ESPN 50th Annual Meeting

ESPN 2017


 
A case of rapidly progressive glomerulonephritis superimposed on X-linked Alport syndrome.
Petr Ananin 1 Olga Komarova 1 Tatyana Vashurina 1 Rustem Tepaev 1 Ekaterina Stolyarevich 2 Alexey Tsygin 1

1- National scientific center of children health
2- Municipal hospital №52
 
Introduction:

The outcome if AKI is strictly dependent of preexisting chronic kidney disease and comorbidities.

Material and methods:

We report case of rapidly progressive glomerulonephritis in 14 years old boy with X-linked Alport syndrome (AS).

Results:

The boy had a history of familial X-linked AS (his elder brother also has morphology of AS with CKD stage 2 and sensoneural deafness stage 3 on third decade), at 1 year he developed hematuria, at 5 years – proteinuria 1,4 g/l. He received nephroprotective and antiproteinuric therapy with fosinopril, losartan and amlodipine, but despite that proteinuria dramatically increased. Since 9 years he had nephrotic range proteinuria. At 14 years next day after vaccination against diphtheria, tetanus and polio he developed nausea, gross hematuria and hypertension and. He was observed and 72 hour he became oliguric. Laboratory findings were increasing of serum creatinine to 1087 µmol/l and urea to 31 mmol/l, serum complement was normal, autoantibodies were negative. He was oliguric and needed dialysis (CVVHD) during 16 days. We performed oral steroid therapy 1 mg/kg, daily steroid pulse-therapy 1000 mg and plasmapheresis with almost complete recovery of kidney function, his serum creatinine was 98 µmol/l, eGFR (CKD-EPI) 99 ml/min/1,73m2. Kidney biopsy showed signs of immunocomplex glomerulonephritis (ICGN) with fibrous and cellular crescents and mesangial granular C3 deposition on immunofluorescence and signs of AS with tubular atrophy and interstitial fibrosis. However within 4 months we observed decline in GFR to 41 ml/min/1,73m2.

Conclusions:

 We suggest that preexisting AS can predispose to immunocomplex glomerulonebphritis because of glomerular basement membrane abnormalities. From another hand, despite acute ICGN superimposed on AS may significantly accelerate CKD progression despite efficient treatment with pulses and plasma exchanges.