Segmental MRI pituitary and hypothalamus volumes post Fontan: An analysis of the Australian and New Zealand Fontan registry.

Objective: Short stature, central hypothyroidism and infertility are common in those with a Fontan circulation. Given that the Fontan circulation often results in hepatic portal venous congestion, we hypothesize that the hypothalamic-pituitary portal circulation is also affected, contributing to subsequent hypothalamic-pituitary axis dysfunction.

Methods: MRI data from the Australian and New Zealand Fontan Registry (86 cases) was compared to 86 age- and sex-matched normal published controls.

Regional deprivation and diabetic ketoacidosis at type 1 diabetes diagnosis in children and adolescents: International comparison among 6 countries.

Introduction: The incidence of diabetic ketoacidosis (DKA) at the time of diagnosis of type 1 diabetes in youth varies between countries and is influenced by socioeconomic factors. We investigated the relationship between regional deprivation indices and DKA at diagnosis of type 1 diabetes in six countries.

Methods: We analyzed children 0.

Use of Freestyle Libre 2.0 in children with type 1 diabetes mellitus and elevated HbA: Extension phase results after a 12-week randomized controlled trial.

Aim: To investigate extension phase outcomes with intermittently scanned continuous glucose monitoring (isCGM 2.0) in children with type 1 diabetes mellitus (T1DM) and elevated HbA (7.5-12.

Parental perspectives following the implementation of advanced hybrid closed-loop therapy in children and adolescents with type 1 diabetes and elevated glycaemia.

Aims: To identify from a parental perspective facilitators and barriers of effective implementation of advanced hybrid closed-loop (AHCL) therapy in children and adolescents with type 1 diabetes (T1D) with elevated glycaemia.

Methods: Semi-structured interviews were conducted with parents of participants while in a post-trial extension phase of the CO-PILOT randomized controlled trial. The Capability, Opportunity, Motivation, Behaviour Model and Theoretical Domain Framework informed the interviews and framework analysis.

The phenotypic spectrum of syndromic optic atrophy associated with variants in : with reclassification of p.Val606Gly as a likely benign variant.

Introduction: Wolfram syndrome due to bi-allelic variants in and mono-allelic Wolfram-like syndrome have variable ocular and syndromic associations. In this report, eight patients are described.

Methods: A retrospective observational case series with detailed ophthalmic and systemic phenotyping, optical coherence tomography (OCT), and neuroimaging.

Mx1-ing it up-Mitochondrial relay for interferon-dependent, unconventional IL-1β release in SLE monocytes.

The role of type I interferon (IFN-I) in systemic lupus erythematosus (SLE) is well documented, but the role of interleukin (IL)-1β remains elusive. In this issue of Immunity, Caielli et al. identified an SLE monocyte population coproducing IL-1β and IFN-I and described how mitochondrial nucleic-acid-containing RBCs engage cGAS/STING, RIG-I, MDA5, and NLRP3 for unconventional IL-1β release.

Frequency and clinical characteristics of children and young people with type 2 diabetes at diagnosis from five world regions between 2012 and 2021: data from the SWEET Registry.

Aims/hypothesis: The diagnosis of type 2 diabetes is increasing in young people worldwide. This study evaluated the frequency and clinical characteristics of young people presenting with type 2 diabetes from the multinational SWEET e.V Registry 2012-2021, including the first years of the COVID-19 pandemic.

Automated Insulin Delivery for Young People with Type 1 Diabetes and Elevated A1c.

Background: Automated insulin delivery is the treatment of choice in adults with type 1 diabetes. Data are needed on the efficacy and safety of automated insulin delivery for children and youth with diabetes and elevated glycated hemoglobin levels.

Methods: In this multicenter, open-label randomized controlled trial, we assigned patients with type 1 diabetes in a 1:1 ratio either to use an automated insulin delivery system (MiniMed 780G) or to receive usual diabetes care of multiple daily injections or non--automated pump therapy (control).

Sexually dimorphic DNA methylation and gene expression patterns in human first trimester placenta.

Background: Fetal sex and placental development impact pregnancy outcomes and fetal-maternal health, but the critical timepoint of placenta establishment in first trimester is understudied in human pregnancies.

Methods: Pregnant subjects were recruited in late first trimester (weeks 10-14) at time of chorionic villus sampling, a prenatal diagnostic test. Leftover placenta tissue was collected and stored until birth outcomes were known, then DNA and RNA were isolated from singleton, normal karyotype pregnancies resulting in live births.

Emergent inequity of glycaemic metrics for Māori children with type 1 diabetes is negated by early use of continuous glucose monitoring.

Aim: We investigated if continuous glucose monitoring (CGM) in children with type 1 diabetes (T1D) within 12 months of being diagnosed modifies the development of glycaemic outcome inequity on the basis of either ethnicity or socio-economic status (SES).

Method: De-identified clinical and SES data from the KIWIDIAB data network were collected 12 months after diagnosis in children under 15 years diagnosed with T1D between 1 October 2020 and 1 October 2021.

Results: There were 206 children with new onset T1D: CGM use was 56.

Protocol for a prospective, multicenter, parallel-group, open-label randomized controlled trial comparing standard care with Closed lOoP In chiLdren and yOuth with Type 1 diabetes and high-risk glycemic control: the CO-PILOT trial.

Purpose: Advanced hybrid closed loop (AHCL) systems have the potential to improve glycemia and reduce burden for people with type 1 diabetes (T1D). Children and youth, who are at particular risk for out-of-target glycemia, may have the most to gain from AHCL. However, no randomized controlled trial (RCT) specifically targeting this age group with very high HbA has previously been attempted.

Children and adolescents with type 1 diabetes in Aotearoa New Zealand: An online survey of workforce and outcomes 2021.

