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INTRODUCTION: Upper gastrointestinal bleeding (UGIB) secondary to portal hypertension (PHT), without endoscopic or surgical treatment options due to an ectopic or unidentified bleeding site or the patient's anatomic characteristics, is challenging in pediatric hepatology. The usual treatment in these cases includes intravenous Octreotide. Recently, the availability of long-acting release Octreo tide (OCT-LAR) for monthly intramuscular administration has become an interesting therapeutic alternative. OBJECTIVE: To report the case of an infant with UGIB due to PHT who was successfully treated with OCT-LAR. CLINICAL CASE: Eight-month-old patient with repeated episodes of UGIB due to extrahepatic portal vein malformation, requiring blood transfusions, and intravenous octreotide infusions. As neither endoscopic nor surgical treatment were feasible, we decided to start IM OCT- LAR monthly. After ten months of treatment, the patient did not present bleeding episodes. No medication-related events were observed. CONCLUSION: We consider that this report could help in the management of similar pediatric patients with UGIB due to PHT without conventional therapeutic possibilities.

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Resumen: Introducción: La hemorragia digestiva por hipertensión portal, sin alternativa de tratamiento endos- cópico o quirúrgico por localizaciones ectópicas, no identificadas del sitio de sangrado o caracterís ticas anatómicas, constituye un desafío terapéutico en Pediatría. El tratamiento habitual incluye la infusión de octreótido endovenoso. En los últimos años, la presentación de octreótido de liberación prolongada (OCT-LAR) para administración mensual intramuscular, resulta una alternativa tera péutica atractiva. Objetivo: Reportar el caso de un lactante con hemorragia digestiva por hiperten sión portal que recibió tratamiento exitoso con OCT-LAR. Caso Clínico: Paciente de 8 meses de vida, con malformación de vena porta extrahepática y episodios reiterados de sangrados digestivos con re querimientos transfusionales e infusiones de octréotido, sin posibilidad de tratamiento endoscópico o quirúrgico. Indicamos OCT-LAR intramuscular mensualmente. Después de diez meses de iniciado el tratamiento, el paciente no repitió sangrados digestivos y no presentó efectos adversos relacionados a la medicación. Conclusión: Consideramos que el reporte de este caso puede resultar de utilidad al presentar una nueva alternativa para el tratamiento de pacientes pediátricos con sangrado digestivo por hipertensión portal sin posibilidades terapéuticas convencionales.

Abstract: Introduction: Upper gastrointestinal bleeding (UGIB) secondary to portal hypertension (PHT), without endoscopic or surgical treatment options due to an ectopic or unidentified bleeding site or the patient's anatomic characteristics, is challenging in pediatric hepatology. The usual treatment in these cases includes intravenous Octreotide. Recently, the availability of long-acting release Octreo tide (OCT-LAR) for monthly intramuscular administration has become an interesting therapeutic alternative. Objective: To report the case of an infant with UGIB due to PHT who was successfully treated with OCT-LAR. Clinical Case: Eight-month-old patient with repeated episodes of UGIB due to extrahepatic portal vein malformation, requiring blood transfusions, and intravenous octreotide infusions. As neither endoscopic nor surgical treatment were feasible, we decided to start IM OCT- LAR monthly. After ten months of treatment, the patient did not present bleeding episodes. No medication-related events were observed. Conclusion: We consider that this report could help in the management of similar pediatric patients with UGIB due to PHT without conventional therapeutic possibilities.

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We have previously reported a strong association between HLA-DRB1*1301 and type 1 pediatric autoimmune hepatitis (PAH) and between HLA-DR*0405 and adult autoimmune hepatitis (AAH). Because human killer cell immunoglobulin-like receptors are known to be associated with susceptibility to autoimmune diseases, we investigated the frequencies of HLA-A, B, C, DRB1 and KIR genes in 144 type 1 PAH and 86 AAH patients, which were compared with 273 healthy controls. We demonstrated in PAH the increased frequency of the functional form of KIR2DS4-Full Length (KIR2DS4-FL), which in combination with HLA-DRB1*1301 revealed a strong synergistic effect (odds ratio=36.5). PAH-KIR2DS4-FL+ subjects have shown an increased frequency of their putative HLA-C*02, 04 and 06 ligands. KIR analysis of PAH also revealed a decreased frequency of KIR2DL2 gene and its ligand. In contrast, AAH cases have shown a weaker increased frequency of KIR2DS4-FL, a lack of synergistic effect with HLA class II antigens and a moderate association with HLA-DRB1*0405. Of note, we demonstrated that liver T cells have a unique pattern of KIR expression. These results show a KIR gene involved in autoimmune hepatitis and suggest a stronger genetic influence for the early onset type I autoimmune hepatitis.

