German national consensus recommendations on nutrition and lifestyle in pregnancy by the 'Healthy Start - Young Family Network'.

Diet and physical activity before and during pregnancy affect short- and long-term health of mother and child. The energy needs at the end of pregnancy increase only by about 10% compared to nonpregnant women. An excessive energy intake is undesirable since maternal overweight and excessive weight gain can increase the risks for a high birth weight and later child overweight and diabetes. Maternal weight at the beginning of pregnancy is especially important for pregnancy outcome and child health. Women should strive to achieve normal weight already before pregnancy. Regular physical activity can contribute to a healthy weight and to the health of pregnant women. The need for certain nutrients increases more than energy requirements. Before and during pregnancy, foods with a high content of essential nutrients should be preferentially selected. Supplements should include folic acid and iodine, iron (in case of suboptimal iron stores), the ω-3 fatty acid docosahexaenoic acid (in case of infrequent consumption of ocean fish) and vitamin D (in case of decreased sun exposure and decreased endogenous vitamin D synthesis). Pregnant women should not smoke and not stay in rooms where others smoke or have smoked before (passive smoking). Alcohol consumption should be avoided, since alcohol can harm unborn children.

[Nutrition in pregnancy - Practice recommendations of the Network "Healthy Start - Young Family Network"]. / Ernährung in der Schwangerschaft - Teil 2. Handlungsempfehlungen des Netzwerks "Gesund ins Leben - Netzwerk Junge Familie".

Nutrition, physical activity and lifestyle in pregnancy influence maternal and child health. The "Healthy start - Young Family Network" supported by the German Government with the national action plan IN FORM developed recommendations on nutrition in pregnancy. Folic acid supplements (400 µg/day) should be started before pregnancy and continue for at least the first trimester. Iodine rich foods and salt and an iodine supplement (100-150 µg/day) are recommended. Long-chain omega-3 fatty acids should be provided with ≥ 1 weekly portion of oily sea fish, or a DHA-supplement if regular fish consumption is avoided. Vitamin D supplementation is advisable unless there is regular exposure to sunlight. Iron supplements should be used based on medical history and blood testing. Vegetarian diets with nutritional supplements can provide adequate nutrition, but counselling is recommended. In contrast, a vegan diet is inadequate and requires additional micronutrient supplementation. For risk reduction of listeriosis and toxoplasmosis, raw animal foods, soft cheeses and packed fresh salads should be avoided; fresh fruit, vegetables and salad should be washed well and consumed promptly. Pregnant women should remain physically active and perform sports with moderate intensity. They should avoid alcohol, active and passive smoking. Up to 3 daily cups of coffee are considered harmless, but energy drinks should be avoided. Childhood allergy is not reduced by avoiding certain foods in pregnancy whereas oily sea fish is recommended. Health care professions should lead parents to health-promoting lifestyles. Subjects of part 1 of the article are practice recommendations on nutrition, on energy needs, micronutrient needs and body weight/weight gain in pregnancy.

[Nutrition in pregnancy - Practice recommendations of the Network "Healthy Start - Young Family Network"]. / Ernährung in der Schwangerschaft - Teil 1.

Nutrition, physical activity and lifestyle in pregnancy influence maternal and child health. The "Healthy start - Young Family Network" supported by the German Government with the national action plan IN FORM developed recommendations on nutrition in pregnancy. Energy needs increase by only ≈10 % by the end of pregnancy whereas micronutrient needs increase much more. Normal weight should preferably be achieved before pregnancy. Dietary recommendations follow those for the general population. Folic acid supplements (400 µg/day) should be started before pregnancy and continue for at least the first trimester. Iodine rich foods and salt and an iodine supplement (100-150 µg/day) are recommended. Long-chain omega-3 fatty acids should be provided with ≥ 1 weekly portion of oily sea fish, or a DHA-supplement if regular fish consumption is avoided. Vitamin D supplementation is advisable unless there is regular exposure to sunlight. Health care professions should lead parents to health-promoting lifestyles. Subjects of part 2 of the article are practice recommendations on nutrition in pregnancy, especially vegetarian diets, recommendations on micronutrient supplementation, risk reduction of listeriosis and toxoplasmosis, alcohol, tobacco, caffeine and physical activity in pregnancy.

Evaluation of scientific criteria for identifying allergenic foods of public health importance.

