Successful treatment of severe carbamyl phosphate synthetase I deficiency.

We describe a girl with neonatal hyperammonaemia due to carbamyl phosphate synthetase I deficiency. Treatment consisted of protein restriction from the second day of life. Sodium benzoate was given for three weeks after birth and again from 7 months of age together with sodium phenylacetate to improve protein tolerance. Growth and development are normal at 15 months of age.     

Do Parents Follow Breastfeeding and Weaning Recommendations Given by Pediatric Nurses? A Study With Emphasis on Introduction of Cow’s Milk Protein in Allergy Risk Families

INTRODUCTION: The aim of this study was to retrospectively examine weaning practices during the first year of life in a representative sample of Swedish children and how parents with a history of atopy introduced milk protein in their infant's diet. METHODS: Data were derived from 467 infants visiting Child Health Centers in three different counties in Sweden for a health check up at 12 months of age. RESULTS: The children were breastfed for an average of 7 months (range, 0.2-15 months), and 18% were still breastfed at the age of 12 months. Few infants had received solid food before the age of 4 months (6%) or after the age of 6 months (12%). Cow's milk protein was introduced in disagreement with the current recommendation for children at risk of developing atopy. CONCLUSION: Breastfeeding and weaning recommendations seem to be followed by most families. The creation of routines for the distribution of information concerning weaning foods should be encouraged in order to reach families with special needs; otherwise, implementation of current recommendations and preventive strategies will be less useful.     

Protein C levels in infancy and early childhood. Influence of breast feeding

Protein C antigen levels were measured in the plasma of healthy full term infants by electroimmunoassay. During the first three months of life (on day four, at one month, two months and three months of age) protein C antigen levels were compared in breast-fed and bottle-fed infants. None of the two groups of infants received vitamin K at birth. Only at the age of three months there was a significant difference between the groups. Unexpectedly infants, who were breast-fed, had a higher protein C level at three months of age. Levels were also measured in 15 healthy children between one and three years of age. The antigen levels increase with age to reach adult values at about three years of age.     

Protein C Levels in Infancy and Early Childhood

ABSTRACT. Protein C antigen levels were measured in the plasma of healthy full term infants by electroimmunoassay. During the first three months of life (on day four, at one month, two months and three months of age) protein C antigen levels were compared in breast-fed and bottle-fed infants. None of the two groups of infants received vitamin K at birth. Only at the age of three months there was a significant difference between the groups. Unexpectedly infants, who were breast-fed, had a higher protein C level at three months of age. Levels were also measured in 15 healthy children between one and three years of age. The antigen levels increase with age to reach adult values at about three years of age.     

北京地区人群中人偏肺病毒血清抗体水平的初步调查

目的通过对人类偏肺病毒（hMPV）特异性IgG抗体检测,初步了解北京地区人群中该病毒感染的状况。方法以大肠杆菌表达的hMPV核蛋白（N蛋白）为抗原,用Western-blot检测随机选取的116例血清标本中hMPVN蛋白特异性IgG抗体,以确定表达的hMPVN蛋白的抗原特异性。随后对1996年4月至1997年3月自首都儿科研究所附属儿童医院和北京宣武医院采集的门诊非呼吸道感染患者及正常儿童的体检血清710例进行hMPVN蛋白特异性IgG抗体检测。结果hMPVN蛋白的抗原特异性试验证明表达的hMPV的N蛋白特异性好。本组710份血清中,抗hMPVN蛋白IgG抗体总阳性率为17．2％（122／710）。其中〈1个月的婴儿抗体阳性率为13．2％,1个月～的婴儿中抗体阳性率下降至6．1％,2个月～的婴儿中抗体阳性率降至最低（3．1％）,6个月～的婴儿中抗体阳性率上升至13．9％,在随后的几个年龄段内抗体阳性率维持在13．0％左右,30岁以后的人群中抗体阳性率有所升高,30岁～人群中达28．1％,40岁～人群中达32．3％,≥50岁的人群中达38．5％。结论研究中所应用的hMPVN蛋白的抗原特异性好;抗体的检测结果提示在北京地区的人群中hMPV的感染并不少见,血清抗体阳性率低的年龄组与文献报道感染发生率高的年龄组相符。     

