified regarding decision-making. They involved the search for reliable, accurate, and positive information; the role of caretakers on the medical team; the relationships between caretakers and the medical team; and effective communication. Themes around anemia treatment included identification of anemia by bloodwork; proactive supplementation; individualized regimens; prioritizing safety and convenience.

Understanding caregiver perspectives regarding anemia treatment in pediatric IF identifies opportunities for systematic quality improvement.

Understanding caregiver perspectives regarding anemia treatment in pediatric IF identifies opportunities for systematic quality improvement.

Genetic susceptibility has been proposed as etiopathogenic in several pediatric liver diseases including autoimmune hepatitis (AIH). High throughput sequencing (HTPS) has been applied to archived needle liver biopsies obtained from adults but rarely to pediatric biopsies. For conclusive diagnosis of AIH, most subjects have an initial formalin-fixed paraffin embedded (FFPE) needle liver biopsy that is eventually archived and may be stored for decades.

Our goal was to develop methods to utilize tissue from archived needle liver biopsies for extraction of RNA sufficient to produce HTPS data.

We extracted total RNA from 45 FFPE needle liver biopsy samples (24 AIH type 1 patients and 21 controls [ages 15_11 and 19_10]; biopsy storage time 0.5-20 years) and constructed cDNA libraries that were then sequenced on an Illumina HiSeq2000 platform.

Forty (89%) of the libraries produced high-quality sequences for further analyses. The average number of sequences obtained per library from HTPS was 55,136,519 (range 14,914,291-184,027,499). There was a significant inverse relationship between the number of human reads obtained and the age of the specimen (P < 2_10_7). It was possible to classify more than 90% of the reads as known genes in samples that had been stored for less than 10 years.

Archived needle liver biopsies can be used for sequence based interrogation of the etiologic origins of complex liver diseases of young subjects, such as AIH.

Archived needle liver biopsies can be used for sequence based interrogation of the etiologic origins of complex liver diseases of young subjects, such as AIH.

Learning health systems (LHS) integrate research, improvement, management, and patient care, such that every child receives "the right care at the right time...every time," that is, evidence-based, personalized medicine. Evobrutinib molecular weight Here, we report our efforts to establish a sustainable, productive, multicenter LHS focused on pediatric liver transplantation.

The Starzl Network for Excellence in Pediatric Transplantation (SNEPT) is the first multicenter effort by pediatric liver transplant families and providers to develop shared priorities and a shared agenda for innovation in clinical care. This report outlines SNEPT's structure, accomplishments, and challenges as an LHS.

We prioritized 4 initial projects immunosuppression, perioperative anticoagulation, quality of life, and transition of care. We shared center protocols/management to identify areas of practice variability between centers. We prioritized actionable items that address barriers to providing "the right care at the right time" to every pediatric liver transplant recipient facilitating transparency of practice variation and the connection of practices to patient outcomes, harnessing existing datasets to reduce the burden of tracking outcomes, incorporating patient-reported outcomes into outcome metrics, and accelerating the implementation of knowledge into clinical practice.