Background Immune thrombocytopenia (ITP) is the most common etiology of acquired thrombocytopenia diseases in children. ITP is characterized by the immune-mediated decreased formation and excessive destruction of platelets. The pathogenesis and management of pediatric ITP are distinct from adult ITP. A disintegrin and metalloproteinase 17 (ADAM17) mediates the shedding of platelet receptor glycoprotein Ib α (GPIb α) in extracellular domain, functioning in the platelet activation and clearance. Our study aims to probe the roles and mechanisms of ADAM17 in pediatric ITP. Methods The differently expressed ADAM17 in megakaryocytes was obtained from children with ITP through the next-generation RNA-Sequence. Hematoxylin-eosin and Giemsa staining were performed for cell morphology identification. Flow cytometry was applied to assess autoantibodies against platelets, subtypes of lymphocytes, the surface expression level of ADAM17 and polyploidization of megakaryocytes, as well as the full-length GP Ib α. Results ADAM17 was significantly downregulated in megakaryocytes and platelets in children with ITP. Higher values of PDW and positive autoantibodies presence were observed in children with ITP. Loss of ADAM17 in mice led to defects in proplatelet formation and significantly elevated expression of phosphorylated myosin light chain (p-MLC) in megakaryocytes. Conclusions Our study indicated that the downregulation of ADAM17 might be an innate cause of inefficient platelet production in pediatric ITP.

The clinical use of the cardiac troponin T (cTnT) assay was limited to the adult population in the diagnosis and prognosis of myocardial injury. However, emerging studies indicated its significant value in the assessment of pediatric cardiology, and it has been routinely measured in most hospitals. Our study investigated the normative values of cTnT in Chinese children and reported the age-specific 99th percentile cut-off for them.A total of 1280 apparently healthy Chinese children were enrolled in our study. Serum levels of cTnT were analyzed on the Roche Elecsys Troponin T Gen 5 STAT assay. According to the Clinical and Laboratory Standards Institute C28-A3 guideline, the 99th percentile upper reference limits (URLs) with 90% confidence intervals (CIs) were calculated in different age subgroups.The 99th percentile URL was 38 (90%CI: 37.0-51.0) ng/L for 1 to <4 months old, 26 (90%CI: 25.2-28.5) ng/L for 4 to 12 months old, and 12 (90%CI: 11.1-12.9) ng/L for 1 to 18 years old, respectively. For subjects aged from 1 to 18 years, boys had slightly higher cTnT levels than girls (P = .003), while our assay could not measure low cTnT concentrations (≥the limit of detection) in 50% girls.Our study provided age-specific URLs of cTnT for Chinese children, with the 5th generation cTnT assay from Roche Diagnostics. It had significant clinical implications in the interpretation and use of test results for pediatric cardiology.