Michel Vidailhet

Vitamin D: Still a topical matter in children and adolescents. A position paper by the Committee on Nutrition of the French Society of Paediatrics

The aims of the present position paper by the Committee on Nutrition of the French Society of Paediatrics were to summarize the recently published data on vitamin D in infants, children and adolescents, i.e., on metabolism, physiological effects, and requirements and to make recommendations on supplementation after careful review of the evidence. Scientific evidence indicates that calcium and vitamin D play key roles in bone health. The current evidence, limited to observational studies, however, does not support other benefits for vitamin D. More targeted research should continue, especially interventional studies. In the absence of any underlying risk of vitamin D deficiency, the recommendations are as follows: pregnant women: a single dose of 80,000 to 100,000 IU at the beginning of the 7th month of pregnancy; breastfed infants: 1000 to 1200 IU/day; children less than 18 months of age, receiving milk supplemented with vitamin D: an additional daily dose of 600 to 800 IU; children less than 18 months of age receiving milk not supplemented with vitamin D: daily dose of 1000 to 1200 IU; children from 18 months to 5 years of age: 2 doses of 80,000 to 100,000 IU every winter (November and February). In the presence of an underlying risk of vitamin D deficiency (dark skin; lack of exposure of the skin to ultraviolet B [UVB] radiation from sunshine in summer; skin disease responsible for decreased exposure of the skin to UVB radiation from sunshine in summer; wearing skin-covering clothes in summer; intestinal malabsorption or maldigestion; cholestasis; renal insufficiency; nephrotic syndrome; drugs [rifampicin; antiepileptic treatment: phenobarbital, phenytoin]; obesity; vegan diet), it may be justified to start vitamin D supplementation in winter in children 5 to 10 years of age as well as to maintain supplementation of vitamin D every 3 months all year long in children 1 to 10 years of age and in adolescents. In some pathological conditions, doses of vitamin D can be increased. If necessary, the determination of 25(OH) vitamin D serum concentration will help determine the level of vitamin D supplementation.

Selenium, glutathione peroxidase (GSH-Px) and lipid peroxidation products before and after selenium supplementation

Treated phenylketonuric (PKU) children are at risk of selenium deficiency. We have studied 15 treated PKU children and 30 control children. We observed significantly lower (P less than 0.0005) plasma and erythrocyte selenium, as well as significantly lower (P less than 0.0005) plasma and erythrocyte glutathione peroxidase activities (GSH-Px) in PKU children than in controls. The lipid peroxidation products, evaluated as plasma malondialdehyde (MDA), was higher (P less than 0.0005) in PKU children than in controls. Specific oral sodium selenite supplementation (Selenium: 0.13 mumol/kg/day) resulted in a rapid increase of plasma selenium and GSH-Px activity, and after 10 days and 1 month respectively significant difference is no longer observed between PKU children and controls values. Statistically significant differences in erythrocyte selenium, erythrocyte GSH-Px activity and plasma MDA between PKU and control children disappear after respectively 2 months, 4 months and 6 months of selenium supplementation.

Alimentation des premiers mois de vie et prévention de l’allergie

Allergy consists in the different manifestations resulting from immune reactions triggered by food or respiratory allergens. Both its frequency and severity are increasing. The easiest intervention process for allergy prevention is the reduction of the allergenic load which, for a major allergen such as peanuts, has to begin in utero. The primary prevention strategy relies first on the detection of at risk newborns, i.e. with allergic first degree relatives. In this targeted population, as well as for the general population, exclusive breastfeeding is recommended until the age of 6 months. The elimination from the mothers diet of major food allergens potentially transmitted via breast milk may be indicated on an individual basis, except for peanut, which is systematically retrieved. In the absence of breastfeeding, prevention consists in feeding at-risk newborns until the age of 6 months with a hypoallergenic formula, provided that its efficiency has been demonstrated by well-designed clinical trials. Soy based formulae are not recommended for allergy prevention. Complementary feeding should not be started before the age of 6 months. Introduction of egg and fish into the diet can be made after 6 months but the introduction of potent food allergens (kiwi, celery, crustaceans, seafood, nuts, especially tree nuts and peanuts) should be delayed after 1 year. This preventive policy seems partially efficacious on early manifestations of allergy but does not restrain the allergic march, especially in its respiratory manifestations. Probiotics, prebiotics as well as n-3 fatty polyunsaturated acids have not yet demonstrated any definitive protective effect.

