Purpura In Children Research Articles

Dapsone for immune thrombocytopenic purpura in children and adults

Dapsone is one of the second line treatments of immune thrombocytopenic purpura (ITP). Dapsone is cheap and has response rates comparable to other second line treatment options like azathioprine, danazol, cyclophosphamide, cyclosporine, and vincristine. This retrospective analysis includes 38 patients (out of total 313 patients) of ITP treated with dapsone from 2004 to 2012. All male patients were screened for G6PD deficiency before starting dapsone. Out of 38 patients (12 children and 26 adults), one was newly diagnosed ITP, seven were persistent ITP, and 30 were chronic ITP. Five patients had side effects of dapsone; two required discontinuation due to skin rashes. The average dose of dapsone was 1.57 mg/kg/day and time to response was 57 days (19–108 days). The response was irrespective of previous treatments and response to them. The response rate was 48.6% (complete response = 40.5%). Only two adult patients had sustained response (> 6 months) after dapsone discontinuation. There were no predictors identified for dapsone response. Dapsone is a safe and cheap second-line therapy for ITP with a response rate of about 50% (majority being CR). A response to dapsone is slow, sustained, and relapses are uncommon on therapy. Dapsone withdrawal leads to relapse in most of the patients.

10.5937/mckg48-3381 = Clinical and laboratory characteristics of immune thrombocytopenic purpura in children

Objective. Description of clinical and laboratory characteristics of immune thrombocytopenic purpura in children, treatment choice and therapeutic response. Methods. Retrospective study with data from medical records of 87 patients of both sexes, aged up to 16 years, hospitalized at the Pediatric Clinic, Clinical Center, Kragujevac, from 01/10/2006 to 01/10/2012. All patients underwent a clinical examination (history, physical examination) and a laboratory examination (complete blood count, blood haemostatic tests - prothrombin time, International normalized ratio - INR, activated partial thromboplastin time - aPTT) combined with additional diagnostics (myelogram, abdominal ultrasound examination, serum immunoglobulins). Results. ITP appears with similar frequency in male and female children. The greatest incidence is at school age (55.2%). The peak of incidence is in autumn (34.5%) and winter (36.8%). The acute form of the disease appears in 90.8%, and the chronic form appears in 9.2% of patients. The majority of affected children (52.9%) had been suffering from respiratory infection in the previous three weeks. Predominant signs were petechiae (70.1%) and skin hematoma (59.8%). The greatest number of responders (66.7%) had platelet count below 20x109/l. Out of the total number of patients, 77.1% were treated only with corticosteroids. The treatment results were satisfactory. The signs of chronic ITP were developed by 8 (9.2%) patients who had not responded to therapy. Conclusion. ITP in children is usually an acute condition, equally represented by gender, in school age, in autumn and winter months, preceded by respiratory infection, manifested with the signs of hemorrhagic syndrome, particularly with petechiae and hematoma and it responds well to corticosteroids.

Genetic susceptibility of Henoch-Schönlein purpura in children

Background Henoch-Schonlein purpura (HSP) is a small vessel vasculitis typically observed in children, 3-10 years old. The aetiology is unclear. Interaction of several environmental factors, including infections and multiple genes has been proposed to play a role in pathogenesis. An increased familial occurrence is an indicator of genetic predisposition; association with a major histocompatibility complex is plausible. The aim of the study was to investigate the association of HLA-DRB1 (HLA class II antigen) with HSP.

알레르기성 자반증의 치료법에 관한 문헌고찰 -중의학 저널을 중심으로-

Objectives Allergic purpura is frequent disease in children. The purpose of this study is to analyze traditional Chinese medicine treatments of Allergic purpura in children through traditional Chinese medical journal research. Methods 22 studies were selected based on title keyword "Allergic purpura" "Child" published in CAJ (China Academic Journal). The studies were about clinical effect, herbs, injections, treatments, and nursing in Allergic purpura in children. Results & Conclusions There are so many treatments studied on purpura in children based on CAJ. A Single or combination traditional Chinese medicine treatment is effective in Allergic purpura in children. Therefore, we need to study more about Allergic purpura in children and should make more clinical cases about it.

Henoch–Schönlein purpura in children

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.

