Abstract Heart failure is the prevalent complication of acute myocardial infarction. We aim to identify a biomarker for heart post-acute This observational study includes 1062 and 1043 patients with infarction in discovery validation cohorts, respectively. The outcomes are in-hospital long-term events. S100A8/A9 screened out through proteomic analysis, elevated circulating independently associated cohorts. Furthermore, predictive value superior traditional biomarkers, addition improves risk...

Early-onset high myopia (eoHM) in children is a kind of that occurs preschool children. While affecting the visual development process, eoHM can cause series complications such as retinal rupture and detachment, posterior scleral staphyloma, which not only damage function, but also delay overall Therefore, early prevention, diagnosis, timely treatment, long-term management are crucial. This article discusses necessity key points blinding caused by children, order to attract doctors'...

The first second-line international recommendation for children with severe persistent/chronic immune thrombocytopenia is thrombopoietin receptor agonist (TPO-RA)-based treatment; however, <30% can achieve sustained response off-treatment (SRoT), leading to a heavy medical burden. This study aimed confirm the efficacy of stepwise response-guided treatment protocol compared TPO-RA-based therapy P/CITP. an individualized stratified starting high-dose dexamethasone, then adding rituximab and...

Abstract Background Atopic dermatitis (AD) is a prevalent chronic inflammatory and highly pruritic skin condition characterized by the infiltration of immune cells, notably eosinophils mast cells. Mast cells (MCs) critically participate in complex pathogenesis AD through multiple pathways have recently garnered growing attention research. Despite abundance related studies published over years, comprehensive bibliometric analysis on this topic remains lacking. Objective Our objective was to...

Eltrombopag is being investigated for the treatment of aplastic anemia (AA) by stimulating hematopoietic stem cell (HSC) proliferation. To evaluate efficacy and safety eltrombopag in first-line therapy pediatric AA. The present retrospective study assessed patients with newly diagnosed AA administered immunosuppressive (IST) (rabbit ATG combined CSA) at a single center from March to September 2017. All were followed up >2 years. A total 14 (8 males), averagely aged 86 months, enrolled this...

ABSTRACT Importance Immune thrombocytopenic purpura ( ITP ) is the most common bleeding disorder in children. Despite highly spontaneously remission, still almost 20% of cases progress into chronic or refractory , which seriously affects children's quality life. Currently there no method to predict initial stage childhood . Objectives To evaluate platelet‐specific antibodies and compare differences their expression clinical progression. Methods This a single‐center prospective cohort study...

For young patients, HLA-MRD HSCT is the first-line treatment of SAA. However, due to China's birth control policy, few patients could find suitable sibling donors and HLA-MUD. More more transplantation centers have used Haplo-D as donor source for adult pediatric patients. studies with larger amount are rare. We retrospectively analyzed data children AA who were treated allogeneic compared therapeutic efficacy Haplo-HSCT MRD/MUD group. A total 62 enrolled. Implantation was successfully...

Autoimmune lymphoproliferative syndrome (ALPS) usually presents in childhood with fever, nonmalignant splenomegaly, and lymphadenopathy along cytopenia, which is caused by mutations the FAS apoptotic pathway. The TCRαβ + CD4/CD8 double-negative T cells (DNT), one of required criteria ALPS, will rise markedly ALPS. Human Protein kinase C delta (PRKCD) deficiency (OMIM # 615559) was recently identified to be causative for an ALPS-type III significant B-cell proliferation particularly immature...

Objective: To evaluate the efficacy and safety of pulsed high- dose dexamethasone(HDD)treatment in children with primary immune thrombocytopenic purpura(ITP). Method: ITP who failed to first-line therapy from September 2013 2014 were given HDD treatment, dexamethasone was administered at a dosage 0.6 mg ·kg-1·d-1(maximum 40 mg/d)for 4 consecutive days. The cycle repeated every 28 days for 6 months. Results: ①A total 38 cases enrolled, 26 boys 12 girls, median age 54(6-151)months, duration...

