phenylketonurias/triglyceride

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Fish-Free Diet in Patients with Phenylketonuria Is Not Associated with Early Atherosclerotic Changes and Enhanced Platelet Activation.

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OBJECTIVE Since patients with phenylketonuria (PKU) have to follow a lifelong restriction of natural protein to lower phenylalanine-intake, they never eat fish. This diet may lead to a chronic deficit of omega-3 and omega-6 fatty acids with the risk of early atherosclerotic changes. The aim of the

Triglycerides, cholesterol, HDL, LDL, and VLDL cholesterol in serum of phenylketonuric children under dietary control.

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Phenylketonuria is currently treated by a special diet to avoid elevated blood concentration of the essential amino acid phenylalanine. The authors examined 20 phenylketonuric children in the Inborn Error of Metabolism Department of Institute of Child Health in Athens. The children had simultaneous

[Efficacy and safety of a phenylalanine-free amino acid-based enteral formula (AA-PKU2) in 1-8 year-old children with phenylketonuria: a prospective, open, self-controlled and multi-center study in China].

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OBJECTIVE To evaluate the efficacy and safety of a phenylalanine-free amino acid-based enteral formula (AA-PKU2) in the treatment of children with phenylketonuria (PKU) aged 1-8 years. METHODS A prospective, open, self-controlled, multi-center trial was performed, enrolling 121 PKU children (1-8

Serum lipid concentrations in subjects with phenylketonuria and their families.

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To determine if subjects with phenylketonuria receiving diets significantly lower in cholesterol and saturated fat had serum lipid concentrations different from those of their family members, we measured serum concentrations of total cholesterol, high-density lipoprotein cholesterol, and total

Metabolic syndrome in children and adolescents with phenylketonuria.

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OBJECTIVE This study aimed to identify markers of metabolic syndrome (MS) in patients with phenylketonuria (PKU). METHODS This was a cross-sectional study consisting of 58 PKU patients (ages of 4-15 years): 29 patients with excess weight, and 29 with normal weight. The biochemical variables assessed

Nutritional and metabolic parameters of children and adolescents with phenylketonuria.

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Considering that phenylalanine-poor diets may be monotonous and compromise the development and nutritional status of children and adolescents with phenylketonuria, the aim of this study was to evaluate the anthropometric and biochemical characteristics of children and adolescents with

Dietary treatment in phenylketonuria does not lead to increased risk of obesity or metabolic syndrome.

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BACKGROUND Little is known about the consequences of the special energy enriched diet used to treat patients with phenylketonuria (PKU) in terms of obesity and metabolic syndrome (MetSyn) development. OBJECTIVE To investigate the prevalence of overweight and obesity, and its consequences in terms of

Lipid profile status and other related factors in patients with Hyperphenylalaninaemia.

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The mainstay of treating patients with phenylketonuria (PKU) is based on a Phe-restricted diet, restrictive in natural protein combined with Phe-free L-amino acid supplements and low protein foods. This PKU diet seems to reduce atherogenesis and confer protection against cardiovascular diseases but

Plasma cholesterol in adults with phenylketonuria.

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Phenylketonuria (PKU) is an autosomal recessive disorder of phenylalanine (Phe) catabolism resulting from a deficiency of L-phenylalanine hydroxylase (PAH). An association between hyperphenylalaninaemia (HPA) and hypocholesterolaemia has been reported in children. However, controversy exists as to

Lipid status and long-chain polyunsaturated fatty acid concentrations in adults and adolescents with phenylketonuria on phenylalanine-restricted diet.

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Blood lipid studies are reported in 25 adults and 2 adolescents with PKU who had been on phenylalanine-restricted diets for a mean period of 22.6 years (range 7-39 years). Measurements included plasma concentrations of phenylalanine, cholesterol, lipoproteins, triglycerides and fatty acid profiles,

Dietary glycemic index, glycemic load and metabolic profile in children with phenylketonuria.

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No data exist in the current literature on the glycemic index (GI) and glycemic load (GL) of the diet of phenylketonuric (PKU) children. The aims of this study were to examine the dietary GI and GL in PKU children on a low-phenylalanine (Phe)-diet and to evaluate whether an association may exist

beta-Lipoprotein quantitation in cord blood spotted on filter paper: a screening test.

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We describe an electroimmunodiffusion technique for measuring beta-lipoprotein in cord blood spotted on filter paper. A series of cord-blood samples, taken from 916 consecutive live-birth infants, was spotted directly onto filter paper and assayed for beta-lipoprotein. Eleven had above-normal

Cardiovascular disease biomarkers in patients with inborn errors of protein metabolism: a pilot study.

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BACKGROUND Limited data exist so far on cardiovascular disease biomarkers in patients maintained on a protein-restricted diet for inborn errors of protein metabolism. The present study aimed to analyse plasma cholesterol, lipoproteins, triglycerides and total homocysteine in patients with various

Haemostatic variables in phenylketonuric children under dietary treatment.

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Classical phenylketonuria (PKU) (McKusick 261600) is an inborn error of metabolism treated by a controlled low-phenylalanine (Phe) diet started as soon as possible in the first days of life. Such a diet can be achieved with vegetable protein and can be considered non-atherogenic because of the

Clinical pictures of 75 patients with neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD).

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We clarified the clinical features of NICCD (neonatal intrahepatic cholestasis caused by citrin deficiency) by retrospective review of symptoms, management and long-term outcome of 75 patients. The data were generated from questionnaires to paediatricians in charge of the patients. Thirty of the

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