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Biomedical subjects

T J Starc

Publications and source records attributed to T J Starc.

At least 37 records · Page 2Linked to original sources

Greater dietary intake of simple carbohydrate is associated with lower concentrations of high-density-lipoprotein cholesterol in hypercholesterolemic children.

Hypercholesterolemic children are increasingly being treated with lipid-lowering diets, but little research has focused on the effects of specific dietary substitutions on HDL cholesterol. We examined the relation between carbohydrate intake and HDL cholesterol in hypercholesterolemic children consuming fat-restricted diets. We obtained 3-d food records for 67 children (mean age: 5.8 +/- 2.5 y) referred for the treatment of hypercholesterolemia. Mean plasma HDL cholesterol was 1.12 +/- 0.21 mmol/L and total cholesterol was 5.99 +/- 1.39 mmol/L. Dietary intake comprised (percentage of total energy) 24.9 +/- 5.1% fat, 59.9 +/- 6.5% carbohydrate, and 16.5 +/- 3.4% protein. Carbohydrate intake included 30.7 +/- 7.4% from simple and 22.6 +/- 6.2% from complex carbohydrates. HDL cholesterol was positively correlated with intake of total fat (r = 0.44, P < 0.001) and saturated fatty acids (r = 0.43, P < 0.001) and inversely correlated with intake of total carbohydrate (r = -0.55, P < 0.001) and simple carbohydrate (r = -0.40, P < 0.001), but not with complex carbohydrate (r = -0.02). The significant inverse relation between simple carbohydrate intake and HDL cholesterol remained after intakes of saturated, monounsaturated, and polyunsaturated fatty acids; intake of complex carbohydrates; dietary cholesterol; plasma triacylglycerol; and age were adjusted for with multivariate techniques. In summary, higher dietary intake of simple carbohydrates was associated with lower HDL-cholesterol concentrations in hypercholesterolemic children consuming reduced-fat diets.

Body Mass Index↗

Evaluation of hypercholesterolemia in childhood.

For many adults, the risk of atherosclerosis can be reduced by intervention and treatment of known risk factors. Direct proof that similar intervention will be effective in children is not available. However, evidence suggests that prevention beginning in childhood will lead to a decrease in incidence of heart disease later in life. The majority of families are eager to take steps to prevent heart disease in their children, especially if there is a family history of early heart disease. It is the role of the pediatrician to identify those children at risk for early heart disease and to initiate advice on reducing risk factors.

Age Factors↗

Early diagnosis of coarctation of the aorta in children: a continuing dilemma.

UNLABELLED: Because late repair of coarctation of the aorta (COA) is associated with premature cardiovascular disease in adult life, early detection and treatment is important. OBJECTIVES: To determine the timing of referral to see whether early detection of COA has improved in the past decade, to evaluate the pattern of and reasons for medical center referral, and to assess the clinical signs relating to the diagnosis of COA. METHODS: The records of 50 consecutive patients older than 1 year who had surgical repair of COA from 1980 to 1990 were reviewed. The age of referral, pattern of referral, and presence of standard clinical signs of COA were analyzed, and data were compared with those from the previous decade. RESULTS: The mean and median ages at referral were 8.4 and 5.8 years, respectively. Pediatricians accounted for 64% of the referrals. A specific diagnosis of COA was made in 2 (4%) of 50 patients before referral to a pediatric cardiologist. The most consistent clinical findings were a cardiac murmur and a systolic blood pressure gradient between the arms and legs of greater than 10 mm Hg, which were both present in all patients. Lower-extremity pulses were decreased in 37 (74%) and absent in 9 (18%). Forty-seven children (94%) had upper-extremity hypertension (> 95th percentile for age); 25 (50%) had systolic blood pressure higher than 140 mm Hg. COA would have been missed in 82% of children if absent lower-extremity pulses were required as a diagnostic feature. These findings were similar to those reported by our institution in the previous decade, suggesting that early detection has not improved. CONCLUSIONS: The timing of, reasons for, and sources of referral for COA in this study, compared with data from the previous decade, indicate no improvement in early detection of COA by pediatricians. Screening all children for COA by routinely measuring upper- and lower-extremity blood pressures during at least one physical examination after the newborn period is mandatory.

