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Pediatric liver pathology

Rama S Ayyala, Sudha A Anupindi, Michael J Callahan
Magnetic resonance imaging is commonly used to evaluate for hepatic and biliary pathology in the pediatric population. Recently, there has been increased use of hepatocyte-specific contrast agents (HSCAs), such as Gadoxetate disodium in children. Traditionally, HSCAs have been used to characterize focal liver lesions. However, these agents can also be used to problem solve specific hepatic or biliary diagnostic dilemmas. The purpose of this manuscript is to review the practical uses of HSCA in children with both hepatic and biliary indications, and review the corresponding imaging findings...
September 28, 2016: Abdominal Radiology
James Hatten, Aaron McGuffin, Mark Mogul
Neuroblastoma is a malignant tumor arising from nerve tissue that accounts for approximately 15 percent of pediatric cancer fatalities. Primary tumors most commonly arise in sympathetic nervous tissue of the abdomen and metastasize to the bone marrow, liver, and lymph nodes. This case report depicts a 3-year-old girl who presented with a recurring fever, runny nose, and a positive test for rhinovirus suggesting a simple case of the common cold. Further investigation, however, revealed stage 4 neuroblastoma...
July 2016: West Virginia Medical Journal
Rima Fawaz, Ulrich Baumann, Udeme Ekong, Björn Fischler, Nedim Hadzic, Cara L Mack, Valérie A McLin, Jean P Molleston, Ezequiel Neimark, Vicky Lee Ng, Saul J Karpen
Cholestatic jaundice in infancy affects approximately 1 in every 2500 term infants and is infrequently recognized by primary providers in the setting of physiologic jaundice. Cholestatic jaundice is always pathologic and indicates hepatobiliary dysfunction. Early detection by the primary care physician and timely referrals to the pediatric gastroenterologist/hepatologist are important contributors to optimal treatment and prognosis. The most common causes of cholestatic jaundice in the first months of life are biliary atresia (BA, 25-40%) followed by an expanding list of monogenic disorders (25%), plus many unknown or multifactorial (e...
July 16, 2016: Journal of Pediatric Gastroenterology and Nutrition
Veronica Marin, Natalia Rosso, Matteo Dal Ben, Alan Raseni, Manuela Boschelle, Cristina Degrassi, Ivana Nemeckova, Petr Nachtigal, Claudio Avellini, Claudio Tiribelli, Silvia Gazzin
Non Alcoholic Fatty Liver Disease (NAFLD) and Non-Alcoholic Steatohepatitis (NASH) are the hepatic manifestations of the metabolic syndrome; worrisome is the booming increase in pediatric age. To recreate the full spectrum of juvenile liver pathology and investigate the gender impact, male and female C57Bl/6 mice were fed with high fat diet plus fructose in the drinking water (HFHC) immediately after weaning (equal to 3-years old human), and disease progression followed for 16 weeks, until adults (equal to 30-years old human)...
2016: PloS One
T Ueno, M Zenitani, H Yamanaka, N Tanaka, S Uehara, Y Tazuke, K Bessho, H Okuyama
BACKGROUND: Pediatric living donor liver transplant (LDLT) patients sometimes develop graft fibrosis after non-recurrent diseases such as biliary atresia (BA). Donor-specific antibodies (DSA) have recently been shown to play a possible role in graft damage after liver transplantation. We report the impact of DSA on pediatric LDLT for BA patients. METHODS: Patients under age 18 years who received LDLT for BA at our institution and who had at least 5 years' follow-up were identified, and 23 were eventually enrolled in this study...
May 2016: Transplantation Proceedings
Michelle A Veenstra, Alan J Koffron
BACKGROUND: Minimally Invasive Liver Resection (MILR) is an evolving procedure in the adult population for benign and malignant lesions, offering less morbidity while maintaining acceptable outcomes. However, there lacks a published MILR experience in the pediatric population besides case reports. This report describes a pediatric MILR experience in terms of pathology, clinical specifics, and patient outcomes. METHODS: This is a retrospective review of 36 pediatric patients undergoing MILR for benign and malignant conditions...
June 2016: HPB: the Official Journal of the International Hepato Pancreato Biliary Association
Katie Sackett, Maros Cunderlik, Nishant Sahni, Anthony A Killeen, Andrew P J Olson
OBJECTIVES: Hyperferritinemia can be a result of inflammation, infection, chronic iron overload, or other uncommon pathologies including hemophagocytic lymphohistiocytosis (HLH). There is a historical association between extreme hyperferritinemia and HLH, but in reality HLH is associated with a minority of hyperferritinemic states. METHODS: We identified conditions most associated with hyperferritinemia by identifying 65,536 serum ferritin levels at the University of Minnesota Hospital over a five-year period, with 86 values higher than 10,000 ng/mL...
