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1.
ABSTRACT. Nine children with familial hypercholesterolaemia, age range 2 to 12 years, were treated with a low cholesterol diet and probucol (10 mg/kg/day). The year before, the children received, as only treatment, a low fat-cholesterol diet. During this period their mean plasma total cholesterol level fell from 8.2±1.45 mmol/l to 7.17±0.84 mmol/l (12.6%). This level was further reduced to 5.92±0.63 mmol/l (17.1%) after the addition of probucol. Plasma high density lipoprotein cholesterol levels were lowered in absolute terms but not in relation to total cholesterol. No apparent side effects were observed. However, the use of probucol should be restricted for the moment to severe cases of hypercholesterolaemia as the long-term excretion of the drug in children is not yet known.  相似文献   

2.
Hypercholesterolaemia is a major risk factor for the development of coronary heart disease (CHD). Early detection and management of hypercholesterolaemia could retard the atherosclerotic process. Given that CHD and hypercholesterolaemia cluster within families, a screening strategy based on a family history of vascular disease has been advocated. Serum total cholesterol concentrations were measured in a random stratified sample of 1012 children aged from 12-15 years old participating in a coronary risk factor surveillance study in Northern Ireland. Information about vascular disease in close family members was obtained by means of a questionnaire. The study population was divided into two groups according to total cholesterol values: (i) normal, < 5.2 mmol/l (n = 822) and (ii) raised, > or = 5.2 mmol/l (n = 190). A family history identified 63 out of 190 individuals with hypercholesterolaemia yielding a sensitivity of 33.2% and specificity of 71.5%. Our data indicated that a strategy whereby only children from high risk families are screened for hypercholesterolaemia is ineffective. While primary prevention emphasising a healthy diet for all is essential, the role of universal screening deserves further appraisal.  相似文献   

3.
In a country with a high cardiovascular mortality rate, lipid profiles were studied in 929 adolescents (440 from affected and 489 from non-affected families for cardiovascular disease and hypercholesterolaemia). In 334 children with elevated or borderline total cholesterol level, lipid profiles were re-measured after a 1-y period. In boys from affected families, in contrast to boys from non-affected families, significantly higher total cholesterol levels (4.36 +/- 0.81 vs 4.19 +/- 0.78 mmol/L, p < 0.05) and LDL-C level (2.1 +/- 0.72 vs 1.89 +/- 0.79 mmol/L, p < 0.05) and significantly lower HDL-cholesterol levels (1.81 +/- 0.34 vs 1.93 +/- 0.38 mmol/L, p < 0.05) were found. The odds ratio for being in the most unfavourable decile for LDL-cholesterol was significantly higher for girls from affected families (2.17, p = 0.02). A relatively high HDL-C level as well as a favourable TC/HDL-C ratio was demonstrated in all groups, being lowest in boys from affected families. A significant correlation was found between baseline lipids and their values re-measured after 1 y. It is concluded that (1) adolescents with a positive family history are at increased risk for unfavourable lipid profile, (2) adolescents with elevated total cholesterol and LDL-cholesterol levels remain hypercholesterolaemic after a 1-y period and are therefore candidates for further biochemical and clinical monitoring, and (3) children with elevated total cholesterol may not be at high risk for cardiovascular disease owing to the favourable TC/HDL-C ratio. The study results do not indicate that general cholesterol screening in Polish adolescents is necessary, as the proportion of children with elevated LDL-cholesterol is relatively low.  相似文献   

4.
Hypercholesterolaemia is a major risk factor for the development of coronary heart disease (CHD). Early detection and management of hypercholesterolaemia could retard the atherosclerotic process. Given that CHD and hypercholesterolaemia cluster within families, a screening strategy based on a family history of vascular disease has been advocated. Serum total cholesterol concentrations were measured in a random stratified sample of 1012 children aged from 12-15 years old participating in a coronary risk factor surveillance study in Northern Ireland. Information about vascular disease in close family members was obtained by means of a questionnaire. The study population was divided into two groups according to total cholesterol values: (i) normal, < 5.2 mmol/l (n = 822) and (ii) raised, > or = 5.2 mmol/l (n = 190). A family history identified 63 out of 190 individuals with hypercholesterolaemia yielding a sensitivity of 33.2% and specificity of 71.5%. Our data indicated that a strategy whereby only children from high risk families are screened for hypercholesterolaemia is ineffective. While primary prevention emphasising a healthy diet for all is essential, the role of universal screening deserves further appraisal.  相似文献   

