Identifying Frailty and Its Risk Factors in Pediatric Patients with Fontan Physiology.

Frailty is a clinical syndrome common in adults with chronic disease with resultant vulnerability to adverse health outcomes. Little is known about frailty in pediatric patients, including those with single-ventricle heart disease. This study aimed to examine the prevalence of frailty and its associated risk factors in patients with Fontan circulation.

Psychological functioning in paediatric patients with single ventricle heart disease: a systematic review.

Background: Patients with single ventricle heart disease are living into adulthood due to medical and surgical advancements but have significant physical comorbidities and an increased risk for psychological comorbidities compared to healthy subjects or those with other CHD diagnoses. This study aimed to systematically review psychological functioning in paediatric single ventricle heart disease.

Methods: Literature was searched using PubMed, Embase, PsycInfo, CINAHL Complete and Scopus.

Vascular dissection in women with Turner syndrome.

Background: The frequency of ascending aortic dissection in patients with Turner syndrome in the United States remains largely unknown with data surmised from published case reports or case series. Dissection of other vascular structures has only rarely been reported in this patient cohort. Recent European data identified aortic dissection to be a relatively rare event in a group of adult women with Turner syndrome.

Clinical and Echocardiographic Prevalence and Detection of Congenital and Acquired Cardiac Abnormalities in Girls and Women with the Turner Syndrome.

The prevalence of congenital and acquired heart disease in patients with the Turner syndrome (TS) is based on historic cohorts who underwent imaging before the advent of modern day echocardiography. Recent small studies suggest a higher prevalence of cardiac defects. We reviewed clinical and echocardiographic data on 564 girls and women with TS to assess the prevalence of cardiac defects.

Skin cancer screening in the homeless population.

The homeless population is a medically vulnerable patient population in our communities. Of particular concern to dermatologists is the risk for the development of skin cancer in the homeless, in light of their chronic sun exposure and suboptimal sun protection behaviors. Two free skin cancer screenings conducted at the Fourth Street Clinic in Salt Lake City, Utah, in 2011 and 2012, resulted in the diagnosis of 13 skin cancers in 62 patients.

Familial melanoma-associated mutations in p16 uncouple its tumor-suppressor functions.

Familial melanoma is associated with point mutations in the cyclin-dependent kinase (CDK) inhibitor p16(INK4A) (p16). We recently reported that p16 regulates intracellular oxidative stress in a cell cycle-independent manner. Here we constructed 12 different familial melanoma-associated point mutants spanning the p16 coding region and analyzed their capacity to regulate cell cycle phase and suppress reactive oxygen species (ROS).