Associations between TGF-ß1 Levels and Markers of Hemolysis, Inflammation, and Tissue Remodeling in Pediatric Sickle Cell Patients.

Transforming growth factor beta (TGF-ß) is a cytokine with important involvement in biological processes related to the pathogenesis of sickle cell disease (SCD), including endothelial and vascular dysfunction, inflammation, and hematopoietic homeostasis. This study is aimed at investigating associations between levels of TGF-ß1 and classical laboratory biomarkers and inflammatory mediators, as well as the tissue inhibitor of metalloproteases-1 (TIMP-1) and matrix metalloproteinase-9 (MMP-9), in pediatric patients (n = 123) with SCD in steady state: 84 with sickle cell anemia (HbSS) and 39 with hemoglobin SC disease (HbSC). A healthy control (HC) group of 59 individuals was also included. Hematological and biochemical analyses were carried out using electronic methods. TGF-ß1, TIMP-1, and MMP-9 plasma quantifications were performed by ELISA. TGF-ß1 plasma levels were higher in HbSS individuals than in HbSC and HC. In individuals with HbSS, TGF-ß1 levels were positively correlated with red blood cells, hemoglobin, hematocrit, platelets, and TIMP-1. In addition, HbSS individuals with TGF-ß1 levels above the median (≥72.29 ng/mL) also presented increased monocyte counts and decreased albumin levels. In patients with HbSC, TGF-ß1 levels were positively correlated with leukocytes, eosinophils, lymphocytes, monocytes, and platelets, as well as levels of TIMP-1, VLDL-C, triglycerides, heme, and AST. Additionally, HbSC individuals with TGF-ß1 levels above the median (≥47.80 ng/mL) presented increased leukocyte and platelet counts, as well as increased levels of triglycerides, VLDL-C, MMP-9, and TIMP-1, and decreased HDL-C. Our findings suggest that TGF-ß1 may play important roles in vascular remodeling, vasculopathy, angiogenesis, and inflammation in pediatric patients with SCD.

Distúrbios da hemostasia em crianças portadoras de cardiopatias congênitas: [revisão] / Hemostatic defects in children with congenital heart disease: [review]

As cardiopatias congênitas apresentam elevada prevalência no Brasil, sendo responsáveis por morbi-mortalidade importante, principalmente em menores de 5 anos de idade. Relacionam-se a um perfil complexo de alterações hemostáticas, predispondo tanto a eventos trombóticos como hemorrágicos, e cujo conhecimento é fundamental para o manejo adequado de cada paciente. O presente artigo objetiva descrever as alterações da coagulação relacionadas às cardiopatias congênitas, cianogênicas ou não, em menores de 18 anos de idade, submetidos ou não a procedimentos cirúrgicos.

There is a high prevalence of congenital heart diseases in Brazil, which are responsible for high morbidity and mortality, mainly in under 5-year-old children. They are related to a complex profile of hemostatic disorders that lead to thrombotic and hemorrhagic events. Thus knowledge of the diseases is imperative for the adequate management of each patient. The objective of this article is to describe coagulation abnormalities related to congenital cyanotic and non-cyanotic heart diseases, in children, undergoing surgical procedures or not.