Antibodies to Plasmodium falciparum antigens vary by age and antigen in children in a malaria-holoendemic area of Kenya

BACKGROUND: Antibodies are important in protection against infection and disease caused by Plasmodium falciparum, but the frequencies of antibodies to multiple P. falciparum antigens in children are not well-characterized. METHODS: IgG and IgM antibodies to the vaccine candidate antigens circumsporozoite protein, thrombospondin-related adhesive protein, liver stage antigen-1, apical membrane antigen-1, erythrocyte-binding antigen-175 and merozoite surface protein-1 were measured by enzyme-linked immunosorbent assay in 110 children 0-50 months of age in a malaria holoendemic area of Kenya. RESULTS: A similar pattern was seen for IgG antibodies to circumsporozoite protein, thrombospondin-related adhesive protein, apical membrane antigen-1 and erythrocyte-binding antigen-175: high frequencies (70-90%) in children 0-4 months of age; a decrease in children 5-20 months of age (35-71%); and progressive increases in children 21-36 and 37-50 months of age (53-80% and 60-100%, respectively). In contrast, IgG antibodies to liver stage antigen-1 were infrequent in children 0-4 months of age (5%) and increased with age to 64%, and IgG antibody frequencies to merozoite surface protein-1 were similar across age groups (26-52%). IgG antibodies to all antigens were predominantly of the IgG1 and IgG3 subclasses. Frequencies of IgM antibodies to all antigens were low in children 0-4 months of age (0-15%) and increased with age (24-56% in the oldest children). CONCLUSION: In children in a malaria-holoendemic area, IgM antibody to all P. falciparum antigens is infrequent in the first 4 months of life but increases with age and increased exposure. The pattern of age-related IgG response frequencies to P. falciparum antigens varies significantly by antigen.     

Transfer of tuberculin immunity from mother to infant

The purpose of our study was to investigate transfer of tuberculin immunity from mother to infant via breast milk by studying newborn lymphocyte blastogenesis induced by purified protein derivative antigen at 1-5 days of age, 4-6 wk of age, and 3 months of age. Our study consisted of four mother-infant groups: breast-feeding and bottle-feeding infants of tuberculin-positive and tuberculin-negative mothers. A difference in the groups was found only at 4-6 wk of age where 17% (4/23) of breast-feeding infants and 13% (2/15) of bottle-feeding infants of tuberculin positive mothers had lymphocyte blastogenesis to purified protein derivative. None of the infants of tuberculin negative mothers had purified protein derivative-induced blastogenesis. Analysis of covariance with tests for equality of slopes showed that the responses of tuberculin-positive mothers were significantly different from the responses of tuberculin-negative mothers (p less than 0.05). These studies suggest transplacental transfer of tuberculin immunity evident at 4-6 wk of age which wanes by 3 months of age. We could not find evidence of transfer via human milk.     

Cardiovascular disease risk factor variables during the first year of life.

Cardiovascular risk factor variables were measured in a cohort of 440 infants at birth 6 months, and 1 year of age. Blood pressures at 6 months of age were 93/47 mm Hg (systolic/diastolic pressure, fourth phase) and 97/51 mm Hg at 1 year of age. Serum total cholesterol, beta-lipoprotein, and alpha-lipoprotein levels rose dramatically from birth to 1 year of age. Serum triglycerides also showed an increase from birth to 6 months of age, but a decrease from 6 months to 1 years of age. At 6 months of age, the infants were consuming 949 kcal and at 1 year, 1,356 kcal. A statistically significant correlation between serum cholesterol level and protein, fat, cholesterol, and carbohydrate intake was noted at 1 year of age. These observations provide a background for tracing the evolution of risk factor variables as part of the early natural history of arteriosclerosis.     

