Family History Of Hyperlipidemia Research Articles

Application of the back-error propagation artificial neural network (BPANN) on genetic variants in the PPAR-γ and RXR-α gene and risk of metabolic syndrome in a Chinese Han population

This study was aimed to explore the associations between the combined effects of several polymorphisms in the PPAR-γ and RXR-α gene and environmental factors with the risk of metabolic syndrome by back-error propagation artificial neural network (BPANN). We established the model based on data gathered from metabolic syndrome patients (n = 1012) and normal controls (n = 1069) by BPANN. Mean impact value (MIV) for each input variable was calculated and the sequence of factors was sorted according to their absolute MIVs. Generalized multifactor dimensionality reduction (GMDR) confirmed a joint effect of PPAR-γ and RXR-α based on the results from BPANN. By BPANN analysis, the sequences according to the importance of metabolic syndrome risk factors were in the order of body mass index (BMI), serum adiponectin, rs4240711, gender, rs4842194, family history of type 2 diabetes, rs2920502, physical activity, alcohol drinking, rs3856806, family history of hypertension, rs1045570, rs6537944, age, rs17817276, family history of hyperlipidemia, smoking, rs1801282 and rs3132291. However, no polymorphism was statistically significant in multiple logistic regression analysis. After controlling for environmental factors, A1, A2, B1 and B2 (rs4240711, rs4842194, rs2920502 and rs3856806) models were the best models (cross-validation consistency 10/10, P = 0.0107) with the GMDR method. In conclusion, the interaction of the PPAR-γ and RXR-α gene could play a role in susceptibility to metabolic syndrome. A more realistic model is obtained by using BPANN to screen out determinants of diseases of multiple etiologies like metabolic syndrome.

A case of Hypertriglyceridemia (? Transient Infantile Hypertriglyceridemia) with associated Hyperglycinemia and Carnitine deficiency.

Hypertriglyceridemia is an emerging risk factor for atherosclerosis, coronary heart disease and pancreatitis in the developing countries like India. It can be familial or acquired. Hypertriglyceridemia associated with hyperglycinemia and carnitine deficiency is rarely seen. We report a case of 3 months old infant with low birth weight admitted for failure to thrive and lower respiratory tract infection. He was screened for hypertriglyceridemia and inborn error of metabolism. Serum was lipemic with triglyceride level-1236 mg%, Cholesterol-220 mg%, Amino acid Glycine(789 umol/L) was elevated, Fatty acids like Acetylcarnitine( 4.16 umol/L), Octadecanoyl carnitine(0.16 umol/L) were found to be decreased. There is no family history of hyperlipidemia or consanguineous marriage. History, clinical examination and blood reports points towards transient infantile hypertriglyceridemia. Reasons for high glycine level and carnitine deficiency could not be established. Further investigations like mass spectrometry and mutational screening for genetic defects are necessary for confirmatory diagnosis. Meanwhile the infant was treated with lipid lowering agents, medium chain fatty acids and carnitine supplements.

Universal pediatric lipid screening: preventing future cardiovascular disease in high-risk children

