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Failure hepatic pediatric

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https://www.readbyqxmd.com/read/29022907/p38%C3%AE-mapk-antagonizing-jnk-to-control-the-hepatic-fat-accumulation-in-pediatric-patients-onset-intestinal-failure
#1
Yongtao Xiao, Jun Wang, Weihui Yan, Kejun Zhou, Yi Cao, Wei Cai
The p38α mitogen-activated protein kinase (MAPK) has been related to gluconeogenesis and lipid metabolism. However, the roles and related mechanisms of p38α MAPK in intestinal failure (IF)-associated liver steatosis remained poor understood. Here, our experimental evidence suggested that p38α MAPK significantly suppressed the fat accumulation in livers of IF patients mainly through two mechanisms. On the one hand, p38α MAPK increased hepatic bile acid (BA) synthesis by upregulating the expression of the rate-limiting enzyme cholesterol 7-α-hydroxylase (CYP7A1) and peroxisome proliferator-activated receptor γ coactivator-1α (PGC-1α), which in turn activated the transcription of the CYP7A1...
October 12, 2017: Cell Death & Disease
https://www.readbyqxmd.com/read/29019615/haemodynamic-profiles-of-children-with-end-stage-heart-failure
#2
Sharon Chen, John C Dykes, Doff B McElhinney, Robert J Gajarski, Andrew Y Shin, Seth A Hollander, Melanie E Everitt, Jack F Price, Ravi R Thiagarajan, Steven J Kindel, Joseph W Rossano, Beth D Kaufman, Lindsay J May, Elizabeth Pruitt, David N Rosenthal, Christopher S Almond
Aims: To evaluate associations between haemodynamic profiles and symptoms, end-organ function and outcome in children listed for heart transplantation. Methods and results: Children <18 years listed for heart transplant between 1993 and 2013 with cardiac catheterization data [pulmonary capillary wedge pressure (PCWP), right atrial pressure (RAP), and cardiac index (CI)] in the Pediatric Heart Transplant Study database were included. Outcomes were New York Heart Association (NYHA)/Ross classification, renal and hepatic dysfunction, and death or clinical deterioration while on waitlist...
October 7, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28992518/comprehensive-detection-of-viruses-in-pediatric-patients-with-acute-liver-failure-using-next-generation-sequencing
#3
Takako Suzuki, Jun-Ichi Kawada, Yusuke Okuno, Satoshi Hayano, Kazuhiro Horiba, Yuka Torii, Yoshiyuki Takahashi, Syuichiro Umetsu, Tsuyoshi Sogo, Ayano Inui, Yoshinori Ito
BACKGROUND: Pediatric acute liver failure (PALF) is a rare and severe syndrome that frequently requires liver transplantation. Viruses are one of the most frequent causes of this disease, however, pathogenic viruses are not determined in many patients. Recently next-generation sequencing (NGS) has been applied to comprehensively detect pathogens of infectious diseases of unknown etiology. OBJECTIVES: To evaluate an NGS-based approach for detecting pathogenic viruses in patients with PALF or acute hepatitis of unknown etiology...
October 3, 2017: Journal of Clinical Virology: the Official Publication of the Pan American Society for Clinical Virology
https://www.readbyqxmd.com/read/28988422/comparison-of-two-cytoreductive-regimens-for-%C3%AE-%C3%AE-t-cell-depleted-haploidentical-hsct-in-pediatric-malignancies-improved-engraftment-and-outcome-with-tbi-based-regimen
#4
Elad Jacoby, Nira Varda-Bloom, Gal Goldstein, Daphna Hutt, Chaim Churi, Helly Vernitsky, Amos Toren, Bella Bielorai
BACKGROUND: Graft manipulation using selective depletion of αβ-T cells provides a source of haploidentical hematopoietic stem cell transplantation (haplo-HSCT) enriched in effector cells. We report our experience implementing this haplo-HSCT for high-risk malignancies in pediatric patients focusing on the conditioning regimen. PROCEDURE: We performed a retrospective study of patients who underwent T-cell receptor αβ-depleted haplo-HSCT for high-risk pediatric malignancies...
October 8, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28937522/ambulatory-high-dose-methotrexate-administration-in-pediatric-osteosarcoma-patients-at-a-single-institution-in-argentina
#5
Jose A Villegas Rubio, Walter Cacciavillano, Adriana Rose, Pedro Zubizarreta, Marcelo Scopinaro
BACKGROUND: The purpose of this study was to evaluate the feasibility and safety of ambulatory high-dose methotrexate (HDMTX) administration with oral hydration, alkalinization, and leucovorin rescue. HDMTX (12 g/m) was given intravenously over 4 hours after urine alkalinization. Families and patients were instructed to continue ambulatory oral hydration and alkalinization to monitor urine pH and to adjust bicarbonate according to our institution's treatment algorithm. Clinical status and MTX levels were controlled every 24 hours, and oral leucovorin dose was adjusted accordingly...