Effects of Formula Protein Level and Ratio on Infant Growth, Plasma Amino Acids and Serum Trace Elements II. Follow-up Formula

Abstract. Infants, that had been formula-fed from birth, were fed follow-up formula with 1.5, 2.2 or 2.9 g protein/dl together with 25 g of cereal/day as supplemental food, or formula only (15 g/dl). Formulas were started at 4 months of age and daily intake, anthropometric measurements and plasma samples taken at 5, 6 and 7 months. Protein intake was 2.0, 3.0 and 3.7 g/kg/d, respectively. Growth data were similar for all groups, as were hemoglobin and serum protein values. BUN values for the group fed only formula with 1.5 g protein/dl were lower than for the group fed the same formula with cereals and the other groups. Plasma amino acids were not affected by the addition of the small amount of cereals to the formula with 1.5 g protein/dl, but significantly higher levels of valine, leucine and histidine were found at 7 months for infants fed the two higher protein levels. The highest protein level also appeared to have a negative effect on plasma zinc levels. These results suggest that a protein level of 1.5 g/dl in follow-up formula (2.0 g/kg/d) is adequate during 4 to 7 months of age and that higher protein levels may be excessive.     

Effects of formula protein level and ratio on infant growth, plasma amino acids and serum trace elements. II. Follow-up formula

Infants, that had been formula-fed from birth, were fed follow-up formula with 1.5, 2.2 or 2.9 g protein/dl together with 25 g of cereal/day as supplemental food, or formula only (15 g/dl). Formulas were started at 4 months of age and daily intake, anthropometric measurements and plasma samples taken at 5, 6 and 7 months. Protein intake was 2.0, 3.0 and 3.7 g/kg/d, respectively. Growth data were similar for all groups, as were hemoglobin and serum protein values. BUN values for the group fed only formula with 1.5 g protein/dl were lower than for the group fed the same formula with cereals and the other groups. Plasma amino acids were not affected by the addition of the small amount of cereals to the formula with 1.5 g protein/dl, but significantly higher levels of valine, leucine and histidine were found at 7 months for infants fed the two higher protein levels. The highest protein level also appeared to have a negative effect on plasma zinc levels. These results suggest that a protein level of 1.5 g/dl in follow-up formula (2.0 g/kg/d) is adequate during 4 to 7 months of age and that higher protein levels may be excessive.     

北京地区人群中人Boca病毒血清抗体的分析

目的 通过对血清中人Boca病毒(HBoV)主要衣壳蛋白VP2特异性IgG抗体进行检测,初步了解北京地区人群中这种新发现的病毒的感染状况.方法 以大肠杆菌表达的HBoV主要衣壳蛋白VP2为抗原,应用Western-blot方法,对1996年4月至1997年3月取自首都儿科研究所附属儿童医院健康查体者及北京宣武医院非呼吸道感染患者的血清标本共677份进行HBoV VP2蛋白特异性IgG抗体检测.设抗组氨酸抗体及兔抗HBoV-VP2多肽特异性免疫血清为阳性血清对照.结果 (1)677份血清标本中,抗HBoV VP2蛋白的IgG抗体阳性400份,总检出率为59.1%.(2)被检对象中,＜1个月的婴儿抗体阳性率为45.3%,1个月～的婴儿抗体阳性率为41.4%,2个月～的婴儿抗体阳性率最低(31.3%),6个月～至7岁龄抗体阳性检出率在45.6%～69.7%,7岁后直至40岁,抗体阳性检出率维持在70%左右;50岁后则为61.8%～62.8%.结论 早在1996年北京地区的人群中就有59.1%曾经感染过HBoV,说明这种病毒是一种新发现的病毒而不是新出现的病毒,北京地区人群中该病毒的感染较常见.6个月龄以前的婴儿为易感人群.     

Six-year follow up of phenylalanine intakes and plasma phenylalanine concentrations

The daily Phe intakes of normally growing 1- to 6-year-old treated PKU patients were evaluated. The children received protein in amounts that varied from 2.26±0.47 g/kg body weight per day (mean±SD) at the age of 6 to 1.81±0.35 at the age of 72 months. Mean Phe intakes declining from 34±7 at the age of 6 months to 15±5 mg/kg body weight per day at the age of 72 months were required to maintain mean median plasma Phe levels around 6.0 mg/dl.     

