RESUMEN
La hepatitis autoinmunitaria es una enfermedad inflamatoria crónica del hígado caracterizada por una interacción compleja entre factores genéticos, respuesta inmunitaria a antígenos presentes en los hepatocitos y alteraciones de la regulación inmunitaria. Presenta una distribución global, con predominio en individuos de sexo femenino. Se clasifica en dos grupos, según el tipo de autoanticuerpos séricos detectados. La forma de presentación más frecuente es la hepatitis aguda (40 %), con síntomas inespecíficos, elevación de aminotransferasas e hipergammaglobulinemia. El tratamiento estándar consiste en la administración de fármacos inmunosupresores. Es una patología compleja, a veces difícil de diagnosticar. Si no se trata de manera adecuada, la mortalidad puede alcanzar el 75 % a los 5 años de evolución.
Autoimmune hepatitis (AIH) is a chronic inflammatory condition of the liver characterized by a complex interaction among genetic factors, immune response to antigens present in hepatocytes, and immune regulation alterations. Its distribution is global and there is a female predominance. AIH is divided into 2 groups, depending on the type of serum autoantibodies detected. The most common presentation is acute hepatitis (40%), with nonspecific symptoms, high aminotransferase levels, and hypergammaglobulinemia. Standard treatment consists of the administration of immunosuppressive drugs. It is a complex condition, often difficult to diagnose. If not managed adequately, the 5-year mortality rate may reach 75%.
Asunto(s)
Humanos , Niño , Hepatitis Autoinmune/diagnóstico , Hepatitis Autoinmune/terapia , Gastroenterología , Autoanticuerpos , América LatinaRESUMEN
Autoimmune hepatitis (AIH) is a chronic inflammatory condition of the liver characterized by a complex interaction among genetic factors, immune response to antigens present in hepatocytes, and immune regulation alterations. Its distribution is global and there is a female predominance. AIH is divided into 2 groups, depending on the type of serum autoantibodies detected. The most common presentation is acute hepatitis (40%), with non-specific symptoms, high aminotransferase levels, and hypergammaglobulinemia. Standard treatment consists of the administration of immunosuppressive drugs. It is a complex condition, often difficult to diagnose. If not managed adequately, the 5-year mortality rate may reach 75%.
La hepatitis autoinmunitaria es una enfermedad inflamatoria crónica del hígado caracterizada por una interacción compleja entre factores genéticos, respuesta inmunitaria a antígenos presentes en los hepatocitos y alteraciones de la regulación inmunitaria. Presenta una distribución global, con predominio en individuos de sexo femenino. Se clasifica en dos grupos, según el tipo de autoanticuerpos séricos detectados. La forma de presentación más frecuente es la hepatitis aguda (40 %), con síntomas inespecíficos, elevación de aminotransferasas e hipergammaglobulinemia. El tratamiento estándar consiste en la administración de fármacos inmunosupresores. Es una patología compleja, a veces difícil de diagnosticar. Si no se trata de manera adecuada, la mortalidad puede alcanzar el 75 % a los 5 años de evolución.
Asunto(s)
Gastroenterología , Hepatitis Autoinmune , Autoanticuerpos , Niño , Femenino , Hepatitis Autoinmune/diagnóstico , Hepatitis Autoinmune/terapia , Humanos , América Latina , MasculinoRESUMEN
PURPOSE: The aim of this study was to report the long-term results of an institutional protocol of percutaneous biliary balloon dilatation (PBBD) on paediatric patients with benign anastomotic stricture after liver transplantation. As a secondary objective, we evaluated risk factors associated with post-treatment re-stricture. MATERIALS AND METHODS: Fourteen paediatric, post-liver transplant patients with benign anastomotic stricture of Roux-en-Y hepaticojejunostomy were included. All patients underwent the same treatment protocol of three PBBD procedures with 15-day intervals. Clinical outcome was analysed using the Terblanche classification. Primary patency rate was assessed with the Kaplan-Meier test. RESULTS: All patients had an initial successful result (Terblanche grade, excellent/good) after PBBD. At the end of the follow-up time of 35.7 ± 21.1 months (CI95%, 23.5-47.9), 10 patients persisted with excellent/good grading, while the remaining 4 had re-stricture, all of the latter occurring within the first 19 months. Patency rate after percutaneous treatment at 1, 3, and 5 years were 85.7%, 70%, and 70%, respectively. History of major complication after liver transplantation was associated with 5 times higher risk of re-stricture, HR 5.48 [95% CI, 2.18-8.78], p = 0.018. CONCLUSION: In paediatric patients with benign anastomotic stricture of hepaticojejunostomy after liver transplantation, the "Three-session" percutaneous biliary balloon dilatation protocol is associated with a high rate of long-term success. In this limited series, the history of post-liver transplant major complication, defined as complications requiring a reintervention under general anaesthesia or advanced life support, seems to be an independent risk factor for stricture recurrence.
