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1

Genotype and Phenotype Correlation in Hereditary Thrombotic Thrombocytopenic Purpura (Upshaw-Schulman Syndrome)  

ClinicalTrials.gov

Thrombotic Thrombocytopenic Purpura; Congenital Thrombotic Thrombocytopenic Purpura; Familial Thrombotic Thrombocytopenic Purpura; Thrombotic Thrombocytopenic Purpura, Congenital; Upshaw-Schulman Syndrome

2014-06-17

2

Thrombotic thrombocytopenic purpura preceding systemic lupus erythematosus.  

PubMed Central

The case of a patient admitted with thrombotic thrombocytopenic purpura nine years after developing systemic lupus erythematosus (SLE) is reported. Thrombotic thrombocytopenic purpura associated with SLE has been described on other occasions, but in most patients the diagnosis of SLE precedes that of thrombotic thrombocytopenic purpura. The unusual sequence and the chronological separation of the two diseases is emphasised.

Simeon-Aznar, C P; Cuenca-Luque, R; Fonollosa-Pla, V; Bosch-Gil, J A

1992-01-01

3

Management of immune thrombocytopenic purpura in adults.  

PubMed

Primary immune thrombocytopenic purpura (ITP), also referred to as idiopathic thrombocytopenic purpura, is an organ-specific autoimmune disorder in which antibody-coated or immune complex-coated platelets are destroyed prematurely by the reticuloendothelial system, resulting in peripheral blood thrombocytopenia. The disease is heterogeneous with regard to its severity and clinical course and is unpredictable in its response to therapy. Although the basic underlying pathophysiology of ITP has been known for more than 50 years, current treatment guidelines are based on expert opinion rather than on evidence because of a lack of high-quality clinical trials and research. The only patients for whom treatment is clearly required are those with severe bleeding and/or extremely low platelet counts (< 10 x 10(9)/L). Treatment of patients with ITP refractory to corticosteroids and splenectomy requires careful evaluation of disease severity, patient characteristics related to risk of bleeding, and adverse effects associated with treatment. Clinical trials with numerous new agents are under way, which we hope will add more effective and targeted strategies to our therapeutic armamentarium. We describe a logical and structured approach to the clinical management of ITP in adults, based on a literature review and our personal experience. PMID:15065616

Stasi, Roberto; Provan, Drew

2004-04-01

4

Idiopathic thrombocytopenic purpura associated with Crohn's disease.  

PubMed

Besides cytopenia related to treatment, several hematological disorders such as anemia, abnormal platelet activity, thrombosis, presence of anticardiolipin or anti-neutrophil antibodies, cyclic neutropenia, and myelodysplasia, have been reported in patients with Crohn's disease (CD). The case we report here is the first one documenting the association of idiopathic thrombocytopenic purpura (ITP) with CD. PMID:9542329

Baudard, M; Molina, T; Benfiguig, K; Bethoux, J P; Zittoun, R

1998-01-01

5

Recent Advances in Thrombotic Thrombocytopenic Purpura  

Microsoft Academic Search

Thrombotic thrombocytopenic purpura (TTP) is characterized by microangiopathic hemolytic anemia and thrombocytopenia, accompanied by microvascular thrombosis that causes variable degrees of tissue ischemia and infarction. Intravascular coagulation is not a prominent feature of the disorder. Plasma exchange can induce remissions in approximately 80% of patients with idiopathic TTP, but patients have a much worse prognosis when thrombotic microangiopathy is associated

J. Evan Sadler; Joel L. Moake; Toshiyuki Miyata; James N. George

2004-01-01

6

Acute pancreatitis induced thrombotic thrombocytopenic purpura  

PubMed Central

Thrombotic thrombocytopenic purpura (TTP) is a rare syndrome of unknown cause with an estimated incidence of one case per million. The disease is characterized by a pentad of symptoms: Thrombocytopenia, microangiopathic hemolytic anemia, neurologic changes, renal dysfunction, and fever. It causes thrombosis in the microvasculature of several organs, producing diverse manifestations. Acute pancreatitis (AP) is a well-described consequence of TTP. Acute pancreatitis triggering TTP is uncommon.

Ali, Mohammed Arshad; Shaheen, Jalal Syed Shafiuddin; Khan, Mohammed Ali

2014-01-01

7

Thrombotic thrombocytopenic purpura precipitated by thyrotoxicosis.  

PubMed

Thrombotic thrombocytopenic purpura (TTP) may be seen in association with autoimmune disorders such as immune hemolytic anemia and systemic lupus erythematosus, but rarely has it been associated with Graves' disease. We report one such case of a woman with new-onset thyrotoxicosis caused by Graves' disease, who abruptly developed TTP, confirmed by low serum ADAMTS-13 value. She had a dramatic response to plasma exchange, with remission of TTP. Definitive treatment with radioactive iodine resulted in euthyroidism and has prevented recurrence of TTP. PMID:22644892

Chhabra, Saurabh; Tenorio, Grace

2012-11-01

8

Sudden death due to thrombotic thrombocytopenic purpura.  

PubMed

This case studies the clinical, laboratory, and pathologic findings observed in thrombotic thrombocytopenic purpura (TTP). Although TTP is a well-recognized syndrome, it is frequently undetected antemortem and represents a rare cause of sudden death. We recently were involved in a case of TTP in which an 18-year-old woman with no previous history died suddenly. The case was referred to our office for consultation because of a recent history of methamphetamine abuse. We report herewith our approach to the diagnostic workup of TTP and review relevant literature. PMID:3605013

Ross, W K; Newton, N E; Stivers, R R

1987-06-01

9

Idiopathic Thrombocytopenic Purpura after Mastectomy and Axillary Lymph Node Dissection  

PubMed Central

First described in 1916, idiopathic thrombocytopenic purpura (ITP) is an autoimmune disease resulting in the destruction of platelets. Here, we present a case of an 85-year-old patient diagnosed with invasive ductal carcinoma of the breast whose surgical treatment was complicated postoperatively by acute-onset thrombocytopenia with a resultant hematoma at the operative site. Diagnostic Workup revealed no clear etiology for the thrombocytopenia; therefore, a presumptive diagnosis of idiopathic thrombocytopenic purpura was made. Previous literature has associated the development of idiopathic thrombocytopenic purpura with breast cancer. However, to the authors' knowledge, there are no reported cases of ITP presenting immediately following surgical intervention for breast cancer in the absence of other etiologic factors.

Santivasi, Wil L.; Routt, Meghan M.; Terando, Alicia M.

2014-01-01

10

Inherited thrombotic thrombocytopenic purpura in children.  

PubMed

Congenital thrombotic thrombocytopenic purpura (TTP) or Upshaw-Schulman syndrome is caused by homozygous or compound heterozygous mutations in the ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) gene. We investigated 30 patients with congenital TTP and analyzed clinical data and underlying ADAMTS-13 mutations. All patients showed virtually no ADAMTS-13 activity in plasma. Individual disease burden ranged from mild courses with rare episodes of mild thrombocytopenia to severe courses with chronic kidney disease and central nervous system (CNS) lesions. Two patients died due to complications of TTP. If initiated in a timely manner, plasma transfusions offer a reliable treatment to prevent organ damage. We identified 30 different causative mutations in the ADAMTS-13 gene. Our data do not support the idea of a tight correlation between ADAMTS-13 genotype and severity of disease. The type and magnitude of exogenous triggers for acute bouts of TTP as well as endogenous individual factors participating in the inflammatory response likely represent the foremost determinants of individual clinical courses. Future developments should aim at improving early diagnosis of TTP. To improve feasibility of prophylaxis and treatment of congenital TTP, recombinant ADAMTS-13 therapeutics are highly anticipated. PMID:24816970

Hassenpflug, Wolf Achim; Budde, Ulrich; Schneppenheim, Sonja; Schneppenheim, Reinhard

2014-06-01

11

Priapism and penile gangrene due to thrombotic thrombocytopenic purpura.  

PubMed

A 51-year-old man presented with 4 days of idiopathic ischemic priapism. Partial detumescence was achieved with aspiration and injection with intracorporeal adrenaline. On further evaluation, the patient was diagnosed with thrombotic thrombocytopenic purpura. This was treated with plasma exchange and steroids. The penis was initially observed for 3 weeks, which subsequently became necrotic and infected, requiring debridement. After debridement of penile shaft skin, it became apparent that the entire penis was necrotic, necessitating total penectomy. The wound was closed secondarily 2 weeks later. Gangrene of the penis after priapism is very rare. Priapism associated with thrombotic thrombocytopenic purpura has not been reported previously in published data. PMID:19896177

Kwok, Benjamin; Varol, Celi

2010-01-01

12

Immune thrombocytopenic purpura in a liver transplant patient.  

PubMed

Thrombocytopenia is common during the early posttransplant period. Most cases are mild and recover in 1 month. Occasionally, severe thrombocytopenia may occur in the late posttransplant period. We report a 10-year-old boy with severe thrombocytopenia 3.5 years after liver transplant. We exclude secondary causes of thrombocytopenia. Bone marrow aspiration findings and treatment response were suggestive for immune thrombocytopenic purpura. The patient was treated with intravenous immunoglobulin at thrombocytopenia periods successfully. We continued tacrolimus, but preferred a reduced dose. In conclusion, immune thrombocytopenic purpura should be borne in mind as a reason of late onset, severe thrombocytopenia after liver transplant. PMID:24635822

Ünlüsoy Aksu, Aysel; E?rita? Gürkan, Ödül; Sar?, Sinan; Yenicesu, ?dil; Dalg?ç, Buket

2014-03-01

13

Idiopathic thrombocytopenic purpura and percutaneous coronary stenting: A dangerous duo?  

Microsoft Academic Search

Dual antiplatelet therapy is a mainstay in the management of patients undergoing coronary stenting. Whether this is also appropriate in patients with a diagnosis of idiopathic thrombocytopenic purpura (ITP) is unclear. We report the case of a 66-year-old man with ITP admitted for an acute coronary syndrome. On admission platelets were 110×109\\/L without petechiae or purpura, and coronary angiography revealed

Claudio Moretti; Maria Teresa Lucciola; Luisa Morena; Giuseppe Biondi-Zoccai; Antonio Laudito; Matteo Anselmino; Gian Paolo Trevi; Imad Sheiban

2008-01-01

14

Multivessel Coronary Thrombosis in a Patient with Idiopathic Thrombocytopenic Purpura  

PubMed Central

A 49-year-old woman who had idiopathic thrombocytopenic purpura was admitted to our hospital with severe chest pain. Electrocardiography revealed inferolateral myocardial infarction. The patient underwent immediate coronary angiography, which revealed thrombi in the left coronary system. Percutaneous intervention was not indicated, because the thrombi had occluded the distal segments of multiple coronary arteries. Administration of tirofiban satisfactorily dissolved the thrombi.

Yagmur, Julide; Cansel, Mehmet; Acikgoz, Nusret; Yagmur, Murat; Eyupkoca, Ferhat; Ermis, Necip; Akturk, Erdal

2012-01-01

15

Thrombocytopenic purpura as adverse reaction to recombinant hepatitis B vaccine  

Microsoft Academic Search

Three cases of immune thrombocytopenic purpura after the first dose of recombinant hepatitis B vaccine occurred in infants under 6 months of age. Other possible causes of this condition were excluded. Antiplatelet antibodies were present. A defect in platelet production was excluded in two children. Corticosteroid treatment was effective. Subsequent administration of other vaccines (against polio, diphtheria, and tetanus) did

F Ronchi; P Cecchi; F Falcioni; A Marsciani; G Minak; G Muratori; P L Tazzari; S Beverini

1998-01-01

16

Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura  

Microsoft Academic Search

Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura. The term thrombotic microangiopathy (TMA) defines a lesion of vessel wall thickening (mainly arterioles or capillaries), intraluminal platelet thrombosis, and partial or complete obstruction of the vessel lumina. Depending on whether renal or brain lesions prevail, two pathologically indistinguishable but somehow clinically different entities have been described: the hemolytic uremic syndrome

Piero Ruggenenti; Marina Noris; Giuseppe Remuzzi

2001-01-01

17

Idiopathic thrombocytopenic purpura with normal platelet survival time  

PubMed Central

Platelet survival has been measured in 16 patients with idiopathic thrombocytopenic purpura. Of this group, three patients had a normal result and surface counting did not show high splenic uptake. The clinical features of these patients are described. These patients have remained well for several years without treatment.

Donaldson, G. W. K.; Parker, A. C.; McArthur, M.; Richmond, J.

1971-01-01

18

Autopsy case of sudden maternal death from thrombotic thrombocytopenic purpura.  

PubMed

A 31-year-old pregnant woman was transferred to the emergency room at 27?weeks of gestation. She had one-day history of fever and upper abdominal pain. Soon after admission, she underwent cardiopulmonary arrest. Autopsy was performed and multiple microthrombi were seen within the small-caliber vessels of many organs, but not in the lungs. Immunohistochemical staining revealed that the thrombi were rich in von Willebrand factor. We also obtained results which showed severely deficient plasma a disintegrin-like and metalloprotease with thrombospondin motifs (ADAMTS) 13 activity and positive ADAMTS13 inhibitor, confirming a diagnosis of thrombotic thrombocytopenic purpura. As far as we know, in Japan, this is the first autopsy report of sudden maternal death from thrombotic thrombocytopenic purpura. We expect that the routine laboratory application of ADAMTS13 assays for unknown thrombocytopenic patients during pregnancy may help in differential diagnosis at an earlier stage of the disease and facilitate tailor-made therapeutic intervention. PMID:22765161

Yamamoto, Takuma; Fujimura, Yoshihiro; Emoto, Yuko; Kuriu, Yukiko; Iino, Morio; Matoba, Ryoji

2013-01-01

19

Management of Chronic Childhood Immune Thrombocytopenic Purpura: AIEOP Consensus Guidelines  

Microsoft Academic Search

Background\\/Objective: The management of chronic childhood idiopathic thrombocytopenic purpura (ITP) is distinct from acute ITP. Similar to the publication on acute ITP guidelines, the AIEOP (Associazione Italiana di Ematologia e Oncologia Pediatrica) considered it appropriate to develop consensus guidelines for chronic childhood ITP to provide useful and shared information for physicians, healthcare professionals, parents and patients. Design\\/Methods: A preliminary, evidence-based

Domenico De Mattia; Giovanni Carlo Del Vecchio; Giovanna Russo; Attilio De Santis; Ugo Ramenghi; Lucia Notarangelo; Momcilo Jankovic; Angelo Claudio Molinari; Marco Zecca; Bruno Nobili; Paola Giordano

2010-01-01

20

Multivessel coronary thrombosis in a patient with idiopathic thrombocytopenic purpura.  

PubMed

A 49-year-old woman who had idiopathic thrombocytopenic purpura was admitted to our hospital with severe chest pain. Electrocardiography revealed inferolateral myocardial infarction. The patient underwent immediate coronary angiography, which revealed thrombi in the left coronary system. Percutaneous intervention was not indicated, because the thrombi had occluded the distal segments of multiple coronary arteries. Administration of tirofiban satisfactorily dissolved the thrombi. PMID:23304046

Yagmur, Julide; Cansel, Mehmet; Acikgoz, Nusret; Yagmur, Murat; Eyupkoca, Ferhat; Ermis, Necip; Akturk, Erdal

2012-01-01

21

Functional properties of lymphocytes in idiopathic thrombocytopenic purpura  

Microsoft Academic Search

Idiopathic or immune thrombocytopenic purpura (ITP) is characterized by antibody-mediated destruction of platelets. The etiology is unknown. We postulated that increased autoantibody production in ITP might be attributable to either increased or prolonged expression of CD40 ligand (CD40L, CD154) in T or B lymphocytes, as has been previously observed in systemic lupus erythematosus (SLE). In addition, we hypothesized that ITP

Nicholas P Webber; John O Mascarenhas; Mary K Crow; James Bussel; Elaine J Schattner

2001-01-01

22

Perioperative care of a patient with refractory idiopathic thrombocytopenic purpura undergoing total knee arthroplasty.  

PubMed

Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder leading to low platelet count and an increased risk of bleeding. Major joint replacement surgery in a patient with ITP can be associated with severe postoperative bleeding. We present our experience of perioperative management in a patient with severe refractory chronic idiopathic thrombocytopenic purpura who successfully underwent a cemented total knee replacement. PMID:23269964

Singhal, Rohit; Gudimetla, Veera; Stewart, Andrew; Luscombe, Karen L; Charalambous, Charalambos P

2012-12-01

23

A report of disseminated adenocarcinoma presenting as thrombotic thrombocytopenic purpura  

PubMed Central

Thrombotic microangiopathies (TMAs) represent a heterogeneous group of diseases characterized by a microangiopathic hemolytic anemia, peripheral thrombocytopenia, and organ failure of variable severity. TMAs encompass thrombotic thrombocytopenic purpura (TTP), typically characterized by fever, central nervous system manifestations and hemolytic uremic syndrome (HUS), in which renal failure is the prominent abnormality. In patients with cancer TMAs may be related to various antineoplastic drugs or to the malignant disease itself. The reported series of patients with TMAs directly related to cancer are usually heterogeneous, retrospective, and encompass patients with hematologic malignancies with solid tumors or receiving chemotherapy, each of which may have distinct presentations and pathophysiological mechanisms. Patients with disseminated malignancy who present with microangiopathic hemolytic anemia and thrombocytopenia may be misdiagnosed as thrombotic thrombocytopenic purpura (TTP) Only a few cases of TTP secondary to metastatic adenocarcinoma are known in the literature. We present a case of a 34-year-old man with TTP syndrome secondary to metastatic small-bowel adenocarcinoma. Patients with disseminated malignancy had a longer duration of symptoms, more frequent presence of respiratory symptoms, higher lactate dehydrogenase levels, and more often failed to respond to plasma exchange treatment. A search for systemic malignancy, including a bone marrow biopsy, is appropriate when patients with TTP have atypical clinical features or fail to respond to plasma exchange.

Alonso, Joaquin Valle; Fonseca, Javier; Lopera, Elisa Lopera; Aguayo, Miguel Angel; Montes, Yelda Hernandez; Llamas, Jose Carlos

2011-01-01

24

Immune thrombocytopenic purpura in a child with thyroid hormone resistance - a rare presentation.  

PubMed

Resistance to thyroid hormone (RTH) is a rare entity characterized by a decreased target tissue responsiveness of thyroid hormones. Although immune thrombocytopenic purpura (ITP) has been reported with different thyroid disorders in the literature, its coexistence with RTH is not known. A 9-year-old girl presented with ITP and features of hypothyroidism in the form of goiter and growth retardation. She was subsequently found to have RTH. High-dose thyroid hormone replacement was required to overcome the resistance that not only ameliorated the features of hypothyroidism but also brought an apparent remission of ITP. PMID:23382302

Sarkar, Sumantra; Mondal, Rakesh; Nandi, Madhumita; Ghosh, Jagabandhu

2013-01-01

25

Splenectomy during pregnancy: treatment of refractory immune thrombocytopenic purpura.  

PubMed

Immune thrombocytopenic purpura (ITP) complicates 1-2/10 000 pregnancies and accounts for 5% of cases of pregnancy-associated thrombocytopenia. Corticosteroids and intravenous immunoglobulin remain the first-line therapy in pregnancy, and a majority of pregnant women respond to this conventional therapy. Other cytotoxic and immunosuppressive agents used for treatment in non-pregnant patients, for example, danazol, cyclophosphamide, vinca alkaloids and azathioprine, are potential teratogens and cannot be administered during pregnancy. For pregnant women with ITP who fail to respond to medical management and are at a significant risk of haemorrhage due to thrombocytopenia, splenectomy may be considered as an option. We report two cases of splenectomy during pregnancy for refractory ITP. In one patient, it was carried out at 24 weeks, and in the second patient it was carried out during the caesarean section. Splenectomy as a second-line option in cases of refractory severe ITP in pregnancy is discussed. PMID:24363245

Mahey, Reeta; Kaur, Simran Deep; Chumber, Sunil; Kriplani, Alka; Bhatla, Neerja

2013-01-01

26

Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura  

PubMed Central

Discoveries during the past decade have revolutionized our understanding of idiopathic thrombotic thrombocytopenic purpura (TTP). Most cases in adults are caused by acquired autoantibodies that inhibit ADAMTS13, a metalloprotease that cleaves von Willebrand factor within nascent platelet-rich thrombi to prevent hemolysis, thrombocytopenia, and tissue infarction. Although approximately 80% of patients respond to plasma exchange, which removes autoantibody and replenishes ADAMTS13, one third to one half of survivors develop refractory or relapsing disease. Intensive immunosuppressive therapy with rituximab appears to be effective as salvage therapy, and ongoing clinical trials should determine whether adjuvant rituximab with plasma exchange also is beneficial at first diagnosis. A major unanswered question is whether plasma exchange is effective for the subset of patients with idiopathic TTP who do not have severe ADAMTS13 deficiency.

2008-01-01

27

Pulmonary Hyalinizing Granuloma Associated with Idiopathic Thrombocytopenic Purpura  

PubMed Central

Pulmonary hyalinizing granuloma (PHG) is a rare, benign lung disease of unknown etiology. It manifests as discrete, rounded nodules within the lung parenchyma. A 39-year-old woman presented for investigation after pulmonary nodules were found incidentally. Chest computed tomography showed multiple, discrete, non-enhancing pulmonary nodules bilaterally. Positron emission tomography (PET) was negative. Biopsy demonstrated a non-specific lymphoplasmacytic infiltrate. Open resection yielded two nodules consistent with hyalinizing granulomas. The differential for multiple pulmonary nodules is broad. PET scan can help rule out metastatic disease, although some cancers are not hypermetabolic on PET. Furthermore, some non-malignant conditions, including hyalinizing granuloma, can show increased activity on PET. PHG should be included in the differential of multiple pulmonary nodules, especially if nodule stability can be demonstrated and/or needle biopsies are non-diagnostic. Associated immune-mediated conditions, such as idiopathic thrombocytopenic purpura (ITP) in our patient, may also favor HG. In this case report we find an association between PHG and ITP.

Coleman, Christopher; Nassar, Aziza; McComb, Barbara

2014-01-01

28

Pulmonary hyalinizing granuloma associated with idiopathic thrombocytopenic purpura.  

PubMed

Pulmonary hyalinizing granuloma (PHG) is a rare, benign lung disease of unknown etiology. It manifests as discrete, rounded nodules within the lung parenchyma. A 39-year-old woman presented for investigation after pulmonary nodules were found incidentally. Chest computed tomography showed multiple, discrete, non-enhancing pulmonary nodules bilaterally. Positron emission tomography (PET) was negative. Biopsy demonstrated a non-specific lymphoplasmacytic infiltrate. Open resection yielded two nodules consistent with hyalinizing granulomas. The differential for multiple pulmonary nodules is broad. PET scan can help rule out metastatic disease, although some cancers are not hypermetabolic on PET. Furthermore, some non-malignant conditions, including hyalinizing granuloma, can show increased activity on PET. PHG should be included in the differential of multiple pulmonary nodules, especially if nodule stability can be demonstrated and/or needle biopsies are non-diagnostic. Associated immune-mediated conditions, such as idiopathic thrombocytopenic purpura (ITP) in our patient, may also favor HG. In this case report we find an association between PHG and ITP. PMID:24744965

Coleman, Christopher; Nassar, Aziza; McComb, Barbara

2014-01-01

29

Autosensitization and immune complexes in chronic idiopathic thrombocytopenic purpura.  

PubMed Central

Argument exists as to whether platelet damage in chronic idiopathic thrombocytopenic purpura (ITP) is mediated by an autoimmune response to platelet antigen or by immune complexes. We have studied thirty-nine patients with ITP for evidence of (i) sensitization to platelet antigen, using a macrophage migration inhibition factor (MIF) assay, (ii) circulating immune complexes, using a Clq deviation technique, and (iii) serum-induced platelet 'immunoinjury', using a 3H-serotonin release assay. Eighty-one per cent of the patient group had a migration index of less than 0.8 (normal range 0.8-1.3), while 91% of those tested had a serum factor (presumably immune complexes) which bound to labelled Clq. The serotonin release assay was abnormal in 32% of twenty-two patients, and the results of this test bore no clear relationship to either those of the Clq deviation test or the MIF results. We conclude that most patients with ITP are sensitized to platelet-associated antigen, and have circulating immune complexes. The contribution of these factors to platelet destruction and the nature of the antigen in the complexes remain to be demonstrated. The serotonin release assay is less sensitive, non-specific, and has limited value in the diagnosis of ITP.

Clancy, R; Trent, R; Danis, V; Davidson, R

1980-01-01

30

Changes in splenic microcirculatory pathways in chronic idiopathic thrombocytopenic purpura.  

PubMed

The spleen plays a central role in the pathogenesis of chronic idiopathic thrombocytopenic purpura (ITP); it produces massive quantities of antiplatelet antibodies, leading to accelerated phagocytosis of platelets. Lymphoid hyperplasia typically occurs in the spleen, characterized by large numbers of lymphatic nodules with active germinal centers. Whether changes in splenic microcirculatory pathways also occur is not known. We have studied this question by scanning electron microscopy of corrosion casts, comparing spleens removed from patients with ITP with normal spleens obtained from organ transplant donors. The casts demonstrate two major changes in microcirculatory pathways in ITP. Firstly, a striking proliferation of arterioles and capillaries is found in the white pulp and marginal zone (MZ), seen as extensive vascularization in 92.3% of lymphatic nodules (n = 191) versus 0.6% (n = 224) in normal spleens. Secondly, the marginal sinus, a series of flattened, anastomosing vascular spaces between the white pulp and MZ, is absent in 89.4% of lymphatic nodules versus 4.9% in normal spleens. The cause of these microcirculatory changes, which may not be exclusive to ITP, is presently unknown. Absence of the marginal sinus may affect distribution of blood flow through the MZ such that platelets spend increased amounts of time in the proximity of macrophages. In the presence of antiplatelet antibodies found in ITP spleens, this delayed transit would lead to greatly increased platelet destruction. PMID:1884017

Schmidt, E E; MacDonald, I C; Groom, A C

1991-09-15

31

Platelet-associated CD154 in immune thrombocytopenic purpura.  

PubMed

CD40-ligand (CD154) is expressed on activated CD4+ T lymphocytes and is essential for the T cell-dependent activation of B lymphocytes. CD154 is also expressed at the activated platelet surface. In this study, we show that platelet-associated CD154 is increased in immune thrombocytopenic purpura (ITP), a disease characterized by an autoimmune response against proteins of the platelet membrane. CD154 and its messenger RNA were also present in increased amounts in the megakaryocytes of patients with ITP. We found that platelet-associated CD154 is competent to induce the CD40-dependent proliferation of B lymphocytes, and we observed an in vitro CD154-dependent production of antibodies to the GPIIb/IIIa complex (integrin alphaIIbbeta3) when platelets and peripheral blood B lymphocytes from ITP patients with circulating anti-GPIIb/IIIa antibody were cultured together. Therefore, platelet-associated CD154 expression is increased in ITP and is able to drive the activation of autoreactive B lymphocytes in this disease. PMID:15191945

Solanilla, Anne; Pasquet, Jean-Max; Viallard, Jean-François; Contin, Cécile; Grosset, Christophe; Déchanet-Merville, Julie; Dupouy, Maryse; Landry, Marc; Belloc, Francis; Nurden, Paquita; Blanco, Patrick; Moreau, Jean-François; Pellegrin, Jean-Luc; Nurden, Alan T; Ripoche, Jean

2005-01-01

32

Thrombotic thrombocytopenic purpura: survival by "giving a dam".  

PubMed Central

A teenager died suddenly in 1923 of systemic microvascular thrombosis. Dr. Eli Moschcowitz attributed the "hitherto undescribed disease" (now "thrombotic thrombocytopenic purpura," or "TTP") to "some powerful poison" with "both agglutinative and hemolytic properties." In 1982, TTP was found to be a defect in the "processing" of unusually large (UL) von Willebrand factor (VWF) multimers. By 1998, the cause of TTP was known to be either familial absence or acquired inhibition (by autoantibody) of plasma VWF-cleaving metalloprotease. This enzyme, the 13th member of a disintegrin and metalloprotease family with thrombospondin domains (ADAMTS-13), circulates in normal plasma waiting to cleave the long strings of ULVWF multimers emerging from stimulated endothelial cells. Uncleaved ULVWF multimers in TTP induce platelet adhesion and aggregation in the rapidly flowing blood of microvessels. Episodes of TTP are treated by "giving A DAM" (TS-13, that is) contained in normal plasma, either by infusion alone or in combination with plasmapheresis. Images Fig. 1 Fig. 2 Fig. 5

Moake, Joel L.

2004-01-01

33

Platelet antibody in idiopathic thrombocytopenic purpura and other thrombocytopenias  

SciTech Connect

Platelet-associated immunoglobulin was measured by the use of fluorescent anti-1gG antibody. The method is simple, rapid, and sensitive and provides a precise quantitive assay of bound (direct) and free (indirect) 1gG with platelet specificity. We have evaluated this test in 30 normal volunteers and in 50 patients with immune and nonimmune, treated and untreated thrombocytopenias. All patients with immune thrombocytopenias (acute and chronic idiopathic thrombocytopenic purpura and systemic lupus erythematosus) having platelet counts < 100,000/..mu..l had elevated levels of platelet-bound 1gG and 86% had also positive results in the indirect assay. All patients with nonimmunological thrombocytopenias showed normal results in the direct and indirect assay of platelet-associated immunoglobulin. In patients studied repeatedly during the course of their illness, an inverse relation was found between platelet count and level of platelet-bound 1gG. Patients with systemic lupus erythematosus presented clear exceptions to this rule. Investigations of the absorbability of platelet autoantibodies and alloantibodies showed that this assay can readily differentiate between these two antibody species and can also identify specificities of alloantibodies.

Sugiura, K.; Steiner, M.; Baldini, M.G.

1980-10-01

34

Relapses of thrombotic thrombocytopenic purpura after treatment with rituximab.  

PubMed

Rituximab has been added to therapeutic plasma exchange (TPE) in the last 10 years for refractory thrombotic thrombocytopenic purpura (TTP). We performed a retrospective single institution study to determine if patients with TTP treated with TPE and rituximab experienced relapses. We reviewed the electronic and apheresis records of patients treated between 2003 and 2008 and collected the following parameters: demographics, laboratory results, treatment characteristics, and follow-up. We identified 12 patients with ADAMTS13 <5% due to an inhibitor who received TPE and rituximab during the study period. The mean number of TPEs required to achieve remission was 24 ± 3, time to remission was 28 ± 3 days, and hospital length of stay was 36 ± 4 days. During a mean follow-up of 73.4 ± 6 months, four patients (33%) relapsed. On average, relapse occurred at 62 ± 8.5 months postachievement of remission. The one-year, three-year, and five-year relapse free-survival (RFS) rates were 92%, 75%, and 75%, respectively. On multivariate analysis, we failed to identify independent predictors of relapse. This retrospective analysis does not support the notion that rituximab prevents or decreases the rate of relapse in TTP. Prospective randomized studies are needed to confirm this observation. PMID:23857472

Goyal, Jatinder; Adamski, Jill; Lima, Jose L O; Marques, Marisa B

2013-12-01

35

A case associated with comorbidities among cerebral infarction, idiopathic thrombocytopenic purpura, and triple x syndrome.  

PubMed

A 46-year-old female presented to the emergency room due to the chief complaint of left-sided weakness. By imaging study, she was diagnosed with cerebral infarction. Thrombolytic and antiplatelet agents were not considered due to the "golden hour" for treatment having passed and a low platelet count. The peripheral blood smear, bone marrow biopsy, and aspirate findings were consistent with immune thrombocytopenic purpura. The chromosome analysis revealed the 47,XXX karyotype. To the best of our knowledge, this is the first case report associated with the comorbidities of cerebral infarction, idiopathic thrombocytopenic purpura, and triple X syndrome. PMID:25035678

Kim, Hanjun; Hwang, Sang Sun; Uh, Young; Kim, Juwon; Yoon, Kap Jun; Lee, Ji-Yong

2014-06-01

36

A Case Associated with Comorbidities Among Cerebral Infarction, Idiopathic Thrombocytopenic Purpura, and Triple X Syndrome  

PubMed Central

A 46-year-old female presented to the emergency room due to the chief complaint of left-sided weakness. By imaging study, she was diagnosed with cerebral infarction. Thrombolytic and antiplatelet agents were not considered due to the “golden hour” for treatment having passed and a low platelet count. The peripheral blood smear, bone marrow biopsy, and aspirate findings were consistent with immune thrombocytopenic purpura. The chromosome analysis revealed the 47,XXX karyotype. To the best of our knowledge, this is the first case report associated with the comorbidities of cerebral infarction, idiopathic thrombocytopenic purpura, and triple X syndrome.

Kim, Hanjun; Hwang, Sang Sun; Uh, Young; Kim, Juwon; Yoon, Kap Jun; Lee, Ji-Yong

2014-01-01

37

Improvement of thrombocytopenia after treatment for Helicobacter pylori in a patient with immunologic thrombocytopenic purpura  

PubMed Central

Immune thrombocytopenic purpura is the most common autoimmune hematologic disease, affecting individuals of different ages. Recently, the bacterium Helicobacter pylori entered the list of causes of immune thrombocytopenic purpura. Here we present the case of a 55-year-old female patient with low platelet counts initially attributed to chronic vaginal bleeding. As corticosteroid therapy was ineffective she was treated for H. pylori infection. Within four weeks the patient had a platelet count of 87.17 ×109/L accompanied by clinical improvement of the symptoms.

Rocha, Aline Maia; Botelho, Luis Fabio Barbosa; Rocha, David Maia

2014-01-01

38

Improvement of thrombocytopenia after treatment for Helicobacter pylori in a patient with immunologic thrombocytopenic purpura.  

PubMed

Immune thrombocytopenic purpura is the most common autoimmune hematologic disease, affecting individuals of different ages. Recently, the bacterium Helicobacter pylori entered the list of causes of immune thrombocytopenic purpura. Here we present the case of a 55-year-old female patient with low platelet counts initially attributed to chronic vaginal bleeding. As corticosteroid therapy was ineffective she was treated for H. pylori infection. Within four weeks the patient had a platelet count of 87.17 ×10(9)/L accompanied by clinical improvement of the symptoms. PMID:24790544

Rocha, Aline Maia; Botelho, Luís Fábio Barbosa; Rocha, David Maia

2014-03-01

39

Novel Use of a Thrombopoietin Mimetic for Chronic Immune Thrombocytopenic Purpura in Pregnancy  

PubMed Central

BACKGROUND Thrombopoietin mimetic agents are a novel therapeutic option for patients with chronic immune thrombocytopenic purpura (ITP). We report on the use of romiplostim, a thrombopoietin mimetic, throughout pregnancy. CASE A 28 year old primigravida with chronic ITP initiated a planned pregnancy on romiplostim. The second and third trimesters were marked by a cyclic pattern of thrombocytopenia requiring supplemental corticosteroids or intravenous immunoglobulin and resultant thrombocytosis. Increased romiplostim doses and daily corticosteroids stabilized the platelet count prior to induction of labor. The delivery was uneventful, though the newborn manifested anemia and intraventricular hemorrhage. CONCLUSION The decreased efficacy of romiplostim monotherapy is attributed to increased target-mediated drug disposition and the physiologic changes of pregnancy. Safety concerns still exist for the developmental effects of romiplostim on the fetus.

Patil, Avinash S.; Dotters-Katz, Sarah K.; Metjian, Ara D.; James, Andra H.; Swamy, Geeta

2014-01-01

40

Helicobacter pylori eradication in patients with chronic immune thrombocytopenic purpura  

PubMed Central

AIM: To assess the effect of Helicobacter pylori (H. pylori) eradication on platelet counts in patients with chronic immune thrombocytopenic purpura (cITP). METHODS: A total of 36 cITP patients were included in the study. The diagnosis of H. pylori was done by rapid urease test and Giemsa staining of the gastric biopsy specimen. All H. pylori positive patients received standard triple therapy for 14 d and were subjected for repeat endoscopy at 6 wk. Patients who continued to be positive for H. pylori on second endoscopy received second line salvage therapy. All the patients were assessed for platelet response at 6 wk, 3rd and 6th months. RESULTS: Of the 36 patients, 17 were positive for H. pylori infection and eradication was achieved in 16 patients. The mean baseline platelet count in the eradicated patients was 88615.38 ± 30117.93/mm3 and platelet count after eradication at 6 wk, 3 mo and 6 mo was 143230.77 ± 52437.51/mm3 (P = 0.003), 152562.50 ± 52892.3/mm3 (P = 0.0001), 150187.50 ± 41796.68/mm3 (P = 0.0001) respectively and in the negative patients, the mean baseline count was 71000.00 ± 33216.46/mm3 and at 6 wk, 3rd and 6th month follow up was 137631.58 ± 74364.13/mm3 (P = 0.001), 125578.95 ± 71472.1/mm3 (P = 0.005), 77210.53 ± 56892.28/mm3 (P = 0.684) respectively. CONCLUSION: Eradication of H. pylori leads to increase in platelet counts in patients with cITP and can be recommended as a complementary treatment with conventional therapy.

Noonavath, Ravinder Naik; Lakshmi, Chandrasekharan Padma; Dutta, Tarun Kumar; Kate, Vikram

2014-01-01

41

Idiopathic thrombocytopenic purpura as the presenting feature of a primary bilateral adrenal non Hodgkin's lymphoma.  

PubMed

Primary lymphomas arising in the adrenal gland are extremely rare. The presenting symptoms are nonspecific and may be related to lymphoma or to associated adrenal insufficiency. In this report we describe the case of a 61 year old woman with idiopathic thrombocytopenic purpura and primary bilateral non Hodgkin's lymphoma of the adrenals. PMID:9389368

Baudard, M; Pagnoux, C; Audouin, J; Buy, J N; Bethoux, J P; Delmer, A; Zittoun, R

1997-08-01

42

Human Granulocytic Ehrlichiosis Presenting with Acute Renal Failure and Mimicking Thrombotic Thrombocytopenic Purpura  

Microsoft Academic Search

We present the case of an elderly female patient presenting with recurrent acute renal failure, fever, altered mental status, abdominal pain, thrombocytopenia and a small number of fragmented red cells on peripheral smear mimicking recurrent thrombotic thrombocytopenic purpura (TTP). Eventually, however, she was diagnosed to have human granulocytic ehrlichiosis (HGE), and after treatment for HGE her clinical and laboratory abnormalities

Kulwant S. Modi; David C. Dahl; Robert O. Berkseth; Ronald Schut; Edward Greeno

1999-01-01

43

Impact of thrombotic thrombocytopenic purpura on leukemic children undergoing bone marrow transplantation  

Microsoft Academic Search

Thrombotic thrombocytopenic purpura (TTP) has emerged as one of the main transplant-related complications over the last 15 years. The current study defines the incidence and the risk factors for the occurrence of TTP in 131 consecutive leukemic children who were transplanted between January 1994 and December 1997 at four Italian pediatric centers. Patients with ALL (101), AML (21), MDS (9),

C Uderzo; M Fumagalli; P De Lorenzo; A Busca; E Vassallo; S Bonanomi; E Lanino; G Dini; S Varotto; C Messina; R Miniero; MG Valsecchi; A Balduzzi

2000-01-01

44

Splenectomy for Chronic Idiopathic Thrombocytopenic Purpura in Children: A Single Center Study in China  

Microsoft Academic Search

The management of chronic and refractory idiopathic thrombocytopenic purpura (ITP) in children is controversial. We conducted a retrospective review of our single center experience in China between 1990 and 2003 with splenectomy for chronic ITP in children in order to determine the initial and long-term hematological response, morbidity, mortality, predictors of response to splenectomy and the therapy in children who

Tingting Wang; Maoqiang Xu; Linxiang Ji; Renchi Yang

2006-01-01

45

Periarteritis nodosa and thrombotic thrombocytopenic purpura with serous retinal detachment in siblings.  

PubMed Central

Periarteritis nodosa and thrombotic thrombocytopenic purpura in siblings is reported. In both patients a localised serous retinal detachment and lesions of the retinal pigment epithelium had developed owing to choroidal vascular obstruction. These cases support the suggested possible relationship between the two conditions. Images

Stefani, F. H.; Brandt, F.; Pielsticker, K.

1978-01-01

46

Genetic analysis of autoantibodies in idiopathic thrombocytopenic purpura reveals evidence of clonal expansion and somatic mutation  

Microsoft Academic Search

Although idiopathic thrombocytopenic purpura (ITP) is the most common autoim- mune hematologic disorder, little is known about the associated autoantibodies on a molecular level. Consequently, diagnos- tic assays and therapy for ITP lack speci- ficity. To avoid technical limitations im- posed by B-cell immortalization methods, we used repertoire cloning (Fab\\/phage display) to clone platelet autoantibodies and examine the relation between

Jessica H. Roark; James B. Bussel; Douglas B. Cines; Don L. Siegel

2002-01-01

47

Cardiac and pulmonary thrombosis during multidrug treatment in an idiopathic thrombocytopenic purpura patient.  

PubMed

Glucocorticosteroids, intravenous immunoglobulins, vincristine, danazol, and eltrombopag are used in refractory chronic idiopathic thrombocytopenic purpura (ITP). All those treatment modalities are susceptible for thrombosis generation. There is an increased risk of thrombosis in the diseases' natural course. The case we present is a resistant chronic ITP patient who developed pulmonary and intracardiac thrombosis during multidrug treatment. Risk of concomitant usage of drugs and rapid increase in platelet count are discussed. PMID:23320868

Andic, Neslihan; Gunduz, Eren; Akay, Olga Meltem; ?ahin, Deniz; Teke, Hava Üsküdar

2014-01-01

48

Prediction of Response to Splenectomy in Patients with Idiopathic Thrombocytopenic Purpura  

Microsoft Academic Search

Background  Chronic immune thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by destruction of opsonized platelets\\u000a in the reticuloendothelial system, particularly the spleen, and by resulting low platelet counts. Splenectomy is the standard\\u000a second-line treatment for patients with ITP who do not respond to corticosteroids. In the present study we determined predictive\\u000a factors for the response of patients with ITP to

A. Shojaiefard; S. A. Mousavi; S. H. Faghihi; S. Abdollahzade

2008-01-01

49

Differences in T Cell Subsets Between Men and Women With Idiopathic Thrombocytopenic Purpura  

Microsoft Academic Search

Idiopathic thrombocytopenic purpura (ITP) is an autoim- mune disorder, occurring predominantly in women. We studied by flow cytofluorimetry the T cell subsets in men and women with lIP and compared them with healthy sex-matched volunteers. In healthy controls, women were found to have higher proportions of T helper\\/inducer (Th,,) and lower T suppressor\\/cytotoxic (T,,?) lymphocytes and consequently higher Th,,:T$,C ratios

Ravindra Mylvaganam; Yeon S. Ahn; William J. Harrington; Chae I. Kim; Howard G. Gratzner

1985-01-01

50

Thrombotic thrombocytopenic purpura related to severe ADAMTS13 deficiency in children  

Microsoft Academic Search

Thrombotic thrombocytopenic purpura (TTP) related to a severely deficient activity of the von Willebrand factor cleaving protease,\\u000a ADAMTS (A Disintegrin And Metalloprotease with ThromboSpondin type 1 repeats) 13, is a life-threatening event, the onset of\\u000a which may occur as early as childhood. TTP is either inherited (Upshaw-Schulman syndrome) via ADAMTS13 gene mutations (neonatal\\u000a onset) or acquired via anti-ADAMTS13 autoantibodies (childhood

Chantal Loirat; Jean-Pierre Girma; Céline Desconclois; Paul Coppo; Agnès Veyradier

2009-01-01

51

Refractory Thrombocytopenia, an Unusual Myelodysplastic Syndrome with an Initial Presentation Mimicking Idiopathic Thrombocytopenic Purpura  

Microsoft Academic Search

Refractory thrombocytopenia (RTC) is an unusual subtype of myelodysplastic syndrome (MDS) that initially presents as chronic\\u000a pure thrombocytopenia. Because of the lack of distinguishable dysplasia, RTC has often been misdiagnosed as idiopathic thrombocytopenic\\u000a purpura.We describe the case of a patient with RTC and trisomy 8 for whom a bone marrow mononuclear cell (BMNC) gene expression\\u000a profile was obtained by means

Jun Qian; Yongquan Xue; Jinlan Pan; Jiannong Cen; Wei Wang; Zixing Chen

2005-01-01

52

Perinatal management of idiopathic thrombocytopenic purpura in pregnancy: risk factors for passive immune thrombocytopenia  

Microsoft Academic Search

Summary Thirty-nine pregnant women with idiopathic thrombocytopenic purpura (ITP) were studied in order to evaluate the influence of therapies for maternal ITP on fetal passive immune thrombocytopenia (PIT). Neonatal platelet counts were also compared with platelet counts, amount of PAIgG, and presence of circulating antiplatelet antibody in maternal blood. Eight of 41 neonates (19.5%) presented PIT without neonatal mortality. A

H. Yamada; S. Fujimoto

1994-01-01

53

Thrombotic Thrombocytopenic Purpura After Prophylactic Cefuroxime Axetil Administered in Relation to a Liposuction Procedure  

Microsoft Academic Search

Thrombotic thrombocytopenic purpura (TTP) or Moschcowitz’s syndrome is characterized by platelet and von Willebrand factor\\u000a (vWF) deposition in arterioles and capillaries throughout the body, which results in organ ischemia. The diagnostic pentad\\u000a characterizing TTP consists of thrombocytopenia, microangiopathic hemolytic anemia (MAHA), fever, neurologic manifestations,\\u000a and renal insufficiency. In terms of type, TTP can be either idiopathic or secondary. The causes

Ahmet Emre Eskazan; Ayse Salihoglu; Emine Gulturk; Seniz Ongoren; Teoman Soysal

54

Presumptive thrombotic thrombocytopenic purpura following a hump-nosed viper (Hypnale hypnale) bite: a case report  

PubMed Central

Hump-nosed viper bites are frequent in southern India and Sri Lanka. However, the published literature on this snakebite is limited and its venom composition is not well characterized. In this case, we report a patient with thrombotic thrombocytopenic purpura-like syndrome following envenoming which, to the best of our knowledge, has not been reported in the literature before. A 55-year-old woman from southern Sri Lanka presented to the local hospital 12 hours after a hump-nosed viper (Hypnale hypnale) bite. Five days later, she developed a syndrome that was characteristic of thrombotic thrombocytopenic purpura with fever, thrombocytopenia, microangiopathic hemolysis, renal impairment and neurological dysfunction in the form of confusion and coma. Her clinical syndrome and relevant laboratory parameters improved after she was treated with therapeutic plasma exchange. We compared our observations on this patient with the current literature and concluded that thrombotic thrombocytopenic purpura is a theoretically plausible yet unreported manifestation of hump-nosed viper bite up to this moment. This study also provides an important message for clinicians to look out for this complication in hump-nosed viper bites since timely treatment can be lifesaving.

2014-01-01

55

Stroke in thrombotic thrombocytopenic purpura induced by thyrotoxicosis: a case report.  

PubMed

Thrombotic thrombocytopenic purpura (TTP) is a hematologic disease involving the platelet aggregation and resulting in hemolytic anemia, thrombocytopenia, and microvascular occlusion. Although frequent neurologic features are headache and confusion, focal deficit is described in 30% of the cases. There are a lot of causes inducing thrombotic thrombocytopenic, but reports are lacking when associated with Grave disease. We describe the case of a 51-year-old Caucasian woman presenting a 24-hour story of sudden onset of dysarthria and left superior limb palsy. Four months before, she developed severe hyperthyroidism associated with petechiae, hemolytic anemia, thrombocytopenia, and schistocytes at blood film examination. Relapse of TTP in association with Grave disease was diagnosed. There are few reports describing association between Grave disease and TTP with only mild neurologic involvement. We described, to our knowledge, the first case of acute ischemic stroke secondary to thrombotic thrombocytopenic induced by thyrotoxicosis. PMID:24674955

Bellante, Flavio; Redondo Saez, Patricia; Springael, Cecile; Dethy, Sophie

2014-07-01

56

Expression of a structurally constrained von Willebrand factor variant triggers acute thrombotic thrombocytopenic purpura in mice.  

PubMed

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease that presents with thrombocytopenia, disseminated thrombosis, hemolytic anemia, and organ dysfunction. The etiology of TTP has revealed that patients share a deficiency in plasma protease a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13), the enzyme responsible for cleaving ultra-large von Willebrand factor (VWF) multimers into nonthrombogenic fragments. Therefore, existing TTP mouse models were developed by targeted disruption of the ADAMTS13 gene. ADAMTS13(-/-) mice are mostly asymptomatic in the absence of a trigger, as redundant proteases appear to take on VWF processing. As an alternative approach to creating one such model, we devised a strategy based on the expression of a cleavage-resistant VWF mutant in mice. The creation of a disulfide bond within the A2 domain of VWF was found to render VWF multimers resistant to proteolysis by plasma proteases under flow. Furthermore, mice expressing the murine VWF/p.S1494C-p.A1534C mutant present with symptoms characteristics of acute TTP such as thrombocytopenia, red cell shredding, accumulation of VWF-rich thrombi in the microvasculature, and advanced TTP symptoms such as renal dysfunction and splenomegaly. Because this model appears to faithfully emulate the pathophysiology of TTP, it should prove most useful in the study of microangiopathic diseases and their treatment. PMID:24713928

Morioka, Yoko; Casari, Caterina; Wohner, Nikolett; Cho, Sungyun; Kurata, Sachiko; Kitano, Ayumi; Christophe, Olivier D; Lenting, Peter J; Li, Renhao; Denis, Cécile V; Prévost, Nicolas

2014-05-22

57

Coronary intervention for acute coronary syndrome in a 51-year-old man with immune thrombocytopenic purpura: a case report  

PubMed Central

Introduction Treatment of the rare cases of patients with chronic idiopathic thrombocytopenic purpura with acute coronary syndrome can be a significant problem. The patient in our case report was treated successfully with percutaneous coronary intervention. Case presentation A 51-year-old man of Turkish origin who had idiopathic thrombocytopenic purpura was admitted to our hospital with severe chest pain. His electrocardiography was normal on admission but dynamic ischemic changes were observed during follow-up. He underwent immediate coronary angiography. In his angiography, left anterior descending artery stenosis was 90% together with the diagonal ostium. Percutaneous coronary intervention was performed successfully. Bleeding complications were not observed after the procedure. Conclusions We report the presence of a rare case of chronic idiopathic thrombocytopenic purpura in a patient with acute coronary syndrome. In this situation a serious multidisciplinary approach is required before coronary intervention.

2014-01-01

58

Persistence of circulating ADAMTS13-specific immune complexes in patients with acquired thrombotic thrombocytopenic purpura.  

PubMed

Anti-ADAMTS13 autoantibodies are the main cause of acquired thrombotic thrombocytopenic purpura. Binding of these antibodies to ADAMTS13 eventually results in the formation of antigen-antibody immune complexes. Circulating ADAMTS13-specific immune complexes have been described in patients with acquired thrombotic thrombocytopenic purpura, although the prevalence and persistence of these immune complexes over time have hitherto remained elusive. Here, we analyzed a large cohort of patients with acquired thrombotic thrombocytopenic purpura for the presence of free and complexed anti-ADAMTS13 antibodies. In the acute phase (n=68), 100% of patients had free IgG antibodies and 97% had ADAMTS13-specific immune complexes. In remission (n=28), 75% of patients had free antibodies (mainly IgG) and 93% had ADAMTS13-specific immune complexes. Free antibodies were mainly of subclasses IgG1 and IgG4, whereas IgG4 was by far the most prevalent in ADAMTS13-specific immune complexes. Comparison of ADAMTS13 inhibitor and anti-ADAMTS13 IgG (total and subclasses) antibody titers in acute phase and in remission samples showed a statistically significant decrease in all parameters in remission. Although non-significant, a trend towards reduced or undetectable titers in remission was also observed for ADAMTS13-specific immune complexes of subclasses IgG1, IgG2 and IgG3. No such trend was discernible for IgG4; IgG4 immune complexes persisted over years, even in patients who had been treated with rituximab and who showed no features suggesting relapse. PMID:24241492

Ferrari, Silvia; Palavra, Kristina; Gruber, Bernadette; Kremer Hovinga, Johanna A; Knöbl, Paul; Caron, Claudine; Cromwell, Caroline; Aledort, Louis; Plaimauer, Barbara; Turecek, Peter L; Rottensteiner, Hanspeter; Scheiflinger, Friedrich

2014-04-01

59

Immune thrombocytopenic purpura in ulcerative colitis: a case report and systematic review.  

PubMed

Over 100 extraintestinal manifestations are reported in ulcerative colitis (UC). A commonly reported hematological manifestation is autoimmune hemolytic anemia. On rare occasions, immune thrombocytopenic purpura (ITP) has been reported with UC. The presence of thrombocytopenia can complicate the clinical scenario as the number of bloody bowel movements is an important indicator of disease activity in UC. A proposed theory for this association is antigenic mimicry between a platelet surface antigen and bacterial glycoprotein. We are reporting a case of UC and associated ITP managed successfully with anti-TNF therapy. We also performed a systemic review of case reports and a case series reporting this association. PMID:24765254

Chandra, Subhash; Finn, Sarah; Obah, Eugene

2014-01-01

60

Immune thrombocytopenic purpura in ulcerative colitis: a case report and systematic review  

PubMed Central

Over 100 extraintestinal manifestations are reported in ulcerative colitis (UC). A commonly reported hematological manifestation is autoimmune hemolytic anemia. On rare occasions, immune thrombocytopenic purpura (ITP) has been reported with UC. The presence of thrombocytopenia can complicate the clinical scenario as the number of bloody bowel movements is an important indicator of disease activity in UC. A proposed theory for this association is antigenic mimicry between a platelet surface antigen and bacterial glycoprotein. We are reporting a case of UC and associated ITP managed successfully with anti-TNF therapy. We also performed a systemic review of case reports and a case series reporting this association.