October 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28914695/autoimmune-acute-liver-failure-and-seronegative-autoimmune-liver-disease-in-children-are-they-different-from-classical-disease
#6
Vikas Jain, Anshu Srivastava, Surender K Yachha, Niraj Kumari, Rachana Kathuria, Moinak S Sarma, Ujjal Poddar, Narendra Krishnani
OBJECTIVES: Presentation as autoimmune acute liver failure (AI-ALF) and seronegative autoimmune liver disease (SN-AILD) represents two uncommon variants of AILD. We compared the clinical profile and outcome of AI-ALF with autoimmune-non-acute liver failure (AI-non-ALF) and also SN-AILD with seropositive autoimmune liver disease (SP-AILD). MATERIALS AND METHODS: Children managed as AI-ALF and AI-non-ALF including SN-AILD and SP-AILD were enrolled and compared. AI-non-ALF was diagnosed by simplified diagnostic criteria and AI-ALF by Pediatric Acute Liver Failure Study Group criteria with positive autoantibody, exclusion of other etiologies, elevated immunoglobulin G and histology when available...
September 13, 2017: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28898314/-associated-factors-to-non-operative-management-failure-of-hepatic-and-splenic-lesions-secondary-to-blunt-abdominal-trauma-in-children
#7
Adriana Echavarria Medina, Carlos Hernando Morales Uribe, Luis Guillermo Echavarria R, Viviana María Vélez Marín, Jorge Alberto Martínez Montoya, David Fernando Aguillón
INTRODUCTION: The non operative management (NOM) is the standard management of splenic and liver blunt trauma in pediatric patients.Hemodynamic instability and massive transfusions have been identified as management failures. Few studies evaluate whether there exist factors allowing anticipation of these events. The objective was to identify factors associated with the failure of NOM in splenic and liver injuries for blunt abdominal trauma. PATIENTS AND METHOD: Retrospective analysis between 2007-2015 of patients admitted to the pediatric surgery at University Hospital Saint Vincent Foundation with liver trauma and/or closed Spleen...
2017: Revista Chilena de Pediatría
https://www.readbyqxmd.com/read/28870776/consensus-report-by-the-pediatric-acute-lung-injury-and-sepsis-investigators-and-pediatric-blood-and-marrow-transplantation-consortium-joint-working-committees-on-supportive-care-guidelines-for-management-of-veno-occlusive-disease-in-children-and-adolescents
#8
Nadia Ovchinsky, Warren Frazier, Jeffery J Auletta, Christopher C Dvorak, Monica Ardura, Enkyung Song, Jennifer McArthur, Asumthia Jeyapalan, Robert Tamburro, Kris M Mahadeo, Chani Traube, Christine N Duncan, Rajinder P S Bajwa
Some patients with veno-occlusive disease (VOD) have multiorgan dysfunction, and multiple teams are involved in their daily care in the pediatric intensive care unit. Cardiorespiratory dysfunction is critical in these patients, requiring immediate action. The decision of whether to use a noninvasive or an invasive ventilation strategy may be difficult in the setting of mucositis or other comorbidities in patients with VOD. Similarly, monitoring of organ function may be very challenging in these patients, who may have fulminant hepatic failure with or without hepatic encephalopathy complicated by delirium and/or infections...
September 1, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28859030/treatment-of-refractory-infantile-hemangiomas-and-pulmonary-hypertension-with-sirolimus-in-a-pediatric-patient
#9
Kelley K Hutchins, Robert D Ross, Daisuke Kobayashi, Alissa Martin, Madhvi Rajpurkar
Infantile hemangioma is a benign vascular neoplasm that spontaneously involutes over time. Management, when needed, consists of medications, laser treatment and surgical excision. We describe a 3-year-old girl who presented shortly after birth with diffuse cutaneous hemangiomas, hepatosplenomegaly with liver lesions, anemia, and acute heart failure. She was diagnosed with hepatic and cutaneous infantile hemangioma based on skin biopsy. She developed progressive pulmonary hypertension with numerous pulmonary nodules suspicious for pulmonary arteriovenous malformations...