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OBJECTIVES: To study the aetiology, outcome and prognostic indicators in children with acute liver failure (ALF). STUDY DESIGN: Retrospective chart review of 210 patients (107 males/103 females; median age: 5.33 years, range: 1-17.4). Patients were followed until discharge (group 1), death (group 2) or liver transplantation (LT; group 3). Data from group 1 were compared to data from the other two groups and King's College criteria were also assessed. RESULTS: Final diagnoses were: 128 (61%) hepatitis A, 68 (32%) indeterminate and 14 (7%) others. The characteristics of patients who survived (n = 59), died (n = 61) and underwent LT (n = 90) were analysed. In multivariate analysis, prothrombin time and encephalopathy III/IV were the most significant parameters suggesting a high likelihood of death. When King's College criteria were applied on admission in patients with and without transplantation, the positive predictive values were 96% and 95%, and the negative predictive values were 82% and 82%, respectively. CONCLUSIONS: Hepatitis A is the main cause of ALF in children in Argentina. Advanced encephalopathy and prolonged prothrombin time were significantly associated with death or need for LT. King's College criteria for predicting the outcome of ALF are applicable in children, including those with ALF due to hepatitis A infection.

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El síndrome de Crigler Najjar (SCN), de Herencia autosómica recesiva, es un trastorno causado por una deficiencia de la enzima bilirrubin UDP glucuronil transferasa. Se describen dos tipos de acuerdo a la ausencia o reducción de la actividad enzimática. Los pacientes presentan ictericia con aumento de bilirrubina inderecta desde el nacimiento y riesgo de Kernnícterus. El tratamiento consiste en exsanguino transfuciones (EXT) durante el período neonatal y lumino terapia (LMT) diaria prolongada. El transplante hepático es la única opción terapéutica curativa para el tipo 1. El tipo 2 responde al fenobarbital. El objetivo de este trabajo fue analizar retrospectivamente dos pacientes con SCN. En ambas se descartaron otras causas de hiperbilirrubinemia inderecta. Caso Clínico 1: Sexo femenino, eutrófica, comenzó con ictericia a los 3 días de vida, 72 horas más tarde la bilirrubina total (BT) era de 24mg/dl a predominio indirecto (BI), recibió LMT hasta los 25 días de vida, luego fenobarbital y LMT domiciliaria, 8 horas/día, con buena respuesta. A los 8 meses, con una BI 5.5 mg/dl se suspendió la LMT. Los valores de BI posteriores oscilaron entre 6 8 mg/dl, bajo tratamiento con FB. Debido a su evolucion clínica, se planteó como diagnóstico SCN tipo 2. Caso Clínico 2: Sexo femenino, eutrófica, presentó ictericia desde los 12 días de vida, con niveles de BI de 39mg/dl, se realizaron EXT y LMT. El examen neurológico era normal. Se indicó LMT domiciliaria 12 16 Hs/d y FB. La paciente persistió con valores de BI>20mg/dl. Se asumió como probable SCN tipo 1. Se inició evaluación pre trasplante hepático.Palabras clave: síndrome de Crigler Najjar, dianóstico, tratamiento, evolución

