Clinical experience in managing pediatric patients with ultra-short bowel syndrome using omega-3 fatty acid.

Total parenteral nutrition (TPN) remains an important component of the management of short bowl syndrome in pediatric patients. However, prolonged TPN is known to be associated with cholestasis. Recently, the use of omega-3-fatty acid (Omegaven) has been proposed to improve TPN cholestasis. We present the early outcome after administration of Omegaven in four patients with ultra-short bowel syndrome. Based on our experience, it appears that omega-3 fatty acid can reverse and prevent the advent of TPN-related cholestasis, thereby significantly improving the process of intestinal adaptation. We suggest that clinicians consider this treatment option before proceeding to invasive surgery to reverse cholestasis. Prospective randomized trials are necessary to define a standard protocol and elucidate other potential benefits of this novel agent.

Management and outcome of antenatally diagnosed congenital cystic adenomatoid malformation of the lung.

OBJECTIVE: To review the management and outcome of babies with antenatally diagnosed congenital cystic adenomatoid malformation. DESIGN: Retrospective cohort review. SETTING: Tertiary neonatal care unit at Queen Mary Hospital and antenatal diagnostic centre at Tsan Yuk Hospital. PATIENTS: Consecutive patients with antenatally suspected congenital cystic adenomatoid malformation in their concepti among antenatal patients attending Tsan Yuk Hospital from 1994 to 2002. Twenty-four of 33 cases were referred to Queen Mary Hospital for postnatal management and for whom comprehensive records were available for analysis in 23. INTERVENTIONS: Postnatal interventions in their babies included investigational imaging for congenital cystic adenomatoid malformation and surgery. MAIN OUTCOME MEASURES: Antenatal and postnatal outcome, as well as pathology of the excised lesions. RESULTS: Antenatal outcome: termination of pregnancy in two cases and spontaneous abortion in one; in-utero regression was documented in nine cases and in one hydropic change was apparent. Postnatal outcome: only eight of 20 babies born alive had symptoms in neonatal period. Two developed serious infective complications in infancy, one with documented in-utero regression. Pulmonary parenchymal abnormalities were detected on computed tomography of the thorax in six of seven cases with normal or non-specific chest radiograph findings. Among nine cases with in-utero regression, congenital cystic adenomatoid malformation was confirmed by operative histology in five and abnormal computed tomography findings in three. Fifteen babies underwent surgical excision, one of whom died because of severe pre-existing pulmonary hypoplasia and nine endured minor postoperative complications. A favourable outcome was documented at a mean follow-up of 22 months (range, 2 months-7 years). CONCLUSIONS: In-utero regression of congenital cystic adenomatoid malformation on antenatal ultrasound may not represent genuine resolution. Computed tomographic thorax should be considered in all newborns with antenatally diagnosed congenital cystic adenomatoid malformation, and if confirmed early operation before first hospital discharge is recommended.

Relation of arterial stiffness with gestational age and birth weight.

BACKGROUND: The cardiovascular risk of individuals who are born small as a result of prematurity remains controversial. Given the previous findings of stiffer peripheral conduit arteries in growth restricted donor twins in twin-twin transfusion syndrome regardless of gestational age, we hypothesised that among children born preterm, only those with intrauterine growth retardation are predisposed to an increase in cardiovascular risks. AIM: To compare brachioradial arterial stiffness and systemic blood pressure (BP) among children born preterm and small for gestational age (group 1, n = 15), those born preterm but having birth weight appropriate for gestational age (group 2, n = 36), and those born at term with birth weight appropriate for gestational age (group 3, n = 35). METHODS: Systemic BP was measured by an automated device (Dinamap), while stiffness of the brachioradial arterial segment was assessed by measuring pulse wave velocity (PWV). The birth weight was adjusted for gestational age and expressed as a z score for analysis. RESULTS: The 86 children were studied at a mean (SD) age of 8.2 (1.7) years. Subjects from group 1, who were born at 32.3 (2.0) weeks' gestation had a significantly lower z score of birth weight (-2.29 (0.63), p<0.001), compared with those from groups 2 and 3. They had a significantly higher mean blood pressure (p<0.001) and their diastolic blood pressure also tended to be higher (p = 0.07). Likewise, their brachioradial PWV, and hence arterial stiffness, was the highest of the three groups (p<0.001). While subjects from group 2 were similarly born preterm, their PWV was not significantly different from that of group 3 subjects (p = 1.00) and likewise their z score of birth weight did not differ (-0.01 (0.71) v -0.04 (1.1), p = 1.00). Brachioradial PWV correlated significantly with systolic (r = 0.31, p = 0.004), diastolic (r = 0.38, p<0.001), and mean (0.47, p<0.001) BP, and with z score of birth weight (r = -0.43, p<0.001). Multiple linear regression identified mean BP and z score of birth weight as significant determinants of PWV. CONCLUSION: The findings of the present study support the hypothesis that among children born preterm, only those with intrauterine growth retardation are disadvantaged as a result of increase in systemic arterial stiffness and mean blood pressure.

