RESUMEN
INTRODUCTION: Advances in immunosuppression have extended patient and graft survival rates after solid organ transplantation; however, this is not free of side effects. Balancing safety and efficacy is of paramount importance, particularly in the pediatric setting. Current literature comparing different protocols is scarce, and decisions are mostly guided by physician preference. We aimed to compare three different protocols from four different centers to identify differences in outcomes after one year of follow-up. MATERIALS AND METHODS: A retrospective analysis of the databases of the participating centers was performed. Consecutive patients aged <18 years with a first liver-only transplant and no other underlying congenital or acquired immunodeficiency were included. Patients were classified according to the immunosuppression protocol as follows: Group A (Prednisone + Tacrolimus + Basiliximab), Group B (Prednisone + Tacrolimus + Basiliximab + anti-thymocyte globulin), and Group C (Prednisone + Tacrolimus). Differences in survival, frequency of rejection, infections, and other complications were analyzed in the entire group (n=97) and in the group with biliary atresia (n=48). RESULTS: After one year of follow-up, no differences in patient or graft survival were observed when comparing either the entire group (n=97) or patients with biliary atresia only (n=48). The frequencies of rejection and episodes of infection were similar. Renal function showed no differences either before or after transplantation or between the groups. CONCLUSION: Immunosuppression protocols used in this study appeared to be equally safe and effective. This could offer the opportunity to tailor them to the patient's individual characteristics without compromising the outcome.
RESUMEN
INTRODUCTION: Intestinal failure, defined as the loss of gastrointestinal function to the point where nutrition cannot be maintained by enteral intake alone, presents numerous challenges in children, not least the timing of consideration of intestine transplantation. OBJECTIVES: To describe the evolution of care of infants and children with intestinal failure including parenteral nutrition, intestine transplantation, and contemporary intestinal failure care. METHODS: The review is based on the authors' experience supported by an in-depth review of the published literature. RESULTS: The history of parenteral nutrition, including out-patient (home) administration, and intestine transplantation are reviewed along with the complications of intestinal failure that may become indications for consideration of intestine transplantation. Current management strategies for children with intestinal failure are discussed along with changes in need for intestine transplantation, recognizing the difficulty in generalizing recommendations due to the high level of heterogeneity of intestinal pathology and residual bowel anatomy and function. DISCUSSION: Advances in the medical and surgical care of children with intestinal failure have resulted in improved transplant-free survival and a significant fall in demand for transplantation. Despite these improvements a number of children continue to fail rehabilitative care and require intestine transplantation as life-saving therapy or when the burden on ongoing parenteral nutrition becomes too great to bear.
Asunto(s)
Enfermedades Intestinales , Insuficiencia Intestinal , Síndrome del Intestino Corto , Trasplantes , Niño , Lactante , Humanos , Intestinos , Intestino Delgado , Nutrición Parenteral , Enfermedades Intestinales/cirugía , Síndrome del Intestino Corto/cirugíaRESUMEN
The understanding of the mechanisms for the development of ascites has evolved over the years, involving the liver, peritoneum, heart, and kidneys as key responsible for its formation. In this article, we review the pathophysiology of ascites formation, introducing the role of the intestine as a major responsible for ascites production through "a game changer" case.
Asunto(s)
Ascitis , Intestinos , Humanos , Ascitis/fisiopatología , Ascitis/etiología , Intestinos/fisiopatologíaRESUMEN
Resumen El síndrome de intestino corto es una entidad de baja incidencia en los pacientes pediátricos, pero se asocia con elevadas tasas de morbimortalidad. El abordaje de estos pacientes por un equipo interdisciplinario de expertos enfocados en la rehabilitación intestinal mejora los resultados a corto y a largo plazo. Entre los recursos disponibles para el tratamiento se incluye el teduglutide, un análogo del péptido similar al glucagón tipo 2 (GLP-2) elaborado mediante técnicas recombinantes. Por medio de la aplicación del método Delphi, a partir de la evidencia disponible y de la experiencia de los autores, se proponen recomendaciones para el uso de teduglutide, dirigidas a los profesionales de la salud que tratan a los pacientes pediátricos con síndrome de intestino corto, así como a las autoridades sanitarias.
