Case report: Per oral endoscopic myotomy (POEM) during pregnancy.

Esophageal achalasia is the most frequent primary motor disorder of the esophagus, resulting in a peristalsis and failed relaxation of the lower esophageal sphincter (LES). Various treatments have been described. Endoscopic alternatives include botulinum toxin injection and pneumatic balloon dilation, but both tend to fail with time and usually repeated interventions are required. Laparoscopic Heller myotomy (LHM) has been considered the gold standard. The diagnosis of achalasia during pregnancy is extremely rare and optimal management remains unclear. Our aim was to report a successful per oral endoscopic myotomy (POEM) performed during pregnancy. A 40-year-old pregnant woman presented with esophageal achalasia during her first trimester. Her disease was diagnosed with high resolution esophageal manometry (HREM). Initially an expectant conduct was attempted, but dysphagia progressed during the first 6 weeks of follow-up and an important weight loss was noticed. She underwent POEM at 15 weeks of gestation. After the procedure, she reported relief of both dysphagia and regurgitation, and her nutritional status improved. She delivered a healthy boy at term. During follow-up, she remains without dysphagia, her upper GI endoscopy shows no esophagitis and her HREM reports a normal integrated relaxation pressure. In achalasia, as in other clinical conditions, therapeutic decisions during pregnancy should always consider both the welfare of the mother and the fetus. POEM is a purely endoscopic treatment and has been recognized as a safe procedure to treat achalasia, with postoperative clinical success comparable to LHM and potential benefits over it.

[Experience with endoscopic submucosal dissection for the treatment of early neoplasms of the digestive tract]. / Disección endoscópica submucosa para el tratamiento de neoplasias incipientes del tubo digestivo: experiencia en un centro universitario de alto volumen.

BACKGROUND: Endoscopic submucosal dissection (ESD) allows en-bloc resection of early gastro-intestinal neoplasms (EGIN) with healing potential. AIM: To describe the results of patients treated with ESD for EGIN by our team. PATIENTS AND METHODS: Descriptive study of patients with EGIN who underwent ESD with curative intention between January 2008 and March 2020. RESULTS: One hundred thirty-two ESD were performed in 127 patients. 77% were gastric lesions, 14% colorectal, 8% esophageal and 1% duodenal. En-bloc resection was achieved in 98.4% of ESDs. Eighty eight percent of patients met curative standards. Overall, cancer-specific, and recurrence-free survival were 95%, 100% and 98% respectively. CONCLUSIONS: ESD allows en-bloc resections with curative potential in selected patients, but with a significant reduction in morbidity and mortality and less impact on quality of life. Our results suggest the feasibility to perform ESD in our country with results comparable to those reported in the literature.

[Surgical results of video-assisted thoracoscopic thymectomy for treatment of Juvenile Myasthenia Gravis]. / Resultados quirúrgicos de la timectomía por videotoracoscopía en el tratamiento de la Miastenia Gravis Juvenil.

INTRODUCTION: Juvenile myasthenia gravis (JMG) is an autoimmune disease affecting the neuromuscular junction that appears before 19 years of age with varying degrees of weakness of different muscle groups. The main treatment is pharmacological, but thymectomy has also demonstrated to improve remission rates. OBJECTIVE: To describe the clinical characteristics and postoperative course of pediatric patients with JMG who underwent video-assisted thoracoscopic (VATS) thymectomy. Clinical Serie: Six pa tients who underwent VATS thymectomy between March 2011 and June 2019. The age range at diag nosis was between 2 and 14 years and the average age at surgery was 7 years. All patients were under treatment with pyridostigmine bromide associated with immunosuppression with corticosteroids before surgery. The interval between diagnosis and thymectomy was 21.5 months on average. VATS was performed by left approach, and there was no perioperative morbidity or mortality. The average hospital stay was 2 days. Three patients remain with no symptoms and without corticotherapy. Two patients were on corticosteroids, but in smaller doses than previous to surgery. One patient presented a crisis requiring hospitalization and ventilatory support during follow-up. CONCLUSION: VATS thy mectomy is part of the treatment for JMG. In this series, it appears as a safe approach and its results were favorable.

