Long-Term Safety and Efficacy of Transcatheter Microwave and Radiofrequency Denervation in a Chronic Ovine Model.

Background: Transcatheter renal denervation (RDN) has had inconsistent efficacy and concerns for durability of denervation. We aimed to investigate long-term safety and efficacy of transcatheter microwave RDN in vivo in normotensive sheep in comparison to conventional radiofrequency ablation.

Methods And Results: Sheep underwent bilateral RDN, receiving 1 to 2 microwave ablations (maximum power of 80-120 W for 240 s-480 s) and 12 to 16 radiofrequency ablations (180 s-240 s) in the main renal artery in a paired fashion, alternating the side of treatment, euthanized at 2 weeks (acute N=15) or 5.

Developmental delay and non-phenylketonuria (PKU) hyperphenylalaninemia in DNAJC12 deficiency: Case and approach.

Background: Hyperphenylalaninemia is a biomarker for several monogenic neurotransmitter disorders where the body cannot metabolise phenylalanine to tyrosine. Biallelic pathogenic variants in DNAJC12, co-chaperone of phenylalanine, tyrosine, and tryptophan hydroxylases, leads to hyperphenylalaninemia and biogenic amines deficiency.

Methods And Results: A male firstborn to non-consanguineous Sudanese parents had hyperphenylalaninemia 247 µmol/L [reference interval (RI) < 200 µmol/L] at newborn screening.

COVID-19 and Dr. Google: Parents' changing experience using online health information about their children's health during the pandemic.

Aims: To identify how the COVID-19 pandemic influences parents' use of the internet, including social media, when seeking health-related information about the pandemic relevant to their children.

Methods: This study employed semi-structured interviews to explore the factors affecting parents of young children when information-seeking online about their children's health related to the COVID-19 pandemic. Parents of children with and without chronic health conditions were interviewed in July and August 2020.

Beyond Refeeding: The Effect of Including a Dietitian in Eating Disorder Treatment. A Systematic Review.

Eating disorders are potentially life-threatening mental health disorders that require management by a multidisciplinary team including medical, psychological and dietetic specialties. This review systematically evaluated the available literature to determine the effect of including a dietitian in outpatient eating disorder (ED) treatment, and to contribute to the understanding of a dietitian's role in ED treatment. Six databases and Google Scholar were searched for articles that compared treatment outcomes for individuals receiving specialist dietetic treatment with outcomes for those receiving any comparative treatment.

Correlation of ventricular septal defect height and outcomes after complete atrioventricular septal defect repair.

Objectives: There are limited data available on the height of the ventricular component of the septal deficiency (VSD) in patients undergoing complete atrioventricular septal defect (CAVSD) repair. VSD height may influence optimal choice of repair strategy with potential consequences for long-term outcomes. We aimed to measure VSD height using 2-dimensional echocardiography and review its association with postoperative outcomes.

Cerebrospinal fluid neopterin as a biomarker of treatment response to Janus kinase inhibition in Aicardi-Goutières syndrome.

Janus kinase (JAK) 1 inhibition represents a precision medicine approach in the treatment of Aicardi-Goutières syndrome (AGS), through targeting of type I interferon-mediated cell signalling. Blood interferon mRNAseq has been proposed as a biomarker of disease with utility in therapeutic monitoring. Objective cerebrospinal fluid (CSF) biomarkers tracking treatment efficacy are currently lacking.

Balance impairment in pediatric charcot-marie-tooth disease.

Introduction: Balance impairment contributes to gait dysfunction, falls, and reduced quality of life in adults with Charcot-Marie-Tooth disease (CMT) but has been minimally examined in pediatric CMT.

Methods: The CMT Pediatric Scale (CMTPedS) was administered to 520 children with CMT. Associations between balance function (Bruininks-Oseretsky Test of Motor Proficiency [BOT-2]) and sensorimotor and gait impairments were investigated.

Prevalence and orthopedic management of foot and ankle deformities in Charcot-Marie-Tooth disease.

Introduction: Foot deformities are frequent complications in Charcot-Marie-Tooth disease (CMT) patients, often requiring orthopedic surgery. However, there are no prospective, randomized studies on surgical management, and there is variation in the approaches among centers both within and between countries.

Methods: In this study we assessed the frequency of foot deformities and surgery among patients recruited into the Inherited Neuropathies Consortium (INC).

Efficacy and effectiveness of anti-digoxin antibodies in chronic digoxin poisonings from the DORA study (ATOM-1).

Context: We hypothesized that in chronic digoxin toxicity, anti-digoxin antibodies (Fab) would be efficacious in binding digoxin, but this may not translate into improved clinical outcomes.

Objective: This study aims to investigate changes in free digoxin concentrations and clinical effects on heart rate and potassium concentrations in chronic digoxin poisoning when anti-digoxin Fab are given.

Materials And Methods: This is a prospective observational study.

Phenotypic Variability of Childhood Charcot-Marie-Tooth Disease.

Importance: Disease severity of childhood Charcot-Marie-Tooth disease (CMT) has not been extensively characterized, either within or between types of CMT to date.

Objective: To assess the variability of disease severity in a large cohort of children and adolescents with CMT.

Design, Setting, And Participants: A cross-sectional study was conducted among 520 children and adolescents aged 3 to 20 years at 8 universities and hospitals involved in the Inherited Neuropathies Consortium between August 6, 2009, and July 31, 2014, in Australia, Italy, the United Kingdom, and the United States.

A pilot randomized controlled trial of a post-discharge program to support emerging adults with type 1 diabetes mellitus transition from pediatric to adult care.

Aims: There is a paucity of randomized controlled trials (RCT) examining transition from pediatric to adult care in type 1 diabetes mellitus (T1DM). This study aimed to determine if transition in T1DM is more effective with a comprehensive transition program (CTP) compared with standard clinical practice (SCP).

Methods: This RCT recruited as young people left pediatric diabetes services.

Transition to adult care in adolescent obesity: a systematic review and why it is a neglected topic.

Transition in pediatric health care involves the purposeful, planned movement of patients from pediatric to adult services. Following the significant increases in long-term survival of chronic childhood diseases in the 1980s, transition has taken on an increasing importance in the management of these chronic diseases. In Australia, there is a conspicuous lack of programs/guidelines for transitioning adolescents with obesity.