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.

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.

Ocular and systemic manifestations of cerebrotendinous xanthomatosis.

PURPOSE: Cerebrotendinous xanthomatosis is a storage disease that usually leads to severe mental and neurologic deterioration before the diagnosis and start of treatment are established. We identified major ocular and systemic characteristics that may enable a diagnosis to be made earlier. METHODS: Ten patients (group 1) of the University Hospital Nijmegen, with a diagnosis of cerebrotendinous xanthomatosis, were re-examined for detailed ocular and major clinical manifestations. Meanwhile, we looked for similar but undiagnosed cases in patients (group 2) who visited the Institute of Ophthalmology during a 12-month period. RESULTS: A diagnosis of cerebrotendinous xanthomatosis had been made in the patients of group 1 at an average age of 40 years (range, 33 to 48 years). Subsequently, six new cases (group 2) were diagnosed in patients 7 to 37 years old (average age, 18 years). Bilateral cataract was the major ocular manifestation in all 16 patients. Small irregular corticonuclear opacities, anterior polar cataracts, and dense posterior subcapsular cataracts were diagnosed at various ages (mean, 18 years; range, 4 to 40 years). Four patients showed clinical signs of optic neuropathy, whereas retinal function was normal in all patients. Other major clinical signs included a history of chronic diarrhea (since childhood), mental deterioration (mean age, 23 years), neurologic deterioration (mean age, 31 years), and tendon xanthomas (mean age, 37 years). CONCLUSIONS: Appropriate biochemical investigations for cerebrotendinous xanthomatosis should be performed in patients with unexplained juvenile or early-onset adult cataracts, especially if these cataracts are associated with chronic diarrhea since infancy, mental retardation or deterioration, neurologic dysfunction, or xanthomas.

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.

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.

Effects of naso-gastric tube feeding on the nutritional status of children with cancer.

OBJECTIVE: To study the effect of sufficient energy intake, by means of the protocolized administration of naso-gastric tube feeding, on the nutritional status of a child with cancer. DESIGN: A comparative experimental study. SETTING: Tertiary care at the Centre for Pediatric Oncology, South East Netherlands, University Hospital, Nijmegen. SUBJECTS: Seven children, newly diagnosed with cancer, were included in the experimental study and all completed the trial period. Fourteen patients were included in the retrospective study. They were randomly chosen from a group of patients previously treated for a malignancy at our department and who had received naso-gastric tube feeding for at least 16 weeks. INTERVENTION: Protocolized (experimental group) vs non-protocolized (retrospective group) administration of naso-gastric tube feeding over a period of 16 weeks. The main difference was the amount of tube feeding administered. In addition to energy from other foods, children in the experimental group received 106+/-13% of their total daily energy requirements (TDER) by means of tube feeding, whereas children in the retrospective group had received 75+/-24%. MAIN OUTCOME MEASURES: Weight as a percentage of weight for height according to the 50th percentile of a healthy reference population=ideal weight. RESULTS: Weight, expressed as a percentage of the ideal weight, increased significantly in the experimental group (18.2 8.4; P=0.01) and the retrospective study group (5.2 7.3; P=0.001). However, the increase was statistically significant in favour of the experimental group (P=0.003), in which all the children reached their ideal weight, compared to 21% in the retrospective group. CONCLUSION: Aggressive protocolized nutritional intervention during the intensive phase of anti-cancer treatment, in the form of naso-gastric tube feeding that provides the child's total daily energy requirements, results in considerable improvement in the nutritional status.

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.

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.

[Hepatitis B in children in 4 university centers in The Netherlands]. / Hepatitis B bij kinderen in vier universitaire centra in Nederland.

OBJECTIVE: To evaluate the prevalence and the natural course of hepatitis B infection in children. DESIGN: Retrospective longitudinal. SETTING: Four university pediatric centres. METHOD: To explore the possibility of starting a trial with interferon alpha, data of viral and biochemical tests and biopsies of children younger than 16 years were studied. RESULTS: In a period of 10 years (1980-1990) 145 patients, of whom 74% were not of original Dutch descent, were found positive for HBsAg. The data of 142 patients could be analysed. Seroconversion was seen in 27 patients and 42 were already anti-HBe positive at the time of presentation. Chronic hepatitis, representing the category which could benefit from interferon alpha treatment, was found in 24 patients. Severe complications of the hepatitis were found in 4% of the children, including hepatocellular carcinoma and cirrhosis. Follow-up was insufficient so the seroconversion rate could only be estimated at 12% for the first year following the diagnosis. CONCLUSION: As a result of this study the authors present a proposal for a therapeutic trial with interferon alpha. This is a national protocol under the auspices of the section for pediatric gastroenterology of the Nederlandse Vereniging voor Kindergeneeskunde (Netherlands Pediatric Association).

[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.

[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.

[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.

[Neonatal jaundice, a diagnostic approach]. / Icterus neonatorum, een diagnostische benadering.

Jaundice is a common feature in full-term and premature newborns. Considering this high incidence it is important to distinguish physiologic and non-physiologic jaundice. This differentiation is complicated by the presence of a large overlap between ranges of normal and pathologic bilirubin levels in serum. A combination of criteria is proposed on the basis of which on one hand the risk of missing any pathology will be minimal and on the other hand a too extensive diagnostic work-up in patients with a physiologic jaundice is prevented. A systemic approach to neonatal jaundice is described.

[Neonatal hemangiomatosis with prolonged icterus]. / Neonatale haemangiomatosis met icterus prolongatus.

A six week old girl with multiple cutaneous haemangiomas and cholestatic jaundice is described. An exploratory laparotomy revealed neither extra- nor intrahepatic biliary atresia. The diagnosis of a diffuse neonatal haemangiomatosis was made. Prednison therapy induced an almost complete normalization of the liverfunction disturbances. Surgical therapy is not the recommended approach for neonatal haemangiomatosis with cholestatic jaundice.

[An infant with short stature and red cheeks (Rothmund-Thomson syndrome)]. / Een te kleine zuigeling met rode wangen (het syndroom van Rothmund-Thomson.

An infant with short stature and progressive skin lesions of cheeks and dorsum of the hands is described. Further problems such as recurrent diarrhoea and respiratory infections suggested zinc-deficiency, malabsorption-syndrome, Bloom syndrome and early Lupus Erythematosus respectively. Finally Rothmund-Thomson syndrome was diagnosed. This rare genetic disorder is characterized by variable expression of typical cutaneous changes, cataracts, skeletal anomalies, short stature, abnormal hair growth and defective nails and teeth, mental retardation, hypogonadism and a typical facial appearance.