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hepatic failure children

Katia Perruccio, Luisa Sisinni, Antonio Perez-Martinez, Jaime Valentin, Ilaria Capolsini, Maria Speranza Massei, Maurizio Caniglia, Simone Cesaro
BACKGROUND: Human herpesvirus (HHV-6) infection is increasingly recognized among allogeneic hematopoietic stem cell transplantation (HSCT) recipients, 30% being the risk of reactivation in the haploidentical setting. It has been associated with encephalitis, acute graft-versus-host disease (GvHD) and graft failure. Here we report two cohorts of pediatric haploidentical manipulated HSCT in which, despite many differences, HHV-6 reactivation and disease occurred with very high incidence compared to data reported in literature, and represented the main early post-transplant infectious complication compared to other viral, bacterial or fungal infections...
July 29, 2018: Biology of Blood and Marrow Transplantation
Magd A Kotb, Lobna Mansour, Christine William Shaker Basanti, Wael El Garf, Ghada I Z Ali, Sally T Mostafa El Sorogy, Inas E M Kamel, Naglaa M Kamal
Classic galactosemia is caused by deficiency of galactose-1-phosphate uridylyltransferase (GALT). It causes serious morbidity and mortality if left untreated. Screening for galactosemia is not included in Egyptian neonatal screening program. The study aimed to define clinical presentation and complications of galactosemia at Pediatric Hepatology Clinic, Cairo University, Egypt. Thus, the clinical presentation, course and outcome of 37 children with documented galactosemia was studied. Jaundice was the main presentation (67...
July 2018: Journal of Advanced Research
Angelo Di Giorgio, Emanuele Nicastro, Davide Dalla Rosa, Gabriella Nebbia, Aurelio Sonzogni, Lorenzo D'Antiga
BACKGROUND: in adults, the absence of a preexisting chronic liver disease (CLD) is required to diagnose acute liver failure (ALF). The paediatric classification does not considered this aspect, thus previous studies pooled together children with ALF and children with unknown CLD presenting with acute hepatic decompensation (ALF-CLD). We aimed to compare prevalence, features and outcome of children with ALF-CLD to those with a proper ALF. METHODS: Patients admitted between 1996-2017 because of ALF defined by PALF criteria (raised transaminases, INR ≥2...
July 19, 2018: Transplantation
Jingyang Yin, Zuojin Liu, Kang Yang
RATIONALE: Rhabdomyosarcoma (RMS), a malignant tumor with striated muscle differentiation, is the most common type of soft-tissue sarcoma in children and adolescents, but rarely occurs in adults, and especially in human livers. Moreover, this disease has a very poor prognosis. Here we report a case of primary RMS of the liver in a 66-year-old woman. This case is rare with respect to the location and clinical course of the tumor. The tumor had enlarged rapidly, ruptured, and eventually caused the patient's death after a long history of a stable abdominal mass that indicated a hepatic cyst...
July 2018: Medicine (Baltimore)
Alireza Shamsaeefar, Taiser Saleh, Kourosh Kazemi, Saman Nikeghbalian, Masood Dehghani, Mohsen Mansurian, Siavash Gholam, Bahareh Khosravi, Seyyed Ali Malek Hosseini
OBJECTIVES: Liver transplant is the most effective treatment modality for patients with end-stage liver disease, metabolic disorders, hepatic malignancy, and acute liver failure. When a graft fails after primary liver transplant, retransplant of the liver remains the only option. Here, we report the past 12-year experience of the Shiraz Transplant Center regarding liver retransplant. MATERIALS AND METHODS: This is a retrospective cohort study of a 12-year period (2004-2015) of the Shiraz Center in Iran...
July 11, 2018: Experimental and Clinical Transplantation
Shashi N Kapadia, Kristen M Marks
This article proposes a strategy for primary care providers to begin treating patients with hepatitis C virus (HCV). We are motivated by the need to expand HCV treatment and by developments that have simplified treatment for most patients. This article presents 5 steps to achieving quality HCV treatment in the primary care setting: (1) accurate diagnosis via reflex testing; (2) risk stratification and identifying comorbidities via pretreatment evaluation; (3) simple, once-daily, pan-genotypic HCV treatment regimens; (4) minimized on-treatment monitoring: and (5) posttreatment monitoring and high-quality care for comorbidities such as cirrhosis and injection drug use...
July 5, 2018: Clinical Therapeutics
Abdulkerim Temiz, Semire Serin Ezer, Murat Gedikoğlu, Ender Serin, Emine İnce, Hasan Özkan Gezer, Mehmet Oğuz Canan, Akgün Hiçsönmez
PURPOSE: Pediatric experience with biliary tract injuries (BTI) is limited and mostly consists of case presentations. The purpose of this study is to evaluate clinical and radiological findings of possible BTI, treatment strategies, and results. METHODS: The records of nine patients with the diagnosis of BTI between July 2009 and November 2017 were reviewed retrospectively. RESULTS: There were seven boys and two girls (mean 8.05 ± 4.39 years)...