Aortic Coarctation↗

Cardiac size and function during adrenocorticotropic hormone-induced systolic systemic hypertension in infants.

The effects of adrenocorticotropic hormone (ACTH) on systolic blood pressure, and echocardiographic indexes of heart size and function were investigated in 14 infants. After 25 days (range 13 to 46) of treatment with ACTH, systolic blood pressure increased from 93 +/- 9 to 118 +/- 20 mm Hg (p < 0.001; mean +/- 1 SD). Systolic hypertension (systolic blood pressure greater than the 95th percentile for age) developed in 10 of 14 infants and was associated with an increase in left ventricular (LV) shortening fraction from 41 +/- 5% to 52 +/- 8% (p < 0.001). Myocardial hypertrophy and an increase in echocardiographic indexes of myocardial contractility were observed also. To assess the temporal relation between the onset of systolic hypertension and these cardiac changes, data from 8 infants with serial echocardiograms and blood pressure determinations were examined. After a mean 14 days (range 8 to 18) of ACTH, LV shortening fraction increased from 39 +/- 6% to 53 +/- 8% (p < 0.01), whereas systolic blood pressure remained normal in 7 of 8 infants. In addition, a decrease in LV end-systolic dimension was observed during this early phase. This report documents myocardial changes in individual patients, which occur before and during the development of systolic hypertension.

Adrenocorticotropic Hormone↗

Acquired coarctation of the aorta.

Coarctation of the aorta is usually caused by a congenital narrowing of the aorta. This report describes two children who developed hypertension secondary to an acquired coarctation of the aorta. In one patient the coarctation was temporally related to umbilical artery catheterization and was associated with thrombosis and aneurysmal dilatation of the aorta. In the second patient, the coarctation occurred after surgical aortotomy during the removal of an intrathoracic neuroblastoma. Patients who have interventional damage to the aorta should be periodically examined for the appearance of a coarctation. Although an acquired coarctation of the aorta is an infrequent complication of invasive or surgical procedures, it should be identified since it represents a remediable cause of hypertension in children.

Aortic Coarctation↗

Erythrocyte macrocytosis in infants and children with Down syndrome.

Erythrocyte mean corpuscular volume and mean corpuscular hemoglobin levels were higher in children with Down syndrome than in normal control subjects. Reference values for mean corpuscular volume and mean corpuscular hemoglobin level derived from normal populations may be inappropriate for children with Down syndrome. These findings may have important implications for the diagnosis of iron deficiency in these children.

Case-Control Studies↗

Family history fails to identify many children with severe hypercholesterolemia.

Optimal strategies for identifying children with hypercholesterolemia have not been established. Several groups have advocated that testing of serum cholesterol levels be limited to those children who have family histories of hyperlipidemia or premature coronary heart disease. We studied the ability of comprehensive family histories to identify children with hyperlipidemia in a group of 114 children (mean age, 8 +/- 4 years) who were referred for treatment of hypercholesterolemia. A positive family history was defined according to guidelines of the American Academy of Pediatrics. The mean fasting total cholesterol in the children was 5.74 +/- 1.42 mmol/L (222 mg/dL). Family history was negative for hypercholesterolemia or premature coronary heart disease in 22 (22%) of 100 children with total cholesterol levels greater than the 75th percentile for their ages, in 13 (18.3%) of 71 children with total cholesterol levels greater than the 95th percentile for their ages, and in four (11.8%) of 34 children with presumed heterozygous familial hypercholesterolemia. Of the 78 children who had both hypercholesterolemia and positive family histories, hyperlipidemia was reported in 72 families, whereas premature heart disease was reported in only 27. We conclude that in a population of children referred because of known hypercholesterolemia, a detailed family history not only fails to identify many children with mild hypercholesterolemia, but also fails to identify a significant proportion of children with markedly elevated cholesterol levels. Additionally, in families of children with hypercholesterolemia, a history of hyperlipidemia is more common than a history of premature heart disease.

