415 research outputs found
Decreased endovascular trophoblast invasion in first trimester pregnancies with high-resistance uterine artery Doppler indices
No full textPancreatic tumours in children: diagnosis, treatment and outcome
No full textPurpose: Pancreatic tumours in children are exceedingly rare and as a result constitute a diagnostic and therapeutic challenge to paediatric surgeons. We reviewed our experience with these rare entities.Methods: Retrospective single institution study on all paediatric pancreatic tumours over a period of 38 years (1973-2011) and literature review. We recorded data relating to the clinical features at presentation, diagnostic evaluation, treatment and outcome.Results: Fourteen patients were identified (8 male). The most common symptoms at presentation were abdominal pain, anorexia and vomiting. Two cases were discovered incidentally. There were 12 primary and 2 metastatic tumours. Tumour types were Solid Pseudopapillary neoplasm (n=6), Insulinoma (n=3), Pancreatoblastoma (n=1), congenital pancreatic cyst(n=1), Burkitt lymphoma of the pancreas (n=1) and metastatic lesions of other primary tumours(n=2). Twelve were treated with surgical resection including 2 laparoscopically. Post-surgical complications included acute pancreatitis (n=2) and pancreatic pseudocyst (n=2). There was one death from a metastatic tumour and treatment is ongoing in one patient. The remainder have survived. Conclusion: Paediatric pancreatic tumours are rare entities and are usually benign. Clinical symptoms are often non-specific and presentation may be late due to tumour inactivity in case of endocrine neoplasms. For most tumours, surgical resection is the optimal treatment which may be successfully performed laparoscopically when the lesion is in the body or tail of the pancreas. Long term outcome is generally good.<br/
Diagnostic yield and safety of ultrasound-guided bowel mass biopsies in children
No full textBACKGROUND: Traditionally, ultrasound (US)-guided bowel mass biopsies are avoided in favour of endoscopic or surgical biopsies. However, endoscopy cannot easily reach lesions between the duodenojejunal flexure and the terminal ileum and lesions not involving the mucosa may not be accessible via an endoscopic route. OBJECTIVE: The aim of this study was to report our technique and to assess the diagnostic accuracy and safety of US-guided biopsy of bowel masses in children. MATERIALS AND METHODS: We conducted a 14-year retrospective review of US-guided bowel mass biopsies at a single paediatric hospital. RESULTS: Twenty US-guided bowel mass biopsies were performed in 19 patients (median age: 6 years and 6 months, range: 22 months-17 years, median weight: 22 kg, range: 10.2-48.4 kg). For 14 biopsies, there was no other lesion that could potentially be biopsied. A percutaneous coaxial technique was used for 19 biopsies and a transanal non-coaxial biopsy was performed in 1. A median of 9 (range: 2-15) cores of tissue was obtained at each biopsy. The technical success rate and adequacy of diagnostic yield were 100%. The most common diagnosis was lymphoma, which occurred in 16 biopsies. Three biopsies contained mucosa. There was one complication out of 20 biopsies (5%, 95% confidence interval 0-15%): a self-limiting, post biopsy pyrexia. Nineteen procedures were accompanied by a bone marrow aspirate and/or trephine within 2 weeks of the bowel biopsy, only one of which was diagnostic. CONCLUSION: US-guided bowel mass biopsy can be performed safely in children, with a high diagnostic yield and low complication rate.sponsorship: P.D.C. is supported by National Institute for Health Research. P.A.P. is supported by GIFT-SURG Wellcome Trust/ Engineering and Physical Sciences Research Council programme grant. This study was supported by the Great Ormond Street Hospital Children's Charity and the National Institute for Health Research Great Ormond Street Hospital Biomedical Research Centre. The views expressed are those of the author(s) and not necessarily those of the National Health Service, the National Institute for Health Research or the Department of Health in England. (National Institute for Health Research, GIFT-SURG Wellcome Trust/ Engineering and Physical Sciences Research Council programme grant, Great Ormond Street Hospital Children's Charity, National Institute for Health Research Great Ormond Street Hospital Biomedical Research Centre)status: Publishe
Variability of the transition zone length in Hirschsprung disease
