Purpura In Children Research Articles

Rotavirus-associated immune thrombocytopenic purpura in children: A retrospective study.

Certain studies have previously indicated that an association may exist between rotavirus infection and primary immune thrombocytopenic purpura (ITP). The present retrospective study aimed to investigate whether rotavirus may cause ITP in children. Firstly, the incidence of ITP in children with or without rotavirus diarrhea was compared. A 14.58% incident rate was observed in children with rotavirus diarrhea compared with a 7.22% incident rate in children without rotavirus diarrhea. Subsequently, the clinical features of ITP children with or without rotavirus infection were compared. The results indicated that ITP children with rotavirus infection were significantly younger, showed significantly decreased mean platelet volume (MPV) levels and presented a significantly higher frequency of bleeding score of 3 against ITP children without rotavirus infection. In conclusion, these findings suggest that rotavirus serves a causative role in ITP.

Parainfluenza infection is associated with Henoch-Schönlein purpura in children

Abstract Background To investigate the association of respiratory tract viruses with Henoch-Schonlein purpura (HSP) in children. Methods 40 children with HSP and 42 controls with upper respiratory tract infections were enrolled in the study. In both groups, the serum IgM antibodies and viral antigens in peripheral blood mononuclear cells (PBMCs) of common respiratory tract viruses including parainfluenza virus (PIV), influenza, respiratory syncytial virus (RSV), and adenovirus were detected. Children with HSP nephritis (HSPN) received renal biopsy, and antigens of the aforementioned viruses were examined. Results Viral IgM antibodies in sera and antigens in PBMCs were detected in HSP and control groups (55% vs. 42.9% and 60% vs. 42.9% respectively), but the differences were not significant. The prevalence of PIV IgM antibody and antigen in PBMCs in HSP group was significantly higher than that in controls (P Conclusions The presence of respiratory tract viruses was common in HSP children. PIV may be an important contributing factor in the pathogenesis of HSP.

Progress of abdominal type Henoch-Schönlein purpura in children

Henoch-Schonlein purpura(HSP)is the most common disease with small vasculitis as the main pathological changes in childhood.Gastrointestinal symptoms as the main clinical manifestations are called abdominal type allergic purpura, some children show abdominal symptoms before skin purpura, even without skin purpura during the whole course of disease, which usually leads to misdiagnose.This article summaries the clinical diagnosis and treatment progress of abdominal type allergic purpura. Key words: Abdominal type Henoch-Schonlein purpura; Chlidren

Experience with the drug 5 % human normal immunoglobulin for intravenous administration of Ig Vena N.I.V. in the treatment of immune thrombocytopenic purpura in children

Intravenous immunoglobulin (IVIG) is the first-line therapy for immune thrombocytopenia (ITP) at children. However, IVIGs are not absolutely comparable, as routine practice shows, so the choice of IVIG requires analysis of specific clinical cases and studies. IVIGs application requires to take into account not only effectiveness, but also tolerability. We investigated effectiveness and safety of Ig VENA (Kedrion S.p.A., Italy) administration for treatment of 62 patients with initially diagnosed ITP at the age of 6 months to 15 years. Positive effect of application of the medication reached at 85.5 % of children with this disease. None of the patients experienced serious adverse events during the study period. Short-term adverse events (fever, nausea, headache) were registered at 16.1 % of patients and did not require discontinuation of Ig VENA therapy. Thus, our study demonstrated the high effectiveness and safety of Ig VENA therapy for children with ITP.

Henoch-Schonlein Purpura in Children Hospitalized at a Tertiary Hospital during 2004-2015 in Korea: Epidemiology and Clinical Management.

