Current Pediatric Reviews - Volume 13, Issue 4, 2017

Background: Severe Hypertriglyceridemia (HTG), i.e., plasma triglyceride levels exceeding 1000 mg/dL, is one of the established causes of acute pancreatitis and severe abdominal pain. There are no established pediatric guidelines regarding treatment of children and adolescents with severe HTG. Objective: To review the pathophysiology and etiology of severe HTG in the pediatric age group, and to discuss management options. Method and Results: Severe HTG is usually due to deficient or absent Lipoprotein Lipase (LPL) activity, which can be due to primary genetic etiology or secondary causes triggering HTG in those with underlying genetic susceptibility. Hospitalization is indicated for patients with severe HTG who are symptomatic with abdominal pain or pancreatitis, in those with uncontrolled diabetes requiring insulin, or, in those with substantial elevations of plasma TG. Fasting followed by fat free diet until plasma TG declines to <1000mg/dL is essential. Subsequently, stringent fat restriction followed by slowly increasing the dietary fat while maintaining the plasma TG concentration at a targeted level is recommended. Insulin infusions are helpful in patients who have some LPL activity, especially in those with diabetes. Plasmapheresis may be considered in those with severe pancreatitis, shock or multi-organ failure. Medications such as fibrates and omega-3 fatty acids are not effective if LPL activity is absent or when plasma TG is >1800 mg/dL. Medications only have an adjunct role in the management. Low fat diet, lifestyle changes, weight loss, control of secondary causes, and patient education form the mainstay of management once the patient is discharged.

LAL-deficiency (LAL-D) is a rare and systemic condition, secondary to LIPA gene mutations, responsible for lysosomal accumulation of cholesteryl esters and triglycerides, whose manifestations are very heterogeneous in terms of the age of onset, severity and the type of clinical and radiological manifestations. Dyslipidemia, hepatomegaly and hepatosteatosis with increased levels of transaminases are the most common features. The increased risk of premature atherosclerosis and cardiovascular disorders, secondary to a generalized alteration of lipid profile and lipoprotein dysfunction associated with LAL-D, has been increasingly pointed out. Therefore, medical awareness towards LAL-deficiency should be increased, since this condition has to be considered in the differential diagnosis of pediatric conditions manifested with dyslipidemia and hepatic accumulation of intracellular products. On the other hand, early patient identification and management remain challenging.

Background: Atherosclerotic disease is a leading cause of morbidity and mortality in adults and is generally thought of as only affecting adults. However, the pathologic changes in vessels leading to atherosclerosis, and an increased risk of cardiovascular disease, have been shown to begin in early adolescence. Objectives: There is a growing body of literature suggesting that earlier treatment, through lifestyle changes and pharmacotherapy, can help reduce this risk. A growing number of children are presenting with elevated cholesterol because of the increased prevalence of obesity and diabetes mellitus. Methods: In addition, an increasing number of children are living with previously fatal diseases that increase the risk of atherosclerosis, either because of the disease process or as adverse effect of the treatment, such as human immunodeficiency virus, Kawasaki disease, and cardiac transplantation. Result and Conclusion: In addition, specific disorders of cholesterol metabolism, such as Familial Hypercholesterolemia (FH) may be encountered in a pediatric practice.

Background: Cardiovascular diseases are the leading cause of death worldwide. While cardiovascular disease typically does not occur until adulthood, the pathogenesis of atherosclerotic cardiovascular disease starts in early childhood. Objective: The purpose of this review is to describe the evidence that the process of atherosclerosis begins in childhood, as well as identify the risk factors present in childhood that are associated with the presence of atherosclerotic cardiovascular disease in childhood as well as future cardiovascular events in adulthood. Results and Conclusion: There is much accumulating evidence linking modifiable risk factors that are already present in childhood and that strongly predict future cardiovascular disease. The selective targeting of modifiable risk factors in childhood, including body mass index, holds promise in reducing the burden of adult cardiovascular disease. Future research studies should focus on elucidating the mechanisms which drive the pathogenesis of atherosclerosis in childhood and young adulthood, as well as identifying which interventions are most effective at limiting the progression of atherosclerosis and thus reducing future cardiovascular disease.

Familial hypercholesterolemia (FH) is a worldwide common autosomal inherited condition associated with premature cardiovascular diseases, both in men and in women (World frequency has been recently estimated to be as high as 1:250). Identifying FH cases early in life could represent a cornerstone to prevent fatal events in adult's life. Pediatricians are well positioned to evaluate the familial cardiovascular risk factors among their young patients, to make a diagnosis and to perform familial screening. The goal of this paper is therefore to emphasize the recognition of this genetic condition at a younger age and to initiate both familial screening and a safe treatment. Prevention of cardiovascular disease starts at an early age in all children, but it may save lives in FH patients.

Background: The Coronary Artery Risk Detection in Appalachian Communities (CARDIAC) Project is a chronic disease risk factor surveillance, intervention, and research initiative aimed at combating the unacceptably high prevalence of heart disease, diabetes, and other chronic illnesses in West Virginia. Objectives and Methods: The school-based public health project identifies health risk factors in children, educates families, informs primary care physicians, and provides resources to schools to help improve population health, beginning with children. Results and Conclusion: Details regarding methodology, results, and conclusions derived from this unique public health initiative that has screened over 200,000 children are the subject of this 18- year review.

Background: Individuals diagnosed with diabetes mellitus have an increased risk of cardiovascular disease, often leading to premature morbidity and mortality. As the number of youth developing T1D and T2D continues to grow, early onset predisposes patients to a longer burden of disease and earlier onset of cardiovascular complications. Early identification and effective management and treatment of risk factors can lead to decreased cardiovascular events and improved quality of life. Objective: In this study, we describe the prevalence of CVD risk factors in children with diabetes. Results: 274 subjects were included in this study, all > 1 year since the time of diagnosis. 134 participants were male (49%). Mean age was 13.7 years (males); 12.8 years (females). subjects averaged 4 diabetes-related visits per year. At the time of their most recent clinic visit, 33% of subjects had a HbA1c < 8%, and 29% had a HbA1c > 10%. Greater than 50% of subjects were overweight or obese. A significant number of subjects were found to have dyslipidemia, and elevated blood pressure was common. Discussion: CVD risk factors are common in children with diabetes mellitus. Good glycemic control, early identification, and effective life-long management of all CVD risk factors may help decrease the high rate of premature morbidity and mortality in diabetes.