Purpura In Children Research Articles

The relationship between the severity and complications of Henöch-Schönlein purpura in children and dietary inflammatory index: a retrospective cohort study.

To investigate the association between the Dietary Inflammatory Index (DII) and disease severity as well as complications in children diagnosed with Henöch-Schönlein purpura (HSP), shedding light on the potential influence of dietary factors on HSP. A retrospective cohort study was conducted, enrolling children aged 2-14 years diagnosed with HSP. Participants were divided into low and Pro-inflammatory dietary groups based on their DII scores. Biomarkers, nutrient intake, blood lipid profiles and disease complications were compared between the two groups. Spearman correlation analysis was performed to assess the relationship between DII and complications. A total of 115 patients, including 56 patients with anti-inflammatory dietary and 59 with pro-inflammatory dietary, were included. The pro-inflammatory dietary group demonstrated significantly elevated of C-reactive protein, tumor necrosis factor-α, interleukin-6, erythrocyte sedimentation rate, white blood cell count, eosinophils, IgE, consumption of total calories, protein, carbohydrates, fiber, fat intake, total cholesterol, LDL cholesterol, HDL cholesterol, triglycerides, VLDL cholesterol, complications of renal, skin, gastrointestinal, coagulation and respiratory in comparison to the anti-inflammatory dietary group. DII was positively correlated with renal, skin, gastrointestinal, coagulation and respiratory complications. The study highlights the potential influence of dietary inflammatory potential, as quantified by the DII, on disease severity and complications in children with HSP. Understanding the interplay between dietary patterns and inflammatory responses in pediatric vasculitis has implications for the management of HSP, emphasizing the relevance of considering dietary interventions to optimize clinical outcomes and improve the overall well-being of affected children.

Immune thrombocytopenic purpura in children: An update

Immune thrombocytopenic purpura in children: An update

Role of leptin in immune thrombocytopenic purpura in children

Role of leptin in immune thrombocytopenic purpura in children

Alteration of gut microbiota in Henoch-Schönlein purpura children with gastrointestinal involvement.

The compositional and structural changes of gut microbiota were closely related to the status of Henoch-Schönlein purpura (HSP). To investigate if clinical indicators and gut microbiota differ between HSP patients with or without gastrointestinal (GI) involvement and to explore the alterations of fecal microbiota in HSP children with and without GI symptoms. A total of 22 children with HSP were enrolled in the study. Fecal microbiota composition was analyzed by 16S rRNA sequencing. Clinical indicators, fecal microbial diversity, and compositions were compared between the two groups. Respectively, 9 patients with GI involvement (HSP-A) and 13 patients without GI involvement (HSP-N) were enrolled. Prealbumin (PA) and the ratio of immunoglobulin A (IgA) / complement (C)3 were significantly decreased in the HSP-A group and an elevated D-dimer was found in the HSP-N group. The relative abundances of Blautia, Lachnospira, and Haemophilus were significantly higher in the HSP-A group compared to HSP-N. Lower levels of unidentified Prevotellaceae, Parabacteroides, and Romboutsia were found in HSP-A patients. The linear discriminant analysis effect size (LEfSe) showed that the biomarkers for the HSP-A group included Blautia, Anaerostipes, Veillonella, Lachnospira, and Haemophilus. For the HSP-N group, unidentified Prevotellaceae, Intestinibacter, Romboutsia, and Akkermansia were the prominent biomarkers at the genus level. Additionally, the ratio of IgA/C3 exhibited a negative correlation with the genus Blautia. Meanwhile, PA showed negatively correlation with Veillonella. These results provide a broader understanding for future microbial-based therapies to decrease the development of GI involvement and improve the clinical outcome of HSP in children.