Adolescent↗

Response to diet and cholestyramine in a patient with sitosterolemia.

In this report, an 11-year-old boy with diffuse tendinous and tuberous xanthomatosis and a plasma sterol concentration of 555 mg/dL, consisting primarily of cholesterol, is described. Three months after changing from an unrestricted diet to a cholesterol-lowering diet, his plasma sterol concentration decreased to 221 mg/dL. Because of the degree and rapidity of his response to diet, sitosterolemia was suspected. According to results of capillary gas-liquid chromatography of his plasma sterols, there was a sitosterol concentration of 31.3 mg/dL (normal less than 1.0 mg/dL), establishing the diagnosis of sitosterolemia. Addition of cholestyramine therapy (8 g/d) to a low sterol diet further lowered his plasma sterol concentration to 173 mg/dL and led to complete regression of all tuberous xanthomata. Tendinous xanthomata regressed at a slower rate. These findings show that the diagnosis of sitosterolemia should be suspected in severely hypercholesterolemic children (total cholesterol greater than 400 mg/dL) whose plasma cholesterol level is highly responsive to dietary manipulation. The rapid and sustained lowering of plasma cholesterol and regression of xanthomata after treatment with diet and cholestyramine suggest that sitosterolemia is a treatable cause of premature atherosclerosis.

Arteriosclerosis↗

Pulmonary venous obstruction and atrioventricular canal anomalies: role of cor triatriatum and double outlet right atrium.

Two-dimensional echocardiographic findings and surgical repair in three infants with atrioventricular (AV) canal and pulmonary venous obstruction are described. The AV canal was complicated by cor triatriatum in two patients and by double outlet right atrium in the other. In patients with AV canal, anatomic obstruction of pulmonary venous return should be excluded by detailed cardiac ultrasound examination.

Cor Triatriatum↗

Time course of changes of plasma renin activity and catecholamines during hemorrhage in conscious sheep.

The time courses of humoral changes in the renin-angiotensin system and the sympathetic nervous system were studied in conscious sheep in response to slow and fast hemorrhages. In two separate groups of chronically instrumented animals, 18 hemorrhages. In two separate groups of chronically instrumented animals, 18 ml/kg of blood was withdrawn over 10 or 30 minutes. The activation of the renin-angiotensin system was assessed by measurement of plasma renin activity and the sympathetic nervous system was assessed by measurement of circulating epinephrine and norepinephrine concentrations. The activation of both the renin-angiotensin system and the sympathetic nervous system occurred more rapidly in the fast hemorrhage group than the slow hemorrhage group. The peak mean plasma renin activity was 20.83 +/- 5.75 ng angiotensin I/ml/hr during the rapid hemorrhage and 8.8 +/- 1.43 ng angiotensin I/ml/hr during the slow hemorrhage (p less than 0.05). In contrast, the levels of maximal activation of the sympathetic nervous system during the slow and rapid hemorrhages were not significantly different. However, despite the threefold difference in rate of blood removal between the two groups, when the activities of the renin-angiotensin system and the sympathetic nervous system were plotted against the volume of blood removed, the time courses of change of these two humoral defense mechanisms were similar in the slow and fast hemorrhage groups. In both groups, an increase in plasma renin activity began earlier than the increase in circulating concentrations of epinephrine and norepinephrine; the maximal increase in all three humoral agents occurred near the end of the blood withdrawal.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute Disease↗

Progressive obstruction of the foramen ovale in patients with left atrioventricular valve atresia.