Case 1: 11-year-old boy at a routine well-child visit A review of systems is negative. He plays three sports and maintains an optimal diet through his mother’s efforts and, recently, his own volition. His paternal grandfather died at age 40 years in a work-related accident. There is no known family history of lipid disorders. Examination shows normal BMI, normal blood pressure and no other abnormalities. Upon lipid screening, he is noted to have nonfasting non-HDL-C of 255 mg/dl. Upon fasting lipid profile, his LDL-cholesterol (LDL-C) is 240 mg/dl. His parents are subsequently tested and his 33-year-old father is noted to have a LDL-C of 250 mg/dl. In this patient, there were no features that would have triggered concerns for hyperlipidemia, such as a family history of hyperlipidemia or early atherosclerotic events. Despite an optimal lifestyle, he has dramatically elevated LDL-C due to a genetic dyslipidemia, heterozygous familial hypercholesterolemia (FH). The approximately 1 in 500 children born with FH demonstrate persistently elevated LDL-C concentrations of at least 160 mg/dl and typically over 200 mg/dl. Approximately 50% of affected men and 20% of affected women experience coronary heart disease events by the age of 50 [2,3]. Multiple studies have shown that relying on a family history of CVD events or hyperlipidemia to trigger screening is insufficiently sensitive and may miss 30–60% of affected children owing to a lack of knowledge about the family history, lack of In November 2011, after a systematic review of the relevant literature, the National Heart Lung, and Blood Institute (NHLBI) Expert Panel on Integrated Guidelines for Cardiovascular Health and Risk Reduction in Children and Adolescents published evidence-based, integrated guidelines on preventing cardiovascular disease (CVD) by reduction of athero sclerotic disease risk factors in children and adolescents [1]. These guidelines encourage the broad adoption of healthy behavioral patterns, beginning in childhood, to prevent the development of athero sclerotic CVD risk factors – so-called ‘primordial prevention’. The guidelines endorse a healthy diet and activity patterns at a population level, as well as the individual practitioner–child–family unit as cornerstones of CVD prevention. Predicated upon evidence linking childhood CVD risk factors to adult risk factor prevalence, subclinical athero sclerosis and CVD events, the panel also comprehensively updated and rationally extended a number of previous guidelines advocating individual -level identification and management of CVD risk factors including nutrition, physical activity, tobacco exposure, elevated blood pressure, obesity, diabetes mellitus and metabolic syndrome. One significant extension pertained to lipid disorders, where the panel recommended screening of all children once between 9 and 11 years of age with a nonfasting non-HDL-cholesterol (HDL-C). This recommendation was designed to identify those children with extreme lipid abnormalities, usually due to a genetic dyslipid emia, in whom the risk of early CVD is established and treatment is available. When abnormal values are confirmed, identified children should be treated with targeted nutritional changes, physical activity and, when

The use of plasmapheresis in managing primary biliary cirrhosis presenting with profound hypercholesterolaemia

Case history A 39-year-old woman had a fasting lipid profile after a wellwoman check in March 2005. Her TC was 44.9 mmol/L, TG 15.9 mmol/L (normal range 0.6–1.5mmol/L), HDL cholesterol 0.33 mmol/L (normal range 0.9–2.4), TC:HDL ratio 36.1. Other results included fasting glucose 5.7 mmol/L, urea 3.6 mmol/L, creatinine 63 μmol/L, potassium 3.6 mmol/L and sodium 125 mmol/L, consistent with pseudohyponatraemia due to the lipaemic sample. She was asymptomatic with no prior medical illnesses. There was no significant family history of hyperlipidaemia or vascular disease. She did not smoke but drank 20 units of alcohol per week. Pravastatin 20 mg was commenced but stopped a week later because of a generalised rash. She was referred to endocrinology as this was before the establishment of a lipid service. Over the next 10 weeks, she became gradually more unwell with fatigue, widespread itching and easy bruising. She lost 10 kg and became jaundiced. On examination, she had palpable hepatosplenomegaly. No stigmata of chronic liver disease or hyperlipidaemia were found. Bloods showed a TC 83 mmol/L, TG 16.3 mmol/L, HDL 0.46, TC:HDL ratio 181.5. Her LFTs were ALP 1589 iu/L (30–95), AST 220 iu/L (12–40), bilirubin 58 μmol/L (3–20), total protein 81 g/L (63–81), albumin 33 g/L (37–50). Other results were sodium 107 mmol/L, haemoglobin 12.1 g/dL (12–16) and INR 0.9 (0.8–1.2). Abdominal ultrasound showed hepatomegaly, marked splenomegaly of 19 cm, with patent portal vein, hepatic veins and arteries. Further blood results revealed bile acids 363 μmol/L (0–20); hepatitis B and C serology were negative. Her ANA and anti-LKM antibodies were negative but she was moderately positive for anti-SmM and AMA. Thyroid function was normal. Serum electrophoresis showed polyclonal increase in gamma region with uninterpretable immunoglobulin levels due to the lipaemia. In view of the risk of hyperviscosity syndrome, she was started on plasmapheresis, continuing as regular fortnightly sessions. Cholestyramine 4 g four times a day and UDCA 250 mg twice daily were commenced. The use of plasmapheresis in managing primary biliary cirrhosis presenting with profound hypercholesterolaemia