May 2016: American Journal of Clinical Pathology
Ho-Jin Lee, Jung-Man Lee, Chul-Woo Jung, Jiwon Lee
For patients with HPS who require anesthesia for a procedure, HPV should be maintained to prevent worsening hypoxemia. Here, the case of a 9-yr-old girl who was scheduled for a living donor liver transplantation is presented. The patient suffered from end-stage liver disease with HPS due to biliary atresia, which contributed to the development of a diffuse pulmonary AVF. Consequently, anesthetic management of this patient involved two different types of pulmonary shunt. It is important to maintain HPV, not only to prevent worsening of the hypoxia caused by HPS but also to inhibit an increase in PVR that could cause an increase of shunt flow through the pathological fistula...
August 2016: Pediatric Transplantation
Christine Y Louie, Waldo Concepcion, Joseph K Park, Arun Rangaswami, Milton J Finegold, Florette K Hazard
Hepatoblastoma is the most common malignant liver tumor in childhood. It has been associated with a variety of constitutional syndromes and gene mutations. However, there are very few reports of associations with pediatric hepatocellular adenomas (HCAs) and no reported associations with pigmented HCAs (P-HCAs). We present a unique case of hepatoblastoma arising in a background of 2 β-catenin-activated HCAs, one of which is pigmented, in a 4-year-old child. The gross, histologic, and immunohistochemical features are described for each tumor...
July 2016: American Journal of Surgical Pathology
Yohei Yamada, Ken Hoshino, Rie Irie, Hirofumi Tomita, Mototoshi Kato, Naoki Shimojima, Akihiro Fujino, Taizo Hibi, Masahiro Shinoda, Hideaki Obara, Osamu Itano, Shigeyuki Kawachi, Minoru Tanabe, Michiie Sakamoto, Yuko Kitagawa, Tatsuo Kuroda
The outcome of LTx in pediatric patients with FHF of unknown etiology remains inferior to that of LTx in pediatric patients with cholestatic diseases. A higher incidence of steroid-resistant severe rejection has been increasingly recognized among the responsible factors. We assessed the efficacy of the administration of steroids and PGE1 via PVI in the management of LTx for FHF in pediatric patients. In our early cohort (1995-2007), seven patients who underwent LTx for FHF of unknown etiology were treated with conventional immunosuppressive therapy (calcineurin inhibitor and a steroid)...
August 2016: Pediatric Transplantation
Amy E Wagenaar, Jun Tashiro, Juan E Sola, Obi Ekwenna, Akin Tekin, Eduardo A Perez
PURPOSE: We sought to identify factors associated with increased resource utilization and in-hospital mortality for pediatric liver transplantation (LT). METHODS: Kids' Inpatient Database (1997-2009) was used to identify cases of LT in patients <20 years old. RESULTS: Overall, 2905 cases were identified, with an in-hospital survival of 91 %. LT was performed most frequently in < 5 year olds (61 %), females (51 %), and Caucasians (56 %)...
May 2016: Pediatric Surgery International
Piti Techavichit, Prakash M Masand, Ryan W Himes, Rana Abbas, John A Goss, Sanjeev A Vasudevan, Milton J Finegold, Andras Heczey
Undifferentiated embryonal sarcoma of the liver (UESL) is a rare and aggressive pediatric malignancy. The purpose of this study was to review the clinical, radiologic, and pathologic features and outcome of children with UESL at our institution, in the United Network of Organ Sharing database and to review the existing literature to define the state of the art for children with UESL. Six children were diagnosed with UESL at the Texas Children's Cancer Center between 1993 and 2014, 12 children underwent liver transplantation registered in the United Network of Organ Sharing database, and 198 children with UESL were described in 23 case series during 1978 to 2014...
May 2016: Journal of Pediatric Hematology/oncology
Mirco Nacoti, Davide Corbella, Francesco Fazzi, Francesca Rapido, Ezio Bonanomi
Bleeding and coagulopathy are critical issues complicating pediatric liver transplantation and contributing to morbidity and mortality in the cirrhotic child. The complexity of coagulopathy in the pediatric patient is illustrated by the interaction between three basic models. The first model, "developmental hemostasis", demonstrates how a different balance between pro- and anticoagulation factors leads to a normal hemostatic capacity in the pediatric patient at various ages. The second, the "cell based model of coagulation", takes into account the interaction between plasma proteins and cells...
