ABSTRACT
Venous thromboembolism (VTE) in children is a rare condition. An increased incidence has been observed in the last few years due to several factors, such as increased survival in chronic conditions, especially chronic kidney disease (CKD), use of catheters, and increased sensitivity of diagnostic tools. VTE includes deep vein thrombosis (DVT) and pulmonary embolism (PE). VTE in children is associated with a two to six times higher mortality risk and a 5-10% prevalence of post-thrombotic syndrome. Overall, 5% of VTE episodes in children are associated with chronic kidney disease. The etiology of VTE in chronic kidney disease covers a wide range of pathologies. Various types of thrombotic complications may occur during long-term use of a chronic dialysis catheter. VTE occurs in 3% of children with nephrotic syndrome (NS). The risks for VTE and arterial thromboembolism (ATE) were particularly high in the first 6 months after the onset of NS. Other causes of VTE are graft rejection due to thrombosis of vascular anastomoses after kidney transplantation (3%) and autoimmune diseases (lupus nephritis, antiphospholipid syndrome). In this state-of-the-art overview, we have reviewed the physiologic and pathologic mechanisms underlying pediatric thrombosis and updated current diagnostic and treatment options, emphasizing personal experience as well.
ABSTRACT
Background: Nephrotic syndrome (NS) is the term used for the association of edema and massive proteinuria. From a therapeutic point of view, it is important to distinguish between primitive and secondary kidney damage. The clinical evolution, prognosis and therapeutic response in the NS in children are directly determined by the anatomopathological aspect. Steroid resistant nephrotic syndrome was diagnosed in patients with idiopathic NS based on lack of complete remission despite treatment with steroids. Purpose: To analyse the anatomopathological aspects of steroid resistant nephrotic syndrome (SRNS) and their correlation with evolution. Materials and Methods: We made a retrospective study with the aim to analyze the anatomo-pathological aspects and their correlations with evolution in 68 cases of steroid resistant nephrotic syndrome (SRNS) hospitalized in the Pediatric Nephrology Department in Iasi, Romania. We defined SRNS in all cases without response to corticosteroids after the first month of therapy. For all the cases selected, the period of follow-up was the minimal 6 months. Results and Discussions: A 36% case of nephrotic syndrome was corticoresistant, with the mean age at onset of patients with SRNS being 9.18 years, compared to KDIGO studies in which the corticosteroid resistance is 10-20%. Renal biopsy was performed in 80.88% children with SRNS and was allowed the evaluation of the activity and chronicity index. Total remission was obtained in 44.01% children with SRNS. The correlation of the anatomopathological aspects with the evolution is not statistically significant (p = 0.76), observing different therapeutic responses to all the analyzed histological types. Conclusion: Almost half of NS in children are cortico resistant. Remission was obtained in 44% of cases of SRNS. Predicting the response to long-term treatment in SRNS is difficult using only renal biopsy; it is necessary to introduce genetic molecular analyses to establish a judicious therapeutic attitude.