5.
1510 plasma cholesterol estimations were made in 1391 children admitted to hospital as part of a biochemical profile. Babies under 1 year and children known to have familial hyperlipoproteinaemia were excluded. The mean concentration was 4-28 mmol/l +/- 1-04 (1 SD) (165-3 mg/100 ml +/- 38-6), and levels exceeded 5-93 mmol/l (229 mg/100 ml) in 68 children. Repeat estimations on 55 of these children showed 34 still to have values greater than 5-93 mmol/l and family studies were performed in 19 of these. In 8 children hypercholesterolaemia was secondary and no familial lipoprotein disorder was present. Familial hyper-beta-lipoproteinaemia (FH) was diagnosed in 3 children and in 2 of the families there was a history of early ischaemic heart disease. In 2 children the diagnosis was in doubt. In the remaining 6 children FH and secondary hyperlipoproteinaemia were excluded so the hypercholesterolaemia was presumably environmentally induced, possibly in association with polygenic inheritance. In the present state of knowledge screening of the childhood population for FH by means of plasma cholesterol determinations cannot be recommended. Studies of lipoproteins should, however, be made in children from families known to have FH or early coronary heart disease.  相似文献   

6.
A prospective follow-up study of infants selected by cord blood total cholesterol (TC) and low-density-lipoprotein cholesterol (LDL-C) levels from 2000 consecutive live births was undertaken to reassess the role of cord blood screening in the diagnosis of familial hypercholesterolaemia (FH). Mean values for serum cholesterol were (mmol/l +/- S.D.): TC, 1.83 +/- 0.56; LDL-C, 0.90 +/- 0.49; HDL-C, 0.70 +/- 0.33; TG, 0.38 +/- 0.16. Seventy-three of 117 infants who had had a cord TC and/or LDL-C greater than 95th percentile, and 373 control group children (cord TC and/or LDL-C less than 95th percentile) were followed up at age 3--12 months. Six of the 117 were hypercholesterolaemic (HC), and one child had an HC parent: positive detection rate greater than or equal to 0.05%; false positive rate greater than or equal to 3.7%. Four control-group children were HC and had an HC parent; false negative rate greater than or equal to 1.1%. With the possible exception of detecting FH in a child with a known affected parent, cord blood screening appears to be unreliable for the diagnosis of FH.  相似文献   

7.
Aim: To determine whether a moderately reduced fat diet affects longitudinal growth in children with hypercholesterolaemia with a mean duration of 7.42 ± 1.93 y. Methods: Subjects with hypercholesterolaemia, total cholesterol above 5.18 mmol/L and LDL-cholesterol above 3.49 mmol/L (n = 144; 69 males and 75 females) from 2 to 13 y of age were studied. Patients were seen in our outpatient department for regular health check-ups. Weight and height were measured every year. At the same time, cholesterol, triglycerides, LDL-C, HDL-C, A-apoprotein and B-apoprotein levels were analysed. Furthermore, degrees of compliance at 1 mo, 6 mo and each year after starting the dietary treatment were determined. Results: No significant change in height or weight was found after fat restriction. In contrast, significant reductions in total cholesterol, LDL cholesterol and B-apoprotein levels of 19%, 24% and 14%, respectively, were detected. Finally, no significant differences were seen in HDL-cholesterol, A-apoprotein or triglycerides.

Conclusions: These data support the hypothesis that growth is not influenced by moderate fat restriction in healthy children who otherwise have normal nutrition.  相似文献   

