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James N. George - One of the best experts on this subject based on the ideXlab platform.
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Hereditary Thrombotic Thrombocytopenic Purpura.
The New England journal of medicine, 2019Co-Authors: Johanna A. Kremer Hovinga, James N. GeorgeAbstract:Hereditary Thrombotic Thrombocytopenic Purpura Hereditary thrombotic thrombocytopenic Purpura is an autosomal recessive disorder caused by the absence of a functional protease (ADAMTS13) that proce...
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The association of pregnancy with thrombotic thrombocytopenic Purpura-hemolytic uremic syndrome.
Current opinion in hematology, 2003Co-Authors: James N. GeorgeAbstract:Thrombotic thrombocytopenic Purpura-hemolytic uremic syndrome occurs more commonly in women and among women is commonly associated with pregnancy. Case series of thrombotic thrombocytopenic Purpura-hemolytic uremic syndrome from 1964 to 2002 were reviewed (1) to document the reports of occurrence of thrombotic thrombocytopenic Purpura-hemolytic uremic syndrome during pregnancy and (2) to search for reports of women with congenital or familial thrombotic thrombocytopenic Purpura-hemolytic uremic syndrome who were initially diagnosed during their first pregnancy. The time during pregnancy with greatest risk for development of thrombotic thrombocytopenic Purpura-hemolytic uremic syndrome is near term and during the postpartum period. This is also the time of greatest risk for thrombotic events and for the occurrence of other pregnancy-related syndromes: preeclampsia, eclampsia, and hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome. These other syndromes may also be associated with thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms, and renal insufficiency, making their distinction from thrombotic thrombocytopenic Purpura-hemolytic uremic syndrome difficult or impossible. The occurrence of preeclampsia and related syndromes, the hypercoaguable state that occurs in late pregnancy and postpartum, and the progressively decreasing concentration of ADAMTS13 that occurs during late pregnancy may combine to increase the risk for occurrence of thrombotic thrombocytopenic Purpura-hemolytic uremic syndrome.
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self reported diagnostic and management strategies in childhood idiopathic thrombocytopenic Purpura results of a survey of practicing pediatric hematology oncology specialists
Journal of Pediatric Hematology Oncology, 2000Co-Authors: Sara K Vesely, Gary E. Raskob, George R Buchanan, Alan R Cohen, James N. GeorgeAbstract:Purpose: To assess current physician self-reported practices regarding initial management of childhood idiopathic thrombocytopenic Purpura (ITP) and to determine physician self-reported willingness to participate in randomized clinical trials comparing different initial management strategies. Patients and Methods: A questionnaire was mailed in November 1997 to all 720 members of the American Society of Pediatric Hematology/Oncology asking how they would diagnose and manage ITP in children 18 months, 5 years, and 15 years of age who were experiencing either dry Purpura (cutaneous hemorrhage only) or wet Purpura (active mucous membrane hemorrhage). Specific questions dealt with bone marrow aspiration, hospital admittance, treatment strategy, and specific doses of corticosteroids and intravenous immunoglobulin. Results: The response rate to the questionnaire was 57%. Most respondents indicated they usually perform a bone marrow aspirate when corticosteroids are to be prescribed and administer drug therapy to patients with newly diagnosed ITP with wet or dry Purpura. Only 16% of respondents would administer no drug therapy to a child with dry Purpura. Intravenous immunoglobulin (IVIG) was preferred to steroids, with anti-D immunoglobulin prescribed less frequently. Hospital admittance often was used for patients with dry Purpura and usually recommended for patients with wet Purpura. Most respondents expressed willingness to randomize patients with dry Purpura to IVIG versus no therapy and those with wet Purpura to IVIG versus prednisone as part of a randomized controlled clinical trial. Conclusions: The self-reported care of the patient with ITP was influenced by the severity of presentation (dry versus wet Purpura). Most physicians reported they would administer specific drug treatment in both scenarios. This survey illustrates the diverse diagnostic and management strategies currently used in childhood ITP. Because no one therapeutic approach is predominant and a scientific basis for decision making in childhood ITP has not been developed, future randomized trials are warranted. On the basis of these survey results, such trials are desired by most pediatric hematology/oncology specialists.
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Chronic Idiopathic Thrombocytopenic Purpura
The New England journal of medicine, 1994Co-Authors: James N. George, Mayez A. El-harake, Gary E. RaskobAbstract:Idiopathic thrombocytopenic Purpura (ITP), also referred to as primary immune thrombocytopenic Purpura, is defined by a low platelet count, normal bone marrow, and the absence of other causes of th...
Nicolas De Prost - One of the best experts on this subject based on the ideXlab platform.
