Servicio de hepatología y Trasplante hepático infantil del Hospital Universitario La Paz / Hepatology and Pediatric Liver Transplant in the Hospital Universitario La Paz

Las enfermedades hepáticas infantiles son diversas y raras. El tratamiento mediante trasplante hepático es necesario en el 60-80% de casos de hepatopatía de inicio neonatal. El Servicio de Hepatología del Hospital Infantil Universitario (HIU) La Paz, Madrid, ofrece experiencia especializada en el diagnóstico y tratamiento de niños con enfermedad hepática, fue constituido en 1975, acumula una gran casuística de estas enfermedades raras, dispone de programa de trasplante hepático desde 1986, con un total de 626 procedimientos de trasplante hasta 2012, de los cuales 122 fueron realizados con donante vivo relacionado. La supervivencia de pacientes trasplantados en la última década es de 95,5% al año y 88,5% a 10 años. Los resultados en niños con trasplante de donante vivo muestran supervivencia del 95,5% a 7 años. En conjunto, el Servicio de Hepatología y Trasplante Hepático HIU la Paz reúne las características idóneas de un centro de referencia nacional (AU)

Pediatric liver diseases are heterogeneous, rare entities. For those with neonatal onset, liver transplantation will be necessary in 60-80%. Service of Hepatology at Hospital Infantil Universitario La Paz, Madrid, offers specialized skills in diagnosis and treatment in children with liver disease. Activity started in 1975, it sums up a large series of these rare diseases, and has a liver transplantation (LT) program since 1986, 626 procedures of transplantation performed up to 2012, 122 interventions using living-related donors. LT survival results achieved in the last decade are: 95,5% at 1 year and 88,5% at 10th year. Survival is 95,5% at 7th year in children undergoing, living-donor liver transplantation. Overall, the Service of hepatology-Liver Transplantation meets optimum requirements of a national referral centre (AU)

Avances en hepatitis B y hepatitis C / Advances in hepatitis B and hepatitis C

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Fallo hepático neonatal como forma de presentación de colestasis intrahepática familiar progresiva / Progressive familial intrahepatic cholestasis presenting as liver failure

La colestasis intrahepática familiar progresiva (CIFP) es un grupo heterogéneo de colestasis de herencia autosómica recesiva y se inicia en el período neonatal o en los primeros años de vida. Existen 3 formas de CIFP en relación con las distintas mutaciones a nivel de los genes del sistema de transporte hepatocelular causantes de la formación de la bilis. Suele afectar a los niños en edad escolar o a los adultos jóvenes. Las principales manifestaciones son colestasis, ictericia y prurito, con una evolución lenta de la enfermedad hepática hacia la fibrosis en los primeros años de vida adulta. El diagnóstico se basa en la sospecha clínica con hallazgos bioquímicos compatibles (la actividad gamma-glutamiltransferrasa es normal en las CIFP tipo 1 y 2, pero es elevada en las tipo 3), pruebas de imagen que descarten otras causas de colestasis y anatomía patológica confirmatoria. El tratamiento inicial consiste en medidas sintomáticas como el ácido ursodesoxicólico. La derivación biliar parcial y el by-pass ileal representan opciones terapéuticas intermedias. En los casos que no respondan a los tratamientos anteriores, el transplante hepático sería la opción con mayores perspectivas curativas y buenos resultados de supervivencia. A continuación presentamos el caso de un neonato con CIFP tipo 2 y una presentación clínica poco habitual en el período neonatal como es el fallo hepático (AU)

Progressive familial intrahepatic cholestasis (PFIC) is a heterogeneous group of autosomic-recessive inherited cholestatic disorders that begin in the neonatal period or in the first years of life. There are three types of PFIC defined by different mutations located in the gene responsible for the bile flow through the intrahepatic canalicular transporter system. These disorders usually present in children or young adults and the main clinical manifestations are cholestasis, jaundice and pruritus, and they progress slowly towards liver fibrosis in adult life. PFIC diagnosis is based on clinical suspicion, biochemical findings (that include normal gamma-glutamyl transpeptidase in type 1 and 2, but increased levels in type 3), image techniques that rule-out other disorders, and histological confirmation. Initial treatment consists of symptomatic relief of cholestatic symptoms with choleretic agents (urso-deoxycholic acid). Partial biliary derivation and ileal bypass are intermediate therapeutic options. In case of no response to these treatments, liver transplantation is indicated. We report the case of a neonate with PFIC type 2 presenting as a liver failure (AU)

[Progressive familial intrahepatic cholestasis presenting as liver failure]. / Fallo hepático neonatal como forma de presentación de colestasis intrahepática familiar progresiva.

