Progression of Crescentic Poststreptococcal Glomerulonephritis to Terminal Uremia Twelve Years after Recovery from an Acute Episode

Accessible online at: http://BioMedNet.com/karger Dear Sir, Acute poststreptococcal glomerulonephritis is still the most common glomerular disease among children in undeveloped countries [1]. The prognosis is usually benign with prompt clinical recovery and resolution of urinary and histological abnormalities within a year after an acute episode [2, 3]. The unfavorable outcome of the disease is due to the mortality in the acute stage which is now rarely seen owing to better supportive care; or to the rapidoprogressive clinical course with histological evidence for crescentic nephritis. Extensive crescentic formation is associated with worse prognosis and the use of various immunosuppressive protocols remains controversial. Herein, we present a child with crescentic poststreptococcal glomerulonephritis, who recovered from an acute episode and had been followed for 6 years, with near-normal laboratory findings, but went into terminal uremia 12 years after the acute episode. In May 1983, 3 weeks after having scarlet fever, a 9-year-old boy presented with macroscopic hematuria, edema and elevated blood pressure (130/100 mm Hg). Initial tests revealed: urea 8.6 mmol/l, creatinine 145 Ìmol/l, serum protein 48 g/l with albumin 22 g/l, cholesterol 8.3 mmol/l, triglycerides 3.2 mmol/l, ASTO titer 800 Todd units, C3 0.25 g/l, proteinuria 3.2 g/day. Instead of recovery, his clinical and biological parameters worsened. His urinary output diminished to 30 ml/24 h, blood pressure increased to 170/130 mm Hg, as well as urea (34.4 mmol/l), creatinine (870 Ìmol/l) and potassium (6.3 mmol/l); thus hemodialysis was commenced on the 8th hospital day. Renal biopsy revealed exudative proliferative glomerulonephritis with crescents in 7 of 12 glomeruli (58%). On immunofluorescence granular pattern of C3(+3), IgG(+2) and fibrinogen(+3) was seen along the capillary loops and in the mesangium. Prednisone was given at the dose of 2 mg/kg BW and 3 days later his urine output increased with gradual clinical and biological recovery and on the 21st hospital day he was off dialysis. Two months later he was clinically well with normal blood pressure (105/70 mm Hg). C3 returned to a normal value 1.12 g/l. Except for microhematuria (20–25 RBC/hpf), proteinuria (1+, 0.56 g/day) and creatinine (120 Ìmol/l) all other laboratory results were normal. One year later his creatinine was normal (55 Ìmol/l), there was no microhematuria and his 24-hour protein excretion was 0.35 g/day. The child has been followed for 6 years, on the last control his blood pressure was 115/80, creatinine 62 Ìmol/l, creatinine clearance 118 ml/min and proteinuria 0.20 g/day with normal urinary sediment. Between 1992 and 1995, we undertook a campaign to evaluate the renal status of children who had suffered from acute poststreptococcal glomerulonephritis in the period from 1980 to 1990, but we could not localize the patient and check up his renal status. He was well until December 1996 when he complained of fatigue and malaise. Laboratory tests revealed urea 38 mmol/l, creatinine 920 Ìmol/l, uric acid 560 Ìmol/l. In February 1997, an AV fistula was created at the Department of Nephrology, Clinical Center Skopje, and the patient commenced chronic hemodialysis. Schacht et al. [4] reported a series of 6 adult patients who progressed to uremia after remission of acute poststreptococcal glomerulonephritis. Although all but one patient in his series renal function returned to normal within 1 year, renal failure developed over the period ranging from 2 to 12 years. All patients in his series had hypertension or moderate proteinuria during the follow-up. On the contrary, our patient was normotensive with minimal proteinuria during the 6-year follow-up. Considering the very favorable course of the disease during the regular 6-year follow-up one should expect good long-term prognosis, but surprisingly our patient went into terminal uremia. Thus, all children who recovered from crescentic poststreptococcal glomerulonephritis should have indefinite follow-up for close monitoring of the renal function.