Chandra, Subhash; Finn, Sarah; Obah, Eugene

2014-01-01

61

Thrombotic thrombocytopenic purpura-like syndromes following bone marrow transplantation: an analysis of associated conditions and clinical outcomes  

Microsoft Academic Search

The diagnosis and treatment of thrombotic thrombocytopenic purpura (TTP) in patients following BMT are often uncertain and unsuccessful. To better understand the evaluation and management of these patients, we describe 17 patients treated with plasma exchange for a presumptive diagnosis of TTP following BMT during a 10 year period, 1989–1998. Because of the uncertainty of the diagnosis, these patients are

V Roy; MA Rizvi; SK Vesely; JN George

2001-01-01

62

Acute renal failure and severe rhabdomyolysis in a patient with resistant thrombotic thrombocytopenic purpura  

PubMed Central

Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disorder. This paper describes the case of a 39-year-old Sudanese male who presented to the emergency room with fever, jaundice, decreased level of consciousness, and worsening kidney function for 7 days, a high lactate dehydrogenase level (1947), severe thrombocytopenia (platelets 8), and numerous schistocytes in the peripheral blood smear. The patient was admitted with a diagnosis of TTP for plasma exchange. Fourteen days later, his creatinine kinase (CK) level rose to >50,000 IU; rhabdomyolysis was suggested. Continuous venovenous hemodialysis (CVVHD) was started. The patient’s CK level remained high, despite CVVHD, until the 6th day, after which this parameter gradually started to decrease. This report highlights a resistant case of TTP that presented with concomitant severe rhabdomyolysis, which demanded aggressive, continuous intervention.

Qahtani, Saad Al

2011-01-01

63

Chemically dimerized intravenous immunoglobulin has potent ameliorating activity in a mouse immune thrombocytopenic purpura model.  

PubMed

High-dose intravenous immunoglobulin (IVIG) preparations are currently used for the treatment of autoimmune diseases such as immune thrombocytopenic purpura (ITP). Although the mechanisms of IVIG efficacy remain enigmatic, some clinical and laboratory studies suggest that interaction of the Fc domain of IgG, especially the Fc domain of dimeric IgG, with its receptors (Fc gamma receptors; Fc?Rs) plays an essential role. In this study, IVIG was dimerized with chemical crosslinkers to augment its therapeutic efficacy. Dimerized IVIG was found to have a much higher affinity for Fc?Rs than monomeric IVIG. In a mouse ITP model, chemically dimerized IVIG abrogated the decrease in platelet numbers in the blood that was caused by an anti-platelet antibody at a dose that was one tenth of the required dose of IVIG. These results suggest that chemical dimerization of IVIG should greatly improve the efficacy of IVIG therapy of ITP. PMID:22306012

Machino, Yusuke; Suzuki, Emiko; Higurashi, Saki; Ohta, Hiroto; Suzuki, Mami; Kohroki, Junya; Masuho, Yasuhiko

2012-02-24

64

Immune thrombocytopenic purpura induced by intestinal tuberculosis in a liver transplant recipient.  

PubMed

A variety of clinical manifestations are associated directly or indirectly with tuberculosis. Among them, haematological abnormalities can be found in both the pulmonary and extrapulmonary forms of the disease. We report a case of immune thrombocytopenic purpura (ITP) associated with intestinal tuberculosis in a liver transplant recipient. The initial management of thrombocytopenia, with steroids and intravenous immunoglobulin, was not successful, and the lack of tuberculosis symptoms hampered a proper diagnostic evaluation. After the diagnosis of intestinal tuberculosis and the initiation of specific treatment, a progressive increase in the platelet count was observed. The mechanism of ITP associated with tuberculosis has not yet been well elucidated, but this condition should be considered in cases of ITP that are unresponsive to steroids and intravenous immunoglobulin, especially in immunocompromised patients and those from endemic areas. PMID:25009408

Lugao, Renata Dos Santos; Motta, Marina Pamponet; de Azevedo, Matheus Freitas Cardoso; de Lima, Roque Gabriel Rezende; Abrantes, Flávia de Azevedo; Abdala, Edson; Carrilho, Flair José; Mazo, Daniel Ferraz de Campos

2014-07-01

65

Increased percentages of T cells producing interleukin-21 in patients with immune thrombocytopenic purpura.  

PubMed

Interleukin (IL)-21, a cytokine which promotes differentiation of Th17, follicular helper T cell and B cells, may play an important role in controlling the autoimmune process in immune thrombocytopenic purpura (ITP), an acquired organ-specific autoimmune hemorrhagic disease. The frequencies of IL-21-producing T cells and serum levels of IL-21 were determined by flow cytometry analysis and ELISA, respectively, in 24 ITP patients and nine healthy controls. RT-PCR showed that the frequencies of circulating IL-21-producing T cells and serum levels of IL-21 were significantly higher in ITP patients than that in healthy controls. Serum IL-21 and IL-21 expression in patients with ITP significantly decreased after therapy, suggesting IL-21 might be important in ITP. PMID:24323496

Zhang, Qian; Bai, Hai; Wang, Wenhui

2014-04-01

66

Immune thrombocytopenic purpura induced by intestinal tuberculosis in a liver transplant recipient  

PubMed Central

A variety of clinical manifestations are associated directly or indirectly with tuberculosis. Among them, haematological abnormalities can be found in both the pulmonary and extrapulmonary forms of the disease. We report a case of immune thrombocytopenic purpura (ITP) associated with intestinal tuberculosis in a liver transplant recipient. The initial management of thrombocytopenia, with steroids and intravenous immunoglobulin, was not successful, and the lack of tuberculosis symptoms hampered a proper diagnostic evaluation. After the diagnosis of intestinal tuberculosis and the initiation of specific treatment, a progressive increase in the platelet count was observed. The mechanism of ITP associated with tuberculosis has not yet been well elucidated, but this condition should be considered in cases of ITP that are unresponsive to steroids and intravenous immunoglobulin, especially in immunocompromised patients and those from endemic areas.

Lugao, Renata dos Santos; Motta, Marina Pamponet; de Azevedo, Matheus Freitas Cardoso; de Lima, Roque Gabriel Rezende; Abrantes, Flavia de Azevedo; Abdala, Edson; Carrilho, Flair Jose; Mazo, Daniel Ferraz de Campos

2014-01-01

67

[Treatment of a pregnant patient after multiple trauma: rare combination with thrombotic thrombocytopenic purpura].  

PubMed

Multiple trauma during pregnancy is a relatively rare situation which poses a great challenge for the team in charge of treatment. A concomitant disease, such as thrombotic thrombocytopenic purpura (TTP) with thrombocytic coagulopathy increases the complexity of the treatment problems. This article describes the case of a 36-year-old pregnant woman referred to this hospital suffering from multiple trauma with severe liver rupture. Stabilization was achieved after an emergency Caesarean section and packing of the liver. Recurrent massive bleeding from the liver occurred after depacking and was treated successfully with recombinant factor VIIa. The concomitant TTP was treated by transfusion of fresh frozen plasma and corticosteroids. Rapid initiation of therapy was the goal to achieve hemostasis and prevent aggravation of the coagulation disorder and an unfavourable outcome despite severe thrombocytopenia. PMID:23400712

Haffner, E; Pietsch, U; Fösel, T; Lindemann, W

2013-02-01

68

Drug-induced thrombotic thrombocytopenic purpura successfully treated with recombinant human soluble thrombomodulin.  

PubMed

A 61-year-old woman with recurrent non-small cell lung cancer presented with thrombocytopenia, microangiopathic hemolytic anemia, neurological abnormalities, renal failure and a fever that appeared during chemotherapy with gemcitabine and bevacizumab. She was diagnosed with drug-induced thrombotic thrombocytopenic purpura (TTP). After the discontinuation of chemotherapy, the administration of recombinant human soluble thrombomodulin and fresh-frozen plasma rapidly ameliorated the TTP. Hypertension preceded the onset of TTP and required the administration of quadruple therapy on admission. However, after three months, the hypertension was controllable without anti-hypertensive drugs. Twelve months later, the ninth course of vinorelbine was administered safely, preventing the patient's lung cancer from progressing. PMID:23676600

Nishijima, Yu; Hirata, Haruhiko; Himeno, Aiko; Kida, Hiroshi; Matsumoto, Masanori; Takahashi, Ryo; Otani, Yasushi; Inoue, Kouji; Nagatomo, Izumi; Takeda, Yoshito; Kijima, Takashi; Tachibana, Isao; Fujimura, Yoshihiro; Kumanogoh, Atsushi

2013-01-01

69

Rituximab is effective for selected patients with chronic steroid-refractory immune thrombocytopenic purpura.  

PubMed

We report results of Rituximab therapy in four patients with chronic immune thrombocytopenic purpura (ITP) refractory to 3-8 prior therapeutic regimens. Rituximab was administered at a dose of 375 mg/m2 once weekly for 4-6 weeks. Three out of four patients achieved a complete remission (rise to platelet count above 100,000/microl). Response duration was 4, 16+, and 11+ months. Rituximab was well tolerated but one patient (a 77 year-old male) developed two serious infections, pneumonia and a hepatic abscess, at 2 and 4 months. We conclude that Rituximab is effective in patients with refractory ITP; nevertheless, careful patient selection is mandatory. PMID:15621736

Lalayanni, Chrisavgi; Stavroyianni, Niki; Saloum, Riad; Tsompanakou, Aliki; Anagnostopoulos, Achilles

2004-08-01

70

Low dose cepharanthine ameliorates immune thrombocytopenic purpura associated with multiple myeloma.  

PubMed

Immune thrombocytopenic purpura associated with multiple myeloma is extremely rare. Here, we present the successful management of an elderly Japanese patient with multiple myeloma complicated by immune thrombocytopenia with low dose cepharanthine - a plant derived alkaloid. A 78-year-old male patient with IgG? multiple myeloma was repetitively treated with melpharan and prednisolone. In each chemotherapy course, we demonstrated a close relationship between platelet recovery and administration of high dose prednisolone. When further chemotherapy was avoided because of the patient's poor general condition, administration of cepharanthine was effective in halting progressive thrombocytopenia due to abnormal immune mechanisms. We propose the usefulness of cepharanthine in management of this rare disease. PMID:22561120

Tabata, Rie; Tabata, Chiharu; Tazoh, Ayako; Nagai, Tomoko

2012-07-01

71

Light chain-restricted autoantibodies in chronic idiopathic thrombocytopenic purpura, but no evidence for circulating clonal B-lymphocytes  

Microsoft Academic Search

In chronic idiopathic thrombocytopenic purpura (ITP) platelet destruction is caused by antibodies directed against platelet membrane glycoproteins (GP), and the predominant autoantigens are known to be GPIb\\/IX and GPIIb\\/IIIa. In a recent study we reported that these antibodies frequently had a restricted light chain phenotype, thereby supporting a clonal origin. Similar findings and the presence of clonal B-cell populations in

D. Stockelberg; M. Hou; S. Jacobsson; J. Kutti; H. Wadenvik

1996-01-01

72

Clinicopathological Characteristics and Outcome of Chinese Patients with Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome: A 9Year Retrospective Study  

Microsoft Academic Search

Background: The pathogenesis of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) is unclear and the prognosis is poor. Few studies have been published focusing on Chinese patients with TTP-HUS. We performed a retrospective study on the clinical characteristics and outcome of Chinese patients with TTP-HUS. Method: Patients with TTP-HUS, admitted to our hospital from 1998 to 2006, were retrospectively analyzed. Results:

Wen Zhang; Hao Shi; Hong Ren; Ping-Yan Shen; Xiao-Xia Pan; Xiao Li; Yong-Xi Chen; Yao-Wen Xu; Xiao-Nong Chen; Ping Zhu; Nan Chen

2009-01-01

73

Ticlopidine- and clopidogrel-associated thrombotic thrombocytopenic purpura (TTP): review of clinical, laboratory, epidemiological, and pharmacovigilance findings (1989–2008)  

Microsoft Academic Search

Thrombotic thrombocytopenic purpura (TTP) is a fulminant disease characterized by platelet aggregates, thrombocytopenia, renal insufficiency, neurologic changes, and mechanical injury to erythrocytes. Most idiopathic cases of TTP are characterized by a deficiency of ADAMTS13 (a disintegrin and metalloprotease, with thrombospondin-1-like domains) metalloprotease activity. Ironically, use of anti-platelet agents, the thienopyridine derivates clopidogrel and ticlopidine, is associated with drug induced TTP.

Anaadriana Zakarija; Hau C Kwaan; Joel L Moake; Nicholas Bandarenko; Dilip K Pandey; June M McKoy; Paul R Yarnold; Dennis W Raisch; Jeffrey L Winters; Thomas J Raife; John F Cursio; Thanh Ha Luu; Elizabeth A Richey; Matthew J Fisher; Thomas L Ortel; Martin S Tallman; X Long Zheng; Masanori Matsumoto; Yoshihiro Fujimura; Charles L Bennett

2009-01-01

74

Rituximab in the management of chronic immune thrombocytopenic purpura: an effective and safe therapeutic alternative in refractory patients  

Microsoft Academic Search

Rituximab induces B-cell depletion; therefore, it has been used in the treatment of immune thrombocytopenic purpura (ITP). The aim of this retrospective study was to evaluate the effectiveness of rituximab in the treatment of 89 patients with chronic ITP refractory to several treatments. All the patients had platelet counts 9\\/l. They had received a median of five (2–13) previous treatments,

Francisco Javier Peñalver; Victor Jiménez-Yuste; Manuel Almagro; Alberto Alvarez-Larrán; Lluis Rodríguez; Marisol Casado; Laura Gallur; Pilar Giraldo; Roberto Hernández; Dolores Menor; Maria José Rodríguez; Dolores Caballero; Raúl González; José Mayans; Isabel Millán; José Rafael Cabrera

2006-01-01

75

ST-segment elevation myocardial infarction treated with thrombolytic therapy in a patient with thrombotic thrombocytopenic purpura.  

PubMed

Acute myocardial infarction is a common complication of thrombotic thrombocytopenic purpura (TTP), but rarely the presenting manifestation. Anti-thrombotic therapy for myocardial infarction is rarely utilized in the setting of TTP because of elevated bleeding risk. We report a case of TTP presenting with ST-segment elevation myocardial infarction and treated with thrombolytic therapy. The resultant cardiac and neurological complications highlight the challenges of using evidence-based therapy for myocardial infarction in the setting of TTP. PMID:24189934

Doll, Jacob A; Kelly, Jacob P

2014-07-01

76

Evidence for a role of anti-ADAMTS13 autoantibodies despite normal ADAMTS13 activity in recurrent thrombotic thrombocytopenic purpura  

PubMed Central

Background Severe ADAMTS13 deficiency is a critical component of the pathogenesis of idiopathic thrombotic thrombocytopenic purpura but is found only in about 60% of patients clinically diagnosed with this disease. Design and Methods Over a period of 8 years and six episodes of thrombotic thrombocytopenic purpura we studied the evolution of the anti-ADAMTS13 antibody response in a patient using different ADAMTS13 assays and epitope mapping. Results Anti-ADAMTS13 autoantibodies were found in all episodes but were inhibitory only in the last two episodes. In a flow-based assay, normal ADAMTS13 activity was found only during the first disease episode, while ADAMTS13 activity was normal using a static assay in episodes 1 and 3, and severely deficient in the last two episodes. Fluorescence evolution in a modified fluorescence resonance energy transfer assay using a von Willebrand factor A2 domain peptide substrate was linear in episodes 1, 5 and 6, but increased exponentially in episodes 3 and 4. Despite the variable functional characteristics of the anti-ADAMTS13 autoantibodies, their principal epitope was the ADAMTS13 spacer domain in all episodes. Conclusions The patient is unique as he displayed features of maturation or shaping of the anti-ADAMTS13 autoantibody response during the course of multiple episodes of thrombotic thrombocytopenic purpura. Anti-ADAMTS13 autoantibodies may be important in vivo despite normal ADAMTS13 activity in routine assays. Consequently, treatment decisions should not be based solely on activity assay results.

Froehlich-Zahnd, Rahel; George, James N.; Vesely, Sara K.; Terrell, Deirdra R.; Aboulfatova, Khatira; Dong, Jing-Fei; Luken, Brenda M.; Voorberg, Jan; Budde, Ulrich; Sulzer, Irmela; Lammle, Bernhard; Kremer Hovinga, Johanna A.

2012-01-01

77

Platelet destruction in autoimmune thrombocytopenic purpura: kinetics and clearance of indium-111-labeled autologous platelets  

SciTech Connect

Using autologous /sup 111/In-labeled platelets, platelet kinetics and the sites of platelet destruction were assessed in 16 normal subjects (13 with and three without spleens), in 17 studies of patients with primary autoimmune thrombocytopenic purpura (AITP), in six studies of patients with secondary AITP, in ten studies of patients with AITP following splenectomy, and in five thrombocytopenic patients with myelodysplastic syndromes. In normal subjects, the spleen accounted for 24 +/- 4% of platelet destruction and the liver for 15 +/- 2%. Untreated patients with primary AITP had increased splenic destruction (40 +/- 14%, p less than 0.001) but not hepatic destruction (13 +/- 5%). Compared with untreated patients, prednisone treated patients did not have significantly different spleen and liver platelet sequestration. Patients with secondary AITP had similar platelet counts, platelet survivals, and increases in splenic destruction of platelets as did patients with primary AITP. In contrast, patients with myelodysplastic syndromes had a normal pattern of platelet destruction. In AITP patients following splenectomy, the five nonresponders all had a marked increase (greater than 45%) in liver destruction compared to five responders (all less than 40%). Among all patients with primary or secondary AITP, there was an inverse relationship between the percent of platelets destroyed in the liver plus spleen and both the platelet count (r = 0.75, p less than 0.001) and the platelet survival (r = 0.86, p less than 0.001). In a stepwise multiple linear regression analysis, total liver plus spleen platelet destruction, the platelet survival and the platelet turnover were all significant independent predictors of the platelet count. Thus platelet destruction is shifted to the spleen in primary and secondary AITP. Failure of splenectomy is associated with a marked elevation in liver destruction.

Stratton, J.R.; Ballem, P.J.; Gernsheimer, T.; Cerqueira, M.; Slichter, S.J.

1989-05-01

78

Efficiency of curative and prophylactic treatment with rituximab in ADAMTS13-deficient thrombotic thrombocytopenic purpura: a study of 11 cases.  

PubMed

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease that occurs mainly in young adults. Acquired cases are usually a result of antibodies directed against ADAMTS13 (a disintegrin-like and metalloprotease [reprolysin type] with thrombospondin type 1 motif 13), a protease that cleaves the von Willebrand factor multimers. Prognosis has been improved by plasma therapy, but some acute severe forms are refractory to this treatment and achieving a sustained remission is still a challenge in chronic relapsing forms. We therefore conducted a multicentric open-label prospective trial to test the efficacy of rituximab, an anti-B-cell monoclonal antibody, as a curative and prophylactic treatment in patients with TTP as a result of anti-ADAMTS13 antibodies. Six patients were included during an acute refractory TTP episode. Five patients with severe relapsing TTP and persistent anti-ADAMTS13 antibodies were prophylactically treated during remission. All patients received 4 weekly infusions of rituximab. The target of treatment was to restore a significant ADAMTS13 plasma activity (> 10%). Treatment with rituximab led to clinical remission in all cases of acute refractory TTP. In all patients, anti-ADAMTS13 antibodies disappeared, and a significant (18%-75%) plasma ADAMTS13 activity was detected following treatment. Tolerance of rituximab was good. Rituximab is a promising first-line immunosuppressive treatment in patients with acute refractory and severe relapsing TTP related to anti-ADAMTS13 antibodies. PMID:15933059

Fakhouri, Fadi; Vernant, Jean-Paul; Veyradier, Agnès; Wolf, Martine; Kaplanski, Gilles; Binaut, Raynald; Rieger, Manfred; Scheiflinger, Friedrich; Poullin, Pascale; Deroure, Benjamin; Delarue, Richard; Lesavre, Philippe; Vanhille, Philippe; Hermine, Olivier; Remuzzi, Giuseppe; Grünfeld, Jean-Pierre

2005-09-15

79

Evaluation of humoral immune function in patients with chronic idiopathic thrombocytopenic purpura.  

PubMed

Coincidence of autoimmune diseases such as immune thrombocytopenic purpura (ITP) with immunodeficiencies has been reported previously in patients who suffered from primary antibody deficiency (PAD). But there is no original study on immunological profiles of ITP patients to find out their probable immune deficiency. In this case-control study, ITP patients' humoral immunity was investigated for diagnosis of PAD in comparison with normal population. To evaluate the humoral immune system against polysaccharide antigens, patients' serum immunoglobulin levels were measured and a 23-valent pneumococcal capsular polysaccharide vaccine (PPV23) was administrated to evaluate the antibody response to vaccination. In this study, 14 out of 36 patients (39%) were diagnosed with antibody mediated immune deficiency including 2 patients (5.5%) with immunoglobulin class deficiency and 4 (11%) with IgG subclass deficiency. The remaining patients suffered from specific antibody deficiency. The most frequent deficiency in ITP patients was specific antibody deficiency.Therefore, immunological survey on ITP patients may be important especially for those who have undergone splenectomy. PMID:23454778

Rahiminejad, Mohammad Saeid; Mirmohammad Sadeghi, Mehrdad; Mohammadinejad, Payam; Sadeghi, Bamdad; Abolhassani, Hassan; Dehghani Firoozabadi, Mohammad Mehdi; Fathi, Seyed Mohammad; Rezvani, Hamid; Bahoush, Gholamreza; Ehsani, Mohammad Ali; Faranoush, Mohammad; Mehrvar, Azim; Torabi Sagvand, Babak; Ghadiani, Mojtaba; Rezaei, Nima; Aghamohammadi, Asghar

2013-03-01

80

[ADAMTS13 and thrombotic thrombocytopenic purpura retrospective study at the hospital of Le Mans].  

PubMed

Thrombotic thrombocytopenic purpura (TTP) is a rare disease with poor prognosis in the absence of treatment. The aim of the study was to compare with the literature, the clinical, biological and therapeutic management of PTT at the hospital of Le Mans (CHM) and to identify sub groups. The criteria for inclusion were all patients hospitalized between 2006 and 2012 at CHM with a number of platelets, level of hemoglobin and ADAMTS13 activity respectively less than 150 G/L, 110 g/L and 10 %. Eleven patients were included, confirming the epidemiological level rarity of this disease and the prevalence in young women, with immune dysfunction, and Afro-Caribbean origin. Consistent with literature, patients had heterogeneous symptoms but mainly neurological symptoms. Biological results were heteregenous and also different from the diagnostic described by Amorosi. Concerning therapy, all patients underwent plasma exchange with fresh frozen plasma substitution, confirming this as a reference treatment of first intention. Sub-group analysis have highlighted three predictive factors of mortality which were: an age over 70 years of age, a renal failure and a haptoglobin level superior to 0.10 g/dL. This study reminded the clinical, biological and therapeutic recommendations about TTP and described predictive factors of mortality. PMID:24876143

Bovet, Julien; Pineau-Vincent, Fabienne

2014-01-01

81

A case of myelitis with anti-aquaporin 4 antibody concomitant with immune thrombocytopenic purpura.  

PubMed

We report a 44-year-old woman who had anti-aquaporin 4 (AQP4) antibody-positive myelitis and immune thrombocytopenic purpura (ITP). She was admitted to our hospital with paraparesis, dysesthesia below the Th8 dermatome level on her right side and lower extremities, constipation and urinary retention. Magnetic resonance imaging revealed a longitudinally extending lesion at the level of Th4-Th10. Her serum sample was positive for anti-AQP4 antibody. Corticosteroid therapy was initiated, and her symptoms were largely ameliorated. Furthermore, concurrently with the myelitis, her platelet count dropped (99 × 10(9)/l). A diagnosis of ITP was made with positive serum platelet-associated IgG (PA-IgG) and negative work-up for blood malignancies by bone marrow aspiration. Since a causal relationship between Helicobacter pylori (H. pylori) and ITP is suggested by several studies, she was also examined and diagnosed with H. pylori-positive ITP. After the bacteria eradication therapy, her platelet count and PA-IgG returned to normal range. Furthermore, the anti-AQP4 antibody titer declined and her symptoms were almost resolved. We considered that H. pylori might influence progression of the myelitis as well as induction and development of ITP. PMID:24705832

Mizuno, Hideki; Sato, Shigeru; Ohnishi, Yasushi; Takahashi, Toshiyuki; Nakashima, Ichiro; Fujihara, Kazuo; Aoki, Masashi; Ohnuma, Ayumu

2014-01-01

82

[Successful rituximab treatment for acquired amegakaryocytic thrombocytopenic purpura complicated with Coombs-negative autoimmune hemolytic anemia].  

PubMed

Acquired amegakaryocytic thrombocytopenic purpura (AATP) is a rare disorder characterized by severe thrombocytopenia associated with total absence or a selective decrease in bone marrow megakaryocytes. A 67-year-old male presented with a 2-month bleeding tendency. He was referred to our hospital because of severe thrombocytopenia. Bone marrow biopsy showed complete absence of megakaryocytes without dysplasia in cells of the myeloid and erythroid lineages. AATP was diagnosed. In addition, mild normocytic normochromic anemia and reticulocytosis were also observed and haptoglobin was below the detectable level. Coombs-negative autoimmune hemolytic anemia (AIHA) was diagnosed based on the high titer of RBC-bound IgG and negative direct and indirect coombs test results. He was first treated with cyclosporine 200 mg per day and subsequently with prednisolone but only slight temporary improvement was achieved. Administration of eight doses of rituximab 375 mg/m(2) per week ameliorated both thrombocytopenia and anemia. AATP should be considered in the differential diagnosis of thrombocytopenia, and immunosuppressive therapy is a potential first-line treatment. This is the first case report of AATP accompanied by AIHA successfully treated with rituximab. PMID:23823096

Hashimoto, Akari; Fujimi, Akihito; Kanisawa, Yuji; Matsuno, Teppei; Okuda, Toshinori; Minami, Shinya; Doi, Tadashi; Ishikawa, Kazuma; Uemura, Naoki; Tomaru, Utano

2013-06-01

83

Long-term results of laparoscopic splenectomy in pediatric chronic immune thrombocytopenic purpura  

PubMed Central

Purpose Laparoscopic splenectomy (LS) for pediatric chronic immune thrombocytopenic purpura (ITP) patients has recently become widespread. However, its long-term result is rarely reported in children. Methods We retrospectively analyzed the patients who underwent LS for pediatric chronic ITP from June 1998 to April 2007. Results There were 18 patients (14 male and 4 female) with mean age 9.5 ± 3.8 years. 14 complete response, 3 partial response, and 1 no response were occurred. During the 82-month median follow-up period, 9 patients maintained in a remission state without any additional treatment, and 9 patients relapsed. In a comparative analysis of the relapse group and no relapse group, hospital stays were longer in the relapse group and the preoperative platelet counts and platelet counts at 1 month post were lower in relapse group. A relapse-free survival among 17 patients who achieved partial or complete responses following LS showed 76.5%, 61.8%, and 33.0% at 1-, 5-, and 10-year following LS, respectively. Conclusion Although LS in pediatric chronic ITP patients had excellent results immediately after surgery, careful follow up is needed due to late relapse even when a complete response has been noted for several years.

Kim, Dong Jin

2014-01-01

84

[Concomitant use of romiplostim and chemotherapy for advanced rectal cancer associated with idiopathic thrombocytopenic purpura].  

PubMed

A woman in her 60s was referred to our department with advanced rectal cancer and multiple unresectable metastases of the liver and peritoneum. She had been diagnosed with idiopathic thrombocytopenic purpura (ITP) in her 20s, with a platelet count maintained at approximately 1.0×10(4)/?L by prednisolone; on admission, her platelet count was 0.9×10(4)/?L. Romiplostim, a thrombopoietin receptor agonist, was administered prior to chemotherapy. Her platelet count increased to approximately 10.0×10(4)/?L during chemotherapy with oxaliplatin plus capecitabine, and she developed deep venous thrombosis requiring inferior vena cava filter placement and anticoagulation. No other severe adverse events occurred. There is no standard regimen for the treatment of solid tumors in patients with ITP. This is the first reported case of the concomitant use of romiplostim and chemotherapy for advanced rectal cancer. We believe that romiplostim can effectively salvage the platelet count in emergency situations such as during chemotherapy. PMID:24598096

Yamada, Moyuru; Shinzaki, Shinichiro; Nishida, Tsutomu; Yamada, Takuya; Fujinaga, Tetsuji; Yakushijin, Takayuki; Tatsumi, Tomohide; Iijima, Hideki; Hiramatsu, Naoki; Tsujii, Masahiko; Takehara, Tetsuo

2014-03-01

85

Vaccine administration and the development of immune thrombocytopenic purpura in children.  

PubMed

The most important reasons cited by the opponents of vaccines are concerns about vaccine safety. Unlike issues such as autism for which no indisputable documentation of direct relationship with vaccine use is available, immune thrombocytopenic purpura (ITP) is an adverse event that can really follow vaccine administration, and may limit vaccine use because little is known about which vaccines it may follow, its real incidence and severity, the risk of chronic disease, or the possibility of recurrences after new doses of the same vaccine. The main aim of this review is to clarify the real importance of thrombocytopenia as an adverse event and discuss how it may interfere with recommended vaccination schedules. The available data clearly indicate that ITP is very rare and the only vaccine for which there is a demonstrated cause-effect relationship is the measles, mumps and rubella (MMR) vaccine that can occur in 1 to 3 children every 100,000 vaccine doses. However, also in this case, the incidence of ITP is significantly lower than that observed during the natural diseases that the vaccine prevents. Consequently, ITP cannot be considered a problem limiting vaccine use except in the case of children suffering from chronic ITP who have to receive MMR vaccine. In these subjects, the risk-benefit ratio of the vaccine should be weighed against the risk of measles in the community. PMID:23324619

Cecinati, Valerio; Principi, Nicola; Brescia, Letizia; Giordano, Paola; Esposito, Susanna

2013-05-01

86

Retrospective review of 25 cases of thrombotic thrombocytopenic purpura in Pakistan.  

PubMed

Thrombotic thrombocytopenic purpura (TTP) is a disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia, fever, neurological abnormalities, and renal dysfunction. Because of the rarity of TTP, no comprehensive data is available in the Pakistani population. The present study aimed to review the therapeutic interventions, relapses and mortality rate in patients with TTP treated at a tertiary care hospital in Pakistan. This was a retrospective review of patients treated over a period of more than nine years (2001-2010). Medical charts were retrieved using the ICD coding system version 9 and each file was reviewed by the principal author for clinical and laboratory details, along with the therapy utilized and the outcome. Twenty-five patients were diagnosed with TTP, including nine males (36%) and 16 females (64%) with a median age of 30 ± 18.4 years for all patients. Idiopathic TTP was seen in 17 patients (68%) and secondary causes were identified in eight (32%). Patients were treated with plasma exchange once the diagnosis of TTP was established. Only neurological and renal involvement at the time of presentation emerged as important indicators in determining the outcome and response to treatment. Most of our patients tolerated plasmapheresis very well; however, delay in starting plasmapheresis due to late presentation was a major hurdle in our set up. PMID:22248203

Sagheer, Safoorah; Moiz, Bushra; Usman, Mohammad; Khurshid, Mohammad

2012-02-01

87

Prognostic Factors of Response to Laparoscopic Splenectomy in Patients with Idiopathic Thrombocytopenic Purpura  

PubMed Central

Laparoscopic splenectomy (LS) has become the treatment of choice for patients with idiopathic thrombocytopenic purpura (ITP) who do not respond to medical treatment. The aim of this study was to identify factors predictive of outcome after LS for ITP. From May 1997 to December 2002, we performed 30 LS on patients with ITP. A positive response was defined as a postoperative platelet count greater than 50,000/µL and no requirement for maintenance therapy. Chi-square testing was performed to determine the predictive effects of the following variables: age, sex, preoperative response to steroids or immunoglobulin, duration of disease, antiplatelet antibody, platelet associated antibody, and antinuclear antibody. LS was successfully performed in all patients. For a mean follow-up interval of 24.3 months, response to LS was 73.3%. Splenectomy for steroid nonresponders resulted in an inferior complete response rate (10 of 18, 55.6%) as compared with those that experienced relapse after steroid treatment (11 of 12, 91.7%) (p=0.042). The other significant predictor of outcome by univariate analysis was the time between diagnosis and surgery (p=0.049). The other variables showed no significant correlation with successful splenectomy. We conclude that LS can be performed safely with a satisfactory remission rate in patients with ITP who do not respond to medical treatment, and that the factors most frequently associated with surgical success are a response to steroid and disease duration.

Kwon, Hyuk-Chan; Moon, Chang Hoon; Cho, Young Rak; Kim, Min Chan; Kim, Kyeong Hee; Han, Jin Yeong; Lee, Young Ho; Oh, Sung Yong; Kim, Sung-Hyun; Kim, Jae-Seok

2005-01-01

88

Vaccine administration and the development of immune thrombocytopenic purpura in children  

PubMed Central

The most important reasons cited by the opponents of vaccines are concerns about vaccine safety. Unlike issues such as autism for which no indisputable documentation of direct relationship with vaccine use is available, immune thrombocytopenic purpura (ITP) is an adverse event that can really follow vaccine administration, and may limit vaccine use because little is known about which vaccines it may follow, its real incidence and severity, the risk of chronic disease, or the possibility of recurrences after new doses of the same vaccine. The main aim of this review is to clarify the real importance of thrombocytopenia as an adverse event and discuss how it may interfere with recommended vaccination schedules. The available data clearly indicate that ITP is very rare and the only vaccine for which there is a demonstrated cause-effect relationship is the measles, mumps and rubella (MMR) vaccine that can occur in 1 to 3 children every 100,000 vaccine doses. However, also in this case, the incidence of ITP is significantly lower than that observed during the natural diseases that the vaccine prevents. Consequently, ITP cannot be considered a problem limiting vaccine use except in the case of children suffering from chronic ITP who have to receive MMR vaccine. In these subjects, the risk-benefit ratio of the vaccine should be weighed against the risk of measles in the community.

Cecinati, Valerio; Principi, Nicola; Brescia, Letizia; Giordano, Paola; Esposito, Susanna

2013-01-01

89

[Diagnosis model of idiopathic thrombocytopenic purpura based on platelet differential proteome].  

PubMed

This study was purposed to establish a new quick and simple diagnostic method with high sensitivity and good specificity for idiopathic thrombocytopenic purpura (ITP) and to evaluate its significance. 240 platelet lysates (from patients with ITP, leukemia, MDS, and healthy adults, each of 60 cases) were randomly assigned to training set (120 cases) or validation set (120 cases), all of them were detected by surface enhanced laser desorption ionization time of flight mass spectrometry (SELDI-TOF-MS), in order to identify the differentially expressed protein, the diagnostic model was established by means of artificial neural network (ANN), and was validated by blind test with SPSS 17.0. The results showed that 5 marked proteins significantly differentially expressed (P < 0.01), m/z of highly expressed proteins were 2234.30, 3476.36, and 7526.29, m/z of low expressed proteins were 4990.02 and 5152.39, respectively. The sensitivity and specificity of diagnostic model were 80.6% and 77.3% respectively. The area under the ROC curve consisting of the output value of artificial neura1 network was 0.837. Efficacy of the model was validated by means of blinded test. It is concluded that the ANN model is useful for clinical diagnosis of ITP on the basis of platelet protein fingerprint spectrum. PMID:23484706

Zhou, Pan; Ding, Yin-Huan; He, Peng; Wu, Peng-Qiang; Liu, Wen-Jun; Wang, Kai-Zheng

2013-02-01

90

Splenectomy for immune thrombocytopenic purpura: surgery for the 21st century.  

PubMed

Although immune thrombocytopenic purpura (ITP) is the most common autoimmune hematological disorder, there is still controversy regarding the optimal management of this condition. Medical therapy may cure a proportion of patients with ITP but there are still a significant number of these individuals who are at risk for bleeding events. Surgery has become the least popular therapeutic option for ITP as other medical therapies have become available that attempt to avoid splenectomy and its morbidity. However, the clinical response to these therapies has not been overwhelming based on the fairly small number of trials conducted to date. With current minimally invasive surgical techniques, splenectomy should be again regarded as a viable therapeutic option in patients with ITP. The laparoscopic approach avoids much of the morbidity and complications seen with the conventional open surgical approach and studies have demonstrated similar, if not better, outcomes. In addition, the risk of infection following splenectomy is not as high as may be suspected, particularly with current vaccination regiments. It should be a priority for both the hematology and medical community to advocate for clinical trials to rationally study alternatives to splenectomy. In the interim, laparoscopic splenectomy should be considered as an additional front line therapeutic option in ITP patients. PMID:17722078

Dolan, James P; Sheppard, Brett C; DeLoughery, Thomas G

2008-02-01

91

Learning from errors: when a low platelet count in neonate excludes immune thrombocytopenic purpura in mother.  

PubMed

This paper reviews the most common causes of thrombocytopenia in the newborn. It mentions few classification schemes that clearly characterize the most common causes, diagnosis and treatment approaches for neonatal thrombocytopenia. Particular attention is paid to inborn macrothrombocytopenia without congenital anomalies. They represent a rare group of diseases, often captured randomly or during routine examinations. An attention is paid on congenital macrothrombocytopenia variants with mutations in the MYH9 gene. If they are not associated with other disorders (deafness, presenile cataracts, nephritis or renal failure), they may be mistakenly diagnosed as the acquired immune thrombocytopenic purpura (ITP). This distinguishing is essential to avoid potentially harmful and unnecessary treatment. The listed case report points to a situation where a detection of the root causes of neonatal thrombocytopenia led to a review of misdiagnosed ITP in the mother. A platelet size evaluation by both an appropriate cell counter and blood film examination is useful for differentiating a heterogeneous group of rare inherited macrothrombocytopenias. A healthy twin supported autosomal dominant inheritance. The results of investigations of twins and mother confirmed the congenital/inherited macrothrombocytopenia from the group of MYH9-related diseases (Tab. 5, Fig. 2, Ref. 21). PMID:23514558

Gresikova, M

2013-01-01

92

[Antiphospholipid syndrome with autoimmune hemolytic anemia which mimics thrombotic thrombocytopenic purpura].  

PubMed

A 67-year-old woman was admitted to the hospital for lethargy, fever, hemolytic anemia, thrombocytopenia, and consciousness disturbance. Direct Coombs test was positive, and anti-cardiolipin beta2-glycoprotein I antibody was detected. She was diagnosed with antiphospholipid syndrome complicated with autoimmune hemolytic anemia (AIHA). She demonstrated variable consciousness disturbance, inability to distinguish right from left, dysgraphia and dyscalculia. Multiple cerebral infarctions, especially dominant cerebral hemisphere infarctions, were observed on magnetic resonance imaging. A ventilation-perfusion scan demonstrated the presence of a ventilation-perfusion mismatch in both lung fields, and multiple veinous embolisms in the right femoral, bilateral the great saphenous and popliteal veins. Therefore, pulmonary embolism and thrombophlebitis were diagnosed. Based on these findings, it was necessary to distinguish this diagnosis from thrombotic thrombocytopenic purpura (TTP). As ADAMTS-13 activity was within the normal range, TTP was denied. Thereafter, the patient was treated with 1 mg/kg of prednisolone for AIHA, 3 mg of warfarin, and 3500 units of low-molecular-weight heparin for thrombosis, and her condition improved. PMID:20467225

Karasawa, Naoki; Taniguchi, Yasuhiro; Hidaka, Tomonori; Katayose, Keiko; Kameda, Takuro; Side, Kotaro; Shimoda, Haruko; Nagata, Kenji; Kubuki, Yoko; Matsunaga, Takuya; Shimoda, Kazuya

2010-04-01

93

Platelet antibodies of the IgM class in immune thrombocytopenic purpura  

SciTech Connect

The clinical course and response to therapy of patients with immune thrombocytopenic purpura (ITP) are not completely determined by the level of IgG present on the platelet surface. It is possible that antibodies of other immunoglobulin classes also play a role in platelet destruction in some of these patients. Therefore, the authors studied 175 patients with ITP for the presence of IgM anti-platelet antibodies using radiolabeled polyclonal or monoclonal anti-IgM. They observed that 57% of patients with clinical ITP had increased levels of IgM on their platelets, compared with normal controls and patients with thrombocytopenia who did not have ITP. They obtained similar results using either radiolabeled polyclonal or monoclonal anti-IgM, reagents whose integrity was first characterized using erythrocytes coated with defined amounts of IgM antibody. Among patients with increased platelet-IgM there was a significant correlation both with the presence of increased platelet-C3 as well as the amount of platelet-C3. The authors demonstrated the presence of warm-reacting IgM anti-platelet antibodies in the plasma of two of these patients who were further studied. These studies demonstrate the presence of warm-reacting IgM anti-platelet antibodies in some patients with ITP. They suggest that the binding of complement to platelets by IgM antibodies may initiate platelet clearance as well as enhance the effect of IgG antibodies in ITP.

Cines, D.B.; Wilson, S.B.; Tomaski, A.; Schreiber, A.D.

1985-04-01

94

Combined immune thrombocytopenic purpura and immunoglobulin A nephropathy: a similar pathophysiologic process?  

PubMed

IgA nephropathy is one of the most common forms of glomerulopathies. It is an immune complex-mediated glomerulonephritis diagnosed by the presence of mesangial IgA deposits that are often associated with mesangial cell proliferation. The IgG, C3, IgM, or other immunoglobulin light chains may be co-existed with IgA. Its pathogenesis suggested that it is responsible for enhancing the production of proinflammatory cytokines, chemokines, and growth factors. Platelet-derived growth factor (PDGF) has also been implicated as a modulator of disease activity. Immune thrombocytopenic purpura (ITP) is a bleeding disorder caused by thrombocytopenia that is not associated with a systemic disease. Its pathogenesis suggested an autoimmune disease in which IgG is thought to damage megakaryocytes, which are the precursors of platelet cells. Several studies reported that PDGF levels were higher in normal subjects than in patients with ITP. Moreover, ITP is a disease related to the antibody. Thus, our aim is to examine whether a similar pathophysiological relationship exist between ITP and IgAN that may be mediated by PDGF and/or IgG. PMID:24456575

Kahraman, Cüneyt; Emre, Habib; Gulcan, Erim; Bilen, Yusuf; Uludag, Koray; Uyanik, Abdullah; Kele?, Mustafa

2014-04-01

95

Two cases and review of the literature: primary percutaneous angiography and antiplatelet management in patients with immune thrombocytopenic purpura.  

PubMed

We report two cases of immune thrombocytopenic purpura (ITP) associated with acute coronary artery syndrome highlighting the interventions done in every case along with the medications used during intervention and as outpatient. The first case is that of a woman with ITP exacerbation while on dual antiplatelet therapy and the second case is that of a male presenting with non-ST elevation myocardial infarction (NSTEMI) while in a thrombocytopenic crisis. In both cases antiplatelet therapy was held and thrombopoietic therapy was initiated before resuming full anticoagulation and coronary intervention. Given the paucity of data on ITP and antiplatelets treatment in the setting of acute coronary syndrome, no strict recommendations can be proposed, but antiplatelets appear to be safe acutely and in the long term in this category of patients as long as few measures are undertaken to minimize the risks of bleeding and thrombosis. PMID:24459590

Torbey, Estelle; Yacoub, Harout; McCord, Donald; Lafferty, James

2013-12-29

96

A Case of Non-Hodgkin's Lymphoma in Patient with Coombs' Negative Hemolytic Anemia and Idiopathic Thrombocytopenic Purpura  

PubMed Central

Coombs' negative autoimmune hemolytic anemia (AIHA) is a rare disease which shares similar clinical and hematological features with Coombs' positive AIHA, but its exact frequency remains unknown. There have been few reports of idiopathic thrombocytopenic purpura (ITP) and Coombs' negative AIHA associated with other lymphoproliferative disorders (LPDs). Since there is a well known association between LPDs and autoimmune phenomena, it is important to investigate the possibility of an underlying malignancy. We report a case of ITP and Coombs' negative AIHA associated with diffuse large B-cell lymphoma.

Park, So Yeon; Kim, Eun Sil; Choi, Soon Uk; Hyun, Hee Jae; Ahn, Ju Young; Lee, Ju Hyoung; Ryu, Seo Hee; Park, Jae Hyun; Lee, Gyeong In; Lee, Hyo Jin

2012-01-01

97

A Case of Non-Hodgkin's Lymphoma in Patient with Coombs' Negative Hemolytic Anemia and Idiopathic Thrombocytopenic Purpura.  

PubMed

Coombs' negative autoimmune hemolytic anemia (AIHA) is a rare disease which shares similar clinical and hematological features with Coombs' positive AIHA, but its exact frequency remains unknown. There have been few reports of idiopathic thrombocytopenic purpura (ITP) and Coombs' negative AIHA associated with other lymphoproliferative disorders (LPDs). Since there is a well known association between LPDs and autoimmune phenomena, it is important to investigate the possibility of an underlying malignancy. We report a case of ITP and Coombs' negative AIHA associated with diffuse large B-cell lymphoma. PMID:22500164

Park, So Yeon; Kim, Soyon; Kim, Eun Sil; Choi, Soon Uk; Hyun, Hee Jae; Ahn, Ju Young; Lee, Ju Hyoung; Ryu, Seo Hee; Park, Jae Hyun; Lee, Gyeong In; Lee, Hyo Jin

2012-03-01

98

Massive subretinal and vitreous haemorrhages at presentation in idiopathic thrombocytopenic purpura: report of a case and review of literature.  

PubMed

A 21-year-old woman presented with acute decreased vision due to massive subretinal haemorrhage with macular exudation in the right eye and vitreous haemorrhage in the left eye. Haematological work-up revealed thrombocytopenia (platelets 14,000/?l) with anaemia (haemoglobin 6.3?gm/100?ml). A diagnosis of idiopathic thrombocytopenic purpura (ITP) was confirmed by a haematologist. Systemic therapy resulted in dramatic resolution of the fundus features with near complete restoration of visual acuity over three months. ITP can present with intraocular haemorrhages, especially if accompanied by anaemia. A high index of suspicion and appropriate systemic treatment can lead to a successful outcome. PMID:23763572

Goel, Neha; Arora, Supriya; Jain, Pooja; Ghosh, Basudeb

2014-05-01

99

Autoimmune hepatitis-primary biliary cirrhosis overlap syndrome concomitant with immune hemolytic anemia and immune thrombocytopenic purpura (Evans syndrome).  

PubMed

Autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) associated with Evans syndrome; combination of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP) has rarely been reported. We report the case of a 53-year-old patient who presented with weakness, myalgia, arthralgia, shortness of breath and purpura. Initial laboratory investigations revealed liver dysfunction, anemia and thrombocytopenia. Anti-nuclear (ANA) and antimitochondrial M2 (AMA M2) antibodies were positive. Diagnose of PBC-AIH overlap was made by clinical, serological and histological investigations. AIHA and ITP was identified with clinical-laboratory findings and bone marrow puncture. She was treated with IVIG followed by prednisolone and ursodeoxycholic acid. Hemoglobin-thrombocytes increased rapidly and transaminases improved at day 8. We have reported the first case in the literature with AIH-PBC overlap syndrome concurrent by ITP and AIHA which suggest the presence of shared genetic susceptibility factors in multiple autoimmune conditions including AIH, PBC, ITP and AIHA. PMID:23273499

Korkmaz, Huseyin; Bugdaci, Mehmet Sait; Temel, Tuncer; Dagli, Mehmet; Karabagli, Pinar

2013-04-01

100

The isotype of autoantibodies influences the phagocytosis of antibody-coated platelets in autoimmune thrombocytopenic purpura.  

PubMed

Autoimmune thrombocytopenic purpura (AITP) is an acquired autoimmune bleeding disorder, characterized by isolated thrombocytopenia because of destruction of auto-antibody-coated platelets by Fc-receptor-mediated phagocytosis. The destruction of autoantibody-sensitized platelets by Fc?R-bearing phagocytic cells and the following antigen presentation are considered to play a key role for the pathophysiology of AITP. Although different isotypes of AITP-mediating autoantibodies, e.g. IgG, IgM and IgA, are frequently found in AITP patients, their role in the pathophysiology of AITP remains unclear. Using a flow cytometric monocyte-based phagocytosis assay, we investigated the impact of disease-associated autoantibody isotype in antibody-mediated phagocytosis of platelets. Platelets, labelled with 5-chloromethyl fluorescein diacetate (CMFDA), were incubated with AITP patients' serum characterized by pure IgG or IgM antiplatelet autoantibodies. Labelled platelets were incubated with monocytes. Phagocytosis was defined as the product of percentage of CMFDA-positive monocytes and mean fluorescence intensity of CMFDA. Adherence of platelets to monocytes was quantified by anti-CD61-PerCp in a CMFDA(+) CD14(+) gate. IgG-coated platelets showed a significantly higher phagocytic index than IgM-coated platelets (mean 796?±?157 versus 539?±?78, P?

Hoemberg, M; Stahl, D; Schlenke, P; Sibrowski, W; Pachmann, U; Cassens, U

2011-11-01

101

Effect of Prophylactic Cyclosporine Therapy on ADAMTS13 Biomarkers in Patients with Idiopathic Thrombotic Thrombocytopenic Purpura  

PubMed Central

Several reports have been published regarding the use of cyclosporine (CSA) in the treatment of idiopathic thrombotic thrombocytopenic purpura (TTP). We hypothesized that prophylactic CSA therapy may prevent recurrences in patients with a history of multiple relapses of TTP. Nineteen patients with idiopathic TTP were enrolled on prospective studies at Ohio State University between September 2003 and May 2007. Patients achieving remission remained on CSA therapy for 6 months, allowing us to evaluate the efficacy of CSA as prophylactic therapy. CSA was administered orally at a dose of 2–3 mg/kg in a twice a day divided dose in all patients and continued for a total of 6 months. Long-term clinical follow-up with serial analysis of ADAMTS13 biomarkers during and after CSA therapy were performed to evaluate the efficacy of CSA as a prophylactic therapy. 17/19(89%) patients completed 6 months of CSA therapy in a continuous remission. Two patients relapsed during therapy with CSA and 7 patients relapsed after discontinuing CSA therapy. Ten patients have maintained a continuous remission a median of 21 months (range, 5 to 46) after discontinuing CSA. The ADAMTS13 data suggest that CSA resulted in a significant increase in the ADAMTS13 activity during therapy with CSA. 8/9(89%) relapsing patients had severely deficient ADAMTS13 activity (< 5%) suggesting this is a significant risk factor for relapse of TTP. These data support the hypothesis that prophylactic CSA improves the ADAMTS13 activity and may be effective at preventing relapses in patients at risk for recurrences of TTP.

Cataland, Spero R.; Jin, Ming; Lin, Shili; Kraut, Eric H; George, James N.; Wu, Haifeng M.

2010-01-01

102

Acute renal failure in a patient with antiphospholipid syndrome and immune thrombocytopenic purpura treated with eltrombopag.  

PubMed

Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by the presence of antibodies directed against phospholipid-binding proteins such as beta-2 glycoprotein I and prothrombin, venous and/or arterial thromboembolism, and recurrent fetal loss. To fulfill diagnostic criteria, patients must have thrombotic or obstetrical morbidity and consistently positive antiphospholipid testing on two occasions separated by at least 12 weeks [2]. Other clinical manifestations include thrombocytopenia, renal insufficiency, vasculitis, and cardiac valvular abnormalities. Thrombocytopenia occurs in 20-40% of patients with APS while renal dysfunction develops in ~25% of patients with primary APS [3,4]. Thrombocytopenia is thought to be primarily due to the presence of autoantibodies directed against platelet membrane glycoproteins, although platelet activation and aggregation by APS-associated antibodies have also been implicated. Thrombocytopenia in patients with APS is typically moderate in severity with platelet counts remaining greater than 50,000 cells/mm3 in most cases,so therapy is often unnecessary. When treatment is required, therapies used for immune thrombocytopenic purpura (ITP) are often effective. Corticosteroids, intravenous immunoglobulin, dapsone, and rituximab have all been shown to be effective in APS patients with thrombocytopenia[5-7]. Since November 2008, eltrombopag (Promacta; GlaxoSmithKline,Middlesex, United Kingdom), a nonpeptide thrombopoietin receptor agonist that stimulates the development of megakaryocytes, has been approved for use in the management of chronic ITP. To date, no reports of renal toxicity have emerged with its use. We report the case of a patient with APS and steroid-dependent thrombocytopenia who developed partially reversible acute renal failure after initiation of eltrombopag that recurred on rechallenge. PMID:20652968

Sperati, C John; Streiff, Michael B

2010-09-01

103

A cathepsin-like cysteine proteinase proaggregating activity in thrombotic thrombocytopenic purpura.  