Progressive familial intrahepatic cholestasis (PFIC) is a heterogeneous group of autosomic-recessive inherited cholestatic disorders that begin in the neonatal period or in the first years of life. There are three types of PFIC defined by different mutations located in the gene responsible for the bile flow through the intrahepatic canalicular transporter system. These disorders usually present in children or young adults and the main clinical manifestations are cholestasis, jaundice and pruritus, and they progress slowly towards liver fibrosis in adult life. PFIC diagnosis is based on clinical suspicion, biochemical findings (that include normal gamma-glutamyl transpeptidase in type 1 and 2, but increased levels in type 3), image techniques that rule-out other disorders, and histological confirmation. Initial treatment consists of symptomatic relief of cholestatic symptoms with choleretic agents (urso-deoxycholic acid). Partial biliary derivation and ileal bypass are intermediate therapeutic options. In case of no response to these treatments, liver transplantation is indicated. We report the case of a neonate with PFIC type 2 presenting as a liver failure.

Hepatitis C: tratamiento en niños y gestantes / Hepatitis C: treatment in children and pregnant women

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[Crigler-Najjar syndrome: diagnosis and treatment]. / Síndrome de Crigler-Najjar: diagnóstico y tratamiento.

INTRODUCTION: Crigler-Najjar syndrome (CNS) is a very rare disease characterized by severe indirect hyperbilirubinemia from birth with normal liver function. It may cause kernicterus at any age. This disease is due to a total or partial deficiency of the UDP-glucuronosyltransferase enzyme caused by a mutation of the five exons of the ULT1A1 gene. PATIENTS AND METHODS: We reviewed the clinical outcomes of 7 children diagnosed with CNS between 1987 and 2004. RESULTS: There were three boys and four girls (two of which were homozygote twins). Two children had familial consanguinity. Three out of the six families had another healthy child. The mean follow-up was 8.3 years (14 months-17 years). In all patients, jaundice was detected in the first 3 days of life. The children were admitted to hospital between the fourth and the sixtieth day of life with jaundice and indirect bilirubin levels of between 12.5 and 32 mg/dl. In all patients, hemolysis was ruled out and hepatic function was normal. The diagnosis was based on genetic study in 4 patients, on inactive UGT enzyme in liver in 1 patient, and on clinical features exclusively in 2 patients. Treatment consisted of phenobarbital and phototherapy from 8 to 16 hours a day in all patients except three. Associated calcium salts were found in 5 patients and cholestyramine was found in two. Two patients developed kernicterus. Two underwent liver transplantation and bilirubin levels became normal. The remaining patients maintained indirect bilirubin from 15 to 25 mg/dl with no associated neurological alterations. CONCLUSIONS: Patients with CNS are at greater risk of developing kernicterus, mostly associated with indirect bilirubin levels of around 25 mg/dl. Phototherapy is very useful in these patients but the only definitive treatment is liver transplantation.

Síndrome de Crigler-Najjar: diagnóstico y tratamiento / Crigler-Najjar syndrome: diagnosis and treatment