Background The autoimmune lymphoproliferative syndrome (ALPS) is a rare disease characterized by defective function of the FAS death receptor, which results in chronic, non-malignant lymphoproliferation and autoimmunity accompanied elevated numbers double-negative (DN) T cells (T-cell receptor α/β + CD4–CD8–) an increased risk developing malignancies later life. Case description Here, we report patient with de novo mutation severe phenotype ALPS-FAS. gene identified as novel spontaneous...

LPS-responsive beige-like anchor (LRBA) deficiency is one of the most common monogenic disorders causing variable immunodeficiency (CVID) and CVID-like disorders. However, clinical spectrum compound heterozygous (CHZ) LRBA variation should be extended. In this study, we presented five cases with various refractory cytopenias.Retrospective analysis manifestations, management, outcomes (from pedigrees) gene CHZ variants which initially manifested as single/multilineage immune cytopenias was...

The management of children with non-severe aplastic anemia (NSAA) is undefined and the efficacies benefits immunosuppressive therapy remain inconsistent. study aimed to investigate efficacy Cyclosporine (CsA) monotherapy for pediatric NSAA.Clinical data NSAA who had been treated CsA at outpatient department Beijing Children's Hospital, Capital Medical University, National Center from January 2017 March 2021 was collected retrospectively. Patients <1 years until end follow-up were excluded....

Abstract Objective To analyze the effects of escalating treatment strategy in children with severe chronic immune thrombocytopenia (SCITP). Methods This was a single‐center, retrospective cohort study. Data from SCITP who received our center were collected between June 2017 and August 2019. The included three steps: Step I (six courses high‐dose dexamethasone [HDD]), II (HDD combined low‐dose rituximab), III (eltrombopag). Results A total 30 cases (18 males 12 females) included, duration...

To describe clinical characteristics and surgical outcomes of pediatric epiretinal membranes (ERMs) without specific etiologies.

MRD-HSCT is the first-line therapy for children with SAA, while it not easy to find a compatible donor due Chinese one-child policy.IST has high recurrence rate, risk of clonal transformation.Thus, Haplo-HSCT, as treatment, gradually attracted clinicians' attention.To evaluate efficacy Haplo-HSCT in we performed retrospective study (2006.06-2021.01) 210 patients AA who received HSCT or IST Beijing Children's Hospital.The OS and FFS rates were analyzed IST.We found that from 2006 2021, 3-and...

Immune thrombocytopenia (ITP) is an autoimmune-mediated hemorrhagic disease. Emerging evidence indicates that

A poor prognosis is associated with atherosclerotic plaque rupture (PR) despite after conventional therapy for patients acute coronary syndrome (ACS). Timely identification of PR improves the risk stratification and ACS patients.

Thrombopoietin (TPO) is the critical regulator of platelet production. However, role TPO in pediatric patients with thrombocytopenic disorders has not been fully elucidated. In present study, we attempted to investigate serum levels acquired aplastic anemia (aAA) and immune thrombocytopenia (ITP). We analyzed endogenous plasma concentration count at time measurement 166 aAA 280 ITP retrospectively. further observed a correlation between counts TPO. Serum were significantly higher compared...

Abstract Background Non‐severe aplastic anemia is more likely to develop into severe anemia, and there no widely accepted treatment plan at present. Hematopoietic stem cell transplantation might be a new therapeutic strategy. Methods Retrospectively analyzed 32 patients with non‐severe who underwent hematopoietic from September 2007 2020, the 5‐year estimated overall survival rate incidence of graft‐versus‐host disease were evaluate efficacy safety in pediatric anemia. Results Thirty‐two...

Objective This research aimed to assess the effect of Wuzhi capsules (WZC) on blood concentration cyclosporine A (CsA) in renal aplastic anemia recipients.Methods observational study was carried out at Hematology Oncology Center, Beijing Children's Hospital between November 2019 and February 2020. total 102 Chinese AA recipients receiving CsA (6 mg/kg/d) with or without WZC were included this study. Baseline data, such as age, therapeutic drug monitoring follow-up information collected. The...