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https://www.readbyqxmd.com/read/30323368/surgical-treatment-for-intractable-pruritus-in-progressive-familial-intrahepatic-cholestasis
#1
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
https://www.readbyqxmd.com/read/30311141/case-of-neonatal-fatality-from-neuromuscular-variant-of-glycogen-storage-disease-type-iv
#2
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
https://www.readbyqxmd.com/read/30304203/pediatric-wilson-s-disease-findings-in-different-presentations-a-cross-sectional-study
#3
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
https://www.readbyqxmd.com/read/30296573/investigation-of-the-risk-factors-associated-with-the-failure-of-hepatitis-b-vaccination-of-neonates-in-yunnan-province-china
#4
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, remote southwest part of China mainland. METHODS: Multi-stage sampling was used to randomly select study subjects from 9360000 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
https://www.readbyqxmd.com/read/30277727/cerumen-impaction-diagnosis-and-management
#5
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
https://www.readbyqxmd.com/read/30270490/treatment-outcome-and-pattern-of-failure-in-hepatoblastoma-treated-with-a-consensus-protocol-in-hong-kong
#6
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
https://www.readbyqxmd.com/read/30269249/need-for-recognizing-atypical-manifestations-of-childhood-sporadic-acute-viral-hepatitis-warranting-differences-in-management
#7
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
https://www.readbyqxmd.com/read/30239502/microvascular-hepatic-artery-anastomosis-in-pediatric-living-donor-liver-transplantation-73-consecutive-cases-by-a-single-surgeon
#8
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
https://www.readbyqxmd.com/read/30218416/extrapolation-of-praziquantel-pharmacokinetics-to-a-pediatric-population-a-cautionary-tale
#9
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
https://www.readbyqxmd.com/read/30215629/tnf-inhibitors-for-psoriasis
#10
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
https://www.readbyqxmd.com/read/30177126/combined-liver-kidney-transplantation-in-children-single-center-experiences-and-long-term-results
#11
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
https://www.readbyqxmd.com/read/30161273/comparing-monovalent-and-combination-hepatitis-b-vaccine-outcomes-in-children-delivered-by-mothers-with-chronic-hepatitis-b
#12
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
https://www.readbyqxmd.com/read/30159585/surveillance-testing-and-preventive-care-after-fontan-operation-a-multi-institutional-survey
#13
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
https://www.readbyqxmd.com/read/30067953/high-incidence-of-early-human-herpesvirus-6-infection-in-children-undergoing-haploidentical-manipulated-stem-cell-transplantation-for-hematologic-malignancies
#14
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...
July 29, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/30038819/pilot-study-of-classic-galactosemia-neurodevelopmental-impact-and-other-complications-urge-neonatal-screening-in-egypt
#15
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
https://www.readbyqxmd.com/read/30028785/transplant-free-survival-in-chronic-liver-disease-presenting-as-acute-liver-failure-in-childhood
#16
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
https://www.readbyqxmd.com/read/30024506/pleomorphic-rhabdomyosarcoma-of-the-liver-with-a-hepatic-cyst-in-an-adult-case-report-and-literature-review
#17
REVIEW
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)
https://www.readbyqxmd.com/read/29993357/retransplant-of-the-liver-12-year-experience-of-the-shiraz-organs-transplantation-center
#18
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
https://www.readbyqxmd.com/read/29983266/hepatitis-c-management-simplification-from-test-to-cure-a-framework-for-primary-care-providers
#19
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...
August 2018: Clinical Therapeutics
https://www.readbyqxmd.com/read/29948144/management-of-traumatic-bile-duct-injuries-in-children
#20
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
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