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https://www.readbyqxmd.com/read/28632503/the-catastrophic-antiphospholipid-syndrome-in-children
#1
Ellen J L Go, Kathleen M O'Neil
PURPOSE OF REVIEW: To review the difficult syndrome of catastrophic antiphospholipid syndrome, emphasizing new developments in the diagnosis, pathogenesis and treatment. RECENT FINDINGS: Few recent publications directly address pediatric catastrophic antiphospholipid syndrome (CAPS). Most articles are case reports or are data from adult and pediatric registries. The major factors contributing to most pediatric catastrophic antiphospholipid syndrome include infection and the presence of antiphospholipid antibodies, but complement activation also is important in creating diffuse thrombosis in the microcirculation...
June 16, 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28626543/cerebral-stroke-in-a-teenage-girl-with-paroxysmal-nocturnal-hemoglobinuria
#2
Francesco Gervasi, Lucia D'Amelio, Antonino Trizzino, Fabrizia Ferraro, Delia Russo, Giuseppe Santangelo, Francesca Cardella, Angela Trizzino, Floriana Di Marco, Piero Farruggia
We report a case of paroxysmal nocturnal hemoglobinuria (PNH) in a 14 year-old girl presenting a cerebral arterial thrombosis. The initial diagnosis was carential anemia due to menarche following identification of slight macrocytic anemia, leucopenia and mild thrombocytopenia at routine blood analysis. The child was eventually referred to a children's hospital after the onset of progressive fatigue, anorexia and paleness. Severe anemia (hemoglobin 6 g/dL) with negative Coombs test, mild leucopenia (white blood cells 4...
June 1, 2017: Hematology Reports
https://www.readbyqxmd.com/read/28620647/the-effect-and-safety-of-prostaglandin-administration-in-pediatric-liver-transplantation
#3
Céline Lironi, Valérie A McLin, Barbara E Wildhaber
BACKGROUND: Prostaglandins are often administered after liver transplantation (LT) to diminish ischemia-reperfusion injury (IRI), to favor liver recovery and to prevent vascular thrombosis. Possible beneficial effects in adult liver recipients are controversial, but the single existing pediatric small case series shows no significant impact of prostaglandin administration after LT. The purpose of this study was to analyze the effect of the prostaglandin dinoprostone in pediatric liver recipients...
June 2017: Transplantation Direct
https://www.readbyqxmd.com/read/28610742/interventional-treatment-for-hepatic-artery-thrombosis-after-liver-transplantation
#4
Hua Zhang, Sheng Qian, Rong Liu, Wei Yuan, Jian-Hua Wang
PURPOSE: To evaluate short-term and long-term effectiveness of interventional treatment for hepatic artery thrombosis (HAT). MATERIALS AND METHODS: From March 2003 to October 2015, 34 patients (32 male and 2 female; mean age, 45 y; range 7-64 y) with HAT were identified 0-21d (mean 6.5 d ± 6.0) after liver transplantation and underwent interventional treatments. Technical success, clinical success, complications, hepatic artery patency, and survival time were assessed...
June 11, 2017: Journal of Vascular and Interventional Radiology: JVIR
https://www.readbyqxmd.com/read/28606584/impact-of-a-modified-anti-thrombotic-guideline-on-stroke-in-children-supported-with-a-pediatric-ventricular-assist-device
#5
David N Rosenthal, Chacy A Lancaster, Doff B McElhinney, Sharon Chen, MaryLyn Stein, Aileen Lin, Lan Doan, Jenna M Murray, Mary Alice Gowan, Katsuhide Maeda, Olaf Reinhartz, Christopher S Almond
BACKGROUND: Stroke is the most feared complication associated with the Berlin Heart EXCOR pediatric ventricular assist device (VAD), the most commonly used VAD in children, and affects 1 in 3 children. We sought to determine whether a modified anti-thrombotic guideline, involving more intense platelet inhibition and less reliance on platelet function testing, is associated with a lower incidence of stroke. METHODS: All children supported with the EXCOR at Stanford from 2009 to 2014 were divided into 2 cohorts based on the primary anti-thrombotic guideline used to prevent pump thrombosis: (1) the Edmonton Anti-thrombotic Guideline (EG) cohort, which included children implanted before September 2012 when dual anti-platelet therapy was used with doses titrated to Thromboelastrography/PlateletMapping (TEG/PM); and (2) the Stanford Modified Anti-thrombotic Guideline (SG) cohort, which included children implanted on or after September 2012 when triple anti-platelet therapy was used routinely and where doses were uptitrated to high, weight-based dosing targets, with low-dose steroids administered as needed for inflammation...
