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28 papers found.
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Clinical risk stratification of paediatric renal transplant recipients using C1q and C3d fixing of de novo donor-specific antibodies

Introduction We have previously shown that children who developed de novo donor-specific human leukocyte antigen (HLA) antibodies (DSA) had greater decline in allograft function. We hypothesised that patients with complement-activating DSA would have poorer renal allograft outcomes. Methods A total of 75 children developed DSA in the original study. The first positive DSA sample...

Current issues in postmortem imaging of perinatal and forensic childhood deaths

Perinatal autopsy practice is undergoing a state of change with the introduction of evidence-based cross-sectional imaging, driven primarily by parental choice. In particular, the introduction of post mortem magnetic resonance imaging (PMMR) has helped to advance less-invasive perinatal autopsy in the United Kingdom (UK) and Europe. However, there are limitations to PMMR and...

An optimised method for the proteomic profiling of full thickness human skin

Background The skin is the largest organ of the human body and is the first line barrier defence against trauma, microbial infiltration and radiation. Skin diseases can be a result of multi-systemic disease or an isolated condition. Due to its proteolysis resistant properties there are relatively few human skin proteomic datasets published compared with other human organs or body...

Abnormal villous morphology mimicking a hydatidiform mole associated with paternal trisomy of chromosomes 3,7,8 and unipaternal disomy of chromosome 11

Background Pregnancies affected by non-molar chromosomal abnormality may sometimes demonstrate abnormal chorionic villous morphology that is similar to partial hydatidiform mole. Determination of the underlying aetiology may be difficult in such cases. Case Presentation This report describes a case referred to the regional trophoblastic disease unit as a possible hydatidiform...

Desensitization protocol enabling pediatric crossmatch-positive renal transplantation: successful HLA-antibody-incompatible renal transplantation of two highly sensitized children

Background Renal transplantation improves quality of life (QoL) and survival in children requiring renal replacement therapy (RRT). Sensitization with development of a broad-spectrum of anti-HLA antibodies as a result of previous transplantation or after receiving blood products is an increasing problem. There are no published reports of desensitization protocols in children...

Postmortem cardiac imaging in fetuses and children

Fetal and pediatric cardiac autopsies have a crucial role in the counseling of parents with regard to both the cause of death of their child and the implications of such findings for future pregnancies, as well as for quality assurance of antenatal screening programs and antemortem diagnostic procedures. Postmortem imaging allows an opportunity to investigate the heart in situ...

Indications, advantages and limitations of perinatal postmortem imaging in clinical practice

Just as there is a range of paediatric imaging techniques available during life, a similar repertoire is available as part of the foetal and perinatal postmortem examination. In this article, we review the literature regarding the diagnostic utility of postmortem radiography, US, CT and MRI in this clinical setting. There is limited direct evidence on the diagnostic utility of...

Normal perinatal and paediatric postmortem magnetic resonance imaging appearances

As postmortem imaging becomes more widely used following perinatal and paediatric deaths, the correct interpretation of images becomes imperative, particularly given the increased use of postmortem magnetic resonance imaging. Many pathological processes may have similar appearances in life and following death. A thorough knowledge of normal postmortem changes is therefore...

Oncology: histopathology and imaging in the future

Neil J. Sebire 0 ) Great Ormond Street Hospital for Children , Great Ormond Street, London WC1N 3JH, UK - The established gold standard for many diagnoses, in particular the diagnosis of

Optimization of Liver Decellularization Maintains Extracellular Matrix Micro-Architecture and Composition Predisposing to Effective Cell Seeding

Hepatic tissue engineering using decellularized scaffolds is a potential therapeutic alternative to conventional transplantation. However, scaffolds are usually obtained using decellularization protocols that destroy the extracellular matrix (ECM) and hamper clinical translation. We aim to develop a decellularization technique that reliably maintains hepatic microarchitecture and...

