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Pediatric ITP

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https://www.readbyqxmd.com/read/29222284/management-of-newly-diagnosed-immune-thrombocytopenia-can-we-change-outcomes
#1
REVIEW
Cindy E Neunert
Immune thrombocytopenia resulting from antibody-mediated platelet destruction combined with impaired platelet production is a common cause of thrombocytopenia. The decision to treat newly diagnosed patients is based on several factors including ceasing hemorrhagic manifestations, increasing the platelet count, prevention of bleeding, and inducing remission. Current standard first-line therapy is a course of corticosteroids. Although this treatment paradigm increases the platelet count in the majority of patients, a high percentage relapse after discontinuation of corticosteroid therapy...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29173312/treatment-of-children-with-persistent-and-chronic-idiopathic-thrombocytopenic-purpura-4-infusions-of-rituximab-and-three-4-day-cycles-of-dexamethasone
#2
Joseph H Oved, Christina S Y Lee, James B Bussel
OBJECTIVES: To assess initial and long-term outcome of children with persistent/chronic idiopathic thrombocytopenic purpura (ITP) treated with 4 infusions of rituximab and three 4-day cycles of dexamethasone (4R+3Dex) including cohorts with most benefit and/or treatment associated toxicity. STUDY DESIGN: All pediatric patients with ITP at Weill-Cornell who received 4R+3Dex were included in this retrospective study. Duration was median time from first rituximab infusion to treatment failure...
December 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/29068869/dural-venous-sinus-thrombosis-and-pulmonary-embolism-following-immunoglobulin-treatment-in-pediatric-patient-with-immune-thrombocytopenic-purpura
#3
Kyung Mi Park, Eu Jeen Yang, Young Tak Lim
Intravenous immunoglobulin (IVIG) is a widely used agent as the first choice of treatment of immune thrombocytopenic purpura (ITP). IVIG has several side effects, but it is a relatively safe treatment. Life-threatening thrombosis has been reported in adults and rarely in children. We report a case of a 14-year-old boy with dural venous sinus thrombosis and pulmonary embolism after treatment with IVIG for ITP. The patient was treated with low-molecular-weight heparin followed by warfarin and the symptoms were recovered...
November 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29067848/distinct-clinical-correlates-of-immune-thrombocytopenic-purpura-at-diagnosis-of-childhood-onset-and-adult-sle
#4
Gladys Cherres Xavier Esteves, Natali Weniger Spelling Gormezano, Oriany L Pereira, David Kern, Clovis Almeida Silva, Rosa Maria Rodrigues Pereira, Katia Tomie Kozu, Eloisa Bonfá, Nadia Emi Aikawa
OBJECTIVES: To compare clinical and laboratorial features between childhood-onset systemic lupus erythematosus (cSLE) and adult SLE (aSLE) at concomitant diagnosis of immune thrombocytopenic purpura (ITP). METHODS: This study evaluated 56 cSLE and 73 aSLE patients regularly followed at Pediatric and Rheumatology Divisions of the same University hospital with ITP (platelets count <100,000/mm3 in the absence of other causes) at lupus onset. RESULTS: Median current age was 11...
October 25, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/29020887/the-potential-association-of-tumor-necrosis-factor-%C3%AE-eta-252-g-a-cytokine-gene-polymorphism-with-immune-thrombocytopenic-purpura-among-egyptian-children
#5
Dalia Saber Morgan, Rasha Abdel-RaoufAbdel-Aziz Afifi, Shereen Mohamed El-Hoseiny, Dalia Gamil Amin, Sara Yahia Gaber Ibrahim
OBJECTIVES: The main objective of this study was to study tumor necrosis factor beta (TNFB) + 252G/A gene polymorphism, known to be related to autoimmunity, in immune thrombocytopenia (ITP) patients. We also aimed to investigate the association between TNFB + 252G/A polymorphism and susceptibility to develop persistent/chronic ITP. METHODS: One hundred pediatric ITP patients, as well as 50 age- and sex-matched healthy Egyptian subjects, were included. Genotyping of TNF-β gene (G252A) was done using the PCR-RFLP method...