May 20, 2017: Journal of Heart and Lung Transplantation
https://www.readbyqxmd.com/read/28599856/-bilateral-pulmonary-embolism-mimicking-acute-chest-syndrome-in-an-adolescent-with-sickle-cell-disease
#6
P Mornand, F Chalard, A-S Romain, M Rohr, C Paluel-Marmont, A Niakaté, B Quinet, E Grimprel, M-H Odièvre-Montanié
Pulmonary embolism is a life-threatening and potentially lethal disease. Its incidence in children with sickle cell disease is probably underestimated and pediatric case reports in the literature are rare. Moreover, symptoms can mimic an acute chest syndrome. We report on the case of a 17-year-old boy with SS sickle cell disease, admitted for chest pain with dyspnea and tachycardia. Pulmonary angiography revealed a partial bilateral obstructive pulmonary embolism. We did not find any deep venous thrombosis or thrombophilia...
June 6, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28588188/a-young-boy-with-l-asparaginase-induced-seizure
#7
M A Aziz, N K Singh, M H Rahman, M R Khan, A L Kabir, M Begum
L-Asparaginase is a critical component in the treatment of acute lymphoblastic leukemia in children. It is known to cause coagulation abnormalities, thrombosis and hemorrhage in the central nervous system in addition to vasculitis and hypersensitivity reactions. This syndrome generally occurs after a few weeks of therapy and may occur after L-asparaginase therapy is completed. Seizures are uncommon symptoms. We report a case of seizure associated with L-asparaginase therapy but no evidence of hemorrhagic or thrombotic cerebrovascular events, completed in the department of Hematology of Bangabandhu Sheikh Mujib Medical University during March & April 2016...
April 2017: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/28585786/-results-of-the-mesoportal-bypass-rex-shunt-in-the-treatment-of-idiopathic-extrahepatic-portal-vein-obstruction-in-children
#8
E Domínguez Amillo, C De la Torre Ramos, A Andrés Moreno, J L Encinas Hernández, F Hernández Oliveros, M López Santamaría
BACKGROUND: Extrahepatic portal vein obstruction (EPVO) is the principal cause of portal hypertension in children. The objective of this study was to analyze the capacity of the surgical technique that creates a mesoportal shunt to treat changes caused by EPVO. METHODS: Retrospective review of patients with idiopathic EPVO who underwent a mesoportal shunt and analysis of the changes in the number of leucocytes, platelets, prothrombin time and spleen size one year after the surgery...
January 25, 2017: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
https://www.readbyqxmd.com/read/28580631/kawasaki-disease-medical-therapies
#9
Jane W Newburger
Medical therapies in patients with Kawasaki disease (KD) are administered to reduce the prevalence of coronary aneurysms, reduce systemic inflammation, and prevent coronary thrombosis. All patients with acute KD should be treated with intravenous immunoglobulin (IVIG) 2 g/kg, generally administered over 10-12 hours. Aspirin has never been shown to prevent aneurysms, but is given for its anti-inflammatory and antipyretic effects until the patient has been afebrile for ∼2 days, then lowered to an antiplatelet dose...
June 5, 2017: Congenital Heart Disease
https://www.readbyqxmd.com/read/28579360/complications-of-long-and-intermediate-term-venous-catheters-in-cystic-fibrosis-patients-a-multicenter-study
#10
Teresa L May, Alex H Gifford, Thomas Lahiri, Adam Black, Janet Trang, Alexandra G Cornell, Karyll Gonzalez, Scott Morin, Mark Napier, Christine W Duarte, Jonathan B Zuckerman
BACKGROUND: Totally implantable venous access devices (TIVADs) or peripherally inserted central venous catheters (PICCs) are commonly used in the care of patients with cystic fibrosis (CF), but they are associated with various complications, including thrombosis, infection, and insertion site symptoms. METHODS: We conducted a retrospective review of PICC and TIVAD use in adults and children with CF over an 8-year period at 3 accredited care centers. Patient attributes included CFTR genotype, comorbidities, lung function, body mass index, use of anticoagulation, and respiratory tract microbiology...