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El síndrome de Crigler Najjar (SCN), de Herencia autosómica recesiva, es un trastorno causado por una deficiencia de la enzima bilirrubin UDP glucuronil transferasa. Se describen dos tipos de acuerdo a la ausencia o reducción de la actividad enzimática. Los pacientes presentan ictericia con aumento de bilirrubina inderecta desde el nacimiento y riesgo de Kernnícterus. El tratamiento consiste en exsanguino transfuciones (EXT) durante el período neonatal y lumino terapia (LMT) diaria prolongada. El transplante hepático es la única opción terapéutica curativa para el tipo 1. El tipo 2 responde al fenobarbital. El objetivo de este trabajo fue analizar retrospectivamente dos pacientes con SCN. En ambas se descartaron otras causas de hiperbilirrubinemia inderecta. Caso Clínico 1: Sexo femenino, eutrófica, comenzó con ictericia a los 3 días de vida, 72 horas más tarde la bilirrubina total (BT) era de 24mg/dl a predominio indirecto (BI), recibió LMT hasta los 25 días de vida, luego fenobarbital y LMT domiciliaria, 8 horas/día, con buena respuesta. A los 8 meses, con una BI 5.5 mg/dl se suspendió la LMT. Los valores de BI posteriores oscilaron entre 6 8 mg/dl, bajo tratamiento con FB. Debido a su evolucion clínica, se planteó como diagnóstico SCN tipo 2. Caso Clínico 2: Sexo femenino, eutrófica, presentó ictericia desde los 12 días de vida, con niveles de BI de 39mg/dl, se realizaron EXT y LMT. El examen neurológico era normal. Se indicó LMT domiciliaria 12 16 Hs/d y FB. La paciente persistió con valores de BI>20mg/dl. Se asumió como probable SCN tipo 1. Se inició evaluación pre trasplante hepático.Palabras clave: síndrome de Crigler Najjar, dianóstico, tratamiento, evolución

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Orthotopic liver transplantation is the only definitive mode of therapy for children with end-stage liver disease. However, it remains challenging because of the necessity to prevent long-term complications. The aim of this study was to analyze the evolution of transplanted patients with more than one year of follow up. Between November 1992 and November 2001, 238 patients underwent 264 liver transplantations. A total of 143 patients with more than one year of follow up were included. The median age of patients +/- SD was 5.41 years +/- 5.26 (r: 0.58-21.7 years). All children received primary immunosuppression with cyclosporine. The indications for liver replacement were: fulminant hepatic failure (n: 50), biliary atresia (n: 38), cirrhosis (n: 37), chronic cholestasis (n: 13) and miscellaneous (n: 5). The indications for liver re-transplantation were: biliary cirrhosis (n: 7), hepatic artery thrombosis (n: 4) and chronic rejection (n: 3). Reduced-size liver allografts were used in 73/157 liver transplants, 14 of them were from living-related donors and 11 were split-livers. Patient and graft survival rates were 93% and 86% respectively. Death risk was statistically higher in retransplanted and reduced-size grafted patients. Growth retardation and low bone density were recovered before the first 3 years post-transplant. The incidence of lymphoproliferative disease was 7.69%. De novo hepatitis B was diagnosed in 7 patients (4.8%). Social risk did not affect the outcome of our population. The prevention, detection and early treatment of complications in the long-term follow up contributed to improve the outcome.

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Orthotopic liver transplantation is the only definitive mode of therapy for children with end-stage liver disease. However, it remains challenging because of the necessity to prevent long-term complications. The aim of this study was to analyze the evolution of transplanted patients with more than one year of follow up. Between November 1992 and November 2001, 238 patients underwent 264 liver transplantations. A total of 143 patients with more than one year of follow up were included. The median age of patients +/- SD was 5.41 years +/- 5.26 (r: 0.58-21.7 years). All children received primary immunosuppression with cyclosporine. The indications for liver replacement were: fulminant hepatic failure (n: 50), biliary atresia (n: 38), cirrhosis (n: 37), chronic cholestasis (n: 13) and miscellaneous (n: 5). The indications for liver re-transplantation were: biliary cirrhosis (n: 7), hepatic artery thrombosis (n: 4) and chronic rejection (n: 3). Reduced-size liver allografts were used in 73/157 liver transplants, 14 of them were from living-related donors and 11 were split-livers. Patient and graft survival rates were 93% and 86% respectively. Death risk was statistically higher in retransplanted and reduced-size grafted patients. Growth retardation and low bone density were recovered before the first 3 years post-transplant. The incidence of lymphoproliferative disease was 7.69%. De novo hepatitis B was diagnosed in 7 patients (4.8%). Social risk did not affect the outcome of our population. The prevention, detection and early treatment of complications in the long-term follow up contributed to improve the outcome.(AU)

