Telomere length is associated with intima-media thickness in pediatric liver transplant patients: A prospective cohort study.

Leukocyte telomere length (LTL) is a marker for biological age. Pediatric liver transplant recipients show a high rate of subclinical atherosclerosis, indicated by elevated intima-media thickness (IMT). We hypothesized that atherosclerosis is associated with biological age in these patients and investigated the course of LTL over time.

Variability of Care and Access to Transplantation for Children with Biliary Atresia Who Need a Liver Replacement.

Background & Aims: Biliary atresia (BA) is the commonest single etiology indication for liver replacement in children. As timely access to liver transplantation (LT) remains challenging for small BA children (with prolonged waiting time being associated with clinical deterioration leading to both preventable pre- and post-transplant morbidity and mortality), the care pathway of BA children in need of LT was analyzed—from diagnosis to LT—with particular attention to referral patterns, timing of referral, waiting list dynamics and need for medical assistance before LT. Methods: International multicentric retrospective study.

Adjuvant Therapy with Budesonide Post-Kasai Reduces the Need for Liver Transplantation in Biliary Atresia.

Based on the hypothesis that autoimmunological factors coregulate the pathomechanism in biliary atresia (BA), adjuvant therapy with steroids has become routine, although its efficacy has never been proven. In 2010, a study on the advantages of budesonide compared to prednisolone in autoimmune hepatitis gave rise to experimental therapy using budesonide as an adjuvant BA treatment. Ninety-five BA patients prospectively received a budesonide 2 mg/dose rectal foam daily for three months (SG).

A Case Series on Genotype and Outcome of Liver Transplantation in Children with Niemann-Pick Disease Type C.

Background: To report on clinical presentation and outcomes of children who underwent liver transplantation (LTx) and were subsequently diagnosed to have Niemann-Pick type C (NPC).

Methods: Retrospective, descriptive, multi-centre review of children diagnosed with NPC who underwent LTx (2003-2018). Diagnosis was made by filipin skin test or genetic testing.

Identification of Impaired Executive Functioning after Pediatric Liver Transplantation Using Two Short and Easily Applicable Tests: Cognitive Functioning Module PedsQL and Children's Color Trail Test.

We aimed to assess executive functioning in children after liver transplantation compared with healthy controls and in relation to real-life school performance using the PedsQL Cognitive Functioning Scale (CogPedsQL) and the Childrens' Color Trail Test (CCTT). One hundred and fifty five children (78f, median age 10.4 (1.

High Burden of Subclinical Cardiovascular Target Organ Damage After Pediatric Liver Transplantation.

Cardiovascular (CV) events account for 8%-13% of deaths after liver transplantation (LT) in adulthood. Although CV risk factors (RFs) are present, little is known about the prevalence of subclinical CV target organ damage (TOD) in children after LT. The aim of this prospective observational study was to assess the prevalence of subclinical CV TOD in children after LT and to identify RFs contributing to CV damage as potential targets for clinical intervention.

Impact of Immunosuppression on Executive Functioning After Pediatric Liver Transplantation: An Observational Cohort Study.

Objectives: Children after liver transplantation show increased rates of impaired cognitive functioning. We aimed to assess the potential effects of immunosuppressive therapy on executive functioning measured by the Children's Color Trail Test and the cognitive functioning module of the PedsQL (cogPedsQL) in liver transplanted children to explore potential targets for intervention to improve executive functioning.

Methods: We performed a cross-sectional study in 155 children (78 girls) aged 10.

Characterization of Glomerular Sox9 Cells in Anti-Glomerular Basement Membrane Nephritis in the Rat.

Mechanisms of glomerular crescent formation and podocyte repair processes are still unclear. Therefore, we investigated the expression of the transcription factor Sox9 as a potential marker of a subpopulation of parietal epithelial cells (PECs) with potential regenerative properties. Glomerular Sox9 expression was characterized in detail in a rat anti-glomerular basement membrane (GBM) nephritis model using immunofluorescence and confocal laser scanning microscopy.

Immune monitoring after pediatric liver transplantation - the prospective ChilSFree cohort study.

Background: Although trough levels of immunosuppressive drugs are largely used to monitor immunosuppressive therapy after solid organ transplantation, there is still no established tool that allows for a validated assessment of functional degree of immunosuppression or the identification of clinically relevant over- or under-immunosuppression, depending on graft homeostasis. Reliable non-invasive markers to predict biopsy proven acute rejection (BPAR) do not exist. Literature data suggest that longitudinal measurements of immune markers might be predictive of BPAR, but data in children are scarce.

Bi-nucleation of podocytes is uniformly accompanied by foot processes widening in renal disease.