PubMed

The platelet aggregating activity (PAA) of serum and plasma from thrombotic thrombocytopenic purpura (TTP, eight patients) and haemolytic uraemic syndrome (HUS, three patients) was investigated. Sample-induced aggregation of washed platelets and the parallel 3H-serotonin release were studied both in the presence and the absence of purified von Willebrand factor (vWF) or defibrinated cryoprecipitate, according to Kelton et al (1987). All the samples but two were positive in the test, vWF being indispensable for aggregation in all but two of the active cases. Samples from five patients during remission were ineffective under the same conditions, as were 14 controls from healthy donors. An inhibition study using four cysteine proteinase inhibitors (1 mM iodoacetamide, 0.5 mM E-64, 0.1 mM antipain and 0.1 mM leupeptin) and two serine proteinase inhibitors (200 U/ml aprotinin and 0.2 U/ml hirudin) confirmed that TTP/HUS's PAA behaved enzymatically as a cysteine proteinase, as described (Murphy et al, 1987a). These results were confirmed on a protein preparation obtained from pooled plasmas after Al(OH)3 adsorption, ammonium sulphate precipitation, binding to and elution from a p-(chloromercuri)benzoate (PCMB) affinity resin. To further characterize this activity we investigated whether it was a calcium-dependent neutral protease or a lysosomal cathepsin by testing the sensitivity to a series of peptidyl diazomethyl ketones, synthetic inhibitors specifically designed for cysteine proteinases. We confirm here the presence of a circulating platelet aggregating cysteine proteinase in TTP/HUS and provide evidence for the first time that this activity behaves like a lysosomal cathepsin. PMID:1751375

Falanga, A; Consonni, R; Ruggenenti, P; Barbui, T

1991-11-01

104

Detection of expression of IL-18 and its binding protein in Egyptian pediatric immune thrombocytopenic purpura.  

PubMed

Abstract Immune thrombocytopenic purpura (ITP) is an autoimmune disorder, characterized by dysfunctional cellular immunity including the presence of activated platelet specific autoreactive T cells that recognize and respond to autologous platelet antigens. Autoreactive T cells drive the generation of platelet reactive autoantibodies by B cells as well as T-cytotoxic cell-mediated lysis of platelets. Interleukin-18 (IL-18) is a mediator of T helper type 1 cell responses synergistically with IL-12 that initiate and promote host defense and inflammation. IL-18 has a specific binding protein (IL-18BP) which belongs to the immunoglobulin superfamily. In the present study, serum level and messenger RNA( mRNA) expression of IL-18 as well as IL-18BP mRNA expression were measured in peripheral blood mononuclear cells (PBMNCs) of 100 Egyptian pediatric patients with ITP (70 acute and 30 chronic). In addition to this, we recruited 80 healthy volunteers in order to investigate the possible association between the imbalance of IL-18 and IL-18 BP expressions and the pathogenesis of ITP. IL-18 serum level and mRNA expression were not elevated in cases more than in the control group, but IL-18 mRNA was higher in chronic cases when compared to the acute ones (p?=?0.031) and there was a good negative correlation between the platelet count and serum IL-18. IL-18 BP m-RNA was slightly elevated in cases more than in the control group (95% Confidence interval?=?1.15-2.01). Our results were not supportive for previous findings of elevated IL18/BP mRNA ratio in ITP patients. This could be referred to the fact that autoimmune diseases are complex genetic disorders, therefore further studies on polymorphisms affecting IL-18 gene expression as well as kinetics of IL-18 expression are required to evaluate the role of interleukin 18 and its binding protein in the pathogenesis of ITP. PMID:23556537

Shaheen, Iman A; Botros, Shahira K A; Morgan, Dalia S

2014-01-01

105

Plasma microRNA profiling of pediatric patients with immune thrombocytopenic purpura.  

PubMed

Immune thrombocytopenic purpura (ITP) is a commonly acquired autoimmune bleeding disorder in children. MicroRNAs (miRNAs) are small RNAs which are found in cells and circulation, and play a role in protein synthesis and regulation. In this study, we aimed to determine a biomarker for childhood ITP comparing the plasma miRNA levels of children having ITP with healthy children. A total of 86 patients with ITP and 56 healthy children followed up by the Department of Pediatric Hematology and Oncology in University of Gaziantep since July 2011 were enrolled in the study. The 86 patients with ITP were evaluated in two groups as 43 acute ITP (aITP) and 43 chronic ITP (cITP) patients. Plasma expression levels of 379 miRNAs were investigated by RT-PCR (quantitative RT-PCR) technique and they were compared between aITP, cITP, and control groups. For all miRNAs, the average of raw quantification cycle values of three groups separately in the analysis chip was accepted as the reference gene value, and normalization was done according to this value. Statistically significant differences were detected in seven miRNAs (miR-302c-3p, miR-483-5p, miR-410, miR-544a, miR-302a-3p, miR-223-3p, and miR-597) investigated between the groups with respect to the expression levels. The expression rates were found to be over 95% in miR-302c-3p and miR-483-5p, over 75% in miR-410, and over 40% in miR-544, miR-302a-3p, and miR-223-3p in all three groups. The detection of significant differences between plasma miRNA levels of aITP and cITP patients and healthy children may provide useful information in the prediction of the course of disease, determination of disease etiopathogenesis, and the development of new therapeutic modalities. PMID:24418947

Bay, Ali; Coskun, Enes; Oztuzcu, Serdar; Ergun, Sercan; Yilmaz, Fatih; Aktekin, Elif

2014-06-01

106

Common variable immunodeficiency unmasked by treatment of immune thrombocytopenic purpura with Rituximab  

PubMed Central

Background Hypogammaglobulinemia may be part of several different immunological or malignant conditions, and its origin is not always obvious. Furthermore, although autoimmune cytopenias are known to be associated with common variable immunodeficiency (CVID) and even may precede signs of immunodeficiency, this is not always recognized. Despite novel insight into the molecular immunology of common variable immunodeficiency, several areas of uncertainty remain. In addition, the full spectrum of immunological effects of the B cell depleting anti-CD20 antibody Rituximab has not been fully explored. To our knowledge this is the first report of development of CVID in a patient with normal immunoglobulin prior to Rituximab treatment. Case presentation Here we describe the highly unusual clinical presentation of a 34-year old Caucasian male with treatment refractory immune thrombocytopenic purpura and persistent lymphadenopathy, who was splenectomized and received multiple courses of high-dose corticosteroid before treatment with Rituximab resulted in a sustained response. However, in the setting of severe pneumococcal meningitis, hypogammaglobulinemia was diagnosed. An extensive immunological investigation was performed in order to characterize his immune status, and to distinguish between a primary immunodeficiency and a side effect of Rituximab treatment. We provide an extensive presentation and discussion of the literature on the basic immunology of CVID, the mechanism of action of Rituximab, and the immunopathogenesis of hypogammaglobulinemia observed in this patient. Conclusions We suggest that CVID should be ruled out in any patient with immune cytopenias in order to avoid diagnostic delay. Likewise, we stress the importance of monitoring immunoglobulin levels before, during, and after Rituximab therapy to identify patients with hypogammaglobulinemia to ensure initiation of immunoglobulin replacement therapy in order to avoid life-threatening invasive bacterial infections. Recent reports indicate that Rituximab is not contra-indicated for the treatment of CVID-associated thrombocytopenia, however concomitant immunoglobulin substitution therapy is of fundamental importance to minimize the risk of infections. Therefore, lessons can be learned from this case report by clinicians caring for patients with immunodeficiencies, haematological diseases or other autoimmune disorders, particularly, when Rituximab treatment may be considered.

2013-01-01

107

Opana ER abuse and thrombotic thrombocytopenic purpura (TTP)-like illness: a rising risk factor in illicit drug users.  

PubMed

We report the case of a 22 year-old-woman who presented with upper extremity cellulitis secondary to an infiltration of illicit intravenous drug use. She confessed to the intravenous use of Opana ER (an extended release oral formulation of oxymorphone) which is an opioid drug approved only for oral use. She was found to have clinical evidence of profound thrombotic microangiopathy which resulted due to the intravenous use of Opana ER. She showed full clinical improvement after withholding drug and supportive clinical care. Recent report of Opana ER intravenous abuse was published from Tennessee county and has now been increasingly recognised as one of the causes of thrombocytopenia which mimicks clinically as thrombotic thrombocytopenic purpura. Physicians should be aware of this association as the lack of familiarity to this can pose serious management dilemmas for our patients (especially the polysubstance abusers). PMID:24591390

Kapila, Aaysha; Chhabra, Lovely; Chaubey, Vinod K; Summers, Jeffery

2014-01-01

108

Primary Percutaneous Coronary Intervention for Acute Myocardial Infarction in a Young Female with Idiopathic Thrombocytopenic Purpura: A Case Report and Review  

PubMed Central

A 23-year-old female with the diagnosis of idiopathic thrombocytopenic purpura (ITP) was admitted to our hospital with severe chest pain. The electrocardiogram (ECG) revealed acute anterior myocardial infarction. She underwent an immediate cardiac catheterization. An occluded left anterior descending (LAD) was detected by coronary angiography. Reperfusion was performed successfully by angioplasty and stenting with optimal distal flow without any complications. In this report we discussed the management strategies of acute myocardial infarction (AMI) in a patient with ITP.

Yildiz, Ahmet; Coskun, Ugur; Batukan, Ozlem Esen; Keskin, Kudret

2010-01-01

109

The Oklahoma thrombotic thrombocytopenic Purpura-Hemolytic uremic syndrome (TTP-HUS) registry: a community perspective of patients with clinically diagnosed TTP-HUS  

Microsoft Academic Search

The Oklahoma Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome (TTP-HUS) Registry enrolls all consecutive patients for whom plasma exchange treatment is requested for clinically diagnosed TTP-HUS within a defined geographic region. During 14.5 years, from January 1, 1989 until June 30, 2003, 301 patients have been enrolled; follow-up is complete on 300 patients. Clinical categories have been designated based on associated conditions

James N George; Sara K Vesely; Deirdra R Terrell

2004-01-01

110

Human/BALB radiation chimera engrafted with splenocytes from patients with idiopathic thrombocytopenic purpura produce human platelet antibodies.  

PubMed Central

We have previously shown that lethally irradiated normal strains of mice, radioprotected with severe combined immunodeficient (SCID) bone marrow, can be engrafted with human peripheral blood mononuclear cells (PBMC). The human/mouse radiation chimera can mount marked humoral and cellular responses to recall antigens, as well as primary responses. In the present study, we adoptively transferred splenocytes from patients with chronic immune thrombocytopenic purpura (ITP) into lethally irradiated BALB/c mice, radioprotected with SCID bone marrow. High titres of total human immunoglobulin appeared as early as 2 weeks post-transplant and declined after 6 weeks, while human anti-human platelet antibodies were detected 2-8 weeks after the transfer of splenocytes. The immunoglobulin G (IgG) fraction contained antibodies against glycoprotein (GP) IIb/IIIa (CD41) or GPIb/IX (CD42). The human platelet antibodies showed a low level of cross-reactivity with mouse platelets, and thrombocytopenia in the animals was not observed. Splenocytes from individual ITP patients differed in their capacity to produce either human platelet antibodies or total human immunoglobulin. Furthermore, antibodies produced in the murine system were not always identical to the original antibodies present in the serum of the patients. The study of the serological aspects of autoantibodies against human platelets in an animal model might be useful for the investigation of potential therapeutics in ITP.

Dekel, B; Marcus, H; Shenkman, B; Shimoni, A; Shechter, Y; Canaan, A; Berrebi, A; Varon, D; Reisner, Y

1998-01-01

111

R1933X mutation in the MYH9 gene in May-Hegglin anomaly mimicking idiopathic thrombocytopenic purpura.  

PubMed

May-Hegglin anomaly (MHA) is a rare autosomal dominant disorder characterized by the triad of thrombocytopenia, giant platelets, and inclusion bodies in leukocytes. Recent evidence links MHA to mutations in the MYH9 gene. MHA has not been reported in Taiwan before. We report a 25-year-old Taiwanese man who presented with prolonged bleeding after dental extraction. Examination of peripheral blood smear revealed thrombocytopenia (platelet = 35,000/?L), giant platelets, and Döhle-like cytoplasmic inclusions in neutrophils. A strong family history of thrombocytopenia favored hereditary macrothrombocytopenia over idiopathic thrombocytopenic purpura (ITP). Electron microscopy revealed a spindle shape and parallel order of filaments in the inclusions, consistent with the diagnosis of MHA. We performed mutational analysis using polymerase chain reaction followed by direct sequence of the MYH9 gene for the patient, his maternal uncle and cousin, and all showed the same heterozygous R1933X mutation in exon 40. MHA should be considered when a young patient has thrombocytopenia, frequently misdiagnosed as ITP. Morphological examination of peripheral blood smear, family history tracing and genetic studies are required to make an accurate diagnosis and avoid unnecessary and even harmful therapies such as corticosteroids and splenectomy. PMID:23759689

Sung, Chih-Chien; Lin, Shih-Hua; Chao, Tai-Kuang; Chen, Yeu-Chin

2014-01-01

112

Mechanisms of smooth muscle antibody production: a clinical study in children with infections, haemolytic syndromes, and idiopathic thrombocytopenic purpura.  

PubMed Central

Sera from 530 children suffering from various diseases and from 64 controls were tested for smooth muscle autoantibodies (SMA) by indirect immunofluorescence. A high incidence of SMA (51-86%) was found in patients with viral and bacterial infections (viral hepatitis, infectious mononucleosis, measles, mumps, chickenpox, typhoid fever, and brucellosis), independently of liver invovlvement, and in patients with acute haemolytic anaemia due to G-6-PD deficiency (48%). By contrast, the incidence of SMA from patients with beta-thalassaemia major and idiopathic thrombocytopenic purpura was no higher than in the controls. The discrepancy in incidence in haemolytic anaemias due to different causes may reflect the effect of endogenous and extrinsic agents. In the viral infections, SMA were mainly of the IgM class and gave an 'SMA-V' staining pattern. In bacterial infections (typhoid fever and brucellosis), SMA were either IgG only or IgM and IgG, and the staining pattern was also mainly 'SMA-V'. In infections which affect or may affect the liver (viral hepatitis, infectious mononucleosis, typhoid fever, and brucellosis), SMA was present at high titres (1:80-1:320), whereas in infections not affecting the liver (measles, mumps, and chickenpox) the titres were lower (less than or equal to 1:80). In most patients SMA occurred transiently and without apparent pathogenetic significance. The antigen against which infection-induced SMA is directed is not actin; its nature has yet to be identified.

Kanakoudi-Tsakalidis, F; Cassimos, C; Papastavrou-Mavroudi, T; Akoglu, T; Toh, B H; Yildiz, A; Osung, O; Holborow, E J; Sotelo, J

1979-01-01

113

FATAL OUTCOME OF INFECTION BY DENGUE 4 IN A PATIENT WITH THROMBOCYTOPENIC PURPURA AS A COMORBID CONDITION IN BRAZIL  

PubMed Central

Dengue is currently a major public-health problem. Dengue virus (DENV) is classified into four distinct serotypes, DENV 1-4. After 28 years of absence, DENV-4 was again detected in Brazil in 2010 in Roraima State, and one year later, the virus was identified in the northern Brazilian states of Amazonas and Pará, followed by Rio de Janeiro and São Paulo. In Minas Gerais, the first confirmed case of DENV-4 occurred in the municipality of Frutal in 2011 and has now been isolated from a growing number of patients. Although DENV-2 is associated with the highest risk of severe forms of the disease and death due to the infection, DENV-4 has also been associated with severe forms of the disease and an increasing risk of hemorrhagic manifestations. Herein, the first fatal case of confirmed DENV-4 in Brazil is reported. The patient was an 11-year-old girl from the municipality of Montes Claros in northern Minas Gerais State, Brazil. She had idiopathic thrombocytopenic purpura as a comorbid condition and presented with a fulminant course of infection, leading to death due to hemorrhagic complications. Diagnosis was confirmed by detection of Dengue-specific antibodies using IgM capture enzyme-linked immunosorbent assay and semi-nested RT-PCR. Primary care physicians and other health-care providers should bear in mind that DENV-4 can also result in severe forms of the disease and lead to hemorrhagic complications and death, mainly when dengue infection is associated with coexisting conditions.

Amancio, Frederico Figueiredo; Pereira, Maira Alves; Iani, Felipe Campos de Melo; D'anunciacao, Lorena; de Almeida, Jorge Luis Carvalho; Soares, Janer Aparecida Silveira; Ferraz, Marcela Lencine; Vale, Thiago Cardoso; Lambertucci, Jose Roberto; Carneiro, Mariangela

2014-01-01

114

The role of human leukocyte antigens as predisposing and/or protective factors in patients with idiopathic thrombotic thrombocytopenic purpura.  

PubMed

Fifty-four adult German patients suffering from idiopathic thrombotic thrombocytopenic purpura (TTP) have been examined for HLA class II. All patients presented autoantibodies against ADAMTS13 and ADAMTS13 activity levels <5%. Blood samples have been analyzed for HLA-DRB1 and DQB1 alleles using sequence-specific primer PCR and sequence-specific oligonucleotide PCR. Reference data of German bone marrow and blood donors were obtained from www.allelefrequencies.net. The results were evaluated employing two-sided binomial tests, and p values were corrected using the Benjamini-Hochberg procedure. A significant accumulation for DQB1*02:02 (p < 0.001) and DRB1*11 (p = 0.003) was found within the patient group. Twenty percent (DQB1*02:02) or 48.1% (DRB1*11) of TTP patients were tested positive for the particular HLA antigen compared to 1.2% (DQB1*02:02) or 23.5% (DRB1*11) in the control group. A tendency for a reduced occurrence of HLA-DRB1*04 was revealed (7.4% in patients compared to 24.6% in controls). An association between the HLA antigens DQB1*02:02 and DRB1*11 and disease susceptibility for idiopathic TTP has been found. A higher risk for disease outbreak within persons carrying the mentioned alleles can be assumed. The reduced occurrence of HLA-DRB1*04 in TTP patients indicates a possible protective effect of this HLA allele in disease development. PMID:22203269

John, Marie-Luise; Hitzler, Walter; Scharrer, Inge

2012-04-01

115

Fatal outcome of infection by dengue 4 in a patient with thrombocytopenic purpura as a comorbid condition in Brazil.  

PubMed

Dengue is currently a major public-health problem. Dengue virus (DENV) is classified into four distinct serotypes, DENV 1-4. After 28 years of absence, DENV-4 was again detected in Brazil in 2010 in Roraima State, and one year later, the virus was identified in the northern Brazilian states of Amazonas and Pará, followed by Rio de Janeiro and São Paulo. In Minas Gerais, the first confirmed case of DENV-4 occurred in the municipality of Frutal in 2011 and has now been isolated from a growing number of patients. Although DENV-2 is associated with the highest risk of severe forms of the disease and death due to the infection, DENV-4 has also been associated with severe forms of the disease and an increasing risk of hemorrhagic manifestations. Herein, the first fatal case of confirmed DENV-4 in Brazil is reported. The patient was an 11-year-old girl from the municipality of Montes Claros in northern Minas Gerais State, Brazil. She had idiopathic thrombocytopenic purpura as a comorbid condition and presented with a fulminant course of infection, leading to death due to hemorrhagic complications. Diagnosis was confirmed by detection of Dengue-specific antibodies using IgM capture enzyme-linked immunosorbent assay and semi-nested RT-PCR. Primary care physicians and other health-care providers should bear in mind that DENV-4 can also result in severe forms of the disease and lead to hemorrhagic complications and death, mainly when dengue infection is associated with coexisting conditions. PMID:24879007

Amâncio, Frederico Figueiredo; Pereira, Maira Alves; Iani, Felipe Campos de Melo; D'anunciação, Lorena; Almeida, Jorge Luís Carvalho de; Soares, Janer Aparecida Silveira; Ferraz, Marcela Lencine; Vale, Thiago Cardoso; Lambertucci, José Roberto; Carneiro, Mariângela

2014-06-01

116

Downregulation of T-bet/GATA-3 ratio induced by IL-11 treatment is responsible for Th1/Th2 balance restoration in human immune thrombocytopenic purpura (ITP).  

PubMed

Abnormal cellular immunity induced by deranged Th1/Th2 profile has been revealed to play a critical role in the pathogenesis of immune thrombocytopenic purpura (ITP). Correction of the shifted Th1/Th2 balance represents a potential therapeutic approach to treat ITP. Here, we investigated the effects of IL-11 on the restoration of Th1/Th2 balance in the peripheral blood mononuclear cells (PBMCs) isolated from adult ITP patients. As shown here, we observed a higher ratio of T-bet/GATA-3 gene expression by quantitative real-time PCR in the PBMCs from ITP patients, consistent with the presence of an abnormally high Th1/Th2 ratio. Remarkably, upon IL-11 treatment, a reversal of T-bet/GATA-3 ratio in ITP was achieved and was shown to be responsible for the restoration of Th1/Th2 balance, with IL-11 at 100 ng/ml demonstrating the highest efficiency. T-bet and GATA-3 are the two transcriptional factors that have been indicated to be the master regulators for Th1 and Th2 lineage commitment, respectively. In the presence of 100 ng/ml IL-11, GATA-3 transcript abundance rose up to ~85-fold of that measured in untreated cells, whereas T-bet transcripts were lowered merely to ~41 %, suggesting that GATA-3 was the major contributor for the reversal of T-bet/GATA-3 ratio. Thus, our findings may very well encourage the development of novel medicines that specifically target and correct the T-bet/GATA-3 imbalance identified in ITP. PMID:24338248

Yao, Rongxin; Lin, Ying; Li, Qianqian; Zhou, Xieming; Pan, Xiahui; Bao, Yunhua; He, Muqing; Zhu, Baoling; Guo, Wenjian; Lin, Xiaoji; Jin, Limin

2014-08-01

117

Rituximab therapy for thrombotic thrombocytopenic purpura: A proposed study of the Transfusion Medicine\\/Hemostasis Clinical Trials Network with a systematic review of rituximab therapy for immune-mediated disorders  

Microsoft Academic Search

The rationale for immunosuppressive therapy of thrombotic thrombocytopenic purpura (TTP) was estab- lished by observations that TTP may be caused by autoantibodies to ADAMTS13. Patients with high-titer autoantibodies to ADAMTS13 may have a higher mortality, and survivors may require prolonged plasma exchange therapy in spite of adjunctive glucocorticoid treatment. More intensive immunosuppressive therapy with rituximab may provide benefit for many

James N. George; Robert D. Woodson; Joseph E. Kiss; Kiarash Kojouri; Sara K. Vesely

2006-01-01

118

A rare association of localized scleroderma type morphea, vitiligo, autoimmune hypothyroidism, pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case report  

PubMed Central

Background The localized scleroderma (LS) known as morphea, presents a variety of clinical manifestations that can include systemic involvement. Current classification schemes divide morphea into categories based solely on cutaneous morphology, without reference to systemic disease or autoimmune phenomena. This classification is likely incomplete. Autoimmune phenomena such as vitiligo and Hashimoto thyroiditis associated with LS have been reported in some cases suggesting an autoimmune basis. To our knowledge this is the first case of a morphea forming part of a multiple autoimmune syndrome (MAS) and presenting simultaneously with autoimmune thrombocytopenic purpura and central nervous system vasculitis. Case presentation We report an uncommon case of a white 53?year old female patient with LS as part of a multiple autoimmune syndrome associated with pneumonitis, autoimmune thrombocytopenic purpura and central nervous system vasculitis presenting a favorable response with thrombopoietin receptor agonists, pulses of methylprednisolone and cyclophosphamide. Conclusion Is likely that LS have an autoimmune origin and in this case becomes part of MAS, which consist on the presence of three or more well-defined autoimmune diseases in a single patient.

2012-01-01

119

Safety and Efficacy of Gammaplex(R) in Idiopathic Thrombocytopenic Purpura (ClinicalTrials.gov - NCT00504075)  

PubMed Central

Background and Objectives This multicentre, open-label study investigated the safety and efficacy of Gammaplex, a 5% Intravenous Immunoglobulin (IVIg), in patients with idiopathic (immune) thrombocytopenic purpura (ITP). Materials and Methods Patients were between the ages of 6 and 70 years; had ITP for at least six months and had a platelet count ?20×109/L. Eligible patients were dosed with 1 g/kg of Gammaplex on two consecutive days, followed by assessment of safety and efficacy on Days 3, 5, 9, 14, 21, 32 and 90. Response was defined as the increase in platelet count to a threshold of ?50×109/L on or before Day 9 after the first dose of Gammaplex. Results All 35 patients received at least one infusion of Gammaplex. Twenty-nine (83%) patients responded to Gammaplex, similar to the historical control, with a 95% lower one-sided confidence interval of 68.9%. Median duration of response was 10.0 days, with an overall reduction in bleeding episodes. Gammaplex provided supranormal concentrations of total IgG; mean peak concentration (Cmax) of 45.3 g/L (4.53 g/dL), with a mean half-life of 28.5 days. Fifteen patients reported 63 Adverse Drug Reactions (ADRs); the most common were headache (10 patients), vomiting (6 patients) and pyrexia (5 patients). Five of these ADRs were considered serious, one patient had three concurrent Serious Adverse Events (SAEs); these were vomiting, dehydration and headache. Two other patients each had one SAE (headache). There were no unexpected Adverse Events (AEs) or thromboembolic episodes and no significant changes in vital signs, biochemical, haematological and virology results. Conclusion: Gammaplex achieved a very high concentration of serum IgG but was well-tolerated and effective in the treatment of ITP with a similar degree of efficacy to the pre-determined historical control group and the pre-set statistical criteria. Trial Registration ClinicalTrials.gov NCT00504075 Clinical Trials Registry India 000016

Dash, Clive H.; Gillanders, Kate R.; Stratford Bobbitt, Margaret E.; Gascoigne, Ernie W.; Leach, Samantha J.

2014-01-01

120

[The combination therapy with vinca-alkaloid slow infusion and cholchicine was effective to refractory idiopathic thrombocytopenic purpura. The patient could receive femoral head replacement in safety].  

PubMed

A 46-year-old man with idiopathic thrombocytopenic purpura (ITP) refractory to corticosteroid, splenectomy and other drugs was admitted to our hospital in August, 1994, because of aseptic necrosis of the right femoral head. Although high-dose intravenous gamma-globulin was ineffective, the platelet count was increased within two weeks by the combination therapy that consisted of 0.02 mg/kg vincristine alternating with 0.1 mg/kg vinblastine by slow infusion at a 1-week interval, and oral 1.5 mg/day colchicine. He subsequently underwent the femoral head replacement. This combination therapy seems to be useful for refractory ITP in preparation for surgery. PMID:8852035

Nezu, M; Oh, H; Cho, R; Sato, H; Ikegami, T; Yokota, A; Nakaseko, C; Nishimura, M; Matsuura, Y; Morio, S; Hiruma, K; Nakamura, H; Asai, T

1996-02-01

121

Combination of high-dose dexamethasone and antiretroviral therapy rapidly improved and induced long-term remission of HIV-related thrombocytopenic purpura.  

PubMed

We present a case of HIV-related thrombocytopenic purpura (HIV-ITP) successfully treated with high-dose dexamethasone and antiretroviral therapy (ART). Although high-dose dexamethasone is regarded as the first-line therapy in adult patients with non-HIV ITP, there is limited information on treatment of HIV-ITP and long-term prednisone therapy is considered the standard therapy. High-dose dexamethasone is preferable to conventional long-term prednisone therapy, because of fewer side effects mainly due to shorter steroid use. The ART helps achieve long-term remission for HIV-ITP, although this therapy lacks an immediate effect. In our patient, administration of high-dose dexamethasone resulted in rapid rise in platelet count and ART maintained long-term remission of HIV-ITP. The combination therapy is potentially suitable strategy for the treatment of patients with HIV-ITP and severe thrombocytopenia or bleeding. PMID:23532665

Shindo, Takuma; Nishijima, Takeshi; Teruya, Katsuji; Mizushima, Daisuke; Gatanaga, Hiroyuki; Oka, Shinichi

2013-12-01

122

Thrombotic thrombocytopenic purpura  

MedlinePLUS

Plasma exchange (plasmapheresis plus infusion of donor plasma) is used to remove the antibodies that are affecting clotting from the blood and also replace the missing enzyme. First, you will have ...

123

Thrombopoietin receptor agonists administration for acute exacerbation of chronic idiopathic thrombocytopenic purpura and subsequent anticoagulant therapy for accompanying deep venous thrombosis of the lower limbs.  

PubMed

We report two patients (70- and 49-year-old Japanese men) with acute exacerbation of chronic idiopathic thrombocytopenic purpura (ITP) and deep venous thrombosis of the lower extremities. Both were successfully managed with thrombopoietin receptor agonist (TPO-RA) administration. Both had ITP refractory to steroid treatment. Their immature platelet fraction (absolute-IPF) counts were increased and paralleled the platelet recoveries after TPO-RA (eltrombopag and romiplostim, respectively) without progression of thrombosis. Although ITP has recently been evaluated as a thrombophilic disorder, reports on acute exacerbation of ITP with newly diagnosed thrombosis are limited, and the pathophysiology and association between ITP and thrombosis remain to be elucidated. Moreover, the influences of TPO-RA on thrombosis are still controversial. To our knowledge, this is the first case report describing patients with exacerbation of ITP who developed thrombosis and were treated with TPO-RA. The outcomes of our cases underscore the importance of monitoring thrombosis and not delaying the initiation of anticoagulation treatment during the use of TPO-RA. PMID:24975340

Kawano, Hiroki; Suzuki, Tomohide; Ishii, Shinichi; Wakahashi, Kanako; Kawano, Yuko; Sada, Akiko; Minagawa, Kentaro; Takaya, Tomofumi; Yamashita, Tomoya; Hirata, Ken-Ichi; Koriyama, Kenji; Nagamatsu, Yuichi; Matsui, Toshimitsu; Katayama, Yoshio

2014-06-01

124

Randomized trial comparing intravenous immunoglobulin with methylprednisolone pulse therapy in acute idiopathic thrombocytopenic purpura. Danish I.T.P. Study Group.  

PubMed

Forty-three children with newly diagnosed idiopathic thrombocytopenic purpura (ITP), platelet count (PC) below 20 x 10(9)l-1, and either continued bleeding or failure to show a spontaneous rise in the PC after a 3 day observation period were randomized to treatment with either intravenous immunoglobulin (IVIG) infusions 1 g kg-1 (n = 23) or intravenous methylprednisolone pulse therapy (MPPT) 30 mg kg-1 (n = 20) on two consecutive days. After 72 h, IVIG had induced greater platelet responses (mean PC 188 x 10(9) versus 77 x 10(9)l-1, 2p < 0.001) and raised the PC to a haemostatically safe level above 50 x 10(9)l-1 more frequently (91 versus 50%, one-sided exact p = 0.003). Children responding poorly were then given the alternative treatment in addition. After 6 days, a normal PC of over 150 x 10(9)l-1 had been obtained more frequently in the group given first-line IVIG (70 versus 50%, p = 0.16). The relapse rates during 6 months of follow-up were not significantly different (26 versus 40%, p = 0.26). Cross-over treatment in 11 children with relapse confirmed the superior response to IVIG. The treatment given was restricted to the two initial infusions more often in the IVIG group (70 versus 35%, p = 0.05). These results indicate that IVIG may be preferable to MPPT as the initial treatment for ITP. PMID:8863869

Rosthøj, S; Nielsen, S; Pedersen, F K

1996-08-01

125

Micromegakaryocytes in a patient with partial deletion of the long arm of chromosome 11 [del(11)(q24.2qter)] and chronic thrombocytopenic purpura  

SciTech Connect

Thrombocytopenia or pancytopenia is frequently reported in patients with partial 11q deletion but there are no reports on bone marrow morphology of these patients. We report on a patient with partial deletion of the long arm of chromosome 11 [del(11)(q24.2qter)] and its classical clinical manifestations including chronic thrombocytopenic purpura in whom micromegakaryocytes were found in the bone marrow aspirate. This is the first report of the presence of micromegakaryocytes in the bone marrow of a patient with 11q deletion. Accurate examination of the bone marrow of other patients with the 11q deletion may clarify whether the observation of micromegakaryocytes is common in these patients. Micromegakaryocytes may indicate a defect of development. Two genes for two DNA binding proteins that are likely to be involved in hematopoiesis map in the 11q region: Ets-1, that maps to 11q24, close to D11S912, and the nuclear-factor-related-kB gene that maps to 11q24-q25. It is possible that these genes, when present in only one copy, result in thrombocytopenia or pancytopenia as observed in this patient. 23 refs., 2 figs., 1 tab.

Gangarossa, S.; Mattina, T.; Romano, V.; Milana, G.; Mollica, F.; Schiliro, G. [Universita di Catania (Italy)] [Universita di Catania (Italy)

1996-03-15

126

Impact of chronic Immune Thrombocytopenic Purpura (ITP) on health-related quality of life: a conceptual model starting with the patient perspective  

PubMed Central

Background Immune thrombocytopenic purpura (ITP), a condition characterized by autoimmune-mediated platelet destruction and suboptimal platelet production, is associated with symptoms such as bruising, epistaxis, menorrhagia, mucosal bleeding from the gastrointestinal and urinary tracts and, rarely central nervous system bleeding. The aim of this research is to develop a conceptual model to describe the impact of ITP and its treatment on patients' health-related quality of life (HRQoL). Methods A literature search and focus groups with adult ITP patients were conducted to identify areas of HRQoL affected by ITP. Published literature was reviewed to identify key HRQoL issues and existing questionnaires used to assess HRQoL. Focus group transcripts were reviewed, and common themes were extracted by grouping conceptual categories that described the impact on HRQoL. Results The literature synthesis and themes from the focus group data suggest that decreased platelet counts, disease symptoms, and treatment side effects influence multiple domains of HRQoL for ITP patients. Key areas affected by ITP and its treatments include emotional and functional health, work life, social and leisure activities, and reproductive health. Conclusion ITP affects various areas of HRQoL. This conceptual model will help inform the evaluation of therapeutic strategies for ITP.

Mathias, Susan D; Gao, Sue K; Miller, Kimberly L; Cella, David; Snyder, Claire; Turner, Ralph; Wu, Albert; Bussel, James B; George, James N; McMillan, Robert; Wysocki, Diane Kholos; Nichol, Janet L

2008-01-01

127

Ticlopidine-, Clopidogrel-, and Prasugrel-Associated Thrombotic Thrombocytopenic Purpura: A 20-Year Review from the Southern Network on Adverse Reactions (SONAR)  

PubMed Central

Thienopyridine-derivatives (ticlopidine, clopidogrel, and prasugrel) are the primary antiplatelet agents. Thrombotic thrombocytopenic purpura (TTP) is a rare drug-associated syndrome, with the thienopyridines being the most common drugs implicated in this syndrome. We reviewed 20 years of information on clinical, epidemiologic, and laboratory findings for thienopyridine-associated TTP. Four, 11, and 11 cases of thienopyridine-associated TTP were reported in the first year of marketing of ticlopidine (1989), clopidogrel (1998), and prasugrel (2010), respectively. As of 2011, the FDA received reports of 97 ticlopidine-, 197 clopidogrel-, and 14 prasugrel-associated TTP cases. Severe deficiency of ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) was present in 80% and antibodies to 100% of these TTP patients on ticlopidine, 0% of the patients with clopidogrel-associated TTP (p < 0.05), and an unknown percentage of patients with prasugrel-associated TTP. TTP is associated with use of each of the three thienopyridines, although the mechanistic pathways may differ.

Jacob, Sony; Dunn, Brianne L.; Qureshi, Zaina P.; Bandarenko, Nicholas; Kwaan, Hau C.; Pandey, Dilip K.; McKoy, June M.; Barnato, Sara E.; Winters, Jeffrey L.; Cursio, John F.; Weiss, Ivy; Raife, Thomas J.; Carey, Patricia M.; Sarode, Ravindra; Kiss, Joseph E.; Danielson, Constance; Ortel, Thomas L.; Clark, William F.; Rock, Gail; Matsumoto, Masanori; Fujimura, Yoshihiro; Zheng, X. Long; Chen, Hao; Chen, Fei; Armstrong, John M.; Raisch, Dennis W.; Bennett, Charles L.

2013-01-01

128

Endobronchial hemorrhage after intubation with double-lumen endotracheal tube in a patient with idiopathic thrombocytopenic purpura for minimally invasive cardiac surgery: a case report  

PubMed Central

Minimally invasive cardiac surgery (MICS) requires lung isolation. Lung isolation is usually achieved with double-lumen endotracheal tube (DLT). Patients with idiopathic thrombocytopenic purpura (ITP) have an increased risk of bleeding events. We suspected endobronchial hemorrhage after exchange of DLT during induction of anesthesia for replacement of mitral valve in a 62-year-old man with a known ITP. The MICS was stopped and bronchial artery embolization was performed in the angiographic room. In the present case, in order to reduce the risk of bronchial arterial injury in ITP patient we intubated with single lumen endotracheal tube. Lung isolation led to achievement of intermittent total lung deflation. Based on the results, we recommend a high-dose intravenous immunoglobulin therapy and platelet transfusion prior to cardiac surgery in patients with ITP to increase platelet count. Moreover, it is proposed that in order to clear the vision during the operation, ventilation can be held or made intermittent both prior to cardiopulmonary bypass or at its conclusion to permit exposure.

Kim, Hee Young; Kim, Kyoung Hoon; Kim, Nam Won

2014-01-01

129

Platelet turnover and kinetics in immune thrombocytopenic purpura: results with autologous 111In-labeled platelets and homologous 51Cr-labeled platelets differ  

SciTech Connect

Mean platelet survival and turnover were simultaneously determined with autologous 111In-labeled platelets (111In-AP) and homologous 51Cr-labeled platelets (51Cr-HP) in ten patients with chronic immune thrombocytopenic purpura (ITP). In vivo redistribution of the 111In-AP was quantitated with a scintillation camera and computer-assisted image analysis. The patients were divided into two groups: those with splenic platelet sequestration (spleen-liver 111In activity ratio greater than 1.4), and those with diffuse sequestration in the reticuloendothelial system. The latter patients had more severe ITP reflected by pronounced thrombocytopenia, decreased platelet turnover, and prominent early hepatic platelet sequestration. Mean platelet life span estimated with 51Cr-HP was consistently shorter than that of 111In-AP. Platelet turnover determined with 51Cr-HP was thus over-estimated. The difference in results with the two isotope labels was apparently due to greater in vivo elution of 51Cr. Although the limitations of the techniques should be taken into account, these findings indicate that platelet turnover is not always normal or increased in ITP, but is low in severe disease. We suggest that this may be ascribed to damage to megakaryocytes by antiplatelet antibody. The physical characteristics in 111In clearly make this radionuclide superior to 51Cr for the study of platelet kinetics in ITP.

Heyns A du, P.; Badenhorst, P.N.; Loetter, M.G.P.; Pieters, H.; Wessels, P.; Kotze, H.F.

1986-01-01

130

Ten years of prophylactic treatment with fresh-frozen plasma in a child with chronic relapsing thrombotic thrombocytopenic purpura as a result of a congenital deficiency of von Willebrand factor-cleaving protease.  

PubMed

We report the results of 10 years of prophylactic fresh-frozen plasma (FFP) infusion therapy in a 14-year-old girl with chronic relapsing thrombotic thrombocytopenic purpura (TTP), in whom a severe congenital von Willebrand factor (VWF)-cleaving protease deficiency has been documented. Severe haemolytic crises triggered by infections were prevented and her present renal and neurological functions have been preserved. Sequential measurements of protease activity and platelet count after FFP infusion led us to conclude tentatively that 5% may be sufficient to degrade very large and adhesive VWF multimers. PMID:11380451

Barbot, J; Costa, E; Guerra, M; Barreirinho, M S; Isvarlal, P; Robles, R; Gerritsen, H E; Lämmle, B; Furlan, M

2001-06-01

131

Abnormal gamma IFN and alpha TNF secretion in purified CD2+ cells from autoimmune thrombocytopenic purpura (ATP) patients: their implication in the clinical course of the disease.  

PubMed

Gamma inferferon (gamma IFN), alpha tumor necrosis factor (alpha TNF), and interleukin 6 (IL-60) are cytokines produced by a wide variety of cells, including T lymphocytes and NK cells. These cytokines affect B-cell proliferation and differentiation into immunoglobulin secreting cells. In addition, gamma IFN and alpha TNF also enhance the function of macrophages, upregulating the expression of their IgG receptors. Abnormalities in the production of these cytokines may be involved in the clinical course of autoimmune thrombocytopenic purpura (ATP). This paper describes the production of these cytokines in PHA-stimulated peripheral blood CD2+ cells from ATP patients. Both gamma IFN and alpha TNF were significantly increased in PHA-stimulated CD2+ cells from therapy-dependent ATP patients (platelet counts < 50,000/microliter), as compared to ATP patients with stable disease (sustained platelet counts < 50,000/microliter without need treatment) (P < 0.05). No significant differences were found in gamma IFN production by PHA-stimulated CD2+ cells between therapy-dependent ATP patients and healthy controls (P < 0.05). However, the production of alpha TNF by PHA-stimulated CD2+ cells from therapy-dependent ATP patients was significantly higher compared to that found in healthy controls (P < 0.05). There were no significant differences in IL-6 production by PHA-stimulated CD2+ cells from ATP patients and healthy controls (P < 0.05). These findings demonstrate abnormal gamma IFN and alpha TNF secretion in purified CD2 cells from ATP patients. The clinical severity of the disease is associated with the altered secretion of these lymphokines by CD2 cells. PMID:7639270

Garcia-Suarez, J; Prieto, A; Reyes, E; Manzano, L; Arribalzaga, K; Alvarez-Mon, M

1995-08-01

132

Therapy with high-dose dexamethasone (HD-DXM) in previously untreated patients affected by idiopathic thrombocytopenic purpura: a GIMEMA experience.  

PubMed

In idiopathic thrombocytopenic purpura (ITP), corticosteroids have been widely recognized as the most appropriate first-line treatment, even if the best therapeutic approach is still a matter of debate. Recently, a single high-dose dexamethasone (HD-DXM) course was administered as first-line therapy in adult patients with ITP. In this paper we show the results of 2 prospective pilot studies (monocentric and multicentric, respectively) concerning the use of repeated pulses of HD-DXM in untreated ITP patients. In the monocenter study, 37 patients with severe ITP, age at least 20 years and no more than 65 years, were enrolled. HD-DXM was given in 4-day pulses every 28 days, for 6 cycles. Response rate was 89.2%; relapse-free survival (RFS) was 90% at 15 months; long-term responses, lasting for a median time of 26 months (range 6-77 months) were 25 of 37 (67.6%). In the multicenter study, 95 patients with severe ITP, age at least 2 years and no more than 70 years, were enrolled. HD-DXM was given in 4-day pulses every 14 days, for 4 cycles; 90 patients completed 4 cycles. Response rate (85.6%) was similar in patients classified by age (<18 years, 36 of 42=85.7%; >or=18 years, 41 of 48=85.4%, P=not significant), with a statistically significant difference between the second and third cycle (75.8% vs 89%, P=.018). RFS at 15 months 81%; long-term responses, lasting for a median time of 8 months (range 4-24 months) were 67 of 90 (74.4%). In both studies, therapy was well tolerated. A schedule of 3 cycles of HD-DXM pulses will be compared with standard prednisone therapy (eg, 1 mg/kg per day) in the next randomized Gruppo Italiano Malattie EMatologiche dell'Adulto (GIMEMA) trial. PMID:17077333

Mazzucconi, Maria Gabriella; Fazi, Paola; Bernasconi, Sayla; De Rossi, Giulio; Leone, Giuseppe; Gugliotta, Luigi; Vianelli, Nicola; Avvisati, Giuseppe; Rodeghiero, Francesco; Amendola, Angela; Baronci, Carlo; Carbone, Cecilia; Quattrin, Stefano; Fioritoni, Giuseppe; D'Alfonso, Giulio; Mandelli, Franco

2007-02-15

133

Living with Thrombotic Thrombocytopenic Purpura  

MedlinePLUS

... doctor about changing medicines that may raise your risk of TTP, such as ticlopidine and clopidogrel. Rate This Content: Clinical Trials Clinical trials are research studies that explore whether a medical strategy, treatment, or device is safe and effective for humans. Related Director's ...

134

Immune Thrombocytopenic Purpura of Childhood  

Microsoft Academic Search

Immune mediated thrombocytopenia (ITP) is a com- mon manifestation of autoimmune disease in children. Although patients often present with bruises, pete- chiae, and some mucosal bleeding, the incidence of life-threatening hemorrhage is rare (0.2-0.9%) but can be fatal when presenting in vital organs. A wide range of therapeutic regimens are currently in use, including observation alone, as the majority of

Diane J. Nugent

135

What Causes Thrombotic Thrombocytopenic Purpura?  

MedlinePLUS

... which is a substance often found in tonic water and nutritional health products Rate This Content: ... The NHLBI updates Health Topics articles on a biennial cycle based on a thorough review of research findings ...

136

Congenital amegakaryocytic thrombocytopenic purpura (CAMT).  

PubMed

Congenital amegakaryocytic thrombocytopenia (CAMT) is a rare, autosomal recessive disorder induced by mutations of the gene coding for thrombopoietin (TPO) receptor (c-MPL) despite high levels of serum TPO. Patients initially present with isolated thrombocytopenia that subsequently progresses into pancytopenia. Although the mechanisms leading to aplasia are unknown, the age of onset has been reported to depend on the severity of the c-MPL functional defect. The primary treatment for CAMT is bone marrow transplantation. This report describes a newborn girl who presented to us with symptoms of sepsis but septic profile came negative except thrombocytopenia. Bone marrow biopsy was done for thrombocytopenia which revealed amegakaryocytic thrombocytopenia. She was given prednisolone. PMID:24709246

Ghauri, Rooha Ijaz; Naveed, Muhammad; Mannan, Jovaria

2014-04-01

137

Photoletter to the editor: Diffuse cocaine-related purpura.  

PubMed

Diffuse purpura is an uncommon skin manifestation found in platelet and coagulation disorders, meningococcemia, vasculitides and cocaine use. Reports of cocaine-related purpura predominantly involve adulteration with the anti-helminthic, levamisole. Levamisole enhances the effects of cocaine and is known to cause vasculitis. Recently, the CDC also released an advisory of oxymorphone being used intravenously causing thrombogenic thrombocytopenic purpura (TTP). We report the case of a patient with diffuse purpura ultimately diagnosed with cocaine-related thrombogenic vasculopathy. In the current environment of adulterated cocaine usage and increased prescription narcotic abuse, it is crucial to investigate substance abuse as a cause of diffuse purpura. PMID:24421868

Sarkar, Debjeet; Kammona, Hussein A; Lamsen, Leonard N; McAbee, Bradley A; Clark, Christopher T; Lee, Solomon S; Kelley, Shane E

2013-01-01

138

Genetics Home Reference: Thrombotic thrombocytopenic purpura  

MedlinePLUS

... Resulting complications can include neurological problems (such as personality changes, headaches, confusion, and slurred speech), fever, abnormal ... Center . Where can I find general information about genetic conditions? The Handbook provides basic information about genetics ...

139

Thrombotic Thrombocytopenic Purpura Associated with Ticlopidine Therapy  

Microsoft Academic Search

A patient who developed thrombotic microangiopa- thy while on ticlopidine therapy is reported. Thrombotic mi- croangiopathy resolved with discontinuation of the drug and treatment with plasma exchange and has not recurred during 10 months of follow-up. The emerging data on the risk of devel- oping thrombotic microangiopathy while on Ticlopidine and the possible mechanisms underlying this association are re- viewed.

SEAN F. LEAVEY; JOEL WEINBERG

140

How Is Thrombotic Thrombocytopenic Purpura Diagnosed?  

MedlinePLUS

... measures the number of red blood cells, white blood cells, and platelets in your blood. For this test, a sample ... your red blood cells. In TTP, the red blood cells are torn and broken. Platelet Count This test counts the number of platelets ...

141

How Is Thrombotic Thrombocytopenic Purpura Treated?  

MedlinePLUS

... and surgery. Treatments are done in a hospital. Plasma Therapy Plasma is the liquid part of your blood. It ... nutrients to your body. TTP is treated with plasma therapy. This includes: Fresh frozen plasma for people ...

142

A case of Henoch-Schönlein purpura in disseminated tuberculosis.  

PubMed

Tuberculosis is still a common disease, even in some parts of developing countries. Although its major impact is pulmonary, the tuberculosis is actually a disseminated disease. An unusual form of renal involvement of tuberculosis is glomerulonephritis, as a part of systemic vasculitis, Henoch-Schoenlein Purpura (HSP). A 41-year-old man, being treated with antituberculous agents for pulmonary tuberculosis, was transferred to our hospital because of newly developed generalized purpura and pretibial edema. Renal manifestations were proteinuria and hematuria. Renal biopsy disclosed interstitial chronic granulomatous inflammation with caseous necrosis and strong nodular mesangial Ig A deposit, along with trace granular Ig G deposition and perivascular C3 deposit. Skin lesions were non-thrombocytopenic palpable purpurae, proved leukocytoclastic vasculitis by skin biopsy. All clinical symptoms and signs were relieved by antituberculous medication. We concluded that disseminated tuberculosis might be a cause of HSP, an immune complex mediated disease. PMID:7626558

Han, B G; Choi, S O; Shin, S J; Kim, H Y; Jung, S H; Lee, K H

1995-01-01

143

Henoch-Schonlein Purpura  

MedlinePLUS

... sometimes progressing to serious kidney involvement. Who gets Henoch-Schönlein Purpura (the “typical” patient)? Usually, HSP affects ... with HSP is 5.9 years. What causes Henoch-Schönlein Purpura? In two-thirds of the cases, ...

144

Safety and Efficacy Study of Romiplostim to Treat ITP in Pediatric Subjects  

ClinicalTrials.gov

Idiopathic Thrombocytopenic Purpura; Thrombocytopenia; Thrombocytopenia in Pediatric Subjects With Immune (Idiopathic) Thrombocytopenic Purpura (ITP); Thrombocytopenia in Subjects With Immune (Idiopathic) Thrombocytopenic Purpura (ITP); Thrombocytopenic Purpura

2014-01-29

145

The clinical implications of adult-onset henoch-schonelin purpura  

PubMed Central

Henoch-Schonlein Purpura (HSP) is a small vessel vasculitis mediated by IgA-immune complex deposition. It is characterized by the clinical tetrad of non-thrombocytopenic palpable purpura, abdominal pain, arthritis and renal involvement. Pathologically, it can be considered a form of immune complex-mediated leukocytoclastic vasculitis (LCV) involving the skin and other organs. Though it primarily affects children (over 90% of cases), the occurrence in adults has been rarely reported. Management often involves the use of immunomodulatory or immune-suppressive regimens.

2011-01-01

146

Assessment of Regulatory T Cells in Childhood Immune Thrombocytopenic Purpura  

PubMed Central

This study had the objective to assess the frequency of Tregs in children newly diagnosed with ITP and ascertain whether an association exists between Tregs and platelet counts, by means of a comparison with healthy controls. This case-control study included 19 patients newly diagnosed with ITP—whose blood samples were collected at four points in time: before any therapy and 1, 3, and 6 months after diagnosis—and 19 healthy controls. Tregs (CD4+ CD25+Foxp3 T cells) were evaluated by flow cytometry. There was a statistically significant difference in platelet count between the case and control groups. There were no significant differences in Treg counts between cases and controls at any point during the course of the study and no difference in Treg counts between the chronic and nonchronic groups and no significant correlation between Tregs and platelet counts in the case and control groups. The findings of this study did not show any statistically significant correlation between Tregs and number of platelets in the case and control groups. Treg cells did not play a role in the regulation of autoimmunity in children with ITP.

Mazzucco, Karina L. M.; Junior, Lauro M.; Lemos, Natalia E.; Wieck, Andrea; Pezzi, Annelise; Laureano, Alvaro M.; Amorin, Bruna; Valim, Vanessa; Silla, Lucia; Daudt, Liane E.; Marostica, Paulo J. C.

2013-01-01

147

Successful treatment with erythromycin for idiopathic thrombocytopenic purpura  

PubMed Central

Macrolides have both immunomodulatory and antibacterial effects. We report 3 cases of primary immune thrombocytopenia (ITP) patients who were successfully treated with macrolides, irrespective of Helicobacter pylori (H. pylori) infection status. Case 1, an 88-year-old woman who was an H. pylori-positive ITP patient, was treated with clarithromycin (CAM). CAM was effective temporarily. As an alternative to CAM, she was successfully treated with erythromycin (EM) for more than 7 months. Case 2, a 61-year-old man who was an H. pylori-negative ITP patient, was unsuccessfully treated with CAM but successfully treated with EM. Case 3, a 75-year-old woman who was a H. pylori-negative ITP patient, was treated with CAM. CAM was effective temporarily. After approximately 6 months, she was treated with EM for a common cold, and her platelet count increased rapidly. Based on these findings, macrolide treatment was effective for ITP. The effectiveness of macrolides might suggest immunomodulatory effects as well as antibacterial effects for H. pylori.

Hashino, Satoshi

2011-01-01

148

Dexamethasone: Idiopathic Thrombocytopenic Purpura in Children and Adolescents  

PubMed Central

This Hospital Pharmacy feature is extracted from Off-Label Drug Facts, a quarterly publication available from Wolters Kluwer Health. Off-Label Drug Facts is a practitioner-oriented resource for information about specific drug uses that are unapproved by the US Food and Drug Administration. This new guide to the literature enables the health care professional or clinician to quickly identify published studies on off-label uses and determine if a specific use is rational in a patient care scenario. References direct the reader to the full literature for more comprehensive information before patient care decisions are made. Direct questions or comments regarding Off-Label Drug Uses to jgeneral@kumc.edu.

Generali, Joyce A.; Cada, Dennis J.