Lung aeration on post-mortem magnetic resonance imaging is a useful marker of live birth versus stillbirth

Objective Aim of this study was to investigate whether lung assessment on post-mortem magnetic resonance imaging (PMMR) can reliably differentiate between live birth and stillbirth. Materials and methods We retrospectively assessed PMMR imaging features of a group of late foetal terminations following fetocide and stillbirths (without witnessed breathing) and early infant deaths...

Postmortem magnetic resonance appearances of congenital high airway obstruction syndrome

Scientist Fellow award, and Neil J Sebire is funded by an NIHR Senior Investigator award, partially supported by the Great Ormond Street Childrens Charity, the Higher Education Funding Council for England and

Paediatric and perinatal postmortem imaging: the need for a subspecialty approach

. Arthurs is funded by an NIHR Clinician Scientist Fellow award, and Andrew M. Taylor is funded by an NIHR Senior Research Fellow award and by the Foundation Leducq. Neil J. Sebire is Neil J Sebire is funded

Diffusion-weighted perinatal postmortem magnetic resonance imaging as a marker of postmortem interval

Objective To evaluate perinatal body organ apparent diffusion coefficient (ADC) values at postmortem magnetic resonance imaging (PMMR) in order to evaluate postmortem changes. Methods Postmortem diffusion-weighted imaging (DWI) of the thorax and abdomen were performed with diffusion gradient values b = 0, 500, and 1000 s/mm2 on 15 foetal and childhood cases (mean 33.3 ± 7.8 weeks...

TP53 Mutational Status Is a Potential Marker for Risk Stratification in Wilms Tumour with Diffuse Anaplasia

Purpose The presence of diffuse anaplasia in Wilms tumours (DAWT) is associated with TP53 mutations and poor outcome. As patients receive intensified treatment, we sought to identify whether TP53 mutational status confers additional prognostic information. Patients and Methods We studied 40 patients with DAWT with anaplasia in the tissue from which DNA was extracted and analysed...

Comparative methylome analysis identifies new tumour subtypes and biomarkers for transformation of nephrogenic rests into Wilms tumour

Background Wilms tumours (WTs) are characterised by several hallmarks that suggest epimutations such as aberrant DNA methylation are involved in tumour progression: loss of imprinting at 11p15, lack of recurrent mutations and formation of nephrogenic rests (NRs), which are lesions of retained undifferentiated embryonic tissue that can give rise to WTs. Methods To identify such...

Methylome analysis identifies a Wilms tumor epigenetic biomarker detectable in blood

Background Wilms tumor is the most common pediatric renal malignancy and there is a clinical need for a molecular biomarker to assess treatment response and predict relapse. The known mutated genes in this tumor type show low mutation frequencies, whereas aberrant methylation at 11p15 is by far the most common aberration. We therefore analyzed the epigenome, rather than the...

Diagnostic accuracy of post-mortem MRI for thoracic abnormalities in fetuses and children

Objectives To compare the diagnostic accuracy of post-mortem magnetic resonance imaging (PMMR) specifically for non-cardiac thoracic pathology in fetuses and children, compared with conventional autopsy. Methods Institutional ethics approval and parental consent was obtained. A total of 400 unselected fetuses and children underwent PMMR before conventional autopsy, reported...

Clinico-pathological correlations of congenital and infantile nephrotic syndrome over twenty years

Background Nephrotic syndrome (NS) presenting early in life is caused by heterogeneous glomerular diseases. We retrospectively evaluated whether histological diagnosis in children presenting with NS in the first year of life predicts remission or progression to end-stage kidney disease (ESKD). Methods This is a single centre retrospective review of all children diagnosed with NS...

Reconstruction of fetal and infant anatomy using rapid prototyping of post-mortem MR images

Objectives The recent decline in autopsy rates and lack of human anatomical material donated for research and training has resulted in issues for medical training in the United Kingdom. This study aims to examine the feasibility of making accurate three-dimensional (3D) models of the human body and visceral organs using post-mortem magnetic resonance (MR) imaging and rapid...