Cardiac autonomic modulation in children with severe liver disease, before and after liver transplantation.

Background: The cardiovascular system is directly influenced by the autonomic nervous system (ANS); its changes affect heart rate variability (HRV) and are sensitive indicators of physiological changes. Autonomic dysfunction (AD) is manifested in up to 60% of patients with cirrhosis. Therefore, we aim to analyze the indexes of HRV pre- and post-surgery of children submitted for liver transplantation (LT).

Acute upper gastrointestinal bleeding due to portal hypertension in children: What is the best timing of endoscopy?

Objective: To compare initial clinical/laboratory parameters and outcomes of mortality/rebleeding of endoscopy performed <12 h(early UGIE) versus endoscopy performed after 12-24h(late UGIE) of ED admission in children with acute upper gastrointestinal bleeding(AUGIB) due to portal hypertension.

Methods: This is a retrospective cohort study. From January 2010 to July 2017, medical records of all children admitted to a tertiary care hospital with AUGIB due to portal hypertension were reviewed until 60 days after ED admission.

Sonographic Markers in the Prediction of Fetal Complex Gastroschisis.

Objective: To investigate the ultrasound (US) markers predictive of complex gastroschisis (CG), mortality, and morbidity in fetuses with gastroschisis.

Materials And Methods: This was a retrospective cohort study of 186 pregnancies with isolated fetal gastroschisis. Eight US markers were analyzed.

Treatment of short bowel syndrome in children. Value of the Intestinal Rehabilitation Program.

The main cause of acute intestinal failure is short bowel syndrome, generally as a result of resection of extensive segments of small intestine. As a result, the main symptoms are watery diarrhea, malabsorption syndrome, chronic malnutrition, and death, if the patient is not properly treated. If the length of the remaining intestine is greater than 30 cm, complete adaptation is possible and the patient may not require parenteral nutrition.

Long-term evaluation of fecal continence and quality of life in patients operated for anorectal malformations.

Introduction:: Patients operated for correction of anorectal malformations (ARM) can develop fecal incontinence, constipation, and soiling, with loss in quality of life.

Objective:: To evaluate, through the use of questionnaires, fecal continence, and quality of life of children in the late postoperative follow-up of ARM correction, both high and low. In addition, the levels of fecal continence and quality of life were compared with those of a control group.

Long-term results of the Duhamel technique are superior to those of the transanal pullthrough: A study of fecal continence and quality of life.

Background/purpose: The Duhamel and transanal pull-through (TAPT) techniques have been commonly used for the treatment of children with Hirschsprung disease (HD). However, despite adequate treatment, some patients present with fecal incontinence that severely affects the quality of life (QoL) and lead to psychiatric disorders. The objectives of the present study were to evaluate, through previously adapted questionnaires, the incidence of fecal incontinence and the quality of life (QoL) of children with HD who underwent Duhamel or TAPT techniques.

Outcome of isolated gastroschisis; an international study, systematic review and meta-analysis.

Objective: To determine outcome of children born with isolated gastroschisis (no extra-gastrointestinal congenital abnormalities).

Study Design: International cohort study and meta-analysis.

Primary Outcome: time to full enteral feeding (TFEF); secondary outcomes: Duration of mechanical ventilation, length of stay (LOS), mortality and differences in outcome between simple and complex gastroschisis (complex; born with bowel atresia, volvulus, perforation or necrosis).

Prognostic factors for the evolution and reversibility of chronic rejection in pediatric liver transplantation.

Objective: Chronic rejection remains a major cause of graft failure with indication for re-transplantation. The incidence of chronic rejection remains high in the pediatric population. Although several risk factors have been implicated in adults, the prognostic factors for the evolution and reversibility of chronic rejection in pediatric liver transplantation are not known.

Pediatric acute liver failure in Brazil: Is living donor liver transplantation the best choice for treatment?

Acute liver failure (ALF) in children is a life-threatening condition that often leads to urgent liver transplantation (LT). The aim of the present investigation was to describe the experience in Brazil in treating pediatric ALF, with an emphasis on the role of living donor liver transplantation (LDLT) in treating this condition. All children with ALF who fulfilled the criteria for an urgent LT were admitted to the intensive care unit.

[Validation of questionnaires to assess quality of life related to fecal incontinence in children with anorectal malformations and Hirschsprung's disease].

Objective:: Surgical treatment of anorectal malformations (ARMs) and Hirschsprung's disease (HD) leads to alterations in bowel habits and fecal incontinence, with consequent quality of life impairment. The objectives were to create and validate a Questionnaire for the Fecal Incontinence Index (FII) based on the Holschneider score, as well as a Questionnaire for the Assessment of Quality of Life Related to Fecal Incontinence in Children and Adolescents (QQVCFCA), based on the Fecal Incontinence Quality of Life.

Methods:: The questionnaires were applied to 71 children submitted to surgical procedure, in two stages.

New concepts and outcomes for children with hepatoblastoma based on the experience of a tertiary center over the last 21 years.

Objective: The aim of this study was to summarize the experience of a tertiary center in treating hepatoblastoma for the last 21 years.

Patients And Methods: Fifty-eight cases were included. The tumor extent and prognosis were assessed using the PRETEXT system.

Immediate expression of c-fos and c-jun mRNA in a model of intestinal autotransplantation and ischemia-reperfusion in situ.

Objective: Intestinal ischemia-reperfusion injury occurs in several clinical conditions and after intestinal transplantation. The aim of the present study was to investigate the phenomena of apoptosis and cell proliferation in a previously described intestinal ischemia-reperfusion injury autograft model using immunohistochemical markers. The molecular mechanisms involved in ischemia-reperfusion injury repair were also investigated by measuring the expression of the early activation genes c-fos and c-jun, which induce apoptosis and cell proliferation.