Associations of MEFV gene variants, IL-33, and sST2 with the risk of Henoch-Schönlein purpura in children

ObjectiveHenoch-Schönlein purpura (HSP) is the most common systemic vasculitis in children. HSP is a multifactorial inflammatory disease, but its pathogenesis is still unclear. The pathogenicity of familial Mediterranean fever gene (MEFV) variants in HSP remains controversial. The objective of this study was to evaluate relationships between MEFV variants and susceptibility to HSP and their associations with clinical outcomes. We also investigated levels of IL-33 and soluble suppression of tumorigenicity 2 (sST2) in children with HSP and their clinical significance. MethodsWe selected 100 children with HSP as the case group. The control group consisted of 50 children who visited the hospital for physical health examinations. All subjects were screened for MEFV gene exon mutations, and levels of IL-33 and sST2 were measured. ResultsThe frequency of MEFV variants was significantly greater in HSP patients than in healthy controls. The variant with the highest frequency was E148Q. The frequency of the C allele of the MEFV variant E148Q was 32 % in HSP patients and 18 % in controls (P-adjust = 0.04). Patients with the MEFV E148Q variant had more frequent joint involvement and recurrent purpura and higher levels of IL-33 and C-reactive protein (CRP). Levels of IL-33 and sST2 in children with HSP were significantly higher than those in the control group, and the sST2/IL-33 ratio in children with HSP was unbalanced (P-adjust <0.05). Logistic regression analysis revealed the presence of E148Q and an unbalanced sST2/IL-33 ratio to be independent risk factors for HSP. ConclusionThe results of this study suggest that the MEFV variant E148Q is associated with HSP susceptibility in Chinese children and that carriers of the variant may have more severe clinical manifestations and greater inflammatory responses. E148Q and the sST2/IL-33 ratio may play important roles in the pathogenesis of HSP.

Homologous 111In-Radiolabeled Platelet Survival and Sequestration Exploration for Refractory Immunologic Thrombocytopenic purpura in Children: A Strategy to Avoid Unnecessary Splenectomy

Homologous ¹¹¹In-Radiolabeled Platelet Survival and Sequestration Exploration for Refractory Immunologic Thrombocytopenic purpura in Children: A Strategy to Avoid Unnecessary Splenectomy

Analysis of the influencing factors of abdominal Henoch-Schonlein purpura in children with gastrointestinal bleeding and the clinical value of PLR.

To identify the influencing factors of gastrointestinal bleeding in children with abdominal-type Henoch-Schonlein purpura (HSP) and to assess the diagnostic value of PLR (platelet-to-lymphocyte ratio). We retrospectively analyzed the medical records of 112 children with abdominal HSP admitted to Northwest Women's and Children's Hospital from April 2021 to May 2023. Among them, 62 cases with gastrointestinal bleeding constituted the bleeding group, while the other 50 cases without gastrointestinal bleeding comprised the non-bleeding group. We compared PLR and related routine blood indicators between the two groups. Univariate and multivariate logistic regression analyses were performed to identify independent risk factors for gastrointestinal bleeding. HSP children with gastrointestinal bleeding were further categorized based on treatment efficacy, and the predictive value of PLR for treatment efficacy was analyzed. The observation group exhibited significantly higher levels of WBC, NEU, PLT, MPV, C-reactive protein, and PLR, along with lower lymphocyte levels compared to the control group (all P < 0.05). Univariate analysis revealed associations between symptom onset, abdominal pain, vomiting, levels of WBC, NEU, LYM, PLT, PLR, C-reactive protein and gastrointestinal bleeding (all P < 0.05). Multivariate logistic analysis identified onset with abdominal pain, high WBC values, and elevated PLR ratios as risk factors for gastrointestinal bleeding. The ROC curve demonstrated an AUC of 0.914 for PLR in predicting gastrointestinal bleeding. Additionally, PLR was significantly lower in the good efficacy group compared to the poor efficacy group. The AUC of PLR in predicting treatment efficacy was 0.804, indicating high predictive value. Elevated PLR may serve as a potential risk factor for gastrointestinal bleeding in children with abdominal-type allergic purpura. Monitoring changes in PLR could aid in diagnosis and improvements in treatment for this condition.

