To determine the incidence of common-bile-duct lesions and their relation to liver disease in cystic fibrosis, we performed hepatobiliary scanning in 50 of 61 patients with cystic fibrosis who had hepatomegaly, abnormal liver function, or both and in 31 of 92 patients with cystic fibrosis who did not have hepatomegaly or abnormal liver function. Ninety-six percent of the patients with liver disease had evidence of biliary tract obstruction, which was defined cholangiographically as a stricture of the distal common bile duct in the majority of cases. All the patients without liver disease had normal intrahepatic and common-duct excretion of tracer. Abdominal pain was significantly more common in patients with common-duct obstruction (P less than 0.001), and enlarged gallbladders occurred only in such patients. Since fasting levels of serum bile acids were elevated in nearly half these patients, irrespective of the severity of their liver disease, serum bile acids may be markers of the severity of the common-duct lesion. We conclude that strictures of the distal common bile duct are common in patients with cystic fibrosis and liver disease. This association requires further study, since surgical relief of common-duct obstruction may prevent or ameliorate the hepatic complications of cystic fibrosis.

Previous studies have suggested that children with phenylketonuria (PKU) have a reduction in bone mineralization compared with control subjects. To investigate this, bone mineral density (BMD) of the total body (TBMD) was measured in 32 prepubertal children with PKU and in 95 age-matched control subjects. Spine bone mineral density (SBMD) was also recorded in a subset, 24 with PKU and 55 control subjects. The effect of dietary intake on bone mass was assessed in 30 of the children with PKU and in 12 control subjects. In the children with PKU, TBMD and SBMD were significantly lower than in the control subjects after adjustment for height and weight (P = 0.03 and P = 0.003, respectively). The children with PKU had a higher intake of calcium (P < 0.0001), phosphorus (P = -0.0002), and magnesium (P < 0.0001), suggesting that their lower BMD occurred despite an adequate diet based on current recommendations. Further study is needed to establish the cause of this deficit in bone mass and the benefit of additional nutritional support to reverse this problem.

The use of areal bone mineral density (aBMD) in paediatric populations has aroused some concern, as it fails to take the age‐related increase in bone thickness into account. We have developed a measure of true bone density, volumetric bone mineral density (vBMD), which is independent of age and height. In order to examine the relationship between growth parameters, aBMD and vBMD, we studied patients with phenylketonuria (PKU, n = 40), chronic renal failure (CRF, n = 27) and chronic asthma (n = 19). aBMD of the femoral neck and the mid‐femoral shaft was measured using dual energy X‐ray absorptiometry (DXA), vBMD was calculated on the basis of values of bone mineral content and bone dimension provided by DXA, with the assumption that both sites are cylinders. aBMD and vBMD were then compared with the normal reference, expressed as a standard deviation score (SDS). aBMD and vBMD were normal in the femoral neck region of the PKU group, but aBMD, either standardized for age or for height, was low in the femoral shaft region (p < 0.01). In the CRF group, profound growth retardation was seen (mean height SDS, −3.2) and aBMD and vBMD were both low in the femoral shaft region but not in the femoral neck. In the asthma group, aBMD for age was low at both sites, but vBMD did not differ from that seen in normal individuals. We conclude that the true vBMD provides a different interpretation of bone density compared with aBMD and requires further evaluation in paediatrics because of its age and height independence.

Objective: To investigate the growth and feeding practices in first-generation Vietnamese infants living in Australia. Design: Cohort study. Setting: The study was conducted between 1999 and 2002 in Sydney. Subjects: A total of 239 Vietnamese women were recruited randomly from antenatal clinics, and of these 210 were initially seen. During the first year, 20 cases (9.5%) were lost to follow-up. Data were collected at 0.5, 2, 4, 6, 9 and 12 months. Results: Vietnamese infants were significantly longer and heavier than reference data (both Po0.0001). The Vietnamese infants had a significant decline in weight growth with age compared with reference data (Po0.001). The Vietnamese infants had marginally higher s.d. score for ideal weight for length than reference data (P ¼ 0.044). There was a significant decline in ideal weight for length with age compared with reference data (P ¼ 0.0065). Both parents were significantly shorter (mean s.d. height scores: À1.570.8 (mother) and À1.870.8 (father)) than reference data (Po0.001). The incidence of breast feeding was 79%, but half of the breast feeding women had stopped breast feeding by 3 months. A total of 162 (79.8%) infants were given infant formula within the first week, of whom 131 (80.1%) were fed infant formula within the first 24 h after birth. Conclusions: Vietnamese infants in this study had growth comparable with reference data despite their parents being shorter than reference data. Breast feeding duration was short with infant formula being introduced early. Sponsorship:

