| Therapy-resistant anaemia in congenital nephrotic syndrome of the Finnish type--implication of EPO, transferrin and transcobalamin losses. | |
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MedLine Citation:
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PMID: 19153070 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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 approximately 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. |
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Authors:
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Julie Toubiana; Marie-Hélène Schlageter; Bilal Aoun; Olivier Dunand; Renata Vitkevic; Albert Bensman; Tim Ulinski |
Publication Detail:
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Type: Case Reports; Journal Article Date: 2009-01-18 |
Journal Detail:
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Title: Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association Volume: 24 ISSN: 1460-2385 ISO Abbreviation: Nephrol. Dial. Transplant. Publication Date: 2009 Apr |
Date Detail:
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Created Date: 2009-03-23 Completed Date: 2009-08-07 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 8706402 Medline TA: Nephrol Dial Transplant Country: England |
Other Details:
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Languages: eng Pagination: 1338-40 Citation Subset: IM |
Affiliation:
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Department of Pediatric Nephrology, Armand Trousseau Hospital, 26 Avenue du Docteur Arnold Netter, 75012 Paris, France. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Anemia
/
blood,
etiology,
therapy* Blood Transfusion Erythropoietin / administration & dosage, blood, urine* Female Hematinics / administration & dosage, blood, urine* Humans Infant, Newborn Nephrectomy Nephrotic Syndrome / complications, congenital, genetics, therapy* Transcobalamins / analysis, urine* Transferrin / analysis, urine* |
| Chemical | |
Reg. No./Substance:
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0/Hematinics; 0/Transcobalamins; 11096-26-7/Erythropoietin; 11096-37-0/Transferrin |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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