Plasma therapy in atypical haemolytic uremic syndrome: lessons from a family with a factor H mutation View Full Text


Ontology type: schema:ScholarlyArticle      Open Access: True


Article Info

DATE

2008-09-01

AUTHORS

Jean Claude Davin, Lisa Strain, Tim H. J. Goodship

ABSTRACT

Whilst randomised control trials are undoubtedly the best way to demonstrate whether plasma exchange or infusion alone is the best first-line treatment for patients with atypical haemolytic uremic syndrome (aHUS), individual case reports can provide valuable information. To that effect, we have had the unique opportunity to follow over a 10-year period three sisters with aHUS associated with a factor H mutation (CFH). Two of the sisters are monozygotic twins. A similar natural evolution and response to treatment would be expected for the three patients, as they all presented with the same at-risk polymorphisms for CFH and CD46 and no identifiable mutation in either CD46 or CFI. Our report of different modalities of treatment of the initial episode and of three transplantations and relapses in the transplant in two of them, strongly suggest that intensive plasma exchange, both acutely and prophylactically, can maintain the long-term function of both native kidneys and allografts. In our experience, the success of plasma therapy is dependent on the use of plasma exchange as opposed to plasma infusion alone, the prolongation of daily plasma exchange after normalisation of haematological parameters followed by prophylactic plasma exchange, the use of prophylactic plasma exchange prior to transplantation and the use of prophylactic plasma exchange at least once a week posttransplant with immediate intensification of treatment if there are any signs of recurrence. More... »

PAGES

1517-1521

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/s00467-008-0833-y

DOI

http://dx.doi.org/10.1007/s00467-008-0833-y

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1037568386

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/18483746


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212 grid-institutes:grid.5650.6 schema:alternateName Pediatric Nephrology, Emma Children’s Hospital/ Academic Medical Centre, 9 Meibergdreef, 1105, Amsterdam Z-O, The Netherlands
213 schema:name Pediatric Nephrology, Emma Children’s Hospital/ Academic Medical Centre, 9 Meibergdreef, 1105, Amsterdam Z-O, The Netherlands
214 rdf:type schema:Organization
 




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