Aims: To survey the national workforce that manages children and adolescents with type 1 diabetes (T1D) in Aotearoa New Zealand and compare with glycaemic outcomes for 2021.

Methods: A representative from each tertiary and regional diabetes service in Aotearoa New Zealand was asked to participate in an online survey assessing health-care professional (HCP) workforce numbers operating for the 2021 calendar year. Regional full-time-equivalent (FTE), glycaemic outcomes and population demographics were compared to a previously reported workforce surveys (2015 and 2019).

Alterations in genes associated with cytosolic RNA sensing in whole blood are associated with coronary microvascular disease in SLE.

Systemic lupus erythematosus (SLE) patients are 90% women and over three times more likely to die of cardiovascular disease than women in the general population. Chest pain with no obstructive cardiac disease is associated with coronary microvascular disease (CMD), where narrowing of the small blood vessels can lead to ischemia, and frequently reported by SLE patients. Using whole blood RNA samples, we asked whether gene signatures discriminate SLE patients with coronary microvascular dysfunction (CMD) on cardiac MRI (n=4) from those without (n=7) and whether any signaling pathway is linked to the underlying pathobiology of SLE CMD.

Revealing complex interdependencies in surgical instrument reprocessing using SEIPS 101 tools.

Sterile Processing Departments (SPDs) must clean, maintain, store, and organize surgical instruments which are then delivered to Operating Rooms (ORs) using a Courier Network, with regular coordination occurring across departmental boundaries. To represent these relationships, we utilized the Systems Engineering Initiative for Patient Safety (SEIPS) 101 Toolkit, which helps model how health-related outcomes are affected by healthcare work systems. Through observations and interviews which built on prior work system analyses, we developed a SEIPS 101 journey map, PETT scan, and tasks matrices to represent the instrument reprocessing work system, revealing complex interdependencies between the people, tools, and tasks occurring within it.

Distinct RBC alloantibody responses in type 1 interferon-dependent and -independent lupus mouse models.

During transfusion of red blood cells (RBCs), recipients are exposed to both ABO and non-ABO 'minor' antigens. RBC donor units and recipient RBCs are not routinely matched for non-ABO antigens. Thus, recipients are exposed to many RBC alloantigens that can lead to RBC alloantibody production and subsequent clinically significant hemolysis.

Comparing the glycaemic outcomes between real-time continuous glucose monitoring (rt-CGM) and intermittently scanned continuous glucose monitoring (isCGM) among adults and children with type 1 diabetes: A systematic review and meta-analysis of randomized controlled trials.

Aim: To conduct a systematic review and meta-analysis of randomized controlled trials (RCTs) comparing the effectiveness of real-time continuous glucose monitoring (rtCGM) versus intermittently scanned continuous glucose monitoring (isCGM) on key glycaemic metrics (co-primary outcomes HbA1c and time-in-range [TIR] 70-180 mg/dL, 3.9-10.0 mmol/L) among people with type 1 diabetes (T1D).

Exploring the Usability and Acceptability of a Well-Being App for Adolescents Living With Type 1 Diabetes: Qualitative Study.

Background: Adolescents living with either type 1 diabetes (T1D) or type 2 diabetes (T2D) have an increased risk of psychological disorders due to the demands of managing a chronic illness and the challenges of adolescence. Psychological disorders during adolescence increase the risk of suboptimal glycemic outcomes and may lead to serious diabetes-related complications. Research shows that digital health interventions may increase access to psychological support for adolescents and improve physical and mental health outcomes for youth with diabetes.

Interaction effects of significant risk factors on low bone mineral density in ankylosing spondylitis.

Background: To analyze individually and interactively critical risk factors, which are closely related to low bone mineral density (BMD) in patient with ankylosing spondylitis (AS).

Methods: A total of 249 AS patients who visited China-Japan Friendship Hospital were included in this training set. Patients with questionnaire data, blood samples, X-rays, and BMD were collected.

α-Ketoglutarate-Dependent KDM6 Histone Demethylases and Interferon-Stimulated Gene Expression in Lupus.

Objective: We aimed to investigate the hypothesis that interferon (IFN)-stimulated gene (ISG) expression in systemic lupus erythematosus (SLE) monocytes is linked to changes in metabolic reprogramming and epigenetic regulation of ISG expression.

Methods: Monocytes from healthy volunteers and patients with SLE at baseline or following IFNα treatment were analyzed by extracellular flux analysis, proteomics, metabolomics, chromatin immunoprecipitation, and gene expression. The histone demethylases KDM6A/B were inhibited using glycogen synthase kinase J4 (GSK-J4).

Effects of 12-Week Freestyle Libre 2.0 in Children with Type 1 Diabetes and Elevated HbA1c: A Multicenter Randomized Controlled Trial.

To investigate whether intermittently scanned continuous glucose monitoring (isCGM) reduced glycated hemoglobin (HbA1c) compared with capillary self-monitored capillary blood glucose (SMBG) in children with type 1 diabetes (T1D) and elevated glycemic control. This multicenter 12-week 1:1 randomized, controlled, parallel-arm trial included 100 participants with established T1D aged 4-13 years (mean 10.9 ± 2.

Reduced left ventricular function on cardiac MRI of SLE patients correlates with measures of disease activity and inflammation.

Background: Women with SLE have an elevated risk of cardiovascular disease. Many women with SLE frequently report chest pain in the absence of obstructive coronary artery disease (CAD) due to coronary microvascular dysfunction (CMD), a form of ischemia with no obstructive CAD. Echocardiographic studies have shown that SLE patients have reduced left ventricular (LV) function, which may also correlate with higher SLE disease activity scores.