October 12, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28974109/clinical-significance-of-t-cell-immunoglobulin-mucin-3-expression-on-peripheral-blood-mononuclear-cells-in-pediatric-acute-immune-thrombocytopenia
#6
Asmaa M Zahran, Mervat A M Youssef, Khalid I Elsayh, Mustafa M Embaby, Ahmad I M Ibrahim
T-cell immunoglobulin mucin 3 (TIM-3) is a transmembrane protein that plays an important role in several autoimmune diseases. The relationship between TIM-3 and excessive immune responses in immune thrombocytopenia (ITP) is still unknown. In this study, we evaluated the relationship between the expression of TIM-3 on peripheral blood mononuclear cells in patients with ITP and the disease severity. The frequency of lymphocyte and monocyte subsets and their TIM-3 expression were evaluated in patients with acute ITP (n = 45) and in healthy control (n = 20) using flow cytometry...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28971174/investigation-of-celiac-disease-followed-by-immune-thrombocytopenic-purpura-diagnosis-in-patients-and-comparison-with-literature
#7
Hakan Sarbay, Halil Kocamaz, Mehmet Akin, Bayram Ozhan
OBJECTIVE: Celiac disease (CD) and Immune thrombocytopenic purpura (ITP) may occur together as a result of similar autoimmune mechanisms. The aim of this study was to assess the frequency of CD in a group of ITP patients and in the literature. METHODS: A total of 29 patients in Pamukkale University Faculty of Medicine Hospital Pediatric Hematology and Oncology Department with ITP were included in the study. Test was performed for the antibodies related to CD. Positive result for celiac antibodies was confirmed with biopsy...
2017: Northern Clinics of Istanbul
https://www.readbyqxmd.com/read/28921855/utility-of-the-immature-platelet-fraction-in-pediatric-immune-thrombocytopenia-differentiating-from-bone-marrow-failure-and-predicting-bleeding-risk
#8
Alicia McDonnell, Karen L Bride, Derick Lim, Michele Paessler, Char M Witmer, Michele P Lambert
BACKGROUND: Differentiating childhood immune thrombocytopenia (ITP) from other cause of thrombocytopenia remains a diagnosis of exclusion. Additionally factors that predict bleeding risk for those patients with ITP are currently not well understood. Previous small studies have suggested that immature platelet fraction (IPF) may differentiate ITP from other causes of thrombocytopenia and in combination with other factors may predict bleeding risk. METHODS: We performed a retrospective chart review of thrombocytopenic patients with an IPF measured between November 1, 2013 and July 1, 2015...
September 17, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28845713/thrombopoietin-receptor-agonists-for-children-with-immune-thrombocytopenia-a-systematic-review
#9
Jiaxing Zhang, Yi Liang, Yuan Ai, Juan Xie, Youping Li, Wenyi Zheng
OBJECTIVE: We conducted a systematic review to assess the efficacy and safety of Thrombopoietin-receptor agonists (TPOras) for pediatric immune thrombocytopenia (ITP). METHODS: We searched PubMed, Embase and Cochrane Library from their earliest records to January 2017. Randomized controlled trials (RCTs) were included. Primary outcomes were durable response and clinically significant bleeding. Secondary outcomes were overall response, overall bleeding events, the use of rescue medication and adverse events (AEs)...
October 2017: Expert Opinion on Pharmacotherapy
https://www.readbyqxmd.com/read/28698848/treatment-and-response-of-autoimmune-cytopenia-occurring-after-allogeneic-hematopoietic-cell-transplantation-in-children
#10
Seok Hwang-Bo, Seong-Koo Kim, Jae Wook Lee, Pil-Sang Jang, Nack-Gyun Chung, Dae-Chul Jeong, Bin Cho, Hack-Ki Kim
BACKGROUND: Autoimmune cytopenia (AIC) is a rare complication of allogeneic hematopoietic cell transplantation (HCT). In this study, we reviewed the diagnosis, treatment and response to therapy for pediatric patients with post-HCT AIC at our institution. METHODS: Of the 292 allogeneic HCTs performed from January, 2011 to December, 2015 at the Department of Pediatrics, The Catholic University of Korea, seven were complicated by post-HCT AIC, resulting in an incidence of 2...