Congenital thrombotic thrombocytopenic purpura in children

Introduction. Congenital thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening genetic disorder characterized by recurrent episodes of microangiopathic hemolytic anemia, thrombocytopenia, and organ dysfunction due to the defi ciency or dysfunction of the ADAMTS13 enzyme. Despite the availability of clinical and laboratory diagnostic criteria the diagnosis remains challenging due to a wide range of diseases with similar appearance.Aim: to analyze the clinical manifestations and treatment results of identifi ed cases of congenital TTP at the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology and Immunology.Methods. This article reviews 11 clinical cases with congenital TTP genetically confi rmed or with typical clinical and laboratory signs.Results. Clinical and laboratory signs of TTP are highly variable and nonspecifi c, which was observed in the described cohort of patients. One of the trigger factors is often an acute respiratory viral infection, and stabilization of the disease can occur after transfusions of blood components. A delay of diagnosis can often lead not only to a delay of correct therapy, but also to the prescription of wrong therapy. The main diagnosis confi rmatory method was a molecular genetic test, which was carried out in 8 cases. All patients diagnosed with congenital TTP were successfully treated with FFP therapy.Conclusion. Early diagnosis of TTP is diffi cult due to the lack of specifi c clinical manifestations and easily accessible laboratory tests

Serum total bile acid levels assist in the prediction of acute intussusception with abdominal type Henoch-Schonlein purpura in children.

The severe acute abdomen associated with Henoch-Schonlein purpura (HSP) is an acute intussusception (AI). There is no reliable specific marker for AI with abdominal-type HSP. The serum total bile acid (TBA) level is a new prognostic marker associated with the severity of intestinal inflammation. The purpose of this study was to identify the prognostic value of serum TBA levels for the diagnosis of AI in children with abdominal-type HSP. A retrospective study of 708 patients with abdominal-type HSP was conducted, with demographic data, clinical symptoms, hepatic function index, immune function markers, and clinical outcomes assessed. Patients were divided into two groups: HSP (613 patients) and HSP with AI (95 patients). The data were analysed using SPSS 22.0. Of the 708 patients, the serum TBA levels were higher in the HSP with AI group than in the HSP group (P < 0.05). Logistic regression analysis showed that vomiting (OR = 396.492, 95% CI = 14.93-10,529.67, P < 0.001), haematochezia (OR = 87.436, 95% CI = 5.944-1,286.214, P = 0.001), TBA (OR = 16.287, 95% CI = 4.83-54.922, P < 0.001), and D-dimer (OR = 5.987, 95% CI = 1.892-15.834, P = 0.003) were independent risk factors for abdominal-type HSP with AI. Receiver operating characteristic (ROC) curve analysis showed that the optimal cut-off serum TBA value (sensitivity = 91.58%, specificity = 84.67%, AUC = 93.6524%) was >3 μmol/L for predicting AI in children with abdominal-type HSP. In this group of HSP patients with AI, a serum TBA level ≥6.98 μmol/L was significantly associated with an increased incidence of operative treatment (51.85% vs. 75.61%, P = 0.0181), intestinal necrosis (9.26% vs. 29.27%, P = 0.0117), and length of hospital stay [15.76 ± 5.31 vs. 10.98 ± 2.83 (days), P < 0.0001]. In children with HSP and AI, the serum TBA level was significantly higher. A novel but promising haematological indicator, the serum TBA level, helps identify HSP with and without AI and predicts intestinal necrosis in HSP with AI.

Predictor Factors for Chronicity in Immune Thrombocytopenic Purpura in Children.

(1) Background: Immune thrombocytopenia (ITP) is an acute autoimmune blood disorder that is the main cause of thrombocytopenia in children. It is characterized by a decrease in platelets below 100 × 109/L, and limited evolution with severe complications such as intracranial hemorrhage. The chronic form is defined by the persistence of thrombocytopenia more than 12 months after diagnosis. (2) Methods: We performed a retrospective study over a period of 10 years (1 January 2011-31 December 2020) at the Emergency Clinical Hospital for Children "Sf. Maria", Iasi. The aim of the study was to describe the clinical characteristics and to determine the prognostic factors in immune thrombocytopenia in children. (3) Results: In this study we included 271 children with ITP, comprising 123 females (45.4%) and 148 males (54.6%). The remission rate was higher in males, being 68.9% compared to 56.1% in females. Children with ITP under 9 years of age had a higher remission rate. Children with a platelet count > 10 × 109/L at diagnosis had a higher likelihood-of-remission rate compared to patients who presented initial platelet count below this value. (4) Conclusions: The risk factors highly suggestive for chronicity are: age at diagnosis, female sex, and the number of platelets at the onset of the disease.