Asunto(s)
Trasplante de Hígado , Niño , Constricción Patológica/cirugía , Dilatación/métodos , Humanos , Trasplante de Hígado/efectos adversos , Complicaciones Posoperatorias/diagnóstico por imagen , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/terapia , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
OBJECTIVE: To evaluate short- and long-term outcomes after live-donor liver transplantation (LT) with hyper-reduced grafts in low-weight pediatric recipients. LT is an established curative therapy for children with end-stage chronic liver disease or acute liver failure. A major problem in pediatric LT has been the lack of size-matched donor organs. The disadvantage of the use of large-for-size grafts is the insufficient tissue oxygenation and graft compression, which result in poor outcomes. The shortage of suitable donors is most notable in children under 10 kg. To overcome such obstacle, in situ hyper-reduced live-donor liver grafts have been introduced. Available articles in the literature are based on small samples and are deficient in long-term follow-up. METHODS: A single-cohort, retrospective analysis was conducted including 59 pediatric patients under 10 kg who underwent hyper-reduced (in situ "a la carte" left lateral segment reduction) live-donor LT (LDLT) between February 1994 and February 2018. RESULTS: The most frequent cause of liver failure was biliary atresia (70%). Median recipient weight was 8 kg. Vascular complications were confirmed in 15% of the sample, while 45% presented biliary complications. Median follow-up time was 40.3 months. Ten-year overall survival rate was 74%. Pediatric end-stage liver disease score > 23 was associated with a higher risk of post-operative complications. CONCLUSION: LDLT can be undertaken in children with body weight < 10 kg achieving good results in high-volume centers by experienced surgeons.
Asunto(s)
Enfermedad Hepática en Estado Terminal/complicaciones , Enfermedad Hepática en Estado Terminal/cirugía , Trasplante de Hígado/efectos adversos , Donadores Vivos , Complicaciones Posoperatorias/epidemiología , Delgadez/complicaciones , Peso Corporal , Enfermedad Hepática en Estado Terminal/mortalidad , Femenino , Humanos , Lactante , Masculino , Estudios Retrospectivos , Tasa de SupervivenciaRESUMEN
Biliary complications after living donor liver transplantation (LDLT) cause severe morbidity and mortality, with biliary anastomotic stricture being the most common form of presentation. Surgical revision is risky, and it is avoided whenever possible. When a Roux-en-Y hepaticojejunostomy (RYHJ) is used for bilioenteric reconstruction, endoscopic approach is more difficult, if not impracticable. Therefore, percutaneous approach remains as a first-line treatment in these patients. In this case presentation, a percutaneous approach was used to recover patency in an intractable, totally occluded RYHJ stricture in an LDLT paediatric recipient, using a Rösch-Uchida needle to access to the collapsed jejunal loop from the bile duct. Once recanalization of the RYHJ was achieved, a biodegradable stent was placed with middle-term patency at follow-up.
Asunto(s)
Implantes Absorbibles , Procedimientos Endovasculares/métodos , Yeyunostomía , Trasplante de Hígado , Complicaciones Posoperatorias/cirugía , Stents , Grado de Desobstrucción Vascular/fisiología , Conductos Biliares/diagnóstico por imagen , Conductos Biliares/fisiopatología , Conductos Biliares/cirugía , Niño , Pancreatocolangiografía por Resonancia Magnética , Constricción Patológica , Femenino , Humanos , Hígado/cirugía , Complicaciones Posoperatorias/diagnóstico por imagen , Complicaciones Posoperatorias/fisiopatología , Resultado del Tratamiento , Ultrasonografía IntervencionalRESUMEN
BACKGROUND: We had previously described a left lateral segment hyper-reduction technique capable of sizing the graft according to the volume of the abdominal cavity of the recipient. AIM: The purpose of our study was to evaluate our 14-year live-donor liver transplantation experience with in situ graft hyper-reduction in children under 10 kg of weight. PATIENTS AND METHODS: Between January 1997 and May 2011, we performed 881 liver transplants. Two hundred and seventy-seven (n = 277) involved pediatric recipients, of which 102 (37 %) were from live donors. Thirty-five (n = 35) patients under 10 kg of weight underwent hyper-reduced living donor liver transplants. There were 21 females (60 %) and 14 males (40 %), with a median age of 12 months (range 3-23) and a median weight of 7.7 kg (range 5.6-10). RESULTS: Median operative time was 350 min (range 180-510). Median cold ischemia time was 180 min (range 60-300). Twenty-six (n = 26) patients required intraoperative transfusion of blood products. There were 49 postoperative complications involving 26 patients (74 % morbidity rate). One-, 3-, and 5-year survival rates were 87, 79, and 74 %, respectively. Twenty-eight patients are currently alive. CONCLUSIONS: Hyper-reduced grafts provide an alternative approach for low-weight pediatric recipients. The relatively high immediate postoperative morbidity could be related to the complexity of these patients.
