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Introduction and aim The MELD score has been established as an efficient and rigorous prioritization system for liver transplant (LT). Our study aimed to evaluate the effectiveness of the MELD score as a system for prioritization for LT, in terms of decreasing the dropout rate in the waiting list and maintaining an adequate survival post-LT in Chile. Materials and methods We analyzed the Chilean Public Health Institute liver transplant registry of candidates listed from October 15th 2011 to December 31st 2014. We included adult candidates (>15 years old) listed for elective cadaveric LT with a MELD score of 15 or higher. Statistical analysis included survival curves (Kaplan–Meier), log-rank statistics and multivariate logistic regression. Results 420 candidates were analyzed. Mean age was 53.6 ± 11.8 years, and 244 were men (58%). Causes of LT included: Liver cirrhosis without exceptions (HC) 177 (66.4%); hepatocellular carcinoma (HCC) 111 (26.4%); cirrhosis with non-HCC exceptions 102 (24.3%) and non-cirrhotic candidates 30 (7.2%). LT rate was 43.2%. The dropout rate was 37.6% at 1-year. Even though the LT rate was higher, the annual dropout rate was significantly higher in cirrhotic candidates (without exceptions) compared with cirrhotics with HCC, and non-HCC exceptions plus non-cirrhotic candidates (47.9%; 37.2% and 24.2%, respectively, with p = 0.004). Post-LT survival was 84% per year, with no significant differences between the three groups (p = 0.95). Conclusion Prioritization for LT using the MELD score system has not decreased the dropout rate in Chile (persistent low donor's rate). Exceptions generate inequities in dropout rate, disadvantaging patients without exceptions.

The MELD score has been established as an efficient and rigorous prioritization system for liver transplant (LT). Our study aimed to evaluate the effectiveness of the MELD score as a system for prioritization for LT, in terms of decreasing the dropout rate in the waiting list and maintaining an adequate survival post-LT in Chile.We analyzed the Chilean Public Health Institute liver transplant registry of candidates listed from October 15th 2011 to December 31st 2014. We included adult candidates (15 years old) listed for elective cadaveric LT with a MELD score of 15 or higher. Statistical analysis included survival curves (Kaplan-Meier), log-rank statistics and multivariate logistic regression.420 candidates were analyzed. Mean age was 53.6±11.8 years, and 244 were men (58%). Causes of LT included: Liver cirrhosis without exceptions (HC) 177 (66.4%); hepatocellular carcinoma (HCC) 111 (26.4%); cirrhosis with non-HCC exceptions 102 (24.3%) and non-cirrhotic candidates 30 (7.2%). LT rate was 43.2%. The dropout rate was 37.6% at 1-year. Even though the LT rate was higher, the annual dropout rate was significantly higher in cirrhotic candidates (without exceptions) compared with cirrhotics with HCC, and non-HCC exceptions plus non-cirrhotic candidates (47.9%; 37.2% and 24.2%, respectively, with p=0.004). Post-LT survival was 84% per year, with no significant differences between the three groups (p=0.95).Prioritization for LT using the MELD score system has not decreased the dropout rate in Chile (persistent low donor's rate). Exceptions generate inequities in dropout rate, disadvantaging patients without exceptions.

Se presenta el caso de una mujer de 47 años, en muerte encefálica y sin expresiones previas sobre su voluntad de ser donante de órganos. El caso se expone como una narración contextualizada, anonimizada y seguida de un análisis bioético de las decisiones familiares y del rol del coordinador de trasplantes. La presentación, con una descripción del contexto y de los factores que condicionan las decisiones, tiene el propósito de contribuir al desarrollo de la capacidad deliberativa y a tomar mayor conciencia del problema ético de las decisiones familiares, del anonimato de la asignación de órganos y del sentido del final de la vida.