Disección endoscópica submucosa para el tratamiento de neoplasias incipientes del tubo digestivo: experiencia en un centro universitario de alto volumen / Experience with endoscopic submucosal dissection for the treatment of early neoplasms of the digestive tract

Background: Endoscopic submucosal dissection (ESD) allows en-bloc resection of early gastro-intestinal neoplasms (EGIN) with healing potential. Aim: To describe the results of patients treated with ESD for EGIN by our team. Patients and Methods: Descriptive study of patients with EGIN who underwent ESD with curative intention between January 2008 and March 2020. Results: One hundred thirty-two ESD were performed in 127 patients. 77% were gastric lesions, 14% colorectal, 8% esophageal and 1% duodenal. En-bloc resection was achieved in 98.4% of ESDs. Eighty eight percent of patients met curative standards. Overall, cancer-specific, and recurrence-free survival were 95%, 100% and 98% respectively. Conclusions: ESD allows en-bloc resections with curative potential in selected patients, but with a significant reduction in morbidity and mortality and less impact on quality of life. Our results suggest the feasibility to perform ESD in our country with results comparable to those reported in the literature.

Per oral endoscopic myotomy (POEM) in pediatric patients with esophageal achalasia: First Latin-American experience.

BACKGROUND: Achalasia is the most common primary motor disorder of the esophagus, but its incidence in pediatric patients is low. Laparoscopic Heller myotomy (LHM) is the current surgical standard of care treatment. Per-oral endoscopic myotomy (POEM) has emerged as a safe and effective therapeutic alternative in adult patients. We herein report the outcomes of a cohort of pediatric patients with achalasia treated by POEM at a Chilean medical center. METHODS: This is a retrospective analysis of prospectively collected data on children who underwent POEM for esophageal achalasia. Clinical follow-up was evaluated by recording the Eckardt score, a high-resolution esophageal manometry (HREM) three months after the procedure, and an annual upper gastrointestinal endoscopy. RESULTS: Five patients with esophageal achalasia confirmed by HREM and with a mean age of 11 (5 to 15) years underwent POEM between 2017 and 2019. One patient had a previous LHM. No morbidity or mortality was observed. All patients resolved their dysphagia and no patient required further interventions. Mean Eckardt score reduced from 10 points preoperatively to 1 point postoperatively. Two patients currently have mild esophagitis (confirmed by endoscopy). CONCLUSION: Our results support the previously reported safety and effectiveness of POEM. Longer follow-up and larger cohorts will be important to confirm its role in the treatment of children with esophageal achalasia. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: Level IV.

Long-term Results of Endoscopic Submucosal Dissection (ESD) for the Treatment of Early Gastric Cancer (EGC) in a High-volume Latin American Center.

BACKGROUND: Gastric cancer is the second leading cause of death by cancer worldwide. Endoscopic submucosal dissection (ESD) is a technique that allows en bloc resection of early lesions of the digestive tract. It has curative potential in selected patients and benefits over gastrectomy for the treatment of early gastric cancer (EGC). The aim of this study is to present the results of ESD for EGC in a high-volume center in Chile. MATERIALS AND METHODS: Retrospective descriptive study of patients who underwent ESD for EGC at the Doctor Sótero del Río Hospital. RESULTS: A total of 100 ESDs were performed in 96 patients between 2008 and 2020. Fifty-five percent were female patients, the average age was 68 years (range, 45 to 89 y). En bloc resection was achieved in 98% of cases and the rate of complications Clavien grade III or higher was 8.3%. There were no cases of perioperative mortality. Ninety-three percent of the dissections were classified as R0 and 83% met curative standards according to expanded criteria. The mean follow-up was 42 months (range, 1 to 144 mo). Overall survival was 97%. Cancer-specific survival was 100% and recurrence-free survival was 97%. CONCLUSIONS: The present study describes the largest series of ESD for the treatment of EGC reported in Latin America. Our results support the feasibility of implementing ESD in Chile and indicate good oncological outcomes comparable to those reported in the large Asian series published to date.

