Patient's Query
Hello doctor,
I am 15-years-old. My native kidney biopsy is:
Microscopic description:
The sample contains renal cortex, with 21 glomeruli (8 of them globally sclerosed).
Some glomeruli reveal mild mesangial matrix expansion, marked glomerular hypertrophy, segmental glomerulosclerosis in 4 glomeruli.
Tubular epithelial cells show edematous cytoplasm and red blood cells in some tubules.
Interstitial fibrosis is up to 20% of the cortical area.
Arteries and arterioles are unremarkable.
Congo red stain for amyloid was not performed.
Immunofluorescence description:
Results of immunofluorescence for IgA, IgG, IgM, C3, and C1q were performed on paraffin sections. There are weak focal IgM, C3, and equivocal IgA staining in the mesangium.
Electron microscopy description:
Electron microscopy sample contains one (1) glomerulus. Focal effacement of podocyte foot processes is present. No electron-dense deposits are found in the glomerular structures. Glomerular endothelial cells reveal slight swelling of the cytoplasm. Glomerular basement membranes in some areas appear thin, with some very delicate focal splintering.
Clinical information:
Chronic nephritic syndrome.
CKD IIIa.
What type of nephropathy could it be? Like IgA nephropathy or Alport, etc.?
Thank you.
Hello,
Welcome to icliniq.com.
I read your query and the biopsy report.
Can you tell me what were the kidney functions of the patient (urea and creatinine)? Also, tell me the urine examination of the patient and if 24-hour urine protein was done? Is there any family history of kidney failure? Were eye and ear examinations done? Also, was the patient having recurrent urinary tract infections? I do not think this is IgA nephropathy, and it looks more like Alport's syndrome.
Patient's Query
Hello doctor,
Thank you for your answer.
Creatinine 150 (I do not know the units), GFR 45 mL/min. 24-hour was done, just protein traces found. But she is taking ACE inhibitors for six months. Before that, it was about 0.7 - 1 grams of protein per day.
No family history. Eye and ear examination were not done recently, but it was always fine and now looks fine.
No recurrent urinary tract infections.
Alport's is inherited, but the biopsy says to rule out the inherited (they supposed Alport as something additional?)
Anything beyond IgA (IgA should be dominant on immunofluorescence and under an electron microscope?) Is Alport's possible?
Thank you very much.
Hello,
Welcome back to icliniq.com.
Based on the available history and investigations, including biopsy, I think this is secondary FSGS (focal segmental glomerulosclerosis) because most likely Alport's syndrome since GBM (glomerular basement membrane) is showing focal splintering. It is not IgA nephropathy. She needs to be on ACE (angiotensin-converting enzyme) inhibitors. For ruling out Alport's syndrome, we need to do a slit lamp examination of the eyes by an ophthalmologist and pure tone audiometry by an ENT specialist. Another possibility is thin basement membrane disease. Also, she would need a genetic test for a definitive diagnosis.
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Answered byDr. Manzoor Ahmad Parry
Medically reviewed byiCliniq medical review team
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