keyword
https://read.qxmd.com/read/38154976/-zellweger-syndrome-caused-by-pex6-gene-variation-in-2-cases-and-literature-review
#21
JOURNAL ARTICLE
P Yang, C Z Zeng, X W Tao, S W Rong, Y Long, L K Zeng
Objective: To summarize the clinical features and genetic characteristics of Zellweger spectrum disorder caused by PEX6 gene variation. Methods: This was a case series research. Clinical date and genetic results of 2 neonatal cases of Zellweger syndrome caused by PEX6 gene variation in Wuhan Children's Hospital, Tongji Medical College, Huazhong University of Science & Technology and Affiliated Hospital of Guangdong Medical University from July 2021 to July 2022 were retrospectively collected and analyzed...
January 2, 2024: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://read.qxmd.com/read/38138042/high-incidence-of-acute-liver-failure-among-patients-in-egypt-coinfected-with-hepatitis-a-and-hepatitis-e-viruses
#22
JOURNAL ARTICLE
Mohamed A El-Mokhtar, Amal A Elkhawaga, Mona Sedky Hussein Ahmed, Ehsan M W El-Sabaa, Aliaa A Mosa, Ahmed Shawkat Abdelmohsen, Abdelmajeed M Moussa, Eman H Salama, Sahar Aboulfotuh, Ahmed M Ashmawy, Ahmed Ismail Seddik, Ibrahim M Sayed, Haidi Karam-Allah Ramadan
Hepatitis A virus (HAV) and Hepatitis E virus (HEV) are transmitted through the fecal-oral route. HAV outbreaks and one HEV outbreak have been reported in Egypt. However, the impact of HAV-HEV co-infection is not known. In this study, we assessed HEV markers in acute HAV-infected patients ( n = 57) enrolled in Assiut University hospitals. We found that 36.8% of HAV-infected patients were also positive for HEV markers (anti-HEV IgM and HEV RNA), while 63.2% of the patients were HAV mono-infected. Demographic and clinical criteria were comparable in both HAV mono-infected patients and HAV-HEV co-infected patients...
November 30, 2023: Microorganisms
https://read.qxmd.com/read/38089685/a-systematic-review-of-the-proposed-etiologies-of-the-2021-2022-outbreaks-of-pediatric-acute-hepatitis-of-unknown-etiology
#23
REVIEW
Lauren Lewis, Carly van Wylick, Daniel J Mulder
In April 2022, the World Health Organization (WHO) declared a global outbreak of acute hepatitis of unknown etiology (AHUE) with a high risk of severe outcomes, for which various etiologies have been proposed by the literature. This study examines primary reports of pediatric AHUE cases and summarizes the proposed etiologies. This systematic review collected and evaluated published peer-reviewed articles, official data, and clinical reports of AHUE cases that met the WHO working case definition. 19 hypothesized etiologies for AHUE were identified from 36 sources, which fell into eight categories...
2023: Frontiers in Pediatrics
https://read.qxmd.com/read/38088852/-reconstruction-of-total-portosystemic-shunt-into-selective-portosystemic-shunt-in-a-child
#24
JOURNAL ARTICLE
S R Margaryan, A Yu Razumovsky, Z B Mitupov, A I Gurevich, E A Titova
To date, side-to-side splenorenal shunt (SRS) and its analogues (splenosuprarenal shunts (SSRS)) are mainly used for portal hypertension. These are total portosystemic shunts characterized by total blood shunt from portal vein into inferior vena cava. The latter is fraught with a significant risk of complications such as pulmonary hypertension, decreased portal liver perfusion, liver failure and hepatic encephalopathy. Prevention of these complications is still an urgent problem in modern surgery. However, we proposed a new method of treatment, i...
2023: Khirurgiia
https://read.qxmd.com/read/38074262/accidental-iron-poisoning-in-children-experience-from-a-teaching-institution
#25
JOURNAL ARTICLE
Jolly Chandran, R Sanketh, Siva Vyasam, Asha Chrysolyte, Kala Ebenezer
BACKGROUND: Acute iron toxicity is fatal in children resulting from an accidental overdose of maternal iron tablets at home. There is scanty literature on children looking at the profile and outcome. We report a case series of five children presenting after accidental ingestion of iron tablets. Two presented with fulminant hepatic failure at 48 h, and despite supportive management and plasmapheresis in one child, both succumbed to illness. MATERIALS AND METHODS: This retrospective study was conducted in the pediatric intensive care unit (PICU) of a teaching institution in South India between January 2009 and December 2019...
