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Pediatric liver pathology

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https://www.readbyqxmd.com/read/28717867/totally-laparoscopic-living-donor-left-hepatectomy-for-liver-transplantation-in-a-child
#1
Helayel Almodhaiberi, Seok-Hwan Kim, Ki-Hun Kim
BACKGROUND: Minimally invasive surgery has been validated to be a new standard in living donor hepatectomy for adult-to-pediatric transplantation with less morbidity [1]. Laparoscopic donor hepatectomy can reduce the major concerns about pain and morbidity associated with open surgery and a slow return to daily activities of donors [2]. Herein, we present one case of totally laparoscopic living donor left hepatectomy including the middle hepatic vein (MHV). DONOR AND METHOD: A 37-year-old mother volunteered to donate to her 3-year-old son with biliary atresia (PELD score 7)...
July 17, 2017: Surgical Endoscopy
https://www.readbyqxmd.com/read/28714114/split-liver-transplantation-for-retroperitoneal-immature-teratoma-masquerading-as-hepatoblastoma
#2
Dongkyu Oh, Nam-Joon Yi, Sanghee Song, Ok Kyung Kim, Suk Kyun Hong, Kyung Chul Yoon, Sung Woo Ahn, Hyo-Sin Kim, Hyeyoung Kim, Hyun-Young Kim, Hyoung Jin Kang, Myungsu Lee, Kyoung Bun Lee, Kwang-Woong Lee, Kyung Suk Suh
Extragonadal teratoma originating from the retroperitoneum represents less than 5% of all teratomas and accounts for less than 10% of all pediatric retroperitoneal neoplasms. To date, there has been no report of teratoma managed with LT. This study reports an infant aged 3 months with retroperitoneal immature teratoma involving the hepatic hilum, refractory to chemotherapy and treated with LT. The patient was referred to our hospital for management of a growing abdominal mass. Histopathology of a fine needle biopsy of the lesion suggested the possibility of a hepatoblastoma with teratoid features...
July 16, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28708813/application-and-interpretation-of-histocompatibility-data-in-liver-transplantation
#3
Russell N Wesson, Eric W Etchill, Jacqueline Garonzik-Wang
PURPOSE OF REVIEW: There has been a resurgence of interest in histocompatibility as it applies to liver transplantation. The association of persistent and de-novo donor specific antibody (DSA) and outcomes after liver transplantation continues to be investigated. RECENT FINDINGS: Consensus continues to evolve regarding the existence of acute and chronic antibody-mediated rejection (AMR) and pathogenicity of DSA and associated pathologic findings after liver transplantation...
July 13, 2017: Current Opinion in Organ Transplantation
https://www.readbyqxmd.com/read/28689245/imaging-of-renal-medullary-carcinoma-in-children-and-young-adults-a-report-from-the-children-s-oncology-group
#4
Jesse K Sandberg, Elizabeth A Mullen, Mariana M Cajaiba, Ethan A Smith, Sabah Servaes, Elizabeth J Perlman, James I Geller, Peter F Ehrlich, Nicholas G Cost, Jeffrey S Dome, Conrad V Fernandez, Geetika Khanna
BACKGROUND: Renal medullary carcinoma is a rare renal malignancy of childhood. There are no large series describing the imaging appearance of renal medullary carcinoma in children. OBJECTIVE: To characterize the clinical and imaging features of pediatric renal medullary carcinoma at initial presentation. MATERIALS AND METHODS: We retrospectively analyzed images of 25 pediatric patients with renal medullary carcinoma enrolled in the Children's Oncology Group renal tumors classification, biology and banking study (AREN03B2) from March 2006 to August 2016...
July 8, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28675362/liver-resections-in-a-high-volume-center-form-standard-procedures-to-extreme-surgery-and-ultrasound-guided-resections
#5
Florin Botea, Mihnea Ionescu, Vladislav Braşoveanu, Doina Hrehoreţ, Sorin Alexandrescu, Mihai Grigorie, Oana Stanciulea, Diana Nicolaescu, Dana Tomescu, Gabriela Droc, Daniela Ungureanu, Ruxandra Fota, Adina Croitoru, Liana Gheorghe, Cristian Gheorghe, Ioana Lupescu, Mugur Grasu, Mirela Boroş, Radu Dumitru, Mihai Toma, Vlad Herlea, Irinel Popescu
Background: Liver resection (LR) is the treatment of choice for most benign and malignant focal liver lesions, as well as in selected patients with liver trauma. Few other therapies can compete with LR in selected cases, such as liver transplantation in hepatocellular carcinoma (HCC) and ablative therapies in small HCCs or liver metastases. The present paper analyses a single center experience in LR, reviewing the indications of LR, the operative techniques and their short-term results. MATERIAL AND METHOD: Between January 2000 and December 2016, in "œDan Setlacec" Center of General Surgery and Liver Transplantation were performed 3165 LRs in 3016 patients, for pathologic conditions of the liver...
