| A case report of systemic capillary leak syndrome (Clarkson's disease). | |
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MedLine Citation:
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PMID: 20148770 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Systemic capillary leak syndrome (SCLS) is a very rare disorder also known as Clarkson's disease. The condition is characterized by recurrent episodes of severe capillary hyperpermeability resulting in severe hemoconcentration, hypoalbuminemia, hypovolemia and shock. We describe a 41-year-old previously healthy man who was admitted to hospital on several occasions with rapidly developing hypovolemic shock accompanied by extreme hemoconcentration and hypoalbuminemia. Our case is similar to other reports describing patients with SCLS where the initial suspicions have been pointing towards septic shock. He received a combination of prophylactic treatment with theophylline, beta-agonists, immunoglobulins and statins but eventually died after a severe episode of SCLS that ended with recurrent cardiac arrest. Clinical autopsy revealed pulmonary edema and acute and chronical organic fluid overload. SCLS should be kept in mind when treating patients suffering from attacks of severe idiopathic edema and mimics recurrent septic shock where no pathogen is found. The pathogenesis is unknown and the attacks may be lethal. |
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Authors:
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J Hollenberg; J Frykman; L-G Lundberg; S Forsberg |
Publication Detail:
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Type: Case Reports; Journal Article Date: 2010-02-11 |
Journal Detail:
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Title: Acta anaesthesiologica Scandinavica Volume: 54 ISSN: 1399-6576 ISO Abbreviation: Acta Anaesthesiol Scand Publication Date: 2010 May |
Date Detail:
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Created Date: 2010-04-26 Completed Date: 2010-07-27 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 0370270 Medline TA: Acta Anaesthesiol Scand Country: England |
Other Details:
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Languages: eng Pagination: 649-52 Citation Subset: IM |
Affiliation:
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Department of Cardiology, Medical Intensive Care Unit, Karolinska Institute, South Hospital, Stockholm, Sweden. jacob.hollenberg@sodersjukhuset.se |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Blood Chemical Analysis Capillary Leak Syndrome / complications*, diagnosis, therapy Fatal Outcome Humans Hypoalbuminemia / etiology, therapy Male Patient Readmission Shock / diagnosis, etiology*, therapy |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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