Effects of ischemic preconditioning in a pig model of large-for-size liver transplantation.

Objective: In most cases of pediatric liver transplantation, the clinical scenario of large-for-size transplants can lead to hepatic dysfunction and a decreased blood supply to the liver graft. The objective of the present experimental investigation was to evaluate the effects of ischemic preconditioning on this clinical entity.

Methods: Eighteen pigs were divided into three groups and underwent liver transplantation: a control group, in which the weights of the donors were similar to those of the recipients, a large-for-size group, and a large-for-size + ischemic preconditioning group.

Technique advance to avoid hepatic venous outflow obstruction in pediatric living-donor liver transplantation.

HVOO represents a serious critical complication of pediatric living-donor liver transplantation because open surgical repair is virtually impossible. Currently, despite several technical innovations and the introduction of triangulated anastomosis for hepatic vein reconstruction, the reported incidence of HVOO is still considerable. The aim of this study was to propose a new technique for hepatic venous reconstruction that avoids the original orifice of the recipient hepatic veins.

A new simplified technique of arterial reconstruction in pediatric living-donor liver transplantation: a comparison with the classical technique.

Background/aim: Hepatic artery anastomosis (HAA) is the most important aspect of living donor liver transplantation (LDLT), and it is currently performed by a specialized microsurgeon using micro surgical techniques, with interrupted sutures and the aid of an operative microscope. To simplify the procedure, we studied a new, simpler technique performed by pediatric transplant surgeons with continuous sutures and the same 3.5× magnification loupe used during other transplant procedures.

Postoperative care in pediatric liver transplantation.

In the last 25 years, liver transplantation in children has become an effective, definitive, and universally accepted treatment for terminal liver diseases. Long-term survival exceeds 80% and improves each year as the result of constant technical advancements and improvements in immediate postoperative intensive care and clinical control.

Living donor liver transplantation in children: should the adult donor be operated on by an adult or pediatric surgeon? Experience of a single pediatric center.

Background/purpose: Living donor liver transplantation has become a cornerstone for the treatment of children with end-stage hepatic dysfunction, especially within populations or countries with low rates of organ utilization from deceased donors. The objective is to report our experience with 185 living donors operated on by a team pediatric surgeons in a tertiary center for pediatric liver transplantation.

Methods: Retrospective analysis of medical records of donors of hepatic grafts for transplant undergoing surgery between June 1998 and March 2013.

Effects of tacrolimus and insulin in a liver regeneration model in growing animals with portal vein stenosis: immunohistochemical and molecular studies.

The aim of the present investigation was to describe a new model of liver regeneration in growing rats with reduced portal flow. In addition, it was studied whether tacrolimus and insulin could have any pro-regenerative effect under such conditions. Ninety-five rats were divided into five groups: Group 1 (sham), abdominal incision without intervention; Group 2, 70% hepatectomy; Group 3, 70% hepatectomy + PV stenosis; Group 4, 70% hepatectomy + portal vein stenosis + insulin; and Group 5, 70% hepatectomy + portal vein stenosis + tacrolimus.

Should we measure fetal omphalocele diameter for prediction of perinatal outcome?

Objective: To evaluate the fetal omphalocele diameter/abdominal circumference ratio (OD/AC) as a predictor of adverse perinatal outcome.

Methods: Analysis involving 47 singleton pregnancies with fetal omphalocele, normal karyotype and absence of other major abnormalities. The OD/AC ratio was determined antenatally by ultrasound and the best cutoff for the prediction of neonatal death was determined by receiver operating characteristic curve analysis.

A simplified experimental model of large-for-size liver transplantation in pigs.

Objective: The ideal ratio between liver graft mass and recipient body weight for liver transplantation in small infants is unknown; however, if this ratio is over 4%, a condition called large-for-size may occur. Experimental models of large-for-size liver transplants have not been described in the literature. In addition, orthotopic liver transplantation is marked by high morbidity and mortality rates in animals due to the clamping of the venous splanchnic system.

Conjoined twins: twenty years' experience at a reference center in Brazil.

Objective: This study reports on the experience of one hospital regarding the surgical aspects, anatomic investigation and outcomes of the management of 21 conjoined twin pairs over the past 20 years.

Methods: All cases of conjoined twins who were treated during this period were reviewed. A careful imaging evaluation was performed to detail the abdominal anatomy (particularly the liver), inferior vena cava, spleen and pancreas, either to identify the number of organs or to evaluate the degree of organ sharing.

Congenital chylous ascites: a report of a case treated with hemostatic cellulose and fibrin glue.

We report a case of an infant with recurrent chylous ascites who was unresponsive to conventional medical treatment. An exploratory laparotomy revealed no macroscopically visible sites of lymph leakage that could be ligated. Lymph exudation was noted in areas near the subhepatic recess and in the lesser sac surrounding the pancreas, which was not amenable to suture.

Effects of the administration of pentoxifylline and prednisolone on the evolution of portal fibrogenesis secondary to biliary obstruction in growing animals: immunohistochemical analysis of the expression of TGF-β and VEGF.

Objective: During the neonatal and infancy periods, some chronic liver diseases may lead to progressive hepatic fibrosis, which is a condition that can ultimately result in the loss of organ function and severe portal hypertension necessitating hepatic transplantation. In a previous report, pharmacological interventions were demonstrated to modulate hepatic fibrosis induced by bile duct ligation in young rats. The administration of pentoxifylline or prednisolone, or the combination of both, resulted in reduced fibrogenesis in portal spaces.