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pneumococcal Purpura fulminans in asplenic or hyposplenic patients a french multicenter exposed unexposed retrospective cohort study
Critical Care, 2020Co-Authors: Damien Contou, Remi Coudroy, Gwenhael Colin, Jeanmarc Tadie, Martin Cour, Romain Sonneville, Armand Mekontso Dessap, Nicolas De ProstAbstract:Pneumococcal infections remain the main cause of overwhelming post-splenectomy infections, and Purpura fulminans may develop in almost 20% of patients with overwhelming post-splenectomy infection. We aimed at describing the impact of asplenia/hyposplenia on the clinical features and the outcomes of adult patients admitted to the intensive care unit (ICU) for pneumococcal Purpura fulminans. A 17-year national multicenter retrospective cohort study included adult patients admitted to 55 French ICUs for an infectious Purpura fulminans from 2000 to 2016. Patients with pneumococcal Purpura fulminans were analyzed according to the absence or presence of asplenia/hyposplenia. Among the 306 patients admitted to the ICU for Purpura fulminans, 67 (22%) had a pneumococcal Purpura fulminans, of whom 34 (51%) had asplenia (n = 29/34, 85%) or hyposplenia (n = 5/34, 15%) and 33 (49%) had eusplenia. The prevalence of pneumococcal Purpura fulminans was seven times higher in asplenic/hyposplenic patients compared to eusplenic patients with Purpura fulminans (n = 34/39, 87% vs. n = 33/267, 12%; p < 0.001). The median time interval between the occurrence of asplenia/hyposplenia and ICU admission was 20 [9–32] years. Pneumococcal vaccine coverage was 35% in asplenic/hyposplenic patients. Purpura was more frequently reported before ICU admission in asplenic/hyposplenic patients (n = 25/34, 73% vs. n = 13/33, 39%; p = 0.01). The rate of bacteremia did not differ between asplenic/hyposplenic and eusplenic patients (n = 31/34, 91% vs n = 27/33, 82%; p = 0.261). SAPS II (60 ± 14 vs. 60 ± 18; p = 0.244) and SOFA (13 [1–5] vs. 14 [1–4, 6]; p = 0.48) scores did not differ between asplenic/hyposplenic and eusplenic patients. There were no significant differences between asplenic/hyposplenic and eusplenic patients regarding the rate of limb amputation (n = 9/34, 26% vs. 15/33, 45%; p = 0.11) and hospital mortality (n = 20/34, 59% vs. n = 15/33, 45%; p = 0.27). Half of pneumococcal Purpura fulminans episodes occurred in asplenic or hyposplenic patients. Pneumococcal vaccine coverage was reported in one third of asplenic/hyposplenic patients. Half of pneumococcal Purpura fulminans episodes occurred more than 20 years after splenectomy. Outcomes of pneumococcal Purpura fulminans did not show significant differences between patients with or without asplenia or hyposplenia, although the small number of patients included limited our power to detect potential differences between groups.
Simon J Hambidge - One of the best experts on this subject based on the ideXlab platform.
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a population based multisite cohort study of the predictors of chronic idiopathic thrombocytopenic Purpura in children
Pediatrics, 2008Co-Authors: Jason M Glanz, Taru Hayes, Simon J HambidgeAbstract:OBJECTIVE. The objective of this study was to identify risk factors for developing chronic idiopathic thrombocytopenic Purpura in a cohort of pediatric patients with idiopathic thrombocytopenic Purpura. METHODS. We conducted a retrospective cohort analysis of 259 children who were diagnosed with idiopathic thrombocytopenic Purpura between 1991 and 2000 at 1 of 8 managed care organizations that comprise the Vaccine Safety Datalink. We reviewed the charts of 595 potential patients with idiopathic thrombocytopenic Purpura from the 8 Vaccine Safety Datalink sites and excluded patients with known causes of thrombocytopenia. Chronic idiopathic thrombocytopenic Purpura was defined as having thrombocytopenia for 6 months beyond the initial diagnosis. The risk for developing chronic idiopathic thrombocytopenic Purpura was assessed using simple and multivariable analyses. RESULTS. Of the 259 cases of idiopathic thrombocytopenic Purpura, 197 (76%) were acute, 60 (23%) were chronic, and 2 (1%) could not be determined. Among the acute cases, the mean duration of illness was 22 days. There was 1 serious bleeding outcome in the cohort. In multivariable regression analysis, the patients with chronic illness were older, less likely to present with mucosal bleeding, less likely to have had an acute illness before diagnosis, and more likely to present with a platelet count >20000/μL than children with acute idiopathic thrombocytopenic Purpura. In particular, children whose illness was diagnosed at ≥10 years of age and who had platelet counts ≥20000/μL had an approximate fivefold risk for progressing to chronic disease when compared with children who presented at ≤2 years of age with platelet counts CONCLUSIONS. Although idiopathic thrombocytopenic Purpura tends to be a benign and self-limited condition, acute and chronic idiopathic thrombocytopenic Purpura seem to be distinct illnesses defined by age, platelet count, bleeding symptoms, and the presence of acute illness before diagnosis. Physicians should be aware of these differences when advising their patients and families.
