M. K. Patrick

Survival, growth and quality of life in children after orthotopic liver transplantation : a 5 year experience

The aims of this study were to investigate outcome and to evaluate areas of potential ongoing concern after orthotopic liver transplantation (OLT) in children. Actuarial survival in relation to age and degree of undernutrition at the time of OLT was evaluated in 53 children (age 0.58‐14.2 years) undergoing OLT for endstage liver disease. Follow‐up studies of growth and quality of life were undertaken in those with a minimum follow‐up period of 12 months (n= 26). The overall 3 year actuarial survival was 70%. Survival rates did not differ between age groups (actuarial 2 year survival for ages <1,1‐5 and >5 years were 70, 70 and 69% respectively) but did differ according to nutritional status at OLT (actuarial 2 year survival for children with Z scores for weight <−1 was 57%, >−1 was 95%; P=0.004). Significant catch‐up weight gain was observed by 18 months post‐transplant, while height improved less rapidly. Quality of life (assessed by Vineland Adaptive Behaviour Scales incorporating socialization, daily living skills, communication and motor skills) was good (mean composite score 91±19). All school‐aged children except one were attending normal school. Two children had mild to moderate intellectual handicap related to post‐operative intracerebral complications. Satisfactory long‐term survival can be achieved after OLT in children regardless of age but the importance of pre‐operative nutrition is emphasized. Survivors have an excellent chance of a good quality of life and of satisfactory catch‐up weight gain and growth.

Malnutrition in children with chronic liver disease accepted for liver transplantation: Clinical profile and effect on outcome

Abstract  The nutritional profiles of 37 children (aged 0.5–14.0 years) with chronic liver disease at the time of acceptance for orthotopic liver transplantation (OLTP) have been evaluated using clinical, biochemical and body composition methods. Nutritional progress while waiting for a donor has been related to outcome, whether transplanted or not. At the time of acceptance, most children were underweight (mean standard deviation (s.d.) weight = 1.4±0.2) and stunted (mean s.d. height = ‐ 2.2±0.4), had low serum albumin (27/35) and had reduced body fat and depleted body cell mass (measured by total body potassium ‐ mean % expected for age = 58±5%, n= 15). Mean ad libitum nutrient intake was 63±5% of recommended daily intake (RDI). Those who died while waiting (n= 8) had significantly lower mean initial s.d. weight compared with those transplanted. The overall actuarial 1 year survival of those who were transplanted (mean waiting time = 75 days) was 81% but those who were initially well nourished (s.d. weight > ‐ 1.0) had an actuarial 1 year survival of 100%. There were no significant differences in actuarial survival in relationship to age, type of transplant (whole liver or segmental), liver biochemistry or the presence or absence of ascites. Of the total group accepted for OLTP, whether transplanted or not, the overall 1 year survival for those who were relatively well nourished was 88% and for those undernourished (initial s.d. weight > ‐ 1.0) was 38% (P<0.003). Declining nutritional status during the waiting period also adversely affected outcome. We conclude that malnutrition and/or declining nutritional status is a major factor adversely affecting survival in children awaiting OLTP. In transplant units where waiting time is greater than 40 days, earlier referral, prioritization of cases and the use of adult donor livers may reduce this risk and efforts to maintain or improve nutritional status deserve further study.

Colonoscopy preparation in children: safety, efficacy, and tolerance of high- versus low-volume cleansing methods.

