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hepatic failure children

Timothee Bonifay, Christelle Prince, Clarisse Neyra, Magalie Demar, Dominique Rousset, Hatem Kallel, Mathieu Nacher, Félix Djossou, Loïc Epelboin
BACKGROUND: French Guiana (FG) was the first country in South America to declare chikungunya virus infection (CHIKV). The outbreak affected about 16,000 persons between February 2014 and October 2015, with several atypical cases, but only two fatal cases. We aimed to describe the clinical presentation of patients hospitalized for CHIKV infection, to estimate and identify risk factors of unusual and severe forms in adult patients. MATERIALS AND METHODS: A monocentric retrospective study was conducted in Cayenne hospital, the main city and the main hospital in FG, from March 1st 2014 to August 31st 2015...
2018: PloS One
Katherine D Culbreath, Alejandro V Garcia, Ira L Leeds, Mitchell R Ladd, Todd Crawford, Emily Boss, Daniel S Rhee
INTRODUCTION: Complete tumor resection of primary malignant liver tumors offers the best chance of survival. However, many of these children may experience anemia and failure to thrive. This study analyzes the association of preoperative anemia and nutritional support with outcomes in children undergoing major resection of primary malignant liver tumors. METHODS: Using the National Surgical Quality Improvement Program Pediatric database from 2012 to 2015, children undergoing major liver resections for primary malignant hepatic tumors were selected...
December 2018: Journal of Surgical Research
I Astadicko, M F Dresse, M C Seghaye
Sickle cell disease (SCD) is a genetic disorder due to an abnormal gene coding for the chain ? of the hemoglobin. The main clinical manifestations related to the major forms of SCD (SS-, SC-, and S-thalassemia) are chronic hemolysis, susceptibility to infections and vasoconstrictive crisis causing micro-emboli and/or infarction responsible for acute or chronic organ lesions. The latest are enhanced by tissue iron overload due to repeated blood transfusions. Cardiac complications are an important part of morbidity and mortality of SCD...
November 2018: Revue Médicale de Liège
Meena Sivasankaran, M Venkatadesikalu, V Mythili, Srinivas Sankaranarayanan, Dhaarani Jayaraman, Shivani Patel, Venkateswaran Vellaichamy Swaminathan, Ramya Uppuluri, Revathi Raj
Transfusion-transmitted hepatitis C is a major concern among thalassemia patients. Our aim is to estimate the prevalence of Hepatitis C infection among thalassemia patients and to assess the treatment response, adverse effects of Peg-interferon based regimen and the new direct-acting antiviral drugs. Patients with thalassemia receiving regular blood transfusions with positive anti HCV antibodies during a period from January 2012 to June 2017 were analyzed. Serial HCV viral load and genotype and liver function tests were performed...
October 2018: Indian Journal of Hematology & Blood Transfusion
Karin Amrein, Alja Papinutti, Erwin Mathew, Greisa Vila, Dhruv Parekh
The prevalence of vitamin D deficiency in intensive care units ranges typically between 40 and 70 %. There are many reasons for being or becoming deficient in the ICU. Hepatic, parathyroid and renal dysfunction aditionally increase the risk for developing vitamin D deficiency. Moreover, therapeutic interventions like fluid resuscitation, dialysis, surgery, extracorporeal membrane oxygenation, cardiopulmonary bypass and plasma exchange may significantly reduce vitamin D levels. Many observational studies have consistently shown an association between low vitamin D levels and poor clinical outcomes in critically ill adults and children, including excess mortality and morbidity such as acute kidney injury, acute respiratory failure, duration of mechanical ventilation and sepsis...
October 1, 2018: Endocrine Connections
Christiane Sokollik, Valerie A McLin, Diego Vergani, Benedetta Terziroli Beretta-Piccoli, Giorgina Mieli-Vergani
Autoimmune hepatitis (AIH) is a rare, chronic disease that affects both adults and children, including infants. The disease is probably triggered by environmental factors in genetically predisposed individuals. The clinical presentation ranges from asymptomatic patients or patients with non-specific symptoms, such as fatigue, to fulminant liver failure, many children presenting with symptoms indistinguishable from those of acute hepatitis. Raised transaminase and immunoglobulin G (IgG) levels, in association with circulating autoantibodies, guide towards the diagnosis...
October 19, 2018: Journal of Autoimmunity
Ibrar Khan, Muhammad Asif Qureshi, Fowad Karim, Mahmood Shaukat
Progressive familial intrahepatic cholestasis (PFIC) is one of the causes of childhood end stage liver disease. It is an autosomal recessive disorder, characterized by pruritus, coagulopathy, growth retardation, jaundice, and subsequently cirrhosis and hepatic failure due to impaired bile acid transport and metabolism. Diversion of bile, internally or externally, from the terminal ileum, to decrease re-uptake, is a viable option for relieving pruritus. Four children with PFIC type1 were treated with partial internal biliary diversion (PIBD) from June 2014 To March 2017 in the Unit of Paediatric surgery, Jinnah Hospital Lahore...
