Bárbara, una posible donante de órganos / Barbara, a potential organ donnor

This article presents the case of a 47 year old woman, on encephalic death state, who had not expressed her willing or acceptance for organ donation. The case is described as a narrative, contextualized, and in an anonymous way. A bioethical analysis of the family decision making and of the role of transplant coordinator is proposed. This presentation, with the description of its whole context and of the factors that influenced decisions, has the purpose to contribute to the development of deliberation competencies and to increase the awareness of the ethics problems of family decisions, of the conditions of anonymous organ assignation and of the of the end of life's meanings.

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.

Falla hepática fulminante en niños / 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 Parvo virus 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 percent. 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.

Falla hepática fulminante (FHF) en la infancia se define como evidencia bioquímica de daño hepático, sin antecedentes conocidos de enfermedad hepática crónica, coagulopatía no corregida por la administración de vitamina K e INR superior a 1,5 si el paciente tiene encefalopatía o superior a 2 si no tiene encefalopatía. Objetivo: Presentar la experiencia de un centro de trasplante hepático (TH) en el tratamiento de 8 niños con FHF y revisar la literatura. Pacientes y Método: Revisión retrospectiva de la historia clínica de pacientes con FHF. Resultados: La edad media fue de 8 años, rango 0-11, tres sexo femenino. Cinco pacientes fueron sometidos a TH. Dos pacientes fallecieron, uno de ellos con TH. La etiología fue indeterminada en 4 pacientes, 1 autoinmune, 1 enfermedad de Wilson, 1 parvovirus y 1 rechazo crónico del injerto. Todos los injertos fueron de donante cadáver, 3 de ellos reducidos. Dos de cinco pacientes con encefalopatía grado III-IV fallecieron. La tasa de sobrevida al año fue de 75 por ciento. Conclusión: El manejo de la FHF debe realizarse en un centro con capacidad de realizar TH, aunque no todos los pacientes requerirán finalmente esta terapia. El TH debiera ser ofrecido sólo si la enfermedad subyacente es tratable con reemplazo hepático y si el pronóstico del TH es mejor que el de la enfermedad misma.

Sirolimus en trasplante de órgano sólido pediátrico: Experiencia en 5 casos / Use of Sirolimus in five pediatric patients undergoing solid organ transplantation

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). 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 CM, treated with SRL between June 2001 and November 2006. Results: 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 ¡ate 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. Conclusions: 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.

Rescate con Sirolimus por rechazo agudo recurrente y diabetes mellitus en trasplante hepático: caso clínico pediátrico / Sirolimus for rescue of recurrent acute rejection and diabetes mellitus after liver transplantation: report of one case

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.

Falla hepática fulminante por parvovirus B 19 y trasplante hepático, caso clínico / Acute liver failure by Parvovirus B19 and liver transplant: case report

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.

Trasplante combinado hepato-renal: a propósito de un caso / Combined liver-kidney transplantation: report of one case

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 (Rev Méd Chile 2003; 131: 1309-12).

Hiperplasia nodular focal del hígado en niños y transplante hepático / Hepatic focal nodular hyperplasia in children and liver transplantation

La hiperplasia nodular focal (HNF) del hígado es una tumoración benigna probablemente secundaria a una alteración en la vascularización del parénquima hepático, rara en la edad pedíatrica. Objeto: reportar un caso clínico de HNF que requirió transplante hepático ortotópico (THO) y revisar la literatura actual con respecto a la conducta terapéutica a seguir. Caso clínico: niño de 15 años de edad con una masa abdominal palpable. Ecografía y tomografía axial computada de abdomen demostraron un tumor multifocal en ambos lóbulos hepáticos de características irresecables. Biopsia operatoria del hígado confirmo una HNF. El paciente desarrolló hipertensión portal debido a compresión tumoral de la vena porta, con ascitis, encefalopatía hepática e insuficiencia renal, por lo que se decidió su manejo mediante un THO en injerto total, con una excelente evolución a 5 años de seguimiento. Conclusión: la decisión entre un enfoque conservador o quirúrgico depende de las características de cada caso, siendo el THO una alternativa terapéutica en pacientes con tumores hepáticos benignos e irresecables

Trasplante hepático en adultos: casuística de Clínica Alemana de Santiago / Adult liver transplantation: a single center experience from Chile

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 percent 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 percent of cases respectively). The overall 1 and 5 years survival rates were 80 percent and 73 percent respectively. Considering exclusively the last 22 OLT performed since January 1999, the 1 year survival rate has improved to 91 percent. 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