438 research outputs found
Decreased endovascular trophoblast invasion in first trimester pregnancies with high-resistance uterine artery Doppler indices
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Pancreatic 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/
Supplemental Material - Rapid desensitization through immunoadsorption during cardiopulmonary bypass. A novel method to facilitate human leukocyte antigen incompatible heart transplantation
No full textSupplemental Material for Rapid desensitization through immunoadsorption during cardiopulmonary bypass. A novel method to facilitate human leukocyte antigen incompatible heart transplantation by Richard W Issitt, Eamonn Cudworth, Mario Cortina-Borja, Arun Gupta, Delordson Kallon, Richard Crook, Michael Shaw, Alex Robertson, Victor T Tsang, Sophie Henwood7, Vivek Muthurangu, Neil J Sebire, Michael Burch and Matthew Fenton in Perfusion</p
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
Germline melanocortin-1-receptor genotype is associated with severity of cutaneous phenotype in congenital melanocytic nevi: a role for MC1R in human fetal development
No full textCongenital melanocytic nevi (CMN) are pigmented birthmarks that affect up to 80% of the skin surface area. The increased frequency of CMN in families of severely affected individuals is suggestive of a predisposing germline genotype. We noted a high prevalence of red hair in affected families, and considered a role for MC1R in this condition. A cohort of 166 CMN subjects underwent pigmentary phenotyping, with MC1R genotyping in 113. Results were compared with a local control group of 60 unrelated children and with 300 UK children without CMN. CMN subjects had higher prevalences of red hair and a red-haired parent than local controls and had a higher rate of compound heterozygosity and homozygosity for MC1R variants. The presence of a V92M or R allele (D84E, R151C, R160W, D294H) was associated with increasing size of the CMN, implying a growth-promoting effect of these alleles. Unexpectedly, the V92M and R151C alleles were also strongly associated with birth weight in the CMN cohort, a finding confirmed in the control group. The effect of germline MC1R genotype on development and severity of CMN led us to investigate potential broader effects on growth, revealing a role for MC1R in normal fetal development.Journal of Investigative Dermatology advance online publication, 10 May 2012; doi:10.1038/jid.2012.95
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
Outcomes of paediatric kidney transplant recipients using the updated 2013/2017 Banff histopathological classification for antibody-mediated rejection
No full textBackground: After the major changes with regard to acute and chronic ABMR in the Banff classification initiated in 2013, there has been an improvement in diagnosing antibody-mediated rejection (ABMR) in adult studies but no data have been published in the paediatric population. Methods: We assessed 56 paediatric kidney transplant biopsies due to kidney dysfunction in patients with donor-specific antibodies (DSA) in a retrospective single-centre study between January 2006 and March 2012. The results were compared with 2003/2007 Banff classification noting the subsequent 2017 and 2019 modifications do not change the 2013 Banff classification with regard to acute antibody-mediated rejection (apart from the addition of gene transcripts/classifiers that do not affect our analysis). Results: Following the 2013 Banff classification, there were seven cases (12.5%) diagnosed with ABMR that would have been misclassified when applying the 2003/2007 classification. Evaluating the histological features of all ABMR-related cases, we report the importance of v− (intimal arteritis) and t− (tubulitis) lesions: absence of v− and t− lesions in the biopsy is related to significantly higher kidney allograft survival (OR 7.3, 95%CI 1.1–48.8, p = 0.03 and OR 5.3, 95%CI 1.2–25.5, p = 0.04 respectively). Moreover, absence of t− lesions was associated with significantly fewer rejection episodes the year after the initial biopsy (OR 5.1, 95%CI 1.4–19.8, p = 0.01). Conclusions: Our study supports that the updated 2013 Banff classification shows superior clinicopathological correlation in identifying ABMR in paediatric kidney transplant recipients. Our results can be extrapolated to the recently updated 2019 Banff classification.</p
Intussusception and COVID-19 in infants: evidence for an etiopathologic correlation
No full textNonrespiratory conditions related to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections have been largely described. Ileocolic intussusception has been reported in association with SARS-CoV-2 infection in 10 children, raising the possibility of an etiopathologic role for the virus, but none of these cases documented tissue pathology that would have supported SARS-CoV-2 intestinal inflammation. We report 2 cases of intussusception in patients with SARS-CoV-2 infection who were treated at different pediatric tertiary centers in Europe and provide evidence of the presence of the virus in mesenteric and intestinal tissues of the patients
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