June 2018: JPMA. the Journal of the Pakistan Medical Association
Tavleen Sandhu, Michelle Polan, Zhongxin Yu, Rufei Lu, Abhishek Makkar
Glycogen storage disease type IV (GSD-IV), or Andersen disease, is a rare autosomal recessive disorder that results from the deficiency of glycogen branching enzyme (GBE). This in turn results in accumulation of abnormal glycogen molecules that have longer outer chains and fewer branch points. GSD-IV manifests in a wide spectrum, with variable phenotypes depending on the degree and type of tissues in which this abnormal glycogen accumulates. Typically, GSD-IV presents with rapidly progressive liver cirrhosis and death in early childhood...
October 12, 2018: JIMD Reports
Şükrü Güngör, Mukadder Ayşe Selimoğlu, Fatma İlknur Varol, Serdal Güngör
BACKGROUND: Wilson's disease (WD) may present with different manifestations: from an asymptomatic state to liver cirrhosis. Here, we aimed to evaluate clinical presentations and laboratory findings and prognoses among WD cases. DESIGN AND SETTING: Cross-sectional study based on patients' records from the university hospital, İnönü University, Malatya, Turkey. METHODS: The medical records of 64 children with WD were evaluated focusing on the clinical, laboratory and liver biopsy findings in different clinical presentations...
July 2018: São Paulo Medical Journal, Revista Paulista de Medicina
Feng Wang, Wenyu Kang, Wenting Zhou, Qiudong Su, Shengli Bi, Feng Qiu, Qiongfen Li
OBJECTIVE: This study aimed to investigate HBsAg positive rates and risk factors of HBV infection among the children less than 15 years old in Yunnan province, a remote southwest part of mainland China. METHODS: Multi-stage sampling was used to randomly select study subjects from 9,360,000 individuals. Hepatitis B vaccine inoculation rate and HBsAg positive rate were investigated, and then propensity score and generalized linear mixed model (GLMMs) were applied to the case-control study...
October 5, 2018: International Journal of Infectious Diseases: IJID
Charlie Michaudet, John Malaty
Cerumen production is a normal and protective process for the ear canal. However, cerumen should be removed when it causes symptoms (e.g., hearing loss, itching, pain, tinnitus) or prevents assessment of the external auditory canal, the tympanic membrane, or audiovestibular system. Cerumen should also be removed when it limits examination in patients who cannot communicate their symptoms, such as those with dementia or developmental delay, nonverbal patients with behavioral changes, and young children with fever, speech delay, or parental concerns...
October 15, 2018: American Family Physician
Anthony P Y Liu, Janice J K Ip, Alex W K Leung, C W Luk, C H Li, Karin K H Ho, Regina Lo, Edwin K W Chan, Albert C Y Chan, Patrick H Y Chung, Alan K S Chiang
BACKGROUND AND AIM: We reviewed the results and pattern of failure of the consensus HB/HCC 1996 treatment protocol for pediatric hepatoblastoma (HB) in Hong Kong. The role of SIOPEL and Children's Hepatic tumors International Collaboration (CHIC) risk stratification was evaluated. METHODS: Patients enrolled on the protocol from 1996 to 2014 were included. PRETEXT staging, SIOPEL, and CHIC risk groups were retrospectively assigned. RESULTS: Sixty patients were enrolled with median age at diagnosis of 1...
September 30, 2018: Pediatric Blood & Cancer
Sumit Kumar Singh, Vibhor Borkar, Anshu Srivastava, Amrita Mathias, Surender Kumar Yachha, Ujjal Poddar
Various atypical manifestations have been described in acute viral hepatitis (AVH). We evaluated the prevalence, clinical features, response to treatment and outcome of various atypical manifestations of AVH in children. Consecutive children (≤ 18 years) with AVH due to hepatitis A, B, or E were studied while patients with acute or acute on chronic liver failure were excluded. Diagnosis of atypical manifestations was based on standard criteria. A total of 477 children with AVH (median age 7.0 (5-11) years, 74% boys) were seen; 22% (n = 106) had atypical manifestations...
September 29, 2018: European Journal of Pediatrics
Kevin J Zuo, Arman Draginov, Andre Panossian, Annie Fecteau, Gregory H Borschel, Emily S Ho, Ronald M Zuker
BACKGROUND: Living donor liver transplantation (LDLT) is an important strategy of procuring segmental liver allografts for pediatric patients with liver failure, as suitably sized whole donor organs are scarce. Early pediatric LDLT experience was associated with high rates of hepatic artery thrombosis, graft loss, and mortality. Collaboration with microsurgeons for hepatic artery anastomosis in pediatric LDLT has decreased rates of arterial complications; however, reported outcomes are limited...