2013-01-01

149

Dexamethasone: idiopathic thrombocytopenic purpura in children and adolescents.  

PubMed

This Hospital Pharmacy feature is extracted from Off-Label Drug Facts, a quarterly publication available from Wolters Kluwer Health. Off-Label Drug Facts is a practitioner-oriented resource for information about specific drug uses that are unapproved by the US Food and Drug Administration. This new guide to the literature enables the health care professional or clinician to quickly identify published studies on off-label uses and determine if a specific use is rational in a patient care scenario. References direct the reader to the full literature for more comprehensive information before patient care decisions are made. Direct questions or comments regarding Off-Label Drug Uses to jgeneral@kumc.edu. PMID:24421447

Generali, Joyce A; Cada, Dennis J

2013-02-01

150

Ludwig's angina after severe thrombocytopenic purpura associated with dengue fever.  

PubMed

Here, we report a case of Ludwig's angina, which required surgery because of toothache. The patient had dengue and severe thrombocytopenia as confirmed by clinical and laboratory diagnoses. However, dengue is not included among the predisposing factors for Ludwig's angina. PMID:24603747

Campos, Maria Antonia; Junior Prota, Mário Lúcio Cerqueira; Gomes, Carlos Augusto; Amaral, Karina Peisino do; Almeida, Diogo Campos

2014-01-01

151

Mycophenolate mofetil therapy for juvenile dermatomyositis with immune thrombocytopenic purpura  

Microsoft Academic Search

A 6-year-old girl, who had received corticosteroid and cyclosporine on the diagnosis of interstitial pneumonitis related to\\u000a juvenile dermatomyositis, developed severe thrombocytopenia. Her thrombocytopenia was resistant to repeated intravenous immunoglobulin\\u000a administration and methylprednisolone pulse therapy. After additional treatment with mycophenolate mofetil (MMF), instead\\u000a of cyclosporine, the thrombocytopenia improved, facilitating a reduction in the dose of corticosteroid without exacerbation\\u000a of the

Chikara Ogimi; Nazuna Honma; Risa Tanaka; Tsutomu Oh-ishi

152

Pulse Cyclophosphamide Therapy for Refractory Autoimmune Thrombocytopenic Purpura  

Microsoft Academic Search

ceived 1 to 4 doses (mean 2.0) of 1.0 to 1.5 g\\/m2 intravenous cyclophosphamide per course. Of the 20 patients treated with pulse cyclophosphamide therapy, 13 patients (65v0) achieved a complete response (CR), four (20%) a partial re- sponse (PR), and three patients (15%) failed to respond. Of the 13 complete responders, eight have remained in remis- sion with stable

Alex Reiner; Terry Gernsheimer; Sherrill J. Slichter

1995-01-01

153

Thrombotic thrombocytopenic purpura--possibilities of treatment and results.  

PubMed

Results of treatment of 13 patients fulfilling the criteria for TTP are presented. Thrombocytopenia was present in all patients (100%). Eleven of 13 patients (84.6%) had conciousness disorder, and seven of 13 patients (53.8%) had renal impairment. Immunosuppressive therapy with plasmapheresis and replacement of removed volume with fresh frozen plasma in a dosage of 25 ml/kg body weight resulted in statistically significant increase of platelet count (p = 0.0033), and significant improvement of consciousness as defined by increased Glasgow Coma Score (GCS) (p = 0.0524). In two patients, renal function recovered and, in one patient, hemodialysis was no longer needed. This improvement in a small patient group has no statistical significance. PMID:11345402

Gasparovi?, V; Meji?, S; Pisl, Z; Radoni?, R; Radman, I

2001-01-01

154

Postictal hemifacial purpura.  

PubMed

Non-traumatic stereotyped postictal purpura is rare. A 25-year-old woman presented with right facial, cheek and periorbital purpuric eruptions that occurred after secondarily generalized tonic-clonic seizures. The stereotyped, invariably right-sided facial skin eruption, which resolved in 48 hours, falsely raised concerns of spousal abuse. Possible pathophysiological mechanisms include: (a) valsalva-induced capillary hypertension with secondary purpura, (b) ictal corticolimbic stimulation of the autonomic nervous innervation of facial vasomotor structures, and (c) trigeminal-mediated local release of vasoactive substances. Although rare, such stereotyped patterns of purpura should be recognized to avoid incorrect attribution of spousal abuse. PMID:9733412

Reis, J J; Kaplan, P W

1998-08-01

155

Henoch-Schonlein purpura  

MedlinePLUS

Henoch-Schonlein purpura is a disease that involves purple spots on the skin, joint pain, gastrointestinal problems, ... Henoch-Schonlein is caused by an abnormal response of the immune system. It is unclear why this ...

156

Severe Thrombocytopenia in an Immune Thrombocytopenic Parturient Non-responder to Medical Line of Treatment: Anaesthetic Management for Splenectomy Combined with Caesarean Section  

Microsoft Academic Search

We report anaesthesia management of a parturient with severe thrombocytopenia secondary to immune thrombocytopenic purpura\\u000a (ITP). Her platelet count remained around 3 × 109\\/l in spite of optimum medical therapy and hence was posted for splenectomy combined with caesarean section. Anaesthesia implications\\u000a of severe thrombocytopenia comprises risk of central nervous system bleeding, perioperative haemorrhage causing placental\\u000a hypoperfusion and foetal hypoxia, risk of

Vikas Karne; Meenal Patil

157

Fc receptors in immune thrombocytopenias: a target for immunomodulation?  

PubMed Central

In autoimmune disease, Fc receptors (FcRs) form the interface between immune effector cells and their antibody-coated targets, and as such are attractive targets for immunomodulatory therapy. In this issue of the JCI, two highly novel studies of Fc–FcR interactions provide new insights into the role of FcRs in immune thrombocytopenia. Asahi et al. utilized a comprehensive platform of immunological assays to examine the mechanism underlying Helicobacter pylori–associated immune thrombocytopenic purpura, and Ghevaert et al. describe a specially designed antibody that saturates binding sites on fetal platelets without initiating Fc?R-mediated platelet phagocytosis, preventing the binding of pathological maternal anti-HLA antibodies that cause fetomaternal alloimmune thrombocytopenia (see the related articles beginning on pages 2939 and 2929, respectively). These reports illustrate how a remarkably detailed molecular understanding of the FcR network may translate into new therapeutic strategies with high clinical impact.

Psaila, Bethan; Bussel, James B.

2008-01-01

158

Safety and Efficacy Study of Romiplostim (AMG 531) to Treat ITP in Pediatric Subjects  

ClinicalTrials.gov

Idiopathic Thrombocytopenic Purpura; Thrombocytopenia in Pediatric Subjects With Immune (Idiopathic) Thrombocytopenic Purpura (ITP); Thrombocytopenia in Subjects With Immune (Idiopathic) Thrombocytopenic Purpura (ITP)

2011-08-11

159

Nodular purpura in infancy  

PubMed Central

Seventeen infants with nodular purpura are reported. All these infants suffered from a coagulation abnormality due to vitamin K deficiency. Contributing factors were breast-feeding, the use of milk substitutes, diarrhoea and antimicrobial therapy. The patients responded rapidly and completely to parenteral vitamin K. In the management of infantile diarrhoea use of antibiotics should be restricted to specific bacteriologically proven cases. Vitamin K administration is suggested in infants with prolonged gastroenteritis and limited milk intake and particularly those receiving antibiotics. ImagesFig. 1

Nagi, N. A.; Al-Dubooni, H. M.; Al-Shirkat, S. A. R.

1982-01-01

160

Powerlifter's purpura: a valsalva-associated phenomenon.  

PubMed

The causes of purpura can be classified into intravascular, vascular, and extravascular mechanisms. We describe a case of cervicofacial purpura in a powerlifter attributed to the accompanying Valsalva-associated increased arterial pressure. Powerlifting should be added to the list of activities that may cause purpura. PMID:12234164

Pierson, Joseph C; Suh, Philip S

2002-08-01

161

Henoch-Schönlein purpura in children.  

PubMed

Henoch-Schönlein purpura is the most common systemic vasculitis of childhood. In the majority of children, the outcome of Henoch-Schönlein purpura is excellent with spontaneous resolution of symptoms and signs. However, a small subset of patients will develop long-term sequelae in the form of chronic kidney disease. While the clinical presentation and diagnosis of Henoch-Schönlein purpura is straightforward, treatment of Henoch-Schönlein purpura nephritis and long-term renal outcomes of more severely affected children are less certain. This review article gives a general overview of Henoch-Schönlein purpura with emphasis on recently published information, including the new classification of childhood vasculitis, insights into pathogenesis of Henoch-Schönlein purpura and a summary of various treatments of established Henoch-Schönlein purpura nephritis. PMID:24134307

Trnka, Peter

2013-12-01

162

Purpura-associated congenital lymphedema.  

PubMed

An 8-year-old girl referred to our Department for a two-month worsening of congenital primary lymphedema of the lower limb and for the appearance of several purpuric lesions on the right thigh and knee. We diagnosed a lichenoid pigmented purpura of Gougerot and Blum in a patient with Milroy disease, complicated by an insufficiency of anterior saphena. We treated the patient with topical steroids and compression stockings, until surgical intervention of phlebectomy. We report this case for the rarity of the disease, for the even more rare association with lichenoid pigmented purpura and for cutaneous immunopathological findings. PMID:19620038

Berti, Samantha; Pieri, Alessandro; Lotti, Torello; Duranti, Alberto; Panelos, John; De Martino, Maurizio; Moretti, Silvia

2009-01-01

163

The B-cell compartment as the selective target for the treatment of immune thrombocytopenias  

Microsoft Academic Search

utoimmune thrombocytopenias represent a diverse group of diseases associated with the production of antibodies reacting against platelet antigens. They may develop as isolated thrombocytopenias, of unknown etiopathogenesis, and in that case they are called idio- pathic thrombocytopenic purpura (ITP). In other cases, autoimmune thrombocytopenias develop during a lym- phoproliferative disorder, more frequently chronic lym- phocytic leukemia (CLL), or a systemic

FRANCESCO ZAJA; NICOLA VIANELLI; ALESSANDRA SPEROTTO; SALVATORE DE VITA; ISABELLA IACONA; ALFONSO ZACCARIA; PAOLA MASOLINI; VALENTINA TOMADINI; MONICA TANI; ANNA LIA MOLINARI; MICHELE BACCARANI; RENATO FANIN

164

Capnocytophaga canimorsus infection presenting with complete splenic infarction and thrombotic thrombocytopenic purpura: a case report  

PubMed Central

Background Animal bites are typically harmless, but in rare cases infections introduced by such bites can be fatal. Capnocytophaga canimorsus, found in the normal oral flora of dogs, has the potential to cause conditions ranging from minor cellulitis to fatal sepsis. The tendency of C. canimorsus infections to present with varied symptoms, the organism’s fastidious nature, and difficulty of culturing make this a challenging diagnosis. Rarely, bacterial cytotoxins such as those produced by C. canimorsus may act as causative agents of TTP, further complicating the diagnosis. Early recognition is crucial for survival, and the variability of presentation must be appreciated. We present the first known case of C. canimorsus infection resulting in TTP that initially presented as splenic infarction. Case presentation 72-year-old Caucasian male presented with a four-day history of abdominal pain, nausea, vomiting, diarrhea, and intermittent confusion. On presentation, vital signs were stable and the patient was afebrile. Physical examination was unremarkable apart from petechiae on the inner left thigh, and extreme diffuse abdominal pain to palpation and percussion along with positive rebound tenderness. Initial investigations revealed leukocytosis with left shift and thrombocytopenia, but normal liver enzymes, cardiac enzymes, lipase, INR and PTT. Abdominal CT demonstrated a non-enhancing spleen and hemoperitoneum, suggesting complete splenic infarction. Although the patient remained afebrile, he continued deteriorating over the next two days with worsening thrombocytopenia. After becoming febrile, he developed microangiopathic hemolytic anemia and hemodynamic instability, and soon after was intubated due to hypoxic respiratory failure and decreased consciousness. Plasma exchange was initiated but subsequently stopped when positive blood cultures grew a gram-negative organism. The patient progressively improved following therapy with piperacillin-tazobactam, which was switched to imipenem, then meropenem when Capnocytophaga was identified. Conclusions There is a common misconception amongst practitioners that the presence of systemic infection excludes the possibility of TTP and vice versa. This case emphasizes that TTP may occur secondary to a systemic infection, thereby allowing the two processes to coexist. It is important to maintain a wide differential when considering the diagnosis of either TTP or C. canimorsus infection since delays in treatment may have fatal consequences.

2012-01-01

165

Purpura of the Face and Neck: An Atypical Clinical Presentation Revealing a Hepatosplenic T Cell Lymphoma  

PubMed Central

Background Hepatosplenic T cell lymphoma (HSTL) is a rare but very aggressive peripheral T cell lymphoma whose initial silent clinical presentation unfortunately delays the diagnosis and worsens the prognosis of patient survival. Efforts should be aimed at early recognition and treatment. Methods We describe a case of a 62-year-old woman who presented at our clinic with a non-palpable purpuric eruption of the face. Investigations revealed thrombocytopenia with hepatosplenomegaly, which showed rapid progression together with accentuation of the purpura. Two months later, a bone marrow biopsy revealed the diagnosis of a HSTL. Results The patient received six cycles of CHOP chemotherapy (vincristine, cyclophosphamide, doxorubicin, methylprednisolone) followed by a well-tolerated autologous bone marrow graft. Normalization of the platelet count resulted in regression of the purpuric rash. Conclusion To our knowledge, this is the first report of a facial thrombocytopenic purpura as the inaugural symptom of HSTL. It emphasizes the privileged position of the dermatologist for early recognition of potentially lethal HSTL.

Kuonen, Francois; Bucher, Maya; de Leval, Laurence; Vernez, Maxime; Gilliet, Michel; Conrad, Curdin; Feldmeyer, Laurence

2014-01-01

166

[Henoch-Schönlein recurrent purpura: case report].  

PubMed

A 15-year old patient presented several episodes of uncontrollable vomiting with abdominal pain caused by a recurrent Henoch-Schönlein purpura. Renal impairment was demonstrated through the achievement of urinary test strips. Henoch-Schönlein purpura is the most common vasculitis in children. The clinical picture may include palpable purpura despite normal coagulation, arthralgia, abdominal pain and some degree of renal impairment. Most often, symptomatic treatment is effective. In some severe cases, corticosteroids are required. A control of hematuria and measure of blood pressure are recommended during the 6 to 12 months following the initial episode. PMID:24683839

Abomake, Maka M; Laho, D

2014-01-01

167

Henoch-Schönlein purpura with ileitis terminalis  

Microsoft Academic Search

Gastrointestinal manifestations of Henoch-Schönlein purpura commonly include abdominal pain and gastrointestinal bleeding as well as extraintestinal signs and symptoms. We report here on a 24-year-old man with gastrointestinal pain in whom the classical features of Henoch-Schönlein purpura appeared only 6 days after acute abdominal symptoms. At endoscopic investigation inflammation with aphthous lesions was detected at the terminal ileum, which is

W. A. Scherbaum; R. Kaufmann; U. Vogel; G. Adler

1993-01-01

168

Gastrointestinal manifestations of Henoch-Schonlein Purpura.  

PubMed

Henoch-Schonlein Purpura (HSP) is the most common systemic vasculitis in childhood. The diagnostic criteria include palpable purpura with at least one other manifestation -- abdominal pain, IgA deposition, arthritis or arthralgia, or renal involvement. Immune complex deposits result in necrosis of the wall of small- and medium-sized arteries with infiltration of tissue by neutrophils and deposition of nuclear fragments, a process called leukocytoclastic vasculitis (LCV). It is often associated with infections, medications, or tumors. It may coexist with or mimic Crohn's disease. Periumbilical and epigastric pain worsens with meals, from bowel angina. Bleeding is usually occult or, less commonly, associated with melena. Intussusception, the most common surgical complication, is usually ileo-ileo or ileo-colic. Perforations, usually ileal, may occur spontaneously or be associated with intussusception. Ultrasound, recommended as the first diagnostic test, and CT scans may show intussusception and asymmetric bowel wall thickening mainly involving the jejunum and ileum. There are a range of endoscopic findings including gastritis, duodenitis, ulceration, and purpura, with the second portion of the duodenum characteristically being involved more than the bulb. Intestinal biopsies show IgA deposition and LCV in the submucosal vessels. Superficial biopsies may show inflammation, ulceration, edema, hemorrhage, and vascular congestion, presumably due to vasculitis-induced mucosal ischemia. The efficacy of corticosteroids in preventing severe complications or relapses is controversial. The majority of patients, however, improve spontaneously. PMID:18351468

Ebert, Ellen C

2008-08-01

169

Henoch Sch?nlein purpura associated with pulmonary adenocarcinoma  

PubMed Central

Introduction Henoch-Schönlein purpura is a common immunoglobulin A-mediated vasculitis syndrome in children. Henoch-Schönlein purpura can also affect adults and is probably related to malignancy. Case presentation We report the case of a 61-year-old Japanese man who presented for examination after an abnormal shadow was detected by chest radiography. He received a diagnosis of pulmonary adenocarcinoma, stage IV. Purpura on the legs, abdominal pain, diarrhea, hematuria and proteinuria developed at this time. Henoch-Schönlein purpura was diagnosed, base on the clinical symptoms and histological findings of biopsy specimens of the skin, which showed vasculitis with immunoglobulin A deposits. Our patient received chemotherapy with gemcitabine after successful steroid therapy for the Henoch-Schönlein purpura. Conclusion Although hematological malignancies are well-known causes of vasculitides, cases of Henoch-Schönlein purpura associated with lung adenocarcinoma are rare. Our patient was treated with corticosteroid therapy, which cleared the purpura and cytotoxic chemotherapy for the non-small cell lung cancer. However, he died from heart failure due to cardiac tamponade.

2011-01-01

170

Idiopathic Seidlmayer's Purpura: A Case Report  

PubMed Central

Acute hemorrhagic edema of infancy (AHEI) was considered a rare form of Henoch-Schönlein purpura; however, it is now regarded as an independent disease typically involving patients aged 4–24 months. The authors describe the clinical case of a toddler aged 8 months, with skin erythematous pomphoid lesions, treated at home with topical steroids without benefits. The appearance of new lesions and the worsening of the previous skin signs induced the parents to drive the child to the hospital. The medical history revealed the administration of a vaccine dose 2 months before.

Maggio, Maria Cristina; Ferraro, Fabrizia; Ragusa, Saveria Sabrina; Corpora, Umberto; Corsello, Giovanni

2014-01-01

171

Idiopathic Seidlmayer's Purpura: A Case Report.  

PubMed

Acute hemorrhagic edema of infancy (AHEI) was considered a rare form of Henoch-Schönlein purpura; however, it is now regarded as an independent disease typically involving patients aged 4-24 months. The authors describe the clinical case of a toddler aged 8 months, with skin erythematous pomphoid lesions, treated at home with topical steroids without benefits. The appearance of new lesions and the worsening of the previous skin signs induced the parents to drive the child to the hospital. The medical history revealed the administration of a vaccine dose 2 months before. PMID:24932170

Maggio, Maria Cristina; Ferraro, Fabrizia; Ragusa, Saveria Sabrina; Corpora, Umberto; Corsello, Giovanni

2014-05-01

172

Pathogenesis of Henoch-Schönlein purpura nephritis.  

PubMed

The severity of renal involvement is the major factor determining the long-term outcome of children with Henoch-Schönlein purpura (HSP) nephritis (HSPN). Approximately 40% children with HSP develop nephritis, usually within 4 to 6 weeks after the initial onset of the typical purpuric rashes. Although the pathogenetic mechanisms are still not fully delineated, several studies suggest that galactose-deficient IgA1 (Gd-IgA1) is recognized by anti-glycan antibodies, leading to the formation of the circulating immune complexes and their mesangial deposition that induce renal injury in HSPN. PMID:19526254

Lau, Keith K; Suzuki, Hitoshi; Novak, Jan; Wyatt, Robert J

2010-01-01

173

Neonatal purpura fulminans secondary to group B streptococcal infection.  

PubMed

Purpura fulminans is an acute and frequently fatal disorder characterized by sudden onset of progressive cutaneous hemorrhage and necrosis due to dermal vascular thrombosis and disseminated intravascular coagulation. The authors present a neonate with extensive purpura fulminans due to group B streptoccoccal septicemia and evaluated the attributable clinical mortality and morbidity of this potentially lethal syndrome. Clinicians especially neonatologists should be aware that early-onset sepsis of group B Streptococcus in the newborn infant with purpura fulminans could be a cause of maternal carriage due to colonization of this pathogen microorganism. PMID:20795772

Zenciroglu, Aysegul; Karagol, Belma Saygili; Ipek, Mehmet Sah; Okumus, Nurullah; Yarali, Nese; Aydin, Mustafa

2010-11-01

174

Henoch-Schonlein purpura presenting sequentially as nodular rash, erythema nodosum, and palpable purpura.  

PubMed

We describe a 26-year-old woman who presented with a nodular rash on the elbows following an insect bite. Two days later, she developed erythema nodosum. Both these lesions were treated symptomatically. One week later, she had purpura, abdominal pain, hematuria, and arthralgias, following which steroids were administered. Her investigations revealed only microscopic hematuria that disappeared with therapy. This pattern of sequential appearance of rash and a nodular morphology are both unique features not previously reported. PMID:24696039

Balamurugesan, Kandan; Viswanathan, Stalin

2014-01-01

175

Association of Mycoplasma pneumoniae infection with Henoch-Schonlein purpura.  

PubMed

We present a child with Henoch-Schonlein purpura and Mycoplasma pneumoniae infection, an association that was rarely described in the literature. The infection was confirmed serologically and by using PCR. PMID:24093818

Yiallouros, P; Moustaki, M; Voutsioti, A; Sharifi, F; Karpathios, T

2013-01-01

176

Encephalopathy Complicating Henoch-Schönlein Purpura: Reversible MRI Changes  

Microsoft Academic Search

A 10-year-old boy with Henoch-Schönlein purpura complicated by encephalopathy, transient cortical blindness, and a secondary generalized seizure is reported. Reversible changes in the posterior white and gray matter were seen on magnetic resonance imaging. Our patient illustrates uncommon neurologic manifestations of Henoch-Schönlein purpura. The nature and location of the lesions and the normalization of the patient’s magnetic resonance imaging is

Andrew R Woolfenden; Juliette Hukin; Ken J Poskitt; Mary B Connolly

1998-01-01

177

[Localized purpura revealing vascular prosthetic graft infection].  

PubMed

Prosthetic graft infection after vascular reconstruction is a rare but serious complication. We report a case of infection occurring late after implantation of an iliofemoral prosthetic vascular graft. The Staphylococcus aureus infection was revealed by vascular purpura localized on the right leg 7 years after implantation of a vascular prosthesis. This case illustrates an uncommonly late clinical manifestation presenting as an acute infection 7 years after the primary operation. In this situation, the presentation differs from early infection, which generally occurs within the first four postoperative months. Diagnosis and treatment remain a difficult challenge because prosthetic graft infection is a potentially life-threatening complication. Morbidity and mortality rates are high. Here we detail specific aspects of the clinical and radiological presentation. PMID:23746928

Boureau, A S; Lescalie, F; Cassagnau, E; Clairand, R; Connault, J

2013-07-01

178

TWO CASES OF SARCOMATOSIS WITH PURPURA HAEMORRHAGICA  

PubMed Central

The purpura accompanying the two foregoing cases of sarcoimatosis would seem to find its explanation in the coexistence of several factors, the main feature being an involvement of the vascular system by the sarcomatous elements. There existed in Case I a direct lesion of the vessel wall whereby the sarcoma cells invaded directly the various coats, and were found mainly between the intima and the adventitia, dissecting their way, as it were, along these tracts in the vessel walls. There was further an extensive involvement of the perivascular lymphatics, from which point, indeed, it would seem that the sarcoma cells had invaded the walls of the vessels themselves. In Case II, moreover, not only was there a definite invasion of the lymph spaces near the vessels, but, furthermore, there was undoubted evidence of the existence of emboli of sarcoma cells in the lumina of the blood vessels; and in the immediate vicinity of such conditions hæmorrhages were invariably found. While some vessels, and indeed a great many, were quite free from such emboli, in others the lumina were completely occluded by spindle cells, so as to preclude the possibility that these were merely a collection of desquamated endothelial cells, such as is frequently found as the result of post-mortem changes. That such an embolic condition can exist is by no means an unreasonable supposition, and, while it is generally recognised that multiple sarcomata are usually made up of small round cells, in this case we have an undoubted example of sarcomatosis of the spindle-celled variety. There are numerous instances of this " embolic purpura," as it may be called, especially in French and German literature, the condition being associated with rheumatism, valvular lesions of the heart, and other diseases which induce directly or indirectly the formation of emboli. Krauss, Gimard, Leloir, and others have insisted with considerable emphasis on the embolic origin of many purpuric conditions, and in some instances they have verified their observations by histological examination. Leloir assumes that, in addition to the presence of the ordinary emboli and the changes in the vessel walls with desquamative endarteritis, the blood itself may be much altered chemically, and that in the cachectic conditions clots may be thrown down from the circulating blood and be carried onward to form capillary emboli, with resulting hæmorrhagic infarctions. Krogerer, some ten years ago, in examining the skin removed from patients with symptomatic purpura, found definite thromboses in the smaller veins, and even in the arteries. According to his view, the alterations in the vessel walls gave rise to slowed circulation and tendency to thrombosis, bringing about a liability to hæmorrhages. His plates bear out his theories regarding the thrombi, many of which show considerable organization. But a careful examination of the purpuric areas shows further that a mere invasion of the vascular system by sarcoma cells can not explain all the various blood effusions present. On examining the skin, for instance, in those areas where large irregular hæmorrhages had occurred, there was but little evidence of vascular invasion, while the emboli, on the other hand, seemed to exist mainly in the localized smaller and more circumscribed patches. One must therefore conclude that in such instances a combination of factors will alone afford a rational explanation of the purpura, and that in the general condition of the patient we shall find another cause for the enormous effusions of blood. In both of our cases there were high fever, cachexia, and a rapid progressive asthenia, all being the results of a sarcomatosis, and implying also grave alterations in the composition of the blood. From this we may infer an altered condition of the vessel walls, and hence probably a combination of circumstances sufficient to explain the incidence of hæmorrhage. The raised cutaneous nodules in our second case, some of which were hæmorrhagic, can not be regarded as pure sarcomatous metastases

Martin, C. F.; Hamilton, W. F.

1896-01-01

179

Identification of differentially expressed serum proteins in infectious purpura fulminans.  

PubMed

Purpura fulminans (PF) is a life-threatening hemorrhagic condition. Because of the rarity and randomness of the disease, no improvement in treatment has been made for a long time. In this study, we assessed the serum proteome response to PF by comparing serum proteins between healthy controls and PF patient. Liquid chromatography with tandem mass spectrometry (LC-MS/MS) approach was used after depleting 6 abundant proteins of serum. In total, 262 proteins were confidently identified with 2 unique peptides, and 38 proteins were identified significantly up- (? 2) or downregulated (? 0.5) based on spectral counting ratios (SpCPF/N). In the 38 proteins with significant abundance changes, 11 proteins were previously known to be associated with burn or sepsis response, but 27 potentially novel proteins may be specifically associated with PF process. Two differentially expressed proteins, alpha-1-antitrypsin (SERPINA1) and alpha-2 antiplasmin (SERPINF2), were validated by Western blot. This is the first study where PF patient and healthy controls are compared in a proteomic study to elucidate proteins involved in the response to PF. This study provides an initial basis for future studies of PF, and the differentially expressed proteins might provide new therapeutic targets to decrease the mortality of PF. PMID:24659849

He, Ting; Hu, Jiong-yu; Han, Jian; Zhang, Dong-xia; Jiang, Xu-pin; Chen, Bing; Huang, Yue-sheng

2014-01-01

180

Neurologic manifestations of Henoch-Schönlein purpura.  

PubMed

Henoch-Shönlein purpura (HSP) is a systemic small vessel vasculitis. Most patients present during childhood. The characteristic association of purpura, arthralgia, abdominal pain, and nephritis reflects the predominant distribution of vasculitis. Headaches and mild behavioral changes suggest CNS involvement in one-third of HSP patients. Salient central nervous system (CNS) manifestations are rarer and mostly reported in adults and patients with a severe disease course. Diagnosis of CNS vasculitis is rarely confirmed by histopathology and generally relies on "suggestive" imaging showing brain hemorrhages, infarcts and edema, predominantly located in the parieto-occipital regions. Vessel wall friability and thrombogenicity of active vasculitis, antiphospholipid antibody synthesis, and other hemostatic disturbances may contribute to hemorrhagic and thrombotic complications of HSP. Posterior reversible encephalopathy syndrome and hypertensive encephalopathy occur in HSP and can be difficult to differentiate from CNS vasculitis. Some 53% of patients with neurologic complications experience seizures. Cerebral venous thrombosis, subdural hematoma, subarachnoidal hemorrhage, neuro-ophthalmologic complications, myelopathy, and diverse neuromuscular manifestations are also reported. In contrast with other systemic small vessel vasculitides, peripheral nervous system involvement is infrequent in HSP. Systemic involvement of HSP and homeostatic disorders such as hypertension, uremia, and electrolyte disturbances, as well as superimposed infections can affect the nervous system secondarily. Identification of nervous system complications of HSP is often challenging due to prominent systemic manifestations. HSP is usually a self-limiting disease that requires only supportive care. Patients with CNS vasculitis are commonly treated with corticosteroids. One-fifth of patients with CNS involvement remain with sequelae. PMID:24365374

Bérubé, Maxime D; Blais, Normand; Lanthier, Sylvain

2014-01-01

181

[Henoch-Schönlein purpura in the adult].  

PubMed

Henoch-Schönlein purpura is a systemic vasculitis of the small vessels characterized by perivascular leucocyte infiltrates. It is an immunoglobulin A-related immune complex-mediated disease involving the skin, the joints and the gastrointestinal system. Renal disease may sometimes be associated to these clinical manifestations. Prevalence of the nephritis is highly variable, depending on the series. More rarely, other organs such as the lungs, the heart or the nervous system may be involved. The clinical diagnosis is confirmed by histopathology of the skin (leukocytoclastic vasculitis) and kidney (endo-capillary proliferative glomerulonephritis), showing IgA deposits in these tissues. Short-term prognosis depends on the severity of digestive involvement, but long-term prognosis depends on the renal disease. Recent publications of pediatric and adult series show that the chronic renal failure may progress, sometimes more than ten years after the initial flare. Treatment is usually supportive. The benefit of more specific treatments (corticosteroids or immunosuppressive drugs) in severe visceral forms (usually abdominal or kidney) has not yet been established. PMID:24657040

Pillebout, E; Verine, J

2014-06-01

182

[Massive colorectal hemorrhage in adult Schönlein-Henoch purpura].  

PubMed

Schönlein-Henoch purpura is a well known syndrome, rare in adults, and is seldom accompanied with extensive colorectal haemorrhage. In abdominal purpura, the small bowel is more frequently involved, but cases of oesophageal, gastroduodenal and colorectal localisation also have been reported. The cases with colorectal bleeding, appearing in adulthood and elderly patients, could cause differential diagnostic problems. Described endoscopic features of one patient, who was referred to our department, because of colorectal bleeding. The treatment, course of disease and endoscopic follow-up is presented. No previous endoscopic documentation of this type of localisation has been reported in Hungarian literature, so far. PMID:8351133

Novák, J; Libor, J

1993-07-01

183

Leptin levels in Henoch-Schönlein purpura.  

PubMed

The objective of this paper is to assess the possible role of nitric oxide (NO) and leptin in Henoch-Schönlein purpura (HSP). We investigated the serum leptin and total nitrite levels in 22 children with HSP in the acute phase and after remission and in 20 age- and sex-matched healthy control. Serum leptin levels (nanograms per milliliter; median, min-max) were statistically higher in the acute phase (12.9, 9.1-19.5) than those in the remission phase (6.1, 3.7-10.5, p<0.001) and in the control group (4.9, 3.8-7.5, p<0.001). Also, serum nitrite levels (micromole per liter; median, min-max) were higher in children in the acute phase (45.0, 32.0-60.0) compared to those in remission phase (30.5, 23.0-48.0) and in the control group (29.5, 18.0-38.0) (p<0.001, p<0.001, respectively). There was a positive correlation between serum leptin and total nitrite levels (r=0.65, p<0.001). We have demonstrated that serum leptin and NO levels were increased during the acute phase in children with HSP, and returned to normal levels in remission. We suggest that leptin and NO may have a role in the immunoinflammatory process of HSP, especially in the acute phase. Further studies are needed to clearly establish the roles of leptin and NO in the pathogenesis of HSP. PMID:16816886

Aliyazicioglu, Yuksel; Ozkaya, Ozan; Yakut, Husamettin; Islek, Ismail; Alvur, Muhlise

2007-03-01

184

Henoch-Schönlein purpura: a case with atypical presentation  

Microsoft Academic Search

We report on a case of Henoch-Schönlein purpura (HSP) with pulmonary hemorrhage and severe renal involvement. The patient\\u000a also had active carditis related to acute rheumatic fever. He died despite intensive treatment. Regarding this case, we discuss\\u000a the pathogenesis and clinical findings of pulmonary hemorrhage and active carditis in HSP.

Mukaddes Kalyoncu; Murat Cakir; Erol Erduran; Aysenur Okten

2006-01-01

185

[Randomized comparison of intravenous immunoglobulin and methylprednisolone pulse therapy in children with newly diagnosed idiopathic thrombocytic purpura. The Danish ITP Study Group].  

PubMed

Forty three children with newly diagnosed idiopathic thrombocytopenic purpura (ITP), platelet count (pl.c.) below 20 x 10(9)/l, and either clinically significant bleeding or failure to show a spontaneous platelet rise within three days of admission were randomly allocated to treatment with intravenous infusions of either immunoglobulin (IVIG) 1 g/kg or methylprednisolone (MPPT) 30 mg/kg on two consecutive days. Prompt induction of partial remission with pl.c. > 50 x 10(9)/l after 72 hours was seen in 21/23 given IVIG versus 10/20 given MPPT (exact p = 0.003); mean pl.c.s after 72 hours were 188 versus 77 x 10(9)/l (2p < 0.001). Poor responders were then given the alternative infusions in addition. After six days, complete remission with pl.c. > 150 x 10(9)/l was achieved in 16/23 versus 10/20 (p = 0.16). During six months follow-up, there were no significant differences regarding relapse rates or chronic course. Eleven children with relapse were crossed over to the alternative treatment arm: the estimated treatment effect in pl.c. after 72 hours was 134 x 10(9)/l in favour of IVIG. These results indicate that IVIG infusions may be preferable to high-dose corticosteroids as initial treatment for children with ITP. PMID:9522658

Rosthøj, S; Nielsen, S M; Pedersen, F K

1998-03-01

186

Vasculitic purpura in vinyl chloride disease: a case report  

SciTech Connect

Vinyl chloride (VC), a volatile substance mostly used for polyvinyl chloride (PVC) synthesis, is a systemic toxicant particularly noxious to endothelium. Angiosarcoma of the liver, Raynaud's phenomenon, scleroderma-like lesions, acroosteolysis and neuritis are known to be typical vinyl chloride-associated manifestations (VC disease). A so far unknown feature of the disease is purpura. This was first observed by the authors in a worker of a PVC-producing plant. The skin eruption was characterized by small purpuric maculae with tiny, palpable spots and papulae, mostly concentrated on the lower part of the legs, changing into bullae, pustules and crusts and tending to spontaneous regression after withdrawal from VC exposure. A skin biopsy revealed marked inflammatory reaction with a mostly lymphocytic and histiocytic infiltration around and in the walls of dermal arterioles. The finding of increased circulating immune complexes and anti-smooth muscle autoantibodies strengthens the hypothesis that immunologic changes play a role in the appearance of ''vinylic purpura.''

Magnavita, N.; Bergamaschi, A.; Garcovich, A.; Giuliano, G.

1986-05-01

187

Bilateral brachial plexopathy complicating Henoch–Schönlein purpura  

Microsoft Academic Search

An 11-year-old boy presented with convulsion, fever, rash, abdominal pain, swelling on the eyelids, elbow and wrists, oliguria and hematuria. Based on the abnormal findings the patient was diagnosed with Henoch-Schönlein purpura. On the 3rd day of admission, neurological examination showed ataxic gait, loss of deep tendon reflexes, and decreased (4\\/5) of muscle strength on all extremities. Additionally, bilateral loss

Cahide Y?lmaz; Hüseyin Çaksen; ?ükrü Arslan; Ömer Anlar; Bülent Ata?; Ahmet Sami Güven; Dursun Odaba?

2006-01-01

188

Meningococcal purpura fulminans in children: I. Initial orthopedic management  

PubMed Central

Background Purpura fulminans is a rare and extremely severe infection, mostly due to Neisseria meningitidis frequently causing early orthopedic lesions. Few studies have reported on the initial surgical management of acute purpura fulminans. The aim of this study is to look at the predictive factors in orthopedic outcome in light of the initial surgical management in children surviving initial resuscitation. Methods Nineteen patients referred to our institution between 1987 and 2005 were taken care of at the very beginning of the purpura fulminans. All cases were retrospectively reviewed so as to collect information on the total skin necrosis, vascular insufficiency, gangrene, and total duration of vasopressive treatment. Results All patients had multiorgan failure; only one never developed any skin necrosis or ischemia. Eighteen patients lost tissue, leading to 22 skin grafts, including two total skin grafts. There was only one graft failure. Thirteen patients were concerned by an amputation, representing, in total, 54 fingers, 36 toes, two transmetatarsal, and ten transtibial below-knee amputations, with a mean delay of 4 weeks after onset of the disease. Necrosis seems to affect mainly the lower limbs, but there is no predictive factor that impacted on the orthopedic outcome. We did not perform any fasciotomy or compartment pressure measurement to avoid non-perfusion worsening; nonetheless, our outcome in this series is comparable to existing series in the literature. V.A.C.® therapy could be promising regarding the management of skin necrosis in this particular context. While suffering from general multiorgan failure, great care should be observed not to miss any additional osseous or articular infection, as some patients also develop local osteitis and osteomyelitis that are often not diagnosed. Conclusions We do not advocate very early surgery during the acute phase of purpura fulminans, as it does not change the orthopedic outcome in these children. By performing amputations and skin coverage some time after the acute phase, we obtained similar results to those found in the literature.

Mezel, A.; Raux, S.; Fron, D.; Maillet, M.; Herbaux, B.

2010-01-01

189

Purpura and dermal thinning associated with high dose inhaled corticosteroids.  

PubMed Central

OBJECTIVE--To assess the effect of high dose inhaled corticosteroids on skin. DESIGN--Cross sectional study of patients receiving treatment for chest diseases. SETTING--Outpatient chest clinic in a teaching hospital. PATIENTS--68 Patients divided into four groups of similar age--namely, 15 receiving long term oral prednisolone, 21 receiving high dose inhaled corticosteroids, 15 receiving low dose inhaled corticosteroids, and 17 controls. MAIN OUTCOME MEASURES--Skin thickness at three sites measured by A scan ultrasound and clinical assessment of purpura. RESULTS--Compared with controls patients in both the oral prednisolone treated group and the high dose inhaled corticosteroid treated group had significantly thinner skin at all three sites (group median thicknesses: prednisolone treated group 28-33% less than controls; high dose inhaled corticosteroid treated group 15-19% less than controls). Differences in skin thicknesses between the low dose inhaled corticosteroid treated group and the controls were trivial. The prevalence of purpura was significantly greater in patients receiving oral prednisolone (12/15 patients) and high dose inhaled corticosteroids (10/21) than in controls (2/17). CONCLUSION--Skin thinning and purpura represent further evidence of systemic effects of high dose inhaled corticosteroids.

Capewell, S; Reynolds, S; Shuttleworth, D; Edwards, C; Finlay, A Y

1990-01-01

190

Vascular purpura revealing a severe dilated cardiomyopathy with left ventricular apical thrombus  

PubMed Central

We present a case of vascular purpura revealing an intra-cardiac left-sided thrombus complicating an end-stage dilated cardiomyopathy. Vascular purpura main etiologies encompass the wide specturm of vasculitides and microvascular-occlusion syndromes. Among them, cardiac embolism represents an unusal but potentially severe etology.

Seemann, Aurelien; De Prost, Nicolas; Paoletti, Marie-Therese; Sbidian, Emilie; Brun-Buisson, Christian; Valeyrie-Allanore, Laurence

2012-01-01

191

/sup 111/In-oxine platelet survivals in thrombocytopenic infants  

SciTech Connect

Thrombocytopenia is a common occurrence (20%) in sick neonates, but the causes have not been well studied. In this report we demonstrate that thrombocytopenia in the neonate is characterized by increased platelet destruction as shown by shortened homologous /sup 111/In-oxine-labeled platelet life spans. Thirty-one prospectively studied thrombocytopenic neonates were investigated by measuring the /sup 111/In-labeled platelet life span, platelet-associated IgG (PAIgG), and coagulation screening tests. In every infant, the thrombocytopenia was shown to have a destructive component since the mean platelet life span was significantly shortened to 65 +/- 6 (mean +/- SEM) hours with a range of one to 128 hours compared with adult values (212 +/- 8; range, 140 to 260; gamma function analysis). The platelet survival was directly related to the lowest platelet count and inversely related to both the highest mean platelet volume and duration of the thrombocytopenia. In 22 infants the percent recovery of the radiolabeled platelets was less than 50%, which suggested that increased sequestration also contributed to the thrombocytopenia. Infants with laboratory evidence of disseminated intravascular coagulation (n = 8) or immune platelet destruction evidenced by elevated levels of PAIgG (n = 13) had even shorter platelet survivals and a more severe thrombocytopenia compared with the ten infants in whom an underlying cause for the thrombocytopenia was not apparent. Full-body scintigraphic images obtained in 11 infants showed an increased uptake in the spleen and liver, with a spleen-to-liver ratio of 3:1. This study indicates that thrombocytopenia in sick neonates is primarily destructive, with a subgroup having evidence of increased platelet sequestration.

Castle, V.; Coates, G.; Kelton, J.G.; Andrew, M.

1987-09-01

192

An overview of platelet indices and methods for evaluating platelet function in thrombocytopenic patients.  

PubMed

Thrombocytopenia is associated with bleeding risk. However, in thrombocytopenic patients, platelet count does not correlate with bleeding risk and other factors are thus likely to contribute to this risk. This review presents currently available platelet-related markers available on automated haematology analysers and commonly used methods for testing platelet function. The test principles, advantages and disadvantages of each test are described. We also evaluate the current literature regarding the clinical utility of the test for prediction of bleeding in thrombocytopenia in haematological and oncological diseases. We find that several platelet-related markers are available, but information about the clinical utility in thrombocytopenia is limited. Studies support that mean platelet volume (MPV) can aid diagnosing the cause of thrombocytopenia and low MPV may be associated with bleeding in thrombocytopenia. Flow cytometry, platelet aggregometry and platelet secretion tests are used to diagnose specific platelet function defects. The flow cytometric activation marker P-selectin and surface coverage by the Cone-and-Plate[let] analyser predict bleeding in selected thrombocytopenic populations. To fully uncover the clinical utility of platelet-related tests, information about the prevalence of platelet function defects in thrombocytopenic conditions is required. Finally, knowledge of the performance in thrombocytopenic samples from patients is essential. PMID:24400878

Vinholt, Pernille J; Hvas, Anne-Mette; Nybo, Mads

2014-05-01

193

Intracellular signaling as a potential target for antiplatelet therapy.  

PubMed

Three classes of inhibitors of platelet aggregation have demonstrated substantial clinical benfits. Aspirin acts by irreversibly inhibiting COX-1 and therefore blocking the synthesis of proaggregatory thromboxane A (2) (TxA(2)). The indirect acting (ticlopidine, clopidogrel, prasugrel) and the direct acting (ticagrelor) antagonists of P2Y(12) block the thrombus stabilizing activity of ADP. Parenteral GP IIb-IIIa inhibitors directly block platelet-platelet interactions. Despite well-established benefits, all antiplatelet agents have important limitations: increased bleeding and gastrointestinal toxicities (aspirin), high incidence of thrombotic thrombocytopenic purpura (ticlopidine), potentially nonresponders (clopidogrel), severe bleeding (prasugrel, GP IIb-IIIa antagonists) and "complicated" relationships with aspirin ticagrelor). In this chapter, we present the genetic and pharmacological evidence that supports the development and expectations associated with novel antiplatelet strategies directed at intrasignaling pathways. PMID:22918738

Andre, Patrick

2012-01-01

194

Childhood Immune Thrombocytopenia: Long-term Follow-up Data Evaluated by the Criteria of the International Working Group on Immune Thrombocytopenic Purpura.  

PubMed

Objective: Immune thrombocytopenia (ITP) is a common bleeding disorder in childhood, characterized by isolated thrombocytopenia. The International Working Group (IWG) on ITP recently published a consensus report about the standardization of terminology, definitions, and outcome criteria in ITP to overcome the difficulties in these areas. Materials and Methods: The records of patients were retrospectively collected from January 2000 to December 2009 to evaluate the data of children with ITP by using the new definitions of the IWG. Results: The data of 201 children were included in the study. The median follow-up period was 22 months (range: 12-131 months). The median age and platelet count at presentation were 69 months (range: 7-208 months) and 19x10(9)/L (range: 1x10(9)/L to 93x10(9)/L), respectively. We found 2 risk factors for chronic course of ITP: female sex (OR=2.55, CI=1.31-4.95) and age being more than 10 years (OR=3.0, CI=1.5-5.98). Life-threatening bleeding occurred in 5% (n=9) of the patients. Splenectomy was required in 7 (3%) cases. When we excluded 2 splenectomized cases, complete remission at 1 year was achieved in 70% (n=139/199). The disease was resolved in 9 more children between 12 and 90 months. Conclusion: Female sex and age above 10 years old significantly influenced chronicity. Therefore, long-term follow-up is necessary in these children. PMID:24764727

Evim, Melike Sezgin; Baytan, Birol; Güne?, Adalet Meral

2014-03-01

195

Post-Renal Transplant Thrombotic Thrombocytopenic Purpura (TTP): Attributable to Immunosuppression or Graft Rejection: Report of Three Cases and Literature Review.  

National Technical Information Service (NTIS)

The immunomodulatory drug FK-506 (Tacrolimus) is a calcineurin inhibitor that, as such, indirectly inhibits the transcription of a gene encoding interleukin 2, a cytokine that is vital to the immune response process. Since its introduction in 1987, sporad...

J. P. Pelletier O. Fadare Y. Wu

2005-01-01

196

Successful treatment of an elderly frail patient with acquired idiopathic thrombotic thrombocytopenic purpura under close monitoring of ADAMTS13 activity and anti-ADAMTS13 antibody titers.  

PubMed

A 68-year-old woman was admitted to the regional hospital because of hemolytic anemia, thrombocytopenia, and neurological abnormalities including unconsciousness. One week before admission, she suffered from diarrhea and subsequently passed out and hit her face on the ground. She was suspected of having TTP and was transferred to our hospital. We performed the assays of ADAMTS13 activity and anti-ADAMTS13 antibody titers, and confirmed the diagnosis of acquired idiopathic TTP with total deficiency of ADAMTS13 activity with its inhibitor. She was initially treated with plasma exchange combined with corticosteroids, however, we were forced to substitute plasma exchange with fresh frozen plasma infusion due to procedure-associated complications. The infusion of fresh frozen plasma was known as less effective and more likely to boost inhibitor titers compared to plasma exchange. In this circumstance, we could successfully switch the plasma therapy under close monitoring of ADAMTS13 activity and anti-ADAMTS13 antibody titers which precisely revealed the disease status of TTP in our patient, and eventually she achieved complete remission with normal level of ADAMTS13 activity and no inhibitor. Our experience suggested that the measurement of ADAMTS13 activity and inhibitor titer might be valuable not only for making the diagnosis but also for guiding treatment decisions by precise evaluating of disease status in patients with the acquired form of TTP. PMID:24517874

Sano, Keigo; Yagi, Hideo; Hanamoto, Hitoshi; Fujita, Mariko; Iizuka, Takashi; Yamazaki, Keiko; Tsubaki, Kazuo

2014-04-01

197

Connective tissue disease following hepatitis B vaccination; topiramate-associated fatal heat stroke; ramelteon-induced autoimmune hepatitis; acute oxaliplatin-induced thrombotic thrombocytopenic purpura.  

PubMed

The purpose of this feature is to heighten awareness of specific adverse drug reactions (ADRs), discuss methods of prevention, and promote reporting of ADRs to the US Food and Drug Administration's (FDA's) MedWatch program (800-FDA-1088). If you have reported an interesting, preventable ADR to MedWatch, please consider sharing the account with our readers. Write to Dr. Mancano at ISMP, 200 Lakeside Drive, Suite 200, Horsham, PA 19044 (phone: 215-707-4936; e-mail: mmancano@temple.edu). Your report will be published anonymously unless otherwise requested. This feature is provided by the Institute for Safe Medication Practices (ISMP) in cooperation with the FDA's MedWatch program and Temple University School of Pharmacy. ISMP is an FDA MedWatch partner. PMID:24715739

Mancano, Michael A

2014-03-01

198

Connective Tissue Disease Following Hepatitis B Vaccination; Topiramate-Associated Fatal Heat Stroke; Ramelteon-Induced Autoimmune Hepatitis; Acute Oxaliplatin-Induced Thrombotic Thrombocytopenic Purpura  

PubMed Central

The purpose of this feature is to heighten awareness of specific adverse drug reactions (ADRs), discuss methods of prevention, and promote reporting of ADRs to the US Food and Drug Administration’s (FDA’s) MedWatch program (800-FDA-1088). If you have reported an interesting, preventable ADR to MedWatch, please consider sharing the account with our readers. Write to Dr. Mancano at ISMP, 200 Lakeside Drive, Suite 200, Horsham, PA 19044 (phone: 215-707-4936; e-mail: mmancano@temple.edu). Your report will be published anonymously unless otherwise requested. This feature is provided by the Institute for Safe Medication Practices (ISMP) in cooperation with the FDA’s MedWatch program and Temple University School of Pharmacy. ISMP is an FDA MedWatch partner.

2014-01-01

199

Vancomycin-induced Henoch-Sch?nlein purpura: a case report  

PubMed Central

Introduction Henoch-Schönlein purpura is a small-vessel systemic vasculitis. Although its exact pathophysiology remains unknown, Henoch-Schönlein purpura has been reported in association with various medical conditions including hypersensitivity. We report the case of a patient with vancomycin-induced Henoch-Schönlein purpura. Case presentation A 42-year-old Caucasian man who had previously undergone a heart transplant was diagnosed as having an intra-abdominal abscess after he underwent a Hartmann procedure. At 15 days after initiation of antibiotic therapy including vancomycin, he developed a purpuric rash of the lower limbs, arthralgia, and macroscopic hematuria. At that time, our patient was already on hemodialysis for end-stage renal disease. Henoch-Schönlein purpura was diagnosed. After a second 15-day course of vancomycin, a second flare of Henoch-Schönlein purpura occurred. Skin biopsies showed leucocytoclastic vasculitis with IgA deposits and eosinophils in the peri-capillary inflammatory infiltrate, suggesting an allergic mechanism. After vancomycin was stopped, we did not observe any further flares. Only five cases of isolated cutaneous vasculitis, one case of lupus-like syndrome and one case of Henoch-Schönlein purpura after vancomycin treatment have been described to date in the literature. Conclusions Clinicians should be aware that systemic vasculitis can be induced by some treatments. Vancomycin is a widely prescribed antibiotic. Occurrence of rare but serious Henoch-Schönlein purpura associated with vancomycin requires its prompt discontinuation.

2012-01-01

200

Purpura fulminans in a patient with mixed connective tissue disease.  

PubMed

A 43-year-old lady was admitted to the intensive care unit with sepsis. She had a history of mixed connective tissue disease, Raynaud's syndrome and hypothyroidism. 2 days later, she developed a purpuric rash on her face and extremities with a livedoid background. Few days later, her distal fingers and toes became gangrenous which then had to be amputated. Laboratory investigations showed that she was coagulopathic and had multiple organ dysfunctions. Antiphospholipid antibodies were negative; however, protein C and antithrombin III levels were low. A skin biopsy showed fibrinoid necrosis in the vessel wall with microthrombi and red-cell extravasation. A diagnosis of purpura fulminans was made. PMID:23370948

Murad, Aizuri A; Jeffers, Michael; Tobin, Anne-Marie; Connolly, Maureen

2013-01-01

201

Henoch-Schönlein purpura and recurrent renal failure.  

PubMed

We present an 18-year-old patient with Henoch-Schönlein purpura (HSP) who had multiple episodes of severe acute renal failure, including one episode for which he required hemodialysis for 2 months and a second episode for which dialysis was considered before his spontaneous recovery of renal function. Multiple treatment options, including steroids, mycophenolate mofetil, cyclophosphamide, and plasmapheresis, were tried but we could not confidently point to the utility of any of these measures. We highlight the unusual severity and lability of our patient's clinical course and how such a course makes the evaluation of treatment effectiveness extraordinarily difficult. PMID:20662705

Vonend, Catharina; Rifkin, Stephen I; Baliga, Rajendra S; Weinstein, Samuel S

2010-01-01

202

[Purpura fulminans, venous thrombosis and constitutional thrombophilia in an infant].  