BACKGROUND The use of large-volume electrolyte balanced solutions as preparation for colonoscopy often results in poor patient compliance and acceptance. The tolerance, safety, and efficacy of high-versus low-volume colon-cleansing methods as preparation for colonoscopy in children were compared by randomized operator-blinded trial. METHODS Twenty-nine children ages 3.6-14.6 years had either high-volume nasogastric balanced polyethylene glycol electrolyte lavage (20 ml/kg/ h) until the effluent was clear (n = 15), or two oral doses of sodium phosphate solution (22.5-45 ml) separated by oral fluid intake (n = 14). RESULTS Both preparations were equally effective. The low-volume preparation was better tolerated and caused less discomfort that the high-volume preparation, judging by serial nurse observations. The incidence of abdominal symptoms, diarrhea, sleep disturbance, and vomiting was not significantly different between the two groups. Both groups had a small reduction in mean hematocrit and serum calcium levels. The sodium phosphate preparation caused increases in mean serum sodium concentrations from 140 to 145 mmol/L and serum phosphate concentrations from 1.41 to 2.53 mmol/L. Ten hours after the commencement of the preanesthetic fast, these concentrations had returned to normal. CONCLUSIONS There are advantages in terms of tolerance, discomfort, and case of administration with acceptable colonic cleansing with the use of the less-invasive oral sodium phosphate low-volume colon-cleansing preparation in children. Safe use requires ensuring an adequate oral fluid intake during the preparation time and avoidance of use in patients with renal insufficiency.

The sleep patterns of infants and young children with gastro‐oesophageal reflux

Sleep disturbance in gastro‐oesophageal reflux disease (GORD) in infants and young children has not been systematically studied nor has this manifestation been compared with population norms.

Airway cellularity, lipid laden macrophages and microbiology of gastric juice and airways in children with reflux oesophagitis

BackgroundGastroesophageal reflux disease (GORD) can cause respiratory disease in children from recurrent aspiration of gastric contents. GORD can be defined in several ways and one of the most common method is presence of reflux oesophagitis. In children with GORD and respiratory disease, airway neutrophilia has been described. However, there are no prospective studies that have examined airway cellularity in children with GORD but without respiratory disease. The aims of the study were to compare (1) BAL cellularity and lipid laden macrophage index (LLMI) and, (2) microbiology of BAL and gastric juices of children with GORD (G+) to those without (G-).MethodsIn 150 children aged <14-years, gastric aspirates and bronchoscopic airway lavage (BAL) were obtained during elective flexible upper endoscopy. GORD was defined as presence of reflux oesophagitis on distal oesophageal biopsies.ResultsBAL neutrophil% in G- group (n = 63) was marginally but significantly higher than that in the G+ group (n = 77), (median of 7.5 and 5 respectively, p = 0.002). Lipid laden macrophage index (LLMI), BAL percentages of lymphocyte, eosinophil and macrophage were similar between groups. Viral studies were negative in all, bacterial cultures positive in 20.7% of BALs and in 5.3% of gastric aspirates. BAL cultures did not reflect gastric aspirate cultures in all but one child.ConclusionIn children without respiratory disease, GORD defined by presence of reflux oesophagitis, is not associated with BAL cellular profile or LLMI abnormality. Abnormal microbiology of the airways, when present, is not related to reflux oesophagitis and does not reflect that of gastric juices.

Cytomegalovirus-Infection After Liver-Transplantation in Children

Post‐liver transplant cytomegalovirus (CMV) infection (seroconversion or virus isolation) and CMV disease (infection plus clinical signs and symptoms) were studied in relation to pretransplant recipient and donor serology, age, nutritional status and the effect of paediatric versus adult (reduced size) grafts. Of 70 children receiving 79 transplants, 26 (37%) had evidence of CMV infection, and eight (11.5%) had evidence of CMV disease, four of whom died. The primary infection rate (where the recipients were CMV negative) was 71% with mortality of 7% with most receiving a CMV‐positive graft. The active secondary infection rate (reactivation or reinfection, where the recipients were CMV positive) was 60% with mortality of 12.5%. No significant differences in infection or disease rates were found comparing malnourished versus well‐nourished patients, or between those who received whole or reduced‐size grafts. The high prevalence of CMV infections supports the view that clinical signs alone are inadequate to direct investigations for CMV. Both primary and active secondary CMV infection can result in serious morbidity and mortality in children receiving liver transplants. These data do not support the strategy of providing immunoprophylaxis to seronegative recipients only, at least in paediatric liver transplantation.

