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https://www.readbyqxmd.com/read/29443826/higher-risk-of-posttransplant-graft-failure-in-male-recipients-of-female-donor-grafts-might-not-be-due-to-anastomotic-size-disparity
#1
Kyo Won Lee, Sangbin Han, Sanghoon Lee, Hyun-Hwa Cha, Soohyun Ahn, Hyeon Seon Ahn, Justin S Ko, Mi Sook Gwak, Gaab Soo Kim, Jae-Won Joh, Suk-Koo Lee, Gyu-Seong Choi
BACKGROUND: Posttransplant liver graft failure occurs most often in male recipients of livers from female donors. The respective role of donor sex itself and the size disparity in graft vessels/bile ducts according to donor sex are unclear. Thus, we aimed to evaluate the importance of donor sex with adjustment for anastomotic size disparity between female and male donor grafts. METHODS: A total of 309 male patients without hepatic tumors who underwent living donor liver transplantation were analyzed (109 female and 200 male donors)...
February 13, 2018: Transplantation
https://www.readbyqxmd.com/read/29443401/pediatric-intestinal-failure-associated-liver-disease-challenges-in-identifying-clinically-relevant-biomarkers
#2
Kathleen M Gura, Andrew E Mulberg, Paul D Mitchell, John Yap, Clara Y Kim, Minjun Chen, Alexis Potemkin, Mark Puder
BACKGROUND: Intestinal failure-associated liver disease (IFALD) is complex and diagnosed by concurrent use of parenteral nutrition, clinical presentation, and alterations in hepatic biomarkers exclusive of other causes of liver disease. In comparison with individual measures, composite biomarkers may provide a more effective means for assessing disease progression and response to treatment than single parameters. Since IFALD is considered by some to be a type of drug-induced liver injury (DILI), those diagnostic criteria could potentially be used in this population...
February 2018: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/29410290/one-thousand-pediatric-liver-transplants-during-thirty-years-lessons-learned
#3
Robert S Venick, Douglas G Farmer, Jose R Soto, Jorge Vargas, Hasan Yersiz, Fady M Kaldas, Vatche G Agopian, Jonathan R Hiatt, Sue V McDiarmid, Ronald W Busuttil
BACKGROUND: Pediatric liver transplantation (pLTx) has been the standard of care for children with liver failure since the 1980s. This study examined the world's largest single-center experiences and aimed to identify unique pre-operative predictors of early graft and patient survival for primary (1º-pLTx) and re-transplantation (Re-pLTx). STUDY DESIGN: An IRB approved, retrospective study of all consecutive, isolated pLTx patients ≤ 18 years of age was conducted...
February 1, 2018: Journal of the American College of Surgeons
https://www.readbyqxmd.com/read/29407332/antibody-drug-treatment-for-steroid-resistant-rejection-after-pediatric-living-donor-liver-transplantation-a-single-center-experience
#4
Y Hirata, Y Sanada, T Urahashi, Y Ihara, N Yamada, N Okada, T Katano, S Otomo, K Ushijima, K Mizuta
BACKGROUND: Antibody drugs have been used to treat steroid-resistant rejection (SRR) after liver transplantation. Although anti-thymocyte globulin has been used for SRR after liver transplantation in place of muromonab-CD3 since 2011 in Japan, the effectiveness of anti-thymocyte globulin after pediatric living-donor liver transplantation (LDLT) has not yet been reported. The aim of this study was to evaluate the effectiveness of antibody drug treatment for SRR after pediatric LDLT in our single center...
January 2018: Transplantation Proceedings
https://www.readbyqxmd.com/read/29402263/effect-of-the-2015-earthquake-on-pediatric-inpatient-pattern-at-a-tertiary-care-hospital-in-nepal
#5
Bishnu Rath Giri, Ram Hari Chapagain, Samana Sharma, Sandeep Shrestha, Sunita Ghimire, P Ravi Shankar
BACKGROUND: Earthquakes impact child health in many ways. Diseases occurring immediately following an earthquake have been studied in field based hospitals but studies on the inpatient disease pattern among children without trauma in a permanent hospital setup is lacking. METHODS: We examined the diagnoses of all children without trauma, admitted to Kanti Children's Hospital, Kathmandu for fifteen-week duration (from 4th week to end of the 18th week) following the 7...
