Primary FSGS |
Circulating podocyte-toxic factor(s) |
Secondary FSGS |
Maladaptive response to hyperfiltration |
Reduced nephron mass |
Unilateral renal agenesis |
Oligomeganephronia |
Severe obesity |
Low birth weight |
Drugs and toxins |
Heroin |
Interferon (alpha, beta, gamma) |
Bisphosphonates (eg, pamidronate) |
Anabolic steroids |
mTOR inhibitors |
Calcineurin inhibitors |
Anthracyclines |
Lithium |
Viruses |
HIV |
Parvovirus B19 |
Simian virus 40 |
Cytomegalovirus |
Epstein-Barr virus |
Other causes |
Healing phase of focal proliferative glomerulonephritis (ANCA-associated vasculitis, IgA nephropathy, lupus nephritis), membranous nephropathy |
Sickle cell anemia |
Thrombotic microangiopathy |
Fabry disease |
Glucose-6-phosphatase deficiency (glycogen storage disease I, von Gierke disease) |
Hemophagocytic syndrome |
Fibrillary glomerulonephritis |
Genetic FSGS (kidney limited and syndromic) |
Mutations in slit diaphragm complex genes: NPHS1* (nephrin), NPHS2* (podocin), CD2AP*¶, TRPC6¶, PTPRO* (GLEPP1), MYO1E* |
Mutations in cytoskeleton genes: ACTN4¶, MYO1E*, INF2¶, ARHGAP24¶, ARHGDIA*, MYH9¶ (Epstein syndrome, Fechtner syndrome), ITGB4* |
Mutations in cell matrix genes: COL4A3, COL4A4, COL4A5Δ, LAMB2* (Pierson syndrome) |
Mutations in genes involved in DNA repair, transcription, or nuclear transport: WT1¶ (Denys-Drash syndrome, Frasier syndrome), NUP93, XPO5 (exportin 5), PAX2, LMX1B* (Nail-patella syndrome), WDR73 (Galloway-Mowat syndrome, nephro-cerebellar syndrome), LMNA (partial lipodystrophy), SMARCAL1* |
Mutations in genes involved in cell signaling: PLCE1*, TRPC6¶, KANK4 |
Mutations in lysosome genes: SCARB2* (action myoclonus) |
Mutations in cilia genes: TTC21B |
FSGS: focal segmental glomerulosclerosis; mTOR: mammalian (mechanistic) target of rapamycin; ANCA: antineutrophil cytoplasmic autoantibodies; IgA: immunoglobulin A.
* Pattern of inheritance in the most common genetic forms: Recessive.
¶ Pattern of inheritance in the most common genetic forms: Autosomal dominant.
Δ Pattern of inheritance in the most common genetic forms: Monogenic (X-linked/autosomal) and digenic inheritance described.آیا می خواهید مدیلیب را به صفحه اصلی خود اضافه کنید؟