August 2018: Pediatric Surgery International
Paul G Richardson, Enric Carreras, Massimo Iacobelli, Bijan Nejadnik
Hepatic veno-occlusive disease/sinusoidal obstruction syndrome (VOD/SOS) is a potentially life-threatening complication of conditioning during hematopoietic stem cell transplantation (HSCT) or chemotherapy without HSCT, with a historically reported mean incidence of 13.7% post-HSCT. Typical symptoms of VOD/SOS may include hyperbilirubinemia, painful hepatomegaly, weight gain, and ascites. Defibrotide, a polydisperse mixture of predominantly single-stranded polydeoxyribonucleotides, is currently the only therapy approved to treat hepatic VOD/SOS with pulmonary/renal dysfunction (ie, multiorgan dysfunction/multiorgan failure [MOD/MOF]) following HSCT in the United States and to treat severe hepatic VOD/SOS post-HSCT in the European Union...
June 26, 2018: Blood Advances
Alexander Nast, Lasse Amelunxen, Matthias Augustin, Wolf-Henning Boehncke, Corinna Dressler, Matthew Gaskins, Peter Härle, Bernd Hoffstadt, Joachim Klaus, Joachim Koza, Ulrich Mrowietz, Hans-Michael Ockenfels, Sandra Philipp, Kristian Reich, Thomas Rosenbach, Berthold Rzany, Martin Schlaeger, Gerhard Schmid-Ott, Michael Sebastian, Ralph von Kiedrowski, Tobias Weberschock
The German guideline for the treatment of psoriasis vulgaris was updated using GRADE methodology. The guideline is based on a systematic literature review completed on December 1, 2016, and on a formal consensus and approval process. The second part of this short version of the guideline covers the following special patient populations and treatment situations: tuberculosis screening before and during psoriasis treatment, choice of psoriasis treatment for individuals wishing to have children, as well as during pregnancy and breast-feeding, and patients with joint involvement and vaccinations...
June 2018: Journal der Deutschen Dermatologischen Gesellschaft, Journal of the German Society of Dermatology: JDDG
Nesreen A Faqih, Mahmoud A Alfaqih, Khadra Salami, Brian Herron, Iyad Sultan, Maysa Al-Hussaini
Introduction: Hepatitis secondary to Herpes Simplex Virus (HSV) infection is a complication that often leads to fatal hepatic failure. Early treatment with the anti-viral drug, acyclovir, is life-saving. In view of the non-specific nature of the signs and symptoms associated with HSV hepatitis, diagnosis is often made late during the course of the disease; a factor that largely contributes to the high mortality rate of this treatable disease complication. There is thus a growing consensus in the field to initiate empirical treatment with acyclovir once suspicion of HSV hepatitis is raised even before reaching a conclusive diagnosis...
2018: IDCases
Helene Mygind Wolsk, Marianne Hørby Jørgensen, Susan Nielsen
Children with rheumatic diseases often have elevated liver biochemistry. This can be triggered by medical treatment, e.g. methotrexate can induce liver injury ranging from mild to severe. Autoimmune hepatitis and sclerosing cholangitis are also seen in conjunction with rheumatic diseases, and early diagnosis and treatment is crucial to prevent development of cirrhosis and liver transplantation. Macrophage activation syndrome is a rare but important differential diagnosis as it is a potentially fatal complication to systemic rheumatic diseases, causing liver dysfunction and multi-organ failure...
May 28, 2018: Ugeskrift for Laeger
Silvia Nastasio, Marco Sciveres, Lorenza Matarazzo, Giuseppe Maggiore
Autoimmune hepatitis is a rare inflammatory disease of the liver that most frequently affects children and young adults. It is a multifactorial disease of unknown etiology, characteristically progressive in nature, and if left untreated, may lead to cirrhosis and terminal liver failure. It has been known for several decades now that immunosuppressive treatment convincingly alters the outcome of most patients with autoimmune hepatitis and as such it should be started as soon as diagnosis is made. Primary goals of treatment are: normalization of hepatocellular function, extinction of the hepatic necroinflammatory process, and maintenance of a stable remission, thus preventing progression to cirrhosis and its complications...
May 16, 2018: Current Pediatric Reviews
Amin Sheikh, Kai Y Chau, Helen M Evans
Histological abnormalities, including chronic hepatitis, fibrosis, and steatosis, are increasingly reported in liver biopsies of children after LT. These changes may be progressive and represent a form of rejection. Liver biochemistry is often initially normal. Our LT program began in 2002, utilizing tacrolimus and low-dose steroids for the first year post-LT. Patients undergo a protocol biopsy at 1 year post-LT prior to stopping steroids, then at 5 years and every 5 years thereafter. Target tacrolimus levels are 5-8 μg/L and 3-5 μg/L after 3 and 12 months, respectively...
