Religious dietary rules and their potential nutritional and health consequences.

Background: The vast majority of the world population declares affiliation to a religion, predominantly Christianity and Islam. Many religions have special dietary rules, which may be more or less strictly adhered to.

Methods: Religious food rules were collected from holy books and religious websites as well as their translation into dietary practices.

Intermediate-Term Efficacy and Tolerance of Statins in Children.

Objectives: To evaluate the intermediate-term efficacy and tolerance of statins in children and adolescents with familial hypercholesterolemia.

Study Design: A total of 131 children or adolescents treated with statins for familial hypercholesterolemia were prospectively included. The efficacy of treatment was established by the percentage of children who achieved low density lipoprotein-cholesterol (LDL-C) levels <160 mg/dL during treatment.

Global molecular analysis and APOE mutations in a cohort of autosomal dominant hypercholesterolemia patients in France.

Autosomal dominant hypercholesterolemia (ADH) is a human disorder characterized phenotypically by isolated high-cholesterol levels. Mutations in the low density lipoprotein receptor (LDLR), APOB, and proprotein convertase subtilisin/kexin type 9 (PCSK9) genes are well known to be associated with the disease. To characterize the genetic background associated with ADH in France, the three ADH-associated genes were sequenced in a cohort of 120 children and 109 adult patients.

Erratum to: Review of the nutritional benefits and risks related to intense sweeteners.

[This corrects the article DOI: 10.1186/s13690-015-0092-x.].

Review of the nutritional benefits and risks related to intense sweeteners.

Background: The intense sweeteners currently authorised in Europe comprise ten compounds of various chemical natures. Their overall use has sharply risen in the last 20 years. These compounds are mainly used to formulate reduced-calorie products while maintaining sweetness.

Long-term follow-up of statin treatment in a cohort of children with familial hypercholesterolemia: efficacy and tolerability.

Background: Early identification of children with familial hypercholesterolemia (FH) makes it possible to start lipid-lowering therapy at a young age in order to prevent cardiovascular disease. Numerous randomized, often placebo-controlled, studies have assessed the efficacy and safety of statins in children with FH.

Objective: The aim of this pragmatic observational study was to evaluate pravastatin treatment efficacy and tolerability for a long period of time, and to assess how these results translate in 'real-life' clinical practice.

Intractable diarrhea with tufting enteropathy: a favorable outcome is possible.

Background And Objective: Tufting enteropathy (TE) is a congenital abnormality of intestinal mucosa development characterized by severe intestinal failure requiring parenteral nutrition (PN) and, in some cases, small bowel transplantation. A few patients have had a more favorable outcome. The objective of this study was to evaluate possible correlations between histological lesion severity in duodenal biopsies and clinical outcomes in children with TE.

[Recommendations for hypercholesterolemic children].

Some of hypercholesterolemias observed in childhood have a high risk of premature cardiovascular diseases. The monogenic dominantly inherited hypercholesterolemias such as the familial hypercholesterolemia due to mutations on LDL receptor gene corresponds to these diseases. This article, jointly elaborated by the Nouvelle Société Française d’Athérosclérose together with the Nutrition committee of the Société Française de Pédiatrie, is to propose recommendations for a screening strategy and for management of hypercholesterolemia in children.

Natural history of Crohn's disease: comparison between childhood- and adult-onset disease.

Background: Childhood-onset Crohn's disease (CD) might reflect a more severe form of disease. To test this hypothesis we analyzed the long-term natural history of CD in an adult cohort of patients with childhood-onset compared to adult-onset CD.

Methods: We selected 206 childhood-onset CD patients among 2992 adult patients with a diagnosis of CD established before December 31, 2000.

Diagnosis scoring for clinical identification of children with heterozygous familial hypercholesterolemia.

Background: Familial hypercholesterolemia (FH) is a frequent monogenic condition characterized by progressive atherosclerosis requiring preventive therapy from childhood. In a pediatric setting, heterozygous FH (hFH) in children may not be identified from common forms of hypercholesterolemia (HC).