Analysis of sequence variations in low-density lipoprotein receptor gene among Malaysian patients with familial hypercholesterolemia

BackgroundFamilial hypercholesterolemia is a genetic disorder mainly caused by defects in the low-density lipoprotein receptor gene. Few and limited analyses of familial hypercholesterolemia have been performed in Malaysia, and the underlying mutations therefore remain largely unknown.We studied a group of 154 unrelated FH patients from a northern area of Malaysia (Kelantan). The promoter region and exons 2-15 of the LDLR gene were screened by denaturing high-performance liquid chromatography to detect short deletions and nucleotide substitutions, and by multiplex ligation-dependent probe amplification to detect large rearrangements.ResultsA total of 29 gene sequence variants were reported in 117(76.0%) of the studied subjects. Eight different mutations (1 large rearrangement, 1 short deletion, 5 missense mutations, and 1 splice site mutation), and 21 variants. Eight gene sequence variants were reported for the first time and they were noticed in familial hypercholesterolemic patients, but not in controls (p.Asp100Asp, p.Asp139His, p.Arg471Gly, c.1705+117 T>G, c.1186+41T>A, 1705+112C>G, Dup exon 12 and p.Trp666ProfsX45). The incidence of the p.Arg471Gly variant was 11%. Patients with pathogenic mutations were younger, had significantly higher incidences of cardiovascular disease, xanthomas, and family history of hyperlipidemia, together with significantly higher total cholesterol and low density lipoprotein levels than patients with non-pathogenic variants.ConclusionsTwenty-nine gene sequence variants occurred among FH patients; those with predicted pathogenicity were associated with higher incidences of cardiovascular diseases, tendon xanthomas, and higher total and low density lipoprotein levels compared to the rest. These results provide preliminary information on the mutation spectrum of this gene among patients with FH in Malaysia.

Measurement of lipid profile parameters in hypertensivepatients using atenolol or captopril

Objectives: To study the effects of atenolol and captopril on lipid profile parameters including total cholesterol (TC), triglycerides (TG), High density lipoprotein cholesterol (HDL-c), Low density lipoprotein cholesterol (LDL-c) and atherogenic index (AI) in serum. Moreover to compare the effects of these drugs on the above parameters with each other.Patients and Methods: One hundred hypertensive patients were involved in this study which were divided into two groups each of 50 patients. Patients in the first group were on atenolol and the patients in the second group were on captopril. All of the cases of hypertension were of the primary type (essential) as the patients were diagnosed by specialist physicians. The patients included were not chronically using any other drugs, nor having family history of hyperlipidemia, and not suffering from any other chronic disease. The ages of the patients in the first group ranged from 35-74 years with a mean of 55±5.02 years, while the ages of the second group ranged from 36-80 years with a mean of 57±6.0 years. Another group of 50 normal individuals participated in this study as a control group, with ages ranged from 35-72 years with a mean of 53±4.4 years. Results: The results of this study showed that serum TG and AI were significantly higher in atenolol using group in comparison with the control group, while serum HDL-c concentration was significantly lower. Whereas, the remaining lipid profile parameters studied were not significantly different from the control group. Serum LDL-c concentration was significantly low in captopril using group compared with the control group whereas the remaining lipid parameters studied were not significantly changed in this group. Further analysis of the results of the present study indicated significant decreases of serum TG and AI in captopril using group in comparison with Atenolol using group. Whereas, serum HDL-c concentration was significantly higher in captopril using group. Conclusion: The overall analysis of the lipid profile parameters studied might suggest that atenolol has certain undesirable effects on these parameters while captopril has less undesirable effects. This might indicate that captopril seems to be more suitable antihypertensive agent than atenolol for patients with lipid profile abnormalities.Keywords: Serum, lipid profile, triglyceride, total cholesterol, HDL-c, LDL-c, atherogenic index, hypertension.