PubMed

The association of idiopathic purpura fulminans (PF) and venous thrombosis (VT) seldom reveals constitutional thrombophilia in an infant. We report a case of PF in an 18-month-old infant. Laboratory tests showed disseminated intravascular coagulation (DIVC) with normal rates of C and S proteins and antithrombin. The echo-Doppler examination conveyed venous thrombosis of the lower limbs, while the genetic study showed heterozygous mutation of Factor II (G 20210A). Precocious and multidisciplinary management included frozen fresh plasma supplementation and necrosectomy with skin grafts. The diagnosis and therapeutic problems posed by PF combined with deep venous thrombosis are discussed. PMID:23566584

Fadil, F; Elfatoiki, F; Mortaji, K; Lasry, F; Itri, M; Khadir, K; Benchikhi, H; Sarbati, N; Nadifi, S

2013-05-01

203

Organism-specific platelet response and factors affecting survival in thrombocytopenic very low birth weight babies with sepsis  

Microsoft Academic Search

Objective:To study organism-specific platelet response and factors affecting survival in thrombocytopenic very low birth weight (VLBW) babies with sepsis.Study Design:Very low birth weight babies (birth weight <1500 g) admitted to a single level-three intensive care unit from January 2000 to December 2005 were prospectively evaluated for sepsis by rapid screen test, blood counts and blood culture. In thrombocytopenic babies, organism-specific

M A Bhat; J I Bhat; M S Kawoosa; S M Ahmad; S W Ali

2009-01-01

204

Jejunojejunum intussusception as the single initial manifestation of Henoch-Schönlein purpura in a teenager.  

PubMed

We described a 14-year-old boy with initial presentation of recurrent intermittent abdominal pain, with subsequent development of jejunojejunum intussusception. The characteristic purpuric skin rash of Henoch-Schönlein purpura appeared later after the surgical manual reduction. When abdominal pain is the single initial manifestation in patients with undiagnosed Henoch-Schönlein purpura, emergency physicians should evaluate if the patient required early surgical intervention such as intussusception or perforation and always keep Henoch-Schönlein purpura as a possibility in mind. PMID:22306398

Lim, Cherng-Jyr; Chen, Jiann-Hwa; Chen, Wei-Lung; Shen, Ying-Sheng; Huang, Chien-Cheng

2012-11-01

205

Target  

US Patent & Trademark Office Database

The present invention relates to an isolated target sequence. The target sequence is a splice variant of PDE5 called a PDE5a1, a component of which is presented as SEQ ID No 1. The identified target sequence of the present invention may be used to as a target to identify agents (such as modulators) useful in the prevention and/or treatment of a disease associated with scarring and/or fibrosis or to selectively identify smooth muscle cells and myofibroblasts and myoepithelial cells in samples of normal and diseased tissue from individuals.

2004-09-21

206

Purpura fulminans in three cases of early-onset neonatal group B streptococcal meningitis.  

PubMed

The diagnosis of purpura fulminans was associated with three cases of early-onset group B beta-hemolytic streptococcal (GBS) disease. All three infants had confirmed bacterial disease, extensive purpuric lesions involving the extremities, and laboratory evidence of a consumptive coagulopathy. All three children survived but had markedly compromised neurologic outcomes. Purpura fulminans has not been previously reported with early-onset GBS disease. PMID:1890473

Lynn, N J; Pauly, T H; Desai, N S

1991-06-01

207

[Duodenal involvement in Henoch-Schonlein purpura. Report of a case].  

PubMed

Henoch-Schonlein purpura (HSP) is a systemic vasculitis of the small vessels of the skin, joints, gastrointestinal tract and kidneys. It is the most common cause of nonthrombocytopenic purpura in children, but may also occur in adults. Gastrointestinal symptoms and disease occurs in up to 85% of patients and can be of varying intensity. We report a 12 year old boy with gastrointestinal bleeding due to duodenal involvement associated with this condition evidenced by endoscopy. PMID:20924430

Chacaltana Mendoza, Alfonso

2010-01-01

208

Henoch-schönlein purpura associated with gangrenous appendicitis: a case report.  

PubMed

Henoch-Schönlein Purpura (HSP) is a leucocytoclastic vasculitis of unclear aetiology characterised by symmetrical, non-traumatic, nonthrombocytopenic purpura mostly involving the lower limbs and buttocks, as well as arthritis, gastrointestinal manifestations, and occasional nephritis. A 35 years old male presented with purpuric rash on the lower extremities, abdominal pain, fever, arthralgia, and melaena. A diagnosis of HSP with appendicitis was made, which is an exceedingly rare phenomenon. PMID:24876811

Semeena, Nk; Adlekha, Shashikant

2014-03-01

209

Henoch-Sch?nlein Purpura Associated with Gangrenous Appendicitis: A Case Report  

PubMed Central

Henoch-Schönlein Purpura (HSP) is a leucocytoclastic vasculitis of unclear aetiology characterised by symmetrical, non-traumatic, nonthrombocytopenic purpura mostly involving the lower limbs and buttocks, as well as arthritis, gastrointestinal manifestations, and occasional nephritis. A 35 years old male presented with purpuric rash on the lower extremities, abdominal pain, fever, arthralgia, and melaena. A diagnosis of HSP with appendicitis was made, which is an exceedingly rare phenomenon.

SEMEENA, NK; ADLEKHA, Shashikant

2014-01-01

210

Atypical Henoch-Schonlein purpura? Consider polyarteritis nodosa!  

PubMed

We describe the case of a previously healthy 12-year-old boy admitted to a tertiary paediatric centre with the clinical diagnosis of Henoch-Schonlein purpura (HSP). Symptoms on admission included a generalised rash, colicky abdominal pain, hypertension, proteinuria and fresh rectal bleeding. Abdominal pain and distension worsened and serial ultrasounds suggested bowel ischaemia. He underwent repeat laparotomy and bowel resection, with slow improvement after the second laparotomy. The severity of systemic involvement (gastrointestinal, cardiac, renal and skin) made the initial diagnosis of HSP questionable. Immunohistochemistry of skin biopsies was negative for HSP. Histopathology of the bowel specimen revealed features of necrotising small and medium vessel vasculitis in keeping with polyarteritis nodosa. PMID:24717855

Braungart, Sarah; Campbell, Alison; Besarovic, Sanja

2014-01-01

211

[Possible aggravation of a Henoch Schoenlein purpura by isotretinoin].  

PubMed

We report on a 16 year old patient with a hemorrhagic bullous form of a Henoch-Schönlein purpura. The cause for the event could not be determined, but the patient recovered without complications. Since he had begun a therapy with isotretinoin due to an acne inversa 3 weeks before, a possible association between the event and the treatment could not be excluded, and the case was reported to Swissmedic. In the comment, we discuss the pharmacology of isotretinoin, drug-induced vasculitis, and its pathomechanisms. As a general reminder, the criteria for the causality assessment of adverse drug reactions as well as the reporting system of adverse drug reactions in Switzerland are discussed. PMID:22715073

Derungs, A; Eberhardt, K; Heiniger, U; Rudin, C; Brauchli Pernus, Y B

2012-06-20

212

Evaluation of thromboelastography for prediction of clinical bleeding in thrombocytopenic dogs after total body irradiation and hematopoietic cell transplantation.  

PubMed

Objective-To determine whether thromboelastography is more accurate than conventional methods of evaluating hemostasis for the prediction of clinical bleeding in thrombocytopenic dogs following total body irradiation (TBI) and bone marrow transplantation (BMT). Animals-10 client-owned thrombocytopenic dogs with multicentric lymphoma. Procedures-Results of a kaolin-activated thromboelastography assay, platelet count, and buccal mucosal bleeding time were evaluated for correlation to clinical bleeding. Results-Maximum amplitude, derived via thromboelastography, was the only hemostatic variable with significant correlation to clinical bleeding. Buccal mucosal bleeding time had a high sensitivity but poor specificity for identifying dogs with clinical bleeding. Conclusions and Clinical Relevance-Compared with buccal mucosal bleeding time and platelet count, thromboelastography was more reliable at identifying thrombocytopenic dogs with a low risk of bleeding and could be considered to help guide the use of transfusion products in dogs undergoing TBI and BMT. PMID:24762013

Bucknoff, Melissa C; Hanel, Rita M; Marks, Steven L; Motsinger-Reif, Alison A; Suter, Steven E

2014-05-01

213

Cone and platelet analyser (CPA): a new test for the prediction of bleeding among thrombocytopenic patients.  

PubMed

The risk of bleeding among thrombocytopenic patients was evaluated using our new cone and platelet analyser (CPA) test. Using this test, adherence of platelets was quantitated on extracellular matrix and expressed as percent of surface coverage (SC) and the average size (AS) of aggregates. 42 thrombocytopenic patients with ITP (n=23), post chemotherapy (n= 12) and others (n= 7) were tested over a total of 82 visits. On each visit, complete blood count and CPA tests were performed and patients were evaluated for evidence of bleeding (found in 40 visits). Bleeding patients had significantly lower platelet counts (27.4 +/- 22.0 v 47.1 +/- 21.0 x 10(9)/l), lower haematocrit values (30.2 +/- 8.1 v 35.2 +/- 6.6%), lower MPV (6.83 +/-1.89 v 8.98 +/- 1.13 fl), and lower SC (4.87 +/- 3.95 v 10.33 +/-5.48%) and AS (33.99 +/- 14.94 v 52.9 +/- 24.34 microm2). Univariate analysis yielded platelet count < or =20.0 x 10(9)/l, MPV < or =8 fl, haematocrit <35%, SC <5%, AS< or =40 microm2 as significantly associated with bleeding, whereas only MPV and SC were associated with bleeding (OR 6.95, CI 2.25-21.46 and OR 4.27, CI 1.29-14.16, respectively) by multivariate analysis. When taken together, 21/22 of patients (95%) with both low SC (<5%) and low MPV (<8.0 fl) had bleeding symptoms, whereas only 9/43 (21%) patients with both these parameters above these values experienced bleeding symptoms. We conclude that the CPA test and the parameter SC (<5%) together with MPV (< or =8 fl) might be used as independent predictors of bleeding in the management of thrombocytopenic patients. PMID:9609519

Kenet, G; Lubetsky, A; Shenkman, B; Tamarin, I; Dardik, R; Rechavi, G; Barzilai, A; Martinowitz, U; Savion, N; Varon, D

1998-05-01

214

The diagnosis and classification of Henoch-Schönlein purpura: an updated review.  

PubMed

Henoch-Schönlein purpura (HSP) is a common childhood systemic vasculitis with clinical characteristics of cutaneous palpable purpura, arthralgia/arthritis, bowel angina, and hematuria/proteinuria. HSP is identified mainly based on the above presentations. Combined with pathohistological findings of leukocytoclastic vasculitis (LCV) and IgA-immune deposits in vessel walls and/or glomeruli increase the diagnostic sensitivity and specificity. However, considering the accessibility of biopsy and some patients with atypical presentations, there are still medical unmet needs in HSP diagnosis. This article reviews the diagnosis of HSP including the aspects of classification criteria, differential diagnosis, and some laboratory findings as the biomarkers with diagnostic potential. PMID:24424188

Yang, Yao-Hsu; Yu, Hsin-Hui; Chiang, Bor-Luen

2014-01-01

215

Pneumococcal sepsis-induced purpura fulminans in an asplenic adult patient without disseminated intravascular coagulation.  

PubMed

Acute perturbations in the hemostatic balance of anticoagulation and procoagulation antecede the manifestation of purpura fulminans, a rare syndrome of intravascular thrombosis and hemorrhagic infarction of the skin. Hallmarks include small vessel thrombosis, tissue necrosis and disseminated intravascular thrombosis. The course may be rapidly fulminant resulting in multiorgan failure with thrombotic occlusion of the vasculature, leading to distal extremity ischemia and necrosis. Depletion of protein C (PC) has been emphasized in the pathogenesis. Early intravenous antibiotic administration and hemodynamic support are cornerstones in management. Herein, we report a case of pneumococcal sepsis-induced purpura fulminans limited to the skin in an asplenic adult patient without the development disseminated intravascular coagulation. PMID:24185261

Saraceni, Christine; Schwed-Lustgarten, Daniel

2013-12-01

216

Henoch-Schönlein purpura complicated by appendicitis, intussusception and ureteritis.  

PubMed

Henoch-Schönlein purpura (HSP) is the most common childhood systemic small-vessel vasculitis with skin, joint, gastrointestinal (GI) and renal involvement. Uncommon GI complications are intussusception, bowel perforation and rarely reported appendicitis. HSP-associated stenosing ureteritis represents a rare and potentially serious complication. We present a 5-year-old boy with severe and prolonged course of HSP and three very rare complications that occurred sequentially: appendicitis, intussusception and ureteritis. Only three days after admission, he developed clinical signs of acute appendicitis indicating surgical intervention. Histological analysis of excised appendix showed inflammation but without signs typical for vasculitis. Two weeks later, with the reccurence of HSP he again developed clinical picture of acute abdomen. Ultrasound and radiologic evaluation demonstrated ileo-ileal intussusception and the second laparotomy was undertaken. Histological analysis of the resected bowel tissue showed typical signs of leucocytoclastic vasculitis. In the fourth week of his illness, serial urinalysis showed nephritic urinary sediment indicative of renal involvement. Unexpectedly, control abdominal ultrasound demonstrated mild hydronephrosis of the left kidney, not seen on previous ultrasound evaluations. Undertaken excretory urography and computed tomography (CT) scan showed stenosis of upper/ midureter with mild dilation of upper part of the left ureter suggesting unilateral HSP-associated stenosing ureteritis. Eventually, the patient was discharged and closely followed-up for the next two years. He had no further reccurence of HSP, the urinalysis normalized after six months, while mild unilateral hydronephrosis remained unchanged. Our search of the literature did not show reports of HSP complicated by appendicitis, intussusception and ureteritis, and to our knowledge this is the first case with three different illness events that occured sequentially. We emphasize the necessity of repeated ultrasound evaluations in the course of HSR especially in cases with severe GI and renal invovement. PMID:21661371

Jasi?, Mladen; Subat-Dezulovi?, Mirna; Nikoli?, Harry; Jonji?, Nives; Manestar, Koraljka; Dezulovi?, Matko

2011-03-01

217

Endothelin 1 levels in relation to clinical presentation and outcome of Henoch Schonlein purpura  

Microsoft Academic Search

BACKGROUND: Henoch Schonlein purpura (HSP) is a common vasculitis of small vessels whereas endothelin-1 (ET-1) is usually reported elevated in vasculities and systematic inflammation. The aim of the present study was to investigate whether ET-1 levels are correlated with the clinical presentation and the outcome of HSP. METHODS: The study sample consisted of thirty consecutive patients with HSP. An equal

S Fessatou; P Nicolaidou; D Gourgiotis; H Georgouli; K Douros; M Moustaki; A Fretzayas

2008-01-01

218

Effectiveness of early prednisone treatment in preventing the development of nephropathy in anaphylactoid purpura  

Microsoft Academic Search

A prospective study was performed to verify whether early administration of prednisone could be useful in preventing the development of nephropathy in anaphylactoid purpura. Only patients without signs of nephropathy upon initial presentation entered into the study. A total of 84 patients received delta-prednisone (1 mg\\/kg per day per os for 2 weeks), and 84 patients did not receive steroids.

F. Mollica; S. LiVolti; R. Garozzo; G. Russo

1992-01-01

219

Multiple small-intestine intussusceptions: a complication of purpura haemorrhagica in a horse.  

PubMed

A Belgian draft horse stallion presented with classical signs of purpura haemorrhagica: oedema of the distal limbs, ventral abdomen and head, ecchymotic haemorrhage of mucous membranes, epistaxis, fever, lethargy, reluctance to move, and anorexia. Serum chemistry revealed a highly elevated gamma-globulin fraction. Streptococcal lymphadenitis (strangles) had been present on a neighbouring farm in the past few months. After an initial positive response to therapy with benzylpenicillin, flunixin and dexamethasone, the horse's condition deteriorated suddenly on day 4 of hospitalization, with signs of severe endotoxaemia and small-intestine obstruction. The horse was euthanized because of its poor prognosis and because of economic considerations. On necropsy, large serosal haemorrhages in the small and large intestine and multiple jejunojejunal intussusceptions were found. Intussusception is a well-known complication in children with Henoch-Schonlein purpura, a human autoimmune disease resembling purpura haemorrhagica in horses. To the author's knowledge, this is the first report to describe intussusception as a complication of purpura haemorrhagica in a horse. PMID:21699093

Dujardin, C L L

2011-06-01

220

Resident rounds: Part III: Multiple myeloma presenting as fulminant retiform purpura.  

PubMed

Retiform purpura secondary to underlying type 1 cryoglobulinemia can be a presenting sign of multiple myeloma. Severe pain may herald microvascular occlusion and impending ulceration. Recognizing the distinctive cutaneous and histopathologic features of this occlusive vasculopathy allows for timely diagnosis and treatment. PMID:24851244

Hoesly, Fridolin J; Sluzevich, Jason C

2014-04-01

221

Gastrointestinal involvement revealing Henoch Schonlein purpura in adults: Report of three cases and review of the literature  

PubMed Central

The diagnosis of Henoch-Schönlein purpura (HSP) is difficult, especially when abdominal symptoms precede cutaneous lesions. We report three cases of adult HSP revealed by gastrointestinal (GI) involvement.

2011-01-01

222

Occurrence of thrombosis in congenital thrombocytopenic disorders: a critical annotation of the literature.  

PubMed

Patients with a low platelet count are prone to bleeding. The occurrence of a thrombotic event in congenital thrombocytopenic patients is rare and puzzling. At least nine patients with Glanzmann thrombasthenia have been reported to have had a thrombotic event, eight venous and one arterial (intracardiac, in the left ventricle). On the contrary, three patients with Bernard-Soulier syndrome have been shown to have had arterial thrombosis (myocardial infarction) but no venous thrombosis. Finally, seven patients with the familiar macrothrombocytopenia due to alterations of the MYH9 gene have been reported to have had thrombosis (five myocardial infractions, one ischemic stroke, one deep vein thrombosis and one portal vein thrombosis). The significance of these findings is discussed with particular emphasis on the discrepancy between venous and arterial thrombosis seen in patients with Glanzmann thrombasthenia and Bernard-Soulier syndrome. PMID:23037321

Girolami, Antonio; Sambado, Luisa; Bonamigo, Emanuela; Vettore, Silvia; Lombardi, Anna M

2013-01-01

223

Comparison of platelet parameters in thrombocytopenic patients associated with acute myeloid leukemia and primary immune thrombocytopenia.  

PubMed

Thrombocytopenia is caused by insufficient production and excessive destruction of platelets. Recent improvement of automated blood cell analyzers has allowed measurement of several platelet parameters, providing better understanding of the underlying mechanisms of thrombocytopenia. We investigated the significance of platelet parameters in thrombocytopenic patients. Thrombocytopenic patients (platelet <100?×?10/?l) who were newly diagnosed with acute myeloid leukemia and primary immune thrombocytopenia were enrolled, and platelet, mean platelet volume, platelet distribution width, platelet crit, mean platelet component, mean platelet mass, and large platelet count were measured, and the percentages of large platelets were calculated. The parameters were also measured in the reference population. The mean values of each parameter were as follows: platelet, 259?×?10/?l; mean platelet volume, 7.9 fl; platelet distribution width, 51.3%; platelet crit, 0.20%; mean platelet component, 26.0?g/dl; mean platelet mass, 1.9 pg; large platelet, 4.7?×?10/?l; large platelet percentage, 1.7%. In comparison with acute myeloid leukemia patients, patients with primary immune thrombocytopenia showed significantly higher mean platelet volume, platelet distribution width, mean platelet component, mean platelet mass, large platelet, and large platelet % (P?

Kim, Moon Jin; Park, Pil-Whan; Seo, Yiel-Hea; Kim, Kyung-Hee; Seo, Ja Young; Jeong, Ji-Hun; Park, Mi-Jung; Ahn, Jeong-Yeal

2014-04-01

224

[Purpura fulminans: functional results in two paediatric patients after suffering multiple amputations].  

PubMed

Purpura fulminans (PF) is an unusual haemorrhagic process that is usually associated with meningococcal sepsis and other infectious processes. It usually affects neonates and young children, and starts with a benign infection that progresses to a high fever, purpura ecchymosis, disseminated intravascular coagulopathy, necrosis and gangrene. The treatment of these children usually requires making difficult decisions, since the surgeon and the families must come to terms with the possibility of following an aggressive line of treatment that could lead to multiple mutilating sequelae, or follow palliative treatment. In this study, we review the clinical presentation, treatment and results of two cases of PF treated in our hospital between the years 2002 and 2005. The children presented in this study had a good long-term functional result and an acceptable quality of life, despite being subjected to multiple amputations. PMID:23594852

Tamimi-Mariño, I; Hidalgo, J J; Perez, A; Mendez, L I

2012-01-01

225

Successful outcome of a corticodependent henoch-schönlein purpura adult with rituximab.  

PubMed

Henoch-Schönlein purpura (HSP) is a systemic vasculitis involving small vessels with deposition of immunoglobulin A (IgA) complexes, usually affecting children. Compared with children, HSP in adults is more severe and frequently associated with cancer. We report the case of a 49-year-old woman with medical history of kidney transplantation for segmental glomerular hyalinosis. Eight years after the transplantation, while taking combined immunosuppressive therapy with tacrolimus and azathioprine indicated for the prevention against transplant rejection, she developed a Henoch-Schönlein purpura. Vasculitis involves skin and sciatic peroneal nerve and she received systemic corticosteroid treatment. Because of four relapses and corticosteroid dependence, the patient was treated with rituximab (two intravenous infusions of 1000?mg given two weeks apart). Successful outcome was observed along two years of follow-up. This new case of successful use of rituximab in HSP promotes more investigations of this treatment in clinical trials. PMID:24799911

Pindi Sala, Taylor; Michot, Jean-Marie; Snanoudj, Renaud; Dollat, Marion; Estève, Emmanuel; Marie, Bernadette; Taoufik, Yacine; Delfraissy, Jean-François; Lazure, Thierry; Lambotte, Olivier

2014-01-01

226

Successful Outcome of a Corticodependent Henoch-Sch?nlein Purpura Adult with Rituximab  

PubMed Central

Henoch-Schönlein purpura (HSP) is a systemic vasculitis involving small vessels with deposition of immunoglobulin A (IgA) complexes, usually affecting children. Compared with children, HSP in adults is more severe and frequently associated with cancer. We report the case of a 49-year-old woman with medical history of kidney transplantation for segmental glomerular hyalinosis. Eight years after the transplantation, while taking combined immunosuppressive therapy with tacrolimus and azathioprine indicated for the prevention against transplant rejection, she developed a Henoch-Schönlein purpura. Vasculitis involves skin and sciatic peroneal nerve and she received systemic corticosteroid treatment. Because of four relapses and corticosteroid dependence, the patient was treated with rituximab (two intravenous infusions of 1000?mg given two weeks apart). Successful outcome was observed along two years of follow-up. This new case of successful use of rituximab in HSP promotes more investigations of this treatment in clinical trials.

Pindi Sala, Taylor; Snanoudj, Renaud; Esteve, Emmanuel; Marie, Bernadette; Taoufik, Yacine; Delfraissy, Jean-Francois; Lazure, Thierry; Lambotte, Olivier

2014-01-01

227

Combined antithrombin and protein C supplementation in meningococcal purpura fulminans: a pharmacokinetic study  

Microsoft Academic Search

PurposeTo document in patients with meningococcal purpura fulminans (PF), the effects of a combined supplementation with antithrombin (AT) and protein C (PC) plasma concentrates and to estimate the pharmacokinetics and dose requirements of each inhibitor.DesignRetrospective study of 15 patients.SettingOne paediatric and one adult ICU in a university hospital.InterventionsIn addition to standard intensive care, all patients received a 100 IU\\/kg loading dose

François Fourrier; Francis Leclerc; Karl Aidan; Ahmed Sadik; Mercé Jourdain; Antoine Tournoys; Odile Noizet

2003-01-01

228

CCL5, CXCL16, and CX3CL1 are associated with Henoch-Schonlein purpura  

Microsoft Academic Search

Chemokines are involved in the pathogenesis of various vascular inflammations. However, information about chemokines in Henoch-Schonlein\\u000a purpura (HSP) is limited. Herein, we investigated the serum CCL5, CXCL16, and CX3CL1 levels in HSP patients with controls\\u000a and the ability of sera from HSP patients on chemokine production in human dermal microvascular endothelial cells. Enzyme-linked\\u000a immunosorbent assay (ELISA) detected serum CCL5, CXCL16,

Tao ChenZai-pei; Zai-pei Guo; Xiao-yan Jiao; Rui-zhen Jia; Yu-hong Zhang; Jing-yi Li; Xu-lei Huang; Hong-jie Liu; Ting Lai

229

Henoch Schönlein purpura presenting as duodenal ulcer and gastric outlet obstruction.  

PubMed

Henoch-Schönlein purpura (HSP) is an acute small vessel leucocytoclastic vasculitis. It is the commonest vasculitis in children, with an incidence of about 10 cases per 100, 000 a year. Gastrointestinal manifestations are commonly encountered, however hematemesis and gastric outlet obstruction are rarely reported. The authors present the case of a 5-y-old boy having hematemesis, gastric outlet obstruction and multiple duodenal ulcers. He improved with steroids and conservative management. PMID:23564516

Rathore, Mukesh; Shrivastava, Rimjhim; Goyal, Ravinder; Radotra, B D; Thapa, B R

2014-02-01

230

Evolution of immunoglobulin a nephropathy into Henoch-Schönlein purpura in an adult patient  

Microsoft Academic Search

We report the first case of the evolution of immunoglobulin A (IgA) nephropathy into Henoch-Schönlein purpura in an adult patient. A 28-year-old man presented with an episode of gross hematuria and acute renal failure. The serum IgA level was increased and renal biopsy findings were diagnostic of IgA nephropathy. In addition, many renal tubuli were filled by red blood cell

Alicia Araque; Rosa Sánchez; Concepción Alamo; Nemesio Torres; Manuel Praga

1995-01-01

231

Rituximab-induced interstitial lung disease in a patient with immune thrombocytopenia purpura.  

PubMed

We report the case of an 84-year-old man with refractory immune thrombocytopenia purpura (ITP) who was treated with rituximab and subsequently developed severe interstitial lung disease. There has been increasing use of rituximab in the treatment of ITP with success rates of up to 62% in adult patients with recurrent ITP. Interstitial lung disease is a rare but recognised complication of rituximab but has been rarely reported in the setting of ITP. PMID:22432995

Child, N; O'Carroll, M; Berkahn, L

2012-03-01

232

Methylprednisolone pulse therapy in the treatment of severe forms of Schönlein-Henoch purpura nephritis  

Microsoft Academic Search

.  ?Between 1980 and 1994, 38 children with severe forms of Schönlein-Henoch purpura glomerulonephritis were entered into a prospective\\u000a study to evaluate methylprednisolone pulse therapy on the outcome of nephropathy in terms of clinical symptoms and histopathological\\u000a changes. The patients were considered at risk of developing chronic renal failure when they presented with a nephrotic syndrome\\u000a and\\/or had 50% or more

Patrick Niaudet; Renée Habib

1998-01-01

233

Goodpasture syndrome during the course of a Schönlein-Henoch purpura  

Microsoft Academic Search

Two months after surgical resection of a bronchogenic carcinoma, a 69-year-old patient presented with Schönlein-Henoch purpura with kidney involvement followed by pulmonary hemorrhage. The presence of an IgA linear pattern on the kidney biopsy specimen and circulating anti-glomerular basement membrane (GBM) IgA antibodies led to the diagnosis of Goodpasture syndrome, which implies the possibility that the well-known pulmonary involvement during

Luis Carreras; Rafael Poveda; Jordi Bas; Mariona Mestre; Inés Rama; Marta Carrera

2002-01-01

234

Neonatal purpura fulminans manifestation in early-onset group B Streptococcal infection  

PubMed Central

Patient: Male, 0 Final Diagnosis: Purpura fulminans Symptoms: Fever • letargy Medication: — Clinical Procedure: — Specialty: Pediatrics and Neonatology Objective: Rare disease Background: Neonatal purpura fulminans (PF) is a rare but frequently fatal disorder associated with high morbidity and mortality. It may be congenital, as a result of protein C and S deficiency, or acquired due to severe infection. Gram-negative organisms and Staphylococcus species are the most common causes of the acute infectious type, and a few cases of causative neonatal group B Streptococcus (GBS) disease have been reported worldwide. Case Report: We present a full-term male neonate with purpura fulminans secondary to early-onset group B streptococcal (GBS) infection. The mother brought the infant to the emergency department at the age of 43 hours of life, with fever (39.5°C) and lethargy. Neonatal sepsis was suspected, and he was immediately started on intravenous ampicillin and gentamicin. The initial workup revealed disseminated intravascular coagulopathy, and both blood and CSF cultures grew GBS. He had normal levels of protein C and protein S for his age. The infant died 48 hours after admission due to multiorgan system failure despite aggressive neonatal intensive care support. Conclusions: Neonatal PF secondary to early-onset GBS infection is a fatal condition that should not be missed. Screening pregnant women for GBS colonization and use of protocols for preventing perinatal GBS infection is considered the most important preventive measure of this fatal condition, especially among Saudi women, who have a relatively high rate of GBS infection.

AlBarrak, May; Al-Matary, Abdulrahman

2013-01-01

235

Differences in clinical manifestations and outcomes between adult and child patients with Henoch-Schönlein purpura.  

PubMed

We aimed to investigate differences in clinical manifestations and outcomes between adult and child patients with Henoch-Schönlein purpura (HSP), and to analyze the factors associated with poor prognosis for HSP nephritis. This retrospective 10-yr study enrolled 160 patients with HSP who visited Severance Hospital. Purpura was mostly detected in lower extremities, but purpura in upper extremities was more frequently observed in adults than children (41.7% vs 19.3%). Children had a greater frequency of arthralgia (55.4% vs 27.1%), while adults had a greater frequency of diarrhea (20% vs 1.6%). Anemia, elevated C-reactive protein, and level of IgA were more frequently observed in adults (25% vs 7.1%, 65.6% vs 38.4%, 26.3% vs 3.5%). Renal involvement in adults was more severe than in children (79.2% vs 30.4%). Chronic renal failure showed a significant difference in outcomes of HSP between adults (10.4%) and children (1.8%) after a follow up period of an average of 27 months. Furthermore, renal insufficiency at diagnosis was significantly related to the progression to chronic renal failure. Our results showed several differences in the clinical features of HSP between adults and children. Adults with HSP had a higher frequency of renal insufficiency and worse renal outcomes than children. Renal insufficiency at diagnosis might be of predictive value for the progression to chronic renal failure in HSP patients. PMID:24550645

Kang, Yoon; Park, Jin-su; Ha, You-Jung; Kang, Mi-il; Park, Hee-Jin; Lee, Sang-Won; Lee, Soo-Kon; Park, Yong-Beom

2014-02-01

236

[The role of endoscopy in the diagnosis of Schönlein-Henoch purpura].  

PubMed

During a 10 years period 62 adult patients were admitted with diagnosis of Schönlein-Henoch purpura in our hospital. 25 female and 37 male patients ranged from 30 to 87 years (mean: 59.5 years) presenting with cutan, joint, renal and particularly abdominal involvement were investigated retrospectively. During the course of the disease, all patients developed purpuric rash (100%), 14 (22.5%) patients had joint symptoms and renal involvement occurred in 12 (19.3%) patients. In this study, we discuss 15 (24%) patients with gastrointestinal symptoms appearing in Henoch's purpura. Analysis of the gastrointestinal clinical features revealed: abdominal pain 13 (86%), massive colorectal bleeding 3 (20%), occult blood loss 10 (66%) vomiting 6 (40%) and diarrhoea in 3 (20%) patients. All the patients underwent lower and upper endoscopic examination, in 3 cases the author saw purpuric mucosal lesions in duodenum and in 8 patients were also found coin-like elevated lesions, additionally, biopsy from colonic lesions showed leukocytoclastic vasculitis. It is concluded that endoscopy can be helpful in the diagnosis and treatment of Schönlein-Henoch purpura, especially is those without typical skin rash. PMID:9324677

Novák, J

1997-08-31

237

How Is Thrombocytopenia Diagnosed?  

MedlinePLUS

... With Clinical Trials Links Related Topics Aplastic Anemia Immune Thrombocytopenia Thrombotic Thrombocytopenic Purpura Disseminated Intravascular Coagulation Blood Transfusion Related Media Videos ...

238

What Are the Signs and Symptoms of Thrombocytopenia?  

MedlinePLUS

... With Clinical Trials Links Related Topics Aplastic Anemia Immune Thrombocytopenia Thrombotic Thrombocytopenic Purpura Disseminated Intravascular Coagulation Blood Transfusion Related Media Videos ...

239

Living with Thrombocytopenia  

MedlinePLUS

... With Clinical Trials Links Related Topics Aplastic Anemia Immune Thrombocytopenia Thrombotic Thrombocytopenic Purpura Disseminated Intravascular Coagulation Blood Transfusion Related Media Videos ...

240

Sirolimus for Autoimmune Disease of Blood Cells  

ClinicalTrials.gov

Autoimmune Pancytopenia; Autoimmune Lymphoproliferative Syndrome (ALPS); Evans Syndrome; Idiopathic Thrombocytopenic Purpura; Anemia, Hemolytic, Autoimmune; Autoimmune Neutropenia; Lupus Erythematosus, Systemic; Inflammatory Bowel Disease; Rheumatoid Arthritis

2014-02-03

241

A rare presentation of seizures in a not-so-rare disease: henoch-schönlein purpura presenting with repeated seizures.  

PubMed

Henoch-Schönlein purpura represents the most common form of systemic vasculitis in children. Although a very common cause of vasculitis, seizures are a very rare complication of this disorder. We report a 5-year-old boy who presents with no other clinical symptoms of the disorder other than a seizure. By presenting this case, we hope to expand the differential diagnosis of repeated seizures to include diseases in which the pathogenesis of diseases with small vessel vasculitis such as Henoch-Schönlein purpura is considered. PMID:24892684

Camacho, Christina; Leva, Ernest G

2014-06-01

242

Purpura fulminans following late-onset group B beta-hemolytic streptococcal sepsis.  

PubMed

A 16-day-old male infant initially was in septic shock. Following intensive resuscitation, thrombohemorrhagic lesions developed over his extremities, except for the limb with an arterial line maintained by a continuous heparin sodium infusion. Blood and CSF cultures yielded group B beta-hemolytic streptococci. Results of laboratory studies and clinical appearance supported the diagnosis of purpura fulminans (PF). Systemic heparinization was therefore started, and subsequently his condition improved. Because of the distinct difference in limb sparing, we concluded heparin has a beneficial effect on the evolution of PF. PMID:6383017

Issacman, S H; Heroman, W M; Lightsey, A L

1984-10-01

243

Purpura with ulcerative skin lesions and mixed cryoglobulinemia in a quiescent hepatitis B virus carrier.  

PubMed

Mixed cryoglobulinemia is occasionally seen in patients with hepatitis B virus (HBV) infection. This report presents the case of a quiescent HBV carrier who had type II mixed cryoglobulinemia, protracted purpura, ulcerative skin lesions and advanced chronic kidney disease. The cutaneous manifestations of the patient improved along with a decrease in the serum cryoglobulin and HBV-deoxyribonucleic acid levels following the initiation of oral entecavir in combination with plasmapheresis. However, the patient ultimately required prednisolone due to the limited benefits of these treatments. We also discuss various concerns regarding steroid treatment in patients with mixed cryoglobulinemia complicated by HBV infection. PMID:24429450

Yamazaki, Tomoyuki; Akimoto, Tetsu; Okuda, Kousuke; Sugase, Taro; Takeshima, Eri; Numata, Akihiko; Morishita, Yoshiyuki; Iwazu, Yoshitaka; Yoshizawa, Hiromichi; Komada, Takanori; Iwazu, Kana; Saito, Osamu; Takemoto, Fumi; Muto, Shigeaki; Kusano, Eiji

2014-01-01

244

Improved Outcome of Henoch-Schonlein Purpura Nephritis by Early Intensive Treatment  

Microsoft Academic Search

Objective  To evaluate the different therapies used to treat mild, moderate and severe Henoch-Schonlein purpura nephritis (HSPN) patients\\u000a and to identify the most effective treatment.\\u000a \\u000a \\u000a \\u000a \\u000a Methods  One hundred and eighty six children were evaluated retrospectively. They were divided into mild, moderate and severe condition\\u000a groups. Different therapeutic protocols of non-steroid therapy, hydrocortisone sodium succinate (HCSS) therapy, methylprednisolone\\u000a (MP) pulse therapy, and MP

Fang Deng; Ling Lu; Qin Zhang; Bo Hu; Xun Xia

245

Henoch-Schonlein purpura with intestinal perforation and cerebral hemorrhage: A case report  

PubMed Central

Henoch-Schonlein purpura (HSP) with intestinal perforation and cerebral hemorrhage is a very rare clinical condition. There has been no report of HSP complicated with both intestinal perforation and cerebral hemorrhage until October 2012. Here we describe a case of HSP with intestinal perforation and cerebral hemorrhage in a 5-year-old girl. Plain abdominal radiograph in the erect position showed heavy gas in the right subphrenic space with an elevated diaphragm. Partial resection of the small intestine was performed, and pathological analysis suggested chronic suppurative inflammation in all layers of the ileal wall and mesentery. Seventeen days after surgery, cerebral hemorrhage developed and the patient died.

Wang, Hong-Liang; Liu, Hai-Tao; Chen, Qi; Gao, Yang; Yu, Kai-Jiang

2013-01-01

246

Remission of concomitant Henoch-Schöenlein purpura and Sydenham chorea after intravenous corticosteroids.  

PubMed

We report a young girl who developed Henoch-Schoenlein purpura at the age of 11 years. Two weeks later she developed severe asymmetric choreic movements and behavioral disturbances. Sydenham s chorea was diagnosed based on the laboratory evidence and she was given intravenous methylprednisolone for five consecutive days. Both behavioral and movement disorder rapidly resolved. She was asymptomatic at three years of follow-up. The rapid resolution of choreic movements and behavioral disturbances in our patients suggests, intravenous corticosteroids may be an option in the treatment of Sydenham's chorea, more so when the movements are disabling. PMID:19305078

Zúñiga, Carlos; Díaz, Sergio; Fariña, Angeles; Micheli, Federico

2009-01-01

247

Rapid improvement of Henoch-Schonlein purpura associated with the treatment of Helicobacter pylori infection  

PubMed Central

Helicobacter pylori (H. pylori) are one of the most common bacterial infections, seen in humans, worldwide and their possible relationships to different diseases are a focus of attention nowadays. H. pylori may cause some extra intestinal manifestations some of which are dermatological conditions, including Henoch-Schönlein purpura (HSP), chronic urticaria and atopic dermatitis. We describe a 49-year-old man who presented with HSP triggered by gastric H. pylori infection. Treatment of H. Pylori infection was accompanied by prompt resolution of the gastrointestinal manifestations and purpuric rashes. These findings suggest a causative role for H. pylori in the occurrence of HSP.

Ulas, Turgay; Tursun, Irfan; Dal, Mehmet Sinan; Eren, Mehmet Ali; Buyukhatipoglu, Hakan

2012-01-01

248

Successful Treatment of Hemorrhagic Bullous Henoch-Sch?nlein Purpura with Oral Corticosteroid: A Case Report  

PubMed Central

Henoch-Schönlein purpura (HSP) is a vasculitis of small-sized blood vessels, resulting from immunoglobulin-A-mediated inflammation. It is the most common acute systemic vasculitis in childhood and mainly affects skin, gastrointestinal tract, joints, and kidneys. The characteristic rash of HSP consists of palpable purpuric lesions 2 to 10?mm in diameter concentrating in the buttocks and lower extremities. The occurrence of hemorrhagic bullae in children with HSP is rarely encountered. This report describes a 4.5-year-old female patient with HSP associated with hemorrhagic bullous lesions.

Kocaoglu, Celebi; Ozturk, Ramazan; Unlu, Yasar; Akyurek, Fatma Tuncez; Arslan, Sukru

2013-01-01

249

Purpura and fillers: a review of pre-procedural, intra-procedural, and post-procedural considerations.  

PubMed

Injectable aesthetic fillers are associated with the common and expected side effect of purpura or bruising. There are pre-procedural, intra-procedural and post-procedural considerations that can potentially minimize bruising. Traditional and newer techniques are discussed in this review and the benefits and risks of each technique will be provided. PMID:24085049

Schlesinger, Todd E; Cohen, Joel L; Ellison, Sarah

2013-10-01

250

Renal manifestations in Henoch-Schönlein purpura: a 10-year clinical study.  

PubMed

Henoch-Schönlein purpura (HSP) is an IgA-mediated systemic small vessel vasculitis of childhood. It is characterized by the symptoms including nonthrombocytopenic purpura, abdominal pain, hematuria/proteinuria, and arthargia/arthritis. We conducted a retrospective study of 261 patients diagnosed with HSP from December 1991 to December 2001. Of the 261 patients, fifty-three (20.3%) developed renal manifestations after onset of the disease. Two patients developed nephrotic syndrome. Four patients had group A beta-hemolytic streptococcal pharyngitis and subsequent depressed serum C3 concentration typical of post streptococcal glomerulonephritis. During the study period, the renal survival rate after disease onset was 100%. The prognosis of renal involvement was better than reports from other series. In this study we also found factors associated with HSP nephritis; these included older age at onset, GI bleeding, and central nervous system involvement. The long-term morbidity of HSP is predominantly attributed to renal involvement. It is thus recommended that patients with HSP nephritis are followed for longer periods of time. PMID:15947991

Chang, Wen-Liang; Yang, Yao-Hsu; Wang, Li-Chieh; Lin, Yu-Tsan; Chiang, Bor-Luen

2005-09-01

251

Purpura Fulminans in Meningococcal Septicaemia in an Adult: A Case Report  

PubMed Central

Summary Purpura fulminans is a rare and severe complication of meningococcal septicaemia. It presents as a petechial rash spreading rapidly in extent and depth, evolving into full-thickness skin necrosis. The condition is extremely uncommon in the adult population. We report the case of a 28-yr-old man with extensive meningococcal-related skin necrosis. The initial diagnosis was made and first treatment given in the emergency department of a local hospital, from where after 12 days he was transferred to our hospital. Our approach was based on the continuation of intensive treatment and on staged aggressive debridement. Temporary alloplastic skin grafts were used to prepare the wound bed and the wounds were closed with autologous skin grafts. The patient survived but subsequently, owing to chronic skin ulceration and scar instability, he underwent late bilateral below-the-knee amputation. The patient returned to normal deambulation with an orthopaedic prosthesis 18 months after the onset of meningococcal septicaemia.

Bollero, D.; Stella, M.; Gangemi, E.N.; Spaziante, L.; Nuzzo, J.; Sigaudo, G.; Enrichens, F.

2010-01-01

252

Salmonella meningitis complicated with subdural empyema, brain abscess and purpura fulminans: report of one case.  

PubMed

There exists high incidence of bacteremia, sepsis and meningitis in young infants with Salmonella infection. However, focal intracranial abscesses due to Salmonella infections are rare. We reported a 2-month-old male baby presenting salmonella infection with brain abscess and purpura fulminans. The patient's clinical course was fulminant. He was admitted due to fever, irritability, anemia and leukopenia. He developed cardiac arrest, shock and skin diathesis on his second hospitalization day. After resuscitation he became comatous and showed acrocyanosis and gangrenous skin over the hands, feet and left ear lobe. Both blood and cerebrospinal fluid cultures were Salmonella Group B. The patient got worse rapidly in spite of vigorous treatment. Subdural empyema, ventriculitis and later brain abscess were detected by serial brain sonograms. He died of central nervous system failure, gastrointestinal bleeding and renal failure on the eighteenth hospitalization day. PMID:2576904

Hou, J W; Teng, R J; Lee, C Y

1989-01-01

253

Henoch-Schönlein purpura nephritis complicated by reversible posterior leukoencephalopathy syndrome.  

PubMed

We report a young female patient with Henoch-Schönlein purpura (HSP) nephritis complicated by reversible posterior leukoencephalopathy syndrome (RPLS). The patient suddenly showed generalized seizures and cortical blindness with severe hypertension due to renal insufficiency approximately 1 year after cessation of corticosteroid treatment for HSP nephritis. Magnetic resonance imaging (MRI) demonstrated bilateral abnormal signals mainly in the cerebellum and white matter of the occipital lobe. Clinical symptoms quickly improved in conjunction with disappearance of abnormal signals on brain MRI after starting control of hypertension and continuous hemodiafiltration with steroid pulse therapy and plasmapheresis. RPLS may be caused by vasculitis and also by hemodynamic change due to severe hypertension in HSP, particularly in patients with nephropathy. In such cases intensive treatment should be performed as soon as possible to avoid neurological sequelae. PMID:17205213

Sasayama, Daimei; Shimojima, Yasuhiro; Gono, Takahisa; Kaneko, Kazuma; Matsuda, Masayuki; Ikeda, Shu-ichi

2007-10-01

254

Treatment of severe henoch-schonlein purpura nephritis with mycophenolate mofetil.  

PubMed

Henoch-Schonlein purpura (HSP) is the most common childhood vasculitis. Renal involvement in HSP is one of the major causes of chronic renal failure in children. It is important to start effective and relatively safe medication to prevent end-stage renal disease (ESRD). Mycophenolate mofetil (MMF) appears to be a promising therapeutic agent in many autoimmune diseases such as lupus nephritis and vasculitis. Herein, we describe the treatment with MMF of three patients with HSP nephritis. In two cases with rapidly progressive glomerulonephritis without response to steroid, after treatment with MMF, significant improvement in kidney function and proteinuria were observed. In another patient with HSP nephritic-nephrotic syn-drome who showed resistance to steroid, MMF offered a favorable effect. MMF seems to be a promising therapeutic agent in the treatment of the severe HSP nephritis. PMID:24969202

Nikibakhsh, Ahmad Ali; Mahmoodzadeh, Hashem; Karamyyar, Mohamad; Hejazi, Sasan; Noroozi, Mehran; Macooie, Ali Agayar

2014-01-01

255

High Prevalence of Sinusitis in Children with Henoch-Sch?nlein Purpura  

PubMed Central

We evaluated the prevalence and the types of infectious foci in oral as well as ear, nose, and throat diseases, and we examined incidence of renal involvement with active treatment for focal infection in children with Henoch-Schönlein Purpura. A total of 96 children who presented at Aichi Children's Health and Medical Center and were diagnosed as having HSP were evaluated for infectious foci in the ear, nose, throat, and oral cavities. Seventy-one of 96 children (74.0%) had some type of infectious lesion, such as sinusitis or tonsillitis, and the prevalence of sinusitis was the highest (51 cases, 53.7%). In 44 HSP patients without renal involvement at the first examination, the incidence of nephritis was lower (13.6%) than in previous reports (17–54%) due to our aggressive intervention for infectious foci.

Nakaseko, Haruna; Uemura, Osamu; Nagai, Takuhito; Yamakawa, Satoshi; Hibi, Yoshiko; Yamasaki, Yasuhito; Yamamoto, Masaki

2011-01-01

256

An adult patient with Henoch-Sch?nlein purpura and non-occlusive mesenteric ischemia  

PubMed Central

Background Onset of Henoch-Schönlein purpura (HSP) in middle age is uncommon, and adults with renal or gastrointestinal involvement present with more severe disease than do similar pediatric patients. Case presentation We present the case of a 69-year-old male with HSP who, after treatment with steroids, cyclophosphamide, and continuous intravenous prostaglandin E1 (PGE1), died as a result of severe gastrointestinal involvement with non-occlusive mesenteric ischemia (NOMI). Vascular narrowing associated with the NOMI improved after catheter injection of PGE1 and prednisolone, but the patient died of bleeding from an exposed small vessel. At autopsy there was no active vasculitis in the jejunal submucosa. Conclusion Treatment with PGE1 and prednisolone might improve small-vessel vasculitis associated with NOMI.

2013-01-01

257

[The case of Henoch-Schönlein Purpura associated with Blastocystis hominis].  

PubMed

Blastocystis hominis (B. hominis) is a parasite that often causes gastrointestinal symptoms in patients with immune deficiency and has a controversial pathogenicity in healthy people, although some symptoms are reported outside of the gastrointestinal system in healthy persons. Henoch-Schönlein Purpura (HSP) vasculitis is an acute autoimmune disease characterised by IgA storage of small vessels that is believed to include infectious factors in its aetiology. A 30-month follow-up with a boy diagnosed with HSP being treated with steroid therapy showed that he had recurrent symptoms within two days, and B. hominis was detected in the faecal analysis. His symptoms including rash, abdominal pain, and arthritis improved after treatment with steroid and co-trimaksazol. This paper is the first to present a case of HSP associated with B. hominis. PMID:23955912

Tutanç, Murat; Silfeler, Ibrahim; Ozgür, Tümay; Motor, Vicdan Köksald?; Kurto?lu, Ahmet Ibrahim

2013-01-01

258

Autoerythrocyte sensitization syndrome presenting with general neurodermatitis: factitious purpura or psychophysiological entity?  

PubMed

Autoerythrocyte sensitization syndrome (AES) is a disorder characterized by recurrent, spontaneous, painful bruising in patients with underlying psychosis and neurosis. Its pathogenesis is uncertain. Purpuric lesions reproduced by injections of washed autologous erythrocytes suggest an autoimmune etiology. The authors described a 50-year-old woman who presented with recurrent episodes of painful bruising for 8 months, along with a 2-year history of general chronic neurodermatitis. A diagnosis of AES was made on the basis of the clinical symptoms and laboratory examinations results: the positive autoerythrocyte sensitization test and psychophysiologic derangement. However, the noticeable symptoms, simultaneously identical distribution of the ecchymosis and neurodermatitis lesions, as well as concomitant symptom of itching over the ecchymosis, created doubts regarding the real etiopathogenesis of the ecchymosis and whether the ecchymosis in the present patient were just factitious purpura. The authors discuss the rarity of this case, which to the authors' knowledge, had not been previously reported in the literature. PMID:23205328

Zhao, Hengguang; Luo, Fuling; Li, Hui

2012-12-01

259

Advances in our understanding of the pathogenesis of Henoch-Schönlein purpura and the implications for improving its diagnosis.  

PubMed

Henoch-Schönlein purpura (HSP) is a leukocytoclastic vasculitis classically characterized by palpable purpura, arthritis, abdominal pain and renal disease. In this article, we summarize our current understanding of the pathogenesis of HSP and the implications for improving its diagnosis. Although the pathogenesis of HSP is not fully understood yet, exciting new information has emerged in recent years, leading to a better understanding of its pathogenesis. Here, we discuss genetic predisposition, immunoglobulins with a particular emphasis on IgA1, activated complements, cytokines and chemokines, abnormal coagulation and autoantibodies in the underlying pathogenic mechanisms. Finally, diagnostic criteria for HSP developed by institutions such as the American College of Rheumatology and the European League against Rheumatism/Paediatric Rheumatology European Society were proposed to improve early detection and diagnosis. PMID:24215411

Park, Se Jin; Suh, Jin-Soon; Lee, Jun Ho; Lee, Jung Won; Kim, Seong Heon; Han, Kyoung Hee; Shin, Jae Il

2013-12-01

260

Similar disturbances in B cell activity and regulatory T cell function in Henoch-Schonlein purpura and systemic lupus erythematosus  

Microsoft Academic Search

The immunoglobulin synthesizing activities of peripheral mononuclear cells (MNC) from five patients with Henoch-Schonlein purpura (HSP) and eight patients with active systemic lupus erythematosus (SLE) were compared. Cumulative amounts of IgM, IgG, and IgA synthesized and secreted by unstimulated and PWM-stimulated patient cells over a 12-day period were determied in a solid-phase radioimmunoassay. In unstimulated control cultures mean rates of

M. G. Beale; G. S. Nash; M. J. Bertovich; R. P. MacDermott

1982-01-01

261

Anesthetic management of a patient with Henoch-Schonlein purpura for drainage of cervical lymphadenitis: A case report  

PubMed Central

Henoch-Schonlein purpura (HSP) is a multisystem disease and immunoglobulin A-mediated vasculitis with a self-limited course affecting the skin, joints, gastrointestinal tract, and kidneys. Severe renal and central nervous system disease may lead to life-threatening conditions, and immunosuppressive agents and plasmapheresis may be needed. We report successful management of a 6-year-old patient with HSP for drainage of cervical lymphadenitis.

Hasija, Neha; Taxak, Susheela; Bhardwaj, Mamta; Vashist, Kirti

2014-01-01

262

Anesthetic management of a patient with Henoch-Schonlein purpura for drainage of cervical lymphadenitis: A case report.  

