Cytomegalovirus infection and disease in pediatric liver transplantation: Burden of disease under a preemptive therapy approach.

Background: CMV remains a frequent complication after liver transplantation. Few studies exist in children reporting the epidemiology and outcomes of CMV after LT with current prevention strategies. Our goal is to report the incidence of CMV infection and disease in pediatric LT recipients under preemptive therapy, identify risk factors, complications, and adverse reactions to treatment.

Mutational spectrum of Mexican patients with tyrosinemia type 1: In silico modeling and predicted pathogenic effect of a novel missense FAH variant.

Background: Tyrosinemia type 1 (HT1, MIM#276700) is caused by a deficiency in fumarylacetoacetate hydrolase (FAH) and it is associated with severe liver and renal disfunction. At present, the mutational FAH (15q25.1, MIM*613871) spectrum underlying HT1 in the Mexican population is unknown.

Simultaneous Detection of Beta and Gamma Human Herpesviruses by Multiplex qPCR Reveals Simple Infection and Coinfection Episodes Increasing Risk for Graft Rejection in Solid Organ Transplantation.

Herpesviruses are common components of the human microbiome that become clinically relevant when a competent immunosurveillance is compromised, such as in transplantation. Members of the beta and gamma subfamilies are associated with a wide diversity of pathologies, including end-organ disease and cancer. In this study, we developed a multiplex qPCR technique with high specificity, sensitivity, efficiency and predictability that allowed the simultaneous detection and quantification of beta and gamma human herpesviruses.

Pediatric transplantation case conference: Update on cytomegalovirus.

The prevention and management of cytomegalovirus (CMV) remain challenging in children who have undergone solid organ transplantation, despite the availability of effective antiviral medications and sensitive diagnostic assays. The primary objective of this invited commentary is to provide an updated and multidisciplinary approach to persistently challenging CMV cases that commonly occur in pediatric transplantation candidates and recipients, including cases for which published data are frequently lacking.

A Novel c.91dupG Gene Mutation Is Associated with Early Onset and Severe Alagille Syndrome.

Alagille syndrome (MIM 118450) is an autosomal dominant disorder characterized by paucity of intrahepatic bile ducts, chronic cholestasis, pulmonary stenosis, butterfly-like vertebrae, posterior embryotoxon, and dysmorphic facial features. Most cases are caused by gene mutations. We report the case of a 2-year-old Mexican mestizo patient with Alagille syndrome, having exhibited jaundice and cholestatic syndrome as of three weeks of age.

Tolerability of up to 200 days of prophylaxis with valganciclovir oral solution and/or film-coated tablets in pediatric kidney transplant recipients at risk of cytomegalovirus disease.

This multicenter, open-label study evaluated the tolerability of extended prophylaxis with valganciclovir in pediatric kidney transplant recipients at risk of CMV disease. Fifty-six patients aged 4 months to 16 years received once-daily valganciclovir oral solution and/or tablets, dosed by BSA and renal function, for up to 200 days. The most common AEs on treatment were upper respiratory tract infection (33.

Consensus, Dilemmas, and Challenges in Living Donor Liver Transplantation in Latin America.

We reviewed the history, volume, outcomes, uniqueness, and challenges of living donor liver transplantation (LDLT) in Latin America. We used the data from the Latin American and Caribbean Transplant Society, local transplant societies, and opinions from local transplant experts. There are more than 160 active liver transplant teams in Latin America, but only 30 centers have used LDLT in the past 2 years.

A Scheduled Program of Molecular Screening for Epstein-Barr Virus Decreases the Incidence of Post-transplantation Lymphoproliferative Disease in Pediatric Liver Transplantation.

This is a cohort, retrospective, comparative study of all liver transplant recipients from a single center, from May 1998 to July 2015. Patients were divided into two groups according to the type of Epstein-Barr viral load monitoring. For group I (1998-2007), polymerase chain reaction (PCR) was not available or it was only qualitative with limited access.

