Transitioning Adolescents and Young Adults with Lipid Disorders to Adult Health Care.

PURPOSE OF REVIEW: Pediatric healthcare providers have increasingly become aware of the need for timely and informative transition of adolescents and young adults with chronic medical conditions such as diabetes and cystic fibrosis. However, there is paucity of published data on the importance of and most effective way to transition youth with lipid disorders who are at increased risk of premature cardiovascular disease. RECENT FINDINGS: Evidence shows that atherosclerosis begins at a young age. However, there are no guidelines on the transition of adolescents and young adults with dyslipidemia. In addition, there are conflicting guidelines for lipid management in children versus adults, despite advances in medical pharmacotherapies for dyslipidemia. The lack of guidelines for transition and discordant recommendations for management of this vulnerable population places young adults at-risk for worsening of their underlying disease, and premature cardiovascular events.

Heart Transplant, Kawasaki Disease, and Bone Marrow Transplant: Are There Consequences?

PURPOSE OF REVIEW: This article reviews the current landscape of cardiovascular disease (CVD) risk factors, focusing on dyslipidemia, which contribute to atherosclerosis in three unique populations: youth less than 18 years-of-age with a history of Kawasaki disease, and those who have undergone orthotopic heart and bone marrow transplants. RECENT FINDINGS: Atherosclerosis, the major cause of CVD, begins in childhood. Acquired and genetic disorders of lipid and lipoprotein metabolism, present at an early age, are major contributors to early precursors of atherosclerosis, which accelerate after age 20. Treatment of the underlying medical condition and optimum management of all risk factors is critical in improving outcomes. Nonetheless, limited data is available to assist clinical decision-making, with the aim of improving outcomes. Atherosclerosis, beginning in childhood, is multifactorial in origin with complex interplay of inflammation, infection, endothelial dysfunction, and dyslipidemia. Future studies are needed to help elucidate the specific roles of disease mechanisms, with an emphasis on early intervention and prediction of subclinical disease. In addition to a heart healthy lifestyle, there may be a role for use of lipid-lowering medications beginning at an early age.

Construction of Femoral Vessel Nomograms for Planning Cardiac Interventional Procedures in Children 0-4 Years Old.

The objectives of this study were to construct femoral artery (FA) and femoral vein (FV) nomograms in children aged 0-4 years and to construct probability curves for the occurrence of arterial access complications based on the size of the FA. The FV and FA are commonly accessed during cardiac catheterizations in children with congenital heart diseases (CHD). However, nomograms for vessel dimensions based on child's age or size are not available. This knowledge may be helpful for interventional planning. A prospective study was performed on 400 children (age 0-4 years) with CHD undergoing cardiac catheterizations over a 3-year period. Ultrasound evaluation of the right and left FA and FV was performed under anesthesia prior to vascular access. Regression modeling was applied to derive nomograms based on quantile polynomial regression, which yielded good fit to the data judged by R-squared. GAMLSS transformation method was used to formulate smoothed percentiles. A separate prospective evaluation of FA to determine the size below which loss of pulse (LOP) are likely to occur was performed. Nomograms for FA and FV diameter and cross-sectional area against age and body surface area and probability curves for FA LOP were constructed. It is now possible to examine ultrasound-based normal sizes of femoral vein and artery in children 0-4 years of age. Femoral vessel nomograms and LOP probability curves may help with interventional planning. Future studies with larger sample size, including children of other ages may be useful.

Cardiac myxolipoma in a child: diagnosis and surgical management.

We present a case of a rare tumor, a myxolipoma, appearing in a 5-year-old child, along with the imaging evaluation and surgical management. The natural history is expected to be that of a benign lesion, but the rarity precludes large studies.