Literature DB >> 3718853

Iron removal therapy in porphyria cutanea tarda: phlebotomy versus slow subcutaneous desferrioxamine infusion.

E Rocchi, P Gibertini, M Cassanelli, A Pietrangelo, A Borghi, M Pantaleoni, J Jensen, E Ventura.   

Abstract

Twenty-five patients with overt clinical and biochemical findings of porphyria cutanea tarda took part in a study comparing intensive phlebotomy with slow subcutaneous desferrioxamine treatment. Fifteen male patients (Group A) had intensive venesection therapy. Ten patients (Group B) with associated diseases (minor thalassemia, cardiovascular impairment, pulmonary tuberculosis or severe liver cirrhosis) received 1.5 g of desferrioxamine by slow subcutaneous infusion using an automatic syringe pump 5 days a week. No patient complained of appreciable side effects. Serum iron, ferritin and uroporphyrins were normalized in all subjects by the end of treatment. The mean time necessary for complete recovery was 13.8 months (range 9-19) and 11.2 months (range 6-14) in Groups A and B, respectively. Liver function significantly improved during and after the treatments in both groups. We conclude that recovery from porphyria cutanea tarda can be achieved equally well using phlebotomy or desferrioxamine subcutaneous infusion. Phlebotomy is easily performed and remains the treatment of choice; slow subcutaneous desferrioxamine treatment, although expensive, is recommended when severe associated diseases contra-indicate venesection.

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Year:  1986        PMID: 3718853     DOI: 10.1111/j.1365-2133.1986.tb04071.x

Source DB:  PubMed          Journal:  Br J Dermatol        ISSN: 0007-0963            Impact factor:   9.302


  7 in total

1.  Pro-oxidant and antioxidant factors in acute intermittent porphyria: family studies.

Authors:  E Rocchi; P Ventura; A Ronzoni; M C Rosa; C Gozzi; L Marri; G Casalgrandi; M D Cappellini
Journal:  J Inherit Metab Dis       Date:  2004       Impact factor: 4.982

Review 2.  The role of transferrin in the mechanism of cellular iron uptake.

Authors:  K Thorstensen; I Romslo
Journal:  Biochem J       Date:  1990-10-01       Impact factor: 3.857

Review 3.  Current applications of therapeutic phlebotomy.

Authors:  Tarek Bou Assi; Elizabeth Baz
Journal:  Blood Transfus       Date:  2013-10-03       Impact factor: 3.443

4.  Genetic variation of iron-induced uroporphyria in mice.

Authors:  A G Smith; J E Francis
Journal:  Biochem J       Date:  1993-04-01       Impact factor: 3.857

5.  Initial serum ferritin predicts number of therapeutic phlebotomies to iron depletion in secondary iron overload.

Authors:  Sandhya R Panch; Yu Ying Yau; Kamille West; Karen Diggs; Tamsen Sweigart; Susan F Leitman
Journal:  Transfusion       Date:  2014-09-11       Impact factor: 3.157

6.  A tale of nonhormonal hairs.

Authors:  Rajeev Philip; Prem P Patidar; Praveen Ramachandra; Keshav K Gupta
Journal:  Indian J Endocrinol Metab       Date:  2012-05

7.  Porphyria Cutanea Tarda in a Patient with End-Stage Renal Disease: A Case of Successful Treatment with Deferoxamine and Ferric Carboxymaltose.

Authors:  Natacha Rodrigues; Fernando Caeiro; Alice Santana; Teresa Mendes; Leonor Lopes
Journal:  Case Rep Nephrol       Date:  2017-01-22
  7 in total

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