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1.
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
2.
Ann Diagn Pathol ; 17(1): 22-7, 2013 Feb.
Article in English | MEDLINE | ID: mdl-22658343

ABSTRACT

Renal biopsy is an important method of diagnosis and prognosis in children and adolescents with renal diseases, and there are few studies describing the histopathologic alterations in renal biopsies in these age groups. The aim of this study was to evaluate the incidence of morphologic alterations described in renal biopsies carried out in children and adolescents. Patients aged between 1 month and 18 years were observed from 1996 to 2010 and were separated into 3 age groups: 0 to 6 (group 1, n = 29), 6 to 12 (group 2, n = 31), and 13 to 18 (group 3, n = 77) years. Morphologic alterations were evaluated according to light microscopy, immunofluorescence, and electron microscopy findings. The most common glomerulopathies observed in these different age groups were as follows: group 1-podocytopathy (34.78%), hereditary proteinurias 5 (21.73%), lupus nephritis (13.04%), and Berger disease (8.69%); group 2-podocytopathy (44.44%), acute diffuse glomerulonephritis (22.22%), Berger disease (11.11%), and Alport syndrome or thin membrane disease (11.11%); and group 3-lupus nephritis (22.85%), podocytopathy (20.00%), Berger disease (15.71%), and membranous glomerulopathies (11.42%). This study allows for better knowledge of the prevalence of nephropathies in children and adolescents and shows that a well-supported early diagnosis is indispensable for a more adequate treatment of patients with renal diseases.


Subject(s)
Kidney Diseases/epidemiology , Kidney Diseases/pathology , Kidney/pathology , Adolescent , Biopsy , Brazil/epidemiology , Child , Child, Preschool , Female , Glomerulonephritis, IGA/epidemiology , Glomerulonephritis, IGA/ethnology , Glomerulonephritis, IGA/pathology , Humans , Infant , Kidney Diseases/ethnology , Lupus Nephritis/epidemiology , Lupus Nephritis/ethnology , Lupus Nephritis/pathology , Male , Prevalence , Proteinuria/epidemiology , Proteinuria/ethnology , Proteinuria/pathology , Retrospective Studies
3.
Case Rep Med ; 2012: 283948, 2012.
Article in English | MEDLINE | ID: mdl-22567015

ABSTRACT

Ovarian fibroma is the commonest benign tumor of the ovarian stroma. The cellular subtype accounts for around 10% of ovarian fibromatous tumors. The cellular fibroma is a tumor of uncertain malignant potential that may recur or be associated with peritoneal implants. Usually these are solid tumors, sometimes with small areas of cystic degeneration. This case is reported to highlight an unusual feature for an ovarian fibroma: the tumor was predominantly cystic with a small solid part; the multiple cavities contents consisted of viscous liquid that solidified under room temperature. The multiloculated cysts, the mucinous contents, and the solid areas simulated a borderline mucinous ovarian tumor on both CT scan and gross pathologic examination.

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