Thirteen patients with left atrioventricular (AV) valve atresia and a normal aortic root were studied to evaluate the status of the interatrial communication with advancing age. Six patients had cardiac catheterization within the first 2 weeks of age; of these, five had repeat studies before 7 months of age. The seven other patients initially underwent catheterization after 2 weeks of age. In the group with catheterization before 2 weeks of age, the mean left atrial pressure was 7.8 +/- 5.5 mm Hg and the left atrial-right atrial mean pressure gradient was 1.7 +/- 2.4 mm Hg. In the combined group of patients with catheterization after 2 weeks of age, the mean left atrial pressure was 25.9 +/- 5.6 mm Hg and the mean left atrial-right atrial pressure gradient was 21.1 +/- 5.1 mm Hg. Seven of the 13 patients have survived and have now reached a median age of 6.5 years. Balloon atrial septostomy was adequate for long-term survival in one patient; all of the others have required surgical atrial septectomy. Progressive obstruction of the foramen ovale is part of the natural history of left AV valve atresia, and obstruction develops despite the absence of a left atrial-right atrial gradient during newborn study. Balloon atrial septostomy is recommended during the neonatal period in all patients with left AV valve atresia, even in the absence of an interatrial gradient. Because early surgical atrial septectomy is usually necessary for long-term survival, these patients should have serial noninvasive evaluation of the patency of the interatrial communication.

Cardiac Catheterization↗

Normal growth in high-risk hyperlipidemic children and adolescents with dietary intervention.

OBJECTIVE: The aim of this study was to assess the safety and efficacy of lowering dietary intake of total fat, saturated fat, and cholesterol in growing children and adolescents with severe hyperlipidemia. STUDY DESIGN: This is a 3-year follow-up study conducted on a sample of convenience at three pediatric referral centers in New York City and its suburbs. Subjects were 138 children and adolescents 2 to 15 years of age (54% male), who had been referred with a diagnosis of hyperlipidemia. Those selected had total serum cholesterol values greater than the 95th percentile for age and had at least three visits over 3 years. They were placed on diets restricting total fat content to 30% of total calories and saturated fat to 10% of total calories (National Cholesterol Education Program Step I diet). Anthropometric measures, lipid profiles, and dietary assessment were obtained at each visit. Anthropometric data were analyzed by sex and age. Z scores for height and weight were calculated from NHANES II data and were compared by paired t tests (Hamill et al., 1979, Am J Clin Nutr 32:607-29). RESULTS: Total serum cholesterol dropped from 262 mg/dL at baseline to 249 mg/dL at 3-year follow-up (P = 0.003). There was no significant change in height or weight percentile, expressed as Z score, from baseline to 3-year follow up. CONCLUSIONS: In this population the supervised dietary interventions resulted in a sustained improvement of the lipid profile, with no demonstrable adverse effect on growth.

Adolescent↗

Electrocardiography and 24-hour electrocardiographic ambulatory recording (Holter monitor) studies in children infected with human immunodeficiency virus type 1. The Pediatric Pulmonary and Cardiac Complications of Vertically Transmitted HIV-1 Infection Study Group.

Limited data are available on the electrocardiogram and ambulatory electrocardiogram recording (Holter) in children infected with the human immunodeficiency virus type 1 (HIV-1). The purpose of this study was to estimate the prevalence and cumulative incidence of rhythm and conduction abnormalities in HIV-1-infected children. Electrocardiograms and Holter monitoring studies were performed annually on 205 HIV-1-infected children enrolled after 28 days of life (group I), 93 HIV-1-infected infants enrolled during pregnancy or during the first 28 days of life (group IIa), and 463 HIV-1-uninfected infants enrolled during pregnancy or during the first 28 days of life (group IIb). The 5-year cumulative incidence in the group I children of second-degree atrioventricular block or supraventricular or ventricular tachycardia was 13.4%, and the 5-year incidence was higher for the older infected group I children (16.8% for children > or =4 years old at first study and 11.4% for children <4 years, p = 0.04). The mean corrected QT interval was also longer for the older infected group I children (p = 0.002) and prolonged in the HIV-1-infected compared to the HIV-1-uninfected group II children (p = 0.02). None of the children had atrial fibrillation or flutter. Arrhythmias are uncommon in children infected with HIV-1 and in children of HIV-1-infected mothers and the arrhythmias identified tend to be benign. Therefore, routine Holter monitoring does not appear to be indicated in asymptomatic children.

Arrhythmias, Cardiac↗