PubMed

Henoch-Schonlein purpura (HSP) is a multisystem disease and immunoglobulin A-mediated vasculitis with a self-limited course affecting the skin, joints, gastrointestinal tract, and kidneys. Severe renal and central nervous system disease may lead to life-threatening conditions, and immunosuppressive agents and plasmapheresis may be needed. We report successful management of a 6-year-old patient with HSP for drainage of cervical lymphadenitis. PMID:24843348

Hasija, Neha; Taxak, Susheela; Bhardwaj, Mamta; Vashist, Kirti

2014-04-01

263

Effective therapy for severe Henoch-Schonlein purpura nephritis with prednisone and azathioprine: A clinical and histopathologic study  

Microsoft Academic Search

Objectives: To validate a scoring system to assess the severity of renal lesions and to correlate histology with clinical findings. We also examined the efficacy of treatment with prednisone (1 to 2 mg\\/kg\\/d) and azathioprine (1 to 2 mg\\/kg\\/d) for severe Henoch-Schonlein purpura (HSP) nephritis. Methods: Twenty patients were evaluated retrospectively. All underwent biopsy before treatment, and 13 underwent biopsy

Bethany J Foster; Chantal Bernard; Keith N Drummond; Atul K Sharma

2000-01-01

264

Henoch-Schönlein purpura following influenza vaccinations during the pandemic of influenza A (H1N1)  

Microsoft Academic Search

Although the etiology of Henoch-Schönlein purpura (HSP) remains unclear, influenza vaccinations have been implicated as possible\\u000a triggers for HSP. We describe four patients with HSP following influenza vaccinations which developed during the pandemic\\u000a of influenza A (H1N1) 2009 and review the literature concerning HSP associated with this vaccine. HSP in patients developed\\u000a in October and November, 2009. Four patients exhibited

Toru Watanabe

2011-01-01

265

Elevated serum interleukin-33 levels in patients with Henoch-Schönlein purpura.  

PubMed

Henoch-Schönlein purpura (HSP) is the most common systemic vasculitis and is known as an immunoglobulin (Ig) A related immune complex-mediated disease. However, the molecular mechanisms in the development of HSP are not yet fully understood. Herein, we investigated the serum levels of Interleukin (IL)-33 and soluble ST2 (sST2) in HSP patients and their association with disease severity and IgA autoantibodies production. The serum levels of IL-33 and sST2 were measured by double antibody sandwich enzyme-linked immunosorbent assay (ELISA) in the serum of 33 patients with HSP and 22 controls. Serum levels of IgA anti-endothelial cell antibodies (AECA) and IgA anticardiolipin antibodies (ACA) in HSP patients were detected by double antigen sandwich ELISA. Our results indicated that serum levels of IL-33 but not sST2 were significantly elevated in patients with HSP in acute stage and restored to normal levels in convalescent stage. Moreover, serum IL-33 levels were correlated with the severity of HSP and serum concentrations of AECA-IgA and ACA-IgA. Taken together, we show firstly that serum IL-33 is abnormally elevated in HSP patients. IL-33 might be associated with the IgA autoantibodies production in the pathogenesis of HSP. PMID:22836779

Chen, Tao; Jia, Rui-zhen; Guo, Zai-pei; Cao, Na; Li, Meng-meng; Jiao, Xiao-yan

2013-03-01

266

Self-organizing phenomena induced by LLLT in Henoch-Schoenlein purpura  

NASA Astrophysics Data System (ADS)

Henoch-Schoenlein purpura is characterized by vasculitis of small vessels, particularly those of the skin, gastrointestinal tract, and kidney. Patients have characteristic purpuric skin rash plus all or some of the following: migratory polyarthralgias or polyarthritis, colicky abdominal pain, nephritis. Because until now there is no satisfactory treatment, we applied low level laser therapy (LLLT) in order to compare it with the classical therapy. Twenty-three children (2-15 years of age) have been treated at debut of the disease. They were randomly divided: group A (11 children) received LLLT; group B (12 children) was administrated classical therapy. Two GaAlAs diode lasers (670 nm and 830 nm) were used. The density of energy (4-8 J/cm2), irradiating frequency (2.4 Hz) was applied one session daily, using scanning technique under a special treatment protocol on cutaneous purpuric areas (20 sessions). The best results were obtained in laser group. Despite the complex medication, some patients from group B fell back into the former state after apparent improvement, and two children developed nephritis. The results could be explained by self-organization. LLLT is acting as a trigger factor causing certain systemic effects through circulating blood and a response of the entire immune system, by way of synergetic mechanisms.

Ailioaie, Laura; Ailioaie, C.

2001-06-01

267

The diagnostic usefulness of video capsule endoscopy in adolescent immunoglobulin a vasculitis (henoch-schönlein purpura).  

PubMed

Immunoglobulin (Ig) A vasculitis (IgAV), previously known as Henoch-Schönlein purpura, is a systemic IgA-mediated leukocytoclastic vasculitis that usually affects children. We report the usefulness of video capsule endoscopy in 2 adolescent patients with IgAV having gastrointestinal involvement. Both patient 1, a 15-year-old girl, and patient 2, a 14-year-old boy, presented with purpuric rash and abdominal pain. Video capsule endoscopy showed multiple areas of purpuric erythema throughout the small bowel in both patients and showed multiple ulcers with bleeding in patient 2. Patient 1 responded well to oral prednisolone at a dose of 0.5 mg/kg/day. However, in patient 2, prednisolone at a dose of 0.5 mg/kg/day failed to control the symptoms; therefore, the dose was increased to 1 mg/kg/day to provide relief. Video capsule endoscopy was safe in both cases and produced no side effects. In conclusion, video capsule endoscopy is a useful tool for evaluating small bowel lesions in patients with IgAV and provides valuable information for the treatment of IgAV with gastrointestinal involvement. PMID:24805100

Li, Min; Omi, Tokuya; Matano, Yoko; Fujimori, Shunji; Kawana, Seiji

2014-01-01

268

Plastic surgical treatment of purpura fulminans: Long-term follow-up of two patients.  

PubMed

Purpura fulminans (PF) is a rapidly progressing, potentially life-threatening condition characterised by disseminated intravascular coagulation (DIC) and haemorrhagic infarction of the skin. Plastic surgical treatment of PF has never been reported in Scandinavia. The aim of this report was to review plastic surgical treatment of PF and the long-term results of two patients treated in our department. Both patients presented to a community hospital with skin lesions looking like simple traumatic skin bleeds a period after a Varicella infection. They were initially treated at the community hospitals with broad-spectrum antibiotics and adjunctive therapies. When their condition permitted, they were transferred to the department of paediatrics of Skåne University Hospital where their DIC was treated further. The patients were transferred to the department of plastic and reconstructive surgery, when medically stable, and operated on with debridement, and amputation of a toe in one patient, and the application of autologous skin grafts. The children made an excellent recovery and were discharged home after 1.5 months and 3 weeks, respectively. At follow-up, 14 years and 8 years later, respectively, the patients were fully recovered and no secondary corrections were indicated. In conclusion, debridement of necrotic tissue should be performed in a department of plastic and reconstructive surgery as soon as the child is clinically stable, and skin grafting when the wound bed permits it. Follow-up should be performed in the same fashion as for full-thickness burns. PMID:23210495

Hansson, Emma; Tedgård, Ulf; Becker, Magnus

2013-04-01

269

Successful treatment of recurrent Henoch-Schönlein purpura in a renal allograft with plasmapheresis.  

PubMed

Acute and severe cases of Henoch-Schönlein purpura (HSP) nephritis have been treated with plasmapheresis (PA) in both adults and children. It has been used either alone or with steroids, antiplatelets or cytoxic drugs. Generally, renal function has been shown to improve when PA is utilized. The role of PA in recurrent HSP after renal transplantation is unclear and has not been well described in the literature. We report a 29-year-old female with HSP who developed end-stage renal disease and subsequently underwent a renal transplantation with eventual loss of the allograft 5 years later due to recurrent HSP nephritis. Retransplantion was performed and the patient developed active HSP nephritis in her second allograft within a week after transplantation. In an effort to preserve her allograft, four cycles of PA were performed. Her proteinuria resolved and renal biopsies afterwards demonstrated marked reduction in mesangial IgA deposition. We conclude that PA may be useful in recurrent HSP nephritis, especially when used early. The risk of additional immunosuppression caused by PA needs to be considered. More studies need to be done to evaluate the efficacy of PA in this setting as well as to define the optimal treatment regimen. PMID:17973963

Lee, J; Clayton, F; Shihab, F; Goldfarb-Rumyantzev, A

2008-01-01

270

Immune thrombocytopaenic purpura: an autoimmune cross-link between infections and vaccines.  

PubMed

Immune thrombocytopaenic purpura (ITP) is an autoimmune systemic disease detectable by the presence of low blood platelets count (<10(5)/µl) and the production of autoantibodies against glycoproteins expressed on the platelet surface. The clinical course is often acute, and life-threatening events may occur especially in children, with 52% of paediatric patients recovering either spontaneously or after treatment. A chronic ITP evolution is observed in 64% of adults, of whom 12% will develop an overlapping autoimmune disease. Several microbial agents such as CagA-positive Helicobacter pylori or Candida albicans and a number of viruses including CMV, EBV or HIV can potentially trigger ITP through molecular mimicry. Moreover, ITP improves after treatment of the underlying infection. Similarly, vaccines such as MMR may prompt ITP (IRR 5.48, 1.61-18.64, p < 0.006). Early recognition of the underlying microbial trigger and the removal of modifiable aetiopathogenetic factors should be integrated as a complementary treatment strategy in all patients who do not readily improve with standard ITP care. PMID:24763539

Rinaldi, M; Perricone, C; Ortega-Hernandez, O-D; Perricone, R; Shoenfeld, Y

2014-05-01

271

Elevated Serum Levels of Visfatin in Patients with Henoch-Sch?nlein Purpura  

PubMed Central

Background Henoch-Schönlein purpura (HSP) is an immune complex-mediated disease predominantly characterized by the deposition of circulating immune complexes containing immunoglobulin A (IgA) on the walls of small vessels. Although the pathogenesis of HSP is not yet fully understood, some researchers proposed that B-cell activation might play a critical role in the development of this disease. Objective To investigate the serum levels of visfatin (pre-B-cell colony-enhancing factor), B-cell-activating factor (BAFF), and CXCL13, and to analyze their association with disease severity. Methods The serum levels of visfatin, BAFF, and CXCL13 were measured by using a double-antibody sandwich enzyme-linked immunosorbent assay (ELISA) in 43 patients with HSP and 45 controls. The serum levels of IgA anticardiolipin antibodies (ACA) were detected by using a double-antigen sandwich ELISA. Results Levels of visfatin but not BAFF and CXCL13 were significantly elevated in the sera of patients with HSP in the acute stage, and restored to normal levels in the convalescent stage. Furthermore, serum levels of visfatin were significantly higher in patients with HSP having renal involvement than in those without renal involvement. Serum levels of visfatin were correlated with the severity of HSP and serum concentration of ACA-IgA. Conclusion We show for the first time that the serum levels of visfatin are abnormally elevated in patients with HSP. Visfatin may be associated with the pathogenesis of HSP.

Cao, Na; Chen, Tao; Li, Meng-Meng; Jiao, Xiao-Yan

2014-01-01

272

Elevated serum heme oxygenase-1 and insulin-like growth factor-1 levels in patients with Henoch-Schonlein purpura  

Microsoft Academic Search

The aim of this study is to investigate the levels of heme oxygenase-1 (HO-1), insulin-like growth factor-1 (IGF-1) and oxidative\\u000a stress parameters including malondialdehyde (MDA), total antioxidant capacity (T-AOC) and the activities of total superoxide\\u000a dismutase (SOD), glutathione peroxidase (GSH-PX), catalase (CAT) for exploring the correlations between these biological indexes\\u000a and the clinical manifestations in Henoch-Schonlein purpura (HSP) patients. 36

Tao Chen; Zai-pei Guo; Yu-hong Zhang; Ying Gao; Hong-jie Liu; Jing-yi Li

2011-01-01

273

HLA-B35, a common genetic trait, in a familial case of Henoch-Schoenlein purpura and Berger's disease.  

PubMed

Nephritis characterized by IgA mesangial depositions has been described both in Henoch-Schoenlein purpura (HSP) and in Berger's disease (BD), but common genetic traits are still uncertain. We report here the case of two brothers, the first affected by HSP with persistent nephritis and the second by BD, accidentally discovered as silent microhematuria 1 year after HSP onset in the first brother. HLA genotyping demonstrated the presence of HLA-B35 in both patients. Our findings reinforce the need to screen for urinary abnormalities in family members of patients affected by HSP nephritis to identify a silent IgA nephropathy. PMID:24782055

Pellegrin, M C; Matarazzo, L; Neri, E; Pennesi, M; Crovella, S

2014-01-01

274

Increased serum HMGB1 levels in patients with Henoch-Schönlein purpura.  

PubMed

High-mobility group box-1 (HMGB1) has been implicated as a pro-inflammatory cytokine in the pathogenesis of various inflammatory and autoimmune diseases. However, information about HMGB1 in Henoch-Schönlein purpura (HSP) is still unclear. Herein, we investigated the role of HMGB1 in patients with HSP and the pro-inflammatory effects of HMGB1 on human dermal microvascular endothelial cell line (HMEC-1). Serum HMGB1 levels in patients with HSP together with patients with allergic vasculitis (AV) and urticarial vasculitis (UV) were detected by enzyme-linked immunosorbent assay (ELISA). HMEC-1 cells were treated with HMGB1 at concentrations ranging from 4 ng/ml to 100 ng/ml. Serum HMGB1 levels were significantly increased in patients with HSP, AV and UV, when compared with those in control group. Moreover, abundant cytoplasmic expression of HMGB1 was observed in endothelial cells in lesional skin of HSP patients. Using membrane cytokine antibody array, we indicate that HMGB1 markedly induced TNF-? and IL-6 release in cultured supernatant. Furthermore, by real-time quantitative PCR and ELISA, the effects of HMGB1 on these cytokines production in HMEC-1 cells were established. Finally, Western blot data revealed that HMGB1 can induce phosphorylation of inhibitor of ?B-? (I?B?) and the nuclear translocation of nuclear factor-?B (NF-?B) p65 in HMEC-1 cells. In conclusion, this study provides first observations on the association of HMGB1 with HSP. We suggest that HMGB1 may be an important mediator of endothelial inflammation through the induction of TNF-? and IL-6 production and may play a crucial role in the pathogenesis of HSP. PMID:24758390

Chen, Tao; Guo, Zai-Pei; Wang, Wen-Ju; Qin, Sha; Cao, Na; Li, Meng-Meng

2014-06-01

275

Role of free oxygen radicals and prostanoids in the pathogenesis of Henoch-Schönlein Purpura.  

PubMed

The pathogenesis of Henoch-Schonlein Purpura (HSP) is still controversial. The aim of our study was to investigate the role of oxidative stress and cyclooxygenase (CO) pathway products in the pathogenesis of HSP. In order to investigate this, malondialdehyde (MDA) levels, indicating lipid peroxidation, prostaglandin E (PGE)-like activity as inflammatory mediator and vitamin E (vit-E) levels indicating anti-oxidant status were studied in a group of 10 children with HSP (five girls and five boys, aged 6-21 years, mean 10.7 years), both in the acute and recovery phase of the disease and in five age and sex-matched healthy children as a control group. The patients were also grouped into low and high clinical score groups. Plasma levels of MDA and PGE-like activity were significantly elevated in the active phase of HSP compared to the recovery phase. Vit-E levels were significantly reduced in the active phase compared to the recovery phase. The plasma levels of PGE-like activity of the patients obtained in the active phase were significantly higher than the levels of the control group, whereas the levels of the recovery phase were significantly lower than in the control group. No such difference between the controls and MDA and vit-E levels in the patient group was shown. No correlation between the clinical scores and the parameters studied could be found. Our findings indicate that oxidant stress and CO pathway products may play a role in the pathogenesis of HSP. PMID:9844990

Buyan, N; Erba?, D; Akkök, N; Oz, E; Bibero?lu, G; Hasano?lu, E

1998-09-01

276

Human protein C concentrate in the treatment of purpura fulminans: a retrospective analysis of safety and outcome in 94 pediatric patients  

Microsoft Academic Search

ABSTRACT: INTRODUCTION: Purpura fulminans (PF) is a devastating complication of uncontrolled systemic inflammation, associated with high incidence of amputations, skin grafts and death. In this study, we aimed to clarify the clinical profile of pediatric patients with PF who improved with protein C (PC) treatment, explore treatment effects and safety, and to refine the prognostic significance of protein C plasma

Alex Veldman; Doris Fischer; Flora Y Wong; Wolfhart Kreuz; Michael Sasse; Bruno Eberspächer; Ulrich Mansmann; Rudolf Schosser

2010-01-01

277

Henoch-Schönlein purpura nephritis in a patient with IgG4-related disease: a possible association.  

PubMed

We report a case of Henoch- Schönlein purpura nephritis (HSPN) associated with tubulointerstitial nephritis (TIN) and chronic sclerosing sialoadenitis. The patient is a 75-year-old Japanese woman who had bilateral submandibular gland swelling, palpable purpura on the lower legs, and decreased renal function with hematoproteinuria and marked hypocomplementemia, but no skin lesion suggestive of systemic lupus erythematosus (SLE), and did not fulfill the classification criteria for SLE. Her serum IgG4 level was high and immunostaining of renal biopsies revealed marked infiltration by IgG4-positive plasma cells in the interstitium, confirming the diagnosis of IgG4- related disease. On the other hand, glomeruli showed endocapillary proliferative glomerulonephritis with mesangial IgA and C3 deposition demonstrated by immunofluorescence staining, which were typical glomerular lesions for HSPN. The glomerular and tubulointerstitial lesions responded to steroid therapy dramatically, and her renal function recovered to within the normal range within a month. This case suggests a possible new association between HSPN and IgG4-related disease. PMID:22841022

Ito, Kiyoaki; Yamada, Kazunori; Mizushima, Ichiro; Aizu, Motohiko; Fujii, Hiroshi; Mizutomi, Kazuaki; Matsumura, Masami; Hayashi, Kenshi; Yamagishi, Masakazu; Umehara, Hisanori; Yamaguchi, Yutaka; Nagata, Michio; Kawano, Mitsuhiro

2013-03-01

278

Hemostatic Function and Transfusion Efficacy of Apheresis Platelet Concentrates Treated with Gamma Irradiation in Use for Thrombocytopenic Patients  

PubMed Central

Summary Background During the transfusion of blood components, the transfer of allogeneic donor white blood cells (WBCs) can mediate transfusion-associated graft-versus-host disease (TA-GVHD). To minimize the reaction, exposure of blood products to gamma irradiation is currently the standard of care. The aim of our study was to evaluate and compare hemostatic function, transfusion efficacy, and safety of gamma-irradiated single-donor apheresis platelet concentrates (PCs) and of conventional non-irradiated PCs in patients with chemotherapy-induced thrombocytopenia. Methods 20 double-dose single-donor leukoreduced PCs were split in two identical units; one was gamma-irradiated with 25 Gy (study arm A) and the other remains non-irradiated (study arm B). Both units were stored under equal conditions. Hematologic patients were randomly assigned to receive gamma-irradiated or conventional non-irradiated PCs. Hemostatic function was evaluated by thrombelastography (TEG). TEG measurements were taken pre transfusion and 1 and 24 h post transfusion. TEG profiles were measured, noting the time to initiate clotting (R), the angle of clot formation (?), and the maximum amplitude (clot strength (MA)). Whole blood samples were collected from these thrombocytopenic patients at 1 and 24 h for PLT count increments (CIs) and corrected count increments (CCIs) with assessments of transfusion efficacy. Time to next PLT transfusion, transfusion requirement of RBCs, active bleeding, and adverse events (AEs), were analyzed. Results No differences could be found in hemostatic function parameters (MA, R, and ?) between study arms A and B (all p values > 0.096) pre transfusion as well as 1 and 24 h post transfusion. No differences between study arms A and B were observed for mean (± standard deviation (SD)) 1-hour CCI (12.83 ± 6.33 vs. 11.59 ± 5.97) and 24-hour CCI (6.56 ± 4.10 vs. 5.76 ± 4.05). Mean 1-hour CI and 24-hour CI were not significantly different in both study arms (p = 0.254 and p = 0.242 respectively). Median time to the next PC transfusion after study PC was not significantly different between groups: (2.4 vs. 2.2 days, p = 0.767). No differences could be found in transfusion requirement of red blood cells (p = 0.744) between both study arms. There were also no regarding bleeding, adverse events, and acute transfusion reaction(s). Conclusions This study confirms safety of gamma-irradiated PCs for treatment thrombocytopenia. Hemostatic function, transfusion efficacy, bleeding, and safety of single-donor apheresis PCs treated with gamma irradiation versus untreated control PCs are comparable.

Zhu, Mei; Xu, Wei; Wang, Bao-Long; Su, Hong

2014-01-01

279

Medical Journal. Volume 92, Number 1, 1970.  

National Technical Information Service (NTIS)

Contents: The immunology of tumors; The appearance of antigamma globulin in acute glomerulonephritis and some other diseases in childhood; Generalized lupus erythematosus - A diagnostic problem; Megakaryocytic thrombocytopenic purpura (A report on 56 case...

1971-01-01

280

Vitamin K Deficiency Bleeding Presenting as Nodular Purpura in Infancy: A Rare and Life-Threatening Entity  

PubMed Central

Vitamin K deficiency bleeding (VKDB) disorder is an uncommon entity, which occurs due to inadequate activity of vitamin K-dependant coagulation factors. An 8-months-old exclusively breast-fed male infant presented with multiple, purpuric and nodular non-collapsible swellings on trunk of 4 days duration. Investigations revealed raised activated partial thromboplastin time and prothrombintime. Fibrinogen level and platelet counts were normal. Late VKDB usually presents as intra-cranial or mucosal hemorrhages.[1] Though skin and mucosal bleeding may occur in 1/3rd of infants with VKDB, ‘nodular purpura’ is not the common presenting feature. Earlier recognition of VKDB and immediate investigation/treatment helps prevent the potentially fatal outcome of the disease. Very little is mentioned about this entity in dermatology literature.

Gahalaut, Pratik; Chauhan, Sandhya

2013-01-01

281

A case of isotretinoin-induced purpura annularis telangiectodes of Majocchi and review of substance-induced pigmented purpuric dermatosis.  

PubMed

IMPORTANCE Medications as well as chemical and food exposures have been causally linked to the development of pigmented purpuric dermatosis (PPD). We describe herein what is to our knowledge the first reported case of isotretinoin-induced PPD. OBSERVATIONS A woman in her 30s presented with purpura annularis telangiectodes of Majocchi on the lower extremities 2 months after initiating isotretinoin for the treatment of refractory nodulocystic acne. CONCLUSIONS AND RELEVANCE We believe isotretinoin was the most likely causative agent in this case because the lesions began after initiation of isotretinoin treatment and resolved shortly after its termination, and the pathologic findings were consistent with other described cases of drug-induced PPD. The lesions have continued to fade, and no new lesions have developed in a 3-month follow-up period. Drug-induced PPD is distinct from idiopathic PPD, and it is important to consider isotretinoin as a potential inciting agent. PMID:24284887

Kaplan, Rachel; Meehan, Shane A; Leger, Marie

2014-02-01

282

[Influence of trematode invasion and zinc ions on the histometric peculiarities of haemocytes and some hematological indices of Planorbarius purpura (Gastropoda: Pulmonata: Bulinidae)].  

PubMed

Cellular components of the Planorbarius purpura hemolymph are represented by three phyla of haemocytes (prohemocytes, eosinophilis microgranulocytes, and basophilis granulocytes) and vesicular cells. As a result of the invasions of P. purpura with the trematode Echinoparyphium aconiatum, changes of the linear dimensions of granular hemocytes and their nuclei took place. Moreover, an increase of the hemocytes' number per l mm3 of hemolymph and change of the percentages of different hemocyte types were recorded. Under the influence of zinc ions, linear dimensions of prohemocytes and their nuclei (at 10 MPCns of the toxicant) were changed. In granular hemocytes and abnormalities of all histometrical and hematological parameters were observed. All cytometrical, karyometrical, and hematological alterations were expressed more clearly in infested mollusks than in non-infested ones, and occurred usually under lower concentrations of zinc ions. PMID:20349633

Kirichuk, G E; Stadnichenko, A P

2010-01-01

283

Drotrecogin alfa (activated) in patients with severe sepsis presenting with purpura fulminans, meningitis, or meningococcal disease: a retrospective analysis of patients enrolled in recent clinical studies  

Microsoft Academic Search

INTRODUCTION: We report data from adult and pediatric patients with severe sepsis from studies evaluating drotrecogin alfa (activated) (DrotAA) and presenting with purpura fulminans (PF), meningitis (MEN), or meningococcal disease (MD) (PF\\/MEN\\/MD). Such conditions may be associated with an increased bleeding risk but occur in a relatively small proportion of patients presenting with severe sepsis; pooling data across clinical trials

Jean-Louis Vincent; Simon Nadel; Demetrios J Kutsogiannis; RT Noel Gibney; S Betty Yan; Virginia L Wyss; Joan E Bailey; Carol L Mitchell; Samiha Sarwat; Stephen M Shinall; Jonathan M Janes

2005-01-01

284

Cocaine-associated retiform purpura: a C5b-9-mediated microangiopathy syndrome associated with enhanced apoptosis and high levels of intercellular adhesion molecule-1 expression.  

PubMed

Cocaine-associated retiform purpura is a recently described entity characterized by striking hemorrhagic necrosis involving areas of skin associated with administration of cocaine. Levamisole, an adulterant in cocaine, has been suggested as the main culprit pathogenetically. Four cases of cocaine-associated retiform purpura were encountered in the dermatopathology practice of C. M. Magro. The light microscopic findings were correlated with immunohistochemical and immunofluorescence studies. All 4 cases showed a very striking thrombotic diathesis associated with intravascular macrophage accumulation. Necrotizing vasculitis was noted in 1 case. Striking intercellular adhesion molecule-1 (ICAM-1)/CD54 expression in vessel wall along with endothelial expression of caspase 3 and extensive vascular C5b-9 deposition was observed in all biopsies examined. Cocaine-induced retiform purpura is a C5b-9-mediated microvascular injury associated with enhanced apoptosis and prominent vascular expression of ICAM-1, all of which have been shown in prior in vitro and in vivo murine models to be a direct effect of cocaine metabolic products. Antineutrophilic cytoplasmic antibody and antiphospholipid antibodies are likely the direct sequelae of the proapoptotic microenvironment. The inflammatory vasculitic lesion could reflect the downstream end point reflective of enhanced ICAM-1 expression and the development of antineutrophilic cytoplasmic antibody. Levamisole likely works synergistically with cocaine in the propagation of this syndromic complex. PMID:23392134

Magro, Cynthia M; Wang, Xuan

2013-10-01

285

Autologous Peripheral Blood Stem Cell Transplantation in Patients With Life Threatening Autoimmune Diseases  

ClinicalTrials.gov

Purpura, Schoenlein-Henoch; Graft Versus Host Disease; Anemia, Hemolytic, Autoimmune; Rheumatoid Arthritis; Churg-Strauss Syndrome; Hypersensitivity Vasculitis; Wegener's Granulomatosis; Systemic Lupus Erythematosus; Giant Cell Arteritis; Pure Red Cell Aplasia; Juvenile Rheumatoid Arthritis; Polyarteritis Nodosa; Autoimmune Thrombocytopenic Purpura; Takayasu Arteritis

2005-06-23

286

Henoch-Schönlein purpura (HSP) and high-dose immunoglobulin treatment in patient with familiar prostatic adenocarcinoma.  

PubMed

A 52-y old man was admitted to our Department because of abdominal pains and diarrhea with fresh blood, with concomitant purpura over the whole body and edema of the both tarsal joints. The medical history of the patient included skin changes of similar character identified once 10-12 y before. The family history revealed prostate cancer (brother and father) and pulmonary carcinoma (mother and mother's sister). An administration of immunoglobulins in the course of HSP is a non-standard clinical procedure and in case of our patient--clinically effective. In the literature, we have found only few articles about intravenous immunoglobulin treatment for acute, adult-onset HSP and only one article about GI bleeding from colonic ecchymoses in HSP. In these cases HSP wasn't associated with prostate cancer. In the first article, authors had seen dramatic responses to intravenous immunoglobulin, like in the case presented by us. IV-IG acts as an immunomodulator by suppression of antibody production, Fc-receptor blockade and anti-idiotypic reaction. In our case, the last two mechanisms could be perceived as favorable effects of IV-IG. PMID:24231833

Sobieszcza?ska, Ma?gorzata; Tubek, S?awomir; Poplicha, Dagmara; Grabelus, Anna; Pawe?czak, Jacek

2014-02-01

287

Adhesion of Neisseria meningitidis to Dermal Vessels Leads to Local Vascular Damage and Purpura in a Humanized Mouse Model  

PubMed Central

Septic shock caused by Neisseria meningitidis is typically rapidly evolving and often fatal despite antibiotic therapy. Further understanding of the mechanisms underlying the disease is necessary to reduce fatality rates. Postmortem samples from the characteristic purpuric rashes of the infection show bacterial aggregates in close association with microvessel endothelium but the species specificity of N. meningitidis has previously hindered the development of an in vivo model to study the role of adhesion on disease progression. Here we introduced human dermal microvessels into SCID/Beige mice by xenografting human skin. Bacteria injected intravenously exclusively associated with the human vessel endothelium in the skin graft. Infection was accompanied by a potent inflammatory response with the secretion of human inflammatory cytokines and recruitment of inflammatory cells. Importantly, infection also led to local vascular damage with hemostasis, thrombosis, vascular leakage and finally purpura in the grafted skin, replicating the clinical presentation for the first time in an animal model. The adhesive properties of the type IV pili of N. meningitidis were found to be the main mediator of association with the dermal microvessels in vivo. Bacterial mutants with altered type IV pili function also did not trigger inflammation or lead to vascular damage. This work demonstrates that local type IV pili mediated adhesion of N. meningitidis to the vascular wall, as opposed to circulating bacteria, determines vascular dysfunction in meningococcemia.

Melican, Keira; Michea Veloso, Paula; Martin, Tiffany; Bruneval, Patrick; Dumenil, Guillaume

2013-01-01

288

Circulating adhesion molecules ICAM-1, E-selectin, and von Willebrand factor in Henoch-Sch?nlein purpura.  

PubMed Central

Adhesion molecules play an important part in leucocyte transendothelial migration and thus may provide a useful marker of surface expression at inflammatory sites. In 20 patients with Henoch-Schönlein purpura serum intercellular adhesion molecule 1 (ICAM-1), E-selectin, and plasma von Willebrand factor (vWF) were determined by ELISA during the active and inactive phase of the disease. Twelve healthy children were studied as a control group. Serum ICAM-1 concentrations increased during the active phase of the disease and differed significantly compared with the inactive phase (p < 0.05). However ICAM-1 in the active phase did not differ significantly compared with controls (p = 0.08). Serum E-selectin concentrations did not differ in the active and inactive phase of the disease. By contrast, vWF increased in the active phase of the disease and differed significantly compared with inactive disease and control groups (p < 0.01). Considering the adhesion molecules and vWF, only vWF correlated well with the C reactive protein measurement in the active phase, which is considered a good marker of disease activity. These data suggest that plasma vWF is a good marker of vascular inflammation and endothelial damage. Circulating ICAM-1 might be an additional parameter in some of the patients.

Soylemezoglu, O; Sultan, N; Gursel, T; Buyan, N; Hasanoglu, E

1996-01-01

289

Targets Directory.  

National Technical Information Service (NTIS)

This directory is intended to serve as a quick-look reference of target information. Section 1 describes target command and control systems, while section 2 discusses specific aerial, land, seaborne, and tow targets. Appendixes contain government points o...

1988-01-01

290

Using the Oxford classification of IgA nephropathy to predict long-term outcomes of Henoch-Schönlein purpura nephritis in adults.  

PubMed

Recently, there has been emerging concern that crescents, the main histologic feature of Henoch-Schönlein purpura nephritis, merely reflect active inflammation, and may not be useful in predicting long-term outcomes. We therefore conducted a single-center retrospective study to evaluate whether the new Oxford classification of immunoglobulin A nephropathy can be used to predict long-term outcome in patients with Henoch-Schönlein purpura nephritis. We included 61 biopsy-proven patients with Henoch-Schönlein purpura nephritis between January 1991 and August 2010. In addition to the International Study of Kidney Disease in Children classification, pathologic findings were also evaluated by the Oxford classification. Primary outcomes were defined as either the onset of estimated glomerular filtration rate <60?ml/min per 1.73?m(2) with ?30% decrease in estimated glomerular filtration rate from baseline or end-stage renal disease. During a median follow-up of 49.3 months, 13 (21%) patients reached the primary end point. A Kaplan-Meier plot showed that renal event-free survival was significantly longer in patients with <50% crescents than in those with crescents in ?50% of glomeruli (P=0.003). Among the components of the Oxford classification, patients with endocapillary hypercellularity (E1; P=0.016) and tubular atrophy/interstitial fibrosis (T1/T2; P=0.018) had lower renal survival rates than those with E0 and T0. In a multivariate Cox model adjusted for clinical and pathologic factors, E1 (hazard ratio=8.91; 95% confidence interval=1.47-53.88; P=0.017) and T1/T2 (hazard ratio=8.74; 95% confidence interval=1.40-54.38; P=0.020) were independently associated with reaching a primary outcome, whereas the extent of crescentic lesions was not. Our findings suggest that the Oxford classification can be used in predicting long-term outcomes of Henoch-Schönlein purpura nephritis. PMID:24390221

Ho Kim, Chan; Jin Lim, Beom; Sung Bae, Yoon; Eun Kwon, Young; Ly Kim, Yung; Heon Nam, Ki; Sook Park, Kyoung; Yeong An, Seong; Mo Koo, Hyang; Mee Doh, Fa; Jung Lee, Mi; Jung Oh, Hyung; Yoo, Tae-Hyun; Kang, Shin-Wook; Hun Choi, Kyu; Joo Jeong, Hyun; Han, Seung Hyeok

2014-07-01

291

Interferon-gamma gene polymorphism +874 (a/t) in chinese children with henoch-schönlein purpura.  

PubMed

The aim of this study was to investigate the possible influence of Interferon-gamma (IFN-?) gene polymorphism +874 (A/T) (rs2430561) in the susceptibility and renal complications of patients with Henoch-Schonlein purpura (HSP). We also studied the effects of IFN-? allelic variation on serum levels of pro-and anti-inflammatory cytokines in HSP patients. The study population comprised 97 patients suffering from HSP and 97 control participants. Patients and controls were genotyped for a single nucleotide polymorphism +874 (A/T) in the first intron of the IFN-? gene by the TaqMan PCR method. Frequencies of individuals with IFN-? +874 AA, AT and TT genotypes were 77.3%, 21.6% and 1% in HSP patients and 79.4%, 17.5% and 3.1% in controls, respectively. The frequency of the AA genotype in HSP patients with nephritis was slightly higher (83.3%) than in HSP patients without nephritis (73.8%). The allele A occurred more commonly in HSP patients with nephritis (92%) than in HSP patients without nephritis (86%), but these differences were not statistically significant (p= 0.469 and p= 0.244, respectively). In addition, significant difference in serum IL-10 levels between IFN-? +874 different genotype groups was found. Our results do not support a role for IFN-? gene polymorphism +874 (A/T) in the susceptibility to HSP and allelic variation at IFN-? +874 locus had no effect on serum levels of cytokines in patients with HSP except for IL-10. PMID:24659122

Xu, Hui; Li, Wei; Fu, Haidong; Jiang, Guizheng

2014-06-01

292

Similar disturbances in B cell activity and regulatory T cell function in Henoch-Schonlein purpura and systemic lupus erythematosus  

SciTech Connect

The immunoglobulin synthesizing activities of peripheral mononuclear cells (MNC) from five patients with Henoch-Schonlein purpura (HSP) and eight patients with active systemic lupus erythematosus (SLE) were compared. Cumulative amounts of IgM, IgG, and IgA synthesized and secreted by unstimulated and PWM-stimulated patient cells over a 12-day period were determied in a solid-phase radioimmunoassay. In unstimulated control cultures mean rates of IgM, IgG, and IgA synthesis were less than 250 ng/ml. The synthetic activities of patient MNC were markedly increased. In HSP cultures IgA was the major immunoglobulin class produced (2810 x/divide 1.33 ng/ml) followed by IgG (1754 x/divide 1.32 ng/ml) and IgM (404 x/divide 1.16 ng/ml). In SLE cultures IgA and IgG syntheses were equally elevated (4427 x/divide 1.20 and 4438 x/divide 1.49 ng/ml, respectively) whereas IgM synthesis averaged 967 x/divide 1.66 ng/ml. PWM stimulation of pateient MNC caused a sharp decline in the synthesis of all three immunoglobulin classes. After T cell depletion B cell-enriched fractions from HSP and SLE patients maintained high levels of IgA and IgG synthesis that were inhibited by PWM and by normal allogeneic but not autologous T cells. In PWM-stimulted co-cultures, patient T cells nonspecifically suppressed the synthetic activities of autologous and control B cells. in contrast patient B cells achieved normal levels of immunoglobulin synthesis when cultured with control T cells plus PWM. In longitudinal studies patient B and T cell disturbances persisted despite clinical improvement.

Beale, M.G. (St. Louis Children's Hospital, MO); Nash, G.S.; Bertovich, M.J.; MacDermott, R.P.

1982-01-01

293

Sputter target  

DOEpatents

The disclosure relates to an improved sputter target for use in the deposition of hard coatings. An exemplary target is given wherein titanium diboride is brazed to a tantalum backing plate using a gold-palladium-nickel braze alloy.

Gates, Willard G. (Kansas City, MO); Hale, Gerald J. (Overland Park, KS)

1980-01-01

294

Henoch-Schönlein purpura in northern Spain: clinical spectrum of the disease in 417 patients from a single center.  

PubMed

The severity of clinical features and the outcomes in previous series of patients reported with Henoch-Schönlein purpura (HSP) vary greatly, probably due to selection bias. To establish the actual clinical spectrum of HSP in all age groups using an unselected and wide series of patients diagnosed at a single center, we performed a retrospective review of 417 patients classified as having HSP according to the criteria proposed by Michel et al. Of 417 patients, 240 were male and 177 female, with a median age at the time of disease diagnosis of 7.5 years (interquartile range [IQR], 5.3-20.1 yr). Three-quarters of the patients were children or young people aged 20 years or younger (n = 315), and one-quarter were adults (n = 102). The most frequent precipitating events were a previous infection (38%), usually an upper respiratory tract infection, and/or drug intake (18.5%) shortly before the onset of the vasculitis. At disease onset the most common manifestations were skin lesions (55.9%), nephropathy (24%), gastrointestinal involvement (13.7%), joint symptoms (9.1%), and fever (6.2%). Cutaneous involvement occurring in all patients, mainly purpuric skin lesion, was the most common manifestation when the vasculitis was fully established, followed by gastrointestinal (64.5%), joint (63.1%), and renal involvement (41.2%). The main laboratory findings were leukocytosis (36.7%), anemia (8.9%), and increased serum IgA levels (31.7%). The most frequent therapies used were corticosteroids (35%), nonsteroidal antiinflammatory drugs (14%), and cytotoxic agents (5%). After a median follow-up of 12 months (IQR, 2-38 mo), complete recovery was observed in most cases (n = 346; 83.2%), while persistent, usually mild, nephropathy was observed in only 32 (7.7%) cases. Relapses were observed in almost a third of patients (n = 133; 31.9%).In conclusion, although HSP is a typical vasculitis affecting children and young people, it is not uncommon in adults. The prognosis is favorable in most cases, depending largely on renal involvement. PMID:24646467

Calvo-Río, Vanesa; Loricera, Javier; Mata, Cristina; Martín, Luis; Ortiz-Sanjuán, Francisco; Alvarez, Lino; González-Vela, M Carmen; González-Lamuño, Domingo; Rueda-Gotor, Javier; Fernández-Llaca, Héctor; González-López, Marcos A; Armesto, Susana; Peiró, Enriqueta; Arias, Manuel; González-Gay, Miguel A; Blanco, Ricardo

2014-03-01

295

ISAC targets  

NASA Astrophysics Data System (ADS)

The TRIUMF-ISAC radioactive ion beam facility was designed and constructed to allow irradiation of thick targets with up to 100 A proton beam intensities. Since beginning operation in 1998, beam intensities on ISAC targets have progressively increased toward the 100 A design limit. Routine operation with p + intensities up to 75 A is currently possible for both refractory metal target materials and for composite metal carbide materials; full 99 A p + intensity has been achieved for Nb foil target material. Consideration must be given to the beam power deposition, the power dissipation and the limiting temperature criteria of each target material. Increased beam power dissipation has been achieved by modifying target materials and target containers. Increasing irradiation currents have produced benefits, drawbacks and unexpected results for ISOL operations.

Dombsky, M.; Kunz, P.

2014-01-01

296

No evidence of association between functional polymorphisms located within IL6R and IL6ST genes and Henoch-Schönlein purpura.  

PubMed

Henoch-Schönlein purpura (HSP) is the most common type of primary small-sized blood vessel vasculitis in children and an uncommon condition in adults. Interleukin (IL)-6 is a proinflammatory cytokine whose effect is controlled by the IL-6 receptor (IL-6R). IL-6 transducer (IL-6ST/gp130) is the signal-transducing subunit of the IL-6R. Two hundred and eighty five Spanish HSP patients and 877 sex and ethnically matched controls were genotyped for the IL6R rs2228145 and IL6ST/gp130 rs2228044 functional polymorphisms. No significant differences in the genotype and allele frequencies between HSP patients and controls were observed. Moreover, there were no differences between HSP patients according to the age at disease onset, presence of nephritis or gastrointestinal manifestations. Our results do not confirm association of IL6R rs2228145 and IL6ST/gp130 rs2228044 polymorphisms with HSP. PMID:24498998

López-Mejías, R; Sevilla Pérez, B; Genre, F; Castañeda, S; Ortego-Centeno, N; Llorca, J; Ubilla, B; Ochoa, R; Pina, T; Marquez, A; Sala-Icardo, L; Miranda-Filloy, J A; Rueda-Gotor, J; Martín, J; Blanco, R; González-Gay, M A

2013-12-01

297

Lack of association between macrophage migration inhibitory factor gene promoter (-173 G/C) polymorphism and childhood Henoch-Schönlein purpura in Turkish patients.  

PubMed

Henoch-Schönlein purpura (HSP) is a small-vessel vasculitis of autoimmune hypersensitivity with rash, arthritis, abdominal pain and renal involvements. Macrophage migration inhibitory factor (MIF) is a immunoregulatory proinflammatory cytokine, and a major mediator at the inflammatory sites. The pathogenesis of HSP has not been fully elucidated. Here we aimed to assess the influence of macrophage migration inhibitory factor gene (-173 G/C) polymorphism in the susceptibility and clinical expression of patients with Henoch-Schönlein purpura (HSP). HSP patients (n:139) and ethnically matched healthy controls (n:100) were genotyped by PCR-RFLP. Genotype analysis of both polymorphisms did not reveal a significant deviation from Hardy-Weinberg equilibrium in any group (p > 0.05). No significant difference was obtained in genotype distribution (p > 0.05) and allele frequencies (p > 0.05) between patients and controls. A statistically significant genotype-phenotype correlation was not obtained when HSP patients were stratified by the presence of certain systemic complications and the macrophage migration inhibitory factor gene (-173 G/C) polymorphism (p > 0.05). A significant risk was not observed in the subjects both with the GC+CC genotype (p = 0.06, OR: 0.5538, 95% CI: 0.2985-1.0274) and C allele (odds ratio: C vs. G: 1.799, 95% CI: 1.002-3.23, p = 0.05). Our findings suggest that MIF gene -173 G/C polymorphism is not associated with HSP in the present Turkish population. PMID:23523092

Nalbantoglu, Sinem; Tabel, Y?lmaz; Mir, Sevgi; Berdeli, Afig

2013-04-01

298

Target discovery  

Microsoft Academic Search

Target discovery, which involves the identification and early validation of disease-modifying targets, is an essential first step in the drug discovery pipeline. Indeed, the drive to determine protein function has been stimulated, both in industry and academia, by the completion of the human genome project. In this article, we critically examine the strategies and methodologies used for both the identification

Mark A. Lindsay

2003-01-01

299

Inflation Targeting and Target Instability.  

National Technical Information Service (NTIS)

Monetary policy is modeled as being governed by a known rule, except for a time-varying target rate of inflation. The variable target is taken as representing either discretionary deviations from the rule, or as the outcome of a policymaking committee tha...

R. J. Tetlow

1999-01-01

300

Henoch-Schönlein Purpura  

MedlinePLUS

... and Kidney Diseases (NIDDK) supports several programs and studies devoted to improving treatment for patients with progressive kidney disease and kidney failure. The NIDDK maintains the Pediatric Nephrology Program, which supports research into the causes, ...

301

PlayStation purpura.  

PubMed

A 16-year-old boy presented with a number of asymptomatic pigmented macules on the volar aspect of his index fingers. Dermoscopy of each macule revealed a parallel ridge pattern of homogenous reddish-brown pigment. We propose that these lesions were induced by repetitive trauma from a Sony PlayStation 3 (Sony Corporation, Tokyo, Japan) vibration feedback controller. The lesions completely resolved following abstinence from gaming over a number of weeks. Although the parallel ridge pattern is typically the hallmark for early acral lentiginous melanoma, it may be observed in a limited number of benign entities, including subcorneal haematoma. PMID:20695869

Robertson, Susan J; Leonard, Jane; Chamberlain, Alex J

2010-08-01

302

Human protein C concentrate in the treatment of purpura fulminans: a retrospective analysis of safety and outcome in 94 pediatric patients  

PubMed Central

Introduction Purpura fulminans (PF) is a devastating complication of uncontrolled systemic inflammation, associated with high incidence of amputations, skin grafts and death. In this study, we aimed to clarify the clinical profile of pediatric patients with PF who improved with protein C (PC) treatment, explore treatment effects and safety, and to refine the prognostic significance of protein C plasma levels. Methods In Germany, patients receiving protein C concentrate (Ceprotin®, Baxter AG, Vienna, Austria) are registered. The database was used to locate all pediatric patients with PF treated with PC from 2002 to 2005 for this national, retrospective, multi-centered study. Results Complete datasets were acquired in 94 patients, treated in 46 centers with human, non-activated protein C concentrate for purpura fulminans. PC was given for 2 days (median, range 1-24 days) with a median daily dose of 100 IU/kg. Plasma protein C levels increased from a median of 27% to a median of 71% under treatment. 22.3% of patients died, 77.7% survived to discharge. Skin grafts were required in 9.6%, amputations in 5.3%. PF recovered or improved in 79.8%, remained unchanged in 13.8% and deteriorated in 6.4%. Four adverse events occurred in 3 patients, none classified as severe. Non-survivors had lower protein C plasma levels (P < 0.05) and higher prevalence of coagulopathy at admission (P < 0.01). Time between admission and start of PC substitution was longer in patients who died compared to survivors (P = 0.03). Conclusions This retrospective dataset shows that, compared to historic controls, only few pediatric patients with PF under PC substitution needed dermatoplasty and/or amputations. Apart from epistaxis, no bleeding was observed. Although the data comes from a retrospective study, the evidence we present suggests that PC had a beneficial impact on the need for dermatoplasty and amputations, pointing to the potential value of carrying out a prospective randomised controlled trial.

2010-01-01

303

Target: Lifestyle.  

ERIC Educational Resources Information Center

"Target: Lifestyle" is a physical education curriculum adopted by Detroit Country Day School which incorporates instruction in nutrition, physical fitness, first aid, and lifetime sports. This curriculum aims to influence student attitudes and lifestyles in health and physical fitness. Four levels of instruction are described. (DF)

Poehlman, Eric T.

1985-01-01

304

Targeting Subgroups.  

ERIC Educational Resources Information Center

By requiring limited-English-proficient students and students with disabilities to meet the same targets at the same times as all other students, the NCLB imposes a one-size-fits-all approach that ignores the individual child. Challenges school leaders to take care not to blame the students and still hold themselves accountable for continual…

Schwartzbeck, Terri Duggan

2003-01-01

305

Internet Targets  

Microsoft Academic Search

This article discusses the assets typically targeted by Internet robbers. It also provides a brief overview of the increased importance of ‘intangible’ information assets to the knowledge-based businesses and government agencies of the Information Age and some of the ways that the global adoption of Internet technology brings increased risk to these assets.

Gerald L. Kovacich; William C. Boni

2000-01-01

306

Target assembly  

DOEpatents

A target for a proton beam which is capable of generating neutrons for absorption in a breeding blanket includes a plurality of solid pins formed of a neutron emissive target material disposed parallel to the path of the beam and which are arranged axially in a plurality of layers so that pins in each layer are offset with respect to pins in all other layers, enough layers being used so that each proton in the beam will strike at least one pin with means being provided to cool the pins. For a 300 mA, 1 GeV beam (300 MW), stainless steel pins, 12 inches long and 0.23 inches in diameter are arranged in triangular array in six layers with one sixth of the pins in each layer, the number of pins being such that the entire cross sectional area of the beam is covered by the pins with minimum overlap of pins.

Lewis, Richard A. (Falls Church, VA)

1980-01-01

307

On Target  

NSDL National Science Digital Library

In this design challenge activity, learners modify a cup so it can carry a marble down a zip line and also drop it onto a target. Learners are encouraged to brainstorm answers to design questions, build a prototype using simple materials, and test, evaluate, and redesign their structure. The activity guide includes troubleshooting tips. The related Leader's Notes guide contains information on how to connect this to NASA and aerospace engineering.

Wgbh

2010-01-01

308

Deorbit targeting  

NASA Technical Reports Server (NTRS)

The navigation and control of the space shuttle during atmospheric entry are discussed. A functional flow diagram presenting the basic approach to the deorbit targeting problem is presented. The major inputs to be considered are: (1) vehicle state vector, (2) landing site location, (3) entry interface parameters, (4) earliest desired time of landing, and (5) maximum cross range. Mathematical models of the navigational procedures based on controlled thrust times are developed.

Tempelman, W. H.

1973-01-01

309

Efficacy of Triptolide for Children with Moderately Severe Henoch-Sch?nlein Purpura Nephritis Presenting with Nephrotic Range Proteinuria: A Prospective and Controlled Study in China  

PubMed Central

Objective. To observe the clinical efficacy of the Chinese herb, Triptolide, in children with moderately severe Henoch-Schönlein purpura nephritis (HSPN). Methods. From January 2007 to December 2011, 56 HSPN children manifested by nephrotic range proteinuria with normal kidney function and <50% crescents or sclerosing lesions on biopsy were hospitalized in the Children's Hospital of Zhejiang University School of Medicine. They were divided into two groups: the treatment group (n = 42; Triptolide at a dosage of 1?mg/kg·d, combined with prednisone at a dosage of 2?mg/kg·d, within a course of medium-to-long-term therapy of 6 to 9 months) and the control group (n = 14; prednisone alone, with the same procedure). Results. Short-term remission was observed in 95% of patients from treatment group and in 72% of patients from control group, respectively. There was a significant difference between both groups (?2 = 6.222, P = 0.029) for short-term effects. Meanwhile, no significant difference, as proteinuria, hematuria, hypertension, and decreased eGFR, was observed between the two groups in long-term followup (?2 = 3.111, P = 0.097). The Kaplan-Meier plot analysis also revealed no significant difference (?2 = 2.633, P = 0.105). Conclusion. Triptolide is effective in relieving short-term symptoms for moderately severe HSPN children, though its long-term effects need to be observed further.

Wu, Li; Jin, Xia; Fu, Haidong; Shen, Huijun; Wang, Jingjing; Liu, Aimin; Shu, Qiang; Du, Lizhong

2013-01-01

310

Elevated urinary levels of cystatin C and neutrophil gelatinase-associated lipocalin in henoch-schönlein purpura patients with renal involvement.  

PubMed

Henoch-Schönlein purpura (HSP) is a commonest systemic vasculitis in childhood. The long-term prognosis of HSP is determined by the degree of renal involvement. The aim of this study is to search novel clinically applicable biomarkers to evaluate renal involvement in HSP patients. 20 bio-indexes in urine samples were simultaneously screened by antibody array assay. We indicated that urinary levels of cystatin C (Cys C) and neutrophil gelatinase-associated lipocalin (NGAL) in HSP patients with renal involvement were significantly higher than those without renal involvement and healthy controls. Furthermore, ELISA was used to analyze urinary Cys C and NGAL levels in HSP patients with or without renal involvement, atopic dermatitis (AD) patients and healthy controls. Our results demonstrated that urinary Cys C and NGAL levels in HSP patients with renal involvement were significantly elevated, when compared with those without renal involvement, AD patients and control subjects. In addition, by receiver operating characteristic (ROC) curve analysis, we demonstrated that the area under the ROC curve of NGAL (0.789) was larger than that of Cys C (0.692). Taken together, we show firstly that urinary Cys C and NGAL levels is abnormally elevated in HSP patients with renal involvement. We suggest that urinary Cys C and NGAL are novel useful biomarkers of renal involvement in HSP patients. PMID:24963810

Chen, Tao; Lu, Yong-Hong; Wang, Wen-Ju; Bian, Cai-Yun; Cheng, Xiao-Yun; Su, Yu; Zhou, Pei-Mei

2014-01-01

311

Elevated Urinary Levels of Cystatin C and Neutrophil Gelatinase-Associated Lipocalin in Henoch-Sch?nlein Purpura Patients with Renal Involvement  

PubMed Central

Henoch-Schönlein purpura (HSP) is a commonest systemic vasculitis in childhood. The long-term prognosis of HSP is determined by the degree of renal involvement. The aim of this study is to search novel clinically applicable biomarkers to evaluate renal involvement in HSP patients. 20 bio-indexes in urine samples were simultaneously screened by antibody array assay. We indicated that urinary levels of cystatin C (Cys C) and neutrophil gelatinase-associated lipocalin (NGAL) in HSP patients with renal involvement were significantly higher than those without renal involvement and healthy controls. Furthermore, ELISA was used to analyze urinary Cys C and NGAL levels in HSP patients with or without renal involvement, atopic dermatitis (AD) patients and healthy controls. Our results demonstrated that urinary Cys C and NGAL levels in HSP patients with renal involvement were significantly elevated, when compared with those without renal involvement, AD patients and control subjects. In addition, by receiver operating characteristic (ROC) curve analysis, we demonstrated that the area under the ROC curve of NGAL (0.789) was larger than that of Cys C (0.692). Taken together, we show firstly that urinary Cys C and NGAL levels is abnormally elevated in HSP patients with renal involvement. We suggest that urinary Cys C and NGAL are novel useful biomarkers of renal involvement in HSP patients.