Apoptosis of human umbilical vein endothelial cells (HUVEC) induced by IgA1 isolated from Henoch-Schonlein purpura children

It had been shown that apoptosis of vascular endothelial cells played an important role in the pathogenesis of HSP. The present study was designed to investigate the apoptosis of vascular endothelial cells induced by isolated IgA1 from sera of HSP patients. HUVEC were cultured in 3 different types of media with IgA1 from HSP patients, normal healthy children and simply medium (blank control). Serum IgA1 was purified by jacalin affinity chromatography. The rates of apoptosis in HUVEC incubated with IgA1 were determined by the TUNEL method and flow cytometry, respectively. The expression of bax/bcl-2 and p53 was detected with the methods of Real-time PCR and Westernblot, respectively. The results showed that the apoptosis rate of HUVEC by IgA1 isolated from HSP patients was higher than that the normal controls (14.77±2.23% vs 9.97±1.48%) and blank controls (14.77±2.23% vs 2.25±0.77%) (P <0.01). Moreover the rate of HUVEC by IgA1 from normal healthy children was higher than the blank controls (9.97±1.48% vs 2.25±0.77%) (P <0.01). In addition, the bax and P53 expression were up-regulated and the Bcl-2 expression was down-regulated in HUVEC co-cultured with IgA1 isolated from HSP patients for 24 hours. These findings suggested that IgA1 from HSP patients could induce the apoptosis of HUVEC, which might be related to the vascular endothelial injury of HSP.

Granulomatosis With Polyangiitis Presenting as Henoch-Schönlein Purpura in Children

Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, in children is an uncommon chronic organ- and life-threatening systemic vasculitis that may share at time of initial presentation a number of clinical features in common with Henoch-Schönlein purpura (HSP), a very common and comparatively benign form of childhood vasculitis. Diagnosis of GPA requires a high index of suspicion, and antineutrophil cytoplasmic antibody tests along with tissue biopsy are helpful tools for diagnosis. We report 2 patients with GPA masqueraded as HSP at time of initial presentation. Both patients presented with nonthrombocytopenic purpura on lower extremities, in addition to abdominal pain, and/or microscopic hematuria and fulfilled both the American College of Rheumatology and the Pediatric Rheumatology European Society classification criteria for HSP. Both patients eventually developed significant renal and pulmonary disease and were diagnosed with GPA. We aim to raise awareness of such atypical presentations of GPA to avoid delayed management.

Thrombotic thrombocytopenic purpura in children

Thrombotic thrombocytopenic purpura (TTP) is a rare life-threatening disease in children, due to a severe deficiency of ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 repeats, member 13), inherited in congenital TTP or secondary to anti-ADAMTS13 antibodies in acquired TTP. Rapid techniques for ADAMTS 13 assays, long-term follow-up of patients, phenotype-genotype analysis, improved therapeutic schedules, and new therapies have emerged. Rapid techniques for ADAMTS13 assays now permit rapid confirmation of diagnosis. In congenital TTP, mutations affecting the N-terminal domains of ADAMTS13 are associated with lower residual ADAMTS13 activity and more severe phenotype. Early initiation of plasma infusion treatment and lifelong prophylactic plasma infusion have decreased mortality and sequels and prevent relapses. In acquired TTP, a disease of adolescents but also of children less than 2, adding rituximab to plasma exchange is beneficial. Recombinant ADAMTS13 ought to be soon available for congenital TTP, while acquired TTP children might benefit from its administration, alone or in association with rituximab, to avoid or limit plasma exchange duration. Progress in the understanding of TTP has boosted physicians' awareness that diagnosis and treatment are medical emergencies. New therapies hopefully will decrease treatment burden and improve prognosis.

Dexamethasone: Idiopathic Thrombocytopenic Purpura in Children and Adolescents

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 .

Association of Matrix Metalloproteinase-9 Level with the Risk of Renal Involvement for Henoch–Schönlein Purpura in Children

Matrix metalloproteinase-9 (MMP-9) is an enzyme that implicates in the pathogenesis of renal diseases. Henoch–Schönlein purpura nephritis (HSPN) is the most serious long-term complication of Henoch–Schönlein purpura, and it is one of the most common renal diseases in children. There was no any meta-analysis to explore the association of MMP-9 level with the risk of HSPN, and this meta-analysis was performed to evaluate the association between MMP-9 level and HSPN risk in children. A predefined literature search and selection of eligible relevant studies were performed to collect data from electronic databases. Eight articles were identified for the analysis of association between MMP-9 level and HSPN risk in children. In this meta-analysis, urinary MMP-9 level was associated with the risk of HSPN [odds ratio (OR) = 77.21, 95% confidence interval (CI): 54.56–99.86, p < 0.00001]. Furthermore, there was a notably difference for the association of the serum MMP-9 level with HSPN risk (OR = 135.91, 95% CI: 82.54–189.27, p < 0.00001). In conclusion, urinary/serum MMP-9 level is associated with the risk of HSPN. However, the data used for the meta-analysis in this article were from some low-level studies with small samples, and more studies should be performed in the future.

