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Recenti Prog Med. 2005 Oct;96(10):488-91.

[Clarkson syndrome: a rare clinical condition characterized by generalized edema associated to monoclonal gammopathy].

[Article in Italian]

Author information

  • 1Sezione di Medicina Interna, Dipartimento di Medicina Interna, Immunologia e Malattie Infettive, Universit√†, Bari. f.vella@intmed.uniba.it

Abstract

Generalized edema is commonly due to cardiac failure, renal changes, hepatic and metabolic disturbances, or it can be idiopathic, i.e. primitive lymphedema. Here we describe a patient with several episodes of fluid extravasation characterized by hypotension, hemoconcentration and functional renal insufficiency. These findings, associated to a monoclonal gammopathy, lead to the diagnosis of systemic capillary leak syndrome or Clarkson Syndrome. This rare and perplexing disorder, characterized by a typical three-phase clinical feature, is due to an endothelium permeability alteration, rather responsible of these paroxysmal manifestations. Interleukin-2-pathway is considered as one of the underlying mechanisms. During acute phase the patient underwent therapy with plasma-expanders and glucocorticoids, although in quiescent phase we administered aminophylline, salbutamol and prednisone. After three months, the patient is asymptomatic.

PMID:
16491771
[PubMed - indexed for MEDLINE]
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