Pathology of Podocytopathies Causing Nephrotic Syndrome in Children
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Ranganathan S
Front Pediatr
2016[]; 4
(?): 32
PMID27066465
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Nephrotic syndrome (NS) in children includes a diverse group of diseases that
range from genetic diseases without any immunological defects to causes that are
primarily due to immunological effects. Recent advances in molecular and genomic
studies have resulted in a plethora of genetic defects that have been localized
to the podocyte, the basic structure that is instrumental in normal filtration
process. Although the disease can manifest from birth and into adulthood, the
primary focus of this review would be to describe the novel genes and pathology
of primary podocyte defects that cause NS in children. This review will restrict
itself to the pathology of congenital NS, minimal change disease (MCD), and its
variants and focal segmental glomerulosclerosis (FSGS). The two major types of
congenital NS are Finnish type characterized by dilated sausage shaped tubules
morphologically and diffuse mesangial sclerosis characterized by
glomerulosclerosis. MCD has usually normal appearing biopsy features on light
microscopy and needs electron microscopy for diagnosis, whereas FSGS in contrast
has classic segmental sclerosing lesions identified in different portions of the
glomeruli and tubular atrophy. This review summarizes the pathological
characteristics of these conditions and also delves into the various genetic
defects that have been described as the cause of these primary podocytopathies.
Other secondary causes of NS in children, such as membranoproliferative and
membranous glomerulonephritis, will not be covered in this review.
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