Acquired Thrombotic Thrombocytopenic Purpura in Children: A Single Institution Experience

To describe the clinical features, treatment and prognosis of acquired thrombotic thrombocytopenic purpura (TTP) in children based on a single institution experience. This study is a retrospective review of all 12 children with TTP seen at New York Medical College- Westchester Medical Center during a period of 15 y from 1993 to 2008. There were 7 females and 5 males with acquired TTP, with a median age of 13 (range, 6-17); and no cases of congenital TTP. The classic pentad of TTP (microangiopathic hemolytic anemia, thrombocytopenia, neurologic symptoms, renal dysfunction and fever) was seen in only three patients. Nine had renal involvement; eight had neurologic symptoms; and four had fever. All 12 patients had thrombocytopenia, anemia, and elevated LDH. Nine had idiopathic TTP. Three patients had one of the following underlying disorders: systemic lupus erythematosus, mixed connective tissue disorder, and aplastic anemia (post-bone marrow transplant on cyclosporine). ADAMTS13 level was decreased in 7 of 8 patients studied. Eight of 10 patients achieved remission with plasmapheresis alone. Two needed additional treatment before achieving remission. Two had one or more relapses, requiring immunosupressive treatment with vincrisine, prednisone, or rituximab. The patient with aplastic anemia died of pulmonary hypertension 5 y after bone marrow transplantation. All other 11 patients are alive and free of TTP for a median follow-up of 12 mo (range, 3-72 mo). Acquired pediatric TTP responds well to plasmapheresis. However, many patients do require additional treatment because of refractoriness to plasmapheresis or relapse. The clinical features, response to treatment, and relapse rate of pediatric TTP appear similar to those of adult TTP.

Henoch-Schönlein purpura in children: its relation to oral and to oral and oral and dental health

&gt; Background: Henoch-Schönlein purpura (HSP) is a rare systemic small vessel vasculitis, which commonly occur in childrenbetween 2 and 10 years of age. The course of the disease is often self-limiting, although may manifest long-term renal morbidity. Theseverity of renal involvement decides about the prognosis of this disease. Many factors can trigger the disease attack, which is the mostcommon is bacterial invasion. Since the oral cavity is often refer as infectious foci to other part of the body, it seemed rationally tobe part that contribute the course of disease, thus management of these infectious foci, if possible, gives rise to an astoundingly goodprognosis. Purpose: This paper will describe a review on HSP and the possible association with oral and dental health since it mightbe related to the prognosis of HSP. reviews: Rashes in children are common; they may develop a rash after prescription of antibiotics.Nevertheless there are some childhood diseases that may manifest a rash presentation, such as HSP. It is important for pediatric dentistto have knowledge about HSP and consider the possibility of dental treatment or disease as potential triggers. Conclusion: Oral anddental condition may be the trigger cause of HSP attack. Therefore, it is important for pediatric dental practitioner to be aware of thecourse of the disease in order to limit the expanding complications.&gt; latar belakang: Henoch-Schönlein purpura (HSP) merupakan vaskulitis pembuluh darah kecil sistemik yang jarang terjadi danbiasanya menyerang anak usia 2 hingga 10 tahun. Penyakit tersebut seringkali dapat sembuh sendiri, tetapi pada jangka panjangdapat bermanifestasi dengan morbiditas ginjal. Keparahan keterlibatan ginjal menentukan prognosis penyakit. Banyak faktor yangdapat memicu serangan penyakit, tersering adalah invasi bakteri. Karena rongga mulut sering kali merupakan fokus infeksi terhadapbagian lain dari tubuh, maka mempunyai peluang sebagai faktor pemicu timbulnya penyakit, sehingga penatalaksanaan fokus infeksidalam rongga mulut, jika ada, dapat memberikan prognosis yang baik pada pasien. tujuan: Makalah ini akan menggambarkantinjauan mengenai HSP dan hubungannya dengan kesehatan gigi dan mulut berkaitan pengaruhnya terhadap prognosis HSP. tinjauanPustaka: Ruam sering terjadi pada anak; pasien anak dapat memperlihatkan gejala ruam setelah pemberian antibiotika. Selain itu,beberapa penyakit dapat bermanifestasi sebagai ruam, misalnya HSP. Merupakan hal yang penting bagi dokter gigi anak untuk memilikipengetahuan mengenai HSP dan mempertimbangkan perawatan atau penyakit gigi sebagai pemicu potensial. Kesimpulan: Keadaangigi dan mulut dapat merupakan pencetus serangan HSP, oleh karena itu penting bagi dokter gigi anak untuk memahami perjalananpenyakit sehingga dapat membatasi komplikasi yang terjadi.