October 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28774273/thrombotic-microangiopathy-associated-with-valproic-acid-toxicity
#10
Sean A Hebert, Timothy P Bohan, Christian L Erikson, Rita D Swinford
BACKGROUND: Thrombotic microangiopathy (TMA) is a serious, sometimes life-threatening disorder marked by the presence of endothelial injury and microvascular thrombi. Drug-induced thrombotic microangiopathy (DI-TMA) is one specific TMA syndrome that occurs following drug exposure via drug-dependent antibodies or direct tissue toxicity. Common examples include calcineurin inhibitors Tacrolimus and Cyclosporine and antineoplastics Gemcitabine and Mitomycin. Valproic acid has not been implicated in DI-TMA...
August 3, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28759025/diagnosis-and-severity-criteria-for-sinusoidal-obstruction-syndrome-veno-occlusive-disease-in-pediatric-patients-a-new-classification-from-the-european-society-for-blood-and-marrow-transplantation
#11
S Corbacioglu, E Carreras, M Ansari, A Balduzzi, S Cesaro, J-H Dalle, F Dignan, B Gibson, T Guengoer, B Gruhn, A Lankester, F Locatelli, A Pagliuca, C Peters, P G Richardson, A S Schulz, P Sedlacek, J Stein, K-W Sykora, J Toporski, E Trigoso, K Vetteranta, J Wachowiak, E Wallhult, R Wynn, I Yaniv, A Yesilipek, M Mohty, P Bader
The advances in hematopoietic cell transplantation (HCT) over the last decade have led to a transplant-related mortality below 15%. Hepatic sinusoidal obstruction syndrome/veno-occlusive disease (SOS/VOD) is a life-threatening complication of HCT that belongs to a group of diseases increasingly identified as transplant-related, systemic endothelial diseases. In most cases, SOS/VOD resolves within weeks; however, severe SOS/VOD results in multi-organ dysfunction/failure with a mortality rate >80%. A timely diagnosis of SOS/VOD is of critical importance, given the availability of therapeutic options with favorable tolerability...
July 31, 2017: Bone Marrow Transplantation
https://www.readbyqxmd.com/read/28705427/invasive-disease-by-streptococcus-pyogenes-patients-hospitalized-for-6-years
#12
Vanessa Arias-Constantí, Victoria Trenchs-Sainz de la Maza, Nuria Elvira Sanz-Marcos, Carmina Guitart-Pardellans, Amadeu Gené-Giralt, Carles Luaces-Cubells
INTRODUCTION AND OBJECTIVE: The last years an increase of severe cases of invasive disease (ID) due to Streptococcus pyogenes or streptococcus b-hemolytic group A (SGA) had been detected. The aim of this study was to analyze the epidemiology and the clinical features of ID due to SGA in a tertiary Pediatric Hospital. MATERIAL AND METHODS: Retrospective study in a Pediatric hospital, of all in-patients with final diagnosis of ID due to SGA during 6 years (2009-2014)...
July 10, 2017: Enfermedades Infecciosas y Microbiología Clínica
https://www.readbyqxmd.com/read/28669107/outcomes-and-costs-of-cardiac-surgery-in-adults-with-congenital-heart-disease
#13
Viviane G Nasr, David Faraoni, Anne Marie Valente, James A DiNardo
Advances in pediatric cardiac surgical and medical care have led to increased survival of patients with congenital heart disease (CHD). Consequently, many CHD patients survive long enough to require cardiac surgery as adults. Using the 2013 Nationwide Inpatient Sample (NIS) database, we compared costs and outcomes for adult patients undergoing surgery for treatment of CHD to a reference population of adults undergoing CABG. Patients were identified using International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9 CM) procedure codes...
July 1, 2017: Pediatric Cardiology
https://www.readbyqxmd.com/read/28664942/darunavir-stands-up-as-preferred-hiv-protease-inhibitor
#14
Josep Mallolas
Current antiretroviral therapy reaches and maintains viral suppression over the years in more than 90% of treated HIV-infected individuals. Although integrase inhibitors are the preferred third agent in antiretroviral therapy in the current guidelines, rilpivirine, a non-nucleoside reverse transcrip- tase inhibitor, and darunavir (DRV), a second-generation protease inhibitor, are the preferred third companion to be used along with a backbone of two nucleos(t)ide reverse transcriptase inhibitors as first-line triple HIV combination treatment...
April 2017: AIDS Reviews
https://www.readbyqxmd.com/read/28643816/-budd-chiari-syndrome-and-ulcerative-colitis-in-an-adolescent
#15
Federico Marchetti, Martina Mainetti, Michela Giovannini, Cristina Morelli
In patients with inflammatory bowel disease (IBD) there is an increased incidence of thromboembolic events. We report a case of a female, age 14, with a 6 months history of diarrhea with occasional presence of red blood and with a very distended abdomen with evident ascites. The diagnosis was Budd-Chiari syndrome (BCS) in ulcerative colitis (UC). Therapy with subcutaneous low molecular weight heparin, methylprednisolone, mesalazine was started. Clinical and radiological features quickly improved. No thrombophilia abnormality nor other risk factor for thrombosis were detected...