February 5, 2018: BMC Pediatrics
https://www.readbyqxmd.com/read/29382475/-autoimmune-hepatitis-in-the-pediatric-age
#6
REVIEW
Rubén Peña-Vélez, Enory Almanza-Miranda
In pediatrics, autoimmune hepatitis and sclerosing cholangitis are liver disorders with an immunological damage mechanism. Autoimmune hepatitis is a disease of unknown etiology characterized by interface hepatitis, hypergammaglobulinemia, circulating autoantibodies and a favorable response to immunosuppression. It is an eminently pediatric disease with a prevalent condition in young women. Therapy should be instituted promptly to prevent rapid deterioration, promote remission of disease and long-term survival...
September 2017: Boletín Médico del Hospital Infantil de México
https://www.readbyqxmd.com/read/29381934/pregnancy-associated-hemophagocytic-lymphohistiocytosis-secondary-to-nk-t-cells-lymphoma-a-case-report-and-literature-review
#7
Mengzhou He, Jing Jia, Jingyi Zhang, Rajluxmee Beejadhursing, Lali Mwamaka Sharifu, Jun Yu, Shaoshuai Wang, Ling Feng
RATIONALE: Hemophagocytic lymphohistiocytosis (HLH) occurs primarily in pediatric population, or secondary to malignancy, infection, or autoimmune disease. This disease is rare and prognosis is generally poor. Only a small number of cases during pregnancy have been reported in literature. PATIENT CONCERNS: We report a case of pregnancy-associated HLH secondary to natural killer (NK)/T cells lymphoma. She was admitted at 30 weeks and 3 days of pregnancy with complaints of abdominal pain and fever as high as 39...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29334124/use-of-antiviral-medications-in-drug-reaction-with-eosinophilia-and-systemic-symptoms-dress-a-case-of-infantile-dress
#8
Maggie L Chow, David Kim, Sonia Kamath, David Peng, Minnelly Luu
A 3-month-old girl with Sturge-Weber syndrome presented with a morbilliform rash, eosinophilia, and fulminant liver failure to our tertiary pediatric hospital. She was diagnosed with drug reaction with eosinophilia and systemic symptoms complicated by viremia and evidence of viral hepatitis on liver biopsy. We discuss the role of viral reactivation in drug reaction with eosinophilia and systemic symptoms and the relevance of antiviral therapy in management.
January 15, 2018: Pediatric Dermatology
https://www.readbyqxmd.com/read/29290748/therapeutic-drug-monitoring-of-prolonged-infusion-aztreonam-for-multi-drug-resistant-pseudomonas-aeruginosa-a-case-report
#9
Jeffrey J Cies, Richard J LaCoursiere, Wayne S Moore, Arun Chopra
Aztreonam, a broad-spectrum monobactam, is typically reserved for multidrug resistant (MDR) infections. Pharmacokinetic (PK) data to guide dosing in children, however, are limited to healthy volunteers or nonintensive care unit (ICU) patients. Impaired antibiotic delivery into tissue remains a major concern and may explain the high morbidity and mortality associated with MDR infections. Therefore, evaluating the PK changes in pediatric ICU patients is necessary to elucidate the most appropriate antimicrobial regimen...
November 2017: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
https://www.readbyqxmd.com/read/29234203/cholemic-or-bile-cast-nephropathy-in-a-child-with-liver-failure
#10
Vikrant Sood, Bikrant B Lal, Suman Lata, Archana Rastogi, Seema Alam
Cholemic or bile cast nephropathy is an under-reported entity characterized by acute renal dysfunction in patients with hepatic insult. Limited literature is available regarding its clinical presentation, pathogenesis and prognosis. We hereby present a pediatric case who presented with acute on chronic liver failure with renal dysfunction secondary to cholemic nephropathy.
December 2017: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/29161691/failure-to-thrive-in-the-context-of-carney-complex
#11
Amit Tirosh, Adi Auerbach, Belen Bonella, Phaedon D Zavras, Elena Belyavskaya, Charalampos Lyssikatos, Karen Meir, Ram Weiss, Hagit Daum, Maya B Lodish, David Gillis, Constantine A Stratakis
BACKGROUND/AIMS: Carney complex (CNC) is a rare syndrome associated with multiple tumors and several other unique manifestations. We describe the clinical, genetic, and laboratory findings in a cohort of patients with CNC and failure to thrive (FTT). METHODS: A retrospective case series of pediatric patients with CNC presenting with FTT. RESULTS: We describe a patient with infantile Cushing syndrome (CS) who presented with severe FTT and liver disease; the patient was subsequently diagnosed with CNC...