[Breast feeding: health benefits for child and mother].

The prevalence of breastfeeding in France is one of the lowest in Europe: 65% of infants born in France in 2010 were breastfed when leaving the maternity ward. Exclusive breastfeeding allows normal growth until at least 6 months of age, and can be prolonged until the age of 2 years or more, provided that complementary feeding is started after 6 months. Breast milk contains hormones, growth factors, cytokines, immunocompetent cells, etc., and has many biological properties. The composition of breast milk is influenced by gestational and postnatal age, as well as by the moment of the feed. Breastfeeding is associated with slightly enhanced performance on tests of cognitive development. Exclusive breastfeeding for at least 3 months is associated with a lower incidence and severity of diarrhoea, otitis media and respiratory infection. Exclusive breastfeeding for at least 4 months is associated with a lower incidence of allergic disease (asthma, atopic dermatitis) during the first 2 to 3 years of life in at-risk infants (infants with at least one first-degree relative presenting with allergy). Breastfeeding is also associated with a lower incidence of obesity during childhood and adolescence, as well as with a lower blood pressure and cholesterolemia in adulthood. However, no beneficial effect of breastfeeding on cardiovascular morbidity and mortality has been shown. Maternal infection with hepatitis B and C virus is not a contraindication to breastfeeding, as opposed to HIV infection and galactosemia. A supplementation with vitamin D and K is necessary in the breastfed infant. Very few medications contraindicate breastfeeding. Premature babies can be breastfed and/or receive mothers milk and/or bank milk, provided they receive energy, protein and mineral supplements. Return to prepregnancy weight is earlier in breastfeeding mothers during the 6 months following delivery. Breastfeeding is also associated with a decreased risk of breast and ovarian cancer in the premenopausal period, and of osteoporosis in the postmenopausal period.

Maternal phenylketonuria: the French survey

We report the French experience regarding pregnancies in maternal phenylketonuria (PKU). In 2001, a questionnaire was sent to each referring PKU specialist in the 20 centres of each region of France, collecting reports on 135 pregnancies in 79 women born between 1958 and 1980. The majority of the 135 pregnancies occurred after 1990. A total of 42 women were informed of the risks of untreated pregnancy, while 26 were not informed (no data for 11). A strict diet was achieved in 83% of informed and in 16% of uninformed mothers prior to conception. Healthy offspring were observed in 43% of the 135 pregnancies, spontaneous abortions in 10.4%, elective abortions in 4.4%, therapeutic abortions in 12.6%, and embryopathies (EP) in 21.5%. In 8.1% of cases, the outcomes (in earliest pregnancies) are unknown. The proportion of healthy children increased over time and reached 80% of the pregnancies of informed females. There were seven heart defects, all in cases of EP, but although microcephaly and intrauterine growth retardation (IUGR) were almost constant in EP, we also found nine healthy children with IUGR. A continuum between EP and healthy children is suggested. The anthropometric data of the mothers showed that their body mass index (BMI) distribution was shifted to the left compared to women of the general population. This lower BMI and poor weight gain during pregnancy could contribute to the IUGR observed in normal babies whose mothers received a phenylalanine-restricted diet during pregnancy. Conclusion:the information and the preconception diet are effective for avoiding embryopathies in maternal phenylketonuria. Nutritional parameters can influence fetal growth and the nutritional state must be closely monitored throughout pregnancies of women with phenylketonuria.

La collation de 10 heures en milieu scolaire : un apport alimentaire inadapté et superflu

An apparatus including a camera optical element and a tactile indicator associated with the camera optical element for indicating a property of the camera optical element.