Surgical complications and outcome of pediatric liver transplantation in Hong Kong.

PURPOSE: The aim of this study was to analyze the early and late results of pediatric liver transplantation, with particular reference to complications that required surgical or radiologic intervention. METHODS: The records and code sheets of children who underwent liver transplantation in the authors' institution between September 1993 and December 2001 were reviewed. RESULTS: Twenty-nine children (16 boys and 13 girls) underwent 31 liver transplantations (23 living donor, 8 cadaveric donor) during the study period. The ages of the children ranged from 4 months to 132 months (median, 16 months). Eighteen children had complications that required surgical or radiologic interventional procedures. Complications included, among others, hepatic vein thrombosis (n = 1, 3%), hepatic vein stenosis (n = 2, 7%), portal vein thrombosis (n = 2, 7%), biliary stricture (n = 3, 10%), bile leakage (n = 2, 7%), hepatic artery pseudoaneurysm (n = 1, 3%), jejuno-jejunostomy leakage (n = 1, 3%), graft hepatitis (n = 1, 3%), and posttransplant lymphoproliferative disorder (n = 2, 7%). In addition, 6 children (21%) suffered from intraabdominal bleeding from a variety of causes. After appropriate interventions, at a median follow-up of 38 months (range, 1 to 96 months), patient and graft survival rates were 79% and 74%, respectively. The retransplantation rate was only 7%. There was no incidence of hepatic artery thrombosis. All living donors remain alive and well. CONCLUSIONS: Complications are inevitable in pediatric liver transplantation. However, with timely recognition and active intervention, a good outcome can be achieved.

Airway obstruction in children with congenital heart disease: assessment by flexible bronchoscopy.

We assessed the spectrum of airway disorders in children with congenital cardiac anomalies, and reviewed our experience in using flexible bronchoscopy for assessment of airway problems in this patient group. The clinical records, flexible bronchoscopic findings, and cardiac imaging results of pediatric cardiac patients who presented with either clinical or radiological signs of airway obstruction between 1992-1999 were reviewed. Flexible bronchoscopic assessment was performed with the patients under sedation and topical anesthesia, using one of two bronchoscopes, i.e., an Olympus BFN20 or Olympus BF3C20. Of a total of 52 patients, 33 had acyanotic cardiovascular lesions, the commonest being left-to-right shunts (61%), while 19 had cyanotic heart lesions, with right ventricular outflow obstruction being the commonest (63%). Twenty-seven patients had undergone either surgical or transcatheter interventions. The median age at bronchoscopic assessment was 6 months (range, 4 days to 6 years). None of the patients developed significant procedural complications. A definitive diagnosis was made in 48 (92%) patients, 8 of whom had abnormalities involving only the upper airways, 35 only the lower airways, and 5 both. Abnormalities of the upper airway included laryngomalacia (n = 6), subglottic stenosis (n = 3), pharyngeal collapse (n = 2), and 1 each of choanal stenosis and supraglottitis. Extrinsic compression was the commonest lower airway abnormality that was found in 27/40 patients (67%), with a predilection for the left main bronchus (18/27, 67%). The structures that caused extrinsic compression included dilated pulmonary arteries with or without left atrial dilation (n = 20), an anomalous aortic or pulmonary arterial course (n = 3), a dilated aorta (n = 1), and a shunt (n = 1), but were not obvious in 2 patients. Intrinsic lower airway abnormalities included bronchomalacia (n = 4), tracheal stenosis (n = 4), and one each of variant bronchial bifurcation and a pouch arising from the tracheal wall. Intraluminal mucus plugging of the lower airways occurred in the remaining 3 patients. Children with congenital heart disease are at risk of airway obstruction both before and after surgery. Flexible bronchoscopy, being safe and effective in diagnosing airway disorders in this patient group, should be considered as the first line of investigation.