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La hepatitis autoinmune (HAI) es una enfermedad de origen desconocido que conduce a la destrucción progresiva del parénquima hepático. Predomina en el sexo femenino y se aompaña de la presencia en suero de autoanticuerpos e hipergamaglobulinemia. Generalmente responde al tratamiento inmusupresor con esteroides y azatioprina. Las dosis elevadas de prednisona requeridas para lograr la remisión de la enfermedad, son responsables de severos efectos adversos. El objetivo de este trabajo fue evaluar la efectividad de la ciclosporina (CYA) para obterner la remisión del proceso inflamatorio y de la combinación de bajas dosis de prednisona más azatioprina para mantener esta condición, los parámetros relacionados con la velocidad de respuesta; las complicaciones del tratamiento; la evolución a largo plazo. Material y Métodos: cuarenta y dos pacientes con HAI, atendidos en nuestro servicio durnte el período enero 1994 - marzo 2001, fueron incluidos. El diagnóstico se realizó de acuerdo a los criterios Internacionales publicados. La CyA se indicó como tratamiento inicial, a una dosis de 4 mg/Kg/día durante los primeros 6 meses. En los pacientes con trasaminasas que no superaban el doble de los valores normales, se agregó prednisona a una dosis de 0,5 o 0,3 mg/kg/día en niños que pesaban menos o más de 20 Kg respectivamente, y azatioprina a una dosis de 1,5-2 mg/Kg/día, descendiendo la CyA gradualmente durante 15 días hasta suspenderla. Posteriormente a la discontinuación de la CyA, la dosis de prednisona fue disminuída lentamente, manteniendo la azatioprina sin camibos. Resultados: se obtuvo la normalización de las trasaminasas en 39/42 pacientes; en un 64 por ciento de los mismos la remisión ocurrió durante los primeros 6 meses de tratamiento con CyA en el resto dentro del primer año, con excepción de un caso que remitió a los 14 meses. dos/43 abandonaron los controles antes de los 6 meses de seguimiento y 1/42 casos fue considerado fracaso de tratamiento.

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El trasplante hepático (TH) constituye la única alternativa terapéutica para numerosas enfermedades hepáticas avanzadas. Los adelantos en la técnica quirúrgica y en la inmunosupresión desarrollados en los últimos años permitieron mejorar la sobrevida. En la evolución a largo plazo de los pacientes trasplantados pueden presentarse complicaciones de diversa severidad. Objetivo: analizar la evolución a largo plazo de los pacientes trasplantados con un seguimiento mayor de 1 año post-TH. Material y Métodos: Durante el período 11/92-11/01 se realizaron 264 TH en 238 pacientes. De estos pacientes 143 (157 TH) fueron seguidos más allá de un año post-TH. La mediana de edad (m.a más menos DS) fue de 5,41 años más menos 5,26 (r:0.58 - 21.7 años); 76 pertenecían al sexo femenino. Catorce (9.79 por ciento) recibieron un re-TH. Fueron excluidos los pacientes que no habían cumplido todavía un años post- TH o los que fallecieron antes de ese lapso de seguimiento. Las indicaciones de TH fueron: falla hepática fulminante (FHF) (n:50); atresia de vías biliares (AVB) (n:38); cirrosis (n: 37); colestasis crónica (n: 13) y otras (n: 5). Las indicaciones de Re-TH fueron: cirrosis biliar (n: 7); trombosis de la arteria hepática (n: 4) y rechazo crónico (n: 3). En 73/157 TH se utilizaron injertos reducidos: 14 donantes vivos relacionados (DVR) y 11 biparticiones hepáticas. Se sometieron a análisis estadístico variables potenciales de morbimortalidad. Resultados: La sobrevida global fue: pacientes 93 por ciento: injerto: 86 por ciento. El re-TH y el injerto reducido fueron las variables de mayor significación para aumento del riesgo de muerte en nuestra población. El déficit de talla y masa ósea se recuperó anes de los 3 años post-TH. La incidencia del síndrome linfoproliferativo (SLP) fue del 7.69 por ciento, su diagnóstico y tratamiento temprano permitió una evolución favorable en la mayoria de los casos.