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risk of immune thrombocytopenic Purpura after measles mumps rubella immunization in children
Pediatrics, 2008Co-Authors: Jason M Glanz, Simon J Hambidge, Kristi Yamasaki, Steve Black, Michael Marcy, John P Mullooly, Lisa A Jackson, James D Nordin, Edward A Belongia, Katherine H HohmanAbstract:BACKGROUND. The measles-mumps-rubella vaccine has been associated with immune thrombocytopenia Purpura in 2 small studies. METHODS. By using the Vaccine Safety Datalink, we identified measles-mumps-rubella–vaccinated children aged 1 to 18. A case of immune thrombocytopenia Purpura was defined as a patient with a platelet count of ≤50000/μL with clinical bleeding and normal red and white blood cell indices. The immune thrombocytopenia Purpura incidence rates during exposed (42 days after vaccination) and unexposed time periods were determined. A retrospective cohort of vaccinated children was used to determine incident rate ratios for children aged 1 to 18 years, 12 to 23 months, and 12 to 15 months. RESULTS. A total of 1036689 children received 1107814 measles-mumps-rubella vaccinations; there were 259 confirmed patients with immune thrombocytopenia Purpura. Because only 5 exposed cases occurred after age 2, analyses were limited to children aged 12 to 23 months. Exposed patients aged 12 to 23 months had lower median platelet counts than those who were unexposed and had similar median duration of illness (11 vs 10 days). The incident rate ratio was highest for children aged 12 to 15 months at 7.10. The incident rate ratio for boys aged 12 to 15 months was 14.59, and the incident rate ratio for girls in the same age group was 3.22. Seventy-six percent of immune thrombocytopenia Purpura cases in children aged 12 to 23 months were attributable to measles-mumps-rubella vaccination. This vaccine causes 1 case of immune thrombocytopenia Purpura per every 40000 doses. CONCLUSION. Measles-mumps-rubella vaccine that is given in the second year of life is associated with an increased risk of immune thrombocytopenia Purpura.
Remi Coudroy - One of the best experts on this subject based on the ideXlab platform.
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pneumococcal Purpura fulminans in asplenic or hyposplenic patients a french multicenter exposed unexposed retrospective cohort study
Critical Care, 2020Co-Authors: Damien Contou, Remi Coudroy, Gwenhael Colin, Jeanmarc Tadie, Martin Cour, Romain Sonneville, Armand Mekontso Dessap, Nicolas De ProstAbstract:Pneumococcal infections remain the main cause of overwhelming post-splenectomy infections, and Purpura fulminans may develop in almost 20% of patients with overwhelming post-splenectomy infection. We aimed at describing the impact of asplenia/hyposplenia on the clinical features and the outcomes of adult patients admitted to the intensive care unit (ICU) for pneumococcal Purpura fulminans. A 17-year national multicenter retrospective cohort study included adult patients admitted to 55 French ICUs for an infectious Purpura fulminans from 2000 to 2016. Patients with pneumococcal Purpura fulminans were analyzed according to the absence or presence of asplenia/hyposplenia. Among the 306 patients admitted to the ICU for Purpura fulminans, 67 (22%) had a pneumococcal Purpura fulminans, of whom 34 (51%) had asplenia (n = 29/34, 85%) or hyposplenia (n = 5/34, 15%) and 33 (49%) had eusplenia. The prevalence of pneumococcal Purpura fulminans was seven times higher in asplenic/hyposplenic patients compared to eusplenic patients with Purpura fulminans (n = 34/39, 87% vs. n = 33/267, 12%; p < 0.001). The median time interval between the occurrence of asplenia/hyposplenia and ICU admission was 20 [9–32] years. Pneumococcal vaccine coverage was 35% in asplenic/hyposplenic patients. Purpura was more frequently reported before ICU admission in asplenic/hyposplenic patients (n = 25/34, 73% vs. n = 13/33, 39%; p = 0.01). The rate of bacteremia did not differ between asplenic/hyposplenic and eusplenic patients (n = 31/34, 91% vs n = 27/33, 82%; p = 0.261). SAPS II (60 ± 14 vs. 60 ± 18; p = 0.244) and SOFA (13 [1–5] vs. 14 [1–4, 6]; p = 0.48) scores did not differ between asplenic/hyposplenic and eusplenic patients. There were no significant differences between asplenic/hyposplenic and eusplenic patients regarding the rate of limb amputation (n = 9/34, 26% vs. 15/33, 45%; p = 0.11) and hospital mortality (n = 20/34, 59% vs. n = 15/33, 45%; p = 0.27). Half of pneumococcal Purpura fulminans episodes occurred in asplenic or hyposplenic patients. Pneumococcal vaccine coverage was reported in one third of asplenic/hyposplenic patients. Half of pneumococcal Purpura fulminans episodes occurred more than 20 years after splenectomy. Outcomes of pneumococcal Purpura fulminans did not show significant differences between patients with or without asplenia or hyposplenia, although the small number of patients included limited our power to detect potential differences between groups.