Chronic Idiopathic Thrombocytopenic Purpura in children: predictive factors and outcome

Abstract Idiopathic thrombocytopenic purpura (ITP) is the most common cause of acquired thrombocytopenia in children. In 20% of cases, this condition is classified as chronic when the thrombocytopenia is persistent 6 months after diagnosis. The aim of the present study is to identify the potential factors correlating with a favorable outcome in patients with chronic ITP. Some 71 patients affected by ITP were retrospectively analyzed. Results show a higher rate of spontaneous recovery that is statistically significant for patients with platelet count at diagnosis &lt;20,000/µL. These observations suggest the possibility to delay or avoid aggressive surgical treatment for these patients.

Duration and morbidity of chronic immune thrombocytopenic purpura in children: five‐year follow‐up of a Nordic cohort

To describe the clinical course, morbidity and platelet recovery in an unselected Nordic cohort of children with chronic Immune Thrombocytopenic Purpura (ITP). Prospective 5-year follow-up of 96 children with ITP lasting more than 6 months, with reporting of hospital admissions, severity of bleeding episodes and stabilization of platelet counts above 20, 50 and 150 × 10(9) /L. The estimated 5-year recovery rate was 52%; exclusion of 12 splenectomized children did not change the estimate. Events eliciting admission to hospital occurred in 39 (41%). Major haemorrhages occurred in eight children (8%), including a nonfatal intracranial haemorrhage in one child (1%). The overall admission rate was 0.4/year of thrombocytopenia, decreasing during follow-up as thrombocytopenia converted to milder degrees. Early recovery within 2 years of diagnosis occurred in 35%, was associated with low morbidity and was more likely in young children with abrupt onset of symptoms. In a Nordic cohort of children with chronic ITP, one half had recovered 5 years after diagnosis, more than half never required hospitalization and <10% experienced serious bleeding episodes, always with a platelet count <20 × 10(9) /L. Aggressive management can be restricted to the minority of children with continuing severe thrombocytopenia and frequent, clinically significant bleeding events.

1047a Helicobacter pylori Infection and Chronic Thrombocytopenic Purpura in Children and Adolescents – a Randomized Controlled Trial

1047a Helicobacter pylori Infection and Chronic Thrombocytopenic Purpura in Children and Adolescents – a Randomized Controlled Trial

Henoch–Schönlein purpura in children and adults

Henoch–Schönlein purpura in children and adults

Efficacy of rituximab in treatment of children with refractory idiopathic thrombocytopenic purpura

Fifty children with refractory idiopathic thrombocytopenic purpura (RITP) were treated with rituximab (n =26) or vincristine (n =24).The response rate,adverse reaction and recurrence rate in two groups were compared.The CD19 +/CD20 + B cells in peripheral blood were detected by flow cytometry in 26 patients before and after RITP treatment.The response rates of rituximab group was significantly higher than that of vincristine group ( 69.2％ vs. 37.5％,x2 =9.74,P ＜ 0.01 ). There was no significant difference in the rate of adverse reaction between two groups ( 11.5％ vs.8.3％,x2 =0.62,P ＞ 0.05 ).The recurrence rate of rituximab group ( 22.2％ ) was significantly lower than that of vincristine group (55.6％,x2 =7.24,P ＜ 0.05 ).In rituximab group the platelet count after treatment was significantly higher than that before treatment ( t =12.48,P ＜0.01 ),and the ratio of CD19 +/CD20 + B cells decreased significantly after treatment compare to that before treatment ( t =6.71,P ＜0.05 ).Rituximab is effective in treatment of refractory idiopathic thrombocytopenic purpura in children,which may be associated with decreased B cells in peripheral bloos. Key words: Purpura, thrombocytopenic, idiopathic; Child

Urinary angiotensinogen levels in relation to renal involvement of Henoch‐Schonlein purpura in children