Ten-year experience with liver transplantation at Queen Mary Hospital: retrospective study.

OBJECTIVE: To report the experience with liver transplantation at the Queen Mary Hospital from 1991 to 2000. DESIGN: Retrospective study. SETTING: Liver transplant centre of a University teaching hospital, Hong Kong. PATIENTS: One hundred and forty-eight patients (127 adults and 21 children) who underwent a total of 155 liver transplants using 75 cadaver grafts (full-size, 67; reduced-size, 5; split, 3) and 80 living donor grafts (left lateral segment, 15; left lobe, 6; right lobe, 59) from October 1991 to December 2000 were reviewed. MAIN OUTCOME MEASURES: Graft and patient survival rate. RESULTS: The most common disease indications for liver transplantation were chronic hepatitis B-related liver disease (n=74) in adults and biliary atresia (n=14) in children. Eighteen patients had hepatocellular carcinoma. Forty-eight (31%) liver transplants (three ABO-incompatible) were performed in high-urgency situations for patients requiring intensive care. The proportion of living donor liver transplants was 47.7% in adults and 73.9% in children. The overall 1-year and 5-year patient survival rates were 82% and 77%, respectively. The survival of high-risk recipients, such as those with fulminant hepatic failure (80%), chronic hepatitis B (81%), or hepatocellular carcinoma (94%), was not inferior to that of other patients. CONCLUSION: Over the last decade, the promotion of (cadaver) organ donation through public education coupled with innovative techniques in living donor liver transplantation have enabled a liver transplantation programme to be established in Hong Kong with gratifying results.

Surfactant tracheobronchial lavage for the management of a rabbit model of meconium aspiration syndrome.

We evaluated the effect of tracheobronchial lavage with diluted surfactant solution (bovine lipid extract surfactant, bLES) in a rabbit model of meconium aspiration. All animals were anaesthetized, tracheotomized and given 3-4 ml/kg of 25% slurry of human meconium into the endotracheal tube and mechanically ventilated for 1 h. The animals were then randomly assigned to surfactant lavage (n = 12) with 15 ml/kg of diluted surfactant at a concentration of 5.4 mg phospholipid/ml administered in aliquots of 2 ml; or simple endotracheal suction (control n = 12) when the oxygenation index (OI) was >/=15. Changes in the arterial blood gases and the histomorphological and radiological appearances of the lungs were recorded. The OI and arterial/alveolar oxygen tension (a/A PO2) of the surfactant lavage group improved significantly at 5 min post-treatment, and these improvements were observed throughout the ensuing 4 h of ventilation. There was significantly more solid content recovered by surfactant lavage compared with the control group (p = 0.0001). Radiologically, the post-treatment air space opacification scores of the lavage group were significantly lower compared with the control (p = 0.002). The post-treatment radiographs of the lavage-treated rabbits were rated by the radiologist, who was blinded to the treatment groups, as much improved in 5 and improved in 4, whereas the control rabbits were rated as much worse in 3 and worse in 4. Histological examination showed the lungs of the lavaged rabbits had significantly more normal airway (p < 0.0001), more fields showing completely normal airspace (p = 0.0001) and less fields showing severe overdistension with meconium (p = 0.0005). We concluded that lavage with diluted surfactant solution effectively washed out the meconium, improved gases exchanges, and improved the histological and radiological appearances in the rabbit model of MAS.

Liver transplantation in infants.