Association between 24-hour ambulatory blood pressure and erythrocyte n-3 polyunsaturated fatty acids in Korean subjects with hypertension

N-3 polyunsaturated fatty acids (PUFAs) are known to have antihypertensive properties, but the association between 24-hour ambulatory blood pressure and the tissue content of n-3 PUFA remains controversial. The purpose of the present study was to investigate the hypothesis that the level of erythrocyte n-3 PUFA is inversely related with 24-hour ambulatory blood pressure after adjustment for relevant confounders. Fifty-one male and 49 female Korean patients were included in this study. Twenty-seven of the patients were defined as having hypertension. There were significant differences in age, body mass index, sex, marital status, and family history of hyperlipidemia between hypertensive and nonhypertensive subjects, and these factors were therefore considered to be confounding factors. Multivariate-adjusted regression analysis showed that erythrocyte fatty acids were not significantly associated with the risk of hypertension after adjusting for confounders. However, Pearson correlation analysis showed that 24-hour ambulatory systolic blood pressure (SBP) was significantly and negatively correlated with n-3 PUFA (r = -0.228, P = .027) and eicosapentaenoic acid (r = -0.270, P = .008), but not with docosahexaenoic acid (r = -0.156, P = .131). Multivariate-adjusted regression analysis also showed that intake of protein, vitamin B(2), vitamin E, and cholesterol increased the risk of hypertension after adjusting for confounders. In addition, Pearson correlation analysis showed that fat and cholesterol consumption was positively correlated with SBP, but carbohydrate intake was negatively correlated with SBP. In conclusion, erythrocyte n-3 PUFA did not reduce the risk of hypertension but were negatively correlated with 24-hour ambulatory SBP in the Korean population.

SHA 39. Selected risk factors of cardiovascular diseases among adolescent in Ahad Rufeda southwestern region

Objectives: To study the selected cardiovascular disease risk factor among adolescent school boys in Ahad Rufeda, southwestern, Saudi Arabia. Methods: A cross sectional study was conducted on a sample of students covering 1249 from six secondary schools in Ahad Rufeda Region. the sample of six school was selected using proportional allocation to the of school (governmental or private) and geographical location (urban or rural) during March 2008. Two or three classes from each educational level were randomly chosen in the selected schools. The structured questionnaire was which was used to collect the data was the Arabic version of CDC adolescent health survey, validated and adopted in a previous study in Abha, having information on socio-demographic condition, family history of cardiovascular diseases, smoking habits, behaviors related to physical activity, food choices, as well as, direct measurement of weight, height, waist circumference, systolic and diastolic blood pressure. Waist and height measurement ; were taken to calculate the body mass index (BMI). BMI was classified into four categories: normal (BMI < 25) pre-obese (BMI 25 6 40) obese grade III (+40). Waist circumference was measured at the point of the trunk located midway between the lower costal margin (bottom of lower rib) and iliac crest (tap of pelvic bone) while the person was standing. It was measured to the nearest of the 0.5 cm, at the end of normal expiration. Standard techniques was used to measure the blood pressure. Two or more reading separated by at least 5 min were averaged. student having SBP (systolic blood pressure) over 130 or DBP (diastolic blood pressure) over 85 were considered as having high normal blood pressure. Results: Study shows that 23.4% of students has BMI above 25 and 14.1% has central obesity. About 21.5% of student had SBP over the normal while 19.7% had an increase in DPB – prevalence of smoking was eating found to 11.8% ; cigarette smoking account for 70.7% were 23.1% were shisha smoker ; and 18.4% had experience of smokingothermaterials. About 50%of the studentwere eating healthy food;whilemore than40%were eating junkor fast food. The assessment of physical activity revealed that 25%of the student were not engaged in any type of exercise activity. Family history of hypertensionwas highest among the father with 21.9%aswell as for diabetes mellitus, father were higher 25.6%. Regarding family history of hyperlipidemia it was highest among the father with 33.7% Conclusion: The study has brought out clearly the prevalent risk factors of cardiovascular disease among the student. A general health awareness should be organized at the school level. Tracks: Adult Cardiology.