How to spot Henoch-Schönlein purpura in children and act on it

Advice for nurses on how to identify this disease affecting a child’s small blood vessels, and also rule out other possibilities such as sepsis and meningitis

Expert consensus on diagnosis and treatment of hereditary thrombotic thrombocytopenic purpura in children

儿童遗传性血栓性血小板减少性紫癜（hTTP）是一种少见但严重，甚至可致命的儿童血栓性微血管病，其发病机制为血管性血友病因子裂解酶（ADAMTS13）基因突变所导致的ADAMTS13活性持续严重缺乏。相较于成人，儿童期起病的hTTP更易出现脑或肾远期并发症，对预防性替代治疗的需求更迫切。本共识涵盖hTTP的定义、诊断、鉴别诊断及治疗，旨在为中国儿科医师开展儿童hTTP规范化诊治提供参考。.

Clinical Aspect and Outcome of Henoch-Schoenlein Purpura in Children in Relation to Renal Biopsy Pathologic Findings

The most common childhood vasculitis is Henoch Schoenlein Purpura (HSP). It occurs at any age peaking at around 4-6 years. The classic manifestations of HSP are skin rash, along with any from a triad of other organ system involvement, including gastrointestinal, musculoskeletal, and renal systems. Renal involvement is much more common in children than in adults. 255 HSP patients attending our tertiary referral hospital enrolled in this 10-year retrospective study from 2009 to 2019. According to our study, HSP was more common in males. Most of the patients were between 1-16 years (mean 7.04 years) old. Of these patients, only 19.22% had proteinuria, 17.25% had increased creatinine, 61.7% of patients had hematuria, 67.06 had a fever, and 69.8% had abdominal pain. 24.31% of them had bloody diarrhea. Skin and joint involvement were recorded in 44.7% and 74.9% of patients, respectively. Children affected by HSP may present with different clinical manifestations. We assessed these clinical presentations and outcomes of patients and compared them with renal pathology findings to reveal any prognostic significance of renal pathologic findings in HSP patients.

Effect of MEFV variants on the presentation and clinical course of Henoch-Schonlein purpura in children?

Objective: We aimed to evaluate MEFV mutation frequency and the effects of MEFV mutations on the clinical course including renal involvement in children with Henoch-Schonlein Purpura (HSP).&#x0D; Methods: Children with a diagnosis of HSP who were evaluated for the presence of MEFV mutations were enrolled in this study. Patients were primarily assigned into two groups based on the presence of MEFV mutations as Group 1 including patients without mutations and Group 2 including patients with mutations in at least one allele (heterozygous, homozygous, or compound heterozygous). We also investigated specifically the effects of M694V mutation on the course of HSP by comparing patients with M694V mutation in at least one allele with patients not carrying M694V mutation.&#x0D; Results: Forty-seven patients (23 female) were enrolled. MEFV mutation rate (53%) was 3.5 times the rate in general population. M694V was the most common mutation (48%). Patients with MEFV mutations, especially those with M694V mutation, had lower incidence of preceding infection, but increased inflammatory markers, scalp edema and relapse rate. Renal involvement and long-term prognosis were not affected by the presence of MEFV mutations.&#x0D; Conclusions: MEFV mutations cause susceptibility to develop HSP and are associated with increased inflammation and altered clinical course. However, renal involvement and long-term prognosis were not affected by the presence of MEFV mutations.