CYP3A5 Genotype and Time to Reach Tacrolimus Therapeutic Levels in Renal Transplant Children.

Background: CYP3A5 gene polymorphism rs776746 has been associated with lower tacrolimus dose requirements and bioavailability in both adults and children. This variant causes a loss of CYP3A5 activity owing to a splice site variant leading to a truncated inactive enzyme. The aim of this study was to determine if the rs776746 gene polymorphism is related to the time to reach tacrolimus therapeutic levels in renal transplant children.

Pediatric liver transplantation in Latin America: Where do we stand?

LT started in LA in 1968, and pediatric LT records are available starting in the 1990s. Currently, eight countries perform pediatric LT in LA. Registries by national organizations fail to report robust data on pediatric LT.

Correlation between viral load of cytomegalovirus and tacrolimus and sirolimus levels in transplanted pediatric patients.

Introduction: Survival of transplant patients and grafts depends largely on the use of immunosuppressive drugs. However, a balance remains to be established among immunosuppression, transplant rejection and cytomegalovirus (CMV) infection, which results in a high rate of morbidity and mortality. The aim of this study was to define a better strategy for monitoring transplanted patients based on the analysis of the blood concentration of sirolimus and tacrolimus and the burden of CMV.

Polymorphism analysis and new JAG1 gene mutations of Alagille syndrome in Mexican population.

Alagille syndrome is a multisystem disorder with an autosomic dominant pattern of inheritance that affects the liver, heart, eyes, kidneys, skeletal system and presents characteristic facial features. Mutations of the JAG1 gene have been identified in 20-89% of the patients with Alagille syndrome, this gene encodes for a ligand that activates the Notch signaling pathway. In the present study we analyzed 9 Mexican patients with Alagille syndrome who presented the clinical criteria for the classical presentation of the disease.

Guidelines for the diagnosis and treatment of extrahepatic portal vein obstruction (EHPVO) in children.

Introduction: Extrahepatic portal vein obstruction is an important cause of portal hypertension among children. The etiology is heterogeneous and there are few evidences related to the optimal treatment.

Aim And Methods: To establish guidelines for the diagnosis and treatment of EHPVO in children, a group of gastroenterologists and pediatric surgery experts reviewed and analyzed data reported in the literature and issued evidence-based recommendations.

Liver exhibits thermal variations according to the stage of fibrosis progression: A novel use of modulated-differential scanning calorimetry for research in hepatology.

Aim: Liver fibrosis results in a disproportion of the hepatic composition and architecture, characterized by a progressive accumulation of fibrillar proteins at the liver parenchyma. Modulated-differential scanning calorimetry (mDSC) is an experimental methodology able to determine the specific thermal signature from any biological substance, based on the variation in heat flow and heat capacity. As these physicochemical properties are directly influenced by compositional and structural changes, we decided to study the thermal behavior of the liver during fibrosis using mDSC.

[Pediatric liver transplant program at Hospital Infantil de Mexico Federico Gomez].

This article reports the experience of the largest pediatric liver transplant (LT) program in México. Between June 1998 and May 2011, 76 LT were performed in 74 recipients, including 80% cadaveric-whole organ grafts and 20% segmental grafts, 12% of those coming from live donors and 8% from cadaver reduced donors. The most common indication for LT was biliary atresia (43%), followed by metabolic disorders (13%) and fulminant hepatitis (12%).

Anesthetic risk factors associated with early mortality in pediatric liver transplantation.

Introduction: Early mortality in pediatric patients after liver transplantation (30 days) may be due to surgical and anesthetic perioperative factors.

Objective: To identify anesthetic risk factors associated with early mortality in pediatric patients who undergo liver transplantation (OLT).

