[Congenital adrenal gland insufficiency and myopathy]. / Kongenitale Nebenniereninsuffizienz und Myopathie.

We report a rare case of a female newborn presenting with muscular hypotonia, pneumonia, and cardiovascular and renal insufficiency. Adrenal insufficiency was diagnosed clinically and proven by extremely low cortisone (0.4-0.8 microgram/dl) and high ACTH plasma levels. Myopathy was diagnosed clinically, as well as by muscular biopsy. DNA analysis of both X chromosomes showed no abnormality in the region of the genes for adrenal hypoplasia and Duchenne muscular dystrophy. After 4 weeks of intensive care therapy the patient died of multiorgan failure. At autopsy she had only microscopically visible fetal adrenal cells and multiple porencephalic lesions.

Iron supplementation in children after cardiopulmonary bypass for surgical repair of congenital heart disease.

A controlled study was carried out to evaluate the effects of postoperative iron therapy on iron status in anemic children after cardiopulmonary bypass. The patients were 8 boys and 9 girls (mean age 6.5 years) who underwent elective closure of atrial septal defect, secundum type. On postoperative day 9, patients were randomly assigned to either iron supplementation with iron sulfate 5 mg/kg until day 56 or to a control group. Hemoglobin, reticulocytes, transferrin saturation, free erythrocyte protoporphyrin, and ferritin were measured, the final outcome measure being postoperative iron status on day 56. The treatment group showed higher transferrin saturations (33.5% versus 18.0%), smaller decreases in ferritin level (+3.0 versus--13.7 ng/ml), and a lower incidence of depleted iron stores (0/8 versus 5/9) than the control group (all data: P < 0.05). Anemic children after cardiopulmonary bypass for surgical repair of congenital heart disease thus benefit from iron supplementation within the first postoperative weeks.

Vitamin K in breast milk: no influence of maternal dietary intake.

There is limited information available on the vitamin K intake of lactating mothers, concentration of vitamin K1 in breast milk, and the effect of long-term vitamin K1 supplementation of lactating mothers on the vitamin K1 concentration in breast milk. In a randomized study, we followed 20 mothers who received a daily oral vitamin K1 supplement (average 88 micrograms, supplemented group) and 16 mothers receiving no supplement (control group) from 4 throughout 91 days postpartum. Maternal vitamin K intakes (weighed dietary intake) at 4-6, 25-29 and 87-91 days postpartum ranged between 73 and 1735 micrograms/day. Differences between the groups were statistically not significant. Average intake exceeded the recommended dietary intake for lactating women of 55 micrograms/day by 670%. In the supplemented group, mean breast-milk vitamin K1 concentrations (HPLC) at 5, 26 and 88 days postpartum were 1.73 (SD 0.74), 1.36 (SD 0.81) and 1.67 (SD 2.01) ng/ml, respectively. Corresponding values in the control group were 1.44 (SD 0.57), 1.68 (SD 0.70) and 1.78 (SD 1.05) ng/ml. The latter were not statistically different from values in the supplemented group. Mean daily vitamin K1 intakes of infants breast-fed by supplemented mothers were 0.69 (SD 0.42), 0.93 (SD 0.51) and 1.25 (1.53) micrograms, respectively on days 5, 26 and 88. Corresponding values in the control group were 0.69 (SD 0.30), 1.07 (SD 0.58) and 1.31 (SD 0.95) micrograms and were statistically not different from values in the supplemented group. Average vitamin K1 intakes corresponded to 7-13% of the recommended dietary intake of 10 micrograms/d for infants.(ABSTRACT TRUNCATED AT 250 WORDS)

Iron nutrition and growth of breast- and formula-fed infants during the first 9 months of life.

Feeding of iron (Fe)-fortified (12-15 mg/L) infant formulas is an effective and convenient means to protect infants from Fe deficiency. To study lower levels of Fe fortification of infant formulas (3 or 6 mg/L) compared with those currently in use, we compared Fe intake and Fe nutritional status of three groups of healthy, term infants between 90 and 274 days of age. One group received an Fe-fortified whey-predominant formula (3 mg/L) and the second group received the same formula with a higher Fe level (6 mg/L). A comparison group was breast-fed at least until 274 days of age. All infants received infant foods and cereals according to European Community recommendations. Mean Fe intake of infants fed formula fortified with 3 mg/L was significantly lower at 183 and 274 days of age (p < 0.05) than that of infants fed formula fortified with 6 mg/L. None of the infants fed the formula fortified with 3 mg/L met the recommended daily allowance value (10 mg) for infants between 6 and 12 months of age. Hemoglobin, hematocrit, mean corpuscular volume, free erythrocyte protoporphyrin, and serum ferritin levels were similar in the formula-fed groups; none of the infants had depleted Fe stores (ferritin < 10 micrograms/L) at 183 and 274 days of age. Thirteen percent of breast-fed infants had depleted Fe stores at 183 days of age, but only 3% were depleted at 273 days of age, when Fe-fortified beikost was already part of the diet. No influence of Fe nutritional status was found on zinc and copper nutritional status or on growth.(ABSTRACT TRUNCATED AT 250 WORDS)

[Calcium, phosphorus and vitamin D administration in infancy. Unsolved questions]. / Kalzium, Phosphor- und Vitamin-D-Zufuhr im Säuglingsalter. Ungelöste Fragen.