February 14, 2016: World Journal of Gastroenterology: WJG
Raffaella A Morotti, Dhanpat Jain
This article addresses select liver diseases that are commonly seen in the pediatric group and pose diagnostic challenges in practice. The key genetic/molecular abnormalities, clinical features, histopathologic findings, diagnostic modalities, differential diagnoses, and possible pitfalls in diagnosis are discussed in detail. Although recent advances in understanding the pathophysiology of bile synthesis and transport along with advances in molecular genetics have allowed a better characterization of many of these liver diseases, significant overlap in the histopathologic features of many of these disorders still leads to diagnostic challenges for the pathologist...
June 2013: Surgical Pathology Clinics
Consolato M Sergi
Fibrolamellar hepatocellular carcinoma (FL-HCC) is generally a fairly rare event in routine pathology practice. This variant of hepatocellular carcinoma (HCC) is peculiarly intriguing and,in addition, poorly understood. Young people or children are often the target individuals with this type of cancer. Previously, I highlighted some pathology aspects of FL-HCC, but in this review, the distinctive clinico-pathologic features of FL-HCC and the diagnostic pathologic criteria of FL-HCC are fractionally reviewed and expanded upon...
2015: Diagnostics
Eiso Hiyama
Hepatoblastoma (HBL) is the most common primary liver tumor in children, and is usually diagnosed during the first 3 years of life. Collaborative multicenter studies have led to improved diagnostic and treatment strategies. The pretreatment extent of disease (PRETEXT) staging system has become a consensus classification, and an international pathological classification system has also been developed. Clinical trials examining multimodal therapy, which consists of complete surgical resection plus liver transplantation and chemotherapy, have led to improved outcomes for children with HBL...
October 2014: Translational pediatrics
Lin Su, Xian-Jun Zhou, Qian Dong, Hong Zhang, Feng Shen, Yong-Jian Chen, Xi-Wei Hao, Xiao-Fei Li
We discussed the diagnostic and treatment value and clinical significance of computer assisted surgery system (Higemi) in precision surgeries for pediatric complex liver tumors. A total of 21 pediatric cases receiving hepatectomy for tumors in the portal vein and giant liver tumors from June 2012 to January 2015 were analyzed. Higemi was used for 3-dimensional (3D) reconstruction of thin-slice CT images and surgical planning. Tumors were precisely located and blood vessel neighborhood was determined so as to evaluate surgical feasibility...
2015: International Journal of Clinical and Experimental Medicine
Shyam Mohan, Rahim Moineddin, Govind B Chavhan
PURPOSE: To compare whole-body magnetic resonance imaging (WBMRI) performed at 1.5 and 3 T for technical quality, artifacts, and visibility of selected fixed structures. PATIENTS AND METHODS: 21 children who had WBMRI at both 1.5 and 3 T scanners within a relatively short interval (3-13 months; average-8.6 months) were included. The images were objectively compared with scores from 4 to 1 for five parameters including severity of artifacts; visibility of liver, vertebral column, and marrow in legs; and overall image quality...
October 2015: Indian Journal of Radiology & Imaging
M Bergallo, M Pinon, I Galliano, P Montanari, V Daprà, S Gambarino, P L Calvo
OBJECTIVES: Human endogenous retrovirus (HERVs) constitute approximately 8% of the human genome. Induction of HERV transcription is possible under certain circumstances, and may have a possible role in some pathological conditions. Aim of the present study was to verify whether HERV-W and K activation by EBV might occur also in vivo, during EBV infection, in pediatric liver transplant recipients. METHODS: A total of 35 pediatric liver transplant (LT) patients who received LT at the University Hospital City of Science and Health of Turin, Regina Margherita Children's Hospital were included...
December 17, 2015: Minerva Pediatrica
Joanna M Łotowska, Dariusz M Lebensztejn
The research objective was to identify and quantify the immunohistochemically (IHC) stained hepatic stellate cells (HSCs) in children with chronic hepatitis B (CHB), including staging (S), location in the hepatic lobule, and correlation with hepatocyte count. Retrospective morphological analysis was based on liver biopsies obtained from 70 CHB children before antiviral treatment. To determine fibrosis stage, the Batts and Ludwig scoring system was applied. Immunohistochemical examinations used monoclonal antibodies against - SMA...
September 2015: Polish Journal of Pathology: Official Journal of the Polish Society of Pathologists
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