Per oral endoscopic myotomy in a pediatric patient with achalasia. / Miotomía endoscópica por vía oral (POEM) en un paciente pediátrico para tratamiento de la acalasia esofágica.

INTRODUCTION: Achalasia is the most common primary motor disorder of the esophagus. Its reported incidence is low, even more in pediatric patients. Laparoscopic Heller myotomy is the current stan dard of treatment. During the last years, per-oral endoscopic myotomy (POEM) has been positioned as a safe and effective therapeutic alternative as the Heller procedure for esophageal achalasia. Ob jective: To describe the POEM technique and report the first pediatric case in our country. CLINICAL CASE: 11-year-old patient, previously healthy, who presented with progressive dysphagia for solids and liquids and weight loss. The study concluded a type II achalasia. The patient underwent a POEM and had a postoperative course without incidents. One year after the intervention, symptomatic, endoscopic and manometric resolution have been documented. CONCLUSIONS: The described case is the first POEM in a pediatric patient in our country. Esophageal achalasia is uncommon in pediatrics and POEM has demonstrated clinical success and safety comparable to laparoscopic Heller myotomy in short and medium term. Long-term follow-up will determine its definitive role in the treatment of pediatric patients with esophageal achalasia.

Miotomía endoscópica por vía oral (POEM) en un paciente pediátrico para tratamiento de la acalasia esofágica / Per oral endoscopic myotomy in a pediatric patient with achalasia

INTRODUCCIÓN: La acalasia es el trastorno motor primario más frecuente del esófago. Su incidencia reportada es baja, aún más en pacientes pediátricos. La miotomía de Heller laparoscópica corresponde al estándar actual de tratamiento. Durante los últimos años la miotomía endoscópica por vía oral (POEM) se ha posicionado como una alternativa terapéutica segura y tan efectiva como el Heller para la acalasia esofágica. OBJETIVO: Describir la técnica de POEM y reportar el primer caso pediátrico en nuestro país. CASO CLÍNICO: Paciente de 11 años, previamente sano, que se presentó con disfagia ilógica progresiva y baja de peso. El estudio concluyó una acalasia tipo II. Fue sometido a POEM y cursó un postoperatorio sin incidentes. A un año de la intervención se ha documentado resolución de la sintomatología, seguimiento endoscópico y manométrico sin complicaciones. CONCLUSIONES: El caso descrito corresponde al primer POEM en un paciente pediátrico en nuestro país. La acalasia esofágica es infrecuente en pediatría y el POEM ha demostrado éxito clínico y seguridad comparables a la miotomía de Heller laparoscópica en el corto y mediano plazo. El seguimiento a largo plazo permitirá determinar su rol definitivo en el tratamiento de pacientes pediátricos con acalasia esofágica.

INTRODUCTION: Achalasia is the most common primary motor disorder of the esophagus. Its reported incidence is low, even more in pediatric patients. Laparoscopic Heller myotomy is the current stan dard of treatment. During the last years, per-oral endoscopic myotomy (POEM) has been positioned as a safe and effective therapeutic alternative as the Heller procedure for esophageal achalasia. OBJECTIVE: To describe the POEM technique and report the first pediatric case in our country. CLINICAL CASE: 11-year-old patient, previously healthy, who presented with progressive dysphagia for solids and liquids and weight loss. The study concluded a type II achalasia. The patient underwent a POEM and had a postoperative course without incidents. One year after the intervention, symptomatic, endoscopic and manometric resolution have been documented. CONCLUSIONS: The described case is the first POEM in a pediatric patient in our country. Esophageal achalasia is uncommon in pediatrics and POEM has demonstrated clinical success and safety comparable to laparoscopic Heller myotomy in short and medium term. Long-term follow-up will determine its definitive role in the treatment of pediatric patients with esophageal achalasia.