Jason M Glanz - One of the best experts on this subject based on the ideXlab platform.
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a population based multisite cohort study of the predictors of chronic idiopathic thrombocytopenic Purpura in children
Pediatrics, 2008Co-Authors: Jason M Glanz, Taru Hayes, Simon J HambidgeAbstract:OBJECTIVE. The objective of this study was to identify risk factors for developing chronic idiopathic thrombocytopenic Purpura in a cohort of pediatric patients with idiopathic thrombocytopenic Purpura. METHODS. We conducted a retrospective cohort analysis of 259 children who were diagnosed with idiopathic thrombocytopenic Purpura between 1991 and 2000 at 1 of 8 managed care organizations that comprise the Vaccine Safety Datalink. We reviewed the charts of 595 potential patients with idiopathic thrombocytopenic Purpura from the 8 Vaccine Safety Datalink sites and excluded patients with known causes of thrombocytopenia. Chronic idiopathic thrombocytopenic Purpura was defined as having thrombocytopenia for 6 months beyond the initial diagnosis. The risk for developing chronic idiopathic thrombocytopenic Purpura was assessed using simple and multivariable analyses. RESULTS. Of the 259 cases of idiopathic thrombocytopenic Purpura, 197 (76%) were acute, 60 (23%) were chronic, and 2 (1%) could not be determined. Among the acute cases, the mean duration of illness was 22 days. There was 1 serious bleeding outcome in the cohort. In multivariable regression analysis, the patients with chronic illness were older, less likely to present with mucosal bleeding, less likely to have had an acute illness before diagnosis, and more likely to present with a platelet count >20000/μL than children with acute idiopathic thrombocytopenic Purpura. In particular, children whose illness was diagnosed at ≥10 years of age and who had platelet counts ≥20000/μL had an approximate fivefold risk for progressing to chronic disease when compared with children who presented at ≤2 years of age with platelet counts CONCLUSIONS. Although idiopathic thrombocytopenic Purpura tends to be a benign and self-limited condition, acute and chronic idiopathic thrombocytopenic Purpura seem to be distinct illnesses defined by age, platelet count, bleeding symptoms, and the presence of acute illness before diagnosis. Physicians should be aware of these differences when advising their patients and families.
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risk of immune thrombocytopenic Purpura after measles mumps rubella immunization in children
Pediatrics, 2008Co-Authors: Jason M Glanz, Simon J Hambidge, Kristi Yamasaki, Steve Black, Michael Marcy, John P Mullooly, Lisa A Jackson, James D Nordin, Edward A Belongia, Katherine H HohmanAbstract:BACKGROUND. The measles-mumps-rubella vaccine has been associated with immune thrombocytopenia Purpura in 2 small studies. METHODS. By using the Vaccine Safety Datalink, we identified measles-mumps-rubella–vaccinated children aged 1 to 18. A case of immune thrombocytopenia Purpura was defined as a patient with a platelet count of ≤50000/μL with clinical bleeding and normal red and white blood cell indices. The immune thrombocytopenia Purpura incidence rates during exposed (42 days after vaccination) and unexposed time periods were determined. A retrospective cohort of vaccinated children was used to determine incident rate ratios for children aged 1 to 18 years, 12 to 23 months, and 12 to 15 months. RESULTS. A total of 1036689 children received 1107814 measles-mumps-rubella vaccinations; there were 259 confirmed patients with immune thrombocytopenia Purpura. Because only 5 exposed cases occurred after age 2, analyses were limited to children aged 12 to 23 months. Exposed patients aged 12 to 23 months had lower median platelet counts than those who were unexposed and had similar median duration of illness (11 vs 10 days). The incident rate ratio was highest for children aged 12 to 15 months at 7.10. The incident rate ratio for boys aged 12 to 15 months was 14.59, and the incident rate ratio for girls in the same age group was 3.22. Seventy-six percent of immune thrombocytopenia Purpura cases in children aged 12 to 23 months were attributable to measles-mumps-rubella vaccination. This vaccine causes 1 case of immune thrombocytopenia Purpura per every 40000 doses. CONCLUSION. Measles-mumps-rubella vaccine that is given in the second year of life is associated with an increased risk of immune thrombocytopenia Purpura.