Identification of allergenic foods of public health importance should be based on well-defined criteria. Björkstén et al. (2008) proposed that the criteria should assess the evidence for an IgE mechanism, the reaction, the potency and the severity of the effect of the food and its prevalence. This study evaluated the application of the proposed criteria based on published reports. Publications were selected from two databases to test whether the descriptions for ranking the level of evidence for each criterion were unambiguous and covered the full range of levels of evidence regarding seven foods, five known to be allergenic and two negative controls. The options available to rank the quality of evidence were appropriate but needed refinement to improve clarity and conceptual value. The criteria were helpful to assess known IgE-dependent allergens, and to exclude the non-allergenic substances. The criteria framework discriminated between papers with high, moderate and low quality of evidence. The advantage of using the proposed criteria is to make the decision-making process and rationale explicit. The framework helps to identify gaps in knowledge and to uncover the level of heterogeneity of the evidence thus guiding research and providing a basis for sound risk management decisions.

[Survey on breast-feeding initiation and potential impact factors in hospitals and birth centres in Berlin]. / Untersuchung in Berliner Geburtskliniken und Geburtshäusern über den Stillbeginn und mögliche Einflussfaktoren.

AIMS: The aim of this study was to collect information on and to evaluate the impact of the timing of first suckling and breast-feeding initiation in Berlin and to assess the practicability and acceptance of using a short questionnaire to collect breast-feeding data in hospitals and birth centres. METHODS: A three-month observational study was conducted in 19 maternity units and 4 birth centres, using a short questionnaire to collect quantitative data on the timing of first suckling and breast-feeding from mother-child pairs on the day of discharge. RESULTS: The data indicate a breast-feeding rate of 96.1% at discharge. Infants born in birth centres were more frequently put to their mother's breast within the first hour after birth (p<0.05), and were more frequently mainly (p<0.05) or exclusively (p<0.01) breast-fed at discharge than infants born in hospitals. Hospitals' breast-feeding policies (i.e., following the 'ten steps to successful breast-feeding') were not associated with a higher prevalence of early first suckling and any breast-feeding at discharge, but rather with exclusivity of breastfeeding (p<0.001). CONCLUSIONS: Breast-feeding initiation rates are satisfactorily high in Berlin. Rates of early first suckling and (exclusive) initial breast-feeding are highest in birth centres. No consistent association was found between hospitals' breast-feeding policy and initial breast-feeding variables. The questionnaire was well accepted and is deemed suitable for monitoring purposes.

[Safety of food supplements]. / Sicherheit von Nahrungsergänzungsmitteln.

Food supplements are foodstuffs. Food which is not safe shall not be placed on the market. Adherence to the laws which aim for the safety of food ensures that food supplements are safe. Unfortunately, there are still gaps in the legislation for food supplements. However, even intrinsically safe foods can bear a risk for the consumer if not used appropriately, for example if food supplements are consumed instead of healthy diets or if food supplements are used as substitutes for indicated drugs.

Consideration of possible legislation within existing regulatory frameworks.

Legislation on a particular food or on a particular claim to be used in connection with a food require a definition of the food and unequivocal requirements for the use of the claim. The definitions of prebiotics and probiotics presently place these terms between the categories for conventional foods and foods for special dietary uses. Because probiotics and prebiotics, as a group, do not fulfill the criteria for special dietary uses, they have to comply with the rules and laws for conventional foods even if the requirements for the use of the terms prebiotic and probiotic include effects on body functions. These effects on the health and wellness of the consumer and to stimulatory activity, eg, body defense mechanisms, can be used in claims that should underline the importance of the total dietary pattern. It is suggested that setting up rules for the use of the terms prebiotic and probiotic is preferable to creating new food standards.

Exposition to and health effects of residues in human milk.

A great variety of drugs, cosmetics, food ingredients as well as environmental contaminants are secreted with human milk as a result of actual exposure or the accumulated body burden of the mother. Of great concern and least amenable to short-term intervention are persistent substances in the environment with long half-lives in the body due to their lipophilic properties and minimal degradation. Polyhalogenated aromatic hydrocarbons, namely organochlorine pesticides, polychlorinated biphenyls (PCB) and polychlorinated dibenzodioxins (PCDD) and dibenzofurans (PCDF) are fetotoxic, neurotoxic, immunotoxic, some are promoting carcinogens and/or interfere with hormonal receptors. They pass the placenta and equilibrate among the lipid compartments of the body including breast milk lipids. Transplacental exposure is more relevant with regard to physical development and cognitive functioning of the child than postnatal exposure via breastmilk. Restrictions for production, use and release have been successful in decreasing exposure as shown by a downward trend of their contents both in human milk and serum lipids for the last 15 to 20 years. It is difficult to evaluate the potentially late effects of the exposure via breastmilk which is 10 to 100 times higher in industrialised countries than the tolerable daily intake (TDI) of 1 to 4 toxic equivalents (WHO-TEQ) pg/kg/day established in 1998 by WHO for dioxins and dioxin-like PCBs but which lasts for 0.6% of the expected life span only. Carefully conducted long-term follow-up of cohorts with defined exposure levels, with consideration of numerous biological and psychological parameters, is expected to provide the answer.