Introducción El síndrome de Crigler-Najjar (SCN) es una entidad infrecuente, caracterizada por hiperbilirrubinemia indirecta grave desde el nacimiento con función hepática normal, y que puede ocasionar querníctero a cualquier edad. Se debe a un déficit total o parcial de la enzima UDP-glucuroniltransferasa (UGT) causado por mutaciones en los exones del gen UGT1A1. Pacientes y métodos Se revisa la evolución de 7 niños, diagnosticados de SCN, de 1987 a 2004. Resultados Los pacientes son 3 niños y 4 niñas, dos de ellas gemelas homozigotas. En 2 familias existía consanguinidad y en tres los hermanos eran sanos; el resto eran hijos únicos. El seguimiento medio fue de 8,3 años (14 meses-17 años). La ictericia fue detectada en todos en los primeros 3 días de vida. El ingreso que motivó la sospecha diagnóstica ocurrió entre el día 4 y el 60 con cifras de bilirrubina indirecta (BI) de entre 12,5 y 32 mg/dl. En todos se descartó hemólisis y la función hepática fue normal. El diagnóstico se basó en un estudio genético de 4 casos, en determinación de 0 % de la actividad de la enzima UGT en hígado en uno y en criterios clínicos exclusivamente en los dos restantes. El tratamiento consistió en fenobarbital y fototerapia de 8 a 16 h diarias en todos excepto en tres. En 5 casos se asociaron sales de calcio y en dos colestiramina. Un total de 2 pacientes desarrollaron querníctero durante la evolución; 2 niños fueron trasplantados con normalización del metabolismo de la bilirrubina. El resto presentaron cifras de BI de entre 15 y 25 mg/dl sin desarrollar alteraciones neurológicas. Conclusiones El principal riesgo de los pacientes con SCN es el desarrollo de querníctero, sobre todo con cifras de BI en torno a 25 mg/dl. La fototerapia es útil en el control de estos pacientes. El trasplante hepático es el único tratamiento definitivo

Introduction Crigler-Najjar syndrome (CNS) is a very rare disease characterized by severe indirect hyperbilirubinemia from birth with normal liver function. It may cause kernicterus at any age. This disease is due to a total or partial deficiency of the UDP-glucuronosyltransferase enzyme caused by a mutation of the five exons of the ULT1A1 gene. Patients and methods We reviewed the clinical outcomes of 7 children diagnosed with CNS between 1987 and 2004. Results There were three boys and four girls (two of which were homozygote twins). Two children had familial consanguinity. Three out of the six families had another healthy child. The mean follow-up was 8.3 years (14 months-17 years). In all patients, jaundice was detected in the first 3 days of life. The children were admitted to hospital between the fourth and the sixtieth day of life with jaundice and indirect bilirubin levels of between 12.5 and 32 mg/dl. In all patients, hemolysis was ruled out and hepatic function was normal. The diagnosis was based on genetic study in 4 patients, on inactive UGT enzyme in liver in 1 patient, and on clinical features exclusively in 2 patients. Treatment consisted of phenobarbital and phototherapy from 8 to 16 hours a day in all patients except three. Associated calcium salts were found in 5 patients and cholestyramine was found in two. Two patients developed kernicterus. Two underwent liver transplantation and bilirubin levels became normal. The remaining patients maintained indirect bilirubin from 15 to 25 mg/dl with no associated neurological alterations. Conclusions Patients with CNS are at greater risk of developing kernicterus, mostly associated with indirect bilirubin levels of around 25 mg/dl. Phototherapy is very useful in these patients but the only definitive treatment is liver transplantation

[Chronic liver disease associated with cystic fibrosis: energy expenditure at rest, risk factors, and impact on the course of the disease]. / Hepatopatía crónica asociada a fibrosis quística: gasto energético en reposo, factores de riesgo y repercusión en la evolución de la enfermedad.

INTRODUCTION AND GOALS: Cystic fibrosis is the most frequent recessive disease. There are various hepato-biliary alterations, of which the most important is the development of biliary obstruction and periportal fibrosis. The goal is to assess the influence of liver disease on the nutritional status, the progress of the disease and the associated risk factors. SCOPE: Nutrition Unit of the Gastroenterology Department at La Paz Children's Hospital. MATERIAL AND METHODS: Prospective longitudinal study with 53 patients assessed on three occasions over 3 years: at inclusion, after 1 year and after 3 years. Only 37 of them were followed-up for 3 years. A total of 111 measurements were taken, including analysis of body composition, energy expenditure, intake and energy losses as well as nitrogen balance. Simultaneously, respiratory function tests were performed and the presence of repeated acute respiratory problems was evaluated. RESULTS: 37 patients, 19 females and 18 males (mean age 13.04 years +/- 3.28). Twelve (32.43%) were diagnosed as having liver disease (mean age 12.16% +/- 3.86 SD, 11 males, 1 female), of whom 1 presented meconial ileum, 5 were homozygotic, 5 heterozygotic and the other two presented other mutations. Those with liver disease presented anthropometric parameters that were better than or similar to the patients without liver involvement (p NS). Mean Waterlow index in liver disease sufferers: 93.62% +/- 7.87 SD; without liver disease: 93.06% +/- 10.97 SD (p NS). Mean of LVEF in liver disease sufferers: 88.81 +/- 27.32 SD; without liver disease: 75.21 +/- 27.92 SD (p < 0.05). Mean FVC in liver disease sufferers: 95.38 +/- 22.92 SD; without liver disease: 83 +/- 23.7 SD (p < 0.05). Mean energy expenditure at rest/lean body mass in liver disease sufferers: 38.81 kcal +/- 7.26 SD; without liver disease: 42.36 kcal +/- f 6.91 SD (p < 0.05). CONCLUSIONS: The prevalence of chronic liver disease in patients with cystic fibrosis increases with age and is more frequent in males. Liver disease sufferers present a better evolution in pulmonary function and present less energy expenditure.