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UNLABELLED: There are few cases reported of autoimmune hepatitis (AIH) type 2 presenting as fulminant hepatic failure (FHF) in children. The purpose of this study was to report three girls with AIH type 2 that presented as FHF. METHODS: Over a period of 12 years, 123 patients with AIH diagnosed based on international criteria, 9 (7%) were type 2.3 of them presented as FHF. Other etiologies (viral, metabolic and toxic) were ruled out. The treatment was started with prednisone (2 mg-kg-day) and azathioprine (2 mg-kg-day). EVOLUTION: Patients 1 and 3 died while waiting for liver transplant (LT) at 72 and 48 hours after initiating medical treatment. Patient 2 underwent LT3 days after starting treatment, with excellent evolution at 3 years and 7 months of follow up. CONCLUSIONS: 1--AIH type 2 was very infrequent in our group. 2--33% of cases had initial presentation as FHF. 3--The course of the disease was aggressive, not responding to immunosuppressive therapy. The evolution was unfavorable in all patients. 4--LT is an alternative treatment for this severe disease.

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El propósito de este trabajo es analizar los resultados del trasplante hepático(TH)en la falla hepática fulminante(FHF)por virus A.Entre 1993 y 1999 ingresaron a nuestro hospital 120 pacientes con FHF,de las cuales 72 fueron con virus A.Cincuenta pacientes se incluyeron en lista de espera para realizar un TH.Treinta seis de ellos fueron trasplantados.Se realizó un análisis estadístico uni y multivariado.Las variables utilizadas fueron la edad ,el tiempo en lista de espera,bilirrubinas,tiempo de Quick,factor V,tipo de hígado utilizado(entero o reducido)la encefalopatía y compatibilidad de grupo entre los hígados del donantes y receptor.Se evaluó la sobrevida actuarialcon el test de Kaplan Meyer.Del análisis univariado la utilización de hígados imcompatibles fue estadísticamente significativo para mal pronóstico.El grado de encefalopatía no fue estadísticamente significativo.Del estudio por regresión de Riesgo proporcional de Cox surge que la utillización de los hígados imcompatibles(p<0,0003)y los hígados reducidos(p<0,04)fueron factores independientes de mal pronóstico en la evolución del TH.La sobrevida actuarial de los pacientes fue de los pacientes fue de 77 por ciento al año y a los 3 años.Conclusiones:La utillización de los hígados imcompatibles y reducidos fueron factores de mal pronóstico.La sobrevida actuarial total de este grupo de pacientes fue de 77 por ciento al año y a los 3 años

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There are few cases reported of autoimmune hepatitis (AIH) type 2 presenting as fulminant hepatic failure (FHF) in children. The purpose of this study was to report three girls with AIH type 2 that presented as FHF. METHODS: Over a period of 12 years, 123 patients with AIH diagnosed based on international criteria, 9 (7

) were type 2.3 of them presented as FHF. Other etiologies (viral, metabolic and toxic) were ruled out. The treatment was started with prednisone (2 mg-kg-day) and azathioprine (2 mg-kg-day). EVOLUTION: Patients 1 and 3 died while waiting for liver transplant (LT) at 72 and 48 hours after initiating medical treatment. Patient 2 underwent LT3 days after starting treatment, with excellent evolution at 3 years and 7 months of follow up. CONCLUSIONS: 1--AIH type 2 was very infrequent in our group. 2--33

of cases had initial presentation as FHF. 3--The course of the disease was aggressive, not responding to immunosuppressive therapy. The evolution was unfavorable in all patients. 4--LT is an alternative treatment for this severe disease.

