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ObjectiveDevelop a multi-functional imaging system that combines 1.7μm optical coherence tomography/angiography (OCT/OCTA) to accurately interrogate Hereditary Hemorrhagic Telangiectasia (HHT) skin lesions.MethodsThe study involved imaging HHT skin lesions on five subjects including lips, hands, and chest. We assessed the attributes of both HHT lesions and the healthy vasculature around them in these individuals, employing quantifiable measures such as vascular density and diameter. Additionally, we performed scans on an HHT patient who had undergone anti-angiogenic therapy, allowing us to observe changes in vasculature before and after treatment.ResultsThe results from this pilot study demonstrate the feasibility of evaluating the HHT lesion using this novel methodology and suggest the potential of OCTA to noninvasively track HHT lesions over time. The average percentage change in density between HHT patients' lesions and control was 37%. The percentage increase in vessel diameter between lesion and control vessels in HHT patients was 23.21%.ConclusionIn this study, we demonstrated that OCTA, as a functional extension of OCT, can non-invasively scan HHT lesions in vivo. We scanned five subjects with HHT lesions in various areas (lip, ear, finger, and palm) and quantified vascular density and diameter in both the lesions and adjacent healthy tissue. This non-invasive method will permit a more comprehensive examination of HHT lesions.SignificanceThis method of non-invasive imaging could offer new insights into the physiology, management, and therapeutics of HHT-associated lesion development and bleeding.

Pazopanib (Votrient) is an orally administered tyrosine kinase inhibitor that blocks VEGF receptors potentially serving as anti-angiogenic treatment for hereditary hemorrhagic telangiectasia (HHT). We report a prospective, multi-center, open-label, dose-escalating study [50 mg, 100 mg, 200 mg, and 400 mg], designed as a proof-of-concept study to demonstrate efficacy of pazopanib on HHT-related bleeding, and to measure safety. Patients, recruited at 5 HHT Centers, required ≥ 2 Curacao criteria AND [anemia OR severe epistaxis with iron deficiency]. Co-primary outcomes, hemoglobin (Hgb) and epistaxis severity, were measured during and after treatment, and compared to baseline. Safety monitoring occurred every 1.5 weeks. Seven patients were treated with 50 mg pazopanib daily. Six/seven showed at least 50% decrease in epistaxis duration relative to baseline at some point during study; 3 showed at least 50% decrease in duration during Weeks 11 and 12. Six patients showed a decrease in ESS of > 0.71 (MID) relative to baseline at some point during study; 3/6 showed a sustained improvement. Four patients showed > 2 gm improvement in Hgb relative to baseline at one or more points during study. Health-related QOL scores improved on all SF-36 domains at Week 6 and/or Week 12, except general health (unchanged). There were 19 adverse events (AE) including one severe AE (elevated LFTs, withdrawn from dosing at 43 days); with no serious AE. In conclusion, we observed an improvement in Hgb and/or epistaxis in all treated patients. This occurred at a dose much lower than typically used for oncologic indications, with no serious AE. Further studies of pazopanib efficacy are warranted.

OBJECTIVE. The goal was to determine the diagnostic utility of the National Cholesterol Education Program pediatric guidelines. METHODS. With the use of pediatric lipid data from the Cincinnati Clinic of the Lipid Research Clinics Prevalence Study and lipid and cardiovascular disease data collected for the same subjects as adults in the Princeton Follow-up Study, the sensitivity and specificity of the National Cholesterol Education Program pediatric guidelines were calculated overall and according to age. Furthermore, whether use of parental cardiovascular disease history during childhood influenced the sensitivity 6and specificity was assessed. RESULTS. Overall sensitivities were 43% to 46% and specificities were 82% to 86% for total and low-density lipoprotein cholesterol levels. There was considerable variation in sensitivities according to age, with the lowest sensitivities at ages 14 to 16 years and the highest sensitivities at ages 5 to 10 years and 17 to 19 years. Results were similar whether or not the population was restricted to children with a positive parental history of cardiovascular disease. CONCLUSIONS. Results of our analyses suggest that the sensitivity and specificity for evaluating total cholesterol or low-density lipoprotein cholesterol levels that are elevated in adulthood are not improved by selecting children with a positive parental history. These data also show the strong role that age (particularly the pubertal years between 10 and 15 years of age) plays in lipid measurements for children and adolescents. Continued prospective and longitudinal studies designed with age as well as other risk parameters are needed to determine the best guidelines for clinical screening in the future. www.pediatrics.org/cgi/doi/10.1542/ peds.2005-2968 doi:10.1542/peds.2005-2968 Key Words low-density lipoprotein cholesterol, adolescent, child, sensitivity, specificity, lipoproteins Abbreviations NCEP--National Cholesterol Education Program CVD--cardiovascular disease LDL--low-density lipoprotein PFS--Princeton Follow-up Study LRC--Lipid Research Clinics, CURRENTLY, CHILDREN AND adolescents are important targets for cardiovascular disease (CVD) risk factor screening. The proportions of pediatricians and all physicians who screen children for serum cholesterol levels are 88% [...]

