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1.
Dis Markers ; 2019: 1070495, 2019.
Article in English | MEDLINE | ID: mdl-31191741

ABSTRACT

Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are primary glomerulopathies leading to proteinuria, known as podocytopathies, which share syndromic and morphological similarities. Morphological similarity occurs in cases of FSGS in which the sclerotic lesion was not sampled in renal biopsy, due to the focal nature of the disease. Differentiating these entities is very important, especially in cases of suspected FSGS but with sclerotic lesion not sampled, as they are diseases that apparently have different pathogenic mechanisms and prognosis. The difference in uPAR expression in situ among these two entities may be related to a distinct molecular mechanism involved in pathogenesis. Thus, finding biomarkers involved in the pathogenesis and that can also help in differential diagnosis is very relevant. The aim of this work was to evaluate the potential of urokinase-type plasminogen activator receptor (uPAR) as a biomarker in renal biopsies of patients with podocytopathies (n = 38). Immunohistochemistry showed that FSGS (n = 22) had increased uPAR expression in podocytes compared with both the MCD group (n = 16; p = 0.0368) and control group (n = 21; p = 0.0076). ROC curve (p = 0.008) showed that this biomarker has 80.95% of specificity in biopsies of patients with FSGS. Therefore, uPAR presented a high specificity in cases of podocytopathies associated with sclerosis and it can be considered a potential biomarker for FSGS.


Subject(s)
Glomerulosclerosis, Focal Segmental/metabolism , Kidney/metabolism , Receptors, Urokinase Plasminogen Activator/metabolism , Adolescent , Adult , Aged , Biomarkers/metabolism , Female , Glomerulosclerosis, Focal Segmental/pathology , Humans , Kidney/pathology , Male , Middle Aged , Podocytes/metabolism , Receptors, Urokinase Plasminogen Activator/genetics , Sensitivity and Specificity
2.
Ultrastruct Pathol ; 40(6): 311-316, 2016.
Article in English | MEDLINE | ID: mdl-27669128

ABSTRACT

Congenital or infantile nephrotic syndromes (CNS/INS) correspond to a heterogeneous group of rare diseases in which glomerular renal dysfunction and proteinuria are prominent. The aim of this study is to present six cases of possible CNS/INS with diagnoses based on clinical findings and especially histological, ultrastructural, and immunohistochemical characteristics of renal biopsies. Four cases are presented with diffuse mesangial sclerosis, one of them possibly part of Denys-Drash syndrome and two cases with CNS probably of the Finnish type in patients between 3 months old and 13 years old. The study focuses on the late evolution of Denys-Drash syndrome to end-stage renal disease in a 13-year-old patient and the diagnosis of diffuse mesangial sclerosis in an 8-year-old patient. Thus, it contributes to a better epidemiological characterization of these syndromes, demonstrating cases of CNS/INS in infrequent age groups.


Subject(s)
Nephrotic Syndrome , Biopsy , Brazil , Humans , Kidney Diseases , Kidney Glomerulus
3.
Fetal Pediatr Pathol ; 31(1): 48-54, 2013 Feb.
Article in English | MEDLINE | ID: mdl-22515549

ABSTRACT

Markers of fetal inflammatory response syndrome (FIRS) can influence the morphologic alterations in liver of autopsied neonates. The IL-6, TNF-α, and C-reactive protein (CRP) expression in liver fragments were marked by immunohistochemistry and the intensity of steatosis, percentage of fibrosis, and the number of foci of extramedullary erythropoiesis were evaluated. The degree of steatosis correlated positively with IL-6 (p = 0.06), positively with CRP (p ≤ 0.001), and negatively with TNF-α (p = 0.06). The collagen percentage correlated positively with IL-6 (p = 0.055) and positively with TNF-α (p ≤ 0.001). Erythropoiesis correlated positively with IL-6 (p ≤ 0.001) and negatively with CRP (p = 0.00754). The analyzed markers of FIRS have an important role in triggering hepatic morphologic alterations.


Subject(s)
Infant, Newborn, Diseases/metabolism , Infant, Newborn, Diseases/pathology , Liver/metabolism , Liver/pathology , Autopsy , Biomarkers/analysis , C-Reactive Protein/analysis , C-Reactive Protein/biosynthesis , Female , Humans , Infant, Newborn , Interleukin-6/analysis , Interleukin-6/biosynthesis , Male , Pregnancy , Pregnancy Complications/metabolism , Pregnancy Complications/pathology , Syndrome , Tumor Necrosis Factor-alpha/analysis , Tumor Necrosis Factor-alpha/biosynthesis
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