Objective: To elaborate a clinical scoring system for the diagnosis of hFH, defined by the presence of a disease-causing mutation of the gene for the low-density lipoprotein receptor (LDLR).

Plasma citrulline concentration reflects enterocyte mass in children with short bowel syndrome.

Plasma citrulline was recently shown to reflect the residual functional enterocyte mass in various situations characterized by intestinal failure. However, few data are available in children with short bowel syndrome. The objective of this study was to assess the value of citrulline assays in this situation.

Association of arterial stiffness and endothelial dysfunction with metabolic syndrome in obese children.

Objective: We investigated whether metabolic syndrome, defined in 3 different ways (2 commonly used and 1 novel) is associated with arterial alterations in obese children.

Study Design: The study group comprised 384 obese children age 2.5 to 18 years.

Insulin resistance and ferritin as major determinants of abnormal serum aminotransferase in severely obese children.

Objectives: Liver involvement is a common complication of obesity related in part to insulin resistance. The role of ferritin has not been investigated in children. The aim was to determine the prevalence of liver enzyme abnormalities in severely obese children and to look for relationships between fat mass distribution, insulin resistance, and plasma ferritin.

Pulmonary function and sleep-related breathing disorders in severely obese children.

Background & Aims: To evaluate the frequency of pulmonary function and sleep-breathing disorders in severely obese children and to search for their association with obesity phenotypes.

Methods: Sleep studies and spirometry were performed for 54 severely obese children.

Results: Upper airway resistances (RAWs) were increased with RAW>200% and forced 25s expiratory volume<80% in 83% and 60% of individuals, respectively.

[Allergy to food colouring. A prospective study in ten children].

In order to know more about allergy to food colouring, we conducted a prospective open study over 8 months in a group of 10 atopic children with repeated urticaria. The mean age was 6.5 years (4.

Novel mutations of the PCSK9 gene cause variable phenotype of autosomal dominant hypercholesterolemia.

Autosomal dominant hypercholesterolemia (ADH) is a frequent (1/500) monogenic inherited disorder characterized by isolated elevation of LDL leading to premature cardiovascular disease. ADH is known to result from mutations at two main loci: LDLR (encoding the low density lipoprotein receptor), and APOB (encoding apolipoprotein B100), its natural ligand. We previously demonstrated that ADH is also caused by mutations of the PCSK9 (proprotein convertase subtilisin/kexin type 9) gene that encodes Narc-1 (neural apoptosis-regulated convertase 1).

Role of the DGAT gene C79T single-nucleotide polymorphism in French obese subjects.

Acyl-coenzyme A, diacylglycerol acyltransferase (DGAT), is a key enzyme involved in adipose-cell triglyceride storage. A 79-bp T-to-C single-nucleotide polymorphism (SNP) on the 3' region of the DGAT transcriptional site has been reported to increase promoter activity and is associated with higher BMI in Turkish women. To validate the possible role of this genetic variant in obesity, as well as the variant's possible cellular-functional significance, we performed an association study between the T79C change and several obesity-related phenotypes in 1357 obese French adults and children.

[Anticholesteremic agents in children].

The treatment of hypercholesterolaemia in children is often discussed as part of the primary prevention strategy for premature coronary disease in adults. Cholesterol-lowering drugs are appropriate in children with hereditary autosomal dominant diseases such as familial hypercholesterolaemia, familial Apo B100 deficiency, or combined familial dyslipidaemia. Indeed, these diseases are associated with a high risk of cardiovascular attacks in young adults.

Intronic mutations outside of Alu-repeat-rich domains of the LDL receptor gene are a cause of familial hypercholesterolemia.

Familial hypercholesterolemia (FH), a frequent monogenic condition complicated by premature cardiovascular disease, is characterized by high allelic heterogeneity at the low-density lipoprotein receptor ( LDLR) locus. Despite more than a decade of genetic testing, knowledge about intronic disease-causing mutations has remained limited because of lack of available genomic sequences. Based on the finding from bioinformatic analysis that Alu repeats represent 85% of LDLR intronic sequences outside exon-intron junctions, we designed a strategy to improve the exploration of genomic regions in the vicinity of exons in 110 FH subjects from an admixed population.