Paediatric necrotizing pancreatitis

A 13-year-old boy was admitted with a 7-week history of worsening epigastric pain and vomiting. There was no prior history of similar episodes, jaundice or viral infection. There was no family history of hyperlipidaemia. His only comorbidity was obesity with a body mass index of 36.6 kg/m2. On examination he was tachycardic and tachypnoeic with a tender epigastrium. Blood results showed a leucocytosis, normal liver function tests and normal cholesterol, C-reactive protein and amylase levels. An ultrasound scan was performed but did not reveal any detail because of his body habitus. A computed tomography scan showed a necrotic pancreas and that gallstones were present. The patient deteriorated, becoming increasingly septic, and was transferred across to a paediatric intensive care unit. Initial management consisted of fluid resuscitation, intravenous protein pump inhibitors, intravenous antibiotics, parenteral nutrition and surgical review. He initially responded to treatment, but then deteriorated with worsening sepsis requiring readmission to the paediatric intensive care unit. The repeat computed tomography scan (Figure 1) revealed bilateral pleural effusions, free fluid in the abdomen and peripancreatic inflammation. He showed signs of abdominal compartment syndrome and was taken to theatre for a laparotomy. The operative findings were a necrotic pancreas with over 5 litres of free fluid. A pancreatic necrosectomy and laparostomy was performed (Figure 2). Between 60 and 70% of his pancreas was involved and removed. The rest of his postoperative course was uneventful. He was discharged on Creon supplements, insulin and an antihypertensive. His weight had dropped from 106 kg on admission to 84 kg. After further surgical and paediatric review as an outpatient he later underwent an open cholecystectomy with an ontable cholangiogram.

The clinical utility of screening of biochemical parameters in elite athletes: analysis of 100 cases

Objective:To determine the clinical utility of screening for biochemical parameters in elite athletes.Design:A prospective sequential case series.Setting:The Department of Sports Medicine at the Australian Institute of Sport.Participants:100 elite athletes from...

Coronary risk factors in school children in relation to their family history of coronary heart disease and hyperlipidemia.

A school-based study was implemented to assess the family history of coronary heart disease (CHD) and hyperlipidemia (HL) in relation to serum lipoprotein and apolipoprotein levels. One hundred and twenty-five elementary school students (aged 9-10 years) and 297 junior high school students (aged 12-13 years) participated. Family history was evaluated by the following scoring method: positive family history in a parent, 2 points; in a grandparent, 1 point; and onset of CHD before age 60, 1 additional point. Family history of HL was positive in 8.2% of elementary school students, and 4.2% in junior high school students. Family history of CHD was positive in 11.5% of elementary students, and 11.0% in junior students. Family history score (FHS) for HL was related to serum total cholesterol (TC), low density lipoprotein cholesterol (LDLC), high density lipoprotein cholesterol, apolipoprotein A-I, apolipoprotein B (apoB) and lipoprotein (a) in elementary students, and to TC, LDLC, triglyceride and apoB in junior students. There was no relationship between FHS for CHD and serum lipoprotein or apolipoprotein levels in any student. The children with a positive FH of HL already demonstrated an atherogenic lipid profile while those with FH of CHD did not, which was probably because lipid profiles in children are more genetically mediated by a FH of HL than of CHD.