8.
Thirty-six children identified as having primary type IIa hypercholesterolemia were treated with a diet restricting the intake of saturated fat to 10% of total energy and supplemented with soluble fiber for 8.1 +/- 2.4 (mean +/- SEM) months. In 14 of 36 patients first treated with the American Heart Association "Step-One" diet for 8.0 +/- 1.1 months, total cholesterol level dropped from 257.9 +/- 15.8 mg/dL to 240.6 +/- 10.9 mg/dL; the low-density lipoprotein cholesterol level dropped from 191.8 +/- 17.4 mg/dL to 175.0 +/- 11.7 mg/dL; the high-density lipoprotein cholesterol level dropped from 42.8 +/- 1.94 mg/dL to 41.6 +/- 1.68 mg/dL; and triglyceride concentration rose from 115.9 +/- 13.7 mg/dL to 128.1 +/- 14.1 mg/dL. The 36 patients treated with saturated fat-restrictive diets and supplemental soluble fiber diets had a reduction of total cholesterol levels from 249.2 +/- 7.66 mg/dL to 207.1 +/- 6.31 mg/dL and a low-density lipoprotein cholesterol level reduction from 184.7 +/- 7.55 mg/dL to 142.6 +/- 6.7 mg/dL, a reduction of 18% and 23%, respectively. There was no significant change in high-density lipoprotein cholesterol levels (46.4 +/- 1.9 mg/dL vs 44.3 +/- 2.1 mg/dL) or in triglyceride concentrations (94.2 +/- 7.43 mg/dL vs 102.2 +/- 8.45 mg/dL). In addition, the reduction in serum lipoprotein concentrations resulting from this program was significantly greater than the concentrations resulting from the American Heart Association diet alone. The different responses to these regimens suggest that a simplified diet and soluble fiber supplementation is well tolerated and reduces total and low-density lipoprotein cholesterol levels more effectively than the standard American Heart Association diet in children.  相似文献   

9.
A boy with a total plasma cholesterol concentration of 20.9 mmol/l which fell significantly with a low fat diet, cholestyramine and simvastatin, was shown to have two different mutations in the low density lipoprotein receptor gene, demonstrating that some patients with homozygous familial hypercholesterolaemia show a good lipid lowering response to treatment.  相似文献   

10.
A boy with a total plasma cholesterol concentration of 20.9 mmol/l which fell significantly with a low fat diet, cholestyramine and simvastatin, was shown to have two different mutations in the low density lipoprotein receptor gene, demonstrating that some patients with homozygous familial hypercholesterolaemia show a good lipid lowering response to treatment.  相似文献   

11.
单纯性肥胖儿童外周血单核细胞载脂蛋白E基因的表达   总被引:4,自引:0,他引:4  
Xiang W  Ma YL  Chen C  Fu SM  Yang JF  Zhao SP  Guo DX  Zhao DC  Nie S  Wang FL 《中华儿科杂志》2003,41(10):755-760
目的 探讨单纯性肥胖儿童外周血单核细胞载脂蛋白E基因表达及其与血脂、脂蛋白、载脂蛋白的相关关系。方法 采用竞争性逆转录 聚合酶链式反应方法分析 3 2例单纯性肥胖儿童和 3 2例正常健康儿童外周血单核细胞的载脂蛋白E基因表达。结果 载脂蛋白E基因能在儿童外周血单核细胞表达 ,与健康儿童比较 ,单纯性肥胖儿童外周血单核细胞载脂蛋白E基因表达水平显著下调 (P <0 0 1) ,重度肥胖儿童尤其明显 ,载脂蛋白E基因表达水平与肥胖度呈负相关 (P <0 0 5)。肥胖儿童存在明显的血脂代谢紊乱 ,载脂蛋白E基因表达水平与低密度脂蛋白 胆固醇呈负相关 ,与血载脂蛋白E浓度呈正相关 (P <0 0 5) ,与血总胆固醇、甘油三酯、高密度脂蛋白 胆固醇、脂蛋白 (a)、载脂蛋白AⅠ水平无明显相关 (P >0 0 5)。结论 单纯性肥胖儿童外周血单核细胞载脂蛋白E基因表达水平明显下调 ,并与肥胖程度及血脂代谢异常相关联 ,提示载脂蛋白E基因表达变化可能与肥胖的发生发展及肥胖的心血管病变相关联  相似文献   

