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Pediatric liver pathology

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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
#1
Nadja Fratzl-Zelman, Helena Valta, Renata C Pereira, Barbara M Misof, Paul Roschger, Hannu Jalanko, Kathrine 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...
February 18, 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
#2
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
https://www.readbyqxmd.com/read/28043933/clinical-biochemical-and-histopathology-features-of-patients-with-glycogenic-hepatopathy
#3
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
https://www.readbyqxmd.com/read/27959665/retrospective-analysis-of-the-safety-and-cost-implications-of-pediatric-contrast-enhanced-ultrasound-at-a-single-center
#4
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...
February 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/27940759/human-parechovirus-as-a-cause-of-isolated-pediatric-acute-liver-failure
#5
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
https://www.readbyqxmd.com/read/27924361/prevalence-of-non-cardiovascular-findings-on-ct-angiography-in-children-with-congenital-heart-disease
#6
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
https://www.readbyqxmd.com/read/27895749/a-single-center-retrospective-study-of-pediatric-hepatoblastoma
#7
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
https://www.readbyqxmd.com/read/27825058/huge-focal-nodular-hyperplasia-presenting-in-a-6-year-old-child-a-case-presentation
#8
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
https://www.readbyqxmd.com/read/27805526/histologic-changes-in-bone-marrow-biopsies-from-liver-transplant-patients
#9
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
https://www.readbyqxmd.com/read/27680015/practical-use-and-pitfalls-of-hepatocyte-specific-contrast-agents-hscas-for-pediatric-hepatic-and-biliary-magnetic-resonance-imaging
#10
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
https://www.readbyqxmd.com/read/27491101/inconspicuous-presentation-of-metastatic-neuroblastoma
#11
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
https://www.readbyqxmd.com/read/27429428/guideline-for-the-evaluation-of-cholestatic-jaundice-in-infants-joint-recommendations-of-the-north-american-society-for-pediatric-gastroenterology-hepatology-and-nutrition-and-the-european-society-for-pediatric-gastroenterology-hepatology-and-nutrition
#12
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
https://www.readbyqxmd.com/read/27391242/an-animal-model-for-the-juvenile-non-alcoholic-fatty-liver-disease-and-non-alcoholic-steatohepatitis
#13
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
https://www.readbyqxmd.com/read/27320565/impact-of-donor-specific-antibodies-on-graft-fibrosis-after-pediatric-living-donor-liver-transplantation-for-biliary-atresia
#14
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
https://www.readbyqxmd.com/read/27317956/minimally-invasive-liver-resection-in-pediatric-patients-initial-experience-and-outcomes
#15
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
https://www.readbyqxmd.com/read/27247369/extreme-hyperferritinemia-%C3%A2-causes-and-impact-on-diagnostic-reasoning
#16
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
https://www.readbyqxmd.com/read/27126572/anesthetic-management-of-a-pediatric-patient-with-pulmonary-arteriovenous-fistula-undergoing-liver-transplantation-a-case-report
#17
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
https://www.readbyqxmd.com/read/27096257/hepatoblastoma-arising-in-a-pigmented-%C3%AE-catenin-activated-hepatocellular-adenoma-case-report-and-review-of-the-literature
#18
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
https://www.readbyqxmd.com/read/27090203/the-optimal-immunosuppressive-protocol-for-the-portal-vein-infusion-of-pge1-and-methylprednisolone-in-pediatric-liver-transplantation-for-fulminant-hepatic-failure-of-unknown-etiology
#19
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
https://www.readbyqxmd.com/read/27001031/pediatric-liver-transplantation-predictors-of-survival-and-resource-utilization
#20
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
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