Acute liver failure in children. Experience of a liver transplant center Background: Acute liver failure (ALF) in childhood is defined as biochemical evidence of liver injury, absence of known chronic liver disease and coagulopathy not corrected by vitamin K administration, with INR greater than 1.5 if the patient has encephalopathy or greater than 2.0 if the patient does not have encephalopathy. Objective: Report the experience of a single liver transplant center (LT) in the treatment of 8 children with ALF and review the literature. Method: Retrospective review of clinical charts of patients with ALF. Results: The median age was 8 years-old (range 0-11), three females. Five patients underwent LT. Two patients died, one of them LT. The etiologies were 4 undetermined, 1 autoimmune, 1 Wilson Disease, 1 Parvovirus and 1 chronic graft rejection. All grafts were from cadaver donor, 3 of them reduced. Two out of five patients with encephalopathy grade III-IV died. The one year survival rate was 75%. Conclusions: Children with ALF should be treated in experienced centers with facilities for liver transplant. Transplantation should be offered only if the underlying disease is treatable by liver replacement and if transplant prognosis is better than that of the underlying disease. (Key words: acute liver failure, liver transplant). Rev Chil Pediatr 2009; 80 (2): 144-149

Sirolimus (SRL) is an immunosuppressive drug increasingly used in children undergoing solid organ transplantation. SRL does not cause glucose intolerance, hypertension, nephrotoxicity or neurotoxicity offering significant potential advantages over calceneurin inhibitors (CM).To report five children treated with SRL.A retrospective review of four children undergoing orthotopic liver transplantation (OLT) and one undergoing renal transplantation with recurrent acute rejection (RAR), chronic rejection (CR) or toxicity due to CM, treated with SRL between June 2001 and November 2006.As primary immunosuppressive therapy, all patients received 3 drugs: CM (Tacrolimus (FK) or Cyclosporine), mycophenolate mofetil and steroids. Mean age at treatment with SRL was 98 months. Children undergoing OLT had a late introduction of SRL (mean time after OLT: 37 months), and mean follow-up was 24 months. In this group rescue indications of SRL were RAR in one, CR in one, thrombotic thrombocytopenic purpura (TTP) in one, food allergy in one and other CM toxicity in three. Only one did not experience adverse events due to SRL, but no one required discontinuation of SRL. There were remissions of RAR, CR, TTP and food allergy. The patient with RT was switched from FK to SRL at day 18th after RT, but he had severe neutropenia that led to discontinuation of SRL.SRL may be useful in pediatric solid organ transplant recipients suffering from RAR, CR, TTP, food allergy and CM toxicity. Careful attention should be directed to detect side effects and avoid severe complications.

Background: Sirolimus (SRL) is an immunosuppressive drug increasingly used in children undergoing solid organ transplantation. SRL does not cause glucose intolerance, hypertension, nephrotoxicity or neurotoxicity, offering significant potential advantages over calceneurin inhibitors (CNI). Aim: To report five children treated with SRL. Material and methods: A retrospective review of four children undergoing orthotopic liver transplantation (OLT) and one undergoing renal transplantation with recurrent acute rejection (RAR), chronic rejection (CR) or toxicity due to CNI, treated with SRL between June 2001 and November 2006. Results: As primary immunosuppressive therapy, all patients received 3 drugs: CNI (Tacrolimus (FK) or Cyclosporine), mycophenolate mofetil and steroids. Mean age at treatment with SRL was 98 months. Children undergoing OLT had a late

Sirolimus (SRL) is a new immunosuppressive drug approved for renal transplantation, but is being used increasingly in orthotopic liver transplantation (OLT). Compared with the calcineurin inhibitors, SRL has different mechanisms of action and side effects profile. Thus, this drug offers significant potential advantages over other immunosuppressive agents. SRL does not cause glucose intolerance, hypertension or renal failure, but it may cause dyslipidemia, hepatic artery thrombosis, thrombocytopenia, anemia, leukopenia, oral mucosa ulcers, edema, arthralgias and wound complications. SRL inhibits the signal of interleukin 2 at a post-receptor level, inhibiting lymphocyte proliferation and fibroblast proliferation. It also has antineoplastic and antifungal effects. We report a 10 years old girl who underwent OLT, experiencing a biopsy-proven recurrent acute rejection (AR) in spite of using three immunosuppressive agents (tacrolimus, mofetil micofenolate and steroids). She developed diabetes mellitus as a consequence of the immunosuppressive therapy. She was rescued with SRL, not experiencing AR again. Mofetil micofenolate, steroids and insulin could be discontinued and tacrolimus doses were reduced, without experiencing severe complications. SRL is a new and safe immunosuppressive agent for rescue in patients with OLT and recurrent AR.