June 1, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28570120/characterization-of-the-coagulation-profile-in-children-with-liver-disease-and-extrahepatic-portal-vein-obstruction-or-shunt
#11
William Beattie, Maria Magnusson, Winita Hardikar, Paul Monagle, Vera Ignjatovic
INTRODUCTION: Chronic liver disease causes a disruption of procoagulant and anticoagulant factors, resulting in a fragile state, prone to increased rates of both bleeding and thrombosis. Currently, there is limited literature describing the changes observed in pediatric liver disease and extrahepatic portal vein obstruction or shunt. This study aimed to describe the changes that occur in children with chronic liver disease and extrahepatic portal vein obstruction or shunt. MATERIALS AND METHODS: We measured the concentration and activity of key procoagulant and anticoagulant factors in children with liver disease, children with extrahepatic portal vein obstruction or shunt, and healthy children...
June 1, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/28552449/safety-and-efficacy-of-warfarin-therapy-in-kawasaki-disease
#12
Annette L Baker, Christina Vanderpluym, Kimberly A Gauvreau, David R Fulton, Sarah D de Ferranti, Kevin G Friedman, Jenna M Murray, Loren D Brown, Christopher S Almond, Margaret Evans-Langhorst, Jane W Newburger
OBJECTIVE: To describe the safety and efficacy of warfarin for patients with Kawasaki disease and giant coronary artery aneurysms (CAAs, ≥8 mm). Giant aneurysms are managed with combined anticoagulation and antiplatelet therapies, heightening risk of bleeding complications. STUDY DESIGN: We reviewed the time in therapeutic range; percentage of international normalization ratios (INRs) in range (%); bleeding events, clotting events; INRs ≥6; INRs ≥5 and <6; and INRs <1...
May 25, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28552079/clinical-epidemiology-and-centre-variation-in-chylothorax-rates-after-cardiac-surgery-in-children-a-report-from-the-pediatric-cardiac-critical-care-consortium
#13
Jason R Buckley, Eric M Graham, Michael Gaies, Jeffrey A Alten, David S Cooper, John M Costello, Yuliya Domnina, Darren Klugman, Sara K Pasquali, Janet E Donohue, Wenying Zhang, Mark A Scheurer
Introduction Chylothorax after paediatric cardiac surgery incurs significant morbidity; however, a detailed understanding that does not rely on single-centre or administrative data is lacking. We described the present clinical epidemiology of postoperative chylothorax and evaluated variation in rates among centres with a multicentre cohort of patients treated in cardiac ICU. METHODS: This was a retrospective cohort study using prospectively collected clinical data from the Pediatric Cardiac Critical Care Consortium registry...
May 29, 2017: Cardiology in the Young
https://www.readbyqxmd.com/read/28540196/significance-of-platelet-count-in-children-admitted-with-bronchiolitis
#14
Amar Al Shibli, Najla Alkuwaiti, May Hamie, Dima Abukhater, Muhammad B Noureddin, Abdulla Amri, Salwa Al Kaabi, Aysha Al Kaabi, Mariam Harbi, Hassib Narchi
AIM: To determine the true prevalence of thrombocytosis in children less than 2 years of age with bronchiolitis, its association with risk factors, disease severity and thromboembolic complications. METHODS: A retrospective observational medical chart review of 305 infants aged two years or less hospitalized for bronchiolitis. Clinical outcomes included disease severity, duration of hospital stay, admission to pediatric intensive care unit, or death. They also included complications of thrombocytosis, including thromboembolic complications such as cerebrovascular accident, acute coronary syndrome, deep venous thrombosis, pulmonary embolus, mesenteric thrombosis and arterial thrombosis and also hemorrhagic complications such as bleeding (spontaneous hemorrhage in the skin, mucous membranes, gastrointestinal, respiratory, or genitourinary tracts)...
May 8, 2017: World Journal of Clinical Pediatrics
https://www.readbyqxmd.com/read/28532240/ventricular-assist-devices-for-the-failing-univentricular-circulation
#15
Edward Buratto, William Y Shi, Xin Tao Ye, Igor E Konstantinov
Improved survival following single ventricle palliation has led to a large population of patients with a univentricular circulation, many of whom develop heart failure. Increasing experience with ventricular assist devices (VAD) in children has paved the way for VAD support in those with failing univentricular circulation. Areas covered: The use of VADs to support the failing univentricular circulation is a relatively new concept. Most studies have focused on supporting patients with the failing systemic ventricle...