Nutrition, physical growth, and bone density in treated phenylketonuria.

UNLABELLED: Dietary treatment of phenylketonuria is well established to be safe and to prevent developmental and mental impairment in patients with low or absent phenylalanine hydroxylase activity. The use of semi-synthetic diets necessitates careful and longitudinal control not only of physical and intellectual development, which are both near normal in well treated patients, but also of potential diet inherent insufficiencies of essential nutrients. Concern has been raised by some reports on growth retardation in young patients on strict diets and on decreased bone density in older phenylketonuric children. The clinical significance of these findings is not known. CONCLUSION: Changes have been found, although inconsistently, in connection with selenium, zinc, iron, retinol and polyunsaturated fatty acid status in dietetically treated patients with phenylketonuria. Both the mechanism and significance of these changes is doubtful at present.

Rationale for the German recommendations for phenylalanine level control in phenylketonuria 1997.

UNLABELLED: Treatment of hyperphenylalaninaemias due to phenylalanine hydroxylase deficiency with a low phenylalanine (Phe) diet is highly successful in preventing neurological impairment and mental retardation. There is consensus that, for an optimal outcome, treatment should start as early as possible, and that strict blood Phe level control is of primary importance during the first years of life, but for adolescent and adult patients international treatment recommendations show a great variability. A working party of the German Working Group for Metabolic Diseases has evaluated research results on IQ data, speech development, behavioural problems, educational progress, neuropsychological results, electroencephalography, magnetic resonance imaging, and clinical neurology. Based on the actual knowledge, recommendations were formulated with regard to indication of treatment, differential diagnosis, and Phe level control during different age periods. The development of the early-and-strictly-treated patient in middle and late adulthood still remains to be investigated. Therefore, the recommendations should be regarded as provisional and subject to future research. Efficient treatment of phenylketonuria has to go beyond recommendations for blood Phe level control and must include adequate dietary training, medical as well as psychological counselling of the patient and his family, and a protocol for monitoring outcome. CONCLUSIONS: Early-and-strictly-treated patients with phenylketonuria show an almost normal development. During the first 10 years treatment should aim at blood Phenylalanine levels between 40 and 240 micromol/L. After the age of 10, blood phenylalanine level control can be gradually relaxed. For reasons of possible unknown late sequelae, all patients should be followed up life-long.

Regulating food products without impeding innovation.

Industry and regulatory bodies share a common goal of making beneficial products available to consumers, but the relationship between industry and regulators can become adversarial if it is not handled properly. When industry views the regulatory process as an obstacle to product development and marketing, and regulators view petitioners as having only a profit motive, the opportunity to work together efficiently to get products to market is lost. While it is difficult to codify how industry and regulators should interact, it is worthwhile to look at some of the most provocative issues and see how they might be addressed. The discussion that follows will examine the functioning of regulatory bodies in the European Union, consider how more flexibility might be added to the regulatory process, and raise the issue of how regulatory bodies can function to serve the consumer while promoting innovation.

Recommendations for protein and amino acid intake in phenylketonuria patients.

Nitrogen requirement in phenylketonuria patients is similar to that of normal persons. Prescribed protein intake for patients should be based on age-related safe levels of protein intake modified by the digestibility-corrected amino acid score. Adequate energy intakes and distribution of dietary protein over meals are important determinants for the quality of dietary treatment.

Solomon's epidermal nevus syndrome (type: linear nevus sebaceus) and hypophosphatemic vitamin D-resistant rickets.