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UNLABELLED: Hepatitis A Virus (HAV) infection has been proposed as a possible trigger of autoimmune hepatitis type I. We have previously reported the presence of anti-actin antibodies en protracted hepatitis A. At present the presence of anti-actin antibodies in acute uncomplicated hepatitis A is unknown. The aim of this study was to evaluate the incidence and persistence of anti-actin antibodies un children with acute hepatitis A. MATERIALS AND METHODS: 38 patients, 21 female and 17 male, with mean age of 6.5 years (range 2-13 years) were included. All patients were anti HAV IgM positive. The patients were clinically controlled and laboratory determinations such as ALT/AST, gammaglobulin, gamma GT, nuclear, smooth muscle (anti-actin specificity) and liver-kidney-microsome type I (anti-LKM) antibodies, were evaluated at admission and at the first, third and fifth month. Anti-actin antibodies were determined by indirect immunofluorescense (IIF) on rat kidney, stomach and liver sections and also on monolayers of cultured fibroblasts. Titers higher than 1/40 were considered positive. RESULTS: 18 patients (47.3%) were anti-actin positive in the first determination (titers 1/40 and 1/80). In 4 patients (12.9%) these antibodies remained positive up to one month. All patients were negative 5 months after the onset of illness. ANA and anti-LKM were negative in all cases. CONCLUSIONS: 1) This data demonstrate the presence of anti-actin antibodies in children with uncomplicated HAV hepatitis. 2) The antibodies remained positive for a short period of time. 3) Titers were lower than in autoimmune hepatitis type I. 4) Taken together these results suggest that anti-actin antibodies would be an expression of non specific stimulation of lymphocyte B.

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El trasplante hepático (T. H.) constituye una opción válida en diversas enfermedades hepáticas de la infancia que no pose en una alternativa. El objetivo del trabajo es utilizar el crecimiento como un indicador para evaluar el exito de un plan T.H. en los niños sobrevivientes. De un total de 64 trasplantes realizados en 62 niños, desde noviembre de 1992 a enero de 1996, se estudiaron 31 niños seguidos por un período de 0.50 a 3.55 años, en los que se efectuaron mediciones antroprométricas seriadas, y evaluación de la maduración esquelética. Los resultados muestran que el crecimiento post- T-H., es diferente según distintos grupos. Los pacientes con atresia de vias biliares (A. V. B) y cirrosis autoinmune (CA) presentan un deficit de estatura similar al inicio del seguimiento (-1.14 y -1.39 DS) pero difieren en su evolución post T.H.: el grupo de AVB presenta crecimiento recuperatorio (CR) de 1.18 más menos 0.24 (P <0.005); los niños con (CA) 3n en cambio no mostraron CR y crecieron a una velocidad normal. Los pacientes con fallo hepático fulminante no presentan al momento del T.H deterioro alguno de su estatura y tienen un crecimiento post T.H normal. El cuarto grupo grupo (miscelánea) tiene un deficit inicial severo de talla, sin C.R. ulterior. La edad ósea pre T.H. estuvo retrasada con respecto a la edad cronológica -0.67 en grupo de pacientes crónicos, siendo la velocidad de edad ósea post T.H. normal. El crecimiento físico resulta un adeacudo indicador para evaluar la evolución a largo plazo de los niños con T.H. exitoso.

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Biliary atresia (BA) is one of the biliary tree anomaly more frequent. Occurs in about 0.8 to 1/10.000 live births. BA is defined as a progressive biliary tree. The prognosis depends on the age of the diagnosis and precocity surgery. We present the results of a retrospective analysis of 71 RA carried out at the Garrahan Hospital from 1987 to 1993. 47 were female and 24 were male. Age ranged from 45 to 120 days of life. This study involved a consecutive series of 58 patients with histopathologic study of Porta-hepatis (PH) and liver biopsy obtained during the Kasai. The purpose of this study was to determine the value of histological factors as type of PH and hepatocytic giant cell transformation (GCT). 82.8% had favorable type of PH and the CCT was mild in 84.5%. 72.4% had bad outcome and was independent of the type of PH. Neither of them were statistically significant with survive and evolution. In our service neither PH non CGT were predictors of a bad outcome. There were good outcome in 27.5%, died 37.9% and 10.3% undergo liver transplantation. The precocity in a diagnosis and surgical procedure before two months of age are the most important factors in correlation with survival. Others immunomorphologic factors must be studied in BA that explained the etiopathogenic process. Orthotopic liver transplantation is the successful therapy in children with BA.