Sirolimus (SRL) is a new immunosuppressive drug approved for renal transplantation, but is being used increasingly in orthotopic liver transplantation (OLT). Compared with the calcineurin inhibitors, SRL has different mechanisms of action and side effects profile. Thus, this drug offers significant potential advantages over other immunosuppressive agents. SRL does not cause glucose intolerance, hypertension or renal failure, but it may cause dyslipidemia, hepatic artery thrombosis, thrombocytopenia, anemia, leukopenia, oral mucosa ulcers, edema, arthralgias and wound complications. SRL inhibits the signal of interleukin 2 at a post-receptor level, inhibiting lymphocyte proliferation and fibroblast proliferation. It also has antineoplastic and antifungal effects. We report a 10 years old girl who underwent OLT, experiencing a biopsy-proven recurrent acute rejection (AR) in spite of using three immunosuppressive agents (tacrolimus, mofetil micofenolate and steroids). She developed diabetes mellitus as a consequence of the immunosuppressive therapy. She was rescued with SRL, not experiencing AR again. Mofetil micofenolate, steroids and insulin could be discontinued and tacrolimus doses were reduced, without experiencing severe complications. SRL is a new and safe immunosuppressive agent for rescue in patients with OLT and recurrent AR (Rev Méd Chile 2005; 133: 1221-4)

Introducción: La infección por parvovirus humano B19 (PHB 19) produce un amplio rango de enfermedades que van desde eritema infeccioso en niños hasta artritis aguda en adultos. Algunos estudios sugieren un rol patogénico del PHB 19 en el desarrollo de la hepatitis aguda (HA) y falla hepática fulminante (FHF) en niños y adultos. La Anemia aplástica (AA) es una complicación reconocida de la HA y FHF por PHB 19. Objetivo: Reportar un caso de FHF por infección por PHB 19 y revisar la literatura. Caso clínico: Niña de 7 años de edad con HA que en una semana desarrolló FHF con serología IgM anti-PHB 19 positiva. Otras causas virales, autoinmunes, metabólicas o toxicas fueron descartadas. Fue sometida a trasplante hepático ortotópico (THO) y un año después no ha presentado complicaciones. Conclusiones: El PHB 19 puede causar HA y FHF, su oportuno diagnóstico y tratamiento, que en el caso de la FHF incluye el THO puede resultar en un pronóstico favorable Acute liver failure by Parvovirus B19 and liver transplant: Case report Introduction: Human Parvovirus B19 (HPV) infection produces a wide variety of clinical manifestations including erythema infectiosum in children and acute arthritis in adults. Different studies have suggested a pathogenic role of HPV in the development of acute hepatitis (AH) and acute liver failure (ALF) in children and adults. Aplastic anemia (AA) is a well-recognized complication of AH and ALF by HPV infection. Case: A 7 years-old girl with AH that in one week developed ALF with HPV IgM antibody titer (+). Other viral, metabolic and toxic etiologies were ruled out. She underwent orthotopic liver transplantation (OLT), with no complications one year after still. Conclusions: HPV can cause AH and ALF. Early diagnosis and treatment including OLT in case of ALF may have a favorable outcome

End stage renal disease is not an absolute contraindication for liver transplantation (LT) in patients with end stage liver disease. Actuarial patient and graft survival are comparable for children and adults who undergo LT alone and liver-kidney transplantation (LKT). The most common indications for LKT are the primary hyperoxaluria type I (PH1) and the liver and renal polycystic disease. We report a 12 years old boy with congenital hepatic fibrosis with severe portal hypertension, encephalopathy and polycystic kidney disease with end stage renal disease on dialysis that underwent LKT. During the second postoperative week, he had a biopsy-proven acute liver and renal rejection, that had a good response to corticosteroids. Thirty days after surgery, the liver biopsy was without rejection. No other complications were observed.