November 21, 2017: Hormone Research in Pædiatrics
https://www.readbyqxmd.com/read/29159818/c-ebp%C3%AE-dependent-pre-neoplastic-tumor-foci-are-the-origin-of-hepatocellular-carcinoma-and-aggressive-pediatric-liver-cancer
#12
Ashley Cast, Leila Valanejad, Mary Wright, Phuong Nguyen, Anita Gupta, Liqin Zhu, Soona Shin, Nikolai Timchenko
Recent publications show that classic hepatoblastoma (HBL) is the result of failure of hepatic stem cells to differentiate into hepatocytes; while hepatocellular carcinoma (HCC) is caused by the de-differentiation of hepatocytes into Cancer Stem Cells (CSCs). However, the mechanisms of aggressive HBL and mechanisms that cause de-differentiation of hepatocytes into CSCs are unknown. We found that, similar to HCC but opposite to classic HBL, aggressive HBL is the result of de-differentiation of hepatocytes into CSCs...
November 21, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29110981/distributive-shock-in-a-neonate-with-diffuse-infantile-hepatic-hemangioma
#13
James Brown, Rajan Arora, Usha Sethuraman
BACKGROUND: Hemangiomas are common cutaneous findings on healthy infants. These vascular malformations are generally benign, though in rare circumstances they can potentially be fatal. This is particularly true when the hemangiomas are large or numerous and occurring in visceral organs. Previously unrecognized visceral hemangiomas are part of the differential for any neonate presenting unexpectedly in shock. CASE REPORT: A 10-day-old neonate presented to the pediatric emergency department with difficulty breathing...
October 27, 2017: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/29080066/choosing-first-line-biologic-treatment-for-moderate-to-severe-psoriasis-what-does-the-evidence-say
#14
Mina Amin, Daniel J No, Alexander Egeberg, Jashin J Wu
An advanced understanding of the pathogenesis of psoriasis has led to the development of multiple therapeutic options for moderate-to-severe psoriasis. Tumor necrosis factor inhibitors, ustekinumab, interleukin-17 inhibitors, and guselkumab (an interleukin-23 inhibitor recently approved for psoriasis) are commercially available biologic agents for psoriasis. Evidence from clinical trials provides pertinent information regarding the safety and efficacy of biologic agents for psoriasis, which should be integrated into clinical decision making...
October 27, 2017: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/29039050/the-protective-effect-of-fish-oil-lipid-emulsions-on-intestinal-failure-associated-liver-disease-in-a-rat-model-of-short-bowel-syndrome
#15
Seiro Machigashira, Tatsuru Kaji, Shun Onishi, Waka Yamada, Keisuke Yano, Koji Yamada, Ryuta Masuya, Takafumi Kawano, Kazuhiko Nakame, Motoi Mukai, Satoshi Ieiri
PURPOSE: Pediatric patients with intestinal failure need long-term parenteral nutrition (PN), but this nutritional support causes liver dysfunction, such as intestinal failure-associated liver disease (IFALD). Several studies have shown that the lipid emulsion produced by soybean oil (SO) is associated with the occurrence of IFALD. In this study, we evaluated the effect of SO and fish oil (FO) lipid emulsion on hepatic steatosis. METHODS: Sprague-Dawley rats underwent jugular vein catheterization and were divided into three groups: sham operation with normal chow (Sham group), 80% small bowel resection (80% SBR) + TPN with SO lipid emulsion (SO group), and 80% SBR + TPN with FO lipid emulsion (FO group)...
October 16, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/29022907/p38%C3%AE-mapk-antagonizing-jnk-to-control-the-hepatic-fat-accumulation-in-pediatric-patients-onset-intestinal-failure
#16
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
#17
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
#18
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
#19
COMPARATIVE STUDY
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...
February 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28937522/ambulatory-high-dose-methotrexate-administration-in-pediatric-osteosarcoma-patients-at-a-single-institution-in-argentina
#20
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
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