Resección endoscópica por tunelización submucosa de un leiomioma de la unión gastroesofágica: reporte de un caso / Submucosal tunneling endoscopic resection of a gastroesophageal junction leiomyoma: report of a case

Resumen Introducción Los tumores submucosos del tracto gastrointestinal alto tienen potencial de malignidad y también pueden ser una carga para el paciente, por lo que se requiere disponer de un tratamiento seguro y eficaz. El surgimiento de la técnica de resección endoscópica por tunelización submucosa (STER) en los últimos años ha mostrado resultados prometedores. Caso clínico Se presenta el caso de un hombre de 47 años asintomático, en quien, en el contexto de un estudio preoperatorio de cirugía bariátrica, se pesquisa una lesión subepitelial en la unión gastroesofágica. La endosonografía alta es compatible con un leiomioma esofágico. Se realiza STER con éxito y sin complicaciones; a las 48 h es dado de alta. El estudio histopatológico confirmó un leiomioma esofágico. Discusión La técnica STER ha demostrado consistentemente ser segura y eficaz en el tratamiento de los tumores submucosos de la unión gastroesofágica. El desafío es difundir la técnica por el resto del país, para mayor beneficio de los pacientes.

Introduction Upper gastrointestinal submucosal tumors are potentially malignant lesions; so safe and efficient treatments are needed. In recent years, submucosal tunneling endoscopic resection (STER) has emerged as a novel therapeutic technique, with promising results. Case report 47-year-old male patient, previously asymptomatic, who was found to have a subepithelial lesion at the gastroesophageal junction. Upper endoscopic ultrasound was compatible with a leiomyoma. He underwent STER to remove the tumor and recovered with any complications. The biopsy confirmed an esophageal leiomyoma. Discussion STER has become a highly feasible and safe therapeutic option for submucosal tumors of the gastroesophageal junction. The challenge is to spread knowledge about this technique, to maximize patient's benefit.

Quiste esplénico no parasitario: revisión de la experiencia en un hospital clínico universitario / Non parasitic splenic cysts: single-center experience at a university hospital

Introducción: Las lesiones quísticas esplénicas son poco frecuentes; pueden ser clasificadas en primarias y secundarias según la presencia de revestimiento epitelial. Los quistes parasitarios son provocados por especies de Equinococcus y su manejo es específico. Suelen ser lesiones asintomáticas y el tratamiento depende de su tamaño y de la aparición de manifestaciones clínicas. El abordaje quirúrgico tradicional ha sido la esplenectomía, pero con el reconocimiento de las complicaciones inmunológicas, han tenido auge las alternativas que conservan parénquima esplénico. Objetivo: Comunicar la experiencia en el manejo quirúrgico de esta enfermedad en nuestro centro. Resultados: Se presenta la serie de 11 casos de quistes esplénicos no parasitarios que recibieron tratamiento quirúrgico durante los últimos 15 años. La mayoría de las intervenciones fueron laparoscópicas y no se registró morbimortalidad. Discusión: En el tratamiento de los quistes del bazo no parasitarios actualmente se reconoce el valor de preservar parénquima y función esplénica. La esplenectomía, cirugía cuyo estándar actual es mediante laparoscopia, ha sido progresivamente desplazada por alternativas conservadoras. Las opciones terapéuticas dependen del desarrollo de síntomas, del tamaño y de la ubicación de las lesiones. Conclusiones: Los quistes esplénicos son poco frecuentes. Ante la necesidad de tratamiento quirúrgico el abordaje laparoscópico y las cirugías que conservan parénquima esplénico deberían ser de elección.