Asunto(s)
Peso Corporal , Fallo Hepático/cirugía , Trasplante de Hígado/métodos , Hígado/patología , Argentina , Femenino , Humanos , Terapia de Inmunosupresión/métodos , Lactante , Fallo Hepático/congénito , Donadores Vivos , Masculino , Tamaño de los Órganos , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/mortalidad , Tasa de Supervivencia , Recolección de Tejidos y Órganos/métodos , Ultrasonografía IntervencionalRESUMEN
Although the etiologies of pediatric acute liver failure (ALF) are diverse, ultimate pathophysiologic pathways and management challenges for these disorders, usually lethal in the pre-transplant era, are similar. This review considers particularly the mechanisms of, and monitoring for, intracranial hypertension and coagulopathy; summarizes detailed advice for management of the ALF-associated failures of multiple body systems; and reviews the variety of prognostic scores available to guide management and assist in choosing the patients most apt to benefit from liver transplantation and the optimal timing for such transplantation.
Asunto(s)
Fallo Hepático Agudo/terapia , Trastornos de la Coagulación Sanguínea/etiología , Niño , Encefalopatía Hepática/diagnóstico , Encefalopatía Hepática/terapia , Humanos , Presión Intracraneal/fisiología , Fallo Hepático Agudo/complicaciones , Fallo Hepático Agudo/diagnóstico , Trasplante de Hígado , Monitoreo Fisiológico/métodos , Selección de Paciente , PronósticoRESUMEN
The aim of this study was to study the incidence of chronic renal dysfunction in patients with more than 5 yr of follow-up following liver transplantation and to evaluate the benefit of decreasing cyclosporine A (CsA) dose combined with mycophenolate mofetil (MMF) on renal function and immune response in these patients. Between 1988 and 1994, 60 children were transplanted, and 86% survived >5 yr post-liver transplantation. Fourteen patients developed chronic renal dysfunction secondary to CsA toxicity as evaluated by renal biopsy. In 11 patients CsA dose was decreased to 40-90 mg/ml target levels and MMF 600 mg/m(2) twice daily was added to the immunosuppressive regimen. Plasma creatinine decreased (from 1.0 +/- 0.03 to 0.8 +/- 0.03 ng/dl, p < 0.007), creatinine clearance increased (from 66.8 +/- 3.0 to 99.2 +/- 6.3 ml/min/1.73 m(2), p < 0.002) and microalbuminuria decreased (from 21.0 +/- 8.6 to 3.6 +/- 1.1 mg/24 h, p < 0.05) after 12 months of CsA combined with MMF therapy. During combined therapy the proliferative, cytolytic response and cytotoxic antibodies showed no significant changes, whereas CD4/CD8 ratio increased (from 1.2 +/- 0.2 to 1.4 +/- 0.1, p < 0.05). Tumor necrosis factor-alpha secretion increased (p < 0.005) during MMF therapy. The release of interleukin-10 was strikingly augmented under both immunosuppressive regimens, but the release of transforming growth factor-beta and interferon-gamma did not change. Our findings indicate that initiation of MMF combined with reduced doses of CsA allowed the recovery of renal function with minor changes in the immune response.
Asunto(s)
Ciclosporina/uso terapéutico , Inmunosupresores/uso terapéutico , Fallo Renal Crónico/fisiopatología , Trasplante de Hígado , Ácido Micofenólico/análogos & derivados , Ácido Micofenólico/uso terapéutico , Adolescente , Niño , Ciclosporina/administración & dosificación , Ciclosporina/efectos adversos , Citocinas/efectos de los fármacos , Citocinas/metabolismo , Relación Dosis-Respuesta a Droga , Quimioterapia Combinada , Estudios de Seguimiento , Humanos , Inmunosupresores/administración & dosificación , Inmunosupresores/efectos adversos , Interferón-alfa/metabolismo , Interferón gamma/metabolismo , Interleucina-10/metabolismo , Fallo Renal Crónico/inducido químicamente , Fallo Renal Crónico/inmunología , Pruebas de Función Renal , Pruebas de Función Hepática , Factores de Tiempo , Factor de Crecimiento Transformador beta/efectos de los fármacos , Factor de Crecimiento Transformador beta/metabolismoRESUMEN
Twenty-four-hour ambulatory blood pressure monitoring (ABPM) has proven to have better reproducibility than office blood pressure (BP) and is increasingly used for the study of hypertension in children and adolescents. The aim of our study was to assess 24-h BP profiles and to compare the results of office BP measurements with ABPM in stable liver transplant recipients transplanted before the age of 18 yr. ABPM was performed in 29 patients (nine males, 20 females), aged 3.9-24.8 yr (median 10.8 yr). The investigation was conducted 1.1-11.5 yr (median 5.1 yr) following transplantation. ABPM confirmed hypertension in one out of three office hypertensive patients. Seven patients (24%), whose office BP recordings were within the normotensive range, were reclassified as hypertensive. Non-dippers (n = 17), arbitrarily defined as patients with less than 10% nocturnal fall in BP, were similarly distributed among patients with ambulatory normotension and ambulatory hypertension (chi(2), p = 0.79). In addition, non-dippers showed a negative correlation between 24-h total urinary albumin excretion and both systolic and diastolic nocturnal decline in BP (Rho = -0.48, p < 0.05 and Rho = -0.86, p < 0.01, respectively). Our study found office BP readings to be poorly representative of 24-h BP profile. Larger studies are needed to confirm our observations as well as to determine whether routine BP measurements in the follow-up of paediatric liver transplant recipients should be based solely on office BP.