May 2017: Chirurgia
https://www.readbyqxmd.com/read/28632515/p4-stump-approach-for-intraoperative-portal-vein-stenting-in-pediatric-living-donor-liver-transplantation-an-innovative-technique-for-a-challenging-problem
#6
Chao-Long Chen, Yu-Fan Cheng, Viola Huang, Ting-Lung Lin, Yi-Chia Chan, Hsin-You Ou, Chee-Chien Yong, Shih-Ho Wang, Chih-Che Lin
OBJECTIVE: The aim of this study was to evaluate the utility of the P4 stump stenting approach for treating portal vein (PV) complications in pediatric living donor liver transplantation (LDLT). BACKGROUND: PV complications cause significant morbidity and mortality in pediatric LDLT. Biliary atresia in the backdrop of pathological PV hypoplasia and sclerosis heightens the complexity of PV reconstruction. The authors developed a novel approach for intraoperative PV stenting via the graft segment 4 PV stump (P4 stump) to address this challenge...
June 19, 2017: Annals of Surgery
https://www.readbyqxmd.com/read/28586157/role-of-point-shear-wave-elastography-in-the-determination-of-the-severity-of-fibrosis-in-pediatric-liver-diseases-with-pathologic-correlations
#7
Mehmet Burak Özkan, Meltem Ceyhan Bilgici, Esra Eren, Gonul Caltepe, Gulay Yilmaz, Cengiz Kara, Seda Gun
OBJECTIVES: Our aims in this study were as follows: (1) to determine the cutoff value that can distinguish between advanced liver fibrosis and normal liver tissue for two different elastographic techniques; (2) to determine the cutoff value that can distinguish mild liver fibrosis from normal liver tissue for the techniques; and (3) to assess tissue stiffness in nonalcoholic fatty liver disease (NAFLD). METHODS: Seventy-five patients assessed for liver biopsy on the same day were evaluated by point shear wave elastography...
June 6, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/28575039/assessment-of-pathologic-increase-in-liver-stiffness-enables-earlier-diagnosis-of-cfld-results-from-a-prospective-longitudinal-cohort-study
#8
Victoria Klotter, Caroline Gunchick, Enno Siemers, Timo Rath, Helge Hudel, Lutz Naehrlich, Martin Roderfeld, Elke Roeb
About 30% of patients with Cystic Fibrosis (CF) develop CF-associated liver disease (CFLD). Recent studies have shown that transient elastography (TE), as a method to quantify liver stiffness, allows non-invasive diagnosis of CFLD in adults and children with CF. Within this study we aimed to prospectively identify patients at risk for development of CFLD by longitudinal analysis of liver stiffness and fibrosis scores in a 5-year follow-up. 36 pediatric and 16 adult patients with initial liver stiffness below the cut-off value indicative of CFLD (6...
2017: PloS One
https://www.readbyqxmd.com/read/28540988/pathologic-impact-of-insulin-resistance-and-sensitivity-on-the-severity-of-liver-histopathology-in-pediatric-non-alcoholic-steatohepatitis
#9
Byung Han Park, Jung Min Yoon, Ja Hye Kim, Jin Hwa Moon, Young Ho Lee, Se Min Jang, Yong Joo Kim
PURPOSE: Insulin resistance (IR) has an important role in the development of non-alcoholic steatohepatitis (NASH). We aimed to analyze the association between liver histopathology and IR in pediatric patients with NASH. MATERIALS AND METHODS: In 24 children with non-alcoholic fatty liver disease (NAFLD), we investigated whether the hepatic pathologic characteristics have relations with following three biochemical indices; IR index including homeostasis model assessment of IR (HOMA-IR), quantitative insulin sensitivity check index (QUICKI), and insulin sensitivity indices-free fatty acid (ISI-FFA)...
