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Pediatric liver pathology

Takao Togawa, Tatsuki Mizuochi, Tokio Sugiura, Hironori Kusano, Ken Tanikawa, Takato Sasaki, Fumio Ichinose, Seiichi Kagimoto, Takahisa Tainaka, Hiroo Uchida, Shinji Saitoh
OBJECTIVE: To clarify the clinical, pathologic, and genetic features of neonatal Dubin-Johnson syndrome. STUDY DESIGN: Ten patients with neonatal Dubin-Johnson syndrome were recruited from 6 pediatric centers in Japan between September 2013 and October 2016. Clinical and laboratory course, macroscopic and microscopic liver findings, and molecular genetic findings concerning ATP-binding cassette subfamily C member 2 (ABCC2) were retrospectively and prospectively examined...
February 28, 2018: Journal of Pediatrics
Andrew T Trout, Rachel M Sheridan, Suraj D Serai, Stavra A Xanthakos, Weizhe Su, Bin Zhang, Daniel B Wallihan
Purpose To assess the diagnostic performance of magnetic resonance (MR) elastography-derived liver stiffness to detect liver fibrosis in a pediatric and young adult population with a spectrum of liver diseases. Materials and Methods This retrospective study included patients younger than 21 years of age who underwent MR elastography and liver biopsy within 3 months of one another between January 2012 and September 2016 for indications other than liver transplantation or Fontan palliation of congenital heart disease...
February 22, 2018: Radiology
Jungmin Yoon, Byung Han Park, Ja Hye Kim, Jin Hwa Moon, Young Ho Lee, Se Min Jang, Yong Joo Kim
BACKGROUND: Histologically, nonalcoholic steatohepatitis (NASH) is categorized into adult-type (type 1) and pediatric-type (type 2). The origination of the histological difference between the two types and how they differ clinically remain uncertain. We aimed to understand the incidence and clinical characteristics of the two types of NASH in Korean children, and to investigate the association between their pathological type and clinical characteristics, using anthropometric and laboratory data...
January 29, 2018: Journal of Korean Medical Science
Mohamed R Abdelfattah, Hussein El-Siesy, Hadeel Al-Manea, Dieter C Broering
BACKGROUND: Hepatocellular carcinoma (HCC) is a leading cause of cancer-related deaths worldwide. Currently, liver transplantation (LT) for HCC is the only hope for cure from the tumor and from end-stage liver disease (ESLD). The organ pool shortage in deceased donor LT and the donor-related ethical concerns in living-donor LT necessitate the use of rigorous criteria for LT for HCC. In this respect, two main criteria for LT for HCC were implemented with good outcome, namely, the Milan and the University of California San Francisco criteria...
December 26, 2017: European Journal of Gastroenterology & Hepatology
Soichi Narumoto, Seisuke Sakamoto, Hajime Uchida, Kengo Sasaki, Takanobu Shigeta, Akinari Fukuda, Shunsuke Nosaka, Rie Irie, Takako Yoshioka, Mureo Kasahara
NEC is an idiopathic intestinal mucosal injury that may progress to transmural bowel necrosis without mesenteric ischemia. NEC usually affects 7- to 10-day-old neonates following enteral feeding. A 10-month-old girl with no history of laparotomy underwent LDLT for acute liver failure. After starting enteral feeding on postoperative day 5, she developed abdominal distention. Diffuse PVG and PI were detected by radiologic modalities. Exploratory laparotomy revealed patchy necrosis of the intestine without perforation...
February 2018: Pediatric Transplantation
Stephanie B Shamir, Jessica Kurian, Debora Kogan-Liberman, Benjamin H Taragin
Neonatal liver disease is an important source of morbidity in the pediatric population. The manifestation of liver disease in young infants may be different than in older patients, and there are a number of diagnoses that are unique to this age group. Familiarity with these entities is important as imaging plays a key role in the diagnostic workup, and prompt diagnosis is necessary to prevent complications. This article reviews the spectrum of liver pathologies that can manifest in the first 6 months of life and is intended to educate the general radiologist who may be faced with interpretation of neonatal liver imaging...