Long-Term Course of Lipoprotein Lipase (LPL) Deficiency Due to Homozygous LPLAritain a Patient with Recurrent Pancreatitis, Retained Glucose Tolerance, and Atherosclerosis

Lipoprotein lipase (LPL) deficiency is a rare autosomal recessive disorder caused by LPL gene mutation and is characterized by severe hyperchylomicronemia. Patients with LPL deficiency suffer from the frequent recurrence of acute pancreatitis, but the underlying mechanisms are not fully understood. A 22-yr-old male Japanese patient with severe hyperchylomicronemia was admitted to our hospital in 1973. He had no consanguinity and no family history of hyperlipidemia. He was genetically diagnosed as LPL deficiency (homozygous for LPL(Arita)) with no LPL mass or activity in postheparin plasma. He has experienced recurrent acute pancreatitis 22 times during our 31-yr clinical follow-up, but no pancreatic pseudocyst, irregularity of the pancreatic duct, or abnormal pancreatic calcification was observed in computed tomography. Moreover, his pancreatic endocrine function, as assessed by the oral glucose tolerance test, has preserved more than 30 yr. Although he was a current smoker, no clinically significant atherosclerotic lesion had been observed. From the long-term observation of this patient, we propose that LPL deficiency is not invariably associated with high mortality and that even with repeated episodes of acute pancreatitis, pancreatic function may be slow to decline.

Remember the Endocrine System When Using Psychoactive Drugs

Remember the Endocrine System When Using Psychoactive Drugs

Is hypertriglyceridaemia a new concept for visceral leishmaniasis?

"Is hypertriglyceridaemia a new concept for visceral leishmaniasis?." Annals of Tropical Paediatrics, 24(4), p. 369

Pinpoint skin lesions in a familial hypercholesterolaemia homozygote

The case is reported of a 2-y-old girl referred to the outpatient lipid clinic because of a tiny cutaneous xanthoma on the dorsum of the left foot and a family history of hyperlipidaemia and coronary heart disease (CHD). Fasting serum total cholesterol levels were remarkably high (27.1 mmol l(-1), 1050 mg dl(-1)) and DNA analysis confirmed homozygous familial hypercholesterolaemia (class II mutation). Serum lipids were not affected by dietary intervention and cholestyramine treatment, so low-density lipoprotein apheresis was scheduled to commence at the age of 4 y. An early lipid profile determination should be performed in children with a family history of premature CHD, since the physical examination may be unremarkable even in cases of severe hyperlipidaemia during the first years of life.

O uso de anti-retrovirais em gestantes modifica o perfil lipídico?

OBJETIVO: avaliar o efeito das medicações anti-retrovirais sobre o metabolismo lipídico em gestantes portadoras do HIV. MÉTODOS: estudo prospectivo realizado em 57 gestantes que foram divididas em três grupos: grupo AZT, com 20 pacientes portadoras do HIV em uso de AZT; grupo TT, com 25 pacientes portadoras do HIV em uso de esquemas contendo três anti-retrovirais (AZT + 3TC + NFV) e grupo controle, com 12 pacientes. Os dados demográficos e antropométricos foram homogêneos entre os grupos estudados. Foram excluídas as pacientes com antecedentes pessoais ou familiares de hiperlipidemia. Amostras sanguíneas foram obtidas para avaliação dos lipídeos (colesterol total, frações LDL e HDL-colesterol e triglicerídeos) em quatro oportunidades durante a gravidez (1º = 14-20 semanas; 2º = 21-26 semanas; 3º = 27-32 semanas e 4º = 33-38 semanas). Os dados foram analisados utilizando-se os testes não paramétricos do chi², teste de Friedman e teste de Kruskal-Wallis. RESULTADOS: o uso de anti-retrovirais durante a gestação não induziu diferenças nas taxas do colesterol total e HDL, no entanto, o uso destes agentes esteve associado a elevação de 76,5 mg/dL e 84 mg/dL para 96 mg/dL e 105 mg/dL na concentração da fração LDL-colesterol para os grupos AZT e TT, respectivamente (p<0,01). Observou-se associação positiva significante entre os triglicerídeos e a carga viral nas gestantes do grupo AZT (r = 0,53; p = 0,015). CONCLUSÃO: a utilização dos anti-retrovirais durante a gestação eleva significativamente a concentração da lipoproteína LDL. Persistem as dúvidas se a gestação potencializa a longo prazo os efeitos dos anti-retrovirais sobre o metabolismo lipídico.