Nitric oxide can oxidize proteins, which may be a risk factor for cardiovascular disease. This was the conclusion of a study of 100 patients with cardiovascular disease and 108 healthy people. This study also showed that a group of drugs called statins can decrease the amount of these oxidized proteins, in addition to lowering blood cholesterol levels. Nitric oxide is a natural substance in the body that relaxes blood vessels.

Certain disorders of fat metabolism that predispose people to cardiovascular disease are likely to occur in both children and their parents. With the growing interest in performing blood serum lipid profiles in children, this knowledge can serve as a means of alerting parents to their need for evaluation. A group of 232 families including 497 children underwent lipid profiles as part of the Cincinnati Myocardial Infarction and Hormone Study. Only white males and their families were recruited. Lipid disorders consisted of high levels of low-density lipoprotein cholesterol (LDL-C), high triglycerides (TG), low levels of high-density lipoprotein cholesterol (HDL-C), and combinations thereof. Children with disorders of LDL-C or a combination of TG and HDL-C were statistically more likely to have parents with the same disorder. Children with a combination of disorders were statistically more likely to have parents with a lipid disorder of some kind. Overweight status in children and adults correlated with lipid disorders., Objective. To determine whether lipid disorders can be predicted in parents after such disorders are identified initially in their children. Although this relation has been well determined for children with high cholesterol or low-density lipoprotein cholesterol (LDL-C), it has not been as well described for disorders involving triglycerides (TG) or high-density lipoprotein cholesterol (HDL-C), or their interaction with LDL-C. Methods. Serum lipid values were obtained from 232 families in the comparison population of a large genetics study. Subjects were classified into four groups based on their lipid status: 1) isolated LDL-C disorder, defined by a high LDL-C level and normal TG and HDL-C levels; 2) isolated TG/HDL-C disorder, defined by either high TG, low HDL-C, or both, and normal LDL-C; 3) combined disorder, defined by high LDL-C in addition to either high TG, low HDL-C, or both; and 4) normal, defined by the absence of any of the above disorders. The frequencies of these disorders were noted in both parents and children, and logistic regression analyses were conducted to determine whether the presence of these disorders in at least one child in the family could predict similar disorders in the parents. Results. Children with isolated LDL-C or TG/HDL-C disorder were more likely to have parents with the same disorder as themselves (P = .002 and P = .04, respectively). Children with the combined disorder were more likely to have parents with any lipid disorder (P = .009), but especially isolated LDL-C (P = .002) and isolated TG/HDL-C (P = .05). Conclusion. A classification scheme defining disorders of TG and HDL-C, LDL-C, or a combination can be useful for predicting lipid disorders in parents after such disorders are identified initially in their children. Pediatrics 1994;94:824-831; children, cholesterol, low-density lipoprotein cholesterol, triglycerides, high-density lipoprotein cholesterol, lipid disorders.

The experience of a myocardial infarction and the accompanying anxiety may lead to behavioral disturbances in the patient (1) and the spouse (2). The consequences of such a cardiac event [...]

The American Heart Association Step-One diet may be an effective treatment for children with hyperlipidemia. Hyperlipidemia is a disorder characterized by increased blood levels of different types of lipids (fats). Among 59 children who followed the American Heart Association Step-One diet, 22 initially had elevated blood levels of low density lipoprotein cholesterol (LDL-C), 21 had elevated blood levels of triglycerides (TG) and reduced blood levels of high density lipoprotein cholesterol (HDL-C), 15 had slightly elevated blood levels of different types of lipids and one had normal blood levels of lipids. After an average of five months on the diet, the average blood level of LDL-C dropped in children who initially had elevated LDL-C. The average blood level of TG dropped and that of HDL-C increased over an average of five months in the children who initially had abnormal blood levels of TG and HDL-C., Patients seen at a pediatric lipid clinic over a 27-month period were reviewed retrospectively to evaluate types of primary lipid disorders and effect of dietary treatment at the first follow-up visit. One hundred eighty-two patients were classified into one of four categories: (1) elevated low-density lipoprotein cholesterol (LDL-C) with LDL-C > 95th percentile (32%); (2) isolated triglyceride (TG)/high-density lipoprotein cholesterol (HDL-C) abnormalities, with TG > 95th percentile and/or HDL-C < 5th percentile (30%); (3) borderline LDL-C, TG, or HDL-C (29%); (4) normal (9%). The American Heart Association Step-One Diet was prescribed for all patients older than 2 years, and they received extensive nutritional and risk-management counseling. Of these patients, 59 (32%) returned for at least one follow-up visit and mean changes in lipid values between initial and first follow-up visits were evaluated. Levels of LDL-C decreased by 24 mg/dL in 22 patients with elevated LDL-C levels. Triglyceride levels decreased by a mean of 22 mg/dL and HDL-C increased by a mean of 4 mg/dL in 21 patients with isolated TG/HDL-C abnormalities. Levels of LDL-C tended to rise in this group, but not to a significant degree. A new finding of this report is that screening for total cholesterol results in the identification of many children with TG or HDL-C abnormalities alone and that the Step-One Diet appears to be effective in improving both TG and HDL-C levels in these patients. Pediatrics 1993; 91:92-96; cholesterol, low-density lipoprotein cholesterol, triglycerides, high-density lipoprotein cholesterol, dietary therapy.