Prévention par l’acide folique des défauts de fermeture du tube neural : la question n’est toujours pas réglée

Between 1981 and 1996, several interventional studies proved the efficacy of periconceptional folic acid supplementation in the prevention of neural tube closure defects (NTCD), first in women at risk (with a previous case of NTCD) and also in women of the general population in age to become pregnant. The poor observance of this supplementation led several countries (USA, Canada, Chile...) to decide mandatory folic acid fortification of cereals, which permitted a 30% (USA) to 46% (Canada) reduction in the incidence of NTCD. Moreover, this benefit was accompanied by a diminished incidence of several other malformations and of stroke and coronary accidents in elderly people. However, several papers drew attention to an increased risk of colorectal and breast cancer in relation with high blood folate levels and the use of folic acid supplements. A controlled interventional study showed a higher rate of recurrence of colic adenomas and a higher percentage of advanced adenomas in subjects receiving 1mg/day of folic acid. A recent study demonstrated an abrupt reversal of the downward trend in colorectal cancer 1 year after the beginning of cereal folic acid fortification in the USA and Canada. Two studies also reported impaired cognitive functions in elder persons with defective vitamin B(12) status. Taken in aggregate, these studies question the wisdom of a nationwide, mandatory, folic acid fortification of cereals. As of today, despite their limited preventive efficacy, a safe approach is to keep our current French recommendations and to increase the awareness of all caregivers, so as to improve the observance of these recommendations.

Cobalamin R binder as a possible model molecule for glycoprotein study in cystic fibrosis

The isoprotein pattern of semi-purified R binder (an acidic glycoprotein which binds cobalamin) from saliva and sera of 8 cystic fibrosis patients was compared to that of R binder from samples of 5 healthy children. In cases of cystic fibrosis, the mean isoelectric point of salivary R binder was increased from 3.78 up to 4.34 and its microheterogeneity was reduced. These significant physicochemical modifications were not observed with R binder from cystic fibrosis sera and they did not correlate with the beta-galactosidase, alpha-mannosidase, alpha-L-fucosidase nor neuraminidase activity of saliva. We propose the R binder as a model molecule to study the glycoprotein metabolism in cystic fibrosis since it contains 30-40% carbohydrate, is easily complexed with cyano[57Co]cobalamin and is present in most tissues and fluids of the human organism.

Contrast-Enhanced Ultrasonography in Patients With Glycogen Storage Disease Type Ia and Adenomas

Objective. The purpose of this series was to evaluate the value of contrast‐enhanced ultrasonography (CEUS) in the characterization of focal liver lesions (FLLs) in patients with glycogen storage diseases (GSDs). Methods. Contrast‐enhanced ultrasonographic data obtained for characterization of 8 FLLs (size, 0.9–10.2 cm) in 2 patients with GSD type Ia (GSD‐Ia) and lesion growth or recurrent abdominal pain were reviewed and compared with computed tomographic (CT) and magnetic resonance imaging (MRI) data. After total and left hepatectomy, pathologic examination confirmed benign adenomas in 6 of the evaluated lesions. Follow‐up confirmed benignity in the 2 remaining lesions. Results. In all FLLs, CEUS showed marked hypervascularity in the early arterial phase. Centripetal filling was shown in only 1 lesion, and diffuse enhancement without any clear direction was shown in all other lesions. During the portal and late phases, 6 of the 8 lesions showed sustained enhancement, including 2 lesions that appeared heterogeneous during all phases of CT and MRI. In an aspect of 1 of these 6 large adenomas, late wash‐out could be explained by sinusoid compression. The other 2 adenomas showed moderate wash‐out but remained homogeneous. Conclusions. Focal liver lesions found in patients with GSD‐Ia have similar patterns on CEUS compared with incidental adenomas. Global or partial hypoenhancement observed in the late phase did not indicate a transition to hepatocellular carcinoma but may have been related to ischemia.

Recommandations diététiques pour les femmes enceintes atteintes de phénylcétonurie

Prevention of embryopathy due to maternal phenylketonuria is possible thanks to a maternal-specific low-phenylalanine diet, which has to be started before conception and followed during the whole gestation. The setup of this diet implies knowing the recommended dietary allowances for normal pregnant women as well as for women with nutritional deficiencies. Women with phenylketonuria must be considered at risk for nutritional imbalance for two main reasons. First, most adult women with phenylketonuria have been on a vegetarian diet for many years without protein substitutes or medical control. Secondly, the strict diet for pregnant women with phenylketonuria may induce anorexia or nutritional deficits if it is not well tolerated or understood. Protein, iron, calcium, selenium, vitamin B 12 and caloric intakes are the most sensitive parameters. Close cooperation with an experienced medical and dietician team is required.