May 2017: Recenti Progressi in Medicina
https://www.readbyqxmd.com/read/28621023/pediatric-liver-transplantation-for-fulminant-hepatic-failure-secondary-to-intentional-iron-overdose
#16
Joanne Lai, Jaime Chu, Ronen Arnon
Acute iron poisoning may lead to life-threatening hepatotoxicity. We present the cases of two pediatric patients with hepatotoxicity following intentional iron ingestion that progressed rapidly to fulminant hepatic failure despite treatment with deferoxamine. Liver transplantation was lifesaving in both patients. These cases emphasize the need for a high index of suspicion for iron ingestion, close monitoring for liver toxicity, and timely consideration for liver transplantation.
September 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28583557/in-situ-posterior-graft-segmentectomy-for-large-for-size-syndrome-in-deceased-donor-liver-transplantation-in-adults-a-case-report
#17
A Nagatsu, T Yoshizumi, T Ikegami, N Harimoto, N Harada, Y Soejima, A Taketomi, Y Maehara
Large-for-size syndrome (LFSS) is controversial in pediatric living donor liver transplantation patients and is associated with a poor graft outcome. Similar situations in deceased donor liver transplantation (DDLT) in adults have not been reported frequently, and there are no official guidelines worldwide. Deceased donation is extremely limited in Japan, and when a larger liver is allocated for a very sick small recipient in Japan, transplantation with a plan to address LFSS might be necessary. The patient is a 58-year-old female patient who had acute liver failure with coma...
June 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28535186/fxr-gankyrin-axis-is-involved-in-development-of-pediatric-liver-cancer
#18
Leila Valanejad, Kyle Lewis, Mary Wright, Yanjun Jiang, Amber D'Souza, Rebekah Karns, Rachel Sheridan, Anita Gupta, Kevin Bove, David Witte, James Geller, Gregory Tiao, David L Nelson, Lubov Timchenko, Nikolai Timchenko
The development of hepatoblastoma (HBL) is associated with failure of hepatic stem cells (HSC) to differentiate into hepatocytes. Despite intensive investigations, mechanisms of the failure of HSC to differentiate are not known. We found that oncogene Gankyrin (Gank) is involved in the inhibition of differentiation of HSC via triggering degradation of tumor suppressor proteins (TSPs) Rb, p53, C/EBPα and HNF4α. Our data show that the activation of a repressor of Gank, farnesoid X receptor, FXR, after initiation of liver cancer by Diethylnitrosamine (DEN) prevents the development of liver cancer by inhibiting Gank and rescuing tumor suppressor proteins...
July 1, 2017: Carcinogenesis
https://www.readbyqxmd.com/read/28461212/risk-factors-and-outcomes-related-to-pediatric-intensive-care-unit-admission-after-hematopoietic-stem-cell-transplantation-a-single-center-experience
#19
Marta Pillon, Angela Amigoni, Annaelena Contin, Manuela Cattelan, Elisa Carraro, Emiliana Campagnano, Manuela Tumino, Elisabetta Calore, Antonio Marzollo, Chiara Mainardi, Maria Paola Boaro, Marta Nizzero, Andrea Pettenazzo, Giuseppe Basso, Chiara Messina
To describe incidence, causes, and outcomes related to pediatric intensive care unit (PICU) admission for patients undergoing hematopoietic stem cell transplantation (HSCT), we investigated the risk factors predisposing to PICU admission and prognostic factors in terms of patient survival. From October 1998 to April 2015, 496 children and young adults (0 to 23 years) underwent transplantation in the HSCT unit. Among them, 70 (14.1%) were admitted to PICU. The 3-year cumulative incidence of PICU admission was 14...
August 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28433098/wilson-disease-in-children
#20
Eve A Roberts, Piotr Socha
Wilson disease (WD) is an inherited disorder mainly of hepatocellular copper disposition, due to dysfunction of the Wilson ATPase, a P1B-ATPase encoded by the gene ATP7B. In children, as in older age brackets, clinical disease is highly diverse. Although hepatic disease is the common presentation in children/adolescents, neurologic, psychiatric, and hematologic clinical presentations do occur. Very young children may have clinically evident liver disease due to WD. Early diagnosis, preferably when the child/adolescent is asymptomatic, is most likely to result in near-normal longevity with generally good health so long as the patient tolerates effective medication, is adherent to the lifelong treatment regimen, and has consistent access to the medication...
2017: Handbook of Clinical Neurology
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