June 2017: Expert Review of Medical Devices
https://www.readbyqxmd.com/read/28511552/r147w-in-proc-gene-is-a-risk-factor-of-thromboembolism-in-thai-children
#16
Nongnuch Sirachainan, Ampaiwan Chuansumrit, Werasak Sasanakul, Najwa Yudhasompop, Lalita Mahaklan, Jarin Vaewpanich, Pimlak Charoenkwan, Somjai Kanjanapongkul, Anannit Visudtibhan, Pakawan Wongwerawattanakoon
The p.R147W mutation, the c.C6152T in exon 7, causing a change in amino acid from arginine to tryptophan of the PROC gene has been reported as a common mutation in Taiwanese populations with venous thromboembolism (VTE). The present study aimed to identify the prevalence of p.R147W in the Thai population and children with TE and the risk of developing TE. Patients aged ≤18 years diagnosed with TE were enrolled. The PROC gene was amplified by polymerase chain reaction using a specific primer in exon 7. The restriction fragment length polymorphism was designed using MwoI restriction enzyme...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28474980/risk-of-deep-venous-thrombosis-in-elective-neurosurgical-procedures-a-prospective-doppler-ultrasound-based-study-in-children-12-years-of-age-or-younger
#17
Andrea G Scherer, Ian K White, Kashif A Shaikh, Jodi L Smith, Laurie L Ackerman, Daniel H Fulkerson
OBJECTIVE The risk of venous thromboembolism (VTE) from deep venous thrombosis (DVT) is significant in neurosurgical patients. VTE is considered a leading cause of preventable hospital deaths and preventing DVT is a closely monitored quality metric, often tied to accreditation, hospital ratings, and reimbursement. Adult protocols include prophylaxis with anticoagulant medications. Children's hospitals may adopt adult protocols, although the incidence of DVT and the risk or efficacy of treatment is not well defined...
May 5, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28472942/evaluation-for-inherited-and-acquired-prothrombotic-defects-predisposing-to-symptomatic-thromboembolism-in-children-with-acute-lymphoblastic-leukemia-a-protocol-for-a-prospective-observational-cohort-study
#18
Uma H Athale, Caroline Laverdiere, Trishana Nayiager, Yves-Line Delva, Gary Foster, Lehana Thabane, Anthony Kc Chan
BACKGROUND: Thromboembolism (TE) is a serious complication in children with acute lymphoblastic leukemia (ALL). The incidence of symptomatic thromboembolism is as high as 14% and case fatality rate of ~15%. Further, development of thromboembolism interferes with the scheduled chemotherapy with potential impact on cure rates. The exact pathogenesis of ALL-associated thromboembolism is unknown. Concomitant administration of asparaginase and steroids, two important anti-leukemic agents, is shown to increase the risk of ALL-associated TE...
May 4, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28472748/in%C3%A2-vivo-cardiovascular-toxicity-induced-by-acetochlor-in-zebrafish-larvae
#19
Hongcui Liu, Tianyi Chu, Lili Chen, Wenjun Gui, Guonian Zhu
The risk of acetochlor to human health is still unclear, prompting concern over its risk, especially to pesticide suicides population, occupational population (farmers, retailers and pharmaceutical workers), and special population (young children and infants, pregnant women, older people, and those with compromised immune systems). This study was to explore the toxic effect and the possible mechanism of toxic action of acetochlor using zebrafish larvae whose toxicity profiles have been confirmed to be strikingly similar with mammalian...
April 21, 2017: Chemosphere
https://www.readbyqxmd.com/read/28468093/-the-incidence-and-risk-factors-of-catheter-related-thrombosis-during-induction-chemotherapy-in-acute-lymphocytic-leukemia-children
#20
Y Y Wei, Y Y Zhang, Y Z Zhen, L Q Zhang, C G Jia, R D Zhang, H Y Zheng, X Y Wu, R H Wu
Objective: To investigate the current status of catheter-related-thrombosis (CRT) and the risk factors of Chinese acute lymphocytic leukemia (ALL) children with peripherally inserted central catheter (PICC) . Methods: The clinical data of the 116 inpatients preliminarily diagnosed ALL in the Leukemia Ward of Beijing Children's Hospital with PICC from 1(st) March 2014 to 31(st) December 2014 were collected prospectively. Results: ①Refer to the B-ultrasound on the 15(th) day after catheterization, the incidence of CRT was 28...
April 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
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