Isolated liver transplantation in children with cystic fibrosis--an Australian experience.

CF liver disease is an uncommon indication for pediatric LT. Determining optimal timing and type (isolated liver versus multi-organ) of transplantation for those with severe liver disease can be challenging and involves consideration of the extent of liver disease (PHT, synthetic dysfunction) and extrahepatic factors such as pulmonary function. We present the experience of isolated LT for CF at our center. Eight children received one allograft each (3.9% of all grafts). One- and four-yr survivals are both 75%. The two deaths occurred within the first two months after LT, and in both cases, invasive fungal infections were implicated, one following treatment for acute severe rejection. All had significant PHT, and six had synthetic dysfunction. All had roux-en Y biliary anastomoses and none developed long-term biliary complications. Seven had pulmonary colonization with Pseudomonas aeruginosa and six with fungus at time of transplantation. Mean pre-LT FEV1 was 80% (range 59-116%) predicted, and lung function post-LT was stable. Isolated LT in children with CF is successful in those with relatively preserved pulmonary function, which does not appear to deteriorate as a consequence. Roux-en Y biliary anastomosis and antifungal prophylaxis should be a part of management of these patients.

Chimerism and tolerance in a recipient of a deceased-donor liver transplant.

Complete hematopoietic chimerism and tolerance of a liver allograft from a deceased male donor developed in a 9-year-old girl, with no evidence of graft-versus-host disease 17 months after transplantation. The tolerance was preceded by a period of severe hemolysis, reflecting partial chimerism that was refractory to standard therapies. The hemolysis resolved after the gradual withdrawal of all immunosuppressive therapy.

Prospective peer-review audit of paediatric upper gastrointestinal endoscopy.

AIM: To describe the findings of paediatric upper gastrointestinal endoscopy (UGE) and to reduce the rate of normal findings in children undergoing diagnostic UGE. METHODS: Upper gastrointestinal endoscopy were performed at a single tertiary referral children's hospital over a 3-year period by four endoscopists. Patients were subgrouped into diagnostic categories (recurrent abdominal pain syndrome (RAP), oesophagitis, coeliac disease and enteropathy/inflammatory bowel disease) and endoscopists recorded their clinical diagnosis as above before each procedure. Endoscopic and biopsy findings were compared with clinical diagnosis. After the first year of audit each endoscopist was appraised of their practice and strategies implemented to reduce the normal UGE. In years 2 and 3 all endoscopists were audited by their peers on a monthly basis. RESULTS: A total of 1172 UGE were performed over a 3-year period. Ninety per cent were diagnostic procedures, of which 48% were normal, 16% identified oesophagitis, 11% coeliac disease, 6% gastritis, 3%Helicobacter pylori and 1% peptic ulcer disease. Peer-review audit significantly reduced the number of normal findings in coeliac disease, RAP and overall (P < 0.01) but not in the groups with presumed oesophagitis or investigation of enteropathy/inflammatory bowel disease. CONCLUSIONS: A high proportion of patients undergoing UGE have normal procedures. Peer-review audit can reduce the number of normal procedures particularly in RAP and in the diagnosis of coeliac disease.

Liver transplantation utilizing pediatric cadaver donor livers.

This is a review of a unit's experience with the use of pediatric (up to 15 yr of age), cadaver donor livers for orthotopic liver transplantation (OLTx). The records of 86 patients who underwent 90 OLTx with pediatric donor allografts from January 1986 to September 2002 were reviewed. A total of 69 of 90 (77%) allografts from pediatric donors were used for pediatric (P) recipients, and 21 of 90 (23%) for adult (A) recipients. Donor demographics were: mean donor age 8.5 (0-15) yr; donor sex 53 males/37 females; cause of death head injury 55/90 (61%), cerebral ischaemia 14 of 90 (15%), cerebrovascular 13 of 90 (15%), other eight of 90 (9%). Whole OLTx were performed in 44 of 69 (64%) P group and 20 of 21 (95%) A group. Reduced-size OLTx (13 left lateral lobe, 10 left lobe, one right lobe, one split left lateral lobe) were performed in 25 of 69 (36%) P group vs. in one of 21 (5%) A group, (right lobe split) (p < 0.005). The median follow up was 8.8 yr. The graft loss in the P recipients was 19 of 69 (27.5%) vs. eight of 21 (38.1%) in A recipients. Graft loss for reduced size P OLTx was nine of 25 (36%) vs. 10/44 (22.7%) for whole allograft P OLTx (p = NS). Underlying cause of P graft loss were: chronic rejection six (32%); patient mortality six (32%); vascular four (21%); other three (15%). Underlying cause of A graft loss were: patient mortality four (50%), acute rejection two (25%) and vascular two (25%). OLTx-utilizing pediatric cadaver donor allografts provides acceptable outcomes, including use of reduced-size grafts in pediatric recipients. Vascular complications and chronic rejection tend to be more common in pediatric recipients, whilst patient mortality and graft loss from acute rejection are more common in adult recipients.

99mTc-stannous colloid white cell scintigraphy in childhood inflammatory bowel disease.

UNLABELLED: 99mTc-Labeled white cell scintigraphy (WCS) has been used for the investigation of inflammatory bowel disease (IBD) in adults, but data on children are limited. The most common agent used is (99m) Tc-hexamethylpropyleneamine oxime (HMPAO); however, this agent has limitations. In a retrospective study, we assessed the use of (99m)Tc-stannous colloid WCS for the initial evaluation of children with suspected IBD. METHODS: Diagnostic, endoscopic, and contrast radiography results were retrospectively collected from the medical records. Two experienced nuclear physicians unaware of the patient data interpreted the WCS results, with agreement reached by consensus. Statistical analysis was performed on the ability of WCS to detect active disease and localize it topographically and on a comparison of diagnostic methods, using a combination of clinical features and endoscopy as the reference standard. RESULTS: Between 1996 and 1999, 64 patients (35 male and 29 female; mean age, 12.5 y; age range, 2-19 y) had WCS performed, with IBD subsequently diagnosed in 34 patients. (99m)Tc-Stannous colloid WCS had an 88% sensitivity, 90% specificity, and 8.8 likelihood ratio for initial investigation of IBD. Agreement was poor for topographic localization of disease. Small-bowel series had a 75% sensitivity, 50% specificity, and 1.5 likelihood ratio for detecting endoscopic disease of the terminal ileum and proximal colon. CONCLUSION: Our results confirm that WCS is a useful imaging technique for the initial evaluation of patients with suspected IBD. (99m)Tc-Stannous colloid had results at least comparable to those of other WCS agents, and in children, (99m)Tc-stannous colloid WCS should be preferred in view of lower cost, shorter preparation time, and the smaller blood volumes required.