Introduction: Splenic cysts are rare. They have been classified in primary or secondary lesions based on the presence or absence of an epithelial lining. Parasitic cysts are caused by Echinococcus spp. and have a particular management. Splenic cysts are usually asymptomatic, treatment depends on the development of symptoms and diameter. Traditionally, splenectomy has been the standard surgery, but updated knowledge about the role of the spleen in preventing some infections has led to more conservative options. Objective: To report our experience in surgical management of the disease. Results: Our series includes 11 patients with non-parasitic splenic cysts treated surgically, during the last 15 years. Most were laparoscopic interventions and no morbidity or mortality was registered. Discussion: Surgical treatment for non-parasitic splenic cysts actually favours conservative techniques; salvage of the spleen whenever possible is fully justified based on updated knowledge of the role it plays in promoting protection against infection. Splenectomy, nowadays performed by laparoscopy, has been partially displaced. Treatment options depends on the cyst diameter, development of symptoms and localization. Conclusions: Splenic cysts are unfrequent. If surgical treatment is needed, laparoscopy and interventions conserving splenic parenchyma should be the election.

[Vertebral fractures in children with Type I Osteogenesis imperfecta]. / Fracturas vertebrales en niños con osteogénesis imperfecta tipo I.

Osteogenesis imperfecta (OI) is an hereditary disease affecting conective tissue, mainly associated to growth retardation and pathological fractures. OI type I (OI type I), is the mildest, most often, and homogeneous in its fenotype. Vertebral fractures are the most significant complications, associated to skeletical and cardiopulmonary morbidity. OBJECTIVES: To characterize clinically a cohort of children with OI type I. PATIENTS AND METHODS: A cohort of OI type I children younger than 20 year old was evaluated. Demographic, clinical, biochemical and radiological data were registered. RESULTS: Sixty seven patients were included, 55% male, 69% resident in the Metropolitan Region. The mean age of diagnose was 2.9 years, 70% presented vertebral fractures on follow-up, mostly thoracic, and 50% before the age of 5 years. Fifty percentage presented vertebral fractures at diagnose, which was about the age of 5 years. Bone metabolic parameters were in the normal range, without significant change at the moment of vertebral fractures. Calcium intake was found to be below American Academy of Pediatrics recommendations at the time of the first fracture. CONCLUSIONS: In this study OI type I has an early diagnose, and vertebral fractures show a high incidence, mostly in toddlers. Calcium intake was found to be below reccomended values, and should be closely supervised in these patients.

[Congenital hyperinsulinism of the newborn: a case report]. / Hiperinsulinismo congénito del recién nacido: A propósito de un caso clínico.

Congenital hyperinsulinism (CH) is the most frequent cause of persistent hypoglycemia in the newborn and it is characterized by an inappropriately elevated insulin level in presence of hypoglycemia. Initial management is medical treatment, but if it fails, partial pancreatectomy is the surgical procedure of choice. OBJECTIVE: To report a case of a newborn with CH to aware on this condition and update its multidisciplinary management. CASE REPORT: The case of a full term newborn with severe symptomatic hypoglycemia unresponsive to medical treatment is reported . Ultrasonography and abdominal MRI were normal, Gallium 68 PET/CT showed uptake in the body of the pancreas. Laparoscopic corporocaudal pancreatectomy was performed. The patient resolved hypoglycemia and was discharged without incidents. Genetic study was consistent with focal CH, confirmed later by biopsy. DISCUSSION: This case report exposes the clinical presentation of CH and its diagnosis. It emphasizes on the surgical treatment and the advantages of minimally invasive technique.

Fracturas vertebrales en niños con osteogénesis imperfecta tipo I / Vertebral fractures in children with Type I Osteogenesis imperfecta

La Osteogénesis imperfecta (OI) es una enfermedad hereditaria del tejido conectivo, que se caracteriza principalmente por fragilidad ósea, deformidad y alteración del crecimiento. La OI tipo I (OI tipo I) es la más frecuente, leve y clínicamente homogénea. Su mayor complicación es la presencia de fracturas vertebrales, asociadas a morbilidad esquelética y cardiopulmonar. Objetivo: Caracterizar clinicamente una cohorte de niños con OI tipo I. Pacientes y Método: Se examinó una cohorte histórica de pacientes menores de 20 años mediante la revisión de fichas clínicas, rescatando las características demográficas, clínicas, bioquímicas y radiológicas. Resultados: Se incluyeron 67 pacientes, 55% varones, 69% de la Región Metropolitana. La edad media de diagnóstico fue de 2,9 años, el 70% de los pacientes presentó fracturas vertebrales de predominio torácico, y el 50% lo hizo antes de los 5 años. Un 15% presentó fracturas al momento del diagnóstico, siendo en ellos el diagnóstico de OI tipo I cerca de los 5 años. Los parámetros metabólicos óseos estuvieron en rangos adecuados durante el seguimiento, sin cambios significativos al diagnóstico de las fracturas vertebrales, excepto una disminución de la ingesta recomendada de calcio. Conclusiones: En este estudio, la OI tipo I presenta un diagnóstico precoz, principalmente en preescolares, y se asocia a alta frecuencia de fracturas vertebrales. La disminución en la ingesta de calcio demostrada al momento de la primera fractura requiere especial atención en estos pacientes.

