A double-blind randomised controlled trial of fish oil-based versus soy-based lipid preparations in the treatment of infants with parenteral nutrition-associated cholestasis.

BACKGROUND: Infants receiving prolonged parenteral nutrition (PN) are at risk of PN-associated cholestasis (PNAC). This can progress to hepatic failure and death if PN cannot be discontinued. Fish oil-based parenteral lipid preparation (FOLP) has been shown to be beneficial in case studies. OBJECTIVES: (1) To evaluate whether FOLP could halt or reverse the progression of PNAC compared with soy-based parenteral lipid preparation (SLP) and (2) to assess the effects of FOLP on liver function and physical growth. DESIGN: double-blind randomised controlled trial. SETTING: level III neonatal intensive care unit. PARTICIPANTS: infants with PNAC (plasma-conjugated bilirubin concentration ≥ 34 µmol/l or 2 mg/dl) expected to be PN-dependent for >2 weeks. INTERVENTION: to receive either FOLP or SLP at 1.5 g/kg/day. PRIMARY OUTCOME MEASURE: reversal of PNAC within 4 months after commencement of lipid treatment; secondary outcomes: rate of change of weekly liver function tests, infant growth parameters, blood lipid profile and episodes of late-onset sepsis. RESULTS: A total of 9 infants were randomised to the FOLP group and 7 to the SLP group. There was no significant difference in reversal of PNAC at 4 months between groups. Rates of increase of plasma-conjugated bilirubin and alanine aminotransferase in the SLP group were significantly greater than the FOLP group (13.5 vs. 0.6 µmol/l per week and 9.1 vs. 1.1 IU/l per week, respectively, p = 0.03). Increased enteral nutrition was associated with significant improvement of PNAC in infants receiving FOLP compared with SLP (-8.5 vs. -1.6 µmol/l per 10% increase in enteral nutrition, respectively). The study was terminated prematurely. CONCLUSIONS: progression of PNAC in PN-dependent infants can be halted by replacing SLP with FOLP and reversed by increasing the proportion of enteral nutrition in infants receiving FOLP. Replacement of SLP with FOLP in PN-dependent infants who develop PNAC may be considered.

Socioenvironmental factors associated with constipation in Hong Kong children and Rome III criteria.

OBJECTIVE: Data regarding the prevalence of constipation in the general population of Asian children using internationally standardized definitions are scarce. Environmental factors surrounding a child's day to day living may trigger or perpetuate constipation and encourage postponement of defecation. METHODS: A territory-wide cross-sectional questionnaire survey was conducted in 2318 Hong Kong Chinese elementary school students. Constipation was defined by pediatric Rome III criteria. RESULTS: The mean age of the children was 9 ± 1.9 years; 51% were boys. Two hundred eighty-two children (12.2%, 95% confidence interval [CI] 10.9%-13.5%) were found to have constipation. Children ages 6 to 7 years had the highest prevalence (16.8%, 95% CI 13.8%-19.8%). There was no difference in prevalence between boys and girls (11.6% vs 12.3%; P > 0.05) and between obese and nonobese children (11.5% vs 11.1%; P > 0.05). In univariate analysis, constipation was found to be significantly more prevalent among those children who lived with neither parent, had inadequate company of parents at home, refused to pass bowel movements in school, spent long hours doing homework, had inadequate sleep, and had decreased fiber intake and frequent consumption of fast food (P < 0.05). Multivariate analysis identified refusal to pass bowel movements in school toilets (odds ratio [OR] 1.97, 95% CI 1.42%-2.74%), having dinner with one/both parents <50% of time (OR 1.52, 95% CI 1.01%-2.31%), nighttime sleep <7 hours (OR 1.87, 95% CI 1.04%-3.33%), and frequent consumption of fast food (OR 1.14, 95% CI 1.03%-1.26%) to be independent factors associated with constipation. CONCLUSIONS: Socioenvironmental factors are associated with childhood constipation, and bringing them to the awareness of the public may help prevent or stop the progression of childhood constipation at its early stages.

Rescue treatment of infants with intestinal failure and parenteral nutrition-associated cholestasis (PNAC) using a parenteral fish-oil-based lipid.

Four preterm infants with intestinal failure and severe parenteral nutrition-associated cholestasis (PNAC) received fish-oil-based parenteral lipid as rescue treatment in substitution for the standard soybean-based lipid preparation. The progression of liver disease was halted in 3 infants and they recovered with complete resolution of PNAC. The condition in two of these infants would almost certainly have progressed to end-stage hepatic failure if they had continued to receive long-term parenteral nutrition and <30% of total nutrition enterally. The remaining infant with residual inflamed bowel, protracted feeding intolerance and repeated episodes of sepsis did not respond. Our findings suggest that fish-oil-based parenteral lipid emulsion may contribute to effective treatment of PNAC in selected patients, which should be further evaluated in randomized controlled trials.