Chen, Tao; Lu, Yong-hong; Wang, Wen-ju; Bian, Cai-yun; Cheng, Xiao-yun; Su, Yu; Zhou, Pei-mei

2014-01-01

312

Serum levels of galactose-deficient IgA in children with IgA nephropathy and Henoch-Schönlein purpura.  

PubMed

IgA nephropathy and Henoch-Schönlein purpura nephritis (HSPN) are related diseases characterized by deposits of IgA1-containing immune complexes in the renal mesangium. Adult patients with IgA nephropathy have aberrantly glycosylated IgA1 (galactose-deficient O-linked glycans) in the circulation and renal deposits. However, IgA1 glycosylation has not been studied in pediatric patients with IgA nephropathy. Using our quantitative lectin enzyme-linked immunosorbent assay (ELISA) test, we measured serum levels of galactose-deficient IgA1 of children with IgA nephropathy and HSPN and controls. Children with IgA nephropathy and HSPN had serum levels higher than those of healthy children or renal-disease controls with C1q nephropathy. Furthermore, lectin ELISA identified patients with HSPN whose clinical course mimicked that of IgA nephropathy. In summary, pediatric patients with IgA nephropathy and HSPN have an aberrancy in the glycosylation in IgA1 O-linked glycans that is similar to that in adults with IgA nephropathy. PMID:17943324

Lau, Keith K; Wyatt, Robert J; Moldoveanu, Zina; Tomana, Milan; Julian, Bruce A; Hogg, Ronald J; Lee, Jeannette Y; Huang, Wen-Qiang; Mestecky, Jiri; Novak, Jan

2007-12-01

313

Aberrant glycosylation of IgA1 is inherited in both pediatric IgA nephropathy and Henoch-Schönlein purpura nephritis.  

PubMed

Serum galactose-deficient immunoglobulin A1 (Gd-IgA1) is an inherited risk factor for adult IgA nephropathy (IgAN). In this paper, we determined the heritability of serum Gd-IgA1 levels in children with IgAN and Henoch-Schönlein purpura nephritis (HSPN), two disorders with clinical phenotypes sharing common pathogenic mechanisms. Serum Gd-IgA1 concentrations were quantified using a Helix aspersa-lectin-based enzyme-linked immunosorbent assay. As a group, 34 children with either disorder (20 with HSPN and 14 with IgAN) had significantly higher Gd-IgA1 levels compared with 51 age- and ethnicity-matched pediatric controls. Serum levels of Gd-IgA1 were also elevated in a large fraction of 54 first-degree relatives of pediatric IgAN and HSPN patients compared with 141 unrelated healthy adult controls. A unilineal transmission of the trait was found in 17, bilineal transmission in 1, and sporadic occurrence in 5 of 23 families when both parents and the patient were analyzed. There was a significant age-, gender-, and household-adjusted heritability of serum galactose-deficient IgA1 estimated at 76% in pediatric IgAN and at 64% in HSPN patients. Thus, serum galactose-deficient IgA1 levels are highly inherited in pediatric patients with IgAN and HSPN, providing support for another shared pathogenic link between these disorders. PMID:21326171

Kiryluk, Krzysztof; Moldoveanu, Zina; Sanders, John T; Eison, T Matthew; Suzuki, Hitoshi; Julian, Bruce A; Novak, Jan; Gharavi, Ali G; Wyatt, Robert J

2011-07-01

314

Immune thrombocytopenia. Use of a Coombs antiglobulin test to detect IgG and C3 on platelets  

Microsoft Academic Search

We applied a radiolabeled Coombs antiglobulin test to the diagnosis and management of immune thrombocytopenia in adults and children. This assay substantiated that the majority of patients with idiopathic thrombocytopenic purpura have increased levels of IgG on their platelets. Platelets from a patient with the post-transfusion-purpura syndrome also carried increased IgG, indicating a role for IgG antibody or IgG-containing immune

Douglas B. Cines; Alan D. Schreiber

1979-01-01

315

Accelerator target  

DOEpatents

A target includes a body having a depression in a front side for holding a sample for irradiation by a particle beam to produce a radioisotope. Cooling fins are disposed on a backside of the body opposite the depression. A foil is joined to the body front side to cover the depression and sample therein. A perforate grid is joined to the body atop the foil for supporting the foil and for transmitting the particle beam therethrough. A coolant is circulated over the fins to cool the body during the particle beam irradiation of the sample in the depression.

Schlyer, David J. (Bellport, NY); Ferrieri, Richard A. (Patchogue, NY); Koehler, Conrad (Miller Place, NY)

1999-01-01

316

Accelerator target  

DOEpatents

A target includes a body having a depression in a front side for holding a sample for irradiation by a particle beam to produce a radioisotope. Cooling fins are disposed on a backside of the body opposite the depression. A foil is joined to the body front side to cover the depression and sample therein. A perforate grid is joined to the body atop the foil for supporting the foil and for transmitting the particle beam therethrough. A coolant is circulated over the fins to cool the body during the particle beam irradiation of the sample in the depression. 5 figs.

Schlyer, D.J.; Ferrieri, R.A.; Koehler, C.

1999-06-29

317

Target: Terror  

NSDL National Science Digital Library

This week's In the News investigates the US military strike against targets linked to international terrorism. The eight resources discussed provide analysis, commentary, and recent news. On August 7, simultaneous bombings of US embassies in Nairobi, Kenya and Dar es Salaam, Tanzania, injured thousands of civilians and killed more than 250, including a dozen Americans. In a reprisal on August 20, based on "compelling evidence" from US intelligence agencies, President Clinton ordered a Tomahawk cruise missile attack on several sites suspected of being connected to the terrorist network responsible for the embassy bombings. The targets of the assault included six sites within the distributed Zhawar Kili al-Badr guerrilla camp near Khost, Afghanistan, and the El Shifa Pharmaceutical Plant near Khartoum, Sudan--assumed to be a manufacturing center for chemical weapons. Both locations are believed to be supported by Osama Bin Muhammad Bin Laden, who is, according to the Pentagon, linked to the embassy bombings and considered the "world's leading individual sponsor of terrorism against Americans." The transnational missile attack marks the most powerful military offensive against a private sponsor of confederate terrorist groups. The US called the assault a pre-emptive measure against terrorism and justified its use of force under Article 51 of the UN charter, which authorizes nations to act in self-defense if they anticipate aggression. US Defense Secretary William Cohen acknowledged that the military retaliation "will not eliminate the problem" of terrorism, but he believes the disruption and destruction of terrorist sanctuaries sends a clear message that the US will be steadfast in defense of its citizens "against these cowardly attacks."

Osmond, Andrew.

318

The level of IgA antibodies to human umbilical vein endothelial cells can be enhanced by TNF-? treatment in children with Henoch-Sch?nlein purpura  

PubMed Central

Anti-endothelial cell antibodies (AECA) have been found to play an important role in many vascular disorders. In order to determine the presence of AECA in children with Henoch–Schönlein purpura (HSP), and to elucidate the pathogenic and clinical value of their measurement in this disease, AECA were detected by immunofluorescence staining and a human umbilical vein endothelial cell (HUVEC)-based enzyme-linked immunosorbent assay (ELISA) in 20 children with HSP, 10 children with juvenile rheumatoid arthritis (JRA) without vasculitis and 10 normal healthy children. Antibodies against another endothelial cells, human dermal microvascular endothelial cells (HMVEC-d) were also detected by cell-based ELISA. In some experiments, we compared the binding activity of antibodies to HUVEC with and without tumour necrosis factor-? (TNF-?) or interleukin-1 (IL-1) pretreatment. Patients with acute onset of HSP had higher serum levels of IgA antibodies, both against HUVEC and against HMVEC-d, than healthy controls (P = 0·001, P = 0·008, respectively). Forty-five per cent of patients had positive IgA AECA to HUVEC, and 35% had positive IgA AECA to HMVEC-d. The titres of IgA antibodies to HUVEC paralleled the disease activity. After TNF-? treatment, the values of IgA AECA to HUVEC in HSP patients were significantly increased (P = 0·02). For IgG and IgM AECA, there was no difference between HSP patients and controls (P = 0·51, P = 0·91). Ten JRA children without vasculitis had no detectable IgG, IgM or IgA AECA activity. The results of this study showed that children with HSP had IgA AECA, which were enhanced by TNF-? treatment. Although the role of these antibodies is not clear, IgA AECA provide another immunological clue for the understanding of HSP.

Yang, Y -H; Wang, S J; Chuang, Y -H; Lin, Y -T; Chiang, B -L

2002-01-01

319

Renal neutrophil gelatinase-associated lipocalin and kidney injury molecule-1 expression in children with acute kidney injury and Henoch-Sch?nlein purpura nephritis  

PubMed Central

The aim of this study was to investigate the expression of neutrophil gelatinase-associated lipocalin (NGAL) and kidney injury molecule-1 (KIM-1) in the serum, urine and renal tissues of children with acute kidney injury (AKI) and Henoch-Schönlein purpura nephritis (A-on-C). A prospective single-center evaluation of the serum, urine and renal NGAL and KIM-1 levels was performed in a cohort of children. Blood and 5-ml urine samples were collected from each patient for the analysis of NGAL and KIM-1 levels using an ELISA. In addition, the expression of NGAL and KIM-1 in the kidney was examined using immunohistochemistry in patients with A-on-C and HSPN. The expression of serum cystatin C, ?2-macroglobulin and serum creatinine (SCr), as well as urinary ?2-MG and SCr, in the patients with A-on-C was significantly higher than that of HSPN patients, and the expression of NGAL and KIM-1 in the serum and urine in the A-on-C patients was also significantly higher than that of HSPN patients. However, there were no significant differences in the urine protein levels between the two groups. NGAL and KIM-1 were expressed in renal tubular epithelial cells, and the expression of NGAL and KIM-1 in the A-on-C patients was significantly higher than that in HSPN patients. In addition, the urine NGAL and KIM-1 levels were negatively correlated with glomerular filtration rate, but there was no significant correlation between the urine NGAL/KIM-1 and urine protein levels. The changes in serum and urine NGAL and KIM-1 levels may be applied to the diagnosis of A-on-C.

DU, YUE; HOU, LING; GUO, JINJIE; SUN, TINGTING; WANG, XIULI; WU, YUBIN

2014-01-01

320

Clinical significance of TLR3 and TLR4 in peripheral blood mononuclear cells from children with Henoch-Sch?nlein purpura nephritis  

PubMed Central

The aim of the present study was to investigate the expression levels and clinical significance of Toll-like receptor (TLR) 3 and 4 in peripheral blood mononuclear cells (PBMCs) collected from children with Henoch-Schönlein purpura (HSP) nephritis. The randomized controlled trial was conducted between August 2011 and March 2013, and 105 children with a clinical diagnosis of HSP were enrolled in the study. According to the 24-h urinary protein measurements and the presence of renal damage, the 105 cases were divided into groups A, B and C as follows: Group A, children with HSP but without renal damage; group B, children with HSP nephritis but without proteinuria; group C, children with HSP nephritis and proteinuria. A total of 30 healthy children were enrolled in the normal control group (group N). The primary endpoints were the detection of TLR3 and 4 mRNA and protein expression levels in PBMCs by flow cytometry and quantitative polymerase chain reaction. The mRNA and protein expression levels of TLR4 in the PBMCs were significantly higher in groups A, B and C when compared with group N. In addition, the mRNA and protein expression levels of TLR4 in group C were much higher when compared with groups A and B. A positive correlation was identified between TLR4 protein expression and 24-h urinary protein levels in group C. The expression levels of TLR3 did not significantly differ among the groups. Protein and mRNA expression levels of TLR4 in PBMCs significantly increased and exhibited a positive correlation with urinary protein excretion. These results indicate that aberrant activation of TLR4 may be relevant to the development of HSP nephritis.

CHANG, HONG; YU, DONG-SHENG; LIU, XIU-QIN; ZHANG, QIU-YE; CHENG, NA; ZHANG, SHOU-QING; QU, ZHENG-HAI

2014-01-01

321

Tissue Deposits of IgA-Binding Streptococcal M Proteins in IgA Nephropathy and Henoch-Sch?nlein Purpura  

PubMed Central

IgA nephropathy (IgAN) and Henoch-Schönlein purpura (HSP) are diseases characterized by IgA deposits in the kidney and/or skin. Both may arise after upper respiratory tract infections, but the pathogenic mechanisms governing these diseases remain unclear. Patients with IgAN (n = 16) and HSP (n = 17) were included in this study aimed at examining whether IgA-binding M proteins of group A streptococci could be involved. As M proteins vary in sequence, the study focused on the IgA-binding-region (IgA-BR) of three different M proteins: M4, M22, and M60. Renal tissue from IgAN and HSP patients and skin from HSP patients were examined for deposits of streptococcal IgA-BR by immunohistochemistry and electron microscopy using specific antibodies, and a skin sample from a HSP patient was examined by mass spectrometry. IgA-BR deposits were detected in 10/16 IgAN kidneys and 7/13 HSP kidneys. Electron microscopy demonstrated deposits of IgA-BRs in the mesangial matrix and glomerular basement membrane, which colocalized with IgA. Skin samples exhibited IgA-BR deposits in 4/5 biopsies, a result confirmed by mass spectrometry in one patient. IgA-BR deposits were not detected in normal kidney and skin samples. Taken together, these results demonstrate IgA-BR from streptococcal M proteins in patient tissues. IgA-BR, would on gaining access to the circulation, encounter circulatory IgA and form a complex with IgA-Fc that could deposit in tissues and contribute to the pathogenesis of IgAN and HSP.

Schmitt, Roland; Carlsson, Fredric; Morgelin, Matthias; Tati, Ramesh; Lindahl, Gunnar; Karpman, Diana

2010-01-01

322

[Current treatment of primary immune thrombocytopenia].  

PubMed

Primary immune thrombocytopenia, also termed immune thrombocytopenic purpura (ITP) is an autoimmune disorder characterized by premature platelet destruction and impaired platelet production. Traditional treatment of ITP has predominantly consisted of immune suppression and/or modulation. However, the understanding of the immune mediated impairment of platelet production has led to the development of new treatments that target the thrombopoietin receptor, promoting formation of megakaryocytes and increasing platelet counts. Best practice for the management of ITP has not yet been established because data from comparative studies are lacking. While some disagreement might still remain among experts concerning therapy (when, who, and how should be treated), in recent years different evidence-based practice guidelines have been published to assist healthcare professionals in the diagnosis and treatment of ITP. This review describes the current treatment landscape of ITP. PMID:23809409

Lozano, María L; Vicente, Vicente

2014-05-01

323

Advanced Target Tracker Concepts.  

National Technical Information Service (NTIS)

Conventional target tracking approaches rely on numerical correlation over successive frames on a window around the target. They are therefore sensitive to partial obscuration and changes in target and background appearances. Furthermore, multiple-target ...

P. M. Narendra B. L. Westover

1980-01-01

324

Advanced Target Tracker Concepts.  

National Technical Information Service (NTIS)

Conventional target tracking approaches rely on numerical correlation over successive frames on a window around the target. They are therefore sensitive to partial obscuration and changes in target and background appearances. Furthermore, multiple-target ...

P. M. Narendra B. L. Westover

1981-01-01

325

[Particular features of erythropoiesis in high altitude and possibilities of applying of hypoxic hypoxia methodology for the patients with hemopoietic suppression].  

PubMed

Conditions of hypoxic hypoxia at 3200 m height exert significant positive changes in hemopoiesis, normalizing erythropoiesis and coagulation system. Hypoxic climate therapy can be regarded as an additional efficient method to the pathogenetic treatment for patient with unpainful aplastic anemia and idiopathic thrombocytopenic purpura. It should be emphasized that patients must be out of immunosuppressive therapy when getting high altitude stationary. PMID:24341009

Makeshova, A B; Éralieva, M O; Levina, A A; Mamukova, Iu I; Raimzhanov, A R

2013-07-01

326

Laparoscopic splenectomy--a report of two cases.  

PubMed

Laparoscopic splenectomy for haematological diseases has recently attracted much attention and hailed as a viable alternative to traditional splenectomy using the laparotomy route. We report two cases of laparoscopic splenectomy for idiopathic thrombocytopenic purpura; the first of such procedures performed in Brunei. There was reduced post-operative pain, earlier return of gastrointestinal function and a reduced post-operative hospital stay. PMID:9885713

Kok, K Y; Matthew, V V; Yapp, S K

1998-10-01

327

The effect of antiviral therapy on hepatitis C virus-related thrombocytopenia: a case report  

PubMed Central

Background Autoimmune thrombocytopenic purpura is an immunological disorder characterized by increased platelet destruction due to presence of anti-platelet autoantibodies. Hepatitis C virus infection, which is one of the most common chronic viral infections worldwide, may cause secondary chronic immune thrombocytopenic purpura. Case presentation We report a case of a 51-year-old Caucasian female with hepatitis C virus infection who developed a severe, reversible, immune thrombocytopenia. Platelet count was as low as 56.000/mm3, hepatitis C virus serology was positive, serum glutamic oxaloacetic transaminase, serum glutamic pyruvic transaminase and gamma-glutamyltransferase serum levels were elevated. Disorders potentially associated with autoimmune thrombocytopenic purpura were ruled out. A corticosteroid treatment was started and led to an increase in platelet count. The patient was then treated with pegylated-interferon alpha 2a and ribavirin. After four weeks of treatment hepatitis C virus - ribonucleic acid became undetectable and steroid treatment was discontinued. Six months of antiviral therapy achieved a sustained biochemical and virological response together with persistence of normal platelet count. Conclusion In our case report hepatitis C virus seemed to play a pathogenic role in autoimmune thrombocytopenic purpura. Moreover, the successful response (negative hepatitis C virus - ribonucleic acid) to tapered steroids and antiviral therapy was useful to revert thrombocytopenia.

2014-01-01

328

Escherichia coli produtora de toxina shiga (STEC): principais fatores de virulência e dados epidemiológicos Shiga toxin-producing Escherichia coli (STEC): principal virulence factors and epidemiology  

Microsoft Academic Search

Shiga toxin producing Escherichia coli is an important food borne pathogen, mainly beef products, and is associated to mild and severe bloody diarrhea. In some individuals, STEC infection can progress to hemolytic-uremic syndrome (HUS), a sequela characterized by renal failure, and thrombotic thrombocytopenic purpura (TTP), with possible central nervous system involvement. Cattle, usually healthy, is the principal reservoir of STEC,

Ariane Mayumi Saito Bertão; Halha Ostrensky Saridakis

329

Increased nitric oxide formation in recurrent thrombotic microangiopathies: A possible mediator of microvascular injury  

Microsoft Academic Search

The term thrombotic microangiopathy (TMA) has been used extensively to encompass hemolytic uremic syndrome and thrombotic thrombocytopenic purpura, two syndromes of hemolytic anemia, and thrombocytopenia associated with renal or brain involvement or both. There is evidence that endothelial damage is a crucial feature in the sequence of events that precedes the development of microvascular lesions. More recent studies would suggest

Marina Noris; Piero Ruggenenti; Marta Todeschini; Marina Figliuzzi; Daniela Macconi; Carla Zoja; Simona Paris; Flavio Gaspari; Giuseppe Remuzzi

1996-01-01

330

Ultralarge von Willebrand factor multimers and normal ADAMTS13 activity in the umbilical cord blood  

Microsoft Academic Search

Introduction: Recent studies demonstrate that deficiency of ADAMTS13, the metalloprotease that cleaves von Willebrand factor (VWF) in a shear-dependent manner, causes thrombotic thrombocytopenic purpura (TTP). Previously, ultralarge multimers of VWF were detected in the fetuses, the umbilical cords and the newborns. However, the significance of this finding is unknown. Materials and methods: The activity of ADAMTS13 and the multimer pattern

Han-Mou Tsai; Ravindra Sarode; Katharine A Downes

2002-01-01

331

Drug Insight: the mechanism of action of rituximab in autoimmune disease—the immune complex decoy hypothesis  

Microsoft Academic Search

Inflammatory responses to cell-associated or tissue-associated immune complexes are key elements in the pathogenesis of several autoimmune diseases, including rheumatoid arthritis, systemic lupus erythematosus and immune thrombocytopenic purpura. Effector cells, such as monocytes, macrophages and neutrophils, bind immune complexes in a process mediated by Fc? receptors, and these cells then initiate inflammatory reactions that lead to tissue destruction. Rituximab is

Margaret A Lindorfer; Ronald P Taylor

2007-01-01

332

Severe Ehrlichia chaffeensis Infection in a Lung Transplant Recipient: A Review of Ehrlichiosis in the Immunocompromised Patient  

PubMed Central

We describe a case of human ehrlichiosis in a lung transplant recipient and review published reports on ehrlichiosis in immunocompromised patients. Despite early therapy with doxycycline, our patient had unusually severe illness with features of thrombotic thrombocytopenic purpura. Of 23 reported cases of ehrlichiosis in immunocompromised patients, organ failure occurred in all patients and 6 (25%) died.

Love, Robert B.; Maki, Dennis G.

2002-01-01

333

Brain Abscess Due to Listeria Monocytogenes: First Case Report in Thailand  

Microsoft Academic Search

Brain abscess with bacteremia caused by Listeria monocytogenes in a young woman with immune thrombocytopenic purpura was reported. The clinical features included fever, headache, and left-side weakness. Computed tomography and magnetic resonance imaging of the brain showed a large single abscess at the right frontoparietal area. L. monocytogenes was isolated from a blood culture. The patient promptly received a surgical

Somporn Srifeungfung

334

LIFE Target Fabrication Costs.  

National Technical Information Service (NTIS)

Target costs for the LIFE IFE plant comprises a significant portion of the operating costs for the commercial plant. A preliminary target fabrication and cost study was undertaken previously for a fast ignition target. An updated cost model is documented ...

G. Meyer J. Biener M. Wang R. Miles

2009-01-01

335

Autonomous target acquisition techniques  

NASA Technical Reports Server (NTRS)

Target acquisition is often needed in a deep space mission where the detector on board the space vehicle must be able to perform the decision making process in acquiring the target. That is, the system for target acquisition must be autonomous. This paper presents several autonomous target acquisition techniques, applicable to deep space mission, for detecting stationary and moving targets. These techniques are useful for sensors such as radar, star tracker and television since the target must be found before it can be tracked. A minimum signal-to-noise ratio can be specified for successful acquisition of target.

Brock, H. I.; Hung, J. C.

1975-01-01

336

Electrically charged targets  

DOEpatents

Electrically chargeable laser targets and method for forming such charged targets in order to improve their guidance along a predetermined desired trajectory. This is accomplished by the incorporation of a small amount of an additive to the target material which will increase the electrical conductivity thereof, and thereby enhance the charge placed upon the target material for guidance thereof by electrostatic or magnetic steering mechanisms, without adversely affecting the target when illuminated by laser energy.

Goodman, Ronald K. (Livermore, CA); Hunt, Angus L. (Alamo, CA)

1984-01-01

337

Organelle targeting: third level of drug targeting  

PubMed Central

Drug discovery and drug delivery are two main aspects for treatment of a variety of disorders. However, the real bottleneck associated with systemic drug administration is the lack of target-specific affinity toward a pathological site, resulting in systemic toxicity and innumerable other side effects as well as higher dosage requirement for efficacy. An attractive strategy to increase the therapeutic index of a drug is to specifically deliver the therapeutic molecule in its active form, not only into target tissue, nor even to target cells, but more importantly, into the targeted organelle, ie, to its intracellular therapeutic active site. This would ensure improved efficacy and minimize toxicity. Cancer chemotherapy today faces the major challenge of delivering chemotherapeutic drugs exclusively to tumor cells, while sparing normal proliferating cells. Nanoparticles play a crucial role by acting as a vehicle for delivery of drugs to target sites inside tumor cells. In this review, we spotlight active and passive targeting, followed by discussion of the importance of targeting to specific cell organelles and the potential role of cell-penetrating peptides. Finally, the discussion will address the strategies for drug/DNA targeting to lysosomes, mitochondria, nuclei and Golgi/endoplasmic reticulum.

Sakhrani, Niraj M; Padh, Harish

2013-01-01

338

Photolocalized purpura during levofloxacin therapy.  

PubMed

Side-effects associated with levofloxacin treatment include phototoxicity, hypersensitivity and skin disorders. Purpuric eruptions have rarely been reported. We describe the case of a 75-year-old woman who was prescribed a 15-day course of levofloxacin (500 mg twice a day) for hemorrhagic cystitis. On exposure to sunlight, the patient developed a pruritic purpuric eruption on the lower extremities. The acute reaction differed from a classical sunburn, manifesting as confluent petechiae limited to sun-exposed areas and accompanied by pruritus. This was a rare case of solar capillaritis. Purpuric eruptions on photoexposed skin should be considered another unusual side effect of levofloxacin. PMID:22409715

Rubegni, Pietro; Feci, Luca; Pellegrino, Michele; Fimiani, Michele

2012-04-01

339

Magnetically attached sputter targets  

DOEpatents

An improved method and assembly for attaching sputtering targets to cathode assemblies of sputtering systems which includes a magnetically permeable material is described. The magnetically permeable material is imbedded in a target base that is brazed, welded, or soldered to the sputter target, or is mechanically retained in the target material. Target attachment to the cathode is achieved by virtue of the permanent magnets and/or the pole pieces in the cathode assembly that create magnetic flux lines adjacent to the backing plate, which strongly attract the magnetically permeable material in the target assembly. 11 figures.

Makowiecki, D.M.; McKernan, M.A.

1994-02-15

340

Quick response targeting program  

NASA Technical Reports Server (NTRS)

Computer program generates and verifies operational launch vehicle targeting presettings for lunar free-return missions and lunar landing missions. Program is applicable in astronomy and nuclear physics, and in areas where improved targeting techniques are valuable.

Bosley, J. T.

1971-01-01

341

administrative_supplement_target  

Cancer.gov

NCI Guidelines for Administrative Supplements in Support of Expanding the Childhood Cancer TARGET Initiative Title: Childhood Cancer TARGET Initiative Expansion – Tissue Collections and Characterization Announcement Number: NOT-OD-09-056 NIH Announces

342

FLIR target screening  

NASA Technical Reports Server (NTRS)

Methods for the segmentation and recognition of individual targets sensed with forward looking infrared detectors are discussed. Particular attention is given to an adaptive multi-scenario target screener.

Aggarwal, R.

1982-01-01

343

Target Heart Rates  

MedlinePLUS

... learn how to calculate and monitor your target training heart rate, you have to know your resting heart rate. ... Now you’re ready to determine your target training heart rate. As you exercise, periodically: Take your pulse on ...

344

Polarized nuclear targets  

SciTech Connect

The static and dynamic methods for producing polarized targets are briefly discussed and compared. Nuclei that have been polarized by these methods are summarized. The equipment necessary for producing a working target is described, as are the capabilities presently available at LAMPF. A short description is presented of a polarized /sup 13/C target proposed for use at the LAMPF HRS spectrometer, as an example of a polarized target for use in nuclear physics research.

Jarmer, J.J.

1986-01-01

345

Resonances of radar targets and target discrimination  

NASA Astrophysics Data System (ADS)

The topic of the research program covers methods for target discrimination by radar scattering, making use of the natural resonances of the targets. The general plan of the program consists in a study of resonances of targets of simple shape (e.g., sphere) but complex composition (e.g., dielectric coating of conductors) during the first year, followed by simple conducting targets and comparison with parallel measurements performed at NSWC Dahlgren in the second year, with further extension to conductors of more complex shapes, using Waterman:s methods, later on. The emphasis of our study is two-fold, namely: (1) to study ways how the resonance structure of the radar echo can be used for identifying the nature of the target (the "inverse scattering problem"); and (2) to obtain a physical understanding of the phenomena that cause the resonances, and how they manifest themselves in both steady-state and pulsed echoes. Such an understanding, in preference to a more mechanical use of the singularity expansion method, will provide us with the true capability of mastering the target discrimination problem.

Uberall, H.

1980-03-01

346

DTP - Molecular Targets  

Cancer.gov

Thousands of molecular targets have been measured in the NCI panel of 60 human tumor cell lines. Measurements include protein levels, RNA measurements, mutation status and enzyme activity levels. You can choose to search for a target of interest, or you may browse through a list of targets.

347

Inflation Forecast Targeting: Implementing and Monitoring Inflation Targets  

Microsoft Academic Search

Inflation targeting is shown to imply inflation forecast targeting: the central bank's inflation forecast becomes an explicit intermediate target. Inflation forecast targeting simplifies both implementation and monitoring of monetary policy. The weight on output stabilization determines how quickly the inflation forecast is adjusted towards the inflation target. Money growth or exchange rate targeting is generally inferior to inflation targeting and

Lars E. O. Svensson

1997-01-01

348

Targeted cancer therapies  

PubMed Central

With unprecedented understanding of molecular events underlying human cancer in this genomic era, a large number of drugs specifically targeting hypothesized oncogenic drivers to which tumors are potentially addicted to have been developed and continue to be developed. These targeted cancer therapies are being actively tested in clinical trials with mixed successes. This editorial provides an overview on successful targeted cancer drugs on the market and those drugs that are in late clinical development stages. Importantly, the article lays out main challenges in developing molecular targeted therapies and potential path forward to overcome these challenges, as well as opportunities for China in this new era of targeted agents. The editorial serves as an introduction to the Targeted Cancer Therapies series that will review in depth of major pathways and drugs targeting these pathways to be published in the coming issues of the Chinese Journal of Cancer.

Yan, Li; Rosen, Neal; Arteaga, Carlos

2011-01-01

349

Bar coded retroreflective target  

DOEpatents

This small, inexpensive, non-contact laser sensor can detect the location of a retroreflective target in a relatively large volume and up to six degrees of position. The tracker's laser beam is formed into a plane of light which is swept across the space of interest. When the beam illuminates the retroreflector, some of the light returns to the tracker. The intensity, angle, and time of the return beam is measured to calculate the three dimensional location of the target. With three retroreflectors on the target, the locations of three points on the target are measured, enabling the calculation of all six degrees of target position. Until now, devices for three-dimensional tracking of objects in a large volume have been heavy, large, and very expensive. Because of the simplicity and unique characteristics of this tracker, it is capable of three-dimensional tracking of one to several objects in a large volume, yet it is compact, light-weight, and relatively inexpensive. Alternatively, a tracker produces a diverging laser beam which is directed towards a fixed position, and senses when a retroreflective target enters the fixed field of view. An optically bar coded target can be read by the tracker to provide information about the target. The target can be formed of a ball lens with a bar code on one end. As the target moves through the field, the ball lens causes the laser beam to scan across the bar code.

Vann, Charles S. (Fremont, CA)

2000-01-01

350

Effective neutron targets  

SciTech Connect

Because of the lack of a free neutron target, deuterium targets have been used extensively in studying the neutron structure. The unique spin structure of the {sup 3}He ground state wave function and the recent developments in laser technologies made polarized {sup 3}He targets widely used in many experiments from neutron electromagnetic form factor studies to nucleon spin structure function measurements at all major electron accelerator facilities. In this talk, the current status of the polarized {sup 3}He targets will be reviewed. The author will focus on neutron electromagnetic form factor studies using polarized {sup 3}He targets. The polarized nucleon spin structure function measurements using polarized {sup 3}He targets will also be discussed.

Gao, H.

1997-07-01

351

Inertial Confinement fusion targets  

NASA Technical Reports Server (NTRS)

Inertial confinement fusion (ICF) targets are made as simple flat discs, as hollow shells or as complicated multilayer structures. Many techniques were devised for producing the targets. Glass and metal shells are made by using drop and bubble techniques. Solid hydrogen shells are also produced by adapting old methods to the solution of modern problems. Some of these techniques, problems, and solutions are discussed. In addition, the applications of many of the techniques to fabrication of ICF targets is presented.

Hendricks, C. D.

1982-01-01

352

Integrin Targeted MR Imaging  

PubMed Central

Magnetic resonance imaging (MRI) is a powerful medical diagnostic imaging modality for integrin targeted imaging, which uses the magnetic resonance of tissue water protons to display tissue anatomic structures with high spatial resolution. Contrast agents are often used in MRI to highlight specific regions of the body and make them easier to visualize. There are four main classes of MRI contrast agents based on their different contrast mechanisms, including T1, T2, chemical exchange saturation transfer (CEST) agents, and heteronuclear contrast agents. Integrins are an important family of heterodimeric transmembrane glycoproteins that function as mediators of cell-cell and cell-extracellular matrix interactions. The overexpressed integrins can be used as the molecular targets for designing suitable integrin targeted contrast agents for MR molecular imaging. Integrin targeted contrast agent includes a targeting agent specific to a target integrin, a paramagnetic agent and a linker connecting the targeting agent with the paramagnetic agent. Proper selection of targeting agents is critical for targeted MRI contrast agents to effectively bind to integrins for in vivo imaging. An ideal integrin targeted MR contrast agent should be non-toxic, provide strong contrast enhancement at the target sites and can be completely excreted from the body after MR imaging. An overview of integrin targeted MR contrast agents based on small molecular and macromolecular Gd(III) complexes, lipid nanoparticles and superparamagnetic nanoparticles is provided for MR molecular imaging. By using proper delivery systems for loading sufficient Gd(III) chelates or superparamagnetic nanoparticles, effective molecular imaging of integrins with MRI has been demonstrated in animal models.

Tan, Mingqian; Lu, Zheng-Rong

2011-01-01

353

Infrared target array development  

NASA Astrophysics Data System (ADS)

The US Army Yuma Proving Ground (USAYPG) was requested to develop and acquire a series of infrared targets with controllable thermal signatures to support the test and evaluation of the Target Acquisition Designation System/Pilot Night Vision System (TADS/PNVS) subsystems of the Advanced Attack Helicopter (AAH) Fire Control System. Prior to this development effort, no capability beyond the use of real-scene targets existed at USAYPG to provide thermally active targets with characteristic signatures in the infrared band. Three targets were acquired: (1) a detection target; (2) a recognition target; and (3) a laser scoring board. It is concluded that design goals were met and the system was delivered in time to perform its function. The system provides sufficient thermal realism and has advanced the state-of-the-art of infrared imaging system test and evaluation. It is recommended that the Field Equivalent Bar Target (FEBT) system be validated as a potential test standard and that environmentally 'hardened' targets be acquired for continued thermal sight testing.

Scott, E. A.

1980-04-01

354

[Target infrared detection in target spray].  

PubMed

Crops in agriculture and forestry are normally planted discretely. The chemical sprayed between crops would cause great waste and serious environment pollution. Therefore realization of the precision spray has great significance. This research discussed the method to realize automatic target detection using infrared detect technology. The infrared can avoid the interference of the visible light effectively and the response speed is very fast. Therefore it can be used to implement non-tough detection. Photoelectric detection systems based on infrared detect technology are normally stable, reliable, low cost, simple structure, and easy to be practically utilized. Therefore it is widely used in the on-line real time detection field. Its key point is to determinate the characteristic wavelength or wave band. The infrared lights emitted from the infrared light emitting diode were irradiated to the detected objects. The reflected infrared lights could be received by the photoelectric device. Then control signal was triggered and automatic target spray was realized. Code-division infrared detection circuit was used in the system. Modulated pulse infrared signals using different coding were used in different photodetector units in the built system so as to eliminate the light path interference between different detector units and other light signal interferences. Therefore the interference capacity of the system is high. The test results showed that the automatic target spray equipment set up in the study could detect crop targets automatically. The light wavelength used in the test is 850 nm. The detection range was tunable within 0.1-0.5 m. The least targets detectable distance was less than 0.3 m. PMID:19123390

Deng, Wei; He, Xiong-kui; Zhang, Lu-da; Zeng, Ai-jun; Song, Jian-li; Zou, Jian-jun

2008-10-01

355

The Effects of Feedback on Targeting with Multiple Moving Targets  

Microsoft Academic Search

A number of task settings involve selection of objects from dynamic visual environments with multiple moving targets. Target selection is difficult in these settings because objects move, because there are a number of distracter objects for any targeting action, and because objects can occlude the target. Target feedback has been suggested as a way to assist targeting in visual environments.

David Mould; Carl Gutwin

2004-01-01

356

GWAS and drug targets  

PubMed Central

Background Genome wide association studies (GWAS) have revealed a large number of links between genome variation and complex disease. Among other benefits, it is expected that these insights will lead to new therapeutic strategies, particularly the identification of new drug targets. In this paper, we evaluate the power of GWAS studies to find drug targets by examining how many existing drug targets have been directly 'rediscovered' by this technique, and the extent to which GWAS results may be leveraged by network information to discover known and new drug targets. Results We find that only a very small fraction of drug targets are directly detected in the relevant GWAS studies. We investigate two possible explanations for this observation. First, we find evidence of negative selection acting on drug target genes as a consequence of strong coupling with the disease phenotype, so reducing the incidence of SNPs linked to the disease. Second, we find that GWAS genes are substantially longer on average than drug targets and than all genes, suggesting there is a length related bias in GWAS results. In spite of the low direct relationship between drug targets and GWAS reported genes, we found these two sets of genes are closely coupled in the human protein network. As a consequence, machine-learning methods are able to recover known drug targets based on network context and the set of GWAS reported genes for the same disease. We show the approach is potentially useful for identifying drug repurposing opportunities. Conclusions Although GWA studies do not directly identify most existing drug targets, there are several reasons to expect that new targets will nevertheless be discovered using these data. Initial results on drug repurposing studies using network analysis are encouraging and suggest directions for future development.

2014-01-01

357

Antiplatelet antibody testing in thrombocytopenic pregnant women  

Microsoft Academic Search

OBJECTIVE: The purpose of the study was to attempt to distinguish pregnant women with gestational thrombocytopenia from those with idiopathic immune thrombocytopenia by eight different platelet antibody assays. STUDY DESIGN: Sera from pregnant women with presumed gestational thrombocytopenia (n = 160) and idiopathic immune thrombocytopenia (n = 90) were prospectively tested for indirect and platelet-associated immunoglobulins G and M and

Keith B. Lescale; Keith A. Eddleman; Douglas B. Cines; Philip Samuels; Martin L. Lesser; Janice G. McFarland; James B. Bussel

1996-01-01

358

Clinical uses of radiolabeled platelets  

SciTech Connect

Platelets were first successfully radiolabeled in 1953. At that time, investigators were primarily interested in developing a technique to accurately measure platelet life span in both normal and thrombocytopenic patients. Studies using platelets labeled with /sup 51/Cr have shown shortened platelet survival times in a number of diseases including idiopathic thrombocytopenic purpura, coronary artery disease, and diabetes mellitus. More recently, labels such as /sup 111/In have been developed that allow in vivo imaging of platelets. Indium-111 platelets are being used to better understand the pathophysiology of atherosclerosis, thrombophlebitis, pulmonary embolism and clotting disorders, and to improve the clinical diagnosis of these diseases.

Datz, F.L.; Christian, P.E.; Baker, W.J.

1985-12-01

359

Target Motion Analysis Visualisation  

Microsoft Academic Search

Target motion analysis (TMA) visualisation is used by naval ships to locate targets around ownship when more accurate methods such as active sonar are not viable. TMA utilises an array of data, the main source being passive sonar, and results in an almost infinite number of possible solutions. These solutions must be filtered by the TMA operator to identify the

Andrew Cunningham; Bruce H. Thomas

2005-01-01

360

Targets, backgrounds, and discrimination  

Microsoft Academic Search

The present volume discusses a model-based aircraft identification technique, target intensity and angle scintillations, spatiotemporal nonstationary scene generation, an overview of the Strategic Scene Generation Model (SSGM), nuclear backgrounds for SSGM, and an atmospheric and transmittance code for 50-300 km altitudes. Also discussed are a data base for airborne target signatures, the auroral module of the Strategic High Altitude Radiance

J. S. Accetta; G. H. Kelley

1992-01-01

361

Biosonar Target Fabrication.  

National Technical Information Service (NTIS)

An active target for use in the study of dolphin echolocation has been designed, built, and tested by Applied Research Laboratories, The University of Texas at Austin. The active target is an electronic/acoustical device that transmits a programmed synthe...

K. J. Diercks B. R. Criswell

1975-01-01

362

Moving target exploitation  

NASA Astrophysics Data System (ADS)

The understanding of maneuvering forces is invaluable to the warfighter, as it enhances understanding of enemy force structure and disposition, provides cues to potential enemy actions, and expedites targeting of time critical targets. Airborne ground moving target indicator (GMTI) radars are a class of highly-effective, all-weather, wide-area senors that aid in the surveillance of these moving ground vehicles. Unfortunately conventional GMTI radars are incapable of identifying individual vehicles, and techniques for exploiting information imbedded within GMTI radar reports are limited. The Defense Advanced Research Projects Agency (DARPA) Moving Target Exploitation (MTE) program is working to mitigate these deficiencies by developing, integrating, and evaluating a suite of automated and semi-automated technologies to classify moving targets and units, and to provide indications of their activities. These techniques include: aid in the interpretation of GMTI data to provide moving force structure analysis, automatic tracking of thousands of moving ground vehicles, 1-D target classification based upon high-range- resolution (HRR) radar profiles, and 2-D target classification based upon moving target imaging (MTIm) synthetic aperture radar (SAR). This paper shall present the MTE concept and motivation and provide an overview of results to date.

Johnson, Bruce L.; Grayson, Timothy P.

1998-08-01

363

Targeted cryptosporidium biocides  

US Patent & Trademark Office Database

The present invention relates to fusion proteins comprising a microorganism targeting molecule (e.g., immunoglobulin) and a biocide. The present invention also relates to therapeutic and prophylactic methods of using a fusion protein comprising a microorganism targeting molecule and a biocide in diverse fields.

2014-04-22

364

Knowing Your Learning Target  

ERIC Educational Resources Information Center

No matter what we decide students need to learn, not much will happen until students understand what they are supposed to learn during a lesson and set their sights on learning it. Crafting learning targets for each lesson and deliberately sharing them with students is one way to give students the direction they need. Targets that tell students…

Moss, Connie M.; Brookhart, Susan M.; Long, Beverly A.

2011-01-01

365

Conditional gene targeting  

Microsoft Academic Search

Gene targeting: The classical approach Central to an understanding of the in vivo function of genes is their analysis by mutation, that is, inactivation or modification of a gene by mutation and the study of the consequences of the mutation in the mutant organism. In mammals, before gene targeting, this approach was limited to the rare spontaneous mutations reflected in

Klaus Rajewsky; Hua Gu; Ralf Kühn; Ulrich A. K. Betz; Werner Müller; Jürgen Roes; Frieder Schwenk

1996-01-01

366

Scanner analyzer target  

Microsoft Academic Search

This paper describes a black and white photographic chart called the 'Scanner Analyzer Target' developed as a practical aid toward analyzing scanned image quality produced by binary digital document scanners used as input devices in document image processing systems. The target is used by factory-line personnel as an 'adjustment-set' tool, by the quality control department as an image quality judgement

Roland Simonis

1991-01-01

367

Wideband multiple target tracking  

Microsoft Academic Search

We propose a new scheme involving autoregressive parameter estimation and pattern classification with maximum likelihood (ML) direction of arrival (DOA) estimation to track multiple targets moving in the far-field. The targets are sources of wideband signals which impinge on a uniform linear array of passive sensors. These wideband signals are modeled as vector autoregressive (AR) models so that the spectral

A. Satish; Ratagasard L. Kashyap

1994-01-01

368

Setting reference targets.  

National Technical Information Service (NTIS)

Reference Targets are used to represent virtual quantities like the magnetic axis of a magnet or the definition of a coordinate system. To explain the function of reference targets in the sequence of the alignment process, this paper will first briefly di...

R. E. Ruland

1997-01-01

369

Inflation Targeting: Some Extensions  

Microsoft Academic Search

Previous analyses of the implementation of inflation targeting are extended to monetary policy responses to different shocks, consequences of model uncertainty, and effects of interest rate smoothing and stabilization. Model uncertainty, output stabilization, and interest rate stabilization or smoothing all call for a more gradual adjustment of the conditional inflation forecast toward the inflation target. The conditional inflation forecast is

Lars E. o. Svensson

1998-01-01

370

Tissue Targeted Complement Modulators.  

National Technical Information Service (NTIS)

Systemic suppression of the complement system has been shown to be effective to treat inflammatory disease, yet at the potential cost of compromising host defense and immune homeostasis. Herein disclosed are methods for antigen-specific targeting of compl...

R. J. Quigg S. Tomlinson

2005-01-01

371

Target Discrimination Apparatus.  

National Technical Information Service (NTIS)

The target discrimination apparatus utilizes logic circuit which is positioned between the detection window and the clutter averaging cells of a conventional constant false alarm rate (CFAR) radar detector to provide better estimation of the clutter avera...

P. W. Chen

1979-01-01

372

Targeted Information Dissemination.  

National Technical Information Service (NTIS)

Quantum Leap Innovations (QLI) developed a Targeted Information Dissemination (TID) system for rapid gathering and dissemination of the right information to the right people at the right time. The TID user interface shows tasks of an analyst. A hierarchic...

S. Kallurkar

2008-01-01

373

Multiple Target Tracking Study.  

National Technical Information Service (NTIS)

A particular multiple target tracking mission was studied to determine the radar requirements. Several phased array radar configurations are described. A limited scan array is selected as the most cost effective. The phased array radar is compared with a ...

E. M. Brown D. N. Thomson

1974-01-01

374

Radar Target Approach Simulator.  

National Technical Information Service (NTIS)

A technique employed to generate nondispersive, continuously variable microwave time delay for the simulation of the approach of a radar to its target is described. A bulk elastic wave excited in a transparent crystal enables thousands of feet of propagat...

H. A. Cook

1971-01-01

375

Wavelet radar target classification  

Microsoft Academic Search

Ground-based S-band radars with center frequencies in the 3 GHz range, and bandwidths of about 2 MHz, are currently being considered for the classification\\/identification of targets in the 50 - 250 km range (low signal-to-noise (SNR) environment). Its main drawback is that it is time and computation intensive, and, for targets at long range, it usually requires a number of

Pankaj N. Topiwala; Chojan S. Teng

1995-01-01

376

Cooperative Target-capturing with Incomplete Target Information.  

National Technical Information Service (NTIS)

This paper presents a distributed target-centric formation control strategy for multiple unmanned aerial vehicles (UAVs) in the presence of target motion uncertainty. The formation is maintained around a target using a combination of a consensus protocol ...

D. Pack I. Postlethwaite M. Kothari R. Beard R. Sharma

2013-01-01

377

Airborne Passive Target Motion Analysis.  

National Technical Information Service (NTIS)

Kalman filtering techniques are applied to a two sensor bearings only passive target motion analysis problem. An algorithm is developed to simulate tracking long range maneuvering airborne targets. The target tracking performance of the filter is evaluate...

J. A. Gutzler

1987-01-01

378

Penetration of concrete targets  

SciTech Connect

We developed penetration equations for ogive-nosed projectiles that penetrated concrete targets after normal impact. Our penetration equations predict axial force on the projectile nose, rigid-body motion, and final penetration depth. For target constitutive models, we conducted triaxial material experiments to confining pressures of 600 MPa and curve-fit these data with a linear pressure-volumetric strain relation and with a linear Mohr-Coulomb, shear strength-pressure relation. To verify our penetration equations, we conducted eleven penetration experiments with 0.90 kg, 26.9-mm-diameter, ogive-nosed projectiles into 1.37-m-diameter concrete targets with unconfined compressive strengths between 32-40 MPa. Predictions from our penetration equation are compared with final penetration depth measurements for striking velocities between 280--800 m/s.

Forrestal, M.J. [Sandia National Labs., Albuquerque, NM (United States); Cargile, J.D. [Corps of Engineers, Vicksburg, MS (United States). Waterways Experiment Station; Tzou, R.D.Y. [New Mexico Univ., Albuquerque, NM (United States). Dept. of Mechanical Engineering

1993-08-01

379

Setting reference targets  

SciTech Connect

Reference Targets are used to represent virtual quantities like the magnetic axis of a magnet or the definition of a coordinate system. To explain the function of reference targets in the sequence of the alignment process, this paper will first briefly discuss the geometry of the trajectory design space and of the surveying space, then continue with an overview of a typical alignment process. This is followed by a discussion on magnet fiducialization. While the magnetic measurement methods to determine the magnetic centerline are only listed (they will be discussed in detail in a subsequent talk), emphasis is given to the optical/mechanical methods and to the task of transferring the centerline position to reference targets.

Ruland, R.E.

1997-04-01

380

Phoenix Color Targets  

NASA Technical Reports Server (NTRS)

These images of three Phoenix color targets were taken on sols 1 and 2 by the Surface Stereo Imager (SSI) on board the Phoenix lander. The bottom target was imaged in approximate color (SSI's red, green, and blue filters: 600, 530, and 480 nanometers), while the others were imaged with an infrared filter (750 nanometers). All of them will be imaged many times over the mission to monitor the color calibration of the camera. The two at the top show grains 2 to 3 millimeters in size that were likely lifted to the Phoenix deck during landing. Each of the large color chips on each target contains a strong magnet to protect the interior material from Mars' magnetic dust.

The Phoenix Mission is led by the University of Arizona, Tucson, on behalf of NASA. Project management of the mission is by NASA's Jet Propulsion Laboratory, Pasadena, Calif. Spacecraft development is by Lockheed Martin Space Systems, Denver.

2008-01-01

381

Integrin Targeted Therapeutics  

PubMed Central

Integrins are heterodimeric, transmembrane receptors that function as mechanosensors, adhesion molecules and signal transduction platforms in a multitude of biological processes. As such, integrins are central to the etiology and pathology of many disease states. Therefore, pharmacological inhibition of integrins is of great interest for the treatment and prevention of disease. In the last two decades several integrin-targeted drugs have made their way into clinical use, many others are in clinical trials and still more are showing promise as they advance through preclinical development. Herein, this review examines and evaluates the various drugs and compounds targeting integrins and the disease states in which they are implicated.

Millard, Melissa; Odde, Srinivas; Neamati, Nouri

2011-01-01

382

Foam encapsulated targets  

DOEpatents

Foam encapsulated laser-fusion targets wherein a quantity of thermonuclear fuel is embedded in low density, microcellular foam which serves as an electron conduction channel for symmetrical implosion of the fuel by illumination of the target by one or more laser beams. The fuel, such as DT, is contained within a hollow shell constructed of glass, for example, with the foam having a cell size of preferably no greater than 2 .mu.m, a density of 0.065 to 0.6.times.10.sup.3 kg/m.sup.3, and external diameter of less than 200 .mu.m.

Nuckolls, John H. (Livermore, CA); Thiessen, Albert R. (Livermore, CA); Dahlbacka, Glen H. (Livermore, CA)

1983-01-01

383

Targeted polypeptide degradation  

DOEpatents

This invention pertains to compositions, methods, cells and organisms useful for selectively localizing polypeptides to the proteasome for degradation. Therapeutic methods and pharmaceutical compositions for treating disorders associated with the expression and/or activity of a polypeptide by targeting these polypeptides for degradation, as well as methods for targeting therapeutic polypeptides for degradation and/or activating therapeutic polypeptides by degradation are provided. The invention provides methods for identifying compounds that mediate proteasome localization and/or polypeptide degradation. The invention also provides research tools for the study of protein function.

Church, George M. (Brookline, MA) [Brookline, MA; Janse, Daniel M. (Brookline, MA) [Brookline, MA

2008-05-13

384

Laboratory evaluation of a bleeding patient.  

PubMed Central

Most causes of abnormal bleeding can be determined from a complete blood count including platelet count and bleeding, prothrombin, activated partial thromboplastin, and thrombin times. Occasionally, further evaluation is necessary, such as tests of factor XIII function, fibrinolysis, and vascular integrity. Possible diagnoses include disseminated intravascular coagulation, thrombotic thrombocytopenic purpura, vitamin K deficiency, von Willebrand's disease, heparin-induced thrombocytopenia, acquired inhibitors of factor VIII, lupus anticoagulants, and coagulation disorders related to the acquired immunodeficiency syndrome.

Wallerstein, R O

1989-01-01

385

Severe Thrombocytopenia in Aged Rhesus Macaques (Macaca mulatta) Infected with Simian Varicella Virus  

PubMed Central

Simian varicella virus was diagnosed in 2 geriatric rhesus macaques (Macaca mulatta). The macaques presented with typical skin lesions as well as severe thrombocytopenia as a result of infection. Idiopathic thrombocytopenic purpura is a known complication of varicella zoster virus infection in humans; however, this condition has not been reported previously as a complication of SVV infection. This case report discusses the clinical presentation, pathology, and thrombocytopenia of the affected macaques.

Halliday, Lisa C; Fortman, Jeffrey D

2011-01-01

386

Ocular sarcoidosis and immune thrombocytopenia: a rare association.  