Cows' milk protein-sensitive enteropathy. An important factor in prolonging diarrhoea of acute infective enteritis in early infancy.

The possible role of cows'' milk protein in prolonging diarrhoea in very young infants with acute infective enteritis was studied in 14 infants, 9 under the age of 2 months and 5 older than 6 months. Bacterial pathogens were isolated from the stools of 4 infants from the younger age group. After appropriate initial treatment the infants were maintained on a cows'' milk protein-free formula. 6 weeks later jejunal biopsies were performed before and 24 hours after challenge with a low lactose cows'' milk protein formula. The immunoglobulin and complement levels in the serum and duodenal juice were also estimated at these times. Attempts to isolate bacterial and viral pathogens in stools were again made in all patients. The 5 older infants clinically tolerated cows'' milk protein and their pre- and postchallenge jejunal biopsies were within normal limits. However, significant histological changes were observed in the postchallenge jejunal biopsies of all 9 infants under 2 months of age. In addition, 5 of these infants developed diarrhoea. This suggests that the jejunal mucosa of very young infants previously fed a cows'' milk protein-based formula and who contract infective enteritis suffers damage when rechallenged with cows'' milk protein.     

Diets of healthy Swedish children 4-24 months old. III. Nutrient intake

In a semi-longitudinal investigation comprising a total of 81 infants studied at one or more of the ages 4, 9, 15 and 24 months, the food intake was recorded on three consecutive days at 4 months and on seven consecutive days at 9, 15 and 24 months (40, 40, 38 and 40 infants, respectively in the four age groups). The formula-fed 4-month-old infants had a markedly higher intake of all nutrients, except fat and retinol, than the breast-fed ones. In the three oldest age groups the nutrient intake was high as compared with RDA. The protein intake was exceptionally high. Median intakes that were definitely lower than RDA were noted only for vitamin D, iron and zinc. The nutrient density changed from the age of 9 to 24 months, indicating a transition during that period from infant to adult food habits.     

Food intake and growth of children between 30 and 48 months of age in Jerusalem.

The food intake and body size of a sample of children from a western neighborhood of Jerusalem were determined at 30, 36, and 48 months of age. The mean caloric intake was below the 100% of the Recommended dietary allowance (RDA). The percentage of children receiving less than 100% of the RDA increased slightly with age. Less than 20% of the children had intakes below 60% of the RDA. The mean protein intake was 200% of the RDA. Iron intake met the World Health Organization recommendations. The heme component of the iron intake decreased with age. The mean protein and caloric intake increased as the mother's educational level increased. The weight and height distribution of this population does not differ significantly from the US reference population. At 48 months of age, the children are heavy for their height. Some difficulties of dietary intake studies and the association between food intake and growth are discussed.     

Developmental profile of mitochondrial glycine N -acyltransferase in human liver

Objective: To study the developmental profile of glycine N -acyltransferase (GAT) in the livers of children of various ages and to compare the total and specific GAT activity with that of the adult control subjects.Methods: We measured the specific and the total mitochondrial activity of GAT in liver samples taken from 13 children 4 hours to 11 years of age. The samples were compared with those of control adults aged 24 to 40 years. Samples, either from liver-transplant donors or from autopsy, from those who died of a disorder not related to the liver, were obtained between 6 and 36 hours after death.Results: At 4 hours after birth, very low specific activity and the total liver mitochondrial activity were observed (0.19 μmol/min per milligram protein and 210 mmol/min), with a steady increase up to age 7 months (2.51 μmol/min per milligram protein and 812 mmol/min). The mean specific and total GAT activity in children (n = 5) aged 18 months to 11 years was 6.38 ± 0.13 and 1389 ± 43 and in control adults aged 24 to 40 years (n = 3) was 6.5 ± 0.3 and 1461 ± 71 μmol/min per milligram protein and μmol/min, respectively. These specific and total GAT activity values from children aged 18 months to 11 years were not statistically significant (by analysis of variance and Mann-Whitney test) in comparison with the corresponding activity values from the adult control subjects.Conclusions: Our results indicate that up to age 7 months, children have only 5% to 40% of liver GAT-specific activity, whereas the peak activity is achieved at 18 months and remains constant until age 40 years. The delayed development of GAT in children may thus compromise the detoxification of various drugs and xenobiotics. (J Pediatr 1997;130:1003-7)     

IgG1, IgG2 and IgM responses to two Haemophilus influenzae type b conjugate vaccines in young infants.