Severe hyperlipidemia-associated pregnancy: prevention in subsequent pregnancy by diet.

Severe hyperlipidemia-associated pregnancy: prevention in subsequent pregnancy by diet.

Risk factors for hyperlipidemia in long-term pediatric renal transplant recipients.

Hyperlipidemia (HL) is a common problem in adult renal transplant (TP) recipients, contributing to an increased risk of cardiovascular disease and chronic TP nephropathy. There are multiple causes of HL post renal TP in adult patients, including pre TP HL, immunosuppressive agents, renal dysfunction, hypoalbuminemia secondary to nephrotic syndrome, obesity, and conditions that lead to end-stage renal disease (ESRD). We evaluated the incidence and risk factors of HL in 62 pediatric renal TP recipients (15.4+/-4.2 years, range-3.0-22.3 years) with long-term (6.7+/-3.1 years) functioning [glomerular filtration rate (GFR) 66.7+/-23.2 ml/min per 1.73 m(2)] allografts. The mean serum cholesterol (C) level was 205. 5+/-43.6 mg/dl. Thirty-two patients (51.6%) exhibited elevated serum C levels. The mean serum triglyceride (TG) level was 157.3+/-88.4 mg/dl. Serum TG levels were elevated in 32 patients (51.6%). In patients with elevated serum levels of either C or TG, the mean low-density lipoprotein level (LDL) was 138.6+/-44.1 mg/dl (normal <130 mg/dl) and the high-density lipoprotein (HDL) level 54.6+/-15.9 mg/dl (normal>34 mg/dl). Of those patients studied, 45.5% had high LDL levels, whereas 9.1% exhibited low HDL levels. The two risk factors for elevated serum C levels in our patient population were pre-TP HL and increased years since TP. The only risk factor for elevated serum TG levels was reduced GFR. A family history of HL had a significant deleterious impact upon serum levels of C (P=0.01), but did not affect serum TG levels (P=0.7). Years on dialysis prior to TP, history of prior TP, gender, body mass index, and disease leading to ESRD had no influence upon the development of post-TP HL. We conclude that post-renal TP HL is a significant problem in pediatric renal TP recipients.

The advisability of implementing cholesterol screening in school-age children and adolescents with a family history of cardiovascular disease and hyperlipidaemia.

The family basis of coronary heart disease is well recognized and it is important for family physicians to assess whether children have elevated cholesterol levels. We aimed to evaluate the advisability of implementing cholesterol screening in children with a family history of cardiovascular disease and hyperlipidaemia. We conducted a cross-sectional study in Taiwan from February to June 1996. There were 47,800 students in the population. A total of 4520 students were recruited by two-stage sampling. All the participants were required to fill out a structured questionnaire. The response rate was 92.5%. Our results show that 16-18% of the children had a positive family history of cardiovascular disease or hyperlipidaemia. Children with a family history of hyperlipidaemia were significantly more likely to have elevated total cholesterol and low-density lipoprotein cholesterol than those without such a history (both odds ratios: 1.4, P < 0.05). Positive predictive values of hyperlipidaemia were less than 13% based on family history. More than 75% of children with abnormal lipid levels would be missed. The data suggest that parents' self-reported family history is an ineffective means of identifying children with elevated serum lipid levels in Taiwan. Further research and modification of current National Cholesterol Education Program Panel guidelines for selective cholesterol screening in children may be warranted.

Muir-Torre syndrome associated with a family history of hyperlipidemia

The Muir-Torre syndrome is a rare disorder characterized by sebaceous neoplasms of the skin and multiple visceral malignancies. The syndrome appears to be a familial, autosomal dominant condition. We diagnosed this syndrome in a previously unreported patient and found a personal and family history of malignancies and hyperlipidemia. The association of Muir-Torre syndrome with a family history of hyperlipidemia, another autosomal dominant condition, has not been previously reported. The possible genetic relationship between the two disorders is discussed.