Osteogenesis imperfecta (OI) is an hereditary disease affecting conective tissue, mainly associated to growth retardation and pathological fractures. OI type I (OI type I), is the mildest, most often, and homogeneous in its fenotype. Vertebral fractures are the most significant complications, associated to skeletical and cardiopulmonary morbidity. Objectives: To characterize clinically a cohort of children with OI type I. Patients and Methods: A cohort of OI type I children younger than 20 year old was evaluated. Demographic, clinical, biochemical and radiological data were registered. Results: Sixty seven patients were included, 55% male, 69% resident in the Metropolitan Region. The mean age of diagnose was 2.9 years, 70% presented vertebral fractures on follow-up, mostly thoracic, and 50% before the age of 5 years. Fifty percentage presented vertebral fractures at diagnose, which was about the age of 5 years. Bone metabolic parameters were in the normal range, without significant change at the moment of vertebral fractures. Calcium intake was found to be below American Academy of Pediatrics recommendations at the time of the first fracture. Conclusions: In this study OI type I has an early diagnose, and vertebral fractures show a high incidence, mostly in toddlers. Calcium intake was found to be below reccomended values, and should be closely supervised in these patients.

Hiperinsulinismo congénito del recién nacido: A propósito de un caso clínico / Congenital hyperinsulinism of the newborn: a case report

El hiperinsulinismo congénito (HC) es la causa más frecuente de hipoglicemia persistente del recién nacido (RN), y se caracteriza por secreción no regulada de insulina frente a hipoglicemia. El manejo inicial es médico, pero en caso de fracaso, la pancreatectomía parcial es el tratamiento quirúrgico de elección. Objetivo: Comunicar el caso de un RN con HC con énfasis en el estudio genético, de imágenes y manejo quirúrgico, incluyendo la cirugía laparoscópica. Caso clínico: RN de término, con hipoglicemia prolongada por HC que no respondió al tratamiento médico. La ultrasonografía y resonancia nuclear magnética abdominal fueron normales. PET/CT Galio 68 evidenció aumento de metabolismo glucídico focalizado en el cuerpo pancreático. Se realizó pancreatectomía córporo-caudal laparoscópica sin incidentes, con mejoría de la hipoglicemia. El estudio genético fue compatible con HC focal, confirmado luego por biopsia. Conclusión: Se expone acerca de las características clínicas y diagnóstico del HC, en particular el rol de la cirugía y ventajas del abordaje laparoscópico.

Congenital hyperinsulinism (CH) is the most frequent cause of persistent hypoglycemia in the newborn and it is characterized by an inappropriately elevated insulin level in presence of hypoglycemia. Initial management is medical treatment, but if it fails, partial pancreatectomy is the surgical procedure of choice. Objective: To report a case of a newborn with CH to aware on this condition and update its multidisciplinary management. Case report: The case of a full term newborn with severe symptomatic hypoglycemia unresponsive to medical treatment is reported . Ultrasonography and abdominal MRI were normal, Gallium 68 PET/CT showed uptake in the body of the pancreas. Laparoscopic corporocaudal pancreatectomy was performed. The patient resolved hypoglycemia and was discharged without incidents. Genetic study was consistent with focal CH, confirmed later by biopsy. Discussion: This case report exposes the clinical presentation of CH and its diagnosis. It emphasizes on the surgical treatment and the advantages of minimally invasive technique.