BACKGROUND: Epidermal nevus syndrome is very variable in symptoms and associated abnormalities. Synonyms of this syndrome are linear nevus sebaceus syndrome or Schimmelpenning-Feuerstein-Mims syndrome or Solomon syndrome. The combination with vitamin D-resistant rickets is rare and only sporadically described. Less than 10 cases with this combination of symptoms have been described in the literature. OBSERVATIONS: We describe a boy suffering from epidermal nevus syndrome (type: nevus sebaceus). This child also presented with severe rickets with hyperphosphaturia, resistant to vitamin D. Our patient was seen in consultation at birth, but after a delay of 4 years we were consulted again for a second opinion and treatment; the vitamin D-resistant rickets was recognized. Treatment with 1,25-dihydroxy vitamin D3 and phosphorus resulted in healing of rickets. Removal of parts of the tumors did not influence the rickets. This is in contrast with a formerly described case. Removal of fibroangiomas led in that case to normalization of the alkaline phosphatase, calcium, and phosphate serum levels. CONCLUSIONS: The rickets results from massive phosphate excretion by defective renal tubular reabsorption of phosphate. In all patients described, rickets developed at an early age. Clinical symptoms were marked bone abnormalities, muscle weakness, and bone pain.

Functional and morphological deficits in late-treated patients with homocystinuria: a clinical, electrophysiologic and MRI study.

Seven late-treated patients between the ages of 10-30 years suffering from homocystinuria were examined clinically and electrophysiologically; four had MRI. The clinical examination showed extrapyramidal features and slight impairment of proprioception. Electrophysiological evaluation revealed normal results in the acoustic and central motor system; a minor, possibly vitamin B6 related, sensory neuropathy was detected by peripheral conduction studies. MR imaging showed small focal areas of gliosis in the white matter, generalized cortical atrophy in two patients, but only one small cortical infarct. No changes in the basal ganglia were detected. These results support the view that neurological signs and symptoms in patients suffering from homocystinuria are related to morphological findings, as well as pharmacological effects.

Effects of high carnitine supplementation on substrate utilization in low-birth-weight infants receiving total parenteral nutrition.

Parenterally fed preterm neonates are known to be at risk for carnitine deficiency. We studied substrate utilization in low-birth-weight infants receiving total parenteral nutrition (TPN) with (A) and without (B) supplementation of 48 mg carnitine.kg-1.d-1 on days 4-7 (birth weights 1334 +/- 282 vs 1318 +/- 248 g, gestational age 32 +/- 2 vs 32 +/- 2 wk, A vs B, respectively). TPN consisted of 11 g glucose.kg-1.d-1 and 2.4 g.kg-1.d-1 of both protein and fat. Plasma carnitine concentrations at day 7 were for free carnitine 11.8 +/- 5.0 vs 164 +/- 56 mumol/L and for acyl carnitine 3.8 +/- 2.0 vs 33.9 +/- 15.4 mumol/L, respectively. Indirect calorimetry at day 7 showed a higher fat oxidation (0.21, -0.31 to +0.60 vs 1.18, 0.70 to 1.95 g. kg-1.d-1, respectively, P less than 0.02, median and interquartile range) in group B and a higher protein oxidation (0.37, 0.30-0.43 vs 0.63, 0.53-0.88 g.kg-1.d-1, P less than 0.001). The time to regain birth weight was also higher in group B (7, 5.5-9 vs 9, 7-14 d, P less than 0.05). Carnitine supplementation and calorie intake were the best explanatory variables for metabolic rate (R2 = 0.45, P less than 0.002). We conclude that carnitine supplementation of TPN in this dosage does not seem advisable.

[Pathogenesis and pathophysiology of carnitine deficiency, a predictable risk]. / Pathogenese en pathofysiologie van carnitinedeficiëntie, een voorspelbaar risico.

When patients suffer from hepato-encephalopathy, (cardio)myopathy, dystrophy, hypoglycaemia, some metabolic diseases and several other disease states, carnitine deficiency should be considered. In this article a survey is given of the pathophysiology, laboratory diagnostics, clinical symptomatology and some therapeutic approaches. Some different cases will be demonstrated.

Carnitine deficiency in surgical neonates receiving total parenteral nutrition.

Carnitine plays a key role in the oxidation of fatty acids. Most solutions for parenteral nutrition do not contain carnitine. Because endogenous carnitine synthesis is insufficient in newborns, they are prone to developing a carnitine deficiency when they are dependent on total parenteral nutrition (TPN). Stimulated by the clinical observation of manifest clinical symptoms of carnitine deficiency in one patient, a study of 13 consecutive neonates who received TPN for over 2 weeks was begun. Their plasma carnitine levels before and during carnitine supplementation were determined. All patients had a carnitine intake far below the recommended minimal need of 11 mumol/kg per day. Although only three of them clearly showed clinical symptoms described as carnitine deficiency, carnitine supplementation for all neonates receiving TPN for over 2 weeks is recommended.