End stage renal disease is not an absolute contraindication for liv-er transplantation (LT) in patients with end stage liver disease. Actuarial patient and graft sur-vival are comparable for children and adults who undergo LT alone and liver-kidney trans-plantation (LKT). The most common indications for LKT are the primary hyperoxaluria type I(PH1) and the liver and renal polycystic disease. We report a 12 years old boy with congenitalhepatic fibrosis with severe portal hypertension, encephalopathy and polycystic kidney diseasewith end stage renal disease on dialysis that underwent LKT. During the second postoperativeweek, he had a biopsy-proven acute liver and renal rejection, that had a good response to corti-costeroids. Thirty days after surgery, the liver biopsy was without rejection. No other complica-tions were observed (Rev Med Chile 2003; 131: 1309-12).(

Focal nodular hyperplasia (FNH) is a benign tumour probably arising secondary to an altered vascularization of the hepatic parenchyma. It is rare in the paedriatric age group. We report a patient with FNH who underwent orthotopic liver transplantation (OLT) and we review the literature with respect to therapeutic approaches in treatment of FNH. A 15 year old boy presented with a palpable abdominal mass. Abdominal ultrasound and a CT scan showed multifocal tumours in both hepatic lobes. These were unresectable and a biopsy confirmed the diagnosis of FNH. The patient developed portal hypertension secondary to portal vein compression, with ascites, encephalopathy and renal failure. The decision between an expectant approach versus a surgical approach should be based on individual findings in each case. OLT is a therapeutic alternative in patients with benign unresectable liver tumours.

The success of orthotopic liver transplantation (OLT) has resulted in its widespread use for different liver diseases.To report our 8 years experience with adult OLT at Clinica Alemana de Santiago.In all transplantations done at the center, we recorded patient's overall data and survival, postoperative medical and surgical complications and causes of death.Between November 1993 and September 2001, 51 consecutive OLT were performed in 44 patients (22 females, median age 45 years old). Thirty eight patients presented with chronic and 6 with acute or sub-acute liver failure. Cryptogenic cirrhosis and hepatitis C infection were the most common causes for OLT. Postoperative bleeding and extrahepatic biliary complications were seen in 17.6 and 21.5% of cases respectively. Acute rejection, bacterial infections, CMV infection or disease and post OLT hemodialysis were the most common medical complications (51, 31, 19.6 and 19.6% of cases respectively). The overall 1 and 5 years survival rates were 80% and 73% respectively. Considering exclusively the last 22 OLT performed since January 1999, the 1 year survival rate has improved to 91%.Liver transplantation in Chile provides a good long term survival with acceptable morbidity, due to a multidisciplinary approach management. The survival rates have improved over the last few years probably due to better surgical techniques, ICU care and immunosuppression. These overall results are comparable with those from other Centers in developed countries.

Background: The success of orthotopic liver transplantation (OLT) has resulted in its widespread use for different liver diseases. Aim: To report our 8 years experience with adult OLT at Clinica Alemana de Santiago. Patients and methods: In all transplantations done at the center, we recorded patient's overall data and survival, postoperative medical and surgical complications and causes of death. Results: Between November 1993 and September 2001, 51 consecutive OLT were performed in 44 patients (22 females, median age 45 years old). Thirty eight patients presented with chronic and 6 with acute or sub-acute liver failure. Cryptogenic cirrhosis and hepatitis C infection were the most common causes for OLT. Postoperative bleeding and extra-hepatic biliary complications were seen in 17.6 and 21.5% of cases respectively. Acute rejection, bacterial infections, CMV infection or disease and post OLT hemodialysis were the most common medical complications (51, 31, 19.6 and 19.6% of cases respectively). The overall 1 and 5 years survival rates were 80% and 73% respectively. Considering exclusively the last 22 OLT performed since January 1999, the 1 year survival rate has improved to 91%. Conclusions: Liver transplantation in Chile provides a good long term survival with acceptable morbidity, due to a multidisciplinary approach management. The survival rates have improved over the last few years probably due to better surgical techniques, ICU care and immunosuppression. These overall results are comparable with those from other Centers in developed countries (Rev Méd Chile 2002; 130: 779-86)