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.

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.