Immune Thrombocytopenia Purpura in Children in Lebanon: Prevalence, Treatment Modalities, and Clinical Outcomes in a Retrospective Study

Background: Immune thrombocytopenia purpura (ITP) is one of the most common autoimmune diseases in children characterized by a decreased number of circulating platelets combined with impaired platelet production. There is limited literature data on the prevalence and treatment modalities, and outcome of ITP in children from Lebanon. Methods: We retrospectively reviewed the demographic and clinical data of 59 patients aged 0–18 years diagnosed with ITP between January 2007 and April 2016 in different hospitals in Beirut and the south of Lebanon. Results: ITP patients represented 2.5% of the total number of children admitted to these hospitals during this period. Among the ITP children, 55.93% were male and 44.07% were female. The greatest number of ITP children were in the 1–4 year group, followed by the 5–9 year group. As for the clinical course of the disease, 40.68% of the ITP children presented acute ITP, whereas 59.32% presented chronic ITP. Among the different therapeutic approaches adopted to treat these ITP children, intravenous immunoglobulin was the most commonly used, followed by steroids, a combination of these both agents, cyclosporine, and splenectomy. Interestingly, these therapeutic modalities induced a statistically significant increase in the patients’ platelet count. In addition, the clinical course of ITP was not significantly associated with each of the age group, the platelet count at diagnosis, and gender of patients. Conclusion:This study showed the prevalence of ITP among children from Lebanon, where more than half of ITP children presented a chronic disease. Further studies are needed to evaluate additional predictors of chronic ITP among children from Lebanon and help medical providers make informed decisions about treating childhood ITP. Doi: 10.28991/SciMedJ-2022-04-04-02 Full Text: PDF

Development and validation of nomogram prediction model for severe kidney disease in children with Henoch-Schönlein purpura: A prospective analysis of two independent cohorts-forecast severe kidney disease outcome in 2,480 hospitalized Henoch-Schönlein purpura children.

This study aimed to develop and validate a nomogram to forecast severe kidney disease (SKD) outcomes for hospitalized Henoch–Schönlein purpura (HSP) children. The predictive model was built based on a primary cohort that included 2,019 patients with HSP who were diagnosed between January 2009 and December 2013. Another cohort consisting of 461 patients between January 2014 and December 2016 was recruited for independent validation. Patients were followed up for 24 months in development/training and validation cohorts. The data were gathered at multiple time points after HSP (at 3, 6, 12, and 24 months) covering severe kidney disease as the severe outcome after HSP. The least absolute shrinkage and selection operator (LASSO) regression model was utilized to decrease data dimension and choose potentially relevant features, which included socioeconomic factors, clinical features, and treatments. Multivariate Cox proportional hazards analysis was employed to establish a novel nomogram. The performance of the nomogram was assessed on the aspects of its calibration, discrimination, and clinical usefulness. The nomogram comprised serious skin rash or digestive tract purpura, severe gastrointestinal (GI) manifestations, recurrent symptoms, and renal involvement as predictors of SKD, providing favorable calibration and discrimination in the training dataset with a C-index of 0.751 (95% CI, 0.734–0.769). Furthermore, it demonstrated receivable discrimination in the validation cohort, with a C-index of 0.714 (95% CI, 0.678–0.750). With the use of decision curve analysis, the nomogram was proven to be clinically useful. The nomogram independently predicted SKD in HSP and displayed favorable discrimination and calibration values. It could be convenient to promote the individualized prediction of SKD in patients with HSP.

The Effect of Prednisone Dose on the Clinical and Laboratory Course of Henoch–Schönlein Purpura in Children

Objective: Henoch–Schönlein purpura (HSP) is the most common form of systemic vasculitis in children. Most cases are self-limited. Corticosteroids may be considered for HSP patients with severe renal or extrarenal involvement. However, they have some adverse effects related to the dose and the period of treatment. The study aimed to assess the efficacy and safety of dose 1mg/kg/day of prednisone in the treatment of HSP clinical and laboratory manifestations compared with dose 2 mg/kg/day. Methods: A total of 28 patients (14 treated with prednisone 1 mg/kg and 14 treated with prednisone 2 mg/kg, followed up for 6 months) were included in a prospective randomized trial study, at University Children Hospital in Damascus, Syria, between 2020 and 2021. Results: As prednisone was only used in severe manifestations, severe abdominal pain was the most common indication (53.5%), followed by gastrointestinal hemorrhage (32.1%). However, prednisone effect on all symptoms and laboratory findings for all patients was studied. Both doses of prednisone (1 and 2 mg/kg/day for 2 weeks, with weaning over the subsequent 2 weeks) were effective in reducing the intensity of extrarenal symptoms, and no statistical difference was found (rash: P = 0.47, abdominal pain: P = 0.32, gastrointestinal hemorrhage: P = 0.32, arthritis: P = 0.84, and soft-tissues edema: P = 1). In both groups, new renal symptoms and gastrointestinal complications were developed. Elevated inflammatory markers (CRP: 59.3%, ESR: 77.3%) turned up normal at the end of treatment in both groups. Children treated with prednisone 2mg/kg showed increased appetite; three cases developed Cushing’s moon face; one case showed a transient hypertension at the end of treatment. Conclusions: The routine, early use of prednisone in uncomplicated HSP is not recommended. However, when it is indicated, a 2-week course of prednisone 1 mg/kg/day could be an effective alternative to the higher dose (2 mg/kg/day), with less side effects.