PurposeTo investigate the epidemiology, clinical manifestations, investigations and management, and prognosis of patients with Henoch-Schonlein purpura (HSP).MethodsWe performed a retrospective review of 212 HSP patients under the age of 18 years who were admitted to Inje University Sanggye Paik Hospital between 2004 and 2015.ResultsThe mean age of the HSP patients was 6.93 years, and the ratio of boys to girls was 1.23:1. HSP occurred most frequently in the winter (33.0%) and least frequently in the summer (11.3%). Palpable purpura spots were found in 208 patients (98.1%), and gastrointestinal (GI) and joint symptoms were observed in 159 (75.0%) and 148 (69.8%) patients, respectively. There were 57 patients (26.9%) with renal involvement and 10 patients (4.7%) with nephrotic syndrome. The incidence of renal involvement and nephrotic syndrome was significantly higher in patients with severe GI symptoms and in those over 7 years old. The majority of patients (88.7%) were treated with steroids. There was no significant difference in the incidence of renal involvement or nephrotic syndrome among patients receiving different doses of steroids.ConclusionIn this study, the epidemiologic features of HSP in children were similar to those described in previous studies, but GI and joint symptoms manifested more frequently. It is essential to carefully monitor renal involvement and progression to chronic renal disease in patients ≥7 years old and in patients affected by severe GI symptoms. It can be assumed that there is no direct association between early doses of steroids and prognosis.

Association study between matrix metalloproteinase-9 gene (MMP9) polymorphisms and the risk of Henoch-Schönlein purpura in children.

Henoch-Schönlein purpura nephritis (HSPN), the most serious long-term complication of Henoch-Schönlein purpura, is one of the most common renal diseases in children. Matrix metalloproteinase-9 (MMP-9) is implicated in the pathogenesis of renal diseases. Genomic DNA was isolated from the venous blood leukocytes of 220 unrelated patients with HSPN and 205 unrelated healthy individuals. To identify markers contributing to genetic susceptibility to HSPN, this study examined the potential association between HSPN and four single nucleotide polymorphisms of the MMP-9 gene (MMP9) (rs17576, rs3918254, rs3787268, and rs2236416) by using the MassARRAY system. The allelic or genotypic frequencies of the rs17576 (exon 6) and rs3918254 (intron 6) polymorphisms in HSPN were significantly different from those in the healthy controls. The HSPN subjects had a significantly higher frequency of the G allele of rs17576 (χ(2) = 8.416, P = 0.004, OR = 1.556, 95%CI = 1.153-2.100) and a significantly lower frequency of the A allele of rs2236416 (χ(2) = 10.363, P = 0.001, OR = 0.545, 95%CI = 0.375-0.791). Linkage disequilibrium was observed in two blocks (D' > 0.9; r(2) > 0.8 in control). In block 1, significantly more G-C haplotypes (P = 0.011) and significantly fewer A-C haplotypes (P = 0.008) were found in the HSPN subjects. In block 2, significantly more G-G haplotypes (P = 0.016) and significantly fewer A-G haplotypes (P = 0.006) were found in the HSPN subjects. These observations suggest that the rs17576 and rs3918254 polymorphisms of MMP9 are associated with HSPN.

Current views of the relationship between Helicobacter pylori and Henoch-Schonlein purpura in children.

Helicobacter pylori (H. pylori) is one of the factors involved in the pathogenesis of various gastrointestinal diseases and may play a potential role in certain extra-intestinal diseases. H. pylori infection are mainly acquired during childhood, and it has been reported that in endemic areas of China the infection rates are extraordinarily higher in HSP children, particular those with abdominal manifestations. Furthermore, eradication therapy may ameliorate Henoch-Schonlein purpura (HSP) manifestations and decrease the recurrence of HSP. Therefore, results suggested that detection of H. pylori infection by appropriate method ought to be applied in HSP children. Current evidences indicate that local injury of gastric mucosa and immunological events induced by H. pylori infection are involved in the development of HSP. Increased serum IgA, cryoglobulins, C3 levels, autoimmunity, proinflammatory substances and molecular mimicry inducing immune complex and cross-reactive antibodies caused by H. pylori infection might play their roles in the course of HSP. However, there are no investigations confirming the causality between H. pylori infection and HSP, and the pathogenesis mechanism is still unclear. More bench and clinical studies need to be executed to elaborate the complex association between H. pylori and HSP.

