An investigation to determine the nutritional adequacy and individuals experience of a very low fat diet used to treat type V hypertriglyceridaemia.

BACKGROUND: The management of Type V hypertriglyceridaemia at some centres involves the use of a very low fat diet (15% energy as fat). There is no published research examining compliance with, or nutritional adequacy, of this treatment. This study assesses the nutritional adequacy of the diet and examines barriers and enablers to adherence. METHODS: Fifty-four eligible patients were invited to take part. Eight males and one female agreed to participate. One male later withdrew. The mean age of participants was 49.4 years (SD 12.9 years), their mean BMI was 30.4 kg m(-2) (SD 3.4 kg m(-2)) and their mean triglyceride level was 8.5 mmol L(-1) (SD 5.6 mmol L(-1)). To assess nutritional adequacy and compliance, 3-day dietary records and telephone based diet histories were used and analysed using CompEat. Patients' experience was investigated using semi-structured telephone based qualitative interviews. Qualitative data was recorded, transcribed and analysed using thematic analysis to allow elucidation of emerging themes. RESULTS: Fat accounted for 22.5% of dietary energy and compliance was considered difficult. Barriers included lack of accessible nutritional information, increased patient burden defined as inconvenience and the persistent awareness of potential or actual ill health, lack of appropriate food choices, other peoples' ignorance, lack of flavour and variety, a desire to broaden the palate, cost, social pressure and prior negative experiences with dietitians. Enablers to compliance included nutritional awareness, desire to maintain good health, building on their nutritional knowledge base, behaviour and lifestyle modification, developing a routine, the support of family and friends and supportive eating environments. CONCLUSION: Compliance could be improved through extensive education on labelling, eating during special occasions such as Christmas, birthdays and eating out.

Dietary fat clearance in type V hyperlipoproteinaemia secondary to a rare variant of human apolipoprotein E: the apolipoprotein E3 (Arg 136-->Ser)

This present case report describes two siblings with severe type V hyperlipoproteinaemia, diagnosed very early in life and due to the combination of the common apolipoprotein (Apo) E2 allele and rare mutant variant of ApoE, ApoE3 (Arg 136-->Ser). Phenotyping of ApoE falsely identified E2/E2 phenotype. The presence of mutated ApoE was suspected on an unusual restriction polymorphism of a Hha 1 restriction site and confirmed by sequence analysis of the cloned polymerase chain reaction fragment of exon 4 and familial segregation study. The severity of the hypertriacylglycerolaemia was modulated by the lipid content of the diet. A low-fat diet enriched in medium-chain triacylglycerol (TAG) decreased but did not normalize plasma TAG levels in both affected patients of the pedigree. A standardized lipid-enriched test meal showed a marked impairment of TAG-rich lipoprotein (TRL) clearance, especially the exogeneous TRL bearing ApoB-48 which still represented 79% of total TRL 7 h after the fat load. Finally, differences between the male and female siblings with the existence of a consanguine relationship in their parents suggested the involvement of other genetic factors in modulating the severity of phenotypic expression. This observation reinforces the usefulness of genotyping of ApoE for the characterization of genetic hypertriacylglycerolaemia and selection of the appropriate diet and treatment.

Dyslipidaemia in a boy with recurrent abdominal pain, hypersalivation and decreased lipoprotein lipase activity.

UNLABELLED: An 8-year-old boy with frequently recurring pancreatitis-like abdominal pain, Fredrickson type V dyslipidaemia, and significantly decreased post-heparin plasma lipoprotein lipase (LPL) activity is described. In order to exclude familial LPL deficiency, the complete LPL coding gene sequence was analysed revealing compound heterozygosity for two mutations (Asp9Asn, Ser447Ter) which are not supposed to considerably impair lipolytic enzyme activity. However, until now the combination of both these mutations in one patient has not been observed. In addition to the common symptoms of LPL deficiency, a striking feature of unknown origin was hypersalivation. Treatment including a fat-restricted diet, omega-3 fatty acids, and nicotinic acid led to long symptoms-free intervals. Symptoms recurred however when the diet was not strictly adhered to. CONCLUSION: LPL deficiency is a rare cause of abdominal pain in childhood and deserves careful treatment in order to avoid pancreatitis. The presented patients is a unique compound heterozygote for two mutations which do not abolish lipolytic activity in the homozygote state. Identification of other individuals with this genotype is necessary to understand the phenotype in our patient.

Hypertriglyceridemia: severe type V hyperlipidemia in a young woman.

We present a patient with the typical clinical and biochemical features of severe Type V hyperlipidemia associated with alcohol consumption and estrogen use. Prompt medical intervention resulted in normalization of her lipid profile. We review the lipoprotein composition, the role of lipoproteins in lipid transport with special emphasis on triglycerides, and the clinical features, pathogenesis, and management of Type V hyperlipidemia.

A possible contribution of decrease in free fatty acids to low serum triglyceride levels after diets supplemented with n-6 and n-3 polyunsaturated fatty acids.

