Focal segmental glomerulosclerosis

Definition

Focal segmental glomerulosclerosis (FSGS) is a clinicopathological syndrome or diagnostic term for glomerular injury characterized by the partial scarring of glomeruli. Clinical features include proteinuria, reduced glomerular filtration rate and oedema. FSGS is an important cause of renal failure in adults and nephrotic syndrome in children.

Latest Research and Reviews

  • Research |

    Martin Zenker, Corinne Antignac, Friedhelm Hildebrandt and colleagues report that mutations in OSGEP, TP53RK, TPRKB and LAGE3, genes encoding KEOPS-complex subunits, cause Galloway–Mowat syndrome, a recessive disease characterized by early-onset steroid-resistant nephrotic syndrome and microcephaly. Functional studies suggest that the phenotypes result from impaired protein translation, thus leading to endoplasmic reticulum stress and apoptosis.

    • Daniela A Braun
    • , Jia Rao
    • , Geraldine Mollet
    • , David Schapiro
    • , Marie-Claire Daugeron
    • , Weizhen Tan
    • , Olivier Gribouval
    • , Olivia Boyer
    • , Patrick Revy
    • , Tilman Jobst-Schwan
    • , Johanna Magdalena Schmidt
    • , Jennifer A Lawson
    • , Denny Schanze
    • , Shazia Ashraf
    • , Jeremy F P Ullmann
    • , Charlotte A Hoogstraten
    • , Nathalie Boddaert
    • , Bruno Collinet
    • , Gaëlle Martin
    • , Dominique Liger
    • , Svjetlana Lovric
    • , Monica Furlano
    • , I Chiara Guerrera
    • , Oraly Sanchez-Ferras
    • , Jennifer F Hu
    • , Anne-Claire Boschat
    • , Sylvia Sanquer
    • , Björn Menten
    • , Sarah Vergult
    • , Nina De Rocker
    • , Merlin Airik
    • , Tobias Hermle
    • , Shirlee Shril
    • , Eugen Widmeier
    • , Heon Yung Gee
    • , Won-Il Choi
    • , Carolin E Sadowski
    • , Werner L Pabst
    • , Jillian K Warejko
    • , Ankana Daga
    • , Tamara Basta
    • , Verena Matejas
    • , Karin Scharmann
    • , Sandra D Kienast
    • , Babak Behnam
    • , Brendan Beeson
    • , Amber Begtrup
    • , Malcolm Bruce
    • , Gaik-Siew Ch'ng
    • , Shuan-Pei Lin
    • , Jui-Hsing Chang
    • , Chao-Huei Chen
    • , Megan T Cho
    • , Patrick M Gaffney
    • , Patrick E Gipson
    • , Chyong-Hsin Hsu
    • , Jameela A Kari
    • , Yu-Yuan Ke
    • , Cathy Kiraly-Borri
    • , Wai-ming Lai
    • , Emmanuelle Lemyre
    • , Rebecca Okashah Littlejohn
    • , Amira Masri
    • , Mastaneh Moghtaderi
    • , Kazuyuki Nakamura
    • , Fatih Ozaltin
    • , Marleen Praet
    • , Chitra Prasad
    • , Agnieszka Prytula
    • , Elizabeth R Roeder
    • , Patrick Rump
    • , Rhonda E Schnur
    • , Takashi Shiihara
    • , Manish D Sinha
    • , Neveen A Soliman
    • , Kenza Soulami
    • , David A Sweetser
    • , Wen-Hui Tsai
    • , Jeng-Daw Tsai
    • , Rezan Topaloglu
    • , Udo Vester
    • , David H Viskochil
    • , Nithiwat Vatanavicharn
    • , Jessica L Waxler
    • , Klaas J Wierenga
    • , Matthias T F Wolf
    • , Sik-Nin Wong
    • , Sebastian A Leidel
    • , Gessica Truglio
    • , Peter C Dedon
    • , Annapurna Poduri
    • , Shrikant Mane
    • , Richard P Lifton
    • , Maxime Bouchard
    • , Peter Kannu
    • , David Chitayat
    • , Daniella Magen
    • , Bert Callewaert
    • , Herman van Tilbeurgh
    • , Martin Zenker
    • , Friedhelm Hildebrandt
    •  & Corinne Antignac
  • Reviews |

    The incidence of obesity-related glomerulopathy (ORG) — a distinct entity featuring proteinuria, glomerulomegaly, progressive glomerulosclerosis and renal functional decline — is increasing in parallel with the obesity epidemic. Here, Vivette D'Agati and colleagues review the pathology, clinical features, treatment and pathogenesis of ORG.

    • Vivette D. D'Agati
    • , Avry Chagnac
    • , Aiko P.J. de Vries
    • , Moshe Levi
    • , Esteban Porrini
    • , Michal Herman-Edelstein
    •  & Manuel Praga

News and Comment