Dental considerations on the management of Idiopathic Thrombocytopenic Purpura in children: case report

Idiopathic Thrombocytopenic Purpura (ITP) is a hematological disease characterized by decreased number of blood platelets. Clinically, children with ITP may present petechiae, ecchymoses, haematuria, epistaxis and occasionally hemorrhage. Oral manifestations include spontaneous gingival bleeding, petechiae or hematomas of the mucosa, palate and tongue. It is important for dentists to be aware of ITP in order to properly recognize this condition and offer the adequate treatment to the patient. The aim of this report was, therefore, to relate the case of a 4-year-old patient with acute ITP, to review its main clinical signs in children and describe the management of these patients at the dental office.

Investigation of mean platelet volume as a predictor of acute versus chronic immune thrombocytopenic purpura in children: An exploratory analysis

Current difficulty remains in predicting acute versus chronic immune thrombocytopenic purpura (ITP) in children at the time of diagnosis. The purpose of this exploratory retrospective study was to determine the relationship between mean platelet value (MPV) and initial platelet count at time of diagnosis on prediction of acute versus chronic ITP among pediatric patients. When odds ratios were calculated, initial platelet count less than 10,000/μl was the only statistically significant predictor of acute ITP ( i.e. , case resolution). MPV < 8 fl was not a statistically significant predictor of acute ITP, although clinical trends suggested that those with acute ITP had lower MPV at time of diagnosis. This exploratory study does suggest that multi-center prospective study designs are needed for further study to describe if there is a relationship between MPV in determining acute versus chronic ITP. Adequately predicting acute versus chronic ITP at diagnosis could have numerous implications for children and families as they prepare for treatment and help nurses deliver patient-centered interventions.

Clinical profile and pattern of Henoch-Schönlein purpura in children

Introduction: This study was done to evaluate clinical profile of Henoch- Schönlein purpura (HSP) in children admitted in Patan Hospital.Methods: The medical records of all the children admitted in children ward with diagnosis of HSP from January 2008 to December 2015 were analysed for clinical presentation, management and outcome.Results: Of 59 patients, 37 (63%) were boys. The patients’ ages ranged from 15 months to 14 years with a mean of 8.3 years. Approximately, two third cases presented during winter and autumn. Upper respiratory tract infection preceded HSP in 37 and anti streptolysin O titer was positive in 6 of the 13 (46%) children tested at presentation. Skin purpura was seen in 59 (100%), arthritis or arthralgia in 48 (81%), gastrointestinal manifestation in 47 (80%) and renal involvement in 16 (27%). Forty-one (69%) patients received corticosteroid therapy. All the children made a full recovery, two with nephritis continued to have hypertension, hematuria and proteinuria. Symptoms occurred in 6 (10%) over a period one month to two years follow up.Conclusions: The HSP in children had seasonal occurrence, skin rash, pain abdomen with mild diseases and recovered well. Journal of Patan Academy of Health Sciences, Vol. 2, No. 1, 2015. page:17-21

Is Helicobacter pylori infection a risk factor for idiopathic thrombocytopenic purpura in children?

Idiopathic thrombocytopenic purpura (ITP) is an autoimmune bleeding disorder, which occurs as a result of autoantibodies attachment to the platelets surface and subsequent destruction. Several organisms can mimic features of human antigens (Ags) and induce autoantibody production. One of these organisms is Helicobacter pylori (HP). We assessed the prevalence and relationship of HP infection with ITP in a population of children. One hundred and six children younger than 18 years old were enrolled in this case-control study in which 42 children with ITP were in the case group and 64 healthy children were in the control group. Stool exam for detection of HP-Ag were performed and the variables were compared between the two groups. Mean ± standard deviation age of case and control group was 6.4 ± 3.4 and 8.6 ± 4.4 years old, respectively. HP stool Ag differ significantly between the case and the control groups (P < 0.05). Our results support the role of HP in ITP of children, and urea breath test or Ag detection of HP in stool of these patients is recommended.

