Our results encourage the use of NPPV as a first-line treatment in children with malignancies who develops acute respiratory failure, except in those with severe hemodynamic status.

Infants with end-stage liver disease represent a treatment challenge. Living donor liver transplantation (LDLT) is the only option for timely liver transplantation in many areas of the world, adding to the technical difficulties of the procedure. Factors that affect morbidity and mortality can now be determined, which opens a new era for improvement. We have accumulated an 11-year experience with LDLT for children weighing Ͻ10 kg. From October 1995 to October 2006, a total of 222 LDLT in patients Ͻ18 years of age were performed; 129 primary LDLT and 7 retransplants (4 LDLT and 3 deceased donor grafts) were performed in 129 infants weighing Ͻ10 kg. Forty-seven patients received grafts with graft-to-recipient weight ratio (GRWR) of Ͼ4%. Two patients received monosegmental grafts, and 2 patients underwent delayed abdominal wall closure. Portal vein thrombosis occurred in 5.4% of the patients, hepatic artery thrombosis in 3.1%, and both in 1.5%. Among several variables studied, only the bilirubin level at the time of transplantation was associated with increased risk of death (P ϭ 0.009). Grafts with GRWR Ͼ4% had no negative effect on patient survival. There were 7 retransplants, and 4 patients received a second parental LDLT. Patient survival rates at 1, 3, and 10 years after transplantation were 88.8%, 84.7%, and 82% for all children, and 87.5%, 84.9%, and 84.9% for infants weighing Ͻ10 kg. LDLT has results comparable to other modalities of liver transplantation in infants. Monosegment grafts were rarely required in this series, although they may be necessary in patients with lower body weight.

We reviewed the outcome of children undergoing living related liver transplantation (LRLT) for Wilson's disease (WD), and specifically addressed the potential risk associated with the use of donors who were heterozygous for the Wilson genetic defect. LRLTs were carried out in 11 children with WD, nine of whom presented with fulminant hepatic failure and two with end-stage hepatic insufficiency. The age of the patients ranged from 6 to 16 yr. Eight patients had hepatic encephalopathy and were plasmapheresed preoperatively. The donors (all parents: six fathers and five mothers) were all one-haplotype matched with their respective recipients, and were all therefore heterozygote carriers of the WD genetic defect. The serum ceruloplasmin levels were within normal limits in all donors (mean: 20.0 +/- 2.85 mg/dL). All recipients but one had low serum ceruloplasmin levels with a mean value of 11.6 +/- 7.36 mg/dL before transplantation. The serum ceruloplasmin levels had increased to an average of 21.0 +/- 3.76 mg/dL after LRLT at the latest evaluation, which ranged between 7 and 75 months after transplantation. A marked reduction in urinary copper excretion was observed in all recipients after transplantation. Of eight recipients presenting preoperatively with Kayser-Fleischer (K-F) rings, this abnormality resolved completely after LRLT in five patients and partially in three. All recipients are alive and remain well, and none have developed signs of recurrent WD after a mean follow-up period of 31 months (range 7-75 months). In conclusion, LRLT is an excellent choice for effective treatment of WD, and grafts chosen from heterozygote carriers of the condition do not appear to confer any risk of recurrence in the recipients.

This research suggests that early intensive treatment for children with cancer exhibiting severe sepsis and/or septic shock could be an important factor to influence the mortality rate of these patients. Moreover, that noninvasive ventilation could be an option to reduce endotracheal intubation and invasive ventilation.

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