December 2017: Radiology
K Huang, K Y Qiu, L L Deng, J P Fang, Y Li, H X Guo, D H Zhou
Objective: To investigate the efficacy and safety of micafungin (MCF) for pulmonary invasive fungal disease (PIFD) in pediatric patients with acute leukemia or post hematopoietic stem cells transplantation. Method: Twenty-five neutropenic PIFD children with acute leukemia or post hematopoietic stem cells transplantation in Sun Yat-sen Memorial Hospital of Sun Yat-sen University were selected from January 2012 to June 2015, including 12 males and 13 females, age range 2-15 (average 6.2±2.0) years. There were 12 cases of acute leukemia (AL) after chemotherapy, 4 cases of acute leukemia (AL) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) and 9 cases of β-thalassemia major after allo-HSCT...
November 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Rachel Bernard, Ghanim Aljomah, Emily Klepper, Elizabeth McDonough
Cytomegalovirus (CMV) duodenitis is a rare occurrence, especially in pediatric patients. A thirteen-month-old female presented to the Emergency Department for a febrile seizure. She was incidentally admitted for severe malnutrition with an initial workup remarkable for only a slight elevation in her ALT at 48. The patient was found to have an oral aversion requiring nasogastric tube feeds for adequate caloric intake. She continued to fail to gain weight and underwent an EGD that demonstrated a duodenal ulcer...
2017: Case Reports in Pediatrics
T Y Wang, C Pan, Y J Gao, W T Hu, Q D Ye, M Zhou, J Y Tang
Objective: To investigate the long-term efficacy and prognostic factors of pediatric relapsed Wilms tumor (WT) after retreatment. Method: Sixteen children in Shanghai Children's Medical Center with relapsed Wilms tumor were enrolled consecutively in this study between April 2006 and June 2016. All patients were diagnosed according to pathology, imaging and medical and surgical oncologist's assistance. Relapse treatment included surgical excision, chemotherapy and selective radiation therapy. The clinical features, long-term outcomes and prognostic factors of patients were analyzed retrospectively...
October 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Seiro Machigashira, Tatsuru Kaji, Shun Onishi, Waka Yamada, Keisuke Yano, Koji Yamada, Ryuta Masuya, Takafumi Kawano, Kazuhiko Nakame, Motoi Mukai, Satoshi Ieiri
PURPOSE: Pediatric patients with intestinal failure need long-term parenteral nutrition (PN), but this nutritional support causes liver dysfunction, such as intestinal failure-associated liver disease (IFALD). Several studies have shown that the lipid emulsion produced by soybean oil (SO) is associated with the occurrence of IFALD. In this study, we evaluated the effect of SO and fish oil (FO) lipid emulsion on hepatic steatosis. METHODS: Sprague-Dawley rats underwent jugular vein catheterization and were divided into three groups: sham operation with normal chow (Sham group), 80% small bowel resection (80% SBR) + TPN with SO lipid emulsion (SO group), and 80% SBR + TPN with FO lipid emulsion (FO group)...
October 16, 2017: Pediatric Surgery International
Xinchi Hou, Bozena Birkenfeld, Hanna Piwowarska-Bilska, Anna Celler
BACKGROUND: Technetium-99m-hydrazinonicotinamide-Tyr(3)-octreotide ((99m)Tc-HYNIC-TOC) is recognized as a promising radiopharmaceutical for diagnosing neuroendocrine tumors (NETs). However, (99m)Tc-HYNIC-TOC dosimetry has been investigated only for adults. As pediatric radionuclide therapies become increasingly common, similar dosimetric studies for children are urgently needed. The aim of this study is to report personalized image-based biodistributions and dosimetry evaluations for children studies performed using (99m)Tc-HYNIC-TOC and to compare them with those from adult subjects...