PURPOSE: In view of the earlier reports that children below 1 year of age constitute a high-risk group for liver transplantation, the authors reviewed their experience in performing orthotopic liver transplantation in this age group. METHODS: The records of 9 children aged less than 1 year who underwent 6 living-related liver transplants and 3 reduced-size liver transplants between December 1993 and June 1997 were reviewed. RESULTS: Five reexplorations were required for 3 children who had 1 or more of the following early complications: bleeding from hepatic vein to inferior vena cava anastomosis (n = 1), right hepatic vein stump bleeding (n = 1), intraabdominal hematoma (n = 2), jejuno-jejunostomy leakage (n = 1), and colonic perforation (n = 1). Late complications include stricture at the biliary-enteric anastomosis requiring percutaneous balloon dilatation (n = 3) and hepatitis of undetermined etiology requiring retransplantation (n = 1). There was no hepatic artery thrombosis despite the small arteries available for anastomosis. Follow-up ranged from 19 to 61 months (mean, 40 months). Patient survival rate was 100%, and graft survival with good liver function was 89%. All living donors, 2 fathers and 4 mothers, are well. CONCLUSIONS: Liver transplantation in infants less than 1 year of age is technically demanding but feasible and still can be performed with a good outcome. Age alone (under 1 year) should not be considered as a contraindication for liver transplantation.

Ammonia clearance by peritoneal dialysis and continuous arteriovenous hemodiafiltration.

We report the use of continuous arteriovenous hemodiafiltration (CAVHD) in a neonate with severe hyperammonemia due to a urea cycle disorder. We compared the ammonia clearance (C(NH3)) for peritoneal dialysis (PD) and CAVHD. C(NH3) for CAVHD was 7.45 ml/min per m2 at a dialysate flow of 300 ml/h and was 10.55 ml/min per m2 at a dialysate flow rate of 600 ml/h. The mean PD clearance was 2.15 ml/min per m2. Our data suggest that CAVHD is superior to PD for the removal of plasma ammonia. We conclude that CAVHD should be considered a reasonable alternative in the treatment of neonatal hyperammonemia in urea cycle disorders when medical treatment fails.

Paediatric liver transplantation: Queen Mary Hospital experience.

OBJECTIVE: To assess the results of paediatric liver transplantation in our institution. METHODS: From September 1993 to November 1996, 10 living-related liver transplants (LRLT) and 3 reduced-size liver transplants (RSLT) were performed on 12 children at our hospital. The medical records of the patients were reviewed. All patients suffered from end-stage liver disease resulting from biliary atresia with failed Kasai's operations. Their ages at initial transplantation ranged from 8 months to 11 years. Excluding the 2 older children aged 7.5 and 11 years, the remaining patients were aged 10.5 months on the average and weighed 6 to 9.5 kg (mean: 6.8 kg) at the time of initial transplantation. RESULTS: All living donors were discharged on postoperative day 4 to 8 and resumed their previous normal activities. All recipients were alive with normal liver function and growing after a follow-up period of 3-40 months (mean: 21 months). The patient survival rate was 100%. One patient with RSLT had hepatitis of undetermined aetiology and underwent retransplant with a graft from her mother. The graft survival rate was 92%. Postoperative complications included: postoperative bleeding (n = 3), hepatic vein stenosis (n = I), biliary-enteric anastomotic stenosis (n = 3), intestinal perforation (n = I) and portal vein thrombosis (n = I). They were all treated promptly. In all patients, the hepatic artery (diameter ranged from 1.5 to 2.5 mm) anastomosis was achieved by microvascular technique. There was no hepatic artery thrombosis in our patients. CONCLUSION: With technical refinements, early detection and prompt treatment of complications, and advances in immunotherapy, excellent results can be achieved in paediatric liver transplantation.

Liver transplantation in children: the experience of Queen Mary Hospital, Hong Kong.

