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Currently filtering by tag: FSGS

Twitter Poll (August 5, 2020)

Nail-Patella syndrome, Twitter Poll, Arkana Laboratories
Answer: A, Autosomal recessive inheritance Nail-Patella syndrome is a rare autosomal dominant disease with an incidence of approximately 1/50,000. The majority of cases are due to a mutation in LMX1B located at chromosome 9q34.1. The gene is important in development as it encodes a transcription factor important in dorsoventral pattern limb development, proper development of the anterior eye, neuron development within the CNS, and podocyte differentiation and maintenance. The kidney pathology is rather non-specific and can show minimal glomerular changes to variable degrees of FSGS with thickened capillary loops. By EM, the classic finding is that of "Moth-eaten" glomerular basement...

Diagnose This (March 25, 2019)

Nephrotic Syndrome, Arkana Laboratories, renal pathology, kidney injury
What is your diagnosis?     ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​     ​   ​   ​ ​  ...

Membranous with FSGS

Some patients with membranous glomerulopathy also have associated focal segmental glomerulosclerosis (FSGS) lesions. The glomerulus in this biopsy image, for example, shows a prominent area of segmental sclerosis surrounded by mildly thickened capillary loops which have granular IgG deposits by immunofluorescence (see inset). As in other renal diseases, studies have linked the presence of FSGS lesions in patients with membranous glomerulopathy to more severe disease (e.g. higher levels of proteinuria).   Dumoulin A, Hill GS, Montseny JJ, Meyrier A. Clinical and morphological prognostic factors in membranous nephropathy: significance of focal segmental glomerulosclerosis. Am J Kidney Dis. 2003 Jan;41(1):38-48. PMID: 12500220.

Diagnose This (April 23, 2018)

Fabry disease
What is your diagnosis?   ​   ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​     ​   ​   ​ ​...

FSGS Types

Focal segmental glomerulosclerosis (FSGS)
Are there clues to help distinguish among primary, genetic, and secondary focal segmental glomerulosclerosis (FSGS) lesions?  Focal segmental glomerulosclerosis (FSGS) (Fig. 1) represents a morphologic pattern of injury rather than a specific disease.  Current thinking on this important subject is thoroughly presented in a recent review by De Vriese AS, Sethi S, et al (J Am Soc Nephrol 29: 759–774, 2018).   The authors discuss the three main etiologic categories of disease associated with FSGS lesions:  primary FSGS, genetic FSGS, and secondary (“maladaptive”) type FSGS.  The authors also emphasize the importance of determining whether the patient has nephrotic syndrome (defined by...

Diagnose This (April 16, 2018)

secondary focal segmental glomerulosclerosis
The immunofluorescence study is negative. What is your diagnosis?   ​   ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​     ​...

Foot Process Effacement and Focal Segmental Glomerulosclerosis

Focal Segmental Glomerulosclerosis
Knowing whether a patient has clinical nephrotic syndrome and knowing the degree of podocyte foot process effacement can be helpful diagnostic clues in separating “primary” from “secondary” forms of focal segmental glomerulosclerosis (FSGS). For example, in a patient with the nephrotic syndrome whose biopsy shows FSGS lesions and no significant immune deposits by immunofluorescence, the presence of global effacement of podocyte foot processes by electron microscopy (as seen in Fig 1) provides support for a “primary” FSGS (see reference). Sethi S et al. Focal and segmental glomerulosclerosis: clinical and kidney biopsy correlations. Clin Kidney J. 2014 Dec;7(6):531-7.PMID: 25503953.

Focal Segmental Glomerulosclerosis Not Otherwise Specified

FSGS, focal segmental glomerulosclerosis
Focal segmental glomerulosclerosis (FSGS) is a nonspecific pattern of glomerular scarring in which a subset of the glomeruli show partial involvement of the glomerular tuft. It should not be confused with a specific disease. The differential diagnosis is too long to include here but includes genetic etiologies, primary injury to podocytes, and adaptive responses to diseases such as hypertension and obesity. Clinicopathologic correlation can be useful to identify patients with primary FSGS. Specifically, primary FSGS is more likely if >80% foot process effacement is present by electron microscopy and the clinical evaluation reveals nephrotic syndrome (not just nephrotic-range proteinuria) including...