October 2023: Journal of Family Medicine and Primary Care
https://read.qxmd.com/read/38060750/liver-transplantation-for-acute-hepatic-failure-following-intentional-iron-overdose
#26
Rosário Eça, Sofia Ferreira, Judit Gandara, Helena Pessegueiro, Jorge Daniel
The majority of acute iron toxicity cases occur in young children from accidental ingestion. In adults, iron poisoning is rare and mostly due to intentional ingestion. Physicians, particularly those who do not routinely treat pediatric patients, are often unfamiliar with the clinical manifestation of iron poisoning, its management, and its potential for multiple organ failure, especially liver damage. Severe acute hepatotoxicity treated with liver transplantation is rare in adults, with very limited published literature...
November 2023: Curēus
https://read.qxmd.com/read/38059323/combined-liver-kidney-transplantation-in-pediatric-patients
#27
REVIEW
Nam-Joon Yi, Jiyoung Kim, Su Young Hong, Hee Gyung Kang
Combined liver-kidney transplantation (CLKT) is a surgical procedure that involves transplanting both liver and kidney organs. There are two types of CLKT: simultaneous liver-kidney transplantation (smLKT) and sequential LKT (sqLKT). CLKT accounts for a small percentage of liver transplantations (LTs), particularly in pediatric cases. Nevertheless, the procedure has demonstrated excellent outcomes, with high survival rates and lower rejection rates. The main indications for CLKT in pediatric patients differ somewhat from that in adults, in which end-stage kidney disease after LT is the major indication...
December 7, 2023: Pediatric Transplantation
https://read.qxmd.com/read/38050913/a-report-on-a-nationwide-surveillance-system-for-pediatric-acute-hepatitis-of-unknown-etiology-in-korea
#28
JOURNAL ARTICLE
Kyung Jae Lee, Jae Sung Ko, Kie Young Park, Ki Soo Kang, Kunsong Lee, Jeana Hong, Soon Chul Kim, Yoon Lee, Ben Kang, Yu Bin Kim, Hyun Jin Kim, Byung Wook Eun, Hye-Kyung Cho, Yae-Jean Kim, Mi Jin Kim, Jin Lee, Taek-Jin Lee, Seak Hee Oh, Sowon Park, Eun Ha Hwang, Sangjun Sohn, Jin Gyu Lim, YooJin Kim, Yeoun Joo Lee
BACKGROUND: Several cases of pediatric acute hepatitis of unknown etiology related to adenoviral infections have been reported in Europe since January 2022. The aim of this study was to compare the incidence, severity, possible etiology, and prognosis of the disease with those in the past in Korea. METHODS: The surveillance group collected data between May and November 2022 using a surveillance system. Acute hepatitis of unknown etiology was defined in patients aged < 16 years with a serum transaminase level > 500 IU/L, not due to hepatitis A-E or other underlying causes...
December 4, 2023: Journal of Korean Medical Science
https://read.qxmd.com/read/38036869/liver-histology-and-hepatic-progenitor-cell-activity-in-pediatric-acute-liver-failure-implications-for-clinical-outcome
#29
JOURNAL ARTICLE
Kalpana Panda, Vikrant Sood, Bikrant Bihari Lal, Rajeev Khanna, Archana Rastogi, Gayatri Ramakrishna, Seema Alam
BACKGROUND: Hepatic progenitor cell (HPC) activity and regenerative process that follows pediatric acute liver failure (PALF) is still not well understood. This clinicopathological study was thus conducted with an aim to study the correlation of liver histology and HPC activity with outcomes in PALF. METHODS: All PALF patients with available hepatic histological specimens were included and specimens were analyzed for hepatocyte loss, HPC activity [using cytokeratin (CK) 7, CK19, sex-determining region Y-related high mobility group box(SOX)9 and epithelial cell adhesion molecule (EpCAM)], hepatocyte proliferation (using Ki67), and hepatocyte senescence (using p53 and p21)...
November 30, 2023: Pediatric Transplantation
https://read.qxmd.com/read/38034454/a-case-of-reactivated-acute-hepatitis-in-a-12-month-old-infant-with-multiviral-infection-including-sars-cov-2-and-adenovirus
#30
Jimmy Dawood, Emily L Wickersham, Claire P Witmer, Milissa U Jones
Adenovirus hepatitis is of global concern due to its increasing incidence and poorly understood pathogenesis. Historically, adenovirus has contributed to the development of severe hepatitis in immunocompromised patients. The clinical course and management of such infections in previously healthy children remains elusive. We present a case of severe acute hepatitis in a previously healthy 12-month-old infant with a history of SARS-CoV-2 infection followed by multiviral infection including adenovirus. Additional evaluation revealed acute hepatitis without evidence of acute liver failure except for mild coagulopathy...