Hepatopatía crónica asociada a fibrosis quística: gasto energético en reposo, factores de riesgo y repercusión en la evolución de la enfermedad / Chronic liver disease associated with cystic fibrosis: energy expended at rest, risk factors and impact on the disease's progress

Introducción y objetivos: La fibrosis quística es la enfermedad recesiva más frecuente. Existen diferentes alteraciones hepatobiliares; la más importante es el desarrollo de obstrucción biliar y fibrosis periportal. El objetivo es valorar la influencia de la hepatopatía en el estado nutricional, la evolución de la enfermedad y los factores de riesgo asociados. Ámbito: Unidad de Nutrición del Servicio de Gastroenterología del Hospital Infantil La Paz. Material y métodos: Estudio longitudinal prospectivo con 53 pacientes valorados en tres momentos durante 3 años; al inicio, al año y a los 3 años. Solo 37 se siguieron los 3 años. Se realizan 111 mediciones que incluyen: análisis de la composición corporal, del gasto energético, de la ingesta y de las pérdidas energéticas así como balance nitrogenado. Simultáneamente se realizan pruebas de función respiratoria y se valora la presencia de reagudización respiratoria. Resultados: 37 pacientes, 19 mujeres y 18 varones (edad media 13,04 años ñ 3,28). Doce (32,43 por ciento) fueron diagnosticados de hepatopatía (edad media 12,16 años ñ 3,86 DS, 11 varones, 1 mujer) de los cuales 1 presentó íleo meconial, 5 eran homocigotos, 5 heterocigotos y los 2 restantes presentaban otras mutaciones. Los hepatópatas presentan parámetros antropométricos mejores o similares que los pacientes sin hepatopatía (p NS). Media del índice de Waterlow en hepatópatas: 93,62 por ciento ñ 7,87 DS; no hepatópatas: 93,06 por ciento ñ 10,97 DS (p NS). Media de FEV1 en hepatópatas: 88,81 ñ 27,32 DS; no hepatópatas: 75,21 ñ 27,92 DS (p < 0,05). Media de FVC en hepatópatas: 95,38 ñ 22,92 DS; no hepatópatas: 83 ñ, menos satisfactorios han sido los logros obtenidos en cuanto a su caracterización clínica. La prevalencia de la enfermedad hepática crónica en los pacientes con FQ varía según los criterios diagnósticos empleados. Se considera que los datos actuales sobre la prevalencia subestiman el riesgo real debido a la ausencia de marcadores diagnósticos de alta sensibilidad de enfermedad hepática en la FQ y a la cuestionable precisión de la histología8 la 23,7 DS (p < 0,05). Media del gasto energético en reposo/masa corporal magra en hepatópatas: 38,81 kcal ñ 7,26 DS; no hepatópatas: 42,36 kcal ñ 6,91 DS (p < 0,05).Conclusiones: La prevalencia de hepatopatía crónica en pacientes con fibrosis quística aumenta con la edad y es más frecuente en varones. Los pacientes hepatópatas tienen mejor evolución de la función pulmonar y presentan menor gasto energético (AU)