No full textBackground: Surgical management of Hirschsprung disease (HD) involves fully excising the transition zone (TZ). The literature suggests that resection of ≥ 5 cm of ganglionic bowel proximal to the aganglionic segment is sufficient. Our primary aim was to evaluate the lengths of the TZ in a cohort of consecutive patients with HD. We reviewed the impact this had on the need for revision surgery. We hypothesized that the TZ can be highly variable and may lead to a TZ pull-through when the proximal donut is not reviewed intraoperatively. Methods: A retrospective review was conducted for all patients undergoing primary pull-through surgery between January 2012 and September 2018. Data was collected on demographics, need for staged surgery, and complications following surgery. Results: Forty-eight patients were eligible for inclusion. 11/48 (23%) patients presented late (> 6 months). 27/48 (56%) patients needed a stoma prior to definitive surgery. The median age at pull-through was 6 months (1–84 months). The median TZ length was 1.7 cm (0.3–22.9 cm). 11/48 (23%) had a TZ > 5 cm. 36/48 (75%) patients did not have intraoperative review of the donut resulting in three TZ pull-throughs. Conclusions: We would advocate circumferential intraoperative frozen section review of the proximal donut to minimize the risk of a TZ pull-through. Level of Evidence: Level II
Clusterin, a haploinsufficient tumor suppressor gene in neuroblastomas
No full textThis article is available open access through the publisher’s website. Copyright @ 2009 The Authors.Background - Clusterin expression in various types of human cancers may be higher or lower than in normal tissue, and clusterin may promote or inhibit apoptosis, cell motility, and inflammation. We investigated the role of clusterin in tumor development in mouse models of neuroblastoma. Methods - We assessed expression of microRNAs in the miR-17-92 cluster by real-time reverse transcription–polymerase chain reaction in MYCN-transfected SH-SY5Y and SH-EP cells and inhibited expression by transfection with microRNA antisense oligonucleotides. Tumor development was studied in mice (n = 66) that were heterozygous or homozygous for the MYCN transgene and/or for the clusterin gene; these mice were from a cross between MYCN-transgenic mice, which develop neuroblastoma, and clusterin-knockout mice. Tumor growth and metastasis were studied in immunodeficient mice that were injected with human neuroblastoma cells that had enhanced (by clusterin transfection, four mice per group) or reduced (by clusterin short hairpin RNA [shRNA] transfection, eight mice per group) clusterin expression. All statistical tests were two-sided. Results - Clusterin expression increased when expression of MYCN-induced miR-17-92 microRNA cluster in SH-SY5Y neuroblastoma cells was inhibited by transfection with antisense oligonucleotides compared with scrambled oligonucleotides. Statistically significantly more neuroblastoma-bearing MYCN-transgenic mice were found in groups with zero or one clusterin allele than in those with two clusterin alleles (eg, 12 tumor-bearing mice in the zero-allele group vs three in the two-allele group, n = 22 mice per group; relative risk for neuroblastoma development = 4.85, 95% confidence interval [CI] = 1.69 to 14.00; P = .005). Five weeks after injection, fewer clusterin-overexpressing LA-N-5 human neuroblastoma cells than control cells were found in mouse liver or bone marrow, but statistically significantly more clusterin shRNA-transfected HTLA230 cells (3.27%, with decreased clusterin expression) than control-transfected cells (1.53%) were found in the bone marrow (difference = 1.74%, 95% CI = 0.24% to 3.24%, P = .026). Conclusions - We report, to our knowledge, the first genetic evidence that clusterin is a tumor and metastasis suppressor gene.Sport Aiding Medical Research for Kids (SPARKS), Great Ormond Street
Hospital/National Health Service, the National
Cancer Institute and University of Parma
Placenta as a reservoir of stem cells: An underutilized resource?
No full textIntroduction: Both embryonic and adult tissues are sources of stem cells with therapeutic potential but with some limitations in the clinical practice such as ethical considerations, difficulty in obtaining and tumorigenicity. As an alternative, the placenta is a foetal tissue that can be obtained during gestation and at term, and it represents a reservoir of stem cells with various potential.
Sources of data: We reviewed the relevant literature concerning the main stem cells that populate the placenta.
Areas of agreement: Recently, the placenta has become useful source of stem cells that offer advantages in terms of proliferation and plasticity when compared with adult cells and permit to overcome the ethical and safety concern inherent in embryonic stem cells. In addition, the placenta has the advantage of containing epithelia, haematopoietic and mesenchymal stem cells. These stem cells possess immunosuppressive properties and have the capacity of suppress in vivo inflammatory responses.