To investigate whether urinary angiotensinogen (UAGT) levels are correlated with renal involvement of Henoch-Schonlein purpura (HSP) in children, and to explore whether UAGT has any relation to the severity of HSP. The study sample consisted of 107 patients (50 boys and 57 girls, 6.68±2.41 years) with clinical diagnosis of HSP. A 24 h urine sample was collected before treatment. UAGT levels were measured in patients with HSP in the acute and convalescent phases by enzyme linked immunosorbent assay. Urinary angiotensinogen/urinary concentration of creatinine levels were significantly higher in proteinuric HSP in the acute phase and the convalescent phase (32.02±3.95 and 25.31±4.11 µg/g) compared with those with HSP without renal involvement (17.26±2.60 and 15.14±3.81 µg/g) and those with hematuric HSP (19.70±2.21 and 17.28±3.62 µg/g) (P<0.0001 and P<0.01, respectively). Using matched urine samples from the same patients, UAGT/urinary concentration of creatinine (UCr) levels of proteinuric HSP patients were significantly lower in the convalescent phase (25.31 ± 4.11 µg/g, P<0.01) than in the acute phase (32.02±3.95 µg/g). UAGT/UCr levels showed positive correlation with 24 h urine protein or serum creatinine in both hematuric HSP and proteinuric HSP groups during the acute phase (P<0.05). Urinary angiotensinogen levels were remarkably high in the acute phase in the patients with proteinuric HSP, suggesting increased UAGT may indicate a series of functional changes in the kidney and it may be used as a potential biomarker of severity of HSP to monitor the progression of HSP with renal involvement.

Presentation and Outcome of Idiopathic Thrombocytopenic Purpura in Children: A Single Institution Experience

Abstract 4683 PURPOSE:To review the presenting features, response to treatment and outcome in children diagnosed with idiopathic thrombocytopenic purpura (ITP) at the University of South Alabama, Children's and Women's Hospital and Specialty Clinic. METHODS:Using ICD code 287.3, data were collected from the specialty clinic's medical records and hospital database for children diagnosed with ITP between January 2005 and September 2010. Recurrent and chronic ITP were defined as thrombocytopenia recurring within or more than 6 months of diagnosis, respectively. Univariate and multivariate logistic regression analyses were performed to evaluate variables associated with chronic ITP. RESULTS:Eight four patients were identified (M,F 1:1) with an average age of 70 months at diagnosis. Mean platelet count at presentation was 14k. Oral or nasal mucosal bleeding occurred in 19(23%) patients but none experienced a serious hemorrhage. Thirty three (39%) patients had an associated illness prior to diagnosis of ITP. Treatment consisted of intravenous immunoglobulin (IVIG) in 38(45%), WinRho in 11(13%), IVIG or WinRho followed by the other in 20(24%), data not available 8(10%) and no therapy in 7 patients (8%). Average platelet count at discharge and within 2 weeks after IVIG and WinRho was 57k, 337k and 57k, 375 respectively. Forty three (51%) were acute, 17(20%) became recurrent, and 24(29%) became chronic ITP. Bone marrow examination was performed in 26(30%) patients upon subsequent relapse but the diagnosis remained unaltered in all cases. Rituximab therapy was provided to five and splenectomy was performed in 7 patients. Four patients failed both modalities, all of whom currently are IVIG dependant.Age <5year (OR 0.12, 95%CI 0.22, 0.67, p=0.01) was protective against development of chronic ITP while platelet count >20k at presentation (OR 6.50, 95%CI 1.35, 31.30, p=0.02) and race other than white (OR 36.63, 95%CI 4.61, 291.09, p=0.001) were found to be significantly associated with the development of chronic ITP. Gender, mean platelet volume, total white cell count, and absolute lymphocyte count had no significant association. CONCLUSION:Our study supports the published data that patients with an initial platelet count >20k, older age and non-white race have an increased risk of progression to chronic ITP. Other published variables had no significant association in our analyses. Response to IVIG and WinRho was no different in our patients while rituximab or splenectomy did not lead to a complete resolution in refractory cases. Since bone marrow examination did not alter the diagnosis in any patient, we suggest that routine performance of this procedure may be omitted when a diagnosis of ITP is consistent with clinical history, physical examination and laboratory data. Disclosures:No relevant conflicts of interest to declare.

CNR2 functional variant (Q63R) influences childhood immune thrombocytopenic purpura

Immune thrombocytopenic purpura is an acquired autoimmune disorder that is the most common cause of thrombocytopenia in children. The endocannabinoid system is involved in immune regulation. We evaluated a common missense variant (CAA/CGG; Q63R) of the gene encoding the cannabinoid receptor type 2 (GeneID 1269) in 190 children with immune thrombocytopenic purpura and 600 healthy controls. The allelic frequencies and genotype distribution of the polymorphism in the patients were significant compared to control samples (P=0.006 and P=0.0001, respectively). Interestingly, when acute and chronic immune thrombocytopenic purpura patients were analyzed separately with respect to controls, a significant overrepresentation of the RR genotype and of the R allele was observed only for the chronic form (P=0.00021 and P=0.011, respectively). The relative odds ratio suggested the risk of developing chronic form was more than double in immune thrombocytopenic purpura children homozygous for the variant (odds ratio=2.349, 95% CI: 1.544-3.573; P<0.001).