Background: Podocytes are terminally differentiated glomerular cells expressing a highly complex architecture and lacking the ability to proliferate. However, during renal injury or stress these cells can re-enter into the cell cycle but fail to divide. As a consequence, bi- and multi-nucleated podocytes can be identified in renal biopsies from patients with various kidney diseases.

Vascular architecture as a diagnostic marker for differentiation of World Health Organization thymoma subtypes and thymic carcinoma.

Aims: Thymomas and thymic squamous cell carcinomas (TSQCCs) are rare thymic epithelial tumours. Data on angiogenesis and vascular phenotype in these tumours are limited, and no study has taken histological World Health Organization (WHO) subtypes into account. The aim of this study was to compare vascularization, pericytes coverage and expression of angiogenic growth factors in different WHO-defined subtypes of thymoma METHODS AND RESULTS: Vascular density, diameter and architecture and expression of α-smooth muscle actin (SMA), platelet-derived growth factor (PDGF) receptor-β (PDGFRβ), vascular endothelial growth factor (VEGF) receptor 1 (VEGFR1) and VEGF receptor 2 (VEGFR2) were investigated in WHO type A, AB, B1, B2 and B3 thymomas and TSQCCs, by the use of immunostaining, quantitative morphometry, and tumour vessel isolation by trypsin digestion.

Cell cycle re-entry sensitizes podocytes to injury induced death.

Podocytes are terminally differentiated renal cells, lacking the ability to regenerate by proliferation. However, during renal injury, podocytes re-enter into the cell cycle but fail to divide. Earlier studies suggested that re-entry into cell cycle results in loss of podocytes, but a direct evidence for this is lacking.

On the representation of cells in bone marrow pathology by a scalar field: propagation through serial sections, co-localization and spatial interaction analysis.

Background: Immunohistochemical analysis of cellular interactions in the bone marrow in situ is demanding, due to its heterogeneous cellular composition, the poor delineation and overlap of functional compartments and highly complex immunophenotypes of several cell populations (e.g. regulatory T-cells) that require immunohistochemical marker sets for unambiguous characterization.

Personal decision-making processes for living related liver transplantation in children.

Living related liver transplantation (LRLT) is a valuable transplant option for children with end-stage liver disease who face long waiting times on regular waiting lists. The subjection of a healthy adult to a potentially life-threatening operation can raise issues of freedom of choice, fear, and family conflict for the potential donors. We examined attitudes, fears, and influencing factors in the decision-making process for living liver donation for children in order to identify factors to improve support for living liver donors in the future.

Application and limitations of transient liver elastography in children.

Objectives: Transient elastography (TE) using the FibroScan has gained popularity recently for the noninvasive diagnosis of hepatic fibrosis. Data on its use in children younger than 6 years are still scarce, and the influence of technical aspects such as probe choice and site of measurement on FibroScan results is not clear. Our study aims to clarify some technical issues concerning the use of the FibroScan in children and to deliver normal FibroScan values for reference.

Normalization of serum bile acids after partial external biliary diversion indicates an excellent long-term outcome in children with progressive familial intrahepatic cholestasis.

Background/purpose: The surgical treatment for patients with progressive familial intrahepatic cholestasis (PFIC) is either liver transplantation (LTX) or partial external biliary diversion (PEBD). Both procedures achieve a good short-term outcome. However, the treatment strategy for these children remains controversial because the long-term outcome after PEBD is unknown.

Acceptance and adverse events of the 2009 H1N1 vaccination in immunosuppressed pediatric liver transplant recipients.

A retrospective analysis of H1N1 vaccination in 127 children at ≥ 1 year after liver transplantation found only moderate acceptance (56%) of the vaccination. Physical adverse events were of moderate severity, but frequent (74%). Protection against infection was good, with infection rates of 4% in vaccinated children versus 25% in nonvaccinated children.

Endoscopic retrograde cholangiopancreaticography prior to explorative laparotomy avoids unnecessary surgery in patients suspected for biliary atresia.

Background/aims: Timely diagnosis of biliary atresia (BA) requires key investigations that are less invasive but as accurate as possible. Non-invasive imaging preselects patients before explorative laparotomy is performed. The purpose of this prospective study was to evaluate the accuracy of endoscopic retrograde cholangiopancreaticography (ERCP) in these patients and to discuss its relevance to future diagnostic guidelines in neonatal jaundice.

Monitoring of adenovirus infection in pediatric transplant recipients by quantitative PCR: report of six cases and review of the literature.

Adenoviral (AdV) infections after transplantation remain a challenge in pediatric patients. Qualitative and quantitative PCR offer new approaches to early diagnosis and monitoring. However, their role in the management of AdV infections in pediatric transplant recipients remains to be determined.