Intraindividual comparisons of diets supplemented with sunflowerseed oil (rich in linoleic acid, LA, C18:2n-6), linseed oil (enriched with alpha-linolenic acid, LNA, C18:3n-3) and canned mackerel (rich in eicosapentaenoic acid, EPA, C20:5n-3 and docosahexaenoic acid, DHA, C22:6n-3) were made in 30 patients with primary hyperlipoproteinemia (HLP) of phenotypes IIa (n = 9), IIb (n = 7), IV (n = 7) and V (n = 7). The lipid- and blood pressure-lowering effects of polyunsaturated fatty acids (PUFA), particularly those of the EPA- and DHA-rich diet, were confirmed irrespective of the type of HLP. Apolipoproteins A-I and B remained unchanged. The most remarkable finding was a substantial depression of free fatty acids (FFA) within a standardized glucose tolerance test (GTT) associated with the fall of serum triglycerides after diets enriched with n-6 and especially after those supplemented with n-3 PUFA. It was suggested that the decrease of FFA indicates reduced peripheral lipolysis, which might be a hitherto ignored factor involved in the triglyceride-lowering action of n-6 and, more pronounced, of n-3 PUFA.

Tuberoeruptive xanthomas: unusual presentation of type V hyperlipoproteinemia.

We report a case of pedal tuberoeruptive xanthomatosis associated with ethanol-induced type Vhyperlipoproteinemia that was controlled with diet, withdrawal from ethanol use, and the fibric acid derivative gemfibrozil.

Dietary therapy for different forms of hyperlipoproteinemia.

Diet is the first line of therapy for hypercholesterolemia. The major dietary factors raising the plasma cholesterol are saturated fatty acids, cholesterol, and excess total calories. For almost all forms of hyperlipidemia, the first principle of dietary therapy is to reduce saturated fatty acids, decrease cholesterol, and curtail excess calories. In patients with severe hypercholesterolemia, marked restrictions of diet may be necessary. For these patients, drugs may be required to control cholesterol levels. However, the majority of patients with elevated plasma cholesterol can achieve a satisfactory reduction of cholesterol levels by diet, and drugs will not be necessary. Dietary therapy alone is adequate for most patients with familial forms of hypertriglyceridemia, but for a few patients drugs are required.

Morphometric study of human hepatic cell modifications induced by fenofibrate.

We have studied liver biopsies obtained in 12 hyperlipoproteinemic (HLP) patients (type II, 6; type IV, 6) treated with diet and fenofibrate, and in 15 patients (type II, 11; type IV, 4) receiving diet only. Electron microscopy of liver biopsies and the morphometric analysis according to the method of Weibel and Rohr showed mitrochondrial changes in patients treated with fenofibrate, these changes depending on the type of hyperlipoproteinemia. In type II HLP, we found a decreased volume of normal mitochondria (fenofibrate, 125.72 +/- 17.04 X 10(-3) cm3/cm3; diet only, 185.84 +/- 8.96 10(-3), P less than .05). In type IV HLP we found a decreased number of giant mitochondria (fenofibrate, 0.08 +/- 0.03 X 10(10) cm-3; diet only, 0.32 +/- 0.08 X 10(10) cm-3, P less than .05) and a decreased volume of altered mitochondria (fenofibrate, 6.00 +/- 1.44 X 10(-3) cm3/cm3; diet only, 13.61 +/- 1.17 X 10(-3), P less than .05). In contrast with the rodent studies, the present study shows no change in the number of volume of peroxisomes.

Lipoprotein modifications during dietary treatment in patients with primary type V hyperlipoproteinaemia.

Type V hyperlipoproteinaemia is a disorder of lipid transport characterized by the accumulation in serum of chylomicrons and very low density lipoproteins. The purpose of the study was the analysis of serum lipids and lipoproteins by ultracentrifugation in nine patients with primary type V hyperlipoproteinaemia before and during dietary treatment. After 30 days of balanced isocaloric diet mean serum triglycerides fell from 25.4 +/- 15.0 (mean +/- SD) to 2.8 +/- 1.7 mmol l-1. At the same time the chylomicrons and the very low density lipoproteins of flotation rate higher than 100 disappeared from the serum while the remaining very low density lipoproteins maintained unaltered their normal protein-lipid composition. After 30 days the low density lipoproteins increased significantly in concentration (from 1.6 +/- 0.8 to 4.1 +/- 1.1 mmol l-1 cholesterol) and their percentage content of cholesterol and triglyceride was increased and reduced, respectively. The highest concentration of intermediate density lipoprotein cholesterol was observed after 15 days of treatment (1.2 +/- 0.6 mmol l-1. The abnormally low concentrations and the physicochemical properties of the high density lipoproteins remained unchanged throughout the study (from 0.6 +/- 0.2 to 0.8 +/- 0.2 mmol l-1 cholesterol concentration) and no high density lipoproteins two (HDL2) were observed at any time. The effects of this treatment were an increase in low density and marginal change in high density lipoproteins which are considered, respectively, a positive and a negative risk factor for atherosclerosis.

Familial type V hyperlipoproteinaemia in identical twins homozygous for apoliprotein variant E2: report.