June 2017: Blood Research
https://www.readbyqxmd.com/read/28627134/u-s-food-and-drug-administration-approval-summary-eltrombopag-for-the-treatment-of-pediatric-patients-with-chronic-immune-idiopathic-thrombocytopenia
#11
Lori A Ehrlich, Virginia E Kwitkowski, Gregory Reaman, Chia-Wen Ko, Lei Nie, Richard Pazdur, Ann T Farrell
The U.S. Food and Drug Administration (FDA) approved eltrombopag for pediatric patients with chronic immune (idiopathic) thrombocytopenia (ITP) ages ≥6 on June 11, 2015, and ages ≥1 on August 24, 2015. Approval was based on the FDA review of two randomized trials that included 159 pediatric patients with chronic ITP who had an insufficient response to corticosteroids, immunoglobulins, or splenectomy. This manuscript describes the basis for approval of these applications. The FDA concluded that eltrombopag has shown efficacy and a favorable benefit to risk profile for pediatric patients with chronic ITP...
December 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28537785/a-practical-guide-to-the-use-of-eltrombopag-in-children-with-chronic-immune-thrombocytopenia
#12
John D Grainger, Sharon Thind
INTRODUCTION: Pediatric immune thrombocytopenia (ITP) may be associated with significant burden on children and their parents/caregivers. Thrombopoietin (TPO) receptor agonists (RAs) have been used to treat adult patients with chronic ITP (cITP) for nearly a decade and following pediatric studies Eltrombopag has been recently approved for pediatric cITP in the United States and Europe. TPO-RA s may help reduce the risk of bleeding and the need for conventional ITP therapies. REVIEW: In this review, the clinical data demonstrating the efficacy and safety of TPO-RAs in pediatric ITP are evaluated, key recommendations regarding safe administration of eltrombopag are provided, and potential future directions in management of pediatric ITP are discussed...
March 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/28278270/methylation-level-of-cpg-islands-in-ggh-gene-promoter-in-pediatric-acute-leukemia
#13
Yue Li, Sixi Liu, Huihui Wang, Huirong Mai, Xiuli Yuan, Changgang Li, Xiaowen Chen, Feiqiu Wen
BACKGROUND: γ-Glutamyl hydrolase (GGH) regulates intracellular folates and antifolates such as methotrexate (MTX) for proper nucleotide biosynthesis and antifolate-induced cytotoxicity, respectively. In addition to genetic polymorphism and karyotypic abnormalities, methylation of CpG island 1 (CpG1) in the promoter region is found to modulate GGH activity by reducing GGH mRNA expression in acute lymphoblastic leukemia (ALL) cells. We aim to investigate methylation status of two CpG islands (CpG1 and CpG2) in the GGH promoter region in pediatric patients with ALL and acute myelogenous leukemia (AML)...
2017: PloS One
https://www.readbyqxmd.com/read/28267088/sirolimus-as-an-effective-agent-in-the-treatment-of-immune-thrombocytopenia-itp-and-evans-syndrome-es-a-single-institution-s-experience
#14
Sylwia Jasinski, Mark E Weinblatt, Chana L Glasser
BACKGROUND: Autoimmune cytopenias are characterized by immune-mediated destruction of hematopoietic cell lines with immune thrombocytopenia (ITP) affecting platelets and Evans syndrome (ES) affecting platelets and red blood cells. For patients with persistent disease, limited options for effective and well-tolerated therapies exist. OBJECTIVES: Our aim is to describe our institution's experience with sirolimus as therapy for pediatric patients with persistent ITP and ES...
August 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28183683/study-of-cd4-cd8-and-natural-killer-cells-cd16-cd56-in-children-with-immune-thrombocytopenic-purpura
#15
Farida Hussein El-Rashedi, Mahmoud Ahmed El-Hawy, Mohamed Ahmed Helwa, Sameh Said Abd-Allah
OBJECTIVE/BACKGROUND: To assess the percentage of CD4(+), CD8(+), and natural killer cells (CD16(+), CD56(+)) in children with immune thrombocytopenic purpura (ITP) at presentation and study their impact on disease chronicity. METHODS: This case-control study was conducted at the Pediatric Hematology and Oncology Unit, Menoufia University Hospital (tertiary care center in Egypt). The study was held on 30 children presenting with ITP; they were followed-up and classified into two groups: 15 children with acute ITP; and 15 children with chronic ITP...
