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Question: What are the PD-1+ CD4+ T cells percentages and serum interferon gamma (IFN-γ) levels of pediatric patients with immune thrombocytopenia (ITP)? Finding: Compared with healthy controls, the PD-1+ CD4+ T cells percentages and IFN-γ levels were significantly higher in ITP patients before and 1 month after therapy. Meaning: Our findings suggest that PD-1+ CD4+ T cells and IFN-γ are involved in the pathophysiological process of ITP. |
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IgA vasculitis, formerly known as Henoch-Schönlein purpura, is a systemic IgA-mediated vasculitis of the small vessels commonly seen in children. The natural history of IgA vasculitis is generally self-limiting; however, one-third of patients experience symptom recurrence and a refractory course. This systematic review examined the use of dapsone in refractory IgA vasculitis cases. A literature search of PubMed databases retrieved... |
The purpose of this study was to determine the frequency of CD4+CD25+FoxP3+ regulatory T cells (Treg) in the peripheral blood of patients with childhood chronic immune thrombocytopenic purpura (ITP) exhibiting thrombocytopenia and spontaneous remission. The findings of this study indicate the possibility of predicting spontaneous recovery and pathogenesis of childhood chronic ITP. Eleven children with chronic ITP (seven thrombocytopenic and four... |
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy characterized by endothelial cell damage, resulting in microangiopathic hemolytic anemia, thrombocytopenia, and various degrees of neurological and renal impairment caused by microvascular thrombi. It is rare in children and frequently follows a fatal course. TTP is divided into 2 types: one is inherited and associated with ADAMTS-13 gene mutations and the other... |
Purpose : An association between idiopathic thrombocytopenic purpura (ITP) and systemic lupus erythematosus (SLE) has been recognized for decades because thrombocytopenia is the first manifestation in some patients with SLE. However, the risk of later development of SLE in childhood ITP is currently unknown. We retrospectively evaluated the incidence and clinical significance of the positive antinuclear antibody (ANA) in children... |
Purpose : Idiopathic thrombocytopenic purpura (ITP) is a relatively common hematological disease in children. It generally occurs after exposure to a common viral infection episode; however, it may occasionally follow immunization with measles, measles-mumps-rubella (MMR), hepatitis B (HBV), influenza, diphtheria tetanus-pertussis (DTP), or chickenpox vaccines. In this study, the incidence, clinical characteristics, and treatment outcome of vaccination-associated ITP were investigated... |
Purpose : Several cytokines play important roles in the inflammatory process of Henoch-Sch lein Purpura (HSP). It is likely that transforming growth factor-β (TGF-β) is involved in the pathogenesis of HSP. The purpose of this study is to investigate whether TGF-β promoter polymorphism is associated with the renal involvement of childhood HSP. Methods : Thirty-four patients younger than 15 years, who... |
Purpose : The aim of this study was to investigate the incidence and course of neutropenia following intravenous immunoglobulin (IVIG) therapy in children with idiopathic thrombocytopenic purpura (ITP). Methods : From January 2001 to June 2006, fifty-four patients with ITP were enrolled in this study. Forty-two of 54 patients were treated with IVIG, while the other 12 were treated with... |
Purpose : Intravenous immunoglobulin (IVIG) is effective for the treatment of idiopathic thrombocytopenic purpura (ITP) in children. Recently, several reports have been published that show its impact on the absolute neutrophil count. The present study was performed to confirm these findings. Methods : Data on 26 ITP patients were analyzed. Patients with febrile illness or increased C-reactive protein levels... |
The hemolytic uremic syndrome (HUS) is a rare disease of microangiopathic hemolytic anemia, low platelet count and renal impairment. HUS usually occurs in young children after hemorrhagic colitis by shigatoxin-producing enterohemorrhagic E. coli (D+HUS). HUS is the most common cause of acute renal failure in infants and young children, and is a substantial cause of acute mortality and morbidity; however,... |
Acute hemorrhagic edema of infancy (AHEI) is an uncommon form of cutaneous leukocytoclastic vasculitis that occurs in infants and children younger than 2 years. AHEI is characterized clinically by marked peripheral edema and fever as well as large palpable purpuric and ecchymotic skin lesions in a target-like pattern, mainly on the face, ears and extremities, similar to the skin findings... |
Giant platelet syndrome is a group of unique disorders characterized by the presence of abnormally large platelets, and usually accompanied by thrombocytopenia. Most cases of giant platelets are encountered in idiopathic thrombocytopenic purpura(ITP). In contrast, inherited giant platelet disorders, a group of heterogeneous diseases, are rare. Bernard-Soulier syndrome and its variants, and MYH9 related diseases have been defined at the... |
Purpose : Henoch-Sch nlein purpura(HSP) is the most common and benign systemic vasculitis in children. Few reports have focused on worse outcomes of HSP in adults. The age of onset is suggested as a main risk factor. We assessed the characteristics of adolescent-onset HSP. Methods : We retrospectively analyzed 205 cases presented from Aug. 1993 to Oct. 2003. Patients were classified... |
Henoch-Sch nlein purpura(HSP) is an IgA mediated immune complex vasculitic disease characterized by non-thrombocytic purpura, arthritis, gastrointestinal manifestations, and glomerulonephritis. HSP related glomerulonephritis induces hypoproteinemia and edema in some cases. Protein-losing enteropathy is another rare but known manifestation of HSP leading to hypoproteinemia and edema. We report a 6-year-old girl with HSP who showed edema caused by intestinal protein loss,... |
Purpose : We evaluated children with Henoch-Sch nlein purpura(HSP) in terms of epidemiology and clinical characteristics. Methods : A total of 424 medical records of children with HSP admitted to The Catholic University of Korea, Daejeon St. Mary's Hospital, from 1987 to 2003 were retrospectively analyzed. Results : The mean annual number of cases was 25.1?.9 and no one year showed an... |