November 2023: JPGN reports
https://read.qxmd.com/read/38007049/demystifying-the-global-outbreak-of-severe-acute-hepatitis-of-unknown-aetiology-in-children-a-systematic-review-and-meta-analysis
#31
JOURNAL ARTICLE
Julie Phan, Guy D Eslick, Elizabeth J Elliott
BACKGROUND: The sudden outbreak of severe acute hepatitis of unknown aetiology (SAHUA) in the first half of 2022 affected more than 1010 children in 35 countries worldwide. Dire clinical outcomes, such as acute liver failure necessitating transplantation, neurological symptoms, long-term sequelae, and death, highlight the need to determine the pathogenesis of this condition. Hypotheses on the aetiology include adenovirus and SARS-CoV-2 infections and an aberrant immune response to multiple pathogen exposure following lifting of lockdown measures but further investigation is required to reach an informed consensus...
January 2024: Journal of Infection
https://read.qxmd.com/read/37996345/efficacy-of-therapeutic-plasma-exchange-in-pediatric-cases-of-acute-liver-failure-as-an-extracorporeal-liver-support-system
#32
JOURNAL ARTICLE
Mohit Chowdhry, Ankita Sharma, Soma Agrawal, Rohit Vohra, Karunesh Kumar, Neerav Goyal, Arun Kumar V, Nameet Jerath, Smita Malhotra, Anupam Sibal, Manoj Mishra
BACKGROUND: Acute liver failure in the pediatric population is often accompanied by deranged metabolism, severe encephalopathy and coagulopathy. A liver transplant is the most viable option for the management of such patients. Therapeutic plasma exchange (TPE) is helpful in improving the liver biochemistry profile, thereby, increasing their likelihood of undergoing a liver transplant METHOD: The study was conducted over a period of 3 years (January 2018 to December 2021). Indications mainly consisted of ALF with hepatic encephalopathy, worsening liver parameters in spite of medical management, and candidacy for undergoing a liver transplant...
October 21, 2023: Transfusion and Apheresis Science
https://read.qxmd.com/read/37980196/hepatic-metastasectomy-in-pediatric-patients-an-observational-study
#33
JOURNAL ARTICLE
Andrew M Fleming, Sara A Mansfield, Tim Jancelewicz, Ankush Gosain, James W Eubanks, Andrew M Davidoff, Max R Langham, Andrew J Murphy
BACKGROUND: The role of hepatectomy for metastatic disease in children is controversial. Rationales include potential cure, obtaining a diagnosis, and guiding chemotherapy decisions. This study examines the safety and utility of hepatic metastasectomy for children at a single institution. METHODS: After IRB approval (#22-1258), medical records were reviewed from 1995 to 2022 for children undergoing hepatic metastasectomy. En-bloc hepatectomies during primary tumor resection were excluded...
October 23, 2023: Journal of Pediatric Surgery
https://read.qxmd.com/read/37977713/safety-and-efficacy-of-gene-replacement-therapy-for-x-linked-myotubular-myopathy-aspiro-a-multinational-open-label-dose-escalation-trial
#34
RANDOMIZED CONTROLLED TRIAL
Perry B Shieh, Nancy L Kuntz, James J Dowling, Wolfgang Müller-Felber, Carsten G Bönnemann, Andreea M Seferian, Laurent Servais, Barbara K Smith, Francesco Muntoni, Astrid Blaschek, A Reghan Foley, Dimah N Saade, Sarah Neuhaus, Lindsay N Alfano, Alan H Beggs, Ana Buj-Bello, Martin K Childers, Tina Duong, Robert J Graham, Minal Jain, Julie Coats, Vicky MacBean, Emma S James, Jun Lee, Fulvio Mavilio, Weston Miller, Fatbardha Varfaj, Michael Murtagh, Cong Han, Mojtaba Noursalehi, Michael W Lawlor, Suyash Prasad, Salvador Rico
BACKGROUND: X-linked myotubular myopathy is a rare, life-threatening, congenital muscle disease observed mostly in males, which is caused by mutations in MTM1. No therapies are approved for this disease. We aimed to assess the safety and efficacy of resamirigene bilparvovec, which is an adeno-associated viral vector serotype 8 delivering human MTM1. METHODS: ASPIRO is an open-label, dose-escalation trial at seven academic medical centres in Canada, France, Germany, and the USA...
December 2023: Lancet Neurology
https://read.qxmd.com/read/37970614/challenges-and-dilemmas-in-pediatric-hepatic-wilson-s-disease
#35
REVIEW
Upasana Ghosh, Moinak Sen Sarma, Arghya Samanta
Wilson disease is an autosomal recessive disorder affecting the ATP7B gene located on chromosome 13q. This leads to copper deposition in various organs, most importantly in the liver and brain. The genetic mutations are vast, well reported in the West but poorly documented in developing countries. Hence the diagnosis is made with a constellation of clinico-laboratory parameters which have significant overlap with other liver diseases and often pose a significant dilemma for clinicians. Diagnostic scoring systems are not fool-proof...
