Associations Between Exercise Capacity and Psychological Functioning in Children and Adolescents with Fontan Circulation.

Individuals with Fontan circulation (FC) often have diminished exercise capacity and are at risk for psychological problems. The current study examines associations between exercise capacity and psychological functioning in children and adolescents with FC. A multidisciplinary team evaluated participants in a clinic for pediatric patients with FC.

Creating a digital approach for promoting physical activity in pediatric pulmonary hypertension: A framework for future interventions.

Children with pulmonary hypertension (PH) often demonstrate limited exercise capacity. Data support exercise as an effective nonpharmacologic intervention among adults with PH. However, data on exercise training in children and adolescents are limited, and characteristics of the optimal exercise program in pediatric PH have not been identified.

Cardiopulmonary exercise testing on adaptive equipment in children and adults with Friedreich ataxia.

Introduction/aims: Traditional exercise is often difficult for individuals with Friedreich ataxia (FRDA), and evidence is limited regarding how to measure exercise performance in this population. We evaluated the feasibility, reliability, and natural history of adaptive cardiopulmonary exercise test (CPET) performance in children and adults with FRDA.

Methods: Participants underwent CPET on either an arm cycle ergometer (ACE) or recumbent leg cycle ergometer (RLCE) at up to four visits (baseline, 2 weeks, 4 weeks, and 1 year).

Harnessing Patient Life Stories to Engage Medical Trainees in Strengthening Veteran-Provider Relationships.

Introduction: Medical trainees do not have many opportunities to develop communication skills with patients. We established the voluntary "My Life, My Story" (MLMS) program at the Clement J. Zablocki VAMC in Milwaukee, WI, to determine if this pilot narrative medicine program enhanced trainee interpersonal skills and improved patient-centered care.

The First Sign of Recurrent Angioimmunoblastic T-Cell Lymphoma: A Cutaneous Presentation.

Angioimmunoblastic T-cell lymphoma (AITL) is a subtype of peripheral T-cell lymphoma with a nonspecific clinical presentation. Cutaneous manifestations of AITL are variable and include morbilliform eruptions, urticaria, papulonodules, and erythroderma. We present the case of a 74-year-old male with a medical history of AITL presenting with diffuse erythematous macules and papules coalescing into patches and plaques on the trunk and bilateral upper extremities.

The Fontan Udenafil Exercise Longitudinal Trial: Subgroup Analysis.

The Pediatric Heart Network's Fontan Udenafil Exercise Longitudinal (FUEL) Trial (Mezzion Pharma Co. Ltd., NCT02741115) demonstrated improvements in some measures of exercise capacity and in the myocardial performance index following 6 months of treatment with udenafil (87.

A Rare Case of Cutaneous Metastasis of Renal Cell Carcinoma to the Lateral Thigh.

Renal cell carcinoma (RCC) is a common cancer type in the United States, and at the time of diagnosis, many patients already have metastatic disease. RCC typically metastasizes to the lungs, liver, and bones, with few cases manifesting cutaneous metastasis. Most incidences of RCC metastases reported in the literature have been on the face and scalp.

Utility of Gene Expression Profiling in Skin Cancer: A Comprehensive Review.

Understanding the metastatic potential of a skin cancer is essential to effective management. Gene expression profiling (GEP) is an innovative technology that has allowed for a better understanding of tumor biology in various skin cancers. Current methods focus on identifying and quantifying ribonucleic acid (RNA) transcripts in tissue samples.

Otoplasty: The Belfast Experience. A 10-Year Review of 2333 Ear Outcomes.

Background: "Prominent ear" remains one of the most common referrals to pediatric plastic surgery. The perceived deformity has been corrected using a multitude of techniques for over a century, and significant variation in practice still exists. Recent studies suggesting that cartilage-scoring techniques are associated with more major complications than suture techniques may have led to an adverse international perception of the technique.

Peak Work Rate Increases With Lower Extremity-Focused Exercise Training in Adolescents With Fontan Circulation.

Background Skeletal muscle deficits are associated with worse exercise performance in the Fontan circulation and may be improved by exercise training. We aimed to assess the change in leg lean mass (a marker of skeletal muscle), exercise performance, and functional health status after a lower extremity-focused exercise intervention in adolescents with Fontan circulation. Methods and Results Densitometry for measurement of leg lean mass, cardiopulmonary exercise test, exercise cardiac magnetic resonance, peripheral vascular testing, physical activity questionnaire, and quality of life assessment were performed at baseline and after a 24-week, hybrid center- and home-based training program.

Microsurgical Salvage of Neonatal Upper Limb Ischaemia Following Intrauterine Brachial Vessel Constriction.

