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Objectives Obesity has often been associated with high low-density-lipoprotein cholesterol (LDL-C) and triglyceride plasma concentrations, known risk factors for diabetes mellitus and cardiovascular disease. Study objective was to evaluate the association of LDL-C and triglyceride plasma concentration with muscle and fat mass in children and adolescents. Methods We analyzed data of the National Health and Nutrition Examination Survey (1999–2004) to estimate lean muscle and fat mass assessed by dual-energy X-ray absorptiometry (DXA) of participants whose lipid profiles had been examined. Fat mass was operationalized by DXA-determined fat mass index (FMI). Muscle mass was assessed by appendicular lean mass index (aLMI). High LDL-C and triglyceride concentration was defined as above 130 mg/dL. Results For the evaluation of the association of LDL-C and triglyceride plasma concentration with LMI and FMI Z-scores, the data of 2,487 children and adolescents (age 8–19 years) (984 females) were eligible. High aLMI showed no association with LDL-C or triglyceride concentration, but high FMI showed significant association with LDL-C and triglyceride plasma concentration in the bivariate regression analysis. Conclusions Isolated muscle mass increase may not be protective against high LDL-C and triglycerides plasma levels in children and adolescents. Thus, exercise may lead to risk factor reduction mainly through fat mass reduction.

Background:Increased central (or abdominal) fat mass has been associated with cardiometabolic risk factors such as high low-density lipoproteins (LDL)-cholesterol or triglycerides (TG) concentration in children. Objectives:To generate pediatric reference centiles for trunk/leg fat mass ratio (T2L) (assessed by dual-energy X-ray absorptiometry [DXA]) and to evaluate the association of LDL-cholesterol and TG concentrations with T2L in children and adolescents.Methods:Data of the National Health and Nutrition Examination Survey (1999-2004) were used to determine total and regional fat mass by DXA of the participants (aged 8-19 years) who had also an examination of LDL-cholesterol and TG concentrations. Fat mass was assessed by DXA-determined fat mass index (FMI). Central fat mass was quantified by T2L. Results:The DXA results of 6538 children and adolescents (2629 females) were used to generate reference centiles for T2L. In girls, T2L was significantly associated with high LDL-cholesterol and TG concentration (odds ratio [OR] adjusted to FMI 1.69), (95% confidence interval [CI] 1.20-2.40), and 1.45 (95% CI 1.11-1.91,p = 0.003 andp = 0.008). In boys, T2L was significantly associated only with high TG concentration (OR adjusted to FMI 1.81 [95% CI 1.52-2.19,p < 0.001]). Conclusions:A central fat distribution seemed to be an independent risk factor for high TG concentrations in children and for high LDL-cholesterol only in girls. The first ethnicity-specific, pediatric reference centiles for T2L were presented.

Background: Obesity was often associated with low high-density lipoprotein (HDL) cholesterol concentration, which is an established cardiovascular risk factor. Objectives: To evaluate the association of HDL-cholesterol concentration with fat and muscle mass in children and adolescents. Methods: Data of the National Health and Nutrition Examination Survey (1999-2004) were used to estimate fat and muscle mass by dual-energy X-ray absorptiometry (DXA) of the participants who had also an examination of their lipid profiles. Fat mass was assessed by DXA-determined fat mass index (FMI). Muscle mass was operationalized by appendicular lean mass index (LMI). Low HDL-cholesterol concentration was defined as Results: For the evaluation of the association of HDL-cholesterol concentration with FMI and LMI Z-scores, the data of 6288 children and adolescents (age 8-19 years) (2535 females) were eligible. In the study population, the prevalence of low HDL-cholesterol concentration increased with rising FMI and appendicular LMI Z-scores. Conclusions: The study results suggested that there is a counterintuitive, inverse association of muscle mass and HDL-cholesterol concentration.

Increasing knowledge in the field of rare diseases has led to new therapeutic approaches in the last decade. Treatment strategies have been developed after elucidation of the underlying genetic alterations and pathophysiology of certain diseases (e.g., in osteogenesis imperfecta, achondroplasia, hypophosphatemic rickets, hypophosphatasia and fibrodysplasia ossificans progressiva). Most of the drugs developed are specifically designed agents interacting with the disease-specific cascade of enzymes and proteins involved. While some are approved (asfotase alfa, burosumab), others are currently being investigated in phase III trials (denosumab, vosoritide, palovarotene). To offer a multi-disciplinary therapeutic approach, it is recommended that patients with rare skeletal disorders are treated and monitored in highly specialized centers. This guarantees the greatest safety for the individual patient and offers the possibility of collecting data to further improve treatment strategies for these rare conditions. Additionally, new therapeutic options could be achieved through increased awareness, not only in the field of pediatrics but also in prenatal and obstetric specialties. Presenting new therapeutic options might influence families in their decision of whether or not to terminate a pregnancy with a child with a skeletal disease.