Surgical intervention of intestinal malrotations in paediatric patients without other congenital anatomical abnormalities: overview from a single center.

The subject of malrotation in infants and children without other congenital anatomical abnormalities is reviewed from the perspective of experience with 97 patients operated in 11 years. Fifty-five patients were younger than 6 weeks at operation. They often presented with bilious vomiting, in contrast to older children who presented with non-bilious vomiting or feeding problems. Patients younger than 6 weeks were operated more often acutely than older patients. Volvulus was more common in infants younger than 6 weeks. Two patients with a resulting short bowel syndrome died. In 73 of the surviving 95 (76.8%) children their symptoms disappeared. In the children younger than 6 weeks persisting abdominal problems were significantly less frequent than in older children. In the children presenting with proven gastro-esophageal reflux disease before the malrotation operation, abdominal problems persisted significantly more often. Although there remains considerable controversy over how older children without signs of vascular problems should be managed, failure to respond to radiographic evidence of malrotation could be considered malpractice if volvulus was to occur subsequently. For this reason, every patient with a radiological proven malrotation merits diagnostic laparoscopy.

[Treatment of intestinal failure by total parenteral nutrition at home in children and adults]. / De behandeling van darmfalen met parenterale thuisvoeding bij kinderen en volwassenen.

Intestinal failure is characterised by inability of the intestine to absorb sufficient nutrients to maintain the integrity and function of the body. This can be caused by malabsorption due to too short an intestine or an abnormality of the mucosa, or by a severe motility disorder. In addition to dietary measures, the prescription of total parental nutrition (TPN) at home is sometimes necessary. This treatment is a burden on the patient and the risk of complications must be reduced to a minimum. The risks of long-term parenteral nutrition can be limited and the quality of the provision of services can be increased if the co-ordination is in the hands of a centre for home parenteral nutrition. In the Netherlands there are two centres for home-TPN: the St Radboud University Medical Centre in Nijmegen and the University Medical Centre (AMC) in Amsterdam. In both children and adults, the most common indications are the short bowel syndrome and motility disorders. However, the syndromes that cause this are clearly different in the different age groups. Parenteral nutrition can be given for long periods of time. A large variety of complications can occur, related especially to the equipment or the nutrients. When the nutrition is given via a central venous catheter, then sepsis is a serious and possibly life-threatening complication. In case of administration via an arteriovenous shunt, thrombosis of the shunt is the most frequent problem. If the treatment by means of home-TPN fails, then transplantation of the small intestine should be considered.

[Two newborns with both small-bowel atresia and cystic fibrosis]. / Twee pasgeborenen met zowel dunnedarmatresie als cystische fibrose.

Two newborn girls presented with congenital small-bowel atresia; in one case a high intestinal obstruction had been demonstrated by prenatal echography, while in the other case there were feeding problems and a failure to produce meconium. In both infants, the postoperative period was complicated by feeding problems, malabsorption and insufficient growth. Cystic fibrosis (CF) was then diagnosed in both patients. After modification of the diet, both showed rapid growth to a normal weight. The prevalence of CF in children with congenital small-bowel atresia is 6-13%, which is considerably higher than in a normal population. There is still no good explanation for this finding, but it is likely that CF contributes to the development of small-bowel atresia. In view of the high prevalence of CF in children with small-bowel atresia, children with congenital small-bowel atresia should be examined for CF.

[Extreme dietary selectivity following liver transplantation in a 5-year-old girl; management via behavior therapy]. / Extreme voedselselectiviteit na levertransplantatie bij een 5-jarig meisje; gedragstherapeutische behandeling.

In a 5.1-year-old girl who had been treated by surgical correction of biliary atresia and total orthotopic liver transplantation, extreme dietary selectivity was noted; this was treated by behaviour therapy. On entry in the rehabilitation centre, she manifested malnutrition along with a variety of gastro-enterologic complaints. The treatment consisted of a set of behavioural procedures such as stepwise expansion of the diet, verbal prompting, intermittent contingent attention and a list of agreements with a system of rewards; this led to the elimination of the dietary selectivity and to the consumption of a varied diet in a normal tempo.

