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ISN Education: Ask the Experts

  1. Dr. Lim Soo Kun
  2. Glomerular Diseases
  3. Friday, May 20 2016, 04:13 PM
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Dear expert panel,

I have a 34-year-old man who presented with nephrotic syndrome (24-hr urine protein 6.5g/24hr, Albumin 18, Creatinine 67micromol/l) since age of 29. The initial renal biopsy was not conclusive & he was managed by another nephrologist. He came to me recently for second opinion. After 5 years, he has persistent nephrotic-range proteinuria (6.8g/24hr) and hypoalbuminria (27g/L) with relatively normal renal function (creatinine 80micromol/l) and was rather asymptomatic. Repeat biopsy with electron microscopy confirmed fibronectin glomerulopathy with mild chronicity (mild tubular atrophy & 20% intesrtitial fibrosis).

All the secondary causes screens have been negative. He has no family history of renal diseases. He was treated with Losartan but can only tolerate 50mg daily due to lowish blood pressure (around 110/60mmHg).

I understand that there is no well-proven treatment for this condition. I would like to know if there is anything else that I can offer to this desperate young man. Thank you.

Rgds,
SK Lim
Nephrologist
University Malaya Medical Centre
Kuala Lumpur
Malaysia
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Tejas Desai Accepted Answer
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Thank you for your question Dr. Lim,

I invite you to have a look at how our social media community of nephrologists have responded to your question:

Q: How would U treat 29 yo ♂ w/idiopathic, biopsy-proven fibronectin #nephropathy: 6.5 g proteinuria; Cr 67 umol/L?— The ISN (@ISNkidneycare) May 20, 2016
References
  1. https://twitter.com/ISNkidneycare/status/733739759395278848
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  2. Glomerular Diseases
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