Introduction and goals: Cystic fibrosis is the most frequent recessive disease. There are various hepato-biliary alterations, of which the most important is the development of biliary obstruction and periportal fibrosis. The goal is to assess the influence of liver disease on the nutritional status, the progress of the disease and the associated risk factors. Scope: Nutrition Unit of the Gastroenterology Department at La Paz Children’s Hospital. Material and methods: Prospective longitudinal study with 53 patients assessed on three occasions over 3 years: at inclusion, after 1 year and after 3 years. Only 37 of them were followed-up for 3 years. A total of 111 measurements were taken, including analysis of body composition, energy expenditure, intake and energy losses as well as nitrogen balance. Simultaneously, respiratory function tests were performed and the presence of repeated acute respiratory problems was evaluated. Results: 37 patients, 19 females and 18 males (mean age 13.04 years ± 3.28). Twelve (32.43%) were diagnosed as having liver disease (mean age 12.16% ± 3.86 SD, 11 males, 1 female), of whom 1 presented meconial ileum, 5 were homozygotic, 5 heterozygotic and the other two presented other mutations. Those with liver disease presented anthropometric parameters that were better than or similar to the patients without liver involvement (p NS). Mean Waterlow index in liver disease sufferers: 93.62% ± 7.87 SD; without liver disease: 93.06% ± 10.97 SD (p NS). Mean of LVEF in liver disease sufferers: 88.81 ± 27.32 SD; without liver disease: 75.21 ± 27.92 SD (p < 0.05). Mean FVC in liver disease sufferers: 95.38 ± 22.92 SD; without liver disease: 83 ± 23.7 SD (p < 0.05). Mean energy expenditure at rest/lean body mass in liver disease sufferers: 38.81 kcal ± 7.26 SD; without liver disease: 42.36 kcal ±f 6.91 SD (p < 0.05). Conclusions: The prevalence of chronic liver disease in patients with cystic fibrosis increases with age and is more frequent in males. Liver disease sufferers present a better evolution in pulmonary function and present less energy expenditure (AU)

[Lymphoproliferative disorders of Waldeyer's ring]. / Linfomas primarios del anillo de Waldeyer.

OBJECTIVE: To investigate the incidence and characteristics of lymphoproliferative disorders of Waldeyer's ring in our pediatric patients. MATERIAL AND METHODS: We retrospectively reviewed 20 children under 14 years of age who underwent surgery in our department for adenoidectomy and/or tonsillectomy between 1 January 1996 and 30 November 2000. In non-immunocompromised children, surgical indication was the recent development of progressive unilateral tonsillar hyperplasia, and in immunocompromised patients indication was mainly the enlargement of adenoids and/or tonsils, although eradication of local Ebstein-Barr virus infection and recurrent acute tonsillitis were also indications. RESULTS: The mean age was 4.6+/-2.3 years (range: 16.9 months-13.9 years). Sixteen patients (80.0 %) were male. In the nine patients with unilateral hyperplasia, histopathological diagnosis was diffuse lymphoid hyperplasia. Of the 11 immunocompromised patients, 5 (45.5 %) had some type of lymphoproliferative disorder. DISCUSSION AND CONCLUSIONS: Lymphoproliferative disorders of Waldeyer's ring are relatively frequent in immunocompromised children who have undergone surgery of the adenoids and/or tonsils. Although no cases of unilateral hyperplasia of the tonsils were diagnosed in our series, tonsillectomy is indicated in patients with this diagnosis, independent of their immunological status.

Linfomas primarios del anillo de Waldeyer / Lymphoproliferative disorders of waldeyer's ring

Objetivo: Investigar la incidencia y características de los procesos linfoproliferativos del anillo de Waldeyer en pacientes pediátricos. Material y métodos: Se recogieron retrospectivamente 20 pacientes menores de 14 años intervenidos en nuestro servicio de adenoidectomía y/o amigdalectomía entre el 1 de enero de 1996 y el 30 de noviembre de 2000. La indicación en niños no inmunodeprimidos fue la aparición de una hiperplasia unilateral reciente y progresiva de amígdala y en el caso de inmunodeprimidos fue principalmente el crecimiento adeno y/o amigdalar, aunque también se intervinieron para erradicar infección focal por el virus de Epstein-Barr y por amigdalitis de repetición. Resultados: La media de edad fue de 4,6 ± 2,3 años (límites, 16,9 meses-13,9 años). Del total de los pacientes de nuestra serie, 16 fueron varones (80,0 %). El diagnóstico anatomopatológico de los 9 pacientes con hiperplasia unilateral de amígdala fue hiperplasia folicular linfoide. Por otro lado, en 5 de los 11 sujetos con inmunodeficiencias (45,5 %) se evidenció algún tipo de proceso linfoproliferativo. Discusión y conclusiones: Los procesos linfoproliferativos del anillo de Waldeyer son relativamente frecuentes en niños inmunodeprimidos intervenidos de adenoides y/o amígdalas. Aunque no se diagnosticó ningún caso en nuestra serie, consideramos conveniente operar a sujetos pediátricos con hiperplasia unilateral de amígdala, con independencia de su estado inmunológico (AU)

