Nonalcoholic Fatty Liver Disease and the Heart: JACC State-of-the-Art Review

Nonalcoholic fatty liver disease (NAFLD) and cardiovascular disease (CVD) are both manifestations of end-organ damage of the metabolic syndrome. Through multiple pathophysiological mechanisms, CVD and NAFLD are associated with each other. Systemic inflammation, endothelial dysfunction, hepatic insulin resistance, oxidative stress, and altered lipid metabolism are some of the mechanisms by which NAFLD increases the risk of CVD. Patients with NAFLD develop increased atherosclerosis, cardiomyopathy, and arrhythmia, which clinically result in cardiovascular morbidity and mortality. Defining the mechanisms linking these 2 diseases offers the opportunity to further develop targeted therapies. The aim of this comprehensive review is to examine the association between CVD and NAFLD and discuss the overlapping management approaches.     

Capillary leak syndrome: A rare cause of acute respiratory distress syndrome

Capillary leak syndrome (CLS) is a rare clinical syndrome associated with significant morbidity and mortality. Intensive care and supportive therapy constitute the mainstay of the treatment, along with judicious use of crystalloids and colloids such as dextran and starch during the leak phase. The advantages of proning, steroids, and intravenous immunoglobins are worth contemplating in patients with such a presentation. Extracorporeal membrane oxygenation appears to be an excellent strategy to surmount the impediments of the leak and post leak phase of CLS, especially in patients with severe or refractory hypoxemia.     

The Diagnosis and Management of Hyperinsulinaemic Hypoglycaemia

Insulin secretion from pancreatic β-cells is tightly regulated to keep fasting blood glucose concentrations within the normal range (3.5-5.5 mmol/L). Hyperinsulinaemic hypoglycaemia (HH) is a heterozygous condition in which insulin secretion becomes unregulated and its production persists despite low blood glucose levels. It is the most common cause of severe and persistent hypoglycaemia in neonates and children. The most severe and permanent forms are due to congenital hyperinsulinism (CHI). Recent advances in genetics have linked CHI to mutations in 9 genes that play a key role in regulating insulin secretion (ABCC8, KCNJ11, GLUD1, GCK, HADH, SLC16A1, UCP2, HNF4A and HNF1A). Histologically, CHI can be divided into 3 types; diffuse, focal and atypical. Given the biochemical nature of HH (non-ketotic), a delay in the diagnosis and management can result in irreversible brain damage. Therefore, it is essential to diagnose and treat HH promptly. Advances in molecular genetics, imaging methods (18F-DOPA PET-CT), medical therapy and surgical approach (laparoscopic surgery) have completely changed the management and improved the outcome of these children. This review provides an overview of the genetic and molecular mechanisms leading to development of HH in children. The article summarizes the current diagnostic methods and management strategies for the different types of CHI.     

Do Insurance Mandates Affect Racial Disparities in Outcomes for Children with Autism?

Objective The study investigated whether state mandates for private insurers to provide services for children with autism influence racial disparities in outcomes. Methods The study used 2005/2006 and 2009/2010 waves of the National Survey of Children with Special Health Care Needs. Children with a current diagnosis of autism were included in the sample. Children residing in 14 states and the District of Columbia that were not covered by the mandate in the 2005/2006 survey, but were covered in the 2009/2010 survey, served as the mandate group. Children residing in 32 states that were not covered by a mandate in either wave served as the comparison group. Outcome measures assessed included care quality, family economics, and child health. A difference-in-difference-in-differences (DDD) approach was used to assess the impact of the mandates on racial disparities in outcomes. Results Non-white children had less access to family-centered care compared to white children in both waves of data, but this difference was not apparent across mandate and comparison states as only the comparison states had significant differences. Parents of non-white children reported paying less in annual out-of-pocket expenses compared to parents of white children across waves and groups. DDD estimates did not provide evidence that the mandates had statistically significant effects on improving or worsening racial disparities for any outcome measure. Conclusions This study did not find evidence that state mandates on private insurers affected racial disparities in outcomes for children with autism.     

The Diagnosis of Delirium Superimposed on Dementia: An Emerging Challenge

Delirium occurring in patients with dementia is referred to as delirium superimposed on dementia (DSD). People who are older with dementia and who are institutionalized are at increased risk of developing delirium when hospitalized. In addition, their prior cognitive impairment makes detecting their delirium a challenge. The Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition and the International Statistical Classification of Diseases and Related Health Problems, 10th Revision are considered the standard reference for the diagnosis of delirium and include criteria of impairments in cognitive processes such as attention, additional cognitive disturbances, or altered level of arousal. The Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition and the International Statistical Classification of Diseases and Related Health Problems, 10th Revision does not provide guidance regarding specific tests for assessment of the cognitive process impaired in delirium. Importantly, the assessment or inclusion of preexisting cognitive impairment is also not addressed by these standards. The challenge of DSD gets more complex as types of dementia, particularly dementia with Lewy bodies, which has features of both delirium and dementia, are considered. The objective of this article is to critically review key elements for the diagnosis of DSD, including the challenge of neuropsychological assessment in patients with dementia and the influence of particular tests used to diagnose DSD. To address the challenges of DSD diagnosis, we present a framework for guiding the focus of future research efforts to develop a reliable reference standard to diagnose DSD. A key feature of a reliable reference standard will improve the ability to clinically diagnose DSD in facility-based patients and research studies.