PubMed

There is an association between sarcoidosis and immune thrombocytopenia, which can be severe and even fatal if left untreated. Although ocular sarcoidosis is one of the most common extrapulmonary manifestations of sarcoidosis, immune thrombocytopenia has not been reported in isolated ocular sarcoidosis. We present a patient with a remote history of immune thrombocytopenic purpura who developed a sub-conjunctival mass consistent with isolated ocular sarcoidosis. PMID:24144263

Chen, John J; Allen, Richard C

2014-02-01

387

Splenic scintigraphy using Tc99m-labeled heat-denatured red blood cells in pediatric patients: concise communication  

Microsoft Academic Search

Ten children underwent splenic imaging with heat-denatured red blood cells labeled with technetium-99m (Tc-99m DRBC). The presenting problems included the heterotaxia syndrome, recurrent idiopathic thrombocytopenic purpura following splenectomy, mass in the left posterior hemithorax, and blunt abdominal trauma. In nine patients, the presence or absence of splenic tissue was established. A splenic hematoma was identified in the tenth patient. All

C. P. Ehrlich; N. Papanicolaou; S. Treves; R. A. Hurwitz; P. Richards

1982-01-01

388

Characterization of Conformation-Sensitive Antibodies to ADAMTS13, the von Willebrand Cleavage Protease  

Microsoft Academic Search

BackgroundThe zinc metalloprotease ADAMTS13 is a multidomain protein that cleaves von Willebrand Factor (VWF) and is implicated in Thrombotic Thrombocytopenic Purpura (TTP) pathogenesis. Understanding the mechanism of this protein is an important goal. Conformation sensitive antibodies have been used to monitor protein conformation and to decipher the molecular mechanism of proteins as well as to distinguish functional and non-functional mutants.Methodology\\/Principal

Zuben E. Sauna; Chinyere Okunji; Ryan C. Hunt; Tanvi Gupta; Courtni E. Allen; Elizabeth Plum; Adam Blaisdell; Vahan Grigoryan; Geetha S; Robert Fathke; Kenji Soejima; Chava Kimchi-Sarfaty; Floyd Romesberg

2009-01-01

389

47,XXX in an Adolescent with Premature Ovarian Failure and Autoimmune Disease  

Microsoft Academic Search

Background: Premature ovarian failure (POF) may be idiopathic or may be associated with genetic or autoimmune disorders. The 47,XXX karyotype has been associated with POF and other genitourinary anomalies.Case: A 17-year-old woman with a history of immune thrombocytopenic purpura was referred to the adolescent medicine clinic for evaluation of oligomenorrhea with secondary amenorrhea. Evaluation revealed hypergonadotrophic premature ovarian failure, a

C. M Holland

2001-01-01

390

Mutation analysis and clinical implications of von Willebrand factor–cleaving protease deficiency  

Microsoft Academic Search

Mutation analysis and clinical implications of von Willebrand factor–cleaving protease deficiency.BackgroundThe pentad of thrombocytopenia, hemolytic anemia, mild renal dysfunction, neurologic signs, and fever, classically characterizes the syndrome of thrombotic thrombocytopenic purpura (TTP). TTP usually occurs in adults as an acquired form but a congenital form in children has also been described. In the latter case, the initial presentation is often

Karin Assink; Rikke Schiphorst; Sarah Allford; Diana Karpman; Amos Etzioni; Bénédicte Brichard; Nicole Van De Kar; Leo Monnens; Lambertus Van Den Heuvel

2003-01-01

391

A new method for active surveillance of adverse events from diphtheria\\/tetanus\\/pertussis and measles\\/mumps\\/rubella vaccines  

Microsoft Academic Search

We describe a new method for active post-marketing surveillance of vaccine safety based on patient records. We studied the association between diphtheria\\/ tetanus\\/pertussis (DTP) vaccination and febrile convulsion, and between measles\\/mumps\\/rubella (MMR) vaccination and febrile convulsion and idiopathic thrombocytopenic purpura (ITP) in five district health authorities in England by linking vaccination records with computerised hospital admission records. We found an

P Farrington; M Rush; E Miller; S Pugh; A Colville; A Flower; J Nash; P Morgan-Capner

1995-01-01

392

Severe deficiency of the specific von Willebrand factor-cleaving protease (ADAMTS 13) activity in a subgroup of children with atypical hemolytic uremic syndrome  

Microsoft Academic Search

Objective The von Willebrand factor-cleaving protease (VWF-cp) activity has been reported to be deficient in adults with thrombotic thrombocytopenic purpura (TTP) and generally normal in adults with hemolytic uremic syndrome (HUS). The goal of this study was to determine VWF-cp activity in children with typical postdiarrheal (d+) HUS or atypical non-postdiarrheal (d-) HUS. Study design We measured VWF-cp activity in

Agnès Veyradier; Bernadette Obert; Elie Haddad; Sylvie Cloarec; Hubert Nivet; Michel Foulard; François Lesure; Pierre Delattre; Mustapha Lakhdari; Dominique Meyer; Jean-Pierre Girma; Chantal Loriat

2003-01-01

393

Amino acid regions 572-579 and 657-666 of the spacer domain of ADAMTS 13 provide a common antigenic core required for binding of antibodies in patients with acquired TTP  

Microsoft Academic Search

Antibodies directed against ADAMTS13 have been detected in the majority of patients with acquired thrombotic thrombocytopenic purpura (TTP). We have previously localized a major antigenic determinant within the spacer domain of ADAMTS13. To identify the amino acid residues of the spacer domain that are involved in binding of anti-ADAMTS13 antibodies, we constructed a series of fifteen hybrids (designated A-O) in

Brenda M. Luken; Ellen A. M. Turenhout; Paul H. P. Kaijen; Mascha J. Greuter; Wouter Pos; Mourik van J. A; Rob Fijnheer; Jan Voorberg

2006-01-01

394

Evaluation and clinical application of a new method for measuring activity of von Willebrand factor-cleaving metalloprotease (ADAMTS13)  

Microsoft Academic Search

Thrombotic thrombocytopenic purpura (TTP) is associated with acquired or congenital deficiency of a plasma von Willebrand factor-cleaving protease (VWFcp). Based on partial amino acid sequence and genome-wide linkage analysis of pedigrees with congenital TTP, VWFcp was recently identified as a new member of the ADAMTS family and designated ADAMTS13. We developed a new, rapid, and simple method for measuring VWFcp

M. Böhm; T. Vigh; I. Scharrer

2002-01-01

395

Reduced ADAMTS13 activity in delayed cerebral ischemia after aneurysmal subarachnoid hemorrhage  

Microsoft Academic Search

The pathogenesis of delayed cerebral ischemia (DCI) after aneurysmal subarachnoid hemorrhage (SAH) remains unknown. Besides vasospasm, microthrombosis might have an important function. As in patients with thrombotic thrombocytopenic purpura an A Disintegrin And Metalloprotease with ThromboSpondin repeats-13 (ADAMTS13) deficiency leads to higher concentrations of large von Willebrand factor (vWF) multimers resulting in microthrombosis, our purpose was to compare ADAMTS13 and

Mervyn DI Vergouwen; Kamran Bakhtiari; Nan van Geloven; Marinus Vermeulen; Yvo BWEM Roos; Joost CM Meijers; MDI Vergouwen

2009-01-01

396

Rituximab Off Label Use for Difficult-To-Treat AutoImmune Diseases: Reappraisal of Benefits and Risks  

Microsoft Academic Search

Rituximab is increasingly used off label for difficult-to-treat auto-immune diseases. We reviewed the main case series or\\u000a clinical studies to identify the best indications of rituximab and the situations at substantial risks for adverse events.\\u000a Refractory immune thrombocytopenic purpura was the main indication. However, the long term benefit-to-risk ratio of rituximab\\u000a treatment before or after splenectomy is unknown. A single

Laurent Sailler

2008-01-01

397

Plasma Exchange Therapy in Steroid-Unresponsive Relapses in Patients with Multiple Sclerosis  

Microsoft Academic Search

Background:Plasma exchange (PE) is well established for conditions such as rapid progressive vasculitis associated with autoantibodies against neutrophil cytoplasmic antigens (ANCA), anti-glomerular basement membrane (GBM) antibody disease, or thrombotic thrombocytopenic purpura (TTP). Also, several neurological disorders, such as acute worsening in myasthenia gravis, Guillan-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP), can successfully be treated with PE. Only small

Corinna Trebst; Ansgar Reising; Jan T. Kielstein; Carsten Hafer; Martin Stangel

2009-01-01

398

Multiple autoimmune events after autologous bone marrow transplantation  

Microsoft Academic Search

A 36-year-old woman with RAEB-t and severe bone marrow fibrosis undergoing autologous BMT, developed a histologically documented GVHD-like skin rash. Thereafter, autoimmune thyroiditis, autoimmune thrombocytopenic purpura and autoimmune hemolytic anemia and a lupus anti-coagulant (LAC) were diagnosed. The patient is still alive, symptom-free and in first complete remission (CR); however, all of the autoantibodies are still detectable, with the exception

G Lambertenghi Deliliers; C Annaloro; A Della Volpe; A Oriani; E Pozzoli; D Soligo

1997-01-01

399

Inhibition of Autoantibody Binding to Platelet Glycoprotein lib\\/Illa by Anti-Idiotypic Antibodies in Intravenous Gammaglobulin  

Microsoft Academic Search

Intravenous immunoglobulin (lVlgG) causes an acute rise in the platelet count in the majority of patients with chronic immune thrombocytopenic purpura (ITP) but the mecha- nism(s) of action is still unknown. We evaluated the ability of three different IVIgG preparations to inhibit the in vitro binding of autoantibody to platelet glycoprotein (GP) llb\\/ lila. ITP plasma. known to contain anti-GPIIb\\/Illa

Peter Berchtold; George L. Dale; Patricia Tani; Robert McMillan

1989-01-01

400

Autoantibodies Against the Platelet Glycoprotein lib\\/Illa Complex in Patients With Chronic ITP  

Microsoft Academic Search

Chronic idiopathic thrombocytopenic purpura (ITP) is caused by an antibody reactive with platelet-associated antigens. The present studies provide direct evidence that some patients with chronic ITP have autoantibodies against the platelet glycoprotein (GP) lIb\\/Illa complex. Microtiter wells. coated with a monoclonal antibody (2G12) specific for GPIIb\\/GPIlIa were reacted with GPlIb\\/GPlIla contained in a platelet extract. Control wells containing the same

Virgil L. Woods; Esther H. Oh; Donna Mason; Robert McMillan

1984-01-01

401

Hépatite auto-immune familiale et déficit en C4  

Microsoft Academic Search

Familial auto-immune hepatitis and C4 deficit.Introduction. —Familial auto-immune hepatitis is unusual. We report a case in which hepatitis was associated with a deficiency in the C4 component of the complement.Exegesis. —Type I auto-immune hepatitis A was diagnosed in a 38-year-old woman presenting with systemic lupus erythematosus. Her daughter had to undergo a splenectomy for immunologic thrombocytopenic purpura when she was

J Constants; P Bernard; P Bioulac-Sage; D Barcat; C Conri

1998-01-01

402

Development of an underwater target classifier using target specific features  

Microsoft Academic Search

In Sonar, the detection and estimation functions are performed by signal processors, which involve the computation of various statistics, for enhancing the overall performance of the system. This also takes into account all the undesirable propagation effects caused by the underwater channel. Underwater targets can be classified by using certain target specific features such as target strength, target dynamics, and

M. H. Supriya; P. R. Saseendran Pillai

2003-01-01

403

Target-Rich Environment  

ERIC Educational Resources Information Center

Target marketing is defining school enrollment goals and then developing a strategic plan to accomplish those goals through the use of specific communication vehicles and community focus. It is critical to reach the right audience, with the right message, at the right time, for the right cost. In this brief article, the author describes several…

Perna, Mark C.

2005-01-01

404

Tritium high pressure target  

NASA Astrophysics Data System (ADS)

The design of a tritium high pressure target with a volume of 16.5 cm3, developed at the RFNC-VNIIEF for research of muon fusion catalyzed in a H-D-T hydrogen isotopic mixture at pressures up to 120 MPa in the range of operating temperatures 300-800 K, is presented.

Perevozchikov, V. V.; Yukhimchuk, A. A.; Demin, D. L.; Ganchuk, N. S.; Grebinnik, V. G.; Grishechkin, S. K.; Ishkov, P. D.; Khabarov, Yu. A.; Lobanov, V. N.; Malkov, I. L.; Tikhonov, V. I.; Zinov, V. G.

1999-06-01

405

High purity tungsten targets  

NASA Technical Reports Server (NTRS)

High purity tungsten, which is used for targets in X-ray tubes was considered for space processing. The demand for X-ray tubes was calculated using the growth rates for dental and medical X-ray machines. It is concluded that the cost benefits are uncertain.

1975-01-01

406

Target Fragmentation in Radiobiology.  

National Technical Information Service (NTIS)

Nuclear reactions in biological systems produce low-energy fragments of the target nuclei seen as local high events of linear energy transfer (LET). A nuclear-reaction formalism is used to evaluate the nuclear-induced fields within biosystems and their ef...

J. W. Wilson F. A. Cucinotta J. L. Shinn L. W. Townsend

1993-01-01

407

Targeted radionuclide therapy  

SciTech Connect

Targeted radionuclide therapy (TRT) seeks molecular and functional targets within patient tumor sites. A number of agents have been constructed and labeled with beta, alpha, and Auger emitters. Radionuclide carriers spanning a broad range of sizes; e.g., antibodies, liposomes, and constructs such as nanoparticles have been used in these studies. Uptake, in percent-injected dose per gram of malignant tissue, is used to evaluate the specificity of the targeting vehicle. Lymphoma (B-cell) has been the primary clinical application. Extension to solid tumors will require raising the macroscopic absorbed dose by several-fold over values found in present technology. Methods that may effect such changes include multistep targeting, simultaneous chemotherapy, and external sequestration of the agent. Toxicity has primarily involved red marrow so that marrow replacement can also be used to enhance future TRT treatments. Correlation of toxicities and treatment efficiency has been limited by relatively poor absorbed dose estimates partly because of using standard (phantom) organ sizes. These associations will be improved in the future by obtaining patient-specific organ size and activity data with hybrid SPECT/CT and PET/CT scanners.

Williams, Lawrence E.; DeNardo, Gerald L.; Meredith, Ruby F. [Radiology Division, City of Hope National Medical Center, Duarte, California 91010 (United States); Internal Medicine, University of California Davis Medical Center, 1508 Alhambra Boulevard, Suite 3100, Sacramento, California 95816 (United States); Department of Radiation Oncology, Wallace Tumor Institute WTI No. 117, University of Alabama at Birmingham, Birmingham, Alabama 35294 (United States)

2008-07-15

408

Concurrent multiple target tracking  

Microsoft Academic Search

A concurrent algorithm for multiple target tracking is presented. The underlying tracking formalism is first described by way of a sequential program, and the issues in generalizing the tracker for efficient concurrent implementations are discussed in detail. Typical tracking results on the Mark III hypercube are presented.

Thomas D. Gottschalk

1988-01-01

409

Phenylbutyrate targets in yeast  

Microsoft Academic Search

Phenylbutyrate (4-phenylbutyric acid, PB), is a non-toxic compound approved by the FDA for the treatment of urea cycle disorders. PB and its metabolite phenylacetate (PA) are effective antineoplastic agents in tissue culture and have shown promise as therapeutic drugs for prostate and other human cancers in clinical trials. Since the cellular targets of PB for growth inhibition are not known,

Andrew Marion Grzanowski

2000-01-01

410

Personnel Targeting Operations.  

National Technical Information Service (NTIS)

The U.S. military is not currently organized to conduct effective personnel targeting operations outside of the United States. The Department of Defense needs to create an organization that can find, capture or kill designated personnel. With the events o...

C. W. Clark

2003-01-01

411

Target Heart Rate Calculator  

MedlinePLUS

... workout Enter your age to find a target heart rate during exercise. You'll get the most out of your activities by staying within this range of heartbeats/minute. What is your age? years. How to Check Your Heart Rate Right after you stop exercising, take your pulse: ...

412

Genomic targets in saliva.  

PubMed

Saliva, the most accessible and noninvasive biofluid of our body, harbors a wide spectrum of biological analytes informative for clinical diagnostic applications. While proteomic constituents are a logical first choice as salivary diagnostic analytes, genomic targets have emerged as highly informative and discriminatory. This awareness, coupled with the ability to harness genomic information by high-throughput technology platforms such as genome-wide microarrays, ideally positions salivary genomic targets for exploring the value of saliva for detection of specific disease states and augmenting the diagnostic and discriminatory value of the saliva proteome for clinical applications. Buccal cells and saliva have been used as sources of genomic DNA for a variety of clinical and forensic applications. For discovery of disease targets in saliva, the recent realization that there is a transcriptome in saliva presented an additional target for oral diagnostics. All healthy subjects evaluated have approximately 3,000 different mRNA molecules in their saliva. Almost 200 of these salivary mRNAs are present in all subjects. Exploration of the clinical utility of the salivary transcriptome in oral cancer subjects shows that four salivary mRNAs (OAZ, SAT, IL8, and IL1b) collectively have a discriminatory power of 91% sensitivity and specificity for oral cancer detection. Data are also now in place to validate the presence of unique diagnostic panels of salivary mRNAs in subjects with Sjögren's disease. PMID:17435127

Zimmermann, Bernhard G; Park, Noh Jin; Wong, David T

2007-03-01

413

Target fragmentation in radiobiology  

NASA Technical Reports Server (NTRS)

Nuclear reactions in biological systems produce low-energy fragments of the target nuclei seen as local high events of linear energy transfer (LET). A nuclear-reaction formalism is used to evaluate the nuclear-induced fields within biosystems and their effects within several biological models. On the basis of direct ionization interaction, one anticipates high-energy protons to have a quality factor and relative biological effectiveness (RBE) of unity. Target fragmentation contributions raise the effective quality factor of 10 GeV protons to 3.3 in reasonable agreement with RBE values for induced micronuclei in bean sprouts. Application of the Katz model indicates that the relative increase in RBE with decreasing exposure observed in cell survival experiments with 160 MeV protons is related solely to target fragmentation events. Target fragment contributions to lens opacity given an RBE of 1.4 for 2 GeV protons in agreement with the work of Lett and Cox. Predictions are made for the effective RBE for Harderian gland tumors induced by high-energy protons. An exposure model for lifetime cancer risk is derived from NCRP 98 risk tables, and protraction effects are examined for proton and helium ion exposures. The implications of dose rate enhancement effects on space radiation protection are considered.

Wilson, John W.; Cucinotta, Francis A.; Shinn, Judy L.; Townsend, Lawrence W.

1993-01-01

414

ENFORCEMENT TARGETING 2001  

EPA Science Inventory

A GIS based targeting methodology which uses multi-media state and federal regulatory data to identify watersheds in Texas, Louisiana, Arkansas, Oklahoma, and New Mexico that are vulnerable to environmental damage and/or have high chemical emissions to the environment. The assess...

415

Target perceivability and its applications  

Microsoft Academic Search

A concept of target perceivability is introduced, which is related to such concepts as target existence and target observability. Its probability provides a basis for an integrated approach to track initiation, confirmation, termination, and refinement of track maintenance algorithms. This paper proposes the concepts of target perceivability and presents a recursion of its probability based on hidden Markov models (HMMs)

Ning Li; X. Rong Li

2001-01-01

416

Cellular Targeting in Autoimmunity  

PubMed Central

Many biologic agents that were first approved for the treatment of malignancies are now being actively investigated and used in a variety of autoimmune diseases such as rheumatoid arthritis (RA), antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, systemic lupus erythematosus (SLE), and Sjogren’s syndrome. The relatively recent advance of selective immune targeting has significantly changed the management of autoimmune disorders, and in part, can be attributed to the progress made in understanding effector cell function and their signaling pathways. In this review, we will discuss the recent FDA approved biologic therapies that directly target immune cells as well as the most promising investigational drugs affecting immune cell function and signaling for the treatment of autoimmune disease.

Rogers, Jennifer L.; Serafin, Donald S.; Timoshchenko, Roman G.; Tarrant, Teresa K.

2012-01-01

417

Complement-targeted therapeutics.  

PubMed

The complement system is a central component of innate immunity and bridges the innate to the adaptive immune response. However, it can also turn its destructive capabilities against host cells and is involved in numerous diseases and pathological conditions. Modulation of the complement system has been recognized as a promising strategy in drug discovery, and a large number of therapeutic modalities have been developed. However, successful marketing of complement-targeted drugs has proved to be more difficult than initially expected, and many strategies have been discontinued. The US Food and Drug Administration's approval of the first complement-specific drug, an antibody against complement component C5 (eculizumab; Soliris), in March 2007, was a long-awaited breakthrough in the field. Approval of eculizumab validates the complement system as therapeutic target and might facilitate clinical development of other promising drug candidates. PMID:17989689

Ricklin, Daniel; Lambris, John D

2007-11-01

418

Complement-targeted therapeutics  

PubMed Central

The complement system is a central component of innate immunity and bridges the innate to the adaptive immune response. However, it can also turn its destructive capabilities against host cells and is involved in numerous diseases and pathological conditions. Modulation of the complement system has been recognized as a promising strategy in drug discovery, and a large number of therapeutic modalities have been developed. However, successful marketing of complement-targeted drugs has proved to be more difficult than initially expected, and many strategies have been discontinued. The US Food and Drug Administration’s approval of the first complement-specific drug, an antibody against complement component C5 (eculizumab; Soliris), in March 2007, was a long-awaited breakthrough in the field. Approval of eculizumab validates the complement system as therapeutic target and might facilitate clinical development of other promising drug candidates.

Ricklin, Daniel; Lambris, John D

2010-01-01

419

Phoenix on Target  

NASA Technical Reports Server (NTRS)

This topography map illustrates where NASA's Phoenix Mars Lander is targeted to land on May 25, 2008, based on expectations as of noon pacific time (3 p.m. eastern time), May 24, 2008.

Phoenix is most likely to land at the cross-shaped target at the center of the red ellipse and least likely to land at the ellipse's edges. The ellipse is positioned over the northern arctic plains of Mars, and is approximately 70 kilometers (44 miles) long.

The topography data was taken by NASA's Mars Global Surveyor. It shows exaggerated differences in the height of the terrain.

The Phoenix Mission is led by the University of Arizona, Tucson, on behalf of NASA. Project management of the mission is by NASA's Jet Propulsion Laboratory, Pasadena, Calif. Spacecraft development is by Lockheed Martin Space Systems, Denver.

2008-01-01

420

Targeting the Brain  

Microsoft Academic Search

Drug delivery to the brain has remained one of the most vexing problems in translational neuroscience research. This review\\u000a rationalizes the strategies to target drugs to the brain. Factors such as the speed of intervention, the scale of intervention,\\u000a the state of BBB, and the permissible risks, will all be critical in deciding how best to deliver drugs to a

Shailendra Joshi; Eugene Ornstein; Jeffrey N. Bruce

2007-01-01

421

Targeting the Sumoylation Pathway  

Microsoft Academic Search

Sumoylation is a dynamic process where SUMO (small ubiquitin-like modifier) is covalently conjugated and deconjugated from\\u000a target proteins to regulate their cellular localization, stability, and function. Analogous to the ubiquitination pathway,\\u000a sumoylation involves E1, E2, and E3 enzymes that are engaged in the processing, ligation, and recycling of SUMO. Sumoylation\\u000a influences proteins involved in various cellular processes including stress and

Pooja Pungaliya; Eric Rubin

422

FOS Target Acquisition Test  

NASA Astrophysics Data System (ADS)

FOS onboard target acquisition software capabilities will be verified by this test -- point source binary, point source firmware, point source peak-up, wfpc2 assisted realtime, point source peak-down, taled assisted binary, taled assisted firmware, and nth star binary modes. The primary modes are tested 3 times to determine repeatability. This test is the only test that will verify mode-to-mode acquisition offsets. This test has to be conducted for both the RED and BLUE detectors.

Koratkar, Anuradha

1994-07-01

423

Target Zones and Realignments  

Microsoft Academic Search

Recent contributions emphasize that the presence of exchange-rate target zones has important effects on the within-band behavior of exchange rates when agents are forward-looking. The authors find that the implications of available models are inconsistent with European exchange-rate data, and they suggest that the frequent realignments occurring in the period they consider may be responsible for this. They construct a

Giuseppe Bertola; Ricardo J Caballero

1992-01-01

424

Targeting Inactive Enzyme Conformation  

PubMed Central

There has been considerable interest in protein tyrosine phosphatase 1B (PTP1B) as a therapeutic target for diabetes, obesity, as well as cancer. Identifying inhibitory compounds with good bioavailability is a major challenge of drug discovery programs targeted toward PTPs. Most current PTP active site-directed pharmacophores are negatively charged pTyr mimetics which cannot readily enter the cell. This lack of cell permeability limits the utility of such compounds in signaling studies and further therapeutic development. We identify aryl diketoacids as novel pTyr surrogates and show that neutral amide-linked aryl diketoacid dimers also exhibit excellent PTP inhibitory activity. Kinetic studies establish that these aryl diketoacid derivatives act as noncompetitive inhibitors of PTP1B. Crystal structures of ligand-bound PTP1B reveal that both the aryl diketoacid and its dimeric derivative bind PTP1B at the active site, albeit with distinct modes of interaction, in the catalytically inactive, WPD loop open conformation. Furthermore, dimeric aryl diketoacids are cell permeable and enhance insulin signaling in hepatoma cells, suggesting that targeting the inactive conformation may provide a unique opportunity for creating active site-directed PTP1B inhibitors with improved pharmacological properties.

Liu, Sijiu; Zeng, Li-Fan; Wu, Li; Yu, Xiao; Xue, Ting; Gunawan, Andrea M.; Ya-Qiu, Long; Zhang, Zhong-Yin

2009-01-01

425

Interferon ?-targeted therapy.  

PubMed

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by the production of autoantibodies to various cellular components. Although many of therapies have shown great efficacy, they often associate with adverse effects. The development of safer therapies for SLE has led to recent emphasis on targeting selected pathways that can be important in the inflammatory process in SLE. The cytokine family of type I interferons (IFNs), and especially the IFN? subtypes, are implicated in pathogenesis of SLE. Genetic polymorphisms of several components of the IFN signaling pathway have been associated with an increased risk of SLE. Therefore, IFN? subtypes have been identified as a potential target for drug development in SLE. There have been developed three agents, IFN?-targeted therapy, Sifalimumab, Rontalizumab and NNC 0152-0000-0001. They are anti-IFN? monoclonal antibodies that bind to and specifically neutralizes most IFN? subtypes, preventing signaling through the type I IFN receptor. The safety and dose-proportional pharmacokinetics of those agents were demonstrated. A larger study is currently ongoing, further safety profile will be evaluated. This review provides an update on the ongoing clinical trials of anti-IFN? therapy and the promise and obstacles in the use of biologics in SLE. PMID:23994795

Hanaoka, Hironari; Takeuchi, Tsutomu

2013-01-01

426

Apparatus for forming targets  

DOEpatents

Apparatus and method for cryoinduced uniform deposition of cryogenic materials, such as deuterium-tritium (DT) mixtures, on the inner surface of hollow spherical members, such as inertially imploded targets. By vaporizing and quickly refreezing cryogenic materials contained within a hollow spherical member, a uniform layer of the materials is formed on the inner surface of the spherical member. Heating of the cryogenic material, located within a non-isothermal compact freezing cell, is accomplished by an electrical heat pulse, whereafter the material is quickly frozen forming a uniform layer on the inner surface of the spherical member. The method is not restricted to producing a frozen layer on only the inner surface of the innermost hollow member, but where multiple concentric hollow spheres are involved, such as in multiple shell targets for lasers, electron beams, etc., layers of cryogenic material may also be formed on the inner surface of intermediate or outer spherical members, thus providing the capability of forming targets having multiple concentric layers or shells of frozen DT.

Woerner, Robert L. (Livermore, CA)

1980-01-01

427

Novel diuretic targets.  

PubMed

As the molecular revolution continues to inform a deeper understanding of disease mechanisms and pathways, there exist unprecedented opportunities for translating discoveries at the bench into novel therapies for improving human health. Despite the availability of several different classes of antihypertensive medications, only about half of the 67 million Americans with hypertension manage their blood pressure appropriately. A broader selection of structurally diverse antihypertensive drugs acting through different mechanisms would provide clinicians with greater flexibility in developing effective treatment regimens for an increasingly diverse and aging patient population. An emerging body of physiological, genetic, and pharmacological evidence has implicated several renal ion-transport proteins, or regulators thereof, as novel, yet clinically unexploited, diuretic targets. These include the renal outer medullary potassium channel, ROMK (Kir1.1), Kir4.1/5.1 potassium channels, ClC-Ka/b chloride channels, UTA/B urea transporters, the chloride/bicarbonate exchanger pendrin, and the STE20/SPS1-related proline/alanine-rich kinase (SPAK). The molecular pharmacology of these putative targets is poorly developed or lacking altogether; however, recent efforts by a few academic and pharmaceutical laboratories have begun to lessen this critical barrier. Here, we review the evidence in support of the aforementioned proteins as novel diuretic targets and highlight examples where progress toward developing small-molecule pharmacology has been made. PMID:23863472

Denton, Jerod S; Pao, Alan C; Maduke, Merritt

2013-10-01

428

CDTI target selection criteria  

NASA Technical Reports Server (NTRS)

A Cockpit Display of Traffic Information (CDTI) is a cockpit instrument which provides information to the aircrew on the relative location of aircraft traffic in the vicinity of their aircraft (township). In addition, the CDTI may provide information to assist in navigation and in aircraft control. It is usually anticipated that the CDTI will be integrated with a horizontal situation indicator used for navigational purposes and/or with a weather radar display. In this study, several sets of aircraft traffic data are analyzed to determine statistics on the number of targets that will be displayed on a CDTI using various target selection criteria. Traffic data were obtained from an Atlanta Terminal Area Simulation and from radar tapes recorded at the Atlanta and Miami terminal areas. Results are given in the form of plots showing the average percentage of time (or probability) that an aircraft equipped with a CDTI would observe from 0 to 10 other aircraft on the display for range settings on the CDTI up to 30 n. mi. and using various target discrimination techniques.

Britt, C. L.; Davis, C. M.; Jackson, C. B.; Mcclellan, V. A.

1984-01-01

429

Nanocrystal targeting in vivo  

NASA Astrophysics Data System (ADS)

Inorganic nanostructures that interface with biological systems have recently attracted widespread interest in biology and medicine. Nanoparticles are thought to have potential as novel intravascular probes for both diagnostic (e.g., imaging) and therapeutic purposes (e.g., drug delivery). Critical issues for successful nanoparticle delivery include the ability to target specific tissues and cell types and escape from the biological particulate filter known as the reticuloendothelial system. We set out to explore the feasibility of in vivo targeting by using semiconductor quantum dots (qdots). Qdots are small (<10 nm) inorganic nanocrystals that possess unique luminescent properties; their fluorescence emission is stable and tuned by varying the particle size or composition. We show that ZnS-capped CdSe qdots coated with a lung-targeting peptide accumulate in the lungs of mice after i.v. injection, whereas two other peptides specifically direct qdots to blood vessels or lymphatic vessels in tumors. We also show that adding polyethylene glycol to the qdot coating prevents nonselective accumulation of qdots in reticuloendothelial tissues. These results encourage the construction of more complex nanostructures with capabilities such as disease sensing and drug delivery.

Åkerman, Maria E.; Chan, Warren C. W.; Laakkonen, Pirjo; Bhatia, Sangeeta N.; Ruoslahti, Erkki

2002-10-01

430

Targeted therapy in gastrointestinal malignancies  

PubMed Central

Increased understanding of cancer pathogenesis has identified several pathways that serve as potential targets for novel targeted agents in development. The selection of targeted cancer therapy based on biomarkers has instigated a new era of personalized medicine and changed the way we practice oncology. Many targeted agents are approved for treatment of gastrointestinal malignancies most targeting tumor angiogenesis, and many more are in different phases of development. Here we briefly summarize nine different targeted agents that are approved currently in the U.S. and several other agents currently being studied in various gastrointestinal cancers.

Chhatrala, Ravi; Thanavala, Yasmin; Iyer, Renuka

2014-01-01

431

Target Asteroids! Observing Targets for 2014 April through June  

NASA Astrophysics Data System (ADS)

Asteroids to be observed by the Target Asteroids! program during the period of 2014 April through June are presented. In addition to asteroids on the original Target Asteroids! list of easily accessible spacecraft targets, an effort has been made to identify other asteroids that are 1) brighter and easier to observe for small telescope users and 2) analogous to 101955 Bennu, the target asteroid of the OSIRIS-REx sample return mission.

Hergenrother, Carl; Hill, Dolores

2014-04-01

432

A target-based color space for sea target detection  

Microsoft Academic Search

Sea target detection is a vital application for military and navigation purposes. A new supervised clustering method based\\u000a on the combination of the PSO and FCM techniques is presented for the sea target detection problem. The color components of\\u000a the target and non-target pixels in the RGB color space are used as features to train the classification algorithm. The new

Saeed Mirghasemi; Mojtaba Lotfizad

433

Target Asteroids! Observing Targets for 2014 April through June  

NASA Astrophysics Data System (ADS)

Asteroids to be observed by the Target Asteroids! program during the period of July to September 2014 are presented. In addition to asteroids on the original Target Asteroids! list of easily accessible spacecraft targets, an effort has been made to identify other asteroids that are 1) brighter and easier to observe for small telescope users and 2) analogous to (101955) Bennu, the target asteroid of the OSIRIS-REx sample return mission.

Hergenrother, Carl; Hill, Dolores

2014-07-01

434

Target Asteroids! Observing Targets for 2014 January through March  

NASA Astrophysics Data System (ADS)

Asteroids to be observed for the Target Asteroids! program during the period of 2014 January to March are presented. In addition to asteroids on the original Target Asteroids! list of easily accessible spacecraft targets, an effort has been made to identify other asteroids that are 1) brighter and easier to observe for small telescope users and 2) analogous to (101955) Bennu, the target asteroid of the OSIRIS-REx sample return mission.

Hergenrother, Carl; Hill, Dolores

2014-01-01

435

Scanner analyzer target  

NASA Astrophysics Data System (ADS)

This paper describes a black and white photographic chart called the 'Scanner Analyzer Target' developed as a practical aid toward analyzing scanned image quality produced by binary digital document scanners used as input devices in document image processing systems. The target is used by factory-line personnel as an 'adjustment-set' tool, by the quality control department as an image quality judgement tool, and by customers as an incoming inspection tool. The scanner uses a rotary drum paper transport to move the document in front of a fixed scan line. The document is illuminated by a pair of fluorescent lamps and reflected through a reduction type camera to a CCD image sensor. Scanning speeds range from 7 in./s to 12 in./s and scanning resolutions range from 200 to 300 dpi. The scanner control panel allows image quality adjustments by using 9 levels of darkness and 10 levels of sensitivity. The darkness setting controls the level of optical reflectance at which the scanner shifts its output from white to black. At low darkness setting a scanned image appears lighter than the original and at high darkness setting the resulting image is dark. The sensitivity setting controls the amount of adaptive thresholding included in the binary decision. Low sensitivity setting is adequate for high contrast originals; high sensitivity setting allows the scanner to detect low contrast information contained in different background shades (or colors). At maximum sensitivity, the scanner is extremely sensitive to high frequency (text) and tends to drop out the background information. The purpose of the target is to give the user the ability to measure a number of scanned image parameters: text legibility, resolution, focus, darkness range, shading distortion, sensitivity range, photograph dithering, vertical and horizontal pixel count, document or CCD skew, vertical resolution linearity, aspect ratio, video noise and page registration.

Simonis, Roland

1991-02-01

436

Right on Target  

NASA Technical Reports Server (NTRS)

This map shows the estimated location of the Mars Exploration Rover Spirit within Gusev Crater, Mars. Engineers targeted Spirit for the center of the blue ellipse. Measurements taken during the rover's descent by the Deep Space Network predicted its landing site to be the spot marked with a black dot. Later measurements taken on the ground by both the Deep Space Network and the orbiter Mars Odyssey narrowed the predicted landing site to a spot marked with a white dot. When initially choosing a landing site for the rover, engineers avoided hazardous terrain outlined here in yellow and red. This map consists of data from Mars Odyssey and Mars Global Surveyor.

2004-01-01

437

Advances in Targeted Therapies Tutorial  

Cancer.gov

A tutorial for health professionals that gives an overview of targeted therapies, an emerging approach to cancer treatment. Includes examples of the main types of targeted therapies and describes their mechanisms of action.

438

Targeting Organizations: Centralized or Decentralized.  

National Technical Information Service (NTIS)

This study analyzes the evolution of targeting organizations to identify their strengths and evaluate their impact on future organizations. While both strategic and tactical targeting are discussed, the primary emphasis is on the former. On the eve of its...

E. B. Schmidt

1993-01-01

439

Set Classification of Military Targets.  

National Technical Information Service (NTIS)

The present study shows by example the potential amount of information available in a set of observations of targets where there are known relations between these targets. Known relations between objects significantly reduces the set of possible explanati...

R. Korsnes B. J. Hansen

2004-01-01

440

Multisensor Target Detection and Classification.  

National Technical Information Service (NTIS)

In this thesis a new approach to the detection and classification of tactical targets using a multifunction laser radar sensor is developed. Targets of interest were tanks, jeeps, trucks, and other vehicles. Doppler images were segmented by developing a n...

D. W. Ruck

1987-01-01

441

A Note on Inflation Targeting.  

ERIC Educational Resources Information Center

Presents a pedagogical graphical exposition to illustrate the stabilizing effect of price target zones. Finds that authorities' commitment to defend a price target zone affects the public's inflation expectations and, in turn, reduces actual inflation. (RLH)

Lai, Ching-chong; Chang, Juin-jen

2001-01-01

442

Targeted therapy for sarcomas.  

PubMed

Sarcomas are tumors of mesenchymal origin that make up approximately 1% of human cancers. They may arise as primary tumors in either bone or soft tissue, with approximately 11,280 soft tissue tumors and 2,650 bone tumors diagnosed each year in the United States. There are at least 50 different subtypes of soft tissue sarcoma, with new ones described with ever-increasing frequency. One way to look at sarcomas is to divide them into categories on the basis of their genetic make-up. One group of sarcomas has an identifiable, relatively simple genetic signature, such as the X:18 translocation seen in synovial sarcoma or the 11:22 translocation seen in Ewing's sarcoma. These specific abnormalities often lead to the presence of fusion proteins, such as EWS-FLI1 in Ewing's sarcoma, which are helpful as diagnostic tools and may become therapeutic targets in the future. Another group of sarcomas is characterized by complex genetic abnormalities as seen in leiomyosarcoma, osteosarcoma, and undifferentiated sarcoma. It is important to keep these distinctions in mind when contemplating the development of targeted agents for sarcomas. Different abnormalities in sarcoma could be divided by tumor subtype or by the molecular or pathway abnormality. However, some existing drugs or drugs in development may interfere with or alter more than one of the presented pathways. PMID:24669185

Forscher, Charles; Mita, Monica; Figlin, Robert

2014-01-01

443

Mitochondria as pharmacological targets  

PubMed Central

In the last decade, mitochondria have provided a vast area of research for the pharmacologist, with a wealth of potential targets for drug action. Correct target identification and subsequent pharmacological manipulation might greatly help in the prevention and/or treatment of a number of the most prevalent diseases of our time including cancer, neurodegenerative disease and myocardial infarction. This is a commentary to accompany the publication of three papers in this issue of the BJP by Kurz et al., Pravdic et al. and Puerta et al. on different aspects of pharmacology involving mitochondria. This article is a commentary on Pravdic et al., pp. 220–232, Puerta et al., pp. 233–245 and Kurz et al. pp. 246–257 of this issue. To view these papers visit http://dx.doi.org/10.1111/j.1476-5381.2010.00698.xhttp://dx.doi.org/10.1111/j.1476-5381.2010.00663.x and http://dx.doi.org/10.1111/j.1476-5381.2010.00656.x

Moncada, S

2010-01-01

444

Targeting adipose tissue  

PubMed Central

Two different types of adipose tissues can be found in humans enabling them to respond to starvation and cold: white adipose tissue (WAT) is generally known and stores excess energy in the form of triacylglycerol (TG), insulates against cold, and serves as a mechanical cushion. Brown adipose tissue (BAT) helps newborns to cope with cold. BAT has the capacity to uncouple the mitochondrial respiratory chain, thereby generating heat rather than adenosine triphosphate (ATP). The previously widely held view was that BAT disappears rapidly after birth and is no longer present in adult humans. Using positron emission tomography (PET), however, it was recently shown that metabolically active BAT occurs in defined regions and scattered in WAT of the adult and possibly has an influence on whole-body energy homeostasis. In obese individuals adipose tissue is at the center of metabolic syndrome. Targeting of WAT by thiazolidinediones (TZDs), activators of peroxisome proliferator-activated receptor ? (PPAR?) a ‘master’ regulator of fat cell biology, is a current therapy for the treatment of type 2 diabetes. Since its unique capacity to increase energy consumption of the body and to dissipate surplus energy as heat, BAT offers new perspectives as a therapeutic target for the treatment of obesity and associated diseases such as type 2 diabetes and metabolic syndrome. Recent discoveries of new signaling pathways of BAT development give rise to new therapeutic possibilities in order to influence BAT content and activity.

2012-01-01

445

Magnetized Target Fusion  

NASA Technical Reports Server (NTRS)

Magnetized target fusion (MTF) is under consideration as a means of building a low mass, high specific impulse, and high thrust propulsion system for interplanetary travel. This unique combination is the result of the generation of a high temperature plasma by the nuclear fusion process. This plasma can then be deflected by magnetic fields to provide thrust. Fusion is initiated by a small traction of the energy generated in the magnetic coils due to the plasma's compression of the magnetic field. The power gain from a fusion reaction is such that inefficiencies due to thermal neutrons and coil losses can be overcome. Since the fusion reaction products are directly used for propulsion and the power to initiate the reaction is directly obtained from the thrust generation, no massive power supply for energy conversion is required. The result should be a low engine mass, high specific impulse and high thrust system. The key is to successfully initiate fusion as a proof-of-principle for this application. Currently MSFC is implementing MTF proof-of-principle experiments. This involves many technical details and ancillary investigations. Of these, selected pertinent issues include the properties, orientation and timing of the plasma guns and the convergence and interface development of the "pusher" plasma. Computer simulations of the target plasma's behavior under compression and the convergence and mixing of the gun plasma are under investigation. This work is to focus on the gun characterization and development as it relates to plasma initiation and repeatability.

Griffin, Steven T.

2002-01-01

446

Preparation of sputtering targets  

US Patent & Trademark Office Database

Method for preparing sputtering targets according to one embodiment, by reacting a mixture of alkoxides of metals such as titanium, lanthanum and zirconium, with a soluble lead compound such as lead acetate in an organic solvent in the presence of aqueous ammonium hydroxide, to form a sol-gel. The solvent is removed from the sol-gel to form a powder. The powder is mixed with a liquid medium, e.g. an aqueous medium containing an organosol, to form a slurry of the powder and the slurry is cast on a substrate such as a polyester tape and dried to form a tape of the sol-gel. The tape is cut into individual tapes and the tapes are stacked and laminated under heat and pressure into a monolithic unit, which is then filed at high temperature. The dense monolithic unit thus formed is mechanically machined to the desired shape, e.g. in the form of a circular disc, to produce a sputtering target.

1998-01-20

447

Tankyrases as drug targets.  

PubMed

Tankyrase 1 and tankyrase 2 are poly(ADP-ribosyl)ases that are distinguishable from other members of the enzyme family by the structural features of the catalytic domain, and the presence of a sterile ?-motif multimerization domain and an ankyrin repeat protein-interaction domain. Tankyrases are implicated in a multitude of cellular functions, including telomere homeostasis, mitotic spindle formation, vesicle transport linked to glucose metabolism, Wnt-?-catenin signaling, and viral replication. In these processes, tankyrases interact with target proteins, catalyze poly(ADP-ribosyl)ation, and regulate protein interactions and stability. The proposed roles of tankyrases in disease-relevant cellular processes have made them attractive drug targets. Recently, several inhibitors have been identified. The selectivity and potency of these small molecules can be rationalized by how they fit within the NAD(+)-binding groove of the catalytic domain. Some molecules bind to the nicotinamide subsite, such as generic diphtheria toxin-like ADP-ribosyltransferase inhibitors, whereas others bind to a distinct adenosine subsite that diverges from other diphtheria toxin-like ADP-ribosyltransferases and confers specificity. A highly potent dual-site inhibitor is also available. Within the last few years, tankyrase inhibitors have proved to be useful chemical probes and potential lead compounds, especially for specific cancers. PMID:23648170

Lehtiö, Lari; Chi, Nai-Wen; Krauss, Stefan

2013-08-01

448

Liquid Hydrogen: Target, Detector  

NASA Astrophysics Data System (ADS)

In 1952 D. Glaser demonstrated that a radioactive source's radiation could boil 135°C superheated-diethyl ether in a 3-mm Ø glass vessel and recorded bubble track growth on high-speed film in a 2-cm3 chamber. This Bubble Chamber (BC) promised improved particle track time and spatial resolution and cycling rate. Hildebrand and Nagle, U of Chicago, reported Liquid Hydrogen minimum ionizing particle boiling in August 1953. John Wood created the 3.7-cm Ø Liquid Hydrogen BC at LBL in January 1954. By 1959 the Lawrence Berkley Laboratory (LBL) Alvarez group's ``72-inch'' BC had tracks in liquid hydrogen. Within 10 years bubble chamber volumes increased by a factor of a million and spread to every laboratory with a substantial high-energy physics program. The BC, particle accelerators and special separated particle beams created a new era of High Energy Physics (HEP) experimentation. The BC became the largest most complex cryogenic installation at the world's HEP laboratories for decades. The invention and worldwide development, deployment and characteristics of these cryogenic dynamic target/detectors and related hydrogen targets are described.

Mulholland, G. T.; Harigel, G. G.

2004-06-01

449

Extrapolating target tracks  

NASA Astrophysics Data System (ADS)

Steady-state performance of a tracking filter is traditionally evaluated immediately after a track update. However, there is commonly a further delay (e.g., processing and communications latency) before the tracks can actually be used. We analyze the accuracy of extrapolated target tracks for four tracking filters: Kalman filter with the Singer maneuver model and worst-case correlation time, with piecewise constant white acceleration, and with continuous white acceleration, and the reduced state filter proposed by Mookerjee and Reifler.1, 2 Performance evaluation of a tracking filter is significantly simplified by appropriate normalization. For the Kalman filter with the Singer maneuver model, the steady-state RMS error immediately after an update depends on only two dimensionless parameters.3 By assuming a worst case value of target acceleration correlation time, we reduce this to a single parameter without significantly changing the filter performance (within a few percent for air tracking).4 With this simplification, we find for all four filters that the RMS errors for the extrapolated state are functions of only two dimensionless parameters. We provide simple analytic approximations in each case.

Van Zandt, James R.

2012-05-01

450

Target estimation algorithm design using quantity data and target feature  

NASA Astrophysics Data System (ADS)

The estimation algorithm plays an important role in a radar tracking system. An improved estimation approach using both quantity data and target feature is investigated in this article. The advantage of this approach is that the system will have better estimation based on more target information. A data association denoted one-step conditional maximum likelihood algorithm is applied to match between radar measurements and existing target tracks. Moreover, an adaptive estimator is applied to combine the quantity data and target feature for estimation problems. According to the simulation results, this approach can enhance the performance of multiple-target tracking systems.

Lu, Chung-Lain; Lin, Chih-Min

2011-12-01

451

Open-Economy Inflation Targeting  

Microsoft Academic Search

The paper examines inflation targeting in a small open economy with forward-looking aggregate supply and demand with microfoundations, and with stylised realistic lags in the different monetary-policy transmission channels. The paper compares strict and flexible targeting of CPI and domestic inflation and inflation-targeting reaction functions and the Taylor rule. Flexible CPI-inflation targeting does not only limit the variability of CPI

Lars E. O. Svensson

1998-01-01

452

Open-economy inflation targeting  

Microsoft Academic Search

The paper examines inflation targeting in a small open economy with forward-looking aggregate supply and demand with microfoundations, and with stylized realistic lags in the different monetary-policy transmission channels. The paper compares strict and flexible targeting of CPI and domestic inflation, and inflation-targeting reaction functions and the Taylor rule. Flexible CPI-inflation targeting does not limit the variability of CPI inflation

Lars E. O. Svensson

2000-01-01

453

Revised target information for ICF reactor studies.  

National Technical Information Service (NTIS)

This report discusses the following topics: summary of target gain and driver requirements for short wavelength laser driven indirect drive targets; direct drive laser targets; heavy ion target gain; and target costing and output. 5 figs. (LSP)

R. Bangerter

1991-01-01

454

Projectile penetration into representative targets  

Microsoft Academic Search

The differential equation representing the penetration of a 'hard' projectile into semi-infinite, homogeneous target materials is solved for several generic combinations of the target material\\/projectile characteristics. A 'hard' projectile is defined as one that does not change size or shape and does not lose mass during the penetration process. The target materials evaluated range from the structurally 'soft' materials (liquids)

George W. Stone

1994-01-01

455

Policy Rules for Inflation Targeting  

Microsoft Academic Search

Policy rules that are consistent with inflation targeting are examined in a small macroeconomic model of the US economy. We compare the properties and outcomes of explicit instrument rules' as well as targeting rules.' The latter, which imply implicit instrument rules, may be closer to actual operating procedures of inflation-targeting central banks. We find that inflation forecasts are central for

Glenn D. Rudebusch; Lars E. O. Svensson

1998-01-01

456

Split-target neutronics and the MLNSC spallation target system  

SciTech Connect

The Manuel Lujan, Jr., Neutron Scattering Center (MLNSC) at the Los Alamos National Laboratory is one of four operating Short-Pulse Spallation Sources worldwide. The MLNSC target system (composed of targets, moderators, and reflectors) was first installed in 1985. The target system employs a split tungsten spallation target with a void space in between (the flux-trap gap); this target system will be upgraded in 1998. The ability to efficiently split a spallation target allowed us to introduce the concept of flux-trap moderators and ultimately the notion of backscattering and upstream moderators. The upgraded MLNSC target system will employ both flux-trap and upstream/backscattering moderators to simultaneously service 16 neutron flight paths with high-intensity neutron beams for materials science research.

Russell, G.J.; Ferguson, P.D.; Pitcher, E.J.; Court, J.D.

1996-12-31

457

Electromagnetic targeting of guns  

SciTech Connect

This is the final report of a one-year, Laboratory-Directed Research and Development (LDRD) project at the Los Alamos National Laboratory (LANL). Electromagnetic pulse (EMP) signals produced from explosives being fired have been reported in the literature for fifty years. When a gun is fired it produces an EMP muzzle blast signal. The strength and nature of these signals was first analyzed in the early 1970s, while the results were interesting, no follow-up studies were conducted. With modern detection and signal processing technology, we believe that these signals could be used to instantaneously locate guns of virtually all calibers as they fire. The objective of our one-year project was to establish the basic nature of these signals and their utility in the concept of electromagnetic targeting of guns.

Pogue, E.W.; Boat, R.M.; Holden, D.N.; Lopez, J.R. [and others

1996-10-01

458

Targeting Histone Demethylases  

PubMed Central

In addition to genetic disorders, epigenetic alterations have been shown to be involved in cancer, through misregulation of histone modifications. Miswriting, misreading, and mis-erasing of histone acetylation as well as methylation marks can be actually associated with oncogenesis and tumor proliferation. Historically, methylation of Arg and Lys residues has been considered a stable, irreversible process due to the slow turnover of methyl groups in chromatin. The discovery in recent years of a large number of histone Lys demethylases (KDMs, belonging to either the amino oxidase or the JmjC family) totally changed this point of view and suggested a new role for dynamic histone methylation in biological processes. Since overexpression, alteration, or mutation of a number of KDMs has been found in many types of cancers, such enzymes could represent diagnostic tools as well as epigenetic targets to modulate for obtaining novel therapeutic weapons against cancer. The first little steps in this direction are described here.

Rotili, Dante; Mai, Antonello

2011-01-01

459

Novel Therapies Targeting Endometriosis  

PubMed Central

Endometriosis is an often painful disorder in which the endometrial glands and stroma grow outside the uterus. The disease affects women’s quality of life and is a common cause of infertility. In this review, we describe promising new developments in the field based on in vitro assays and rodent models, each of which has the potential to be beneficial in the treatment of this disease. We will specifically describe the role of anti-inflammatory drugs, selective estrogen, or progesterone modulators, statins, antiangiogenic agents, and the potential for targeting stem cells as likely methods to hone in and eliminate endometriosis. The most promising of these potential therapies are currently slated for further testing in both rodent and nonhuman primate trials.

Osteen, Kevin G.; Bruner-Tran, Kaylon L.; Lockwood, Charles J.; Krikun, Graciela; Sokalska, Anna; Duleba, Antoni J.

2011-01-01

460

Stroke neuroprotection: targeting mitochondria.  

PubMed

Stroke is the fourth leading cause of death and the leading cause of long-term disability in the United States. Blood flow deficit results in an expanding infarct core with a time-sensitive peri-infarct penumbra that is considered salvageable and is the primary target for treatment strategies. The only current FDA-approved drug for treating ischemic stroke is recombinant tissue plasminogen activator (rt-PA). However, this treatment is limited to within 4.5 h of stroke onset in a small subset of patients. The goal of this review is to focus on mitochondrial-dependent therapeutic agents that could provide neuroprotection following stroke. Dysfunctional mitochondria are linked to neurodegeneration in many disease processes including stroke. The mechanisms reviewed include: (1) increasing ATP production by purinergic receptor stimulation, (2) decreasing the production of ROS by superoxide dismutase, or (3) increasing antioxidant defenses by methylene blue, and their benefits in providing neuroprotection following a stroke. PMID