Prognosis of the Nephrotic Syndrome in Sickle Glomerulopathy

Abstract

Of 240 adults with sickle cell anemia seen over 11 years, 12 had the nephrotic syndrome. In 9 (75%) the glomerular lesion, sickle glomerulopathy, consisted of mesangial expansion and basement membrane duplication. Six patients had type IV renal tubular acidosis. Four of the 9 Patients died within 24 months (17 ± 5; mean ± SD), while 5 survived 36 months or longer (80 ± 49); no significant differences were seen between the former and the latter in age, admission serum creatinine and C3 levels, urinary protein excretion, or the frequency of renal tubular acidosis. Chronic azotemia developed in 3 and acute renal shutdown in another 2. Of 22 patients with sickle glomerulopathy (our 9 added to 13 from the literature) 11 died within 2 years. Ten of these (91 %) had developed renal failure, compared to only 5 of the 11 (45%) who survived longer than 2 years (p < 0.05). The 5-year mortality in the general population of sickle cell anemia is 3.75%, and 75% of patients aged 15 years or older survive 18 years or longer. The nephrotic syndrome, most often caused by sickle glomerulopathy, occurs in 4% of patients with sickle cell anemia, leading to renal failure in two-thirds and death in 2 years in half the patients. The development of chronic azotemia correlates strongly with early mortality. The prognosis is much worse than that in the general population of sickle cell anemia.

Renal Complications of Sickle Cell Syndrome in Southern Iran

“…However, in the present study proteinuria was not found. The rate of microalbuminuria was similar to the reports of Becton et al (9), but less than reports of Imuetiyan et al, Lenensburger et al, and . It is expected that micro-albuminuria is increased by aging and decreasing GFR (10,18).…”

“…The development of chronic azotemia correlates strongly with poor prognosis and early mortality as compared to the general population of sickle cell anemia (9). GFR < 60 was present in 5.2% of our patients while end stage renal disease was not present.…”

Cyclophosphamide improves engraftment in patients with SCD and severe organ damage who undergo haploidentical PBSCT

“…Sickle cell-specific criteria included homozygous SCD, compound heterozygous hemoglobin S and C disease or hemoglobin S and b 0 -thalassemia disease, stroke, sickle cell-related renal insufficiency (defined by creatinine $1.5 times the upper limit of normal and kidney biopsy consistent with sickle cell nephropathy, or nephrotic syndrome or creatinine clearance ,50 mL/minute or requiring dialysis), [19][20][21] tricuspid regurgitant jet velocity $2.5 m/s, 22,23 sickle hepatopathy (defined as either ferritin $1000 mg/L or direct bilirubin .0.4 mg/dL and platelet count ,250 000/mL at baseline), 24 .1 hospitalization per year for vaso-occulsive crises or any acute chest syndrome while receiving a maximum tolerated dose of hydroxyurea for at least 6 months.…”

How I treat renal complications in sickle cell disease

“…Full nephrotic syndrome is uncommon at approximately 4%, but when it does occur, it is associated with a very poor outcome. 17 A recognized trigger for nephrotic syndrome, which has been highlighted in patients with SCD, is a recent infection with human parvovirus B19. Early renal biopsy may demonstrate the collapsing variant of focal segmental glomerulosclerosis, the classical lesion associated with virus-induced glomerulopathy.…”