Prevalence of iron deficiency at 18 months was high despite appropriate infant feeding practices during the first year. Modification of the diet in the second year of life may decrease the risk of iron deficiency in Vietnamese children.

Objective: To investigate in children with cystic fibrosis (CF) and children without CF: (1) the test -retest reproducibility of a 20 min resting energy expenditure (REE) measurement; and (2) the long-term reproducibility of REE measurements in children with CF using longitudinal data. Design: Cross-sectional study and longitudinal cohort. Setting: A tertiary referral paediatric hospital. Subjects: A total of 31 (11 male, 20 female) children (aged 12.8 AE 3.6 y) with CF and 32 (14 male, 18 female) healthy children without CF (aged 12.2AE 2.3 y) were enrolled in the short-term reproducibility study. Long-term REE measurement reproducibility was assessed in another 14 children (5 male, 9 female) with CF, comparing their initial REE measurement with a subsequent measurement 1 -2 y later. Methods: All children had measurements of height, weight, skinfold thickness and indirect calorimetry. Results: There was no statistically significant difference in REE between repeated measurements in children with CF (mean AE s.d., 6240AE 1280 and 6220 AE 1315 kJ=24 h) and in the children without CF (6040 AE 956 and 6015 AE 943 kJ=24 h). For the children with CF, the intraclass correlation coefficient was 0.99 and for children without CF the intraclass correlation coefficient was 0.97. The measurement errors were 119 and 177 kJ, respectively. Approximately 80% of the variation in REE in the CF group and 70% in the group without CF was explained by fat-free mass (FFM). Analysis of the longitudinal CF data show there was no difference in REE between a child's first measurement (5140 AE 1140 kJ) and their subsequent measurement (5460 AE 1190 kJ), after adjustment for changes in body size between the measurements. Conclusion: This study has demonstrated that a short-term 20 min REE measurement is reproducible and therefore valid in children with CF and children without CF. These results also indicate that in children with CF, long-term REE measurements are reproducible.

The incidence of gastro-oesophageal reflux in children undergoing general anaesthesia has not previously been studied. Onehundred-and-twenty children (ASA Class 1-2) were studied intraoperatively using continuous oesophageal pH monitoring. The incidence of reflux was 2.5% (3 of 120). None of these three patients had an adverse respiratory event. There was no correlation between reflux and adverse respiratory events. Thirteen patients had minor respiratory events without evidence of acid reflux. Gastro-oesophageal reflux does occur in healthy paediatric patients having minor surgery, but was not a significant cause of the adverse respiratory events that occurred in our study.

Nutritional status, growth and development in children undergoing intensive treatment for cystic fibrosis. Acta Paediatr Scand [Suppl] 366: 106,1990.Dietary intakes were measured over a period of 5 days in 36 malnourished and 36 wellnourished patients with cystic fibrosis. Both energy and protein intakes were significantly less in the malnourished patients for the two age groups studied: 4-9.99 years ( p < 0.01 for both parameters), and 10-16 years ( p c 0.05 and p < 0.01, respectively). In both age groups and both patient groups, average protein intakes were well in excess of the recommended daily intake, but energy intake in the malnourished patients was below the recommended daily intake. Nutritional supplementation of 10 malnourished patients with a polymeric formula, infused overnight via a gastrostomy tube, resulted in a seven-fold increase in weight gain ( p < 0.001) and a doubling of linear growth velocity ( p < 0.01) over a period of 18 months, compared to the 18 months prior to gastrostomy feeding. Measurements of total body nitrogen in eight of these patients demonstrated a 38% increase in body nitrogen content over 12 months, indicating a replenishment of the protein deficit.