Abstract Short bowel syndrome is a low-incidence disorder among pediatric patients, but it is associated with high morbidity and mortality rates. Management of these patients by an interdisciplinary team of experts focused on intestinal rehabilitation improves short- and long-term outcomes. Available resources for treatment include teduglutide, a glucagon-like peptide type 2 (GLP-2) analog made by recombinant techniques. Considering the available evi dence and the authors' experience, Delphi-based recommendations for the use of teduglutide are suggested for healthcare professionals who treat pediatric patients with short bowel syndrome, as well as for health authorities.
RESUMEN
RESUMEN Antecedentes: la insuficiencia intestinal, en su grado más grave, se conoce como fallo intestinal crónico (FIC). Las últimas décadas han sido testigo de la incorporación, evolución y perfeccionamiento de tratamientos que, en su conjunto, se denominan rehabilitación intestinal y abarcan tanto procedimientos quirúrgicos como tratamientos médicos que, llevados a cabo por equipos multi e interdisciplinarios, alcanzan altas tasas de éxito. Objetivo: describir los resultados de 16 años en el tratamiento de pacientes con FIC secundario a síndrome de intestino corto (SIC), y la evolución del papel de la cirugía, la rehabilitación médica y el trasplante. Material y métodos: análisis retrospectivo de una base de datos prospectiva de pacientes con fallo intestinal crónico secundario a SIC entre febrero de 2006 y marzo de 2022. Resultados: se incluyeron 492 pacientes (368 adultos-A y 124 pediátricos-P). Grupo A: 111 pacientes recibieron cirugía de reconstrucción autóloga del tracto gastrointestinal (CRATGI), 16 péptido semisintético similar al glucagón de tipo 2 (sGLP-2); el 83% logró la rehabilitación, con un 77% de supervivencia a 10 años; el 6,8% (17 pacientes) requirió trasplante intestinal (TxI), con un 89% de independencia de la nutrición parenteral (NP) al año y supervivencia post-TxI del 29% a los 10 años. Grupo B: 18 recibieron CRATGI; 9, enteroplastia serial transversa (STEP); y 6, sGLP-2; el 52% se rehabilitó, con una supervivencia del 69% a los 10 años; 28 pacientes recibieron TxI, con 69% de independencia de nutrición parenteral (NP) al año y supervivencia del 39% a los 10 años. Conclusión: los resultados presentados resaltan el papel central de la cirugía y la rehabilitación médica para alcanzar la suficiencia intestinal.
ABSTRACT Background: Severe intestinal insufficiency is known as chronic intestinal failure (CIF). In recent decades, medical treatments and surgical procedures have been incorporated, developed and improved under the name intestinal rehabilitation. When performed by multiand interdisciplinary teams, these treatments have high success rates. Objective: The aim of present study is to describe the 16-year outcomes in the management of patients with CIF secondary to short bowel syndrome (SBS) and the role of surgery, medical rehabilitation, and transplantation. Material and methods: We conducted a retrospective analysis on a prospective database of patients treated with chronic intestinal failure due to SBS between February 2006 and March 2022. Results: A total of 492 patients (368 adults (Group A) and 124 pediatric patients (Group B)] were included. Group A: 111 patients underwent autologous gastrointestinal reconstruction surgery (AGIRS), 16 were treated with semisynthetic glucagon-like peptide-2 (sGLP2); 83% achieved rehabilitation, with 77% survival at 10 years; 6.8% (17 patients) required intestinal transplantation (ITx), with 89% independence from parenteral nutrition (PN) at 1 year and post-ITx survival of 29% at 10 years. Group B: 18 patients underwent AGIRS; 9 underwent serial transverse enteroplasty (STEP); and 6 received sGLP2; 52% were rehabilitated, with 69% survival at 10 years; 28 patients received ITx, with 69% independence from PN at 1 year and 39% survival at 10 years. Conclusion: These results highlight the central role of surgery and medical rehabilitation in the recovery of intestinal function.