October 27, 2023: World Journal of Hepatology
https://read.qxmd.com/read/37965976/the-role-of-liver-transplantation-in-coach-syndrome-joubert-syndrome-with-congenital-hepatic-fibrosis-a-review-of-the-literature
#36
JOURNAL ARTICLE
Lijian Chen, Hajime Uchida, Ryuji Komine, Tasuku Kodama, Toshimasa Nakao, Noriki Okada, Yusuke Yanagi, Seiichi Shimizu, Syed Abbas, Akinari Fukuda, Seisuke Sakamoto, Mureo Kasahara
BACKGROUND: COACH syndrome is a rare autosomal recessive genetic disease characterized by liver fibrosis, which leads to severe complications related to portal hypertension. However, only a few patients with COACH syndrome undergoing liver transplantation (LT) have been reported. MATERIALS AND METHODS: We herein report the outcomes of four children who underwent LT for COACH syndrome at our institute and review three previously reported cases to elucidate the role of LT in COACH syndrome...
November 15, 2023: Pediatric Transplantation
https://read.qxmd.com/read/37936530/outcome-of-pediatric-living-donor-liver-transplant-experience-from-pakistan-a-resource-limited-setting
#37
JOURNAL ARTICLE
Sahira Aaraj, Sabeen Abid Khan, Fatima Maroof, Syed Zohaib Hussain, Faisal Saud Dar, Munir Iqbal Malik
INTRODUCTION: Liver transplantation (LT) has emerged as a lifesaving modality for many liver diseases in children. Pediatric LT is an established treatment in the Western world but is relatively a new procedure in resource-limited countries like Pakistan. The study aims to highlight the outcomes and survival of pediatric recipients from the first pediatric liver transplant center in Pakistan. METHOD: A retrospective analysis of pediatric LT was done from 2012 to 2019...
November 7, 2023: Pediatric Transplantation
https://read.qxmd.com/read/37916829/severe-methotrexate-toxicity-following-a-capizzi-cycle-in-an-obese-adolescent-with-acute-lymphoblastic-leukemia-and-hepatic-steatosis
#38
JOURNAL ARTICLE
Julie M Fischer, Oranooj Lertkovit, Scott C Howard, Chatchawin Assanasen, Archie Bleyer
Methotrexate is a major component of pediatric leukemia treatment. While toxicities are common after high-dose methotrexate, escalating dose methotrexate (Capizzi methotrexate) is typically well-tolerated. We report an adolescent Hispanic female with pre-B acute lymphoblastic leukemia, preexisting obesity and hepatic steatosis who developed severe multiorgan failure following an escalating dose of methotrexate with delayed methotrexate excretion of 11 days. We identified one similar report in an obese adult; however, this case is the first to our knowledge involving a pediatric patient...
November 2, 2023: Journal of Pediatric Hematology/oncology
https://read.qxmd.com/read/37880024/liver-transplantation-for-acute-liver-failure-due-to-yellow-phosphorus-poisoning-a-comprehensive-review
#39
JOURNAL ARTICLE
Hirak Pahari, Amruth Raj, Amey Sonavane, Ambreen Sawant, Deepak Kumar Gupta, Amit Gharat, Vikram Raut
Yellow phosphorus or metal phosphide (YP-MP) rodenticide poisoning has been a known cause of acute liver failure (ALF) in many countries of Asia and North and South America over the last decade. It is a highly toxic compound and is a well-known cause of intentional or accidental poisoning in both adults and children. In lower doses, it causes gastrointestinal symptoms and mild hepatic injury, and patients may spontaneously recover. In higher doses, hepatic necrosis and fatty infiltration may cause significant injury and may even lead to ALF, characterized by hepatic encephalopathy, coagulopathy, and lactic acidosis...
October 23, 2023: Transplantation Proceedings
https://read.qxmd.com/read/37837969/acute-liver-failure-in-low-income-and-middle-income-countries
#40
REVIEW
Sandro Vento, Francesca Cainelli
Acute liver failure is a rare condition involving the rapid development, progression, and worsening of liver dysfunction, characterised by coagulopathy and encephalopathy, and has a high mortality unless liver transplantation is performed. Population-based studies are scarce, and most published data are from high-income countries, where the main cause of acute liver failure is paracetamol overdose. This Review provides an overview of the scanty literature on acute liver failure in low-income and middle-income countries, where patients are often admitted to primary care hospitals and viral hepatitis (especially hepatitis E), tropical infections (eg, dengue), traditional medicines, and drugs (especially anti-tuberculosis drugs) have an important role...
November 2023: Lancet. Gastroenterology & Hepatology
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