Areas of controversy: Some studies describe a subpopulation of placenta stem cells expressing pluripotency markers, but for other studies, it is not clear whether pluripotent stem cells are present during gestation beyond the first few weeks. Particularly, the expression of some pluripotency markers such as SSEA-3, TRA-1-60 and TRA-1-81 has been reported by us, but not by others.
Growing points: Placenta stem cells could be of great importance after delivery for banking for autologous and allogeneic applications. The beneficial effects of these cells may be due to secretion of bioactive molecules that act through paracrine actions promoting beneficial effects.
Areas timely for developing research: Understanding the role of placenta stem cells during pregnancy and their paracrine actions could help in the study of some diseases that affect the placenta during pregnancy
Process evaluation of the Teamplay parenting intervention pilot : implications for recruitment, retention and course refinement
No full textBackground
Parenting programs could provide effective routes to increasing children’s physical activity and reducing screen-viewing. Many studies have reported difficulties in recruiting and retaining families in group parenting interventions. This paper uses qualitative data from the Teamplay feasibility trial to examine parents’ views on recruitment, attendance and course refinement.
Methods
Semi-structured interviews were conducted with 16 intervention and 10 control group parents of 6–8 year old children. Topics discussed with the intervention group included parents’ views on the recruitment, structure, content and delivery of the course. Topics discussed with the control group included recruitment and randomization. Interviews were digitally recorded, transcribed and thematically analyzed.
Results
Many parents in both the intervention and control group reported that they joined the study because they had been thinking about ways to improve their parenting skills, getting ideas on how to change behavior, or had been actively looking for a parenting course but with little success in enrolling on one. Both intervention and control group parents reported that the initial promotional materials and indicative course topics resonated with their experiences and represented a possible solution to parenting challenges. Participants reported that the course leaders played an important role in helping them to feel comfortable during the first session, engaging anxious parents and putting parents at ease. The most commonly reported reason for parents returning to the course after an absence was because they wanted to learn new information. The majority of parents reported that they formed good relationships with the other parents in the group. An empathetic interaction style in which leaders accommodated parent’s busy lives appeared to impact positively on course attendance.
Conclusions
The data presented indicate that a face-to-face recruitment campaign which built trust and emphasized how the program was relevant to families positively affected recruitment in Teamplay. Parents found the parenting component of the intervention attractive and, once recruited, attendance was facilitated by enjoyable sessions, empathetic leaders and support from fellow participants. Overall, data suggest that the Teamplay recruitment and retention approaches were successful and with small refinements could be effectively used in a larger trial
Clinico-pathological correlations of congenital and infantile nephrotic syndrome over twenty years
No full textBACKGROUND:
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 before one year of age between 1990 and 2009. All subjects had a renal biopsy, which was independently blindly reviewed by a single renal pathologist for the purpose of this study.
RESULTS:
Forty-nine children (25 female) who presented at 0.1-11.6 (median 1.6) months were included with 31 presenting within the first three months of life. Histopathological review diagnostic categories were; 13 Mesangial proliferative glomerulopathy (MesGN), 12 Focal and segmental glomerulosclerosis (FSGS), 11 Finnish type changes, eight Diffuse Mesangial Sclerosis (DMS), three Minimal change disease (MCD) and one each of Dense Deposit Disease (DDD) and Membranous nephropathy. Two children died from haemorrhagic complications of the biopsy. Eight children achieved remission (four MesGN, one Finnish type changes, one FSGS, one MCD and one membranous) with patient and renal survival of 73 % and 43 %, respectively, at follow-up duration of 5-222 (median 73) months (with five lost to follow-up). All children with Finnish-type histopathological changes presented within five months of age. Due to the historical nature of the cohort, genetic testing was only available for 14 children, nine of whom had an identifiable genetic basis (seven NPHS1, one PLCE1 and one ITGA3) with none of these nine children achieving remission. All of them had presented within four months of age and required renal replacement therapy, and two died.
CONCLUSIONS:
Histopathological findings are varied in children presenting with NS early in life. Whilst groups of histological patterns of disease are associated with differing outcomes, accurate prediction of disease course in a specific case is difficult and more widespread genetic testing may improve the understanding of this group of diseases and their optimal management
- …