The effect of a micronutrient-fortified complementary food on micronutrient status, growth and development of 6- to 12-month-old disadvantaged urban South African infants.

The study was conducted to look at the effectiveness of a multimicronutrient-fortified complementary food on the micronutrient status, linear growth and psychomotor development of 6- to 12-month-old infants from a black urban disadvantaged community in the Western Cape, South Africa. The study was designed as an intervention study. In both the experimental and control groups, serum retinol concentration showed a decline over the intervention period of 6 months. The decline was less pronounced in the experimental group. This resulted in a significantly (P<005) higher serum retinol concentration at 12 months in the experimental group (26.8+/-5.8 microg/dl) compared with the control group (21.4+/-5 microg/dl). Serum iron concentration also declined over the intervention period. The decline was less pronounced in the experimental group. No difference was observed in haemoglobin levels between the groups at 12 months. Serum zinc concentration did not differ significantly between the two groups at follow up. Weight gain over the 6 months period did not differ significantly between the experimental (2.1+/-0.9 kg) and control groups (2.1+/-1.2 kg). There was no difference in linear growth between the experimental (10.0+/-1.5 cm) and control group (10.1+/-2.1 cm) at the end of the follow-up period. Weight and length at 6 months significantly predicted weight and length at 12 months. No difference was observed in psychomotor developmental scores between the two groups after 6 months of intervention. Introducing a multimicronutrient-fortified complementary food into the diet of 6- to 12-month-old infants seemed to have an arresting effect on declining serum retinol and iron concentration in the experimental group. No benefit was observed in serum zinc concentration, linear growth and psychomotor development.

[Microvillus inclusion disease, a rare cause of severe congenital diarrhea]. / Microvillusinclusieziekte, een zeldzame oorzaak van ernstige aangeboren diarree.

To date, microvillus inclusion disease (MID) has been diagnosed in six Dutch patients. It is a rare autosomal recessive hereditary intestinal disorder mostly presenting with malabsorption and severe secretory diarrhoea from birth. The diagnosis is confirmed by electron microscopy of intestinal mucosal biopsies, which show characteristic intracytoplasmic vesicles containing clearly recognisable microvilli and irregularly distributed microvilli in the brush border. The two clinical forms of the disease that have been recognised internationally, a 'congenital' and a 'late-onset' form of MID, have also been observed in the Dutch patients. At the last follow-up five patients had died, the sixth was 17 years old and alive. The pathogenesis and genetics of MID are, as yet, unknown. Eventually, all patients die from complications of the disease, notably from the total parenteral nutrition. The only chance of survival is intestinal or combined liver-intestinal transplantation.

Disadvantaged black and coloured infants in two urban communities in the Western Cape, South Africa differ in micronutrient status.