Hyperchylomicronaemia and elevated very low density lipoproteins were found in relatively obese 47 year old identical twin brothers. Lipoprotein apoprotein studies showed the presence of apoprotein CII, the activator of lipoprotein lipase, and both men were homozygous E2/2. Studies on the ability of the brothers to clear triglyceride rich particles showed some impairment of post heparin lipase activity, and a slower clearance of infused fat emulsion. The values improved after weight loss. There was some evidence of impaired capacity of the patients' high density lipoprotein to activate post heparin lipoprotein lipase.

Influence on serum lipids, lipoproteins and blood pressure of mackerel and herring diet in patients with type IV and V hyperlipoproteinemia.

Eight patients with type IV and V hyperlipoproteinemia were put on a mackerel and herring diet of an isocaloric regimen for 2 weeks, in a cross-over design. At the end of the dietary periods a predominant increase of eicosapentaenoic acid (EPA - C20:5, n-3) in cholesterol esters and of docosahexaenoic acid (DHA - C22:6, n-3) in serum triglycerides, being more pronounced after mackerel as compared to herring diet, could be confirmed. After mackerel diet serum triglycerides and total cholesterol were significantly lower, returning to basal levels 3 months later. High density lipoprotein (HDL) cholesterol appeared slightly increased after mackerel diet and decreased to initial values thereafter. After herring diet, which contained half as much EPA as compared to mackerel diet, the differences were minor. The decline of free fatty acids (FFA) and insulin at the end of the mackerel period reached the level of significance 60 min and 120 min, respectively, after glucose load. A significantly lower systolic blood pressure in recumbent and upright position after the mackerel period could be found, whereas diastolic pressure and blood pressure after herring diet remained unchanged.

Reduction of plasma lipids, lipoproteins, and apoproteins by dietary fish oils in patients with hypertriglyceridemia.

Dietary fish oils, which are rich in omega-3 fatty acids, have been reported to reduce plasma lipid levels in normolipidemic subjects. We examined the effects of fish oil in 20 hypertriglyceridemic patients: 10 with Type IIb hyperlipidemia and 10 with Type V. These patients were put on three diets differing primarily in fatty acid composition and fat content. The control diet contained a fatty acid mixture typical of a low-fat therapeutic diet (ratio of polyunsaturated to saturated fat, 1.4), the fish-oil diet contained omega-3 fatty acids, and the vegetable-oil diet was rich in the omega-6 fatty acid, linoleic acid. Each diet was followed for four weeks. In the Type IIb group, the fish-oil diet led to decreases in both plasma cholesterol (-27 per cent) and triglyceride (-64 per cent), as compared with the control diet. Very-low-density lipoproteins (VLDLs) were also reduced markedly. The vegetable-oil diet had much less effect. With fish oil, the Type V group had marked decreases in total cholesterol and triglyceride levels (-45 and -79 per cent, respectively). VLDL levels were dramatically lowered, as were apoprotein E levels. The vegetable-oil diet (unlike the fish-oil diet) produced a rapid and significant rise in plasma triglyceride levels. We conclude that fish oils and fish may be useful components of diets for the treatment of hypertriglyceridemia.

[Primary hyperlipoproteinemia in childhood (author's transl)]. / Hiperlipoproteinemia primaria tipo V en el niño.

Type V hyperlipoproteinemia is an unusual entity in children. Only 6 cases have been described so far to our knowledge. Authors present a 9 year old male that came for diagnosis of a hepatosplenomegaly. There was no evidence of abdominal pain, xanthomas or pancreatitis. Secondary disorders such as uncontrolled insulinopenic diabetes mellitus, glycogen storage disease, administration of estrogen compounds, nephrotic syndrome or uremia, and dysglobulinemias were excluded. His father presented the same lipoprotein pattern suggesting a dominant mode of inheritance. The administration of heparin showed a good response of serum proteinlipase.

[Comparative status of dietetic and therapeutic measures in idiopathic hyperlipidaemia (author's transl)]. / Situations respectives du traiteament diététique et du traitement médicamenteux des hyperlipidémies idiopathiques.

The indications for dietetic and therapeutic measures vary according to the type of hyperlipidaemia. A low fat diet is difficult to maintain and has exceptional and precise indications. It is indispensable in type I hyperlipidaemia, particularly since there is at present no drug capable of reducing hyperchylomicronaemia. It is also indicated in type V hyperlipidaemia in association with a low carbohydrate diet--a dietetic treatment which may become troublesome in long terme and must be supervised by highly specialized dieticians. In all other types of hyperlipidaemia systematic reduction of fats is wrong on theoretical grounds and must be firmly rejected on account of its detrimental consequences. When the patient's weight is normal, a normolipidic diet should be prescribed, with emphasis on unsaturated fats. In obese patients, but only at the onset of treatment, an overall low calorie diet with restriction of fats and carbohydrates should be advised. As soon as the target weight is reached, however, this should be replaced by another weight-stabilizing, isocaloric and normolipidic diet. It is, in fact, this kind of diet which all patients with type II, III and IV hyperlipidaemia will finally require.