Congenital intrathoracic stomach: short esophagus or hiatal hernia?

We present a rare case of congenital intrathoracic stomach due to short esophagus in a preterm infant. Antenatal ultrasound during the second trimester showed a tubular cystic structure in the posterior mediastinum. The diagnosis of congenital hiatal hernia was initially made. After birth, the diagnosis was confirmed to be intrathoracic stomach associated with a congenitally short esophagus by upper gastrointestinal contrast study and laparotomy. Features that suggest intrathoracic stomach secondary to congenital short esophagus over the relatively more common congenital hiatal hernia include early identification of the intrathoracic stomach during the second trimester and the consistent absence of an abdominal stomach bubble on antenatal ultrasound. The infant's condition could not be surgically repaired due to the extremely malformed and short esophagus and she subsequently died. This is in sharp contrast to the more surgically amenable and non-life-threatening congenital hiatal hernia. Early recognition of intrathoracic stomach associated with congenital short esophagus is important as it is associated with difficult management and significant postnatal complications. Fetal magnetic resonance imaging may be useful in this situation. The prognosis is cautiously guarded, and the parents should be appropriately counseled.

Familial extrarenal Wilms tumor.

BACKGROUND/PURPOSE: This study aimed to illustrate the first report of extrarenal Wilms tumor occurring in a family. MATERIALS AND METHODS: Retrospective case note review of 3 siblings, 2 of which presented with extrarenal Wilms tumor. Immunohistochemical analysis for WT1 gene product was performed together with molecular genetic linkage studies. RESULTS: A 3-year-old boy had excision of a right-sided extrarenal retroperitoneal Wilms tumor and nephrectomy followed by chemotherapy. At follow-up of 4 years, the boy was well and thriving. Aged 2 years, his sister developed a left-sided retroperitoneal extrarenal Wilms tumor. She had a tumor excision and nephrectomy followed by chemotherapy. She was well on follow-up more than a year after completion of treatment. Immunohistochemical analysis identified WT1 gene product within the tumor for both cases. Molecular genetic linkage studies showed no linkage between the index cases at FWT1 locus. Although possible linkage was demonstrated at WT1 locus, no mutation was found in the coding sequence and intron/exon boundaries of WT1 gene in index patient 1. A possible linkage between the index cases was also found at FWT2 locus. This could be a chance event because of the close relationship of the 2 patients. CONCLUSIONS: We could identify extrarenal Wilms tumor in a family for the first time. Immunohistochemical analysis showed WT1 gene products in both cases. Linkage studies for Wilms tumor genes within the family were inconclusive. The possible linkage between the 2 index cases may be a chance event.

Minimally invasive approach in the management of childhood intussusception.

BACKGROUND: Intussusception is one of the most common causes of intestinal obstruction in infancy. Non-operative reduction using air enema or other hydrostatic reduction methods has been the standard treatment in most cases. However, if the non-operative method is not indicated or fails, open surgery is still necessary. With the tremendous development of the minimally invasive approach in handling surgical conditions in children in the last decade, this has been applied recently for the reduction of intussusception in children. We herein reviewed our experience of using the combined approach, namely, pneumatic reduction and, if failed, laparoscopic reduction in the management of childhood intussusception. METHODS: We carried out a retrospective analysis of all children with intussusception managed at Prince of Wales Hospital between December 1998 and December 2004. The minimally invasive approach was used as far as possible. The method of reduction, success rate and the incidence of complication were analysed. RESULTS: Over a 6-year period, there were 146 patients with 167 episodes of intussusception. Pneumatic reduction was carried out in 160 occasions and was successful in 134 (83.8%). In 33 patients, operative reduction was required. Of these, laparoscopic reduction was attempted in 15 and was successful in 13 (86.7%). In those with either pneumatic or laparoscopic reduction, no procedure-related complication was encountered and they had a significant shorter hospital stay (median 3.0 day) than those requiring laparotomy (median 8.0 day) (t-test, P < 0.0001). CONCLUSION: The minimally invasive approach, that is, pneumatic and/or laparoscopic reduction, was successful in reducing intussusception in 88% of patients with minimal morbidity and shorter hospital stay.

Infants born to mothers with severe acute respiratory syndrome.

Severe acute respiratory syndrome (SARS) is a newly discovered infectious disease caused by a novel coronavirus. During the community outbreak in Hong Kong, 5 liveborn infants were born to pregnant women with SARS. A systematic search for perinatal transmission of the SARS-associated coronavirus, including serial reverse transcriptase-polymerase chain reaction assays, viral cultures, and paired serologic titers, failed to detect the virus in any of the infants. In addition, none of the infants developed clinical, radiologic, hematologic, or biochemical evidence suggestive of SARS. One preterm infant developed jejunal perforation and another developed necrotizing enterocolitis with ileal perforation shortly after birth. This case series is the first report to describe the clinical course of the first cohort of liveborn infants born to pregnant women with SARS.