OBJECTIVES: To determine the nutritional and health status of urban infants in two disadvantaged communities in the Western Cape, South Africa with special reference to micronutrient status. The results of this study will serve to plan an intervention study in these communities in the same age group. DESIGN: Cross-sectional study. SETTING: Two disadvantaged urban black and 'coloured' communities in the Western Cape, South Africa. SUBJECTS: Sixty infants aged 6-12 months from each community. OUTCOME MEASURES: Dietary intake, anthropometric measurements, micronutrient status and psychomotor development. RESULTS: Stunting and underweight were more prevalent in coloured infants (18% and 7%, respectively) than in black infants (8% and 2%, respectively). Anaemia (haemoglobin (Hb) < 11 g dl(-1) was prevalent in 64% of coloured and 83% of black infants. Iron-deficiency anaemia (Hb < 11 g dl(-1) and ferritin < 10 ng ml(-1) was found in 32% of coloured infants and in 46% of black infants. Zinc deficiency was prevalent in 35% and 33% of the coloured and black infants, respectively. Marginal vitamin A deficiency (serum retinol < 20 microg dl(-1) was observed in 23% of black infants compared with 2% of coloured infants. Of black infants, 43% and of coloured infants 6% were deficient in two or more micronutrients. Six per cent of coloured infants had C-reactive protein concentrations above 5 mg l(-1) compared with 38% of the black infants. The dietary intake of micronutrients was in general lower in black infants than in coloured infants. The overall psychomotor development, assessed by the Denver Developmental Screening Test, was different between the two groups. The coloured infants scored higher in three out of the four categories as well as in their overall score. CONCLUSIONS: This study shows that information on stunting and wasting only in urban disadvantaged infants is not sufficient to make recommendations about specific community intervention programmes. Information on the micronutrient status, independent of wasting and stunting, is necessary to design nutrition programmes for different communities. The study also showed a substantially higher prevalence of micronutrient deficiencies among black infants.

Basal metabolic rate in children with a solid tumour.

OBJECTIVE: To study the level of and changes in basal metabolic rate (BMR) in children with a solid tumour at diagnosis and during treatment in order to provide a more accurate estimate of energy requirements for nutritional support. DESIGN: An observational study. SETTING: Tertiary care at the Centre for Paediatric Oncology, University Hospital Nijmegen. SUBJECTS: Thirteen patients were recruited from a population of patients visiting the University Hospital Nijmegen for treatment. All patients asked to participate took part in and completed the study. INTERVENTION: BMR was measured by indirect calorimetry, under stringent, standardised conditions, for 20 min and on three different occasions in all patients. Continuous breath gas analysis using a mouthpiece was performed. Weight, height and skinfold measurements were performed before each measurement. MAIN OUTCOME MEASURES: BMR was expressed as percentage of the estimated reference value, according to the Schofield formulas based on age, weight and sex, and in kJ (kcal) per kg of fat-free mass. RESULTS: At diagnosis, the BMR was higher than the estimated reference BMR in all patients and 44% of the patients were considered hypermetabolic. Mean BMR (as percentage of reference) was significantly increased (11.6% (s.d. 6.7%); P=0.001), but decreased during treatment in 12 of the 13 patients (mean decrease 12.7% (s.d. 3.9%); P<0.0001). Furthermore, a significant negative correlation (P=-0.67; P=0.01) was found between the change in BMR and tumour response. CONCLUSIONS: These data suggest that the BMR of children with a solid tumour is increased at diagnosis and possibly during the first phase of oncologic treatment. This may be important when determining energy requirements for nutritional support.

Nasogastric tube feeding in children with cancer: the effect of two different formulas on weight, body composition, and serum protein concentrations.

BACKGROUND: Treatment of cancer cachexia partly involves the administration of adequate amounts of energy. The aim of this study was to assess the tolerance and efficacy of two equal volumes of tube feeding, one with a standard (1 kcal/mL) and one with a high energy density (1.5 kcal/mL), during the intensive phase of treatment. METHODS: Nutritional status was assessed weekly, in 27 children with a solid tumor, by measuring weight, height, midupper arm circumference, biceps and triceps skinfold, and serum proteins. Tolerance was assessed by recording the occurrence of vomiting and by expressing the administered volume as a percentage of the required volume. RESULTS: Both formulas were equally well tolerated, leading to a significantly higher energy intake in the energy-enriched formula group. In both formula groups, all anthropometric variables increased significantly (range of mean increase, 5.2% to 25.5%; p < .05) during the first 4 weeks of intervention. Between 4 and 10 weeks, variables continued to increase significantly in the energy-enriched group, resulting in adequate repletion, in contrast to the standard formula group. The concentration of serum proteins, low at initiation of tube feeding, returned to the normal range within 2 to 4 weeks with no significant differences between the two groups. CONCLUSIONS: The energy-enriched formula was more effective in improving the nutritional status of children with cancer during the intensive phase of treatment than the standard formula. Intensive, protocolized administration of an energy-enriched formula should therefore be initiated as soon as one of the criteria for initiation of tube feeding is met.