October 13, 2017: EJNMMI Physics
Adriana Fonseca, Abha Gupta, Furqan Shaikh, Raveena Ramphal, Vicky Ng, Ian McGilvray, J Ted Gerstle
BACKGROUND: Orthotopic liver transplantation (OLT) is considered the standard for children with hepatoblastoma (HB) in whom complete surgical resection is not possible. However, OLT is not always available or feasible. OBJECTIVE: To describe the outcome of children with HB who were initially deemed unresectable and underwent complex hepatectomy with planned close margins, and ultimately avoided OLT. METHODS: Demographic data, surgical and pathologic details, and survival information were collected from children treated for HB between January 2010 to December 2015...
February 2018: Pediatric Blood & Cancer
Pablo Caro-Domínguez, Abha A Gupta, Govind B Chavhan
BACKGROUND: There are limited data on utility of diffusion-weighted imaging (DWI) in the evaluation of pediatric liver lesions. OBJECTIVE: To determine whether qualitative and quantitative DWI can be used to differentiate benign and malignant pediatric liver lesions. MATERIALS AND METHODS: We retrospectively reviewed MRIs in children with focal liver lesions to qualitatively evaluate lesions noting diffusion restriction, T2 shine-through, increased diffusion, hypointensity on DWI and apparent diffusion coefficient (ADC) maps, and intermediate signal on both, and to measure ADC values...
September 18, 2017: Pediatric Radiology
Yongqin Ye, Zhihan Li, Qi Feng, Zimin Chen, Zhouguang Wu, Jianyao Wang, Xiaoshuo Ye, Dahao Zhang, Lei Liu, Wei Gao, Lihui Zhang, Bin Wang
BACKGROUND AND OBJECTIVES: Biliary atresia (BA) is a pediatric liver disease characterized by fibro-obliteration and obstruction of the extrahepatic biliary system, that invariably leads to cirrhosis and even death, if left untreated for extended time. However, its pathology and etiology still remained unknown. In this study, we tested the expression of adducin 3 (ADD3), the gene identified as a susceptibility gene in BA by GWAS, and uncovered its upstream regulatory microRNA in the pathogenesis of BA...
2017: PloS One
Jacob A Mark, Cara L Mack, Ahmed I Marwan, Robert E Kramer
OBJECTIVE: Endoscopic retrograde cholangiopancreatography (ERCP) is used to manage biliary pathology in pediatric patients. Plastic biliary stents have been utilized in this population for obstructive lesions and bile leaks, however they are sometimes not effective due to migration, occlusion, or ineffective sealing. Fully covered self-expanding metal stents (FCSEMS) have larger diameters making them more suitable for some situations. However, their use in pediatrics has not been defined...
August 23, 2017: Journal of Pediatric Gastroenterology and Nutrition
Zaher K Otrock, Naval Daver, Hagop M Kantarjian, Charles S Eby
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by excessive activation of the immune system, resulting in overproduction of inflammatory cytokines. Patients usually present with high fever, cytopenias, hyperferritinemia, and hepatosplenomegaly, and their disease process ranges from mild to fatal multiorgan failure. HLH is a heterogeneous group of disorders that can be triggered by infections, neoplasms, or autoimmune diseases. The HLH diagnosis can be difficult to confidently confirm in critically ill patients while waiting for pathology or reference laboratory results to return, delaying the diagnosis with significantly worsened outcomes...
July 2017: Clinical Lymphoma, Myeloma & Leukemia
Jing Zhao, Xian-Jun Zhou, Cheng-Zhan Zhu, Ying Wu, Bin Wei, Gang Zhang, Xi-Wei Hao, Hong Zhang, Zhong Jiang, Qian Dong
OBJECTIVE: To evaluate the usefulness of Hisense Computer Assisted Surgery System (Hisense CAS) in pre-operative surgical planning and intra-operative navigation for resection of pediatric giant hepatic mesenchymal hamartoma (HMH). METHODS: Five children with HMH underwent hepatectomy in our hospital. Pre-operative abdominal enhanced CT was performed for diagnosis and treatment planning. Using CT DICOM files, three-dimensional reconstruction was performed in three cases for operation planning and intra-operative navigation, with SID carrying out precise liver resection during the operation with Hisense CAS...
December 2017: Computer Assisted Surgery (Abingdon, England)
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
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...
November 2017: Pediatric Transplantation
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...
October 2017: Current Opinion in Organ Transplantation
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