title: Hemodynamic maladjustment and disease progression in nephrosis with FSGS.
abstract: Idiopathic nephrotic syndrome (NS) associated with focal segmental glomerulosclerosis (FSGS) and severe renal function impairment is usually refractory to the conventional treatment and progresses to end-stage renal disease. Herein, we reported 10 patients with NS-FSGS who had initially had CCr 34 +/- 12 mL/min/1.73 m2 (normal 120 mL/min/1.73 m2), FE Mg 7.8 +/- 2.6% (normal 2.2%), 24-h urinary protein 3.1 g (normal <200 mg) and been followed up for over 10 years. The initial intrarenal hemodynamic study revealed a marked elevation of efferent arteriolar resistance (RE 17289 +/- 8636 dyne x s x cm(-5); normal 3000 dyne x s x cm(-5)), intraglomerular hypertension (PG 57 +/- 1 mm Hg; normal 52 mm Hg), hyperfiltration (FF 0.24; normal 0.2), marked reductions in GFR 35 +/- 17 mL/min/1.73 m2, renal plasma flow (RPF 159 +/- 61 mL/min/1.73 m2; normal 600 mL/min/1.73 m2) and peritubular capillary flow (PTCF 123 +/- 57 mL/min/1.73 m2; normal 480 mL/min/1.73 m2). Such a hemodynamic alteration indicated a hemodynamic maladjustment with a preferential constriction at RE. Treatment consists of multidrugs, namely angiotensin converting enzyme inhibitor, calcium channel blocker, antiplatelet and anticoagulant, with or without angiotensin II receptor antagonist. Following the treatment, correction of hemodynamic maladjustment has been achieved which is characterized by reductions in RE 6046 +/- 2191 dyne x s x cm(-5), PG 52 +/- mm Hg, FF 0.19 +/- 0.1 and increments in RPF 341 +/- 118 mL/min/1.73 m2, PTCF 280 +/- 106 mL/min/1.73 m2 and GFR 64 +/- 17 mL/min/1.73 m2. Coinciding with hemodynamic improvement, there has been a steadily increased creatinine clearance and improvement in FE Mg 4.3 +/- 2.6% and suppression of proteinuria 0.29 +/- 0.4 g/24 h after the period of follow-up of greater than 10 years.
authors.affiliation: Faculty of Medicine, King Chulalongkorn Memorial Hospital, Bangkok, Thailand. fmednft@md2.md.chula.ac.th
authors.forename: Narisa
authors.initials: N
authors.lastname: Futrakul
authors.forename: Prasong
authors.initials: P
authors.lastname: Siriviriyakul
authors.forename: Tawatchai
authors.initials: T
authors.lastname: Deekajorndej
authors.forename: Prasit
authors.initials: P
authors.lastname: Futrakul
mesh_terms: D018450:Disease Progression; D005500:Follow-Up Studies; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology; D006439:Hemodynamics / Q000502:physiology*; D006801:Humans; D007677:Kidney Function Tests; D009404:Nephrotic Syndrome / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology; D013997:Time Factors; D014665:Vasodilator Agents / Q000627:therapeutic use
chemical_list: D014665:Vasodilator Agents