RESUMEN
La falla intestinal secundaria a síndrome de intestino corto en pediatría es una entidad poco frecuente, de alta morbimortalidad. Requiere de un equipo interdisciplinario para su abordaje, lo cual ha demostrado que disminuye la morbimortalidad y aumenta la posibilidad de que los pacientes logren la autonomía intestinal. Existe una falta de evidencia científica en diferentes abordajes de la patología. Consideramos necesario el desarrollo de esta Guía para el Manejo Clínico construida sobre la base de la metodología Delphi modificada, en la Asociación Argentina de Nutrición Enteral y Parenteral, por 16 expertos que se reunieron para discutir y consensuar los principales aspectos de tratamiento clínico. Se analizaron 4 aspectos: definiciones y epidemiología; nutrición enteral, nutrición parenteral; tratamientos farmacológicos y quirúrgicos,y criterios de derivación a centros de alta complejidad. Sin duda este documento será de utilidad para los pacientes, los profesionales y las instituciones, así como para los diferentes financiadores del sistema de salud.
Intestinal failure secondary to short bowel syndrome in pediatrics, is a rare condition with high morbimortality. A follow up multidisciplinary team is necessary to minimize complications and optimize the intestinal rehabilitation. There are no gold standard guidelines for the management of this group of complex patients. The development of clinical guidelines may contribute for an adequate management of patients with intestinal failure and short bowel syndrome. This Clinical Guideline for the Management was developed by 16 experts based on modified Delphi methodology. The meetings were held at the Argentinian Association of Enteral and Parenteral Nutrition (Asociación Argentina de Nutrición Enteral y Parenteral); the topics analyzed were definitions, epidemiology, enteral and parenteral nutrition, pharmacological and surgical treatments, and criteria for referring patients to intestinal rehabilitation centers. The document is aimed to provide basic scientific knowledge for medical institutions, health providers, healthcare providers, patients and families.
Asunto(s)
Humanos , Niño , Pediatría , Síndrome del Intestino Corto/complicaciones , Síndrome del Intestino Corto/terapia , Nutrición Parenteral , Intestino Delgado , IntestinosRESUMEN
El presente trabajo comunica la evolución posoperatoria inmediata y alejada de los pacientes pediátricos trasplantados en un único centro enla Argentina, desde marzo de 2006 hasta marzo de 2010, en variables demográficas, indicaciones, contraindicaciones, evolución y supervivencia de pacientes e injerto. Basándose en los resultados se puede concluir que el trasplante intestinal debe ser considerado como un tratamiento válido para todos aquellos pacientes que presenten insuficiencia intestinal,con complicaciones del soporte parenteral. Los resultados adquiridos en nuestro programa son similares a los comunicados internacionalmentey abren una nueva perspectiva para un grupo especial de niños que carecían de solución en nuestro medio.
The present is a retrospective analysis of all pediatric patients that underwent intestinal transplant from march 2006 to march 2010, describing demographics, indications, contraindications, clinical follow up and survival in a single center in Argentina. Based on the results shown one can conclude that intestinal transplant should be considered as a valid treatment for patients with intestinal insufficiency and complications related to parenteral nutrition. The results of our program are similar to those reported in the international Intestinal Transplant Registry. This opens a new perspective to a special population that otherwise would not have any other therapeutic option.
Asunto(s)
Humanos , Masculino , Femenino , Lactante , Preescolar , Niño , Adolescente , Intestinos , Intestinos/patología , Nutrición Parenteral , Trasplante , Epidemiología Descriptiva , Estudios RetrospectivosRESUMEN
El presente trabajo comunica la evolución posoperatoria inmediata y alejada de los pacientes pediátricos trasplantados en un único centro enla Argentina, desde marzo de 2006 hasta marzo de 2010, en variables demográficas, indicaciones, contraindicaciones, evolución y supervivencia de pacientes e injerto. Basándose en los resultados se puede concluir que el trasplante intestinal debe ser considerado como un tratamiento válido para todos aquellos pacientes que presenten insuficiencia intestinal,con complicaciones del soporte parenteral. Los resultados adquiridos en nuestro programa son similares a los comunicados internacionalmentey abren una nueva perspectiva para un grupo especial de niños que carecían de solución en nuestro medio.(AU)
The present is a retrospective analysis of all pediatric patients that underwent intestinal transplant from march 2006 to march 2010, describing demographics, indications, contraindications, clinical follow up and survival in a single center in Argentina. Based on the results shown one can conclude that intestinal transplant should be considered as a valid treatment for patients with intestinal insufficiency and complications related to parenteral nutrition. The results of our program are similar to those reported in the international Intestinal Transplant Registry. This opens a new perspective to a special population that otherwise would not have any other therapeutic option.(AU)