Hepatitis / Hepatitis

Las hepatitis virales son un conjunto de infecciones causadas por virus que afectan de forma exclusiva al hígado. El número de virus causantes de hepatitis ha ido incrementándose a medida que se han descubierto nuevos virus. En la actualidad conocemos los virus A, B, C, D, E, G y TTV, aunque los virus G y TTV no tienen ninguna relevancia clínica.Las hepatitis A y E presentan diferente prevalencia en relación a las condiciones socio-económicas de los países y no causan clonicidad, si bien pueden dar lugar a un fallo hepático agudo grave.Las Hepatitis C y B son un problema fundamental de salud pública difundido a nivel mundial, son responsables de hepatopatía crónica y hepatocarcinoma. A lo largo de la última década han ocurrido avances en el tratamiento que modifican de forma favorable el curso espontáneo de la hepatitis B y permiten obtener la curación en algunos pacientes con hepatitis C. La prevención mediante vacunas es la única forma eficaz de evitar esta enfermedad (AU)

[Pulmonary function after liver transplantation in cystic fibrosis. Report of four cases]. / Evolución respiratoria de 4pacientes confibrosis quística receptores detrasplante hepático.

OBJECTIVES: The aim of this study was to evaluate pulmonary function in four patients with cystic fibrosis (CF) after liver transplantation. PATIENTS AND METHODS: From 1993 to 1997 three males and one female, aged 12 to 15 years, required liver transplantation for CF with cirrhosis and portal hypertension. Three had a history of esophageal variceal bleeding. In three patients, forced vital capacity (FVC) and forced expiratory volume in one second (FEV1) before liver transplantation were over 80 and 75% of predicted values, respectively; in the fourth patient FVC was 37% and FEV1was 26%. Two patients presented allergic bronchopulmonary aspergillosis before transplantation. Only one patient was chronically infected in sputum with multiresistant Pseudomonas aeruginosa and none had Burkholderia cepacea. RESULTS: After liver transplantation, only the patient with P. aeruginosa in sputum culture and the worst pulmonary function presented a complicated course requiring mechanical ventilation for 43 days followed by non-invasive nasal ventilation for 8 months. This patient died 19 months after transplantation. The remaining three patients, with better pulmonary function before transplantation, presented an uncomplicated course and currently lead normal lives. CONCLUSIONS: We conclude that liver transplantation can improve pulmonary function and is well tolerated in children with CF and mild or moderate pulmonary involvement. When pulmonary involvement is severe, combined lung and liver transplantation should be considered.

Evolución respiratoria de 4 pacientes con fibrosis quística receptores detrasplante hepático / Pulmonary function after liver transplantation in cystic fibrosis. Report of four cases