In premature infants calcium and phosphate supplementation should be continued until the infants weigh more than 2000 g, because osteopenia of prematurity has been described after discontinuation of the supplements at body weights of < 1800 g. Premature infants generally receive substantially more vitamin D than their daily requirements (> 400 IU), but then are no studies on the side effects of high vitamin D intake (hypercalciuria?). The extremely high calzitriol concentrations in plasma of premature infants who receive Ca/P supplements indicate that maximal stimulation of Ca/P absorption is necessary and present supplementation could still be inadequate. The daily vitamin D requirements of term infants are between 100 and 400 IU and no supplements are necessary if the infants are fed vitamin-D-fortified formulas. The high prevalence of rickets in infants on macrobiotic or vegetarian diets indicates that those infants should receive calcium and vitamin D supplements.

[Acute diarrheal diseases. Treatment with carrot-rice viscous solution is more effective than ORS solution]. / Akute Durchfallerkrankungen. Behandlung mit Karotten-Reisschleim ist effektiver als ORS-Lösung.

In infants and children, the treatment of acute diarrhoea with glucose-based electrolyte solutions results in rehydration but does not reduce the severity and duration of diarrhoea. In german-speaking countries, rice- and carrot-based solutions have a long tradition in the treatment of diarrhoea and may also reduce stool output and the duration of diarrhoea. Therefore, we evaluated the efficacy and safety of a carrot-rice-based rehydration solution (Na 57 mmol/L, n = 70) and two conventional glucose-based solutions with high or low sodium concentrations (Na 90 mmol/L, n = 48 or Na 55 mmol/L, n = 60) in a prospective study. The study subjects were Pakistani boys and girls between 3 and 48 months of age with mild or moderate dehydration. We measured duration of diarrhoea, fecal and urine output, fluid intake and serum electrolytes. The duration of diarrhoea was significantly lower (p less than 0.05) in the group receiving the carrot-rice based rehydration solution (59.5 +/- 30.9 h) than in the groups receiving the high-Na (75.5 +/- 30.5) and low-Na (74.8 +/- 32.5) glucose-electrolyte solutions. The mean fecal output (p less than 0.01) and fluid intake (p less than 0.001) were also significantly lower in the infants and children receiving the carrot-rice-based rehydration solution. No major electrolyte imbalances were observed in the three groups. We conclude that the carrot-rice-based rehydration solution was safe and more effective than two conventional glucose-electrolyte solutions in the rehydration of infants and children with acute diarrhoea.

Interactions between infections, malnutrition and iron nutritional status in Pakistani infants. A longitudinal study.

The interactions between infections, malnutrition and poor iron nutritional status in infants at weaning ages are poorly defined. Therefore, four groups of infants from an area with a high incidence of malnutrition (Lahore, Pakistan) were enrolled in a prospective, randomized nutritional intervention study. Between 122 and 365 days of age, the infants from one community received either a milk cereal without iron fortification (n = 29), a milk cereal fortified with ferrous fumarate (7.5 mg/100 g; n = 30), or a milk cereal fortified with ferric-pyrophosphate (7.5 mg/100 g; n = 27). Forty-four infants from a neighbouring community did not receive a nutritional supplement and served as the control group. Calculated mean daily energy- and protein intake with the cereals was between 259-287 kcal, and 9.6-10.6 g at 12 months of age, respectively. Mean daily iron intake with the fortified cereals was between 4.1-5.1 mg at corresponding age. Nutritional supplementation resulted in significantly lower incidence of malnutrition and higher weight gain. Incidence of acute diarrhoea was significantly (p less than 0.05) lower in the supplemented groups. The infants fed the iron-fortified milk cereals had significantly higher hemoglobin (mean 10.4 vs. 9.8 g.dl-1) and serum ferritin (mean 13.3 vs. 8.5 ng.ml-1) values than the infants fed the non-fortified milk cereals. However, no differences in the incidence of infections were found between the supplemented groups. It is concluded that poor nutritional intake between 122 and 365 days of age substantially contributed to the high incidence of diarrhoea and malnutrition in Pakistani infants.

[Therapeutic drugs, illicit poisons and environmental chemicals in breast milk--assessing the risk]. / Medikamente, Suchtgifte und Umweltchemikalien in der Muttermilch--Risikobeurteilung.

Our knowledge about the transfer of drugs and environmental chemicals into breast milk has increased in the last years. This review is mainly based on recently published literature and focuses on all drugs and environmental substances with documented effects on lactation and the nursing infant. Our aim is to provide brief information for physicians who are counseling breastfeeding mothers.

[Does breast feeding protect from atopic diseases?]. / Schützt Stillen vor atopischen Erkrankungen?