Association between the change in nutritional status in response to tube feeding and the occurrence of infections in children with a solid tumor.

In 32 children with a solid tumor, the association between the change in weight for height, in response to 4 weeks of tube feeding during the intensive phase of treatment, and the occurrence of leukopenia, leukopenic infections, and nonleukopenic infections in a period thereafter (4-10 weeks) was studied. Factors possibly influencing the change in weight for height during the first 4 weeks of tube feeding were also assessed. A statistically significant negative correlation (rho = -0.59; p < .001) was found between the change in z-score of weight for height in response to the first 4 weeks of tube feeding, and the occurrence of nonleukopenic infections between 4 and 10 weeks. A reduced occurrence of nonleukopenic infections resulted in a significant reduction of the number of days of infection-related hospital admission (rho = .45; p = .009), which, besides providing advantages for the patient, also had economical benefits. The change in weight for height in response to tube feeding was mainly influenced by the incidence of therapy-induced vomiting (r = -.45; p = .02) and by the amount of energy provided by tube feeding (r = .47; p = .007). Based on these findings, it is recommended that naso-gastric tube feeding be used in children with a solid tumor during the early intensive phase of treatment, and that one should aim for a considerable increase in weight for height during the first 4 weeks of administration, since this has been shown to reduce the number of nonleukopenic infections in a subsequent period. The increase in weight for height may be improved by providing an optimal antiemetic protocol, which will increase energy uptake, and an energy-enriched formula, which will increase energy intake.

Nutritional status of breastfed infants in rural Zambia: comparison of the National Center for Health Statistics growth reference versus the WHO 12-month breastfed pooled data set.

Cross-sectional data for breastfed infants in rural Zambia were used to evaluate the effect of applying two different data sets as a reference, i.e. the WHO 12-month breastfed pooled data set and the National Center for Health Statistics (NCHS) growth reference in terms of prevalence of malnutrition (stunting, underweight, and wasting). A total of 518 infants who were attending mother-and-child health clinics were included. Age, weight and length were recorded. Anthropometric Z-scores were calculated in two ways: by applying the NCHS growth reference and by using the WHO breastfed data set. Anthropometric Z-scores calculated using the breastfed data set were lower during the first 6-7 months of life compared with those calculated by applying the NCHS growth reference. This resulted in a higher proportion of children aged 0-6 months being classified as stunted and underweight using the breastfed data set versus the NCHS growth reference. After the age of 7 months, similar prevalences of stunting or underweight were observed. Relatively few infants were classified as wasted. In order to adequately assess the prevalence of stunting and underweight in breastfed infants, it is recommended that a new growth reference be developed, as has been initiated by WHO.

Consequences of infections for three-month length increment in young children in rural Zambia.

It was the aim of this study to describe the relationship of infections with subsequent 3-mo length increment in children below 2 y of age in rural Zambia. Children aged 6-9 mo ('infants'; n = 84) and 14-20 mo ('toddlers'; n = 81) attending Mother-and-Child Health clinics, were included and followed up after 3.0 mo (min-max; 2.1-3.7 mo). Anthropometric measurements were taken at each visit. At baseline, C-reactive protein (CRP), alpha1-acid glycoprotein (AGP), retinol and malaria parasitaemia were assessed. Length increment during the 3.0+/-0.5 mo was 1.0+/-0.5 cm/mo for infants and 0.6+/-0.4 cm/mo for toddlers; 50-71% of the children showed increased acute phase proteins, 79-83% had malaria parasitaemia and 55-64% had low serum retinol concentrations. In the total group of children, serum AGP concentrations (r = -0.18; p = 0.03) and serum CRP concentrations (r = -0.15; p = 0.05) showed a negative relation with length increment. After correcting for micronutrient status, dietary intake and maternal height, results of multiple regression analyses showed that the relation between serum AGP concentration and subsequent length increment remained significant. We conclude that, within the multifactorial model, presence of infections in these Zambian children contributes to the short-term retardation of linear growth.

