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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?     ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​     ​   ​   ​ ​  ...

Diagnose This (April 23, 2018)

What is your diagnosis?   ​   ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​     ​   ​   ​ ​...

FSGS Types

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)

The immunofluorescence study is negative. What is your diagnosis?   ​   ​ ​   ​   ​ ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​   ​ ​   ​ ​   ​ ​   ​ ​     ​...

Foot Process Effacement and 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

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...

FSGS Tip Lesion – Secondary

The patient is a 64-year-old male with a history of diabetes mellitus, hypertension, and hyperlipidemia, who presents with an increased creatinine of 1.8 mg/dl and proteinuria (UPCR 2.5 g/g). Serologic workup is completely negative. A renal biopsy was performed and shows nodular glomerulosclerosis (Fig 1) and arteriolar hyalinosis characteristic of diabetic glomerulopathy. Additionally, the glomeruli focally show areas of segmental glomerulosclerosis associated with endocapillary foam cells. These lesions are located at the take-off point of the proximal tubule (Fig 1) and reminiscent of the glomerular lesions seen in the tip variant of FSGS. Electron microscopy shows mesangial matrix expansion, mostly...