Unexpectedly High Prevalence of Low Alpha-Galactosidase A Enzyme Activity in Patients with Focal Segmental Glomerulosclerosis

Hasbal N. B., Caglayan F. B., Sakaci T., Ahbap E., KOÇ Y., Sevinc M., ...Daha Fazla

CLINICS, cilt.75, 2020 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 75
  • Basım Tarihi: 2020
  • Doi Numarası: 10.6061/clinics/2020/e1811
  • Dergi Adı: CLINICS
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, EMBASE, MEDLINE, Directory of Open Access Journals
  • Sivas Cumhuriyet Üniversitesi Adresli: Evet

Özet

OBJECTIVES: Fabry disease (FD) is a rare disease associated with sphingolipid accumulation. Sphingolipids are components of plasma membranes that are important in podocyte function and accumulate in various glomerular diseases such as focal segmental glomerulosclerosis (FSGS). Both FD and FSGS can cause podocyte damage and are classified as podocytopathies. In this respect, FD and FSGS share the same pathophysiologic pathways. Previous screening studies have shown that a significant proportion of end-stage renal disease (ESRD) patients receiving hemodialysis (HD) have unsuspected FD, and the prevalence of low alpha-galactosidase A (aGLA) enzyme activity in these patients is higher than that in the normal population. We aimed to compare aGLA enzyme activity in patients with biopsy-proven FSGS and ESRD receiving HD.