[Follow up for a cohort of patients with biliary atresia: late surgery and development of biliary cysts]. / Seguimiento de pacientes con atresia biliar: cirugía tardía y desarrollo de lagunas biliares.

Since the introduction of Kasai’s hepatic portoenterostomy, the prognosis of patients with biliary atresia has improved. The presence of intrahepatic biliary cysts or bile lakes has been reported in some patients after the intervention. Bile lakes have been related to cholangitis and a poor outcome. OBJECTIVE: To describe the clinical features and prognosis of patients with biliary atresia after Kasai portoenterostomy, with special emphasis in those who developed biliary cysts. PATIENTS AND METHOD: Data from a retrospective cohort of 13 patients with biliary atresia with a Kasai portoenterostomy from 2008 to 2016 was analyzed. Demographic variables associated to Kasai portoenterostomy, hepatic transplant, biliary cysts and colangitis episodes were tabulated. Kaplan Meir and Log Rank test were used to evaluate colangitis-free and native liver survival. RESULTS: The mean age at Kasai was 85 months (SD 40.3, 42-193 months), six patients (46%) had a Kasai operation after 90 days of life. Four patients (31%) developed multiple biliary cysts; all of them had at least one episode of cholangitis. Cholangitis-free survival was significantly lower for those who developed bile lakes. Nine patients (69%) underwent liver transplant, 3 of them because of recurrent cholangitis. There were no differences in global survival or native liver survival between patients with or without biliary cysts. CONCLUSIONS: The incidence of biliary cysts after Kasai portoenterostomy in this series is similar to the reported. The results are consistent with the relationship proposed between the development of biliary cysts and cholangitis. Our patients, some already derived for evaluation and liver transplantation, underwent Kasai operation at an advanced age, which determines a poor prognosis.

Seguimiento de pacientes con atresia biliar: cirugía tardía y desarrollo de lagunas biliares / Follow up for a cohort of patients with biliary atresia: late surgery and development of biliary cysts

Las dilataciones quísticas de las vías biliares, conocidas como lagunas biliares, se han descrito en el seguimiento de los pacientes sometidos a portoenterostomía o cirugía de Kasai por atresia de las vías biliares (AVB). Su aparición se ha asociado con el desarrollo de colangitis y consecuentemente a un peor pronóstico. OBJETIVO: Describir las características clínicas y pronóstico de pacientes con AVB, sometidos a cirugía de Kasai, con énfasis en aquellos que desarrollaron lagunas biliares. PACIENTES Y MÉTODO: Estudio retrospectivo de pacientes sometidos a portoenterostomía de Kasai por AVB durante los años 2008 a 2016. Se tabuló la información demográfica y variables asociadas a la portoenterostomía de Kasai, trasplante hepático, lagunas o quistes biliares y episodios de colangitis. Se crearon curvas de Kaplan Meier y comparación mediante el test de Log Rank para evaluar sobrevida global, libre de colangitis y con hígado nativo, considerando un valor p como significativo. RESULTADOS: Se analizó el seguimiento de 13 pacientes. El tiempo promedio de realización del Kasai fue a los 85 meses (rango 42-193, DS 40,3), seis pacientes (46%) fueron sometidos a cirugía de Kasai después de los 90 días de vida por derivación tardía. Cuatro (31%) desarrollaron lagunas biliares múltiples, todos presentaron episodios de colangitis. La sobrevida libre de colangitis fue significativamente menor para los portadores de lagunas biliares. Nueve pacientes (69%) recibieron un trasplante hepático, en promedio a los 16 meses de edad (rango 6-40, DS 12,1), en 3 de ellos la causa fue colangitis recurrente. No se encontraron diferencias significativas en la sobrevida con hígado nativo ni en sobrevida global entre portadores y no portadores de lagunas biliares. CONCLUSIONES: En esta cohorte, la incidencia de lagunas biliares luego de la portoenterostomía es similar a la descrita en la literatura. Los resultados concuerdan con la relación propuesta entre ellas y el desarrollo de colangitis. En los pacientes de nuestra serie, el diagnóstico y derivación de AVB fue realizado tardíamente, determinando un peor pronóstico.