Severe linear growth retardation in rural Zambian children: the influence of biological variables.

BACKGROUND: The prevalence of stunting in preschool children in Zambia is high; stunting has detrimental effects on concurrent psychomotor development and later working capacity. OBJECTIVE: Our objective was to investigate biological variables that may contribute to linear growth retardation in preschool children in Samfya District, Zambia. DESIGN: Children aged 6-9 mo (n = 108) and 14-20 mo (n = 102) attending mother-and-child health clinics were included. With a mixed-longitudinal design, they were followed up 9 and 21 mo later. Height and weight of children and their mothers were measured. Biochemical measures (eg, serum zinc, retinol, thyrotropin, iron, and acute phase protein concentrations), malaria parasitemia, and intestinal parasitosis were assessed. RESULTS: Height-for-age z scores (HAZ) were low, indicating a high prevalence of stunting (36-79%). Ninety percent of the children were anemic, 53-71% had elevated acute phase proteins, and 80% had malaria parasitemia. Regression analyses showed that maternal height predicted the children's height at 6-9 mo (regression coefficient = 0.05; 95% CI: 0.02, 0.08). The children's height at an early age (6-9 and 14-20 mo) showed a strong relation with their height at later ages (22-30 and 34-41 mo). Serum micronutrient status did not show a significant relation with later HAZ. CONCLUSION: Unlike other studies, we did not identify specific biological factors, such as health and micronutrient status, which contribute to the retardation of linear growth. The normal zinc and iodine statuses of the children suggest that at least these factors are not causal.

Food consumption of young stunted and non-stunted children in rural Zambia.

OBJECTIVE: The aim of this study was to assess the quality of diet of rural Zambian pre-school children, and to compare the dietary intake of stunted and non-stunted children. DESIGN: Cross-sectional study, in which dietary intake was assessed with a 24-h recall method. Height and weight were measured according to standard procedures. SETTING: Twelve villages in Samfya District, Zambia. SUBJECTS: Children aged 6-9 months ('infants') and 14-20 months ('toddlers'), attending Mother-and-Child Health Clinics, were eligible for study. Excluded were: 12 wasted, and 18 for other reasons. In total 106 infants and 99 toddlers were included. RESULTS: In infants and toddlers, total daily intake of energy, calcium, iron, and vitamin A was insufficient compared to recommended daily intakes. Only infants had insufficient protein intake. Compared to intake from weaning foods, breast milk was the main source of energy and most nutrients for infants. For toddlers, weaning foods were more important. Stunted infants and toddlers tended to have lower intakes of energy compared to non-stunted age-mates. Daily energy intake per kg bodyweight showed no difference between stunted and non-stunted children. CONCLUSIONS: Overall quality of weaning foods was inadequate. Stunted infants and toddlers showed a tendency of lower energy intakes compared to non-stunted age-mates.

Malaria is associated with reduced serum retinol levels in rural Zambian children.

Vitamin A deficiency increases the risk of illness, while infections impair vitamin A status. Malaria is highly prevalent in rural Zambia. We describe the relationship between malaria and vitamin A status. We examined dietary vitamin A intake, malaria parasitaemia and serum concentrations of retinol, C-reactive protein (CRP) and alpha 1-acid glycoprotein (AGP) in 210 children under the age of 2. Vitamin A intake was low. Serum retinol was negatively correlated with malaria parasite count and to serum levels of CRP and AGP. Increased malaria parasite density resulted in raised CRP and AGP levels, which were negatively associated with serum retinol. We conclude that improvement of dietary vitamin A intake and prevention of infectious diseases, especially malaria, could alleviate vitamin A deficiency in this population.