Objetivos: El objetivo de este estudio es evaluar la función respiratoria de 4 pacientes con fibrosis quística sometidos a trasplante hepático. Pacientes y métodos: Entre 1993 y 1997, 4 pacientes con fibrosis quística, 3 varones y 1 mujer, de edades comprendidas entre los 12 y los 15 años, con cirrosis e hipertensión portal, fueron sometidos a un trasplante hepático. Tres tenían antecedentes de sangrado por varices gastroesofágicas. Antes del trasplante, 3 casos presentaban una capacidad vital forzada (FVC) y un volumen respiratorio máximo en el primer segundo (FEV1) superiores al 80 y 75%, respectivamente, con respecto a los valores teóricos; el cuarto caso presentaba una FVC del 37 por ciento y un FEV1 del 26%. Dos pacientes presentaron un brote de aspergilosis broncopulmonar alérgica en los meses previos al trasplante. Sólo un paciente estaba colonizado de forma crónica en esputo por Pseudomonas aeruginosa multirresistente y ninguno por Burkholderia cepacia. Resultados: Tras el trasplante hepático sólo el paciente colonizado por P. aeruginosa y peor función pulmonar presentó una evolución más tórpida precisando ventilación mecánica durante 43 días y ventilación nasal no invasiva durante 8 meses, y falleció a los 19 meses del trasplante. Los otros 3 casos, con mejor función pulmonar pretrasplante, presentaron una evolución sin complicaciones importantes y en la actualidad llevan una vida normal. Conclusiones: El trasplante hepático es bien tolerado en niños con fibrosis quística y afectación pulmonar leve-moderada, pudiendo incluso mejorar su función pulmonar. En los casos de afectación pulmonar grave debe considerarse la realización de un trasplante combinado hepatopulmonar (AU)

Short-term cyclosporine induces a remission of autoimmune hepatitis in children.

BACKGROUND/AIMS: The current immunosuppressive treatment of patients with autoimmune hepatitis consists of prednisone and azathioprine. High doses of prednisone used to obtain the remission of the disease are associated with serious adverse effects. To avoid harmful consequences of prednisone therapy, we proposed to treat patients with oral cyclosporine to obtain the remission of the inflammatory process. METHODS: This is a pilot, multinational, multicenter, clinical trial involving children with autoimmune hepatitis. Thirty-two children were recruited, who according to international criteria were considered as having definite autoimmune hepatitis. Cyclosporine alone was administered for 6 months, followed by combined low doses of prednisone and azathioprine for 1 month, after which cyclosporine was discontinued. Biochemical remission of the disease was established by the follow-up of serum transaminase activity levels. Growth parameters and adverse effects of the treatment were recorded. RESULTS: Two patients were withdrawn from the study: one for non-compliance and the other for liver failure which did not improve with cyclosporine. Of the 30 remaining patients, 25 normalized alanine aminotransferase activity levels by 6 months and all the patients by 1 year of treatment. Z-scores for height showed a trend towards improvement during treatment. Adverse effects of cyclosporine were mild and disappeared during weaning off the medication. CONCLUSIONS: Cyclosporine induced the biochemical remission of the hepatic inflammatory/necrotic process in children with autoimmune hepatitis, with few and well-tolerated adverse effects.

[A clinical analysis of internal or external diversion in biliary atresia]. / Análisis clínico de la derivación interna o externa en la atresia biliar.

Cholangitis has been the most common postoperative complication of Kasai's operation for biliary atresia. A host of ingenious surgical procedures have been used to prevent this complication, some including exteriorization of the bilioenteric conduit. The purpose of this report was to investigate the role of the derivation on the incidence of cholangitis, liver function and the survival of patients with biliary atresia treated with Kasai's operation. We have analyzed the clinical data of thirty-six patients with biliary atresia treated with Kasai's portoenterostomy during a seven year period (1987-1993). The patients were divided into two groups: Group I, patients treated with portoenterostomy and exteriorization of the bilioenteric conduit (n = 18) and Group II, patients treated with portoenterostomy without enterostomy. Our results suggest that the use of the exteriorization of the bilioenteric conduit was not shown to be effective in the prevention of episodes of cholangitis. The survival for both groups was not statistically significant and there was no increase in morbidity after the postoperative period or during the liver transplant.

[Experience with the impression cytology test for detecting vitamin A deficiency in children]. / Nuestra experiencia con el test de impresión citológica para detectar deficiencia de vitamina A en niños.

Impression cytology has been suggested as a relatively simple, cheap, practical and non invasive technique for documenting physiologically significant vitamin A deficiency. The vitamin A status of 41 patients was evaluated by serum retinol concentration by high power liquid chromatography (HPLC) and conjunctival impression cytology was performed on each child. All children with normal vitamin A status had normal conjunctival impression cytology. The sensibility of this method was 100%. However, in the 8 vitamin A deficient patients by impression cytology, only 6 of them showed low serum vitamin A levels. Impression cytology appears to detect preclinical vitamin A deficiency that this technique may by of potential usefulness as a screening tool for preclinical vitamin A deficiency populations.