Since the introduction of Kasai’s hepatic portoenterostomy, the prognosis of patients with biliary atresia has improved. The presence of intrahepatic biliary cysts or bile lakes has been reported in some patients after the intervention. Bile lakes have been related to cholangitis and a poor outcome. OBJECTIVE: To describe the clinical features and prognosis of patients with biliary atresia after Kasai portoenterostomy, with special emphasis in those who developed biliary cysts. PATIENTS AND METHOD: Data from a retrospective cohort of 13 patients with biliary atresia with a Kasai portoenterostomy from 2008 to 2016 was analyzed. Demographic variables associated to Kasai portoenterostomy, hepatic transplant, biliary cysts and colangitis episodes were tabulated. Kaplan Meir and Log Rank test were used to evaluate colangitis-free and native liver survival. RESULTS: The mean age at Kasai was 85 months (SD 40.3, 42-193 months), six patients (46%) had a Kasai operation after 90 days of life. Four patients (31%) developed multiple biliary cysts; all of them had at least one episode of cholangitis. Cholangitis-free survival was significantly lower for those who developed bile lakes. Nine patients (69%) underwent liver transplant, 3 of them because of recurrent cholangitis. There were no differences in global survival or native liver survival between patients with or without biliary cysts. CONCLUSIONS: The incidence of biliary cysts after Kasai portoenterostomy in this series is similar to the reported. The results are consistent with the relationship proposed between the development of biliary cysts and cholangitis. Our patients, some already derived for evaluation and liver transplantation, underwent Kasai operation at an advanced age, which determines a poor prognosis.

Is racecadotril effective for acute diarrhea in children? -First update. / ¿Es efectivo el racecadotrilo como tratamiento para la diarrea aguda en niños? -Primera actualización.

This article updates the December 2015 Living FRISBEE (Living FRISBEE: Living FRIendly Summary of the Body of Evidence using Epistemonikos), based on the detection of two systematic reviews not identified in the previous version. Gastroenteritis or acute watery diarrhea is usually a self-limited disease, but it is still associated to substantial healthcare costs and remains a frequent demand for medical care. Racecadotril, an intestinal enkephalinase inhibitor, has been used as treatment because it would decrease the duration of acute diarrhea and fluid loss. However there is still no evidence supporting its routine use. Searching in Epistemonikos database, which is maintained by screening 30 databases, we identified five systematic reviews including nine randomized trials relevant for our question. We combined the evidence using meta-analysis and generated a summary of findings table following the GRADE approach. We concluded racecadotril probably reduces the duration of acute diarrhea in pediatric patients, without increasing adverse effects.

Este resumen Epistemonikos (Living FRISBEE: Living FRIendly Summary of the Body of Evidence using Epistemonikos) es una actualización del resumen publicado en Diciembre de 2015, basado en la detección de dos nuevas revisiones sistemáticas que no habían sido identificadas en la versión anterior. La gastroenteritis o diarrea aguda es una enfermedad habitualmente autolimitada, pero que consume recursos sanitarios y constituye un motivo de consulta frecuente en pediatría. El racecadotrilo, un inhibidor de la encefalinasa intestinal, se ha usado como tratamiento porque disminuiría la duración de la diarrea y la pérdida de líquidos, pero no hay evidencia que justifique su uso rutinario. Utilizando la base de datos Epistemonikos, la cual es mantenida mediante búsquedas en 30 bases de datos, identificamos cinco revisiones sistemáticas que en conjunto incluyen nueve estudios aleatorizados relevantes para nuestra pregunta. Realizamos un metanálisis y tablas de resumen de los resultados utilizando el método GRADE. Concluimos que el uso de racecadotrilo probablemente disminuye la duración del cuadro de diarrea aguda en población pediátrica y que no se asociaría a mayor tasa de eventos adversos.