Henoch-Schönlein purpura (HSP) is a systemic disorder characterized by leukocytoclastic vasculitis that can affect multiple organs predominantly the skin, joints, gastrointestinal tract and kidney. Although the specific pathogenesis of HSP is not known, there are several hypotheses. Although the importance of the complement activation in glomerular injury in HSP has been suggested, the complement levels and the blood pressure in... |
Purpose : Henoch-Sch nlein purpura(HSP) is a systemic vasculitis, characterized by cutaneous palpable purpura, gastrointestinal(GI) symptoms, arthritis and renal involvement. In general, the prognosis is determined by GI complication as well as the severity of nephritis. In this study, we analyzed the statistical relationship between the GI symptom and other clinical findings for assessing the prognosis, and evaluated abdominal ultrasonographic... |
Childhood immune thrombocytopenic purpura(ITP) is a typically benign self-limiting bleeding disorder of not fully known autoimmune etiology. Chronic ITP, which is defined by duration of more than 6 months, occurs in approximately 20% of children. Neither the risk of bleeding nor the type of disease, acute or chronic, can be predicted at initial presentation. The indication of treatment, choice... |
Purpose : Henoch-Schönlein purpura(HSP) is a systemic vasculitis affecting predominantly skin, the gastrointestinal tract, the joints and kidneys. Because the scrotal manifestations including swelling and pain (or tenderness) are known to be uncommon in male HSP patients, there has been a little mention of them in the studies of HSP. Therefore, we investigated the clinical and laboratory features of HSP... |
Purpose : Kawasaki disease(KD) is an important acute febrile systemic vasculitis disease. Various cytokines have been studied to evaluate the pathogenesis of vascular injury. Some cytokines have been implicated for vascular injury, but there has been no direct evidence. We compared cytokine profiles of KD with Henoch-Sch nlein purpura(HSP), and febrile infections. Methods : We investigated the serum concentrations of... |
Henoch-Schönlein purpura is the most common vasculitis in childhood and shows unique clinical features characteristic purpura, arthritis, abdominal pain, intestinal bleeding, nephritis and so forth by IgA mediated leukocytoclastic vasculitis. The etiology of Henoch-Schönlein purpura is unknown, but many variable causative factors are reported such as infection, drugs, vaccination, food, insect bite and so on. Henoch-Schönlein purpura precipitated by trauma... |
Purpose : Idiopathic thrombocytopenic purpura(ITP) is a relatively common hematologic disease in children. The optimal strategy for treating ITP during childhood is a matter of controversy. In general, platelet count has been the primary, if not the sole measure, on which treatment decisions have been made and outcomes determined. In this study, we tried to find out the correlation between... |
Purpose : Long-term prognosis of Henoch-Sch nlein purpura(HSP) is determined by the existence and severity of renal involvement. We evaluated the relationship between various clinical features of HSP and the development of renal involvement using univariate and multivariate analyses for early detection and proper management of HSP nephritis. Methods : We performed a retrospective study of 200 children who were diagnosed... |
Purpose : Henoch-Sch nlein purpura(HSP) is a systemic vasculitis, characterized by cutaneous purpura, abdominal pain, arthralgia and renal involvement. The clinical features of HSP have been reasonably well documented but there are still many gaps in our understanding of HSP. The aim of this study was to present the clinical features of 125 children with HSP and compare them with... |
Henoch-Shonlein purpura(HSP) is a systemic small-vessel vasculitis that primarily affects the skin, gastrointestinal tract, joints, and kidneys. The nervous system may be involved, less commonly than other organs. When the central nervous system(CNS) was involved, headache, changes in mental status, seizures, and focal neurologic deficits have been reported. Hypertension, uremic encephalopathy, metabolic abnomalities, electrolyte abnormalities, or cerebral vasculitis were suggested... |
Purpose : For the early diagnosis of Henoch-Schonlein purpura(HSP) presenting with acute abdominal pain preceding skin rash. Methods : The clinical, endoscopic and radiological records of 23 cases of HSP, presenting with gastrointestinal symptoms preceding skin rash were reviewed. Results : The intervals from the onset of abdominal pain to the development of the skin rash were one day to 30 days(median... |
Purpose : This study was performed to analyze the endoscopic findings in Henoch-Schonlein purpura patients, and to compare the differences in endoscopic findings according to age and gastrointestinal symptoms. Methods : We examined children with Henoch-Schonlein purpura aged 3 to 15 years between September 1996 and October 2002. The total number studied was 65, consisting of 41 boys and 24... |
Purpose : Several methods of IV γ-globulin(IVG) infusion are effective in the treatment of autoimmune disease, including idiopathic thrombocytopenic purpura(ITP). But it is not known which method is more effective in the treatment of ITP. The effectiveness of these two methods of IVG infusion was studied in terms of platelet recovery rate, side effects and recurrence rate. Methods : Forty seven... |
Henoch-Sh nlein Purpura(HSP) is an immuologically mediated systemic vasculitis of small blood vessels affecting predominantly the skin, gastrointestinal tract, joints, and kidneys. Clinical neurological manifestations such as headaches, behavioral changes, mental changes, seizures, and visual loss are described, but neurological complication are rare during the course of HSP. We experienced a case of an 8 year-old male with HSP who... |
Purpose : Henoch-Schonlein purpura(HSP) nephritis has been reported to vary from 25 to 50% among HSP patients and is a common cause of chronic glomerulonephritis in children. In our study, we evaluated the distribution and the association of the Insertion/Deletion(I/D) polymorphism of angiotensin converting enzyme(ACE) gene with clinical manifestations, particularly proteinuria in children with HSP nephritis, compared with that in... |
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