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Darbepoetin alfa and ferric gluconate ameliorate the anemia associated with recessive dystrophic epidermolysis bullosa.

Abstract
Our objective was to describe the efficacy of darbepoetin alfa and ferric gluconate complex in the treatment of the anemia associated with recessive dystrophic epidermolysis bullosa. To accomplish this aim, we retrospectively reviewed a series of patients with this disease treated in a single institution with darbepoetin alfa and parenteral iron for anemia. Four patients with recessive dystrophic epidermolysis bullosa were treated for a mean length of treatment of 14.5 months (4-18 months). Three patients received parenteral iron in the form of ferric gluconate complex and one received iron dextran. The mean pretreatment hemoglobin was 6.8 g/dL (4.9-9.6 g/dL). All four had improvements in their hemoglobin levels and energy levels with a mean increase in hemoglobin level of 2.8 g/dL (p = 0.003). We found darbepoetin alfa and ferric gluconate complex to be effective in the treatment of anemia associated with recessive dystrophic epidermolysis bullosa and to have distinct advantages over previously described formulations, and we concluded that they should be considered in the supportive care of this disease. We recommend the development of a standardized protocol for the evaluation and management of recessive dystrophic epidermolysis bullosa-associated anemia.
AuthorsDennis J Kuo, Anna L Bruckner, Michael R Jeng
JournalPediatric dermatology (Pediatr Dermatol) 2006 Nov-Dec Vol. 23 Issue 6 Pg. 580-5 ISSN: 0736-8046 [Print] United States
PMID17156003 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Ferric Compounds
  • Hematinics
  • Erythropoietin
  • Darbepoetin alfa
  • ferric gluconate
Topics
  • Adolescent
  • Anemia (drug therapy, etiology, genetics)
  • Child
  • Darbepoetin alfa
  • Epidermolysis Bullosa Dystrophica (complications, drug therapy, genetics)
  • Erythropoietin (analogs & derivatives, therapeutic use)
  • Female
  • Ferric Compounds (therapeutic use)
  • Genes, Recessive
  • Hematinics (therapeutic use)
  • Humans
  • Male
  • Retrospective Studies

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