Therapeutic upper gastrointestinal endoscopy in children: An audit of 443 procedures and literature review

Abstract The safety, effectiveness and capabilities of therapeutic upper fibreoptic endoscopy in children undergoing therapeutic endoscopic procedures (n= 443) was studied. Therapy for gastrointestinal bleeding formed the major group (injection sclerotherapy for varices, n= 197 procedures; thermocoagulation for haemorrhagic gastritis, n= 1; and photocoagulation for Dieulafoys disease, n= 1). Sclerotherapy was 97% effective in controlling acute bleeding and 84% effective in obliterating varices with no serious complications or deaths. Oesophageal dilatations for surgical, caustic, congenital and peptic strictures and achalasia (n= 193) were performed with no oesophageal perforations or deaths. Foreign bodies were retrieved (n= 34) with no failures or complications. Percutaneous endoscopic gastrostomy was performed (n= 11) with one failure, proceeding to an unsuccessful surgical gastrostomy. Miscellaneous procedures included endoscopic transpyloric tube placement (n= 5) and endoscopic diathermy of pyloric web (n= 1). Therapeutic fibreoptic endoscopy is therefore concluded to be safe and effective in children, replacing rigid oesophagoscopy and some traditional surgical approaches.

Esophageal stricture in children: fiber-optic endoscopy and dilatation under fluoroscopic control.

We made a retrospective analysis of the efficacy and complication rate of 268 esophageal dilatation procedures performed under fluoroscopic control using the fiber-optic endoscope in 45 children with esophageal stricture. Antegrade and retrograde stricture dilatation was performed under general anesthetic, mainly as an outpatient procedure. Thirty-six children had an esophageal stricture following tracheoesophageal fistula and/or esophageal atresia repair, and nine children had severe corrosive stricture of the esophagus following lye ingestion. The procedure was well tolerated and effective.

Chronic copper toxicosis presenting as liver failure in an australian child

&NA; A 20 month old Caucasian male child, after a five week illness, developed liver failure which was successfully treated by liver transplantation. The explanted liver had a histology identical to that seen in Indian childhood cirrhosis and its copper content was increased tenfold. Water used to prepare the childs milk feeds came from a bore via copper conduits and at times contained 120 µmol/l of copper, eight times the recommended maximum for human consumption. Because non‐Indian cases of Indian childhood cirrhosis associated with excess copper ingestion are increasingly being recognised, and as early treatment can restore normal liver morphology, we support the use of the previously suggested alternative term for this condition, ie; ‘copper‐associated liver disease in childhood’. Measurement of hepatic copper concentrations in all children less than six years of age who develop hepatic failure of unknown cause will increase its recognition. On diagnosis sources of increased dietary copper should be investigated to ensure that younger siblings are not similarly exposed.

Survival, Growth and Quality of Life After Liver-Transplant in Children

To investigate outcome and evaluate areas of potential ongoing concern after orthotopic liver transplantation (OLT) in children, overall actuarial survival rates in relation to age and degree of undernutrition at the time of OLT have been evaluated and followup studies of growth parameters and quality of life undertaken in a group of 48 children (age 0.5-14.2 yr) undergoing OLT for end-stage liver disease. Overall 3 yr actuarial survival was 68%. Survival rates did not differ between age groups (actuarial 1 year survival for ages 5-15 yr, 1-5 yr and <1 yr were 68%, 72% and 62% respectively) but did differ according to nutritional status at OLT (actuarial 1 year survival for children with Z scores for weight <-1 was 58%, >-1 was 94% p=0.01)- Significant catchup weight gain and growth were observed by 6, 12 and 18 months post transplant (n=17). Of 28 survivors studied 12 months after OLT, quality of life was assessed by adaptive behaviour and social skills indices. In all but 3 cases, quality of life was judged to be excellent and all school aged children bar one were attending normal school. Two cases had intracerebral perioperative complications with mild to moderate intellectual handicap.Satisfactory long term survival can be achieved after OLT in children regardless of age but the importance of preoperative nutrition is emphasized. Survivors have an excellent chance of good quality of life and cmchup growth by 1 year post-transplant.