July 2017: Yonsei Medical Journal
https://www.readbyqxmd.com/read/28492501/urinary-metabolomics-in-pediatric-obesity-and-nafld-identifies-metabolic-pathways-metabolites-related-to-dietary-habits-and-gut-liver-axis-perturbations
#10
Jacopo Troisi, Luca Pierri, Annamaria Landolfi, Francesca Marciano, Antonella Bisogno, Federica Belmonte, Carmen Palladino, Salvatore Guercio Nuzio, Pietro Campiglia, Pietro Vajro
To get insight into still elusive pathomechanisms of pediatric obesity and non-alcoholic fatty liver disease (NAFLD) we explored the interplay among GC-MS studied urinary metabolomic signature, gut liver axis (GLA) abnormalities, and food preferences (Kid-Med). Intestinal permeability (IP), small intestinal bacterial overgrowth (SIBO), and homeostatic model assessment-insulin resistance were investigated in forty children (mean age 9.8 years) categorized as normal weight (NW) or obese (body mass index <85th or >95th percentile, respectively) ± ultrasonographic bright liver and hypertransaminasemia (NAFLD)...
May 11, 2017: Nutrients
https://www.readbyqxmd.com/read/28482388/-a-follow-up-report-of-childhood-hepatoblastoma-from-74-cases-in-a-single-center
#11
T Y Wang, C Pan, J Y Tang, Q D Ye, M Zhou, Y J Gao, W T Hu
Objective: To investigate the efficacy and the prognostic factors in pediatric hepatoblastoma according to the standard diagnostic and therapeutic regimen. Method: Eighty-four consecutive patients were enrolled in this study between June 2000 and June 2015. Diagnosis and staging was decided by the multi-disciplinary team including oncologists, surgeons, pathologists and sub-specialized radiologists refering to protocol of Children's Oncology Group(COG) and International Society of Pediatric Oncology Liver Tumor Study Group (SIOPEL) in a case observational study...
May 4, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28459463/mycn-induces-neuroblastoma-in-primary-neural-crest-cells
#12
R R Olsen, J H Otero, J García-López, K Wallace, D Finkelstein, J E Rehg, Z Yin, Y-D Wang, K W Freeman
Neuroblastoma (NBL) is an embryonal cancer of the sympathetic nervous system (SNS), which causes 15% of pediatric cancer deaths. High-risk NBL is characterized by N-Myc amplification and segmental chromosomal gains and losses. Owing to limited disease models, the etiology of NBL is largely unknown, including both the cell of origin and the majority of oncogenic drivers. We have established a novel system for studying NBL based on the transformation of neural crest cells (NCCs), the progenitor cells of the SNS, isolated from mouse embryonic day 9...
May 1, 2017: Oncogene
https://www.readbyqxmd.com/read/28439217/pediatric-metabolic-syndrome-pathophysiology-and-laboratory-assessment
#13
Victoria Higgins, Khosrow Adeli
Pediatric overweight and obesity is an emerging public health priority as rates have rapidly increased worldwide. Obesity is often clustered with other metabolic abnormalities including hypertension, dyslipidemia, and insulin resistance, leading to increased risk of cardiovascular disease. This cluster of risk factors, termed the metabolic syndrome, has traditionally been reported in adults. However, with the increased prevalence of pediatric obesity, the metabolic syndrome is now evident in children and adolescents...
March 2017: EJIFCC
https://www.readbyqxmd.com/read/28417472/evaluation-of-liver-kidney-and-spleen-elasticity-in-healthy-newborns-and-infants-using-shear-wave-elastography
#14
Figen Bakirtas Palabiyik, Ercan Inci, Rustu Turkay, Derya Bas
OBJECTIVES: Elasticity measurements of tissues can be valuable in the diagnosis and management of various diseases. The aim of this study was to determine the elasticity values for normal liver, kidney, and spleen of healthy newborns and infants using shear wave elastography (SWE) imaging. METHODS: A total of 50 healthy term newborns and infants (19 girls and 31 boys; mean age 20.1 days, range 1 to 70 days) were examined by an experienced pediatric radiologist using SWE...
April 18, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/28394846/ultrasound-guided-liver-biopsies-in-children-a-single-center-experience-and-risk-factors-for-minor-bleeding
#15
Carlos Bilreiro, Maria José Noruegas, Isabel Gonçalves, Ângela Moreira
OBJECTIVES: To analyze the institution's experience in ultrasound-guided liver biopsies performed on children and identify risk factors for complications, following a previous study performed in our institution. METHODS: retrospective analysis of a consecutive series of ultrasound-guided pediatric liver biopsies, between 2011 and 2016. Demographic and anthropometric data, biopsy indications, INR and platelet count, biopsy technique, complications and pathologic outcomes were recorded...