Development and Validation of a Differential Diagnosis Model for Acute Appendicitis and Henoch-Schonlein Purpura in Children.

Objective: To study and develop a predictive model for the differential diagnosis of acute appendicitis (AA) and Henoch-Schonlein purpura (HSP) in children and to validate the model internally and externally. Methods: The complete data of AA and HSP cases were retrospectively analyzed and divided into internal and external verification groups. SPSS software was used for single-factor analysis and screening of independent variables, and R software was used for the development and verification of the diagnostic model. Lasso regression analysis was used to screen predictors and Lasso-logistic regression model was constructed, and K-fold cross-validation was used for the internal verification. In addition, nonfever patients were selected for model development and validation in the same way. Receiver operating characteristic (ROC) curves and calibration curves were drawn, respectively, to evaluate the 2 models. Results: Internal development and validation of the model showed that fever, neutrophil ratio (NEUT%), albumin (ALB), direct bilirubin (DBIL), C-reactive protein (CRP), and K were predictive factors for the diagnosis of HSP. The model was presented in the form of a nomogram, and the area under ROC curve of the development group and verification group was 0.9462 (95% confidence interval [CI] = 0.9402-0.9522) and 0.8931 (95% CI = 0.8724-0.9139), respectively. In the model of patients without fever, NEUT%, platelets (PLT), ALB, DBIL, alkaline phosphatase (ALP), CRP, and K were predictive factors for the diagnosis of HSP, and the area under ROC curve of the development group and verification group was 0.9186 (95% CI = 0.908-0.9293) and 0.8591 (95% CI = 0.8284-0.8897), respectively. Conclusion: In this study, 2 diagnostic models were constructed for fever or not, both of which had good discrimination and calibration, and were helpful to distinguish AA and HSP in children.

The Use of Clinical Scores in the Management of Immune Thrombocytopenic Purpura in Children.

While the majority of children with recently diagnosed ITP have a benign, self-limiting condition, most often with a spontaneously recovery, 40% of children with ITP progress toward persistent ITP and 10–20% goes toward chronicity. Several clinical scores have been developed with the aim to perform a better monitoring outcome or to differentiate transient vs. persistent ITP (e.g., Donato score). Our paper aims to describe and to compare the most important scores used in the management of ITP in children: bleeding severity scores and chronicity prediction scores. These scores include a combination of different already known risk factors: age, gender, presence of a previous infections or vaccination, bleeding grade, type of onset, platelet count at diagnosis. The real utility of these scores has been a matter of debate and no consensus has been reached so far as to their necessity to be implemented as compulsory tool in the care of children with ITP.

Molecular Diagnosis Is Vital to the Accurate Classification and Management of Thrombotic Thrombocytopenic Purpura in Children.

Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially life-threatening hematologic disease, presenting a myriad of diagnostic and management challenges in children. Here, we provide a review of this disorder and discuss 2 exemplary cases of TTP occurring in adolescents, emphasizing the need for consideration of late-onset congenital TTP (cTTP). We demonstrate the importance of early confirmation of ADAMTS13 enzyme deficiency and the presence or absence of ADAMTS13 inhibitor in order to rapidly initiate the appropriate life-saving therapies. Ultimately, molecular testing is paramount to distinguishing between congenital and acquired immune-mediated TTP.