Diagnosis of congenital and infantile nephrotic syndromes in renal biopsies in Minas Gerais, Brazil: Six case reports.
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.
a General Pathology Division, Biological and Natural Sciences Institute , Federal University of Triângulo Mineiro , Uberaba , Minas Gerais , Brazil.
Laura Penna
LP
Rocha
a General Pathology Division, Biological and Natural Sciences Institute , Federal University of Triângulo Mineiro , Uberaba , Minas Gerais , Brazil.
Fabiano Bichuette
FB
Custódio
b General Pathology Division, Institute of Tropical Pathology and Public Health , Federal University of Goiás , Goiânia , Goiás , Brazil.
Juliana Reis
JR
Machado
a General Pathology Division, Biological and Natural Sciences Institute , Federal University of Triângulo Mineiro , Uberaba , Minas Gerais , Brazil.
Lívia Helena
LH
de Morais Pereira
a General Pathology Division, Biological and Natural Sciences Institute , Federal University of Triângulo Mineiro , Uberaba , Minas Gerais , Brazil.
Maria Luiza Gonçalves Dos Reis
ML
Monteiro
a General Pathology Division, Biological and Natural Sciences Institute , Federal University of Triângulo Mineiro , Uberaba , Minas Gerais , Brazil.
Valéria Lima
VL
Laterza
a General Pathology Division, Biological and Natural Sciences Institute , Federal University of Triângulo Mineiro , Uberaba , Minas Gerais , Brazil.
Marlene Antônia
MA
Dos Reis
a General Pathology Division, Biological and Natural Sciences Institute , Federal University of Triângulo Mineiro , Uberaba , Minas Gerais , Brazil.
Rosana Rosa Miranda
RR
Corrêa
D001706:Biopsy; D001938:Brazil; D006801:Humans; D007674:Kidney Diseases; D007678:Kidney Glomerulus; D009404:Nephrotic Syndrome
Congenital and infantile nephrotic syndromes; Denys–Drashsyndrome; congenital nephroticsyndrome of the Finnish type; diffuse mesangial sclerosis