Seven living-related liver transplants (LRLT) and two reduced-size liver transplants (RSLT) were performed on eight children who suffered from end-stage liver disease, having previously undergone one to three abdominal operations. Their ages at initial transplantation ranged from 8 months to 11 years (mean 35 months, median 12 months). Excluding the two older children aged 7 and 11 years, respectively, the rest of the children weighed 6 to 9.5 kg (mean 7.3 kg) at the time of the initial transplantation. Seven left lateral segments (S2 + 3) and two left lobes (S2 + 3 + 4) were used; of these the smallest graft had a graft-to-recipient body weight ratio of 0.9%. The volunteer living donors were four mothers, two fathers and one sister who were selected after medical and psychiatric evaluations, and their suitability was confirmed by hematological, biochemical, and radiological criteria. During a follow-up period of 3 to 30 months, all eight children are alive and well with normal liver function, one of them having undergone a retransplant LRLT because of hepatitis of undetermined etiology following a RSLT 1.5 years earlier. All seven donors had an uneventful postoperative course and were discharged on day 4 to 7 postoperatively. They have all resumed normal day-to-day activities. There were no complications in the donor group. A variety of complications occurred in the recipients, all of which were overcome. Operating microscope was used to perform all the arterial anastomoses using microvascular techniques. This method has proven to be a major factor in preventing arterial thrombosis even with the smallest of arterial anastomosis where a 1.5-mm diameter recipient artery was anastomosed to a 2.5-mm diameter donor hepatic artery.

Liver transplantation--perspective from Hong Kong.

BACKGROUND/AIMS: We report our experience of 27 orthotopic liver transplantations in 26 patients performed at Queen Mary Hospital, Hong Kong during the period of October 1991 to October 1995. PATIENTS AND METHODS: There were 19 adults and 7 pediatric patients with a mean age of 29 years (range 8 months to 62 years). The underlying liver diseases of the 26 patients were biliary atresia (n = 6), Alagille syndrome (n = 1), primary biliary cirrhosis (n = 2) cryptogenic cirrhosis (n = 2), alcoholic cirrhosis (n = 5), Wilson's disease (n = 1), fulminant hepatic failure (n = 3), polycystic liver (n = 2), secondary biliary cirrhosis (n = 1), HBV cirrhosis (n = 2) and autoimmune hepatitis with hepatocellular carcinoma (n = 1). The pathology leading to re-transplantation in a pediatric patient was post-transplant hepatitis of unknown etiology. The liver grafts were obtained from 19 brainstem dead and 8 living donors. The pediatric patient requiring re-transplantation received a left lateral segment graft from her mother. Two adults received left lobe grafts from their family members. RESULTS: The overall graft survival is 88% and patient survival is 92%. There were only 2 deaths: one patient developed primary graft nonfunction and died from intracerebral bleeding 39 days after transplantation and the other died from graft rejection resistant to salvage by steroid pulse and OKT3. The other patients are well with functioning grafts. CONCLUSIONS: We hope that the current success rate can convince people in our locality in cadaveric organ donation so that living donors do not run the risk of dying from the operation, although the risk is estimated to be very small.

A study of pre-antibiotic bacteriology in 125 patients with necrotizing enterocolitis.

Over a five-year period, 125 newborns with necrotizing enterocolitis (NEC) were managed by us. Their mean birthweight was 1700 g and mean maturity was 32 weeks. Before commencement of antibiotics, routine septic work-up was done in order to define the bacterial spectrum and antibiotic sensitivity. The study includes aerobic and anaerobic cultures of gastric and pharyngeal aspirates, blood cultures, umbilical swabs and culture of umbilical catheter tips in relevant cases. Peritoneal swab results were also analyzed if laparatomy was performed. Positive cultures were present in 45 patients (36%) with 55 positive specimens. Fifteen types of organism were isolated: the commonest was Enterobacter (29%), followed by E. coli (14.5%) and Klebsiella (13%). They were resistant to ampicillin and first-generation cephalosporin. These organisms were usually opportunistic pathogens. Overgrowth of them may be the cause of NEC. Regular review of the antibiotic sensitivity of these organisms allows prompt and appropriate choice of antibiotics. At the same time, antibiotic sensitivity for these organisms was analyzed to guide us in the choice of antibiotic therapy.

Response of preterm infants to hepatitis B vaccine.

Ninety-nine preterm infants with birth weights < 1750 gm had three doses of hepatitis B vaccine. Fifty-seven received the first dose when they weighed > or = 1000 gm (group 1) and 42 when they weighed > or = 2000 gm (group 2). The final seropositive rates and geometric mean titers of group 1 infants (79%, 61 mIU/ml) and group 2 infants (91%, 262 mIU/ml) were less than that of 43 normal term infants (100%, 679 mIU/ml).