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Acute renal failure in dense deposit disease: complete recovery after combination therapy with immunosuppressant and plasma exchange.

Krmar, R T; Holtbäck, U; Linné, T; Berg, U B; Celsi, G; Söderberg, Marcus; Wernerson, A; Szakos, A; Larsson, S and Melander Skattum, Lillemor LU , et al. (2011) In Clinical Nephrology 75(S1). p.4-10
Abstract
We describe the clinical course of a female adolescent who was followed because of isolated microhematuria and hypocomplementemia before admission to hospital with a sudden onset of acute renal failure. At presentation, she exhibited complement consumption through the complement alternative pathway (AP) while other serologic tests were negative. Renal biopsy revealed dense deposit disease (DDD) with a crescentic pattern. Intravenous methylprednisolone, followed by plasma exchange (PE), and intravenous cyclophosphamide pulses were started shortly after admission. C3NeF and anti-factor H antibody tests were negative. Serum factor H and I levels were normal as well as factor H activity. Screening for mutation in the factor H gene revealed the... (More)
We describe the clinical course of a female adolescent who was followed because of isolated microhematuria and hypocomplementemia before admission to hospital with a sudden onset of acute renal failure. At presentation, she exhibited complement consumption through the complement alternative pathway (AP) while other serologic tests were negative. Renal biopsy revealed dense deposit disease (DDD) with a crescentic pattern. Intravenous methylprednisolone, followed by plasma exchange (PE), and intravenous cyclophosphamide pulses were started shortly after admission. C3NeF and anti-factor H antibody tests were negative. Serum factor H and I levels were normal as well as factor H activity. Screening for mutation in the factor H gene revealed the H402 allele variant. Clinical remission, defined as normalization in renal function and in the activity levels of the complement AP, was noted at one month post-presentation and throughout the follow-up. A repeat renal biopsy showed the disappearance of crescent formation, whereas electron microscopy revealed no regression in dense transformation of the lamina densa. In summary, our patient was successfully treated with immunosuppressant and PE. The absence of known factors associated with DDD suggests that, in this particular case, other regulatory mechanisms of complement AP might have been involved in the disease process. (Less)
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Contribution to journal
publication status
published
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Clinical Nephrology
volume
75
issue
S1
pages
4 - 10
publisher
Dustri-Verlag
external identifiers
  • WOS:000288231500002
  • PMID:21269585
  • Scopus:79952000966
ISSN
0301-0430
DOI
10.2379/CN106444
language
English
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yes
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30916fee-ce86-406b-8f94-36ad0c06846e (old id 1777049)
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http://www.ncbi.nlm.nih.gov/pubmed/21269585?dopt=Abstract
date added to LUP
2011-02-01 09:42:13
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2016-12-04 04:40:05
@misc{30916fee-ce86-406b-8f94-36ad0c06846e,
  abstract     = {We describe the clinical course of a female adolescent who was followed because of isolated microhematuria and hypocomplementemia before admission to hospital with a sudden onset of acute renal failure. At presentation, she exhibited complement consumption through the complement alternative pathway (AP) while other serologic tests were negative. Renal biopsy revealed dense deposit disease (DDD) with a crescentic pattern. Intravenous methylprednisolone, followed by plasma exchange (PE), and intravenous cyclophosphamide pulses were started shortly after admission. C3NeF and anti-factor H antibody tests were negative. Serum factor H and I levels were normal as well as factor H activity. Screening for mutation in the factor H gene revealed the H402 allele variant. Clinical remission, defined as normalization in renal function and in the activity levels of the complement AP, was noted at one month post-presentation and throughout the follow-up. A repeat renal biopsy showed the disappearance of crescent formation, whereas electron microscopy revealed no regression in dense transformation of the lamina densa. In summary, our patient was successfully treated with immunosuppressant and PE. The absence of known factors associated with DDD suggests that, in this particular case, other regulatory mechanisms of complement AP might have been involved in the disease process.},
  author       = {Krmar, R T and Holtbäck, U and Linné, T and Berg, U B and Celsi, G and Söderberg, Marcus and Wernerson, A and Szakos, A and Larsson, S and Melander Skattum, Lillemor and Bárány, P},
  issn         = {0301-0430},
  language     = {eng},
  number       = {S1},
  pages        = {4--10},
  publisher    = {ARRAY(0x8062aa0)},
  series       = {Clinical Nephrology},
  title        = {Acute renal failure in dense deposit disease: complete recovery after combination therapy with immunosuppressant and plasma exchange.},
  url          = {http://dx.doi.org/10.2379/CN106444},
  volume       = {75},
  year         = {2011},
}