Henoch Schönlein purpura in children: retrospective analysis of 46 cases and review of literature

AmacHenoch Schonlein purpurasi (HSP) tanili hastalarin klinik ve laboratuvar karakteristiklerinin degerlendirilmesi, prognozu etkileyen risk faktorlerinin literatur esliginde incelenmesi.Gerec ve yontemAbant Izzet Baysal Universitesi Tip Fakultesi Cocuk Sagligi ve Hastaliklari Anabilim Dali'nda izlenen HSP tanili hastalarin tibbi kayitlari geriye donuk olarak incelendi. Bulgular Hasta populasyonu 46 cocuktan olusmaktadir (25 erkek, 21 kiz, ortalama yas: 6,4, yas araligi: 3-15). Petesi- purpura 46 hastada (% 100), artrit- artralji 25 hastada (% 54,3), gastrointestinal sistem tutulumu 27 hastada (% 58,6), renal tutulum 15 hastada (% 32,6) gozlendi.SonucHSP genelde hafif seyirli ve kendini sinirlayan bir hastalik olup nadiren kronik bobrek yetmezligine yol acabilir. HSP'nin uzun donem morbiditesi renal tutulumla iliskilidir. HSP'li her cocuk tanidan itibaren 1 yil boyunca periodik olarak tam idrar analizi yaptirmalidir.

Laboratory markers indicating gastrointestinal involvement of henoch-schönlein purpura in children.

PurposeTo determine clinically useful biochemical markers reflecting disease activity and/or gastrointestinal (GI) tract involvement in Henoch-Schönlein purpura (HSP).MethodsA total of 185 children with HSP and 130 controls were included. Laboratory data indicating inflammation, standard coagulation, and activated coagulation were analyzed for the HSP patients, including measurements of the hemoglobin level, white blood cell (WBC) count, absolute neutrophil count (ANC), platelet count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) level, prothrombin time, activated partial thromboplastin time, and fibrinogen, D-dimer, and fibrin degradation product (FDP) levels. The clinical scores of the skin, joints, abdomen, and kidneys were assessed during the acute and convalescence phases of HSP.ResultsThe WBC count, ANC, ESR, and CRP, fibrinogen, D-dimer, and FDP levels were significantly higher in the acute phase compared with the convalescent phase of HSP (p<0.05). The total clinical scores were more strongly correlated with the D-dimer (r=0.371, p<0.001) and FDP (r=0.369, p<0.001) levels than with inflammatory markers, such as the WBC count (r=0.241, p=0.001), ANC (r=0.261, p<0.001), and CRP (r=0.260, p<0.001) levels. The patients with GI symptoms had significantly higher ANC (median [interquartile range], 7,138.0 [4,446.4-9,470.0] vs. 5,534.1 [3,263.0-8,153.5], p<0.05) and CRP (0.49 [0.15-1.38] vs. 0.23 [0.01-0.67], p<0.05), D-dimer (2.63 [1.20-4.09] vs. 1.75 [0.62-3.39]), and FDP (7.10 [0.01-13.65] vs. 0.10 [0.01-7.90], p<0.05) levels than those without GI symptoms.ConclusionD-dimer and FDPs are more strongly associated with disease activity and more consistently reflect GI involvement than inflammatory markers during the acute phase of HSP.

Association of the paired box 2 gene polymorphism with the susceptibility and pathogenesis of Henoch‑Schönlein purpura in children.