12.
Effects of weight reduction on serum levels of lipids and apolipoproteins were measured in 13 obese children (seven girls, six boys). Mean weight loss of 8.4% of the initial body weight was achieved after 4 weeks of energy intake restriction and exercise. Serum total cholesterol (5.46 +/- 1.01 mmol/L) and triglyceride (2.08 +/- 0.52 mmol/L) levels were significantly high compared with control values before treatment and were significantly reduced to 4.32 +/- 0.75 and 1.31 +/- 0.42 mmol/L, respectively, after treatment. Serum high-density lipoprotein cholesterol level (1.03 +/- 0.23 mmol/L) was significantly low and unchanged after treatment (0.94 +/- 0.25 mmol/L). Serum apolipoprotein A-I level (0.039 +/- 0.009 mmol/L or 111 +/- 0.26 g/L) was normal before treatment and significantly reduced, to 0.032 +/- 0.007 mmol/L or 0.92 +/- 0.19 g/L, after weight reduction. Serum apolipoprotein B level (0.00019 +/- 0.00007 mmol/L or 1.07 +/- 0.21 g/L) was significantly high before treatment and decreased to the normal range after treatment (0.00014 +/- 0.0009 mmol/L or 0.76 +/- 0.24 g/L). The ratio of apolipoprotein B to apolipoprotein A-I (1.09 +/- 0.29) was significantly high on admission and decreased significantly to 0.64 +/- 0.12 after treatment. Serum apolipoprotein E level (0.0014 +/- 0.0006 mmol/L or 0.05 +/- 0.02 g/L) was normal and decreased to 0.0008 +/- 0.0002 mmol/L or 0.03 +/- 0.01 g/L after treatment. In conclusion, weight reduction achieved by energy intake restriction and exercise had beneficial effects on serum lipid and apolipoprotein concentrations for the prevention of future atherosclerosis.  相似文献   

13.
Effects of a low-cholesterol, polyunsaturate-rich diet and a synthetic organic bile sequestrant polymer (U26,597A, colestipol) were studied in 21 children, heterozygous for familial hypercholesterolemia. Total cholesterol, beta-lipoprotein cholesterol, and triglyceride were measured twice on habitual diet, monthly for six months on a low-cholesterol diet, and monthly for six months on low-cholesterol diet plus 10 gm of colestipol per day. Total cholesterol (mean +/- 1 SD) was 295 +/- 37 on habitual diet, 278 +/- 29 on low-cholesterol diet, and fell significantly to 242 +/- 29 mg/100 ml on diet plus colestipol. Low-density lipoprotein (LDL) cholesterol was 234 +/- 37 on habitual diet, 220 +/- 28 on low-cholesterol diet, and fell significantly to 179 +/- 26 mg/100 ml on diet plus drug. Plasma triglyceride levels on habitual diet were 79 +/- 31, remained unchanged on low-cholesterol diet, 86 +/- 22, and were unaffected by low-cholesterol diet plus drug, 85 +/- 17 mg/100 ml. On diet alone, plasma LDL was not normalized (less than 170 mg/100 ml) in any of the 21 children, and cholesterol fell to within normal limits (less than 230 mg/100 ml) in only one child. The combination of diet plus colestipol resin normalized total and LDL cholesterol in 52% of the children. Cholesterol was lowered to a "moderately elevated" range of 230 to 250 mg/100 ml in an additional 14% of the children and LDL was lowered to a range of 170 to 190 mg/100 ml in an additional 29%. In 33% of the children, cholesterol remained greater than 250 mg/100 ml despite diet plus colestipol, while LDL was greater than 190 mg/100 ml in 19%. Colestipol is an effective and well-tolerated cholesterol lowering compound which, in conjunction with diet, may prove to be very useful in the treatment of children heterozygous for familial hypercholesterolemia.  相似文献   