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Biliary atresia (BA) is one of the biliary tree anomaly more frecuent. Occurs in about 0,8 to 1/10.000 live births. BA is defined as a progressive biliary tree. The prognosis depends ond the age of the diagnosis and precocity surgery. We present the resuls of a retrospective analysis of 71 RA carried out at the Garrahan Hospital from 1987 to 1993. 47 were female and 24 were male. Age ranged from 45 to 120 days of life. This study involved a consecutive series of 58 patients with histopathologic study of Porta-hepatis (PH) and liver biopsy obteined during the Kasai. The purpose of this study was to determine the value of histological factors as type of PH and hepatocitic giant cell transformation (GCT). 82.8 per cent had favorable type of PH and the CCT was mild in 84,5 per cent. 72,4 per cent had bad outcome and was independent of the type of PH. Neither of them were statiscaly significant with survive and evolution. In our serice neither PH non CGT were predicators of a bad outcome. There were good outcome in 27.5 per cent, died 37,9 per cent and 10.3 per cent undergo liver transplantation. The precocity in a diagnosis and surgical procedure before two months of age are the most important factors in correlation with survival. Others immunomorphologic factors must be estudied in BA that explained the ethiopatogenic process. Orthotopic liver transplantation is the succesful therapy in childrens with BA. (AU)

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Biliary atresia (BA) is one of the biliary tree anomaly more frecuent. Occurs in about 0,8 to 1/10.000 live births. BA is defined as a progressive biliary tree. The prognosis depends ond the age of the diagnosis and precocity surgery. We present the resuls of a retrospective analysis of 71 RA carried out at the Garrahan Hospital from 1987 to 1993. 47 were female and 24 were male. Age ranged from 45 to 120 days of life. This study involved a consecutive series of 58 patients with histopathologic study of Porta-hepatis (PH) and liver biopsy obteined during the Kasai. The purpose of this study was to determine the value of histological factors as type of PH and hepatocitic giant cell transformation (GCT). 82.8 per cent had favorable type of PH and the CCT was mild in 84,5 per cent. 72,4 per cent had bad outcome and was independent of the type of PH. Neither of them were statiscaly significant with survive and evolution. In our serice neither PH non CGT were predicators of a bad outcome. There were good outcome in 27.5 per cent, died 37,9 per cent and 10.3 per cent undergo liver transplantation. The precocity in a diagnosis and surgical procedure before two months of age are the most important factors in correlation with survival. Others immunomorphologic factors must be estudied in BA that explained the ethiopatogenic process. Orthotopic liver transplantation is the succesful therapy in childrens with BA.

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Apresentan los resultados del análisis retrospectivo de los primeros 50 trasplantes hepáticos ortotópicos (THO) realizados en el hospital Garrahan desde noviembre 1992 hasta septiembre de 1995. Las indicaciones fueron: 14 atresias de vías biliares, 13 hepatitis fulminante, 11 cirrosis autoinmune, 4 cirrosis criptogenética, 3 síndrome de Alagille, 1 glucogenosis con adenomas, 1 tirosemia con hepatocarcinoma, 1 deficiencia de alfa-1 antitripsina y 1 colangitis esclerosante primaria. El 67 por ciento de los pacientes presentó 1 o más episodios de rechazo agudo, y uno evolucionó al rechazo crónico. Las infecciones fueron causa importante de morbi-mortalidad y las complicaciones del tracto biliar se observaron en el 10 por ciento (3 fístulas y 2 estenosis). La supervivencia actuarial a 1 y 2 años de toda la serie fue del 74 por ciento, siendo para las hepatitis fulminantes del 61,54 por ciento y para los enfermos crónicos del 84 por ciento. El THO se presenta actualmentecomo la única alternativa terapéutica para el paciente con enfermedad hepática aguda o crónica terminal, permitiéndole una calidad de vida y desarrollo adecuados (AU)

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