It is well established that food antigens can pass from mothers to infants via the breast milk. Bovine-beta-lactoglobulin has been detected in several breast milk samples from mothers with regular intake of cow's milk. Healthy breastfed infants can produce IgG antibodies against cow's milk protein and in infants at risk for atopic disease specific IgE antibodies were found before cow's milk based infant formula was introduced into the diet. However, several clinical studies in infants at risk for atopic disease indicate that exclusive breastfeeding decreases the incidence of atopic disease. The protective effect of breastfeeding is only relative and it is uncertain, how long protection lasts. Sensitization to food antigens may occur already in utero, because infants whose mothers avoid common allergenic foods during the whole pregnancy and then during the lactation period have a lower incidence of atopic eczema than infants whose mothers are on an unrestricted diet. Avoidance of common allergenic foods only during the last trimester of pregnancy had no effect, because the fetus is capable of forming IgE immune response.

[Preventive use of vitamin D and fluoride in Austria]. / Vitamin-D- und Fluorprophylaxe in Osterreich.

The implementation of the recommended daily vitamin D and fluoride supplements for infants in Austria has been investigated during a nationwide infant nutrition survey (n = 1069). 93.9% of the infants received daily vitamin D supplements, whereas only 38% received fluroide. Rejection of fluoride supplemtation was most common among mothers aged between 30 and 45 years and mothers with university education. In Carinthia, Vorarlberg and Styria only 2 out of 10 infants received fluoride supplements. It seems necessary to reconsider the recommendations for fluoride supplementation in Austria.

Iron intake and iron nutritional status of infants fed iron-fortified beikost with meat.

We compared iron intake and iron nutritional status of two groups of healthy term infants who received meat-containing baby foods fortified with ferrous sulphate (2 mg Fe/100 g). One group received an Fe-fortified formula (1.6 mg Fe/100 kcal) and the other a nonfortified formula. Fe intake of the group fed the nonfortified formula was significantly lower (p less than 0.0001). These infants received Fe mainly from fortification Fe with beikost (75-86%) and less than 10% met the recommended intake of 1 mg.kg-1.d-1; whereas 80-85% of the infants fed the Fe-fortified formula did. Hb, Hct, FEP, and ferritin were similar in both groups with the exception of lower ferritin values at age 365 d (p less than 0.05) in the group fed the nonfortified formula. No infant had hemoglobin less than 100 g/L. We conclude that regular consumption of commercially prepared Fe-fortified beikost with meat prevents most healthy term infants from Fe deficiency even if Fe intake is substantially below the recommended intake.

[Protein malnutrition in pediatric patients with oncologic diseases]. / Protein-Malnutrition bei pädiatrischen Patienten mit onkologischen Erkrankungen.

To study the frequency of protein-caloric malnutrition in children (n = 16) with malignant diseases, we measured serum-albumin, -transferrin, -prealbumin and -retinol-binding protein (Radial immunodiffusion) before treatment was started. In 9 children at least 3 of the 4 rapid-turnover proteins were below the age-specific reference values. 7 patients (44%) with low rapid turnover proteins were free from infection, liver and renal dysfunction and thus, diagnosis of protein-caloric malnutrition was established. Anthropometric parameters as weight for age and the weight/height ratio did not predict protein-caloric malnutrition. Nutritional therapy (enteral, parenteral) during a period of 2 weeks resulted in significant increase of albumin (p less than 0.05), prealbumin (p less than 0.01) and retinol-binding protein (p less than 0.01). Rapid turnover proteins can be easily measured in each laboratory and are useful in diagnosis and treatment of protein-caloric malnutrition of children with malignant diseases as long as the limitations of this method are not neglected.

[Nutritional iron status of infants fed according to current recommendations]. / Der Eisenversorgungsstatus von Säuglingen, die entsprechend den gültigen Empfehlungen ernährt werden.

We examined the iron nutritional status of healthy term infants in a longitudinal study from 15 through 365 days of age. All infants were fed according to the present austrian recommendations. Serum hemoglobin (Hb) decreased from 15 through 122 days of age and remained constant thereafter. At 365 days of age, only 4.7% of the infants had hemoglobin levels below 11 g/dl, which is considered the borderline value for anemia. Mean corpuscular volume (MCV) of erythrocytes was changing during infancy. Free erythrocyte protoporphyrin (FEP) was constant from 122 days through 365 days of age. The upper normal value of 3 micrograms/gHb for infants older than 122 days of age corresponded to that for children older than one year and adults. Serum ferritin (SF) decreased from 15 through 183 days of age and remained constant thereafter. At 365 days of age, only 9.3% of the infants had SF below 10 micrograms/l, which is considered the borderline concentration for depletion of iron stores. We found no differences of iron nutritional status between infants who were breastfed longer than 122 days and infants who were breastfed shorter than 122 days or were fed formula. Our findings indicate that the prevalence of iron deficiency anemia and depletion of the iron stores is lower than in previous studies. Changes in infant nutrition during the last years resulted in higher iron intake and lower prevalence of iron deficiency.