Skip Navigation


NDT Advance Access originally published online on January 18, 2009
Nephrology Dialysis Transplantation 2009 24(4):1338-1340; doi:10.1093/ndt/gfn762
This Article
Right arrow Full Text
Right arrow Full Text (PDF)
Right arrow All Versions of this Article:
24/4/1338    most recent
gfn762v1
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrowRequest Permissions
Right arrow Disclaimer
Google Scholar
Right arrow Articles by Toubiana, J.
Right arrow Articles by Ulinski, T.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Toubiana, J.
Right arrow Articles by Ulinski, T.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

© The Author [2009]. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. For Permissions, please e-mail: journals.permissions@oxfordjournals.org


Therapy-resistant anaemia in congenital nephrotic syndrome of the Finnish type—implication of EPO, transferrin and transcobalamin losses

Julie Toubiana1, Marie-Hélène Schlageter2, Bilal Aoun1, Olivier Dunand1, Renata Vitkevic1, Albert Bensman1 and Tim Ulinski1

1 Department of Pediatric Nephrology, Armand Trousseau Hospital, APHP and University Pierre and Marie Curie, Paris VI 2 Department of Nuclear Medicine, Saint Louis Hospital, APHP, Paris, France

Correspondence and offprint requests to: Tim Ulinski, Department of Pediatric Nephrology, Armand Trousseau Hospital, 26 Avenue du Docteur Arnold Netter, 75012 Paris, France. Tel: +33-144736662; Fax: +33-144736663; E-mail: tim.ulinski{at}trs.aphp.fr



  Abstract

Congenital nephrotic syndrome of the Finnish type (CNF) is due to NPHS1 mutation and is responsible for a variety of urinary protein losses. We report the case of a 4-month-old girl with a particularly severe form (proteinuria ~150 g/l) of CNF. She developed severe non-regenerative anaemia requiring bi-monthly blood transfusions despite daily EPO (600 UI/kg) and iron supplementation. Epoetin pharmacokinetics revealed a urinary loss of 27% of the given dose within the first 24 h after IV injection. However, plasma levels remained increased after 24 h (228 UI/l). Plasma transferrin and transcobalamin levels were undetectable. Atransferrinaemia and atranscobalaminaemia seem to be responsible for disturbed erythropoiesis.

Keywords: congenital nephrotic syndrome of the Finnish type; erythropoietin; transferrin; transcobalamin

Received for publication: 29. 7.08
Accepted in revised form: 19.12.08


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?


This article has been cited by other articles:


Home page
 BloodHome page
T. Ulinski, B. Aoun, J. Toubiana, R. Vitkevic, A. Bensman, and J. Donadieu
Neutropenia in congenital nephrotic syndrome of the Finnish type: role of urinary ceruloplasmin loss
Blood, May 7, 2009; 113(19): 4820 - 4821.
[Full Text] [PDF]


Disclaimer: Please note that abstracts for content published before 1996 were created through digital scanning and may therefore not exactly replicate the text of the original print issues. All efforts have been made to ensure accuracy, but the Publisher will not be held responsible for any remaining inaccuracies. If you require any further clarification, please contact our Customer Services Department.