March 2017: Hematology/oncology and Stem Cell Therapy
https://www.readbyqxmd.com/read/28120600/immediate-adverse-reactions-to-intravenous-immunoglobulin-in-children-a-single-center-experience
#16
S Kaba, G Keskindemirci, C Aydogmus, R Siraneci, F Erol Cipe
Intravenous immunoglobulin (IVIG) is commonly used in primary and secondary immunodeficiency diseases as well as autoimmune conditions as immunomodulatator treatment. Immediate adverse events which are generally mild and occur during infusion are seen in 6 hours. Reported immediate adverse events are in a wide range from 1%-40% in pediatric patients. 115 patients who received IVIG (except newborns) were included into this crosssectional study. IVIG was given to patients for primary immunodeficiencies (n=8), ITP (n=65), Kawasaki disease (n=11), secondary immunosupression (n=28), and passive immunization (n=3)...
January 2017: European Annals of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28099398/utilizing-a-novel-mobile-health-selfie-application-to-improve-compliance-to-iron-chelation-in-pediatric-patients-receiving-chronic-transfusions
#17
Sarah Leonard, Lindsay M Anderson, Jude Jonassaint, Charles Jonassaint, Nirmish Shah
Iron chelation therapy can prevent iron overload for pediatric patients with sickle cell disease and β-thalassemia major; however, adherence is suboptimal. Therefore, we developed an intensive training program (ITP), to improve medication management and disease knowledge. The objectives were to determine feasibility of the ITP and its preliminary impact on adherence, disease knowledge, and health outcomes. Pediatric patients were recruited to participate in the ITP over a 90-day period and were followed for 6 months...
April 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27940665/recurrent-immune-thrombocytopenia-after-influenza-vaccination-a-case-report
#18
Uri Hamiel, Iris Kventsel, Ilan Youngster
Immune thrombocytopenia (ITP) is an isolated autoimmune condition, often preceded by a viral infection. Vaccines, mainly the measles-mumps-rubella vaccine, have also been associated with an increased risk of developing the disease. Although some case reports of ITP after influenza immunization in adults have been published, epidemiologic studies examining the role of the influenza vaccine as a trigger of ITP have not conclusively proven causality. We report a child with 3 occurrences of ITP, each within 1 week of receiving the influenza trivalent inactivated vaccine...
December 2016: Pediatrics
https://www.readbyqxmd.com/read/27784385/-analysis-of-hpa-gene-polymorphisms-in-children-with-acute-or-chronic-itp
#19
Li-Juan Tang, Xiao-Heng Zhou, Qiong Bin, Xiao-Hua Huang, Jiang-Ming Luo
OBJECTIVE: To investigate the frequency distribution of human platelet antigen allele HPA-2 and HPA-15 among the pediatric patients with acute or chronic idiopathic thrombocytopenic purpura (ITP) in Chinese Guangxi area, and to explore the potential correlation of ITP with HPA-2 and HPA-15 gene polymorphisms. METHODS: The clinical and laboratorial data of 46 children diagnosed as acute ITP and 46 children diagnosed as chronic ITP between January 2007 and December 2014 were collected...
October 2016: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/27781392/increasing-observation-rates-in-low-risk-pediatric-immune-thrombocytopenia-using-a-standardized-clinical-assessment-and-management-plan-scamp-%C3%A2
#20
Michelle L Schoettler, Dionne Graham, Wen Tao, Margaret Stack, Elaine Shu, Lauren Kerr, Ellis J Neufeld, Rachael F Grace
An observational approach is recommended in newly diagnosed children with immune thrombocytopenia (ITP) at low risk of bleeding; however, there is no standard definition of risk. A standardized clinical assessment and management plan (SCAMP(®) ), a modifiable practice guideline, was implemented and revised (SCAMP-1 and SCAMP-2) and applied to 71 newly diagnosed patients with ITP. The Buchanan and Adix bleeding score guided treatment and was modified by stratifying by low- and high-risk grade 3 bleeding in SCAMP-2...
May 2017: Pediatric Blood & Cancer
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