Materials And Methods: This retrospective study of all patients who underwent a deceased or living donor liver transplantation evaluated demographic variables of age, weight, gender, degree of malnutrition, and etiology, as well as qualitative variables of anesthesia time, bleeding, massive transfusion, acid-base balance, electrolyte and metabolic disorders, as well as graft prereperfusion postreperfusion characteristics.

Should a complex uropathy be a contraindication for renal transplantation in children?

Background: Anatomic and functional disorders of the lower urinary tract represent up to 40% of the causes of renal failure in children. Several centers avoid renal transplantation in these patients because of the high risk of complications and lower graft survival. The aim of this work was to determine the frequency of urinary tract abnormalities (UTAs) among our pediatric series, and to compare the frequency of complications, function, and long-term graft survival among patients without versus with UTA.

Cellular senescence in livers from children with end stage liver disease.

Background: Senescent cells occur in adults with cirrhotic livers independent of the etiology.

Aim: Investigate the presence rate of cellular senescence and expression of cell cycle check points in livers from children with end stage disease.

Methodology/principal Findings: Livers of five children aged three years or less undergoing liver transplantation due to tyrosinemia (n = 1), biliary atresia (n = 2), or fulminant hepatitis (n = 2) were analyzed for senescence associated beta-galactosidase (SA-betagal) activity and p16INK4a, p21cip1 and p53.

Valganciclovir dosing according to body surface area and renal function in pediatric solid organ transplant recipients.

Oral valganciclovir is effective prophylaxis for cytomegalovirus (CMV) disease in adults receiving solid organ transplantation (SOT). However, data in pediatrics are limited. This study evaluated the pharmacokinetics and safety of valganciclovir oral solution or tablets in 63 pediatric SOT recipients at risk of CMV disease, including 17 recipients < or =2 years old.

[Pediatric liver transplantation].

Pediatric liver transplantation has evolved over the last two decades into an effective and widely accepted therapy for infants and children. Currently, these high-risk patients achieve 85 to 90% one-year patient survival and an excellent quality of life. This paper reviews the special features of the pediatric recipient, the surgical innovations developed to be able to offer them a transplant (reduced size, live donor, split, and auxiliary partial transplantation), the most significant issues in anesthetic, immunosuppressive and postoperative care in children, as well as a global picture of the results.

[Renal transplantation in children].

Despite being considered a high risk procedure, renal transplantation has been recognized for more than 20 years as the best therapeutic option for children with end-stage renal disease since it is superior than any available dialytic procedure in improving the neuropsychological development and the quality of life. Today pediatric patients have similar graft survival than adults, and 10 year-old children or less have better outcome than any other age group. These remarking results are due to the development of specialized pediatric transplant centers and research programs, improvement in the selection and preparation of donors and recipients, refinement of the surgical technique and the use of new immunossupressive drugs.

Experience of a successful pediatric liver transplant program in Mexico.

Orthotopic liver transplantation (OLT) has been very difficult to develop in Mexico and for many years its occurrence was anecdotal. This report presents the results of a pediatric liver transplant program, analyzing the variables that affect outcomes. Between June 1998 and March 2004, 35 OLT were performed in 34 recipients including 80% cadaveric whole-organ grafts and 20% segmental grafts, with 11% from cadaveric and 9% from living donors.

Infantile hypertrophic pyloric stenosis: a comparative study of pyloric traumamyoplasty and Fredet-Ramstedt pyloromyotomy.

Purpose: The aim of this study was to compare the incidence of surgical complications (duodenal perforation, postoperative vomiting, wound infection or dehiscence, incisional hernia) between 2 different surgical techniques for the resolution of hypertrophic pyloric stenosis in children.

Methods: A clinically controlled, randomized study with follow-up from 24 to 36 months was conducted. One hundred children between 15 days and 2 months old, who underwent surgical resolution of hypertrophic pyloric stenosis, were put randomly into 2 groups: I, pyloric traumamyoplasty (n = 43); II, Fredet-Ramstedt pyloromyotomy (n = 57).