The present study aimed to investigate the distribution of paired box 2 (PAX2) gene polymorphisms in healthy populations and in patients with Henoch‑Schönlein purpura (HSP), focusing on the association between PAX2 gene polymorphisms and the susceptibility and clinical characteristics of HSP. Genomic DNA was extracted from the peripheral venous blood of 100 healthy children (mean age: 5 ± 1.9 years) and 118 children with HSP (mean age: 10.2 ± 2.3 years). Polymerase chain reaction (PCR) was used to amplify exons 1‑12 of the PAX2 gene. Denaturing high performance liquid chromatography and DNA sequencing analysis were conducted for screening of mutations in the PAX2 gene in the PCR products. No genetic polymorphism of the PAX2 gene was identified in exons 1‑7, 9, 10 or 12. Two single nucleotide polymorphisms (SNPs), which presented as complete linkage haplotype 798C>T/909A>C, were identified in exon 8. An SNP (1164T>A) was also identified in exon 11. No significant difference in the allele and genotype frequency distribution of exon 8 (798C>T) or 11 (1164T>A) of the PAX2 gene was identified between the HSP and control groups (P>0.05). However, the frequency of the PAX2 heterozygous genotype 798C>T in the HSP with nephritis (HSPN) group was significantly higher than those in the controls and in the HSP without nephritis group (P<0.05). Furthermore, no significant correlation was identified between the PAX2 gene exon 8 polymorphism (798 C>T) and the renal pathology of children with HSPN. An SNP (1164T>A) was identified in exon 11. The PAX2 heterozygous genotype 798C>T did not increase susceptibility to HSP, however, it may be used clinically as a screening indicator for HSP in children with a high risk of renal involvement.

Plasma exchange therapy for severe gastrointestinal involvement of Henoch Schonlein purpura in children

The aim of this report is to evaluate the plasma exchange as a choice for the management of life-threating gastrointestinal system (GIS) involvement in Henoch-Schönlein purpura (HSP) when refractory to conventional therapies. We retrospectively reviewed the medical records of HSP patients who had plasma exchange therapy due to massive GIS involvement. We reported age, gender, initial HSP presentation, etiological or triggering factors and disease course. Treatment modalities, side effects and their outcomes were noted. We reported 7 cases of childhood HSP with severe gastrointestinal involvement refractory to common immunosuppression with systemic steroid and cytotoxic therapy. All patients gave inadequate response to pulse methyl prednisolone or oral prednisolone therapy with ongoing GIS bleeding and severe abdominal pain. Therefore, pulse cyclophosphamide was added to the treatment. Two patients received additional intravenous immunoglobulin (IVIG) therapy. Gastrointestinal manifestations continued and plasma exchange was performed. All patients improved after plasma exchange treatment. Treatment of GI involvement in HSP with plasma exchange has been mainly based on case reports. According to our data, we propose that, plasma exchange may be a safe and efficient management choice in paediatric HSP patients with massive GIS involvement that are refractory to other therapies.

Changes and significance of serum interleukin-17 and matrix metalloproteinase-9 in children of henoch-schonlein purpura

Objective To investigate the chanegs and correla iton between IL-17 and MMP-9 in c hildren of henoch-schonlein purpura,and to explore the pathogenesis of teh IL-17 and MMP-9 in henoch-schonlein pur-pura /henoch-schonlein purpura nephritis.Methods The level of serum IL-17 andMMP-9 in 74 henoch-schon-lein purpura childr en in initial stage and 30 cases of healthy children（ control group） were detetced by ELISA. Results （1） The level of plasma IL-17 was significantly higher in children of henoch-schonlein purpura thna tha t foh ealthy control group （86.59 ±35.50） fg/L vs.（62.38 ±14.65）fg/L,P〈0.01].（2） The level of plasma MMP-9 was significantly higher in children of henoch-schonlein pupru ar than that o fhealt hy control group [（201.82 ±105.87） fg/L vs（89.27 ±27.99）fg/L,P 〈0.01].（3） Henoch-schonlein purpura children were followed up for6 months,dependin g on whether the kidney was involved,they were devided into non-henoch-schonlein purpura nephritsi grou p nad henoch-s chonlein purpura nephritis group.In initial phase of HSP,the level of plasma IL-17 was significant lyhigher in henoch-schonlein purpura nephritis group than non-henoch-schonlein purpura nephrits group [（101.67 ±39.55） fg/L vs.（101.67 ±39.55）fg/L,P〈0.05].The level of p lasma MMP-9 was significantly higher in henoch-schonlein purpura nephritsi group than no n-henoc h-schonlein purpura nephritis group [（249.63 ±97.57） Pg/L vs.（185.30 ±104.39） pg/L,P〈0.05 ].（4） IL-17 and MMP-9 correlation aanlysis didn＇t show sign ificant correlation（r=0.184,P〉0.05）.Conclusion IL -17 and MMP-9 are involved in the pathogenesis of henohc-schonlein purpura/henoch-schonlein pur pura nephritis. Key words: Henoch-Schonlein purpura; Interleukin-17; Matrix metalloproteinase-9