14.
Serum cholesterol levels during and after Kawasaki disease   总被引:2,自引:0,他引:2  
Serum total cholesterol and high-density lipoprotein (HDL) cholesterol concentrations were studied in paired sera from 23 patients (16 boys) with Kawasaki disease (KD) during acute illness and in 35 patients (21 boys) 5.4 to 7.7 years after KD. Total cholesterol and HDL cholesterol concentrations were significantly lower (paired t test, p = 0.0001) in samples taken within 30 days of the onset of illness (3.32 +/- 0.85 mmol/L (128 +/- 33 mg/dl) and 0.54 +/- 0.25 mmol/L (20.8 +/- 9.7 mg/dl) than in the second samples taken 2 to 16 months after onset of disease (4.16 +/- 0.93 mmol/L (161 +/- 35 mg/dl) and 1.24 +/- 0.35 mmol/L (47.2 +/- 13.9 mg/dl). The lowest total cholesterol levels were observed in samples taken 6 to 9 days after the onset of KD (p = 0.019). No correlations were seen between the highest erythrocyte sedimentation rate, C-reactive protein, or thrombocyte counts and the acute or convalescent cholesterol levels. In patients studied 5.4 to 7.7 years after recovery from KD, the mean total cholesterol concentrations were still lower than in healthy Finnish children. In girls the HDL cholesterol concentrations were similar, whereas 3 of the 18 boys studied had HDL cholesterol values more than 2 SDs below the mean for healthy boys. There was no correlation between the serum cholesterol concentrations and coronary artery abnormalities. These data lead us to infer that KD does not cause such permanent changes in cholesterol metabolism as to be considered a risk factor for atherosclerosis beyond that caused by the disease itself.  相似文献   

15.
After a period of stabilisation on a controlled low lipid low cholesterol diet with animal proteins a group of 16 children with familial hypercholesterolaemia were given a textured soybean protein based diet, with a similar fat composition. All the children had a highly significant reduction in total cholesterol, averaging -21.8% against the baseline after eight weeks. Compliance became less strict afterwards, but more than half of the patients have regularly continued the diet and results have been maintained for one year. Minimal changes were noted in triglyceridaemia and in high density lipoprotein cholesterol concentrations, which showed a slight rise only at the end of treatment. The children''s growth during the trial was normal. In view of the psychological difficulties of prescribing treatment with drugs to children with severe hypercholesterolaemia before puberty and of the relative ineffectiveness of standard low lipid diets in this condition the soybean protein diet may offer a satisfactory alternative.  相似文献   

16.
The present study was done to evaluate the effect of short-term dietary therapy on 148 dyslipidaemic children (24 familial hypercholesterolaemia, 105 non-familial hypercholesterolaemia and 19 hypertriglyceridaemia), detected by mass screening in children at 18 months of age. In the model diet used for treatment, 15% of the total calories were obtained from protein, 27% from fat and 57% from carbohydrate. Cholesterol intake was set at <200 mg/day and the ratio of polyunsaturated to saturated fatty acid (P/S ratio) was 1.2. When compared to the composition of the diet ingested by the dyslipidaemic children, only the P/S ratio changed from 0.7 to 1.2. During 12 months treatment, levels of total cholesterol, low density lipoproteins cholesterol and apoB decreased by 10%–15% in children with familial and non-familial hypercholesterolaemia. There was no significant change in the levels of high density lipoproteins. In 19 children with hypertriglyceridaemia, the intake of carbohydrate was limited to 55% of the total calories consumed and after 12 months of treatment, triglyceride levels reverted to normal. Throughout the study period, apprimately 70% of the children on this dietary therapy were seen in our clinics every 3–6 months and physical development was within normal ranges. These results, taken together, indicate that dietary therapy can be effective for correcting dyslipidaemia, even in young children.  相似文献   

17.
The prevalence of dyslipidaemia in children with insulin dependent diabetes mellitus (IDDM) and its relation to glycaemic control was studied in a group of 51 diabetic children and a control population of 132 schoolchildren. The prevalence of dyslipidaemia in the fasting state was increased in the diabetic group (39%) compared with control subjects (17%). Serum cholesterol concentration alone was raised in 25% of diabetic subjects while serum cholesterol and triglycerides were raised in 14%, compared with 16% and 0.7% respectively in control subjects. Serum total cholesterol (5.1 v 4.5 mmol/l), low density lipoprotein cholesterol (3.2 v 2.6 mmol/l), non-esterified fatty acids (0.91 v 0.50 mmol/l), and triglycerides (0.94 v 0.76 mmol/l) were higher in diabetic children. Serum total cholesterol, triglycerides, and apolipoprotein (apo)B concentrations increased with worsening control, while serum high density lipoprotein cholesterol and apoA-I concentrations were unaltered. There were also positive correlations between glycated haemoglobin and total cholesterol, triglycerides, and apoB in diabetic children. Thus, abnormalities in circulating lipids are common in young subjects with IDDM but largely disappear if blood glucose concentrations are reasonably controlled.  相似文献   