PRP-meningococcal outer membrane protein complex (PRP-OMPC) and oligosaccharide linked to variant diphtheria toxin (HbOC) Haemophilus influenzae type b (HIB) conjugate vaccines have both been licensed for United States infants at 2 months of age. Differences in serologic responses for these vaccines have been noted with PRP-OMPC producing an early response at 2 months of age and HbOC producing a higher response after a third dose at 6 months of age. To further characterize the nature of these distinct responses, we measured the IgG1, IgG2 and IgM anti-HIB concentrations by enzyme-linked immunosorbent assay after administration of both vaccines. PRP-OMPC produced an IgM and IgG1 anti-HIB response following the initial dose at 2 months of age. After two doses of HbOC an increase in IgG1 and IgM were noted and after a third dose at 6 months of age an IgG2 anti-HIB response occurred. In addition 33 study subjects were boosted with PRP-OMPC at age 18 months and compared with 34 subjects who received only a primary dose. The anti-HIB IgG1 and IgG2 concentrations following the booster dose were both significantly higher for the primed group (P = 0.0001 and P = 0.001, respectively). Both HIB conjugate vaccines produce predominantly IgG1 anti-HIB antibody responses. The early response to PRP-OMPC vaccine at 2 months of age may result from adjuvant characteristics of the OMPC.     

Influence of protein and energy intakes on body composition of formula-fed preterm infants after term

The aim of the present study was to evaluate changes in body composition in 48 preterm infants in relation to protein and energy intakes from term up to 3 months of corrected age, using air displacement plethysmography. Protein intake (grams per kilogram per day) was negatively associated with percentage of fat mass at 1 month of corrected age. The high-protein-intake group showed greater gain in lean body mass gain than did the low-protein-intake group. This finding suggests that during the first month of corrected age, high protein intake results in a significantly different weight gain composition.     

Protein C activity and antigen levels in childhood

Hereditary protein C deficiency is an important risk factor for thrombosis. To enable its diagnosis shortly after birth, we determined reference values of protein C antigen and activity levels for the first 3 months of life. To establish an age-related range of protein C levels we also determined median values for individuals up to 18 years of age. A good correlation between the two levels was seen from the 3rd/4th month of life onwards, whereas in the first 2 months the activity levels were significantly lower than the antigen levels. This was not due to interference by the increased plasma citrate concentration at high haematocrit values, and may suggest a dysfunctional protein C molecule in the neonatal period. We found a rapid rise in protein C activity and antigen levels until the age of 7–9 months, followed by a slower progression toward adolescence. In contrast to previous reports, our results indicate that adult values are probably not achieved until sometime during the 2nd decade of life.     

The long-term effects of protein energy malnutrition in early childhood on bone age, bone cortical thickness and height.

Three groups of Ugandan children, 18 in each group, and one comparison group of 18 children were examined at 11-17 years of age. The three groups had previously been admitted for treatment of protein energy malnutrition between the ages of 8 to 15, 16 to 21 and 22 to 27 months respectively. The comparison group had not been clinically malnourished throughout the period up to 27 months of age. The children came from one tribe and from similar socio-economic background, and were individually matched on age and sex. The bone age was estimated by hand wrist radiography scored for maturity by the Tanner & Whitehouse method. The metacarpal index, a ratio derived from the medullary width and full diameter of the mid-point of the second metacarpal, was used as a measure of bone cortical thickness. The three malnourished groups are significantly shorter in height than the comparison group, but are not different in bone age and metacarpal index. No differences are observed between the three groups of children who had been admitted for protein energy malnutrition at different ages. The findings are discussed as they relate to the existing literature.