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Pediatric liver pathology

Saurabh Mukewar, Ayush Sharma, Kandace Lackore, Felicity Enders, Michael Torbenson, Patrick S Kamath, Lewis R Roberts, Yogish C Kudva
BACKGROUND & AIMS: Glycogenic hepatopathy, a syndrome characterized by hepatomegaly and increased liver transaminases in patients with type-1 diabetes, has not been well characterized in adults. We describe the clinical, biochemical, and histo-pathology profile of a cohort of patients with glycogenic hepatopathy. We also examined differences between patients with type-1 diabetes with vs without glycogenic hepatopathy. METHODS: We performed a case-control study of patients with type-1 diabetes diagnosed with glycogenic hepatopathy and patients with type-1 diabetes without glycogenic hepatopathy (controls)...
December 30, 2016: Clinical Gastroenterology and Hepatology
Gibran T Yusuf, Maria E Sellars, Annamaria Deganello, David O Cosgrove, Paul S Sidhu
OBJECTIVE: Because of concern over medical ionizing radiation exposure of children, contrast-enhanced ultrasound (CEUS) has generated interest as an inexpensive, ionizing radiation-free alternative to CT and MRI. CEUS has received approval for pediatric hepatic use but remains off-label for a range of other applications. The purposes of this study were to retrospectively analyze adverse incidents encountered in pediatric CEUS and to assess the financial benefits of reducing the number of CT and MRI examinations performed...
December 13, 2016: AJR. American Journal of Roentgenology
Amee M Bigelow, John P Scott, Johnny C Hong, David C Cronin, Bernadette E Vitola, Roger A Fons, Tara L Petersen
Among infants, almost half of acute liver failure cases are classified as indeterminate, whereas only a small number of cases show a documented viral infection. We present the first reported case of isolated acute hepatic failure in an infant in the setting of a human parechovirus (HPeV) infection. HPeV also may have been contributory to the posttransplant complication of 2 intussusceptions. This is a 10-month-old girl who presented with only symptoms of fussiness and was noted to have progressive decline in synthetic liver function as well as worsening coagulopathy requiring a liver transplant...
November 2016: Pediatrics
Archana Malik, Jeffrey C Hellinger, Sabah Servaes, Mathew C Schwartz, Marc S Keller, Monica Epelman
BACKGROUND: CT angiography is gaining broader acceptance in the evaluation of children with known or suspected congenital heart disease. These studies include non-cardiovascular structures such as the mediastinum, lung parenchyma and upper abdominal organs. It is important to inspect all these structures for potential abnormalities that might be clinically important and, in some cases, may impact care plans. OBJECTIVE: To determine the prevalence of non-cardiovascular findings in CT angiography of children with congenital heart disease...
December 6, 2016: Pediatric Radiology
Yi Zhang, Weiling Zhang, Suoqin Tang, Liping Chen, You Yi, Pinwei Zhang, Aiping Liu, Tian Zhi, Dongsheng Huang
Hepatoblastoma is a malignant liver tumor generally diagnosed in infants and children <3 years old. The current retrospective study aimed to investigate the associations of tumor stage, pathological type, metastasis and chemotherapy with clinical outcomes. In the current study, a total of 102 patients with hepatoblastoma were enrolled between September 2006 and June 2014. Clinical records and follow-up information for each of patient were obtained to conduct a Kaplan-Meier survival analysis and log-rank test...
November 2016: Oncology Letters
Lin Zhuang, Chuangye Ni, Wenbing Din, Feng Zhang, Yi Zhuang, Yawei Sun, Dong Xi
INTRODUCTION: Focal nodular hyperplasia (FNH) is a benign lesion of the liver which is usually found in healthy adults, however, FNH is rare in children, and comprises only 2% of all pediatric liver tumors. Herein, we report the case of a 6-year-old child (male) with a huge FNH which size is more than 10cm. This could be the biggest FNH among all children's FNH cases ever reported. CASE PRESENTATION: A 6-year-old boy was found a hepatic space-occupying lesion two years ago...
2016: International Journal of Surgery Case Reports
Pelin Börcek, Binnaz Handan Özdemir, Çiğdem Sercan, Eda Yılmaz Akçay, Sema Karakuş, Mehmet Haberal
OBJECTIVES: Liver transplant may complicated by various hematologic conditions, resulting in indication for bone marrow biopsy. Immunosuppressive therapies, specific infections, and secondary neoplasms affect bone marrow. In the present study, we evaluated the histologic spectrum of bone marrow findings in liver allograft recipients. MATERIALS AND METHODS: Of 338 patients who received liver transplants and were followed at the Başkent University, Faculty of Medicine, 44 patients underwent bone marrow biopsy...
2016: Experimental and Clinical Transplantation
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 L 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 (25%-40%) followed by an expanding list of monogenic disorders (25%), along with many unknown or multifactorial (eg, parenteral nutrition-related) causes, each of which may have time-sensitive and distinct treatment plans...
January 2017: 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
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