Resumen de CD80, suPAR and Nephrotic Syndrome in a case of NPHS2 mutation

G. Cara Fuentes, Carlos Araya, Changli Wei, Christopher Rivard, Takuji Ishimoto, Jochen Reiser, Richard J. Johnson, Eduardo H. Garin

• Background:

  Podocin mutations are characterized by progres - sion to end stage renal disease and histologic findings of Fo - cal Segmental Glomerulosclerosis (FSGS). CD80 is a podocytes protein that may play a role in proteinuria, particularly in Minimal Change Disease whereas the soluble urokinase recep - tor (suPAR) is characteristically elevated in the serum of FSGS patients.

  Methods:

  In a patient with nephrotic syndrome and podocin mutation, urinary and serum CD80 as well as suPAR were measured using commercially available kits. Urinary CD80 molecular size was determined by western blot analysis. Glo - merular staining for CD80 and podocin was performed.

  Results:

  Patient displayed marked elevated CD80 and mildly increased suPAR urinary levels compared to controls. Serum CD80 level was within the range observed in normal controls. Serum su - PAR level was elevated, albeit in the lower range reported for patients with primary FSGS. Immunofluorescence examination of kidney biopsy revealed glomerular CD80 expression.

  Conclu - sion:

  The combination of serum and urinary biomarkers can help differentiate various forms of FSGS. High urinary CD80 and elevated serum and urinary suPAR might represent a profile to differentiate this genetic form of FSGS from primary FSGS.