Helicobacter pylori infection & immune thrombocytopenic purpura in children and adolescents: A randomized controlled trial

Helicobacter pylori and immune thrombocytopenic purpura (ITP) association is not well established in chronic ITP (cITP) in children, although the cure of thrombocytopenia in approximately half of H. pylori eradicated adult patients has been described. The aim of this study was to investigate the effect of H. pylori eradication on platelet (PLT) recovery in cITP children and adolescents through a randomized, controlled trial. A total of 85 children (mean age 11.4 years) with cITP were prospectively enrolled. Diagnosis of H. pylori was established by two locally validated tests, 13C-urea breath test and monoclonal stool antigen test. Twenty-two infected patients were identified, and randomly allocated into two groups: H. pylori treatment group (n = 11) and the non-intervention control group (n = 11). The control group was offered treatment if the thrombocytopenia persisted after the follow-up. At baseline, there were no differences regarding age, sex, duration of disease, and PLT count between groups. Sixty three of 85 patients were uninfected. PLT response was classified as complete response: PLT > 150 × 109 l−1; partial response: PLT 50–150 × 109 l−1, or an increase of 20–30 × 109 l−1; no response: PLT < 50 × 109 l−1 or an increase of <20 × 109 l−1 after at least 6 months of follow-up. Complete response was observed in 60.0% (6/10, one excluded) H. pylori eradicated patients vs. 18.2% (2/11) in non-eradicated patients (p = 0.08; OR = 6.75) after 6–9 months of follow-up. Among uninfected patients, only 13.8% (8/58) presented complete response. Two non-treated controls were treated after 6–12 months of follow-up, and PLT response was observed in 61.5% (8/13) of H. pylori eradicated patients, and in 19.0% (11/58) of uninfected patients (p = 0.004). Cytotoxin associated gene A and vacuolating cytotoxin gene A IgG antibodies were present in almost all infected patients. Therefore, the study suggests that H. pylori eradication plays a role in the management of H. pylori infected cITP children and adolescents.

Inherited Thrombotic Thrombocytopenic Purpura in Children

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.

Artefactual skin lesions in children and adolescents: review of the literature and two cases of factitious purpura

Self harm is a great diagnostic and treatment challenge. In addition, psychocutaneous conditions are rare in the pediatric population and may therefore be misdiagnosed. Dermatitis artefacta is a psychocutaneous syndrome, which is a subgroup of the general spectrum of self-inflicted skin lesions. Dermatitis artefacta encompasses an array of different clinical manifestations, including purpura. Factitious purpura has rarely been reported in children. Case report and review of the literature. We describe two Caucasian patients (9-year-old boy and 10-year-old girl) with striking purpuric lesions diagnosed as factitious purpura. The clinical lesions were similar, but the underlying psychological problems differed significantly (depression and stress). The current state of knowledge of dermatitis artefacta in children and adolescents was reviewed. The presence of purpura in children and adolescents typically causes extensive intervention programs due to the possible serious pathological consequences. The two cases demonstrate a need for a high degree of attention to psychological disturbances, lesional evolution, and distribution once the suspicion is established.

Comparison of dexamethasone and anti-D immune globulin for immune thrombocytopenia purpura in children

Different therapeutic options in children with immune thrombocytopenic purpura include observation alone, periodic treatment with corticosteroids, intravenous immunoglobulin (IVIG) or anti-D, chronic administration of immunosuppressive agents, and splenectomy.Preference of the type of therapy depends on the degree of thrombocytopenia and clinical bleeding manifestations. Dexamethasone is safe but its side effects are the main disadvantages for its usage. Anti-D is more expensive than dexamethason but the side effect is rare and not dangerous and response to treatment is assessed in approximately 3 days after infusion.