August 2018: Pediatric Transplantation
Ryan P Barbaro, Philip S Boonstra, Kevin W Kuo, David T Selewski, David K Bailly, Cheryl L Stone, Chin Ying Chow, Gail M Annich, Frank W Moler, Matthew L Paden
This study evaluates whether three commonly used pediatric intensive care unit (PICU) severity of illness scores, pediatric risk of mortality score (PRISM) III, pediatric index of mortality (PIM) 2, and pediatric logistic organ dysfunction (PELOD), are the appropriate tools to discriminate mortality risk in children receiving extracorporeal membrane oxygenation (ECMO) support for respiratory failure. This study also evaluates the ability of the Pediatric Risk Estimate Score for Children Using Extracorporeal Respiratory Support (Ped-RESCUERS) to discriminate mortality risk in the same population, and whether Ped-RESCUERS' discrimination of mortality is improved by additional clinical and laboratory measures of renal, hepatic, neurologic, and hematologic dysfunction...
May 8, 2018: ASAIO Journal: a Peer-reviewed Journal of the American Society for Artificial Internal Organs
Fiona Fonseca, Bijal Kulkarni, Meenal Hastak, Vinay Kumaran, Vibha Varma, Sorabh Kapoor
INTRODUCTION AND AIM: 1. Study of liver explants - Etiologic types of end-stage chronic liver disease (ESCLD) and acute liver failure (ALF) in adults and children. 2. Assessment of donor steatosis and incidental granulomas. 3. Post-transplant liver biopsies. MATERIAL AND METHODS: Specimens of 180 explant hepatectomies, 173 donor wedge and 30 core liver biopsies, and 58 post transplant liver biopsies received in our department from April 2013 to March 2017. RESULTS: 1...
May 2018: Annals of Hepatology
Rakesh Aggarwal, Amit Goel
Infection with genotype 1 or 2 hepatitis E virus (HEV) results primarily from human-to-human transmission through the fecal-oral route in low-resource countries. It presents primarily as "acute viral hepatitis" syndrome, usually a self-limiting illness. A few cases progress to acute liver failure, a serious illness with high fatality. Clinical disease is infrequent among children. Infection during pregnancy is associated with a higher risk of symptomatic disease, severe liver injury, and mortality...
May 7, 2018: Cold Spring Harbor Perspectives in Medicine
Hakan Leblebicioglu, Resat Ozaras
Hepatitis E virus (HEV), a non-enveloped single stranded RNA virus causes sporadic cases of hepatitis or outbreaks. The disease is generally self-limited although it may cause fulminant hepatitis in pregnant women, elderly, those with underlying chronic hepatitis, immunosuppressed, and transplant recipients. It is transmitted through fecal-oral route and zoonotic transmission. Hepatitis is a main health care problem in Turkey; HBV and HCV prevalences are 4 and 1% respectively. Hepatitis D represents another considerable hepatitis etiology with a prevalence of 5-27%...
May 2, 2018: Annals of Clinical Microbiology and Antimicrobials
Arvind Kumar Bishnoi, Pankaj Garg, Kartik Patel, Chandrasekaran Ananthanarayanan, Ritesh Shah, Atul Solanki, Himani Pandya, Sanjay Patel
BACKGROUND: Retrospective reviews have found the use of stored packed red blood cells (PRBCs) in priming to be associated with increased risk of postoperative complications. The purpose of study was to prospectively investigate the influence of duration of storage of PRBCs used in priming the cardiopulmonary bypass (CPB) circuit on the metabolic profile of the patients, and postoperative outcome after paediatric cardiac surgery. METHODS: Between January 2015 and December 2015, 198 consecutive children operated for cardiac surgery using CPB and received blood for priming the circuit were included...
March 31, 2018: Heart, Lung & Circulation
Adel Ghorani-Azam, Samaneh Sepahi, Bamdad Riahi-Zanjani, Anahita Alizadeh Ghamsari, Seyed Ahmad Mohajeri, Mahdi Balali-Mood
Background: For many years, medicinal plants and herbal therapy have been widely used in different societies for the treatment of various diseases. Besides their therapeutic potency, some of the medicinal plants have strong toxicity in human, especially in children and elderly. Despite common beliefs that natural products are safe, there have been few reports on their toxicities. Materials and Methods: In the present study, we aimed to systematically review the literature wherein acute plant poisoning and herbal intoxication have been reported in pediatric patients...
2018: Journal of Research in Medical Sciences: the Official Journal of Isfahan University of Medical Sciences
Bárbara Neto, Mariana Borges-Dias, Eunice Trindade, José Estevão-Costa, José Miguel Campos
Introduction: Biliary atresia is the main cause of death by hepatic failure and the main indication for liver transplant in children. This study aims to analyze the population with this diagnosis, treated between 2000 and 2015 at Hospital de São João. Material and Methods: Descriptive, observational, and retrospective study, including the patients with biliary atresia, diagnosed and treated between January 1, 2000 and December 31, 2015. We analyzed epidemiologic, clinical, biochemical, and image data, as well as registered complications and present status...
March 2018: GE Portuguese Journal of Gastroenterology
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