September 4, 2018: Plastic and Reconstructive Surgery
Peter L Bonate, Tianli Wang, Paul Passier, Wilhelmina Bagchus, Howard Burt, Christian Lüpfert, Nada Abla, Jana Kovac, Jennifer Keiser
L-praziquantel (PZQ) pharmacokinetic data were analyzed from two relative bioavailability Phase 1 studies in adult, healthy subjects with two new oral dispersion tablet (ODT) formulations of L-PZQ administered under various combinations of co-administration with food, water, and/or crushing. Linear mixed effects models adequately characterized the noncompartmental estimates of the pharmacokinetic profiles in both studies. Dose, food, and formulation were found to significantly affect L-PZQ exposure in both studies...
September 14, 2018: Journal of Pharmacokinetics and Pharmacodynamics
Margot Chima, Mark Lebwohl
Tumor necrosis factor (TNF)-α has been identified as a key cytokine mediating cutaneous inflammation in the pathogenesis of psoriasis. The TNF inhibitors (TNFi's) infliximab, adalimumab, and etanercept are efficacious, Food and Drug Administration-approved medications for the treatment of moderate-to-severe plaque psoriasis. Each drug has a unique pharmacological profile that can have therapeutic implications when choosing a particular TNFi for a patient. An understanding of these idiosyncrasies can help guide therapeutic decisions for patients with psoriasis that also have inflammatory bowel disease, hepatitis C, hepatitis B, latent tuberculosis, obesity, cardiovascular disease, and heart failure...
September 2018: Seminars in Cutaneous Medicine and Surgery
M Szymczak, P Kaliciński, G Kowalewski, M Markiewicz-Kijewska, D Broniszczak, H Ismail, M Stefanowicz, A Kowalski, J Rubik, I Jankowska, B Piątosa, J Teisseyre, R Grenda
Combined liver-kidney transplantation (CLKT) is a rare procedure in pediatric patients in which liver and kidney from 1 donor are transplanted to a recipient during a single operation. The aim of our study was to analyze indications and results of CLKT in children. MATERIALS AND METHODS: Between 1990 and 2017 we performed 722 liver transplantations in children; we performed 920 kidney transplantations in children since 1984. Among them, 25 received CLKT. Primary diagnosis was fibro-polycystic liver and kidney disease in 17 patients, primary hyperoxaluria type 1 in 6 patients, and atypical hemolytic uremic syndrome-related renal failure in 2 children...
September 2018: Transplantation Proceedings
Le Y Lee, Si M Chan, Christina Ong, Marion M Aw, Franco Wong, Sharon Saw, Guan H Lee, Koh C Thoon, Kong B Phua
AIM: We compared the vaccine effectiveness of monovalent and combination hepatitis B vaccine regimens in infants born to chronic hepatitis B carrier mothers. METHODS: An observational cohort of neonates was recruited over 78 months from two public hospital maternity units in Singapore. We enrolled term infants, born to chronic hepatitis B surface antigen-positive mothers regardless of their hepatitis Be antigen status, who completed the hepatitis B virus (HBV) vaccination programme in Singapore...
August 30, 2018: Journal of Paediatrics and Child Health
Michael V Di Maria, David W Brown, Frank Cetta, Salil Ginde, David Goldberg, Shaji C Menon, Heather M Phelps, Jack Rychik, Kurt R Schumacher, Philip Thrush, Gruschen Veldtman, Gail Wright, Adel K Younoszai
More children with single ventricle heart disease are surviving after Fontan surgery. This circulation has pervasive effects on multiple organ systems and has unique modes of failure. Many centers have created multidisciplinary programs to care for these patients. Our aim was to survey such programs to better understand current approaches to care. We hypothesized that significant variability in surveillance testing strategy would be present. Eleven academic institutions with established Fontan care programs performing a combined estimated 300 Fontan surgeries per year, with a total population of 1500-2000 Fontan patients, were surveyed using a REDCap survey regarding surveillance testing and basic practice philosophies...
August 29, 2018: Pediatric Cardiology
Katia Perruccio, Luisa Sisinni, Antonio Perez-Martinez, Jaime Valentin, Ilaria Capolsini, Maria Speranza Massei, Maurizio Caniglia, Simone Cesaro
Human herpesvirus-6 (HHV-6) infection is increasingly recognized among allogeneic hematopoietic stem cell transplantation (HSCT) recipients, with 30% at risk of reactivation in the haploidentical setting. It has been associated with encephalitis, acute graft-versus-host disease, and graft failure. Here we report 2 cohorts of pediatric haploidentical manipulated HSCT in which, despite many differences, HHV-6 reactivation and disease occurred with very high incidence compared with data reported in the literature and represented the main early post-transplant infectious complication compared with other viral, bacterial, or fungal infections...
December 2018: Biology of Blood and Marrow Transplantation
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