April 8, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28333140/metabolic-and-molecular-insights-into-an-essential-role-of-nicotinamide-phosphoribosyltransferase
#16
Li Q Zhang, Leon Van Haandel, Min Xiong, Peixin Huang, Daniel P Heruth, Charlie Bi, Roger Gaedigk, Xun Jiang, Ding-You Li, Gerald Wyckoff, Dmitry N Grigoryev, Li Gao, Linheng Li, Min Wu, J Steven Leeder, Shui Qing Ye
Nicotinamide phosphoribosyltransferase (NAMPT) is a pleiotropic protein implicated in the pathogenesis of acute respiratory distress syndrome, aging, cancer, coronary heart diseases, diabetes, nonalcoholic fatty liver disease, obesity, rheumatoid arthritis, and sepsis. However, the underlying molecular mechanisms of NAMPT in these physiological and pathological processes are not fully understood. Here, we provide experimental evidence that a Nampt gene homozygous knockout (Nampt(-/-)) resulted in lethality at an early stage of mouse embryonic development and death within 5-10 days in adult mice accompanied by a 25...
March 23, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/28302000/expanded-criteria-for-hepatocellular-carcinoma-in-liver-transplant
#17
Mehmet Haberal, Aydıncan Akdur, Gökhan Moray, Gülnaz Arslan, Figen Özçay, Haldun Selçuk, Handan Özdemir
OBJECTIVES: Hepatocellular carcinoma is the sixth most common cancer worldwide and is the third highest cause of malignancy-related death. Because of its typically late diagnosis, median survival is approximately 6 to 20 months, with 5-year survival of < 12%. Hepatocellular carcinoma typically arises in the background of cirrhosis, with liver transplant regarded as the optimal therapy for selected patients. Initially, orthotopic liver transplant was limited to patients with extensive unresectable tumors, resulting in uniformly dismal outcomes due to high tumor recurrence rates...
March 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28296681/adenovirus-hepatitis-clinicopathologic-analysis-of-12-consecutive-cases-from-a-single-institution
#18
Kurt B Schaberg, Neeraja Kambham, Richard K Sibley, John P T Higgins
Adenoviruses are common pathogens that usually cause self-limited infections. However, in the immunocompromised host they can cause severe infections involving multiple organs including the liver. A search of the pathology database at Stanford University Medical Center (1995 to 2016) identified 12 cases of adenovirus hepatitis including biopsy and autopsy specimens. There were 8 pediatric patients, 7 of which had received orthotropic liver transplants and 1 of which was receiving chemotherapy for lymphoblastic leukemia...
June 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28214296/abnormally-high-and-heterogeneous-bone-matrix-mineralization-after-childhood-solid-organ-transplantation-a-complex-pathology-of-low-bone-turnover-and-local-defects-in-mineralization
#19
Nadja Fratzl-Zelman, Helena Valta, Renata C Pereira, Barbara M Misof, Paul Roschger, Hannu Jalanko, Katherine Wesseling-Perry, Klaus Klaushofer, Outi Mäkitie
Chronic renal, liver, and heart failure in children associates with multiple skeletal complications. Increased fracture incidence often persists after transplantation and could be related to alterations in bone material properties. In the present cohort study we evaluated bone mineralization density distribution (BMDD) by quantitative backscattered electron imaging (qBEI) in 23 pediatric solid organ allograft recipients with suspected osteoporosis. We measured BMDD in the entire cross-sectional area of transiliac bone biopsies obtained from kidney (n = 9), liver (n = 9), and heart (n = 5) transplant recipients (aged 7...
May 2017: Journal of Bone and Mineral Research: the Official Journal of the American Society for Bone and Mineral Research
https://www.readbyqxmd.com/read/28124483/mutations-in-ttc21b-cause-different-phenotypes-in-two-childhood-cases-in-china
#20
Hongwen Zhang, Baige Su, Xiaoyu Liu, Huijie Xiao, Jie Ding, Yong Yao
AIM: The TTC21B gene is now known as causative of nephronophthisis-related ciliopathies (NPHP-RC). We reported two Chinese pediatric cases with end-stage renal disease and other phenotypes caused by the TTC21B gene mutations. METHODS: The clinical features of Chinese pediatric cases with NPHP-RC were summarized. Mutation analysis of the TTC21B gene was performed using next-generation sequencing. RESULTS: The two cases both had nephrotic proteinuria, renal failure, hypertension and abnormal liver function (or hepatic fibrosis)...
January 26, 2017: Nephrology
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