18.
The prevalence of dyslipidaemia in children with insulin dependent diabetes mellitus (IDDM) and its relation to glycaemic control was studied in a group of 51 diabetic children and a control population of 132 schoolchildren. The prevalence of dyslipidaemia in the fasting state was increased in the diabetic group (39%) compared with control subjects (17%). Serum cholesterol concentration alone was raised in 25% of diabetic subjects while serum cholesterol and triglycerides were raised in 14%, compared with 16% and 0.7% respectively in control subjects. Serum total cholesterol (5.1 v 4.5 mmol/l), low density lipoprotein cholesterol (3.2 v 2.6 mmol/l), non-esterified fatty acids (0.91 v 0.50 mmol/l), and triglycerides (0.94 v 0.76 mmol/l) were higher in diabetic children. Serum total cholesterol, triglycerides, and apolipoprotein (apo)B concentrations increased with worsening control, while serum high density lipoprotein cholesterol and apoA-I concentrations were unaltered. There were also positive correlations between glycated haemoglobin and total cholesterol, triglycerides, and apoB in diabetic children. Thus, abnormalities in circulating lipids are common in young subjects with IDDM but largely disappear if blood glucose concentrations are reasonably controlled.  相似文献   

19.
Twenty-three children, who were detected by neonatal PKU screening, were followed for 8-18 years in one paediatric centre. Dietary treatment was started if the blood phenylalanine level exceeded 0.72 mmol/l. All 23 infants were initially given a low phenylalanine diet. The growth and development rates of the children did not differ significantly from those in a reference population, although one child had mild mental retardation and another had a short attention span. Fourteen children were still on a strict phenylalanine-restricted diet on their last follow-up (at 8-18 years of age). In nine children who were initially put on a low phenylalanine diet, it was possible to normalize the diet between 1/2 and 10 years of age, while maintaining the blood phenylalanine levels between 0.25 and 0.72 mmol/l. It seems likely that those of our patients who markedly increased their phenylalanine tolerance during childhood had a regulatory mutation of the phenylalanine hydroxylase system. A continuous reevaluation of each child treated with a low phenylalanine diet reduces the use of unnecessarily restricted diets.  相似文献   

20.
Hyperglycemia and hypoinsulinemia in patients with Fanconi-Bickel syndrome   总被引:1,自引:0,他引:1  
Fanconi-Bickel syndrome (FBS) is a rare autosomal recessive disorder characterized by the combination of hepatorenal glycogen accumulation and Fanconi-type nephropathy. Mutations in GLUT2, the gene for facilitative glucose transporter protein 2 (GLUT2), cause FBS. AIM: To evaluate glucose and insulin responses to oral glucose load in patients with FBS. METHODS: Ten children (7.3 +/- 4.8 years) diagnosed with FBS in early infancy underwent a standard oral glucose tolerance test (OGTT); plasma glucose (PG) and serum insulin concentrations were measured at 30-min intervals for 2 hours. HbA1c, insulin-like growth factor-I, and fasting lipid profiles were also measured. RESULTS: Mean fasting and 2-h PG concentrations were 3.8 +/- 0.9 mmol/l and 8.6 +/- 3.0 mmol/1, respectively. 2-hour PG levels were above 11.1 mmol/l in two patients (20%) and between 7.75 and 11.1 mmol/ in four patients (40%). HbA1c was normal in all the patients with a mean of 5.4 +/- 0.3%. Mean fasting and peak serum insulin levels were 8.7 +/- 0.8 pmol/ and 98.6 +/- 43.0 pmol/l, respectively, and did not differ between the patients with normal and abnormal OGTT. Patients with abnormal OGTT were younger (4.8 +/- 3.2 vs 11.0 +/- 4.8 yr; p = 0.04). Fasting PG increased with age (r = 0.80, p < 0.01). Total and LDL cholesterol as well as triglyceride concentrations were elevated. CONCLUSIONS: Most but not all patients with FBS have impaired glucose tolerance/diabetes range hyperglycemia after OGTT while maintaining normal HbA1c. Patients with FBS are relatively hypoinsulinemic. Both fasting hypoglycemia and post-OGTT hyperglycemia seem to improve with age.  相似文献   

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