Establishment of a pediatric liver transplantation program: experience with 100 transplantation procedures.

OBJECTIVE: To analyze the primary factors that influence the development and consolidation of a pediatric liver transplantation program. PATIENTS AND METHODS: This was a retrospective study of 100 liver transplantation procedures performed in 84 pediatric patients between May 1990 and November 2007. The male-female ratio was 40:60. Mean (SD) age was 5 years (40 patients were younger than 2 years); cold ischemia time was 7.10 (3.1) hours; surgery time was 5.2 (2.2) hours; and time on the waiting list for transplantation was 75 (range, 1-1012) days. Indications for transplantation included cholestatic disease (43%), acute hepatic failure (AHF; 34%), metabolic disorders (14%), and cirrhosis (9%). Transplanted organs included 3 split grafts, 29 partial grafts, and 8 living-donor grafts. RESULTS: Mean graft survival was 70.4%, 59.2%, and 58.1% at 1, 3, and 5 years, respectively. Factors that influenced graft outcome were age younger than 2 years; surgery time more than 6 hours; and AHF vs cholestatic disease, metabolic disorders, and cirrhosis. There were no significant differences in long-term (51% vs 59%) and short-term (71% vs 70%) graft survival between procedures performed in 1990-1998 compared with those performed in 1999-2007; however, there was a higher percentage (P = .005) of recipients at high risk (age younger than 2 years or with AHF) in the later period. All data were consistent with those of the European Liver Transplant Registry 2007. CONCLUSIONS: A pediatric liver transplantation program can be established by a group experienced in liver transplantation.

[Invasive mechanical ventilation in cystic fibrosis: Influence in lung transplant]. / Ventilación mecánica invasiva en fibrosis quística: influencia en el trasplante pulmonar.

INTRODUCTION: Invasive mechanical ventilation (IMV) in patients with advanced cystic fibrosis (CF) is a relative contraindication for lung transplant (LT) in adults, although there is currently no data on children. PATIENTS AND METHODS: An 8-year retrospective study on 21 children with CF who underwent LT was performed, analysing their results as they were receiving (n = 8) or not (n = 13) IMV pretransplant. Demographic and surgical data, postoperative course, lung function and survival (immediate and 1-year) were compared between both groups. The role of the IMV pretransplant as a postoperative risk factor was estimated (odds ratio) and Kaplan Meier survival study was performed in both groups. RESULTS: No differences in patient age, sex and nutritional parameters were observed between both groups. Those on IMV who received LT required more frequent and longer bypass, more need for tracheotomy, a higher number of rejection episodes per patient and multiorgan failure, longer PICU stay and longer time on IMV than those who were not on IMV when LT was received. Nevertheless, no differences could be found regarding graft function and immediate and 1-year survivals (62.5% vs. 92.3% with and without IMV respectively). On the other hand, long-term survival was significantly lower than in patients on IMV. CONCLUSIONS: In our experience, children with CF on IMV who receive LT have more complicated surgery and immediate postoperative course. Though immediate and 1-year results and survivals may be encouraging, medium and long-term ones are significantly lower.

[Mechanical ventilation in pediatrics (III). Weaning, complications and other types of ventilation. High-frequency ventilation]. / Ventilación mecánica en pediatría (III). Retirada de la ventilación, complicaciones y otros tipos de ventilación. Ventilación de alta frecuencia.

In the era of lung-protective ventilation strategies, high frequency oscillatory ventilation (HFOV) has attracted renewed interest and its use has dramatically increased in neonatal and pediatric intensive care units. HFOV is able to reduce ventilator-induced lung injury by limiting the incidence of volutrauma, atelectrauma, barotrauma and biotrauma. During HFOV, adequate oxygenation and ventilation is achieved by using low tidal volumes and small pressure swings at supraphysiologic frequencies. Unlike other high-frequency ventilation modes, HFOV has an active expiration phase. HFOV constitutes a safe and successful ventilation mode for managing pediatric patients with respiratory insufficiency refractory to optimized conventional mechanical ventilation and provides better results when initiated early. However, the elective use of HFOV requires further studies to identify its benefits over conventional modes of mechanical ventilation and to support its routine use as a first line therapy. In the present article, the Respiratory Working Group of the Spanish Society Pediatric Critical Care reviews the main issues in the pediatric application of HFOV. In addition, a general practical protocol and specific management strategies, as well as the monitoring, patient care and other special features of the use of HFOV in the pediatric setting, are discussed.

[External ventricular assist devices as circulatory support in patients with dilated cardiomyopathy]. / Asistencia ventricular externa como soporte circulatorio en un paciente con miocardiopatía dilatada.

Ventricular assist devices have demonstrated their utility in patients with intractable cardiac failure, both as support until complete myocardial recovery and as a bridge to transplantation. Specific pediatric pneumatic paracorporeal systems can be applied even in infants. Long-term survival has been reported although experience is limited. We report the case of an 8-year-old boy with dilated cardiomyopathy awaiting cardiac transplantation. The patient developed profound cardiogenic shock with multiorgan failure while being evaluated for heart transplantation. He was given biventricular assistance with the MEDOS-HIA system (MEDOS-Helmholtz Institute). Maximum stroke volume ventricles of 25 and 22 ml were used, achieving a cardiac output of 2.2 l/min in both ventricles. The patient was supported with ventricular assistance for 9 days, but multiple organ failed to improve and transplantation became impossible. Progressive loss of peripheral circulatory resistance unresponsive to treatment developed and ventricular assistance was discontinued. The previous severe shock and advanced and progressive multiorgan failure could be responsible for the poor outcome of our patient despite maintenance of adequate cardiac output. Nevertheless, the use of ventricular assist devices is a real therapeutic alternative in children with severe cardiogenic shock, allowing them to recover completely or undergo heart transplantation. Patient selection, the choice of a system of appropriate size, and early implantation seem to be the cornerstones for obtaining good results.