Neonatal limb ischaemia is a rare disease entity with devastating morbidity, including compartment syndrome, tissue loss, limb loss, reduced limb growth, irreparable neuropathies and Volkmann's syndrome.  We report a case of limb revascularisation and salvage due to intrauterine brachial artery thrombosis. Published literature is limited to case reports and case series, with various treatment modalities discussed.

Skeletal muscle deficits are associated with worse exercise performance in pediatric pulmonary hypertension.

Background: Skeletal muscle deficits are associated with worse exercise performance in adults with pulmonary hypertension (PH) but the impact is poorly understood in pediatric PH.

Objective: To study muscle deficits, physical inactivity, and performance on cardiopulmonary exercise test (CPET) and exercise cardiac magnetic resonance (eCMR) in pediatric PH.

Methods: Youth 8-18 years participated in a prospective, cross-sectional study including densitometry (DXA) for measurement of leg lean mass Z-score (LLMZ), handheld dynamometer with generation of dominant and non-dominant handgrip Z-scores, Physical Activity Questionnaire (PAQ), CPET, and optional eCMR.

Cardiopulmonary Exercise Testing in Pediatric Patients With Hypertrophic Cardiomyopathy.

Background: Exercise stress testing (EST) in pediatric hypertrophic cardiomyopathy (HCM) patients has not well described in a large heterogenous cohort.

Objectives: The objective of the study was to determine the clinical utility of EST in pediatric HCM.

Methods: This was a retrospective single-center analysis of HCM patients younger than 21 years who had EST between January 1, 2000, and January 1, 2019.

A Single Stage Composite Cleft Septorhinoplasty for Correction of the Mature Unilateral Cleft Nose Deformity - The Gujrat Technique.

Objective: To evaluate the results of a single stage composite cleft septorhinoplasty procedure ("The Gujrat Technique") to correct the exaggerated cleft nose deformity after completion of nasal growth in an adult patient cohort.

Methods: Adult patients with a residual unilateral cleft nasal deformity were deemed eligible for the proposed "Gujrat Technique". Over a 10-year period (2007-2017), 96 adult patients underwent this composite cleft septorhinoplasty as a single stage operation.

Lower limb injuries in an english professional football club: injury analysis and recommendations for prevention.

Background: Lower limb injuries constitute a problem in the sport of football. Our aim is to explore patterns and trends of lower limb injuries in an English professional football club.

Methods: This is a descriptive epidemiological study.

Safety and Feasibility of Exercise Rehabilitation in Children with Ventricular Assist Devices.

Children with advanced heart failure may require ventricular assist devices (VAD) while awaiting heart transplantation. Currently, no data exist regarding the safety of exercise rehabilitation (ER) in children on VAD support. The purpose of this study was to determine the safety and feasibility of ER in children on VAD support awaiting heart transplantation.

Observation of Aerosol Generation by Human Subjects During Cardiopulmonary Exercise Testing Using a High-Powered Laser Technique: A Pilot Project.

Purpose: Human respiratory aerosols may have important implications for transmission of pathogens. The study of aerosol production during vigorous breathing activities such as exercise is limited. In particular, data on aerosol production during cardiopulmonary exercise testing (CPET) are lacking.

Development of a Mitochondrial Myopathy-Composite Assessment Tool.

Background: 'Mitochondrial Myopathy' (MM) refers to genetically-confirmed Primary Mitochondrial Disease (PMD) that predominantly impairs skeletal muscle function. Validated outcome measures encompassing core MM domains of muscle weakness, muscle fatigue, imbalance, impaired dexterity, and exercise intolerance do not exist. The goal of this study was to validate clinically-meaningful, quantitative outcome measures specific to MM.

Throat Packs in Cleft and Craniofacial Surgery.

Throat packs are used in a wide range of surgical and dental procedures. Indications for use include; airway stabilization, reducing the risk of aspiration, and postoperative nausea and vomiting. Despite these perceived benefits, a retained throat pack can be fatal.

A review of functional latissimus dorsi transfers for absent elbow flexion and supination.

Aims: To review patients treated with a functional latissimus dorsi flap for congenital and acquired elbow flexion deficits.

Methods: Retrospective review of functional latissimus dorsi flaps performed in one regional unit. Patient notes were reviewed to determine aetiology, pre-op deficits and function, surgical technique, complications and outcomes.

Denture Hygiene as It Relates to Denture Stomatitis: A Review.

The wear of dentures carries with it the possibility of an inflammatory response by the denture-bearing tissues. Lack of or improper hygiene has been shown to contribute to denture stomatitis (DS). Although essential, denture hygiene is often overlooked by patients or performed improperly.