Idiopathic systemic capillary leak syndrome (Clarkson syndrome) in childhood: systematic literature review

Abstract

Approximately 500 cases of idiopathic systemic capillary leak syndrome (Clarkson syndrome) have been reported worldwide. This life-threatening condition is characterized by episodes of increase in vascular permeability with loss of fluid into the interstitium and presents with acute onset of edema, signs of tissue hypoperfusion, hemoconcentration, and low blood protein level. It has been diagnosed mainly in middle-aged adults with a monoclonal gammopathy. We performed a systematic review of the literature on Clarkson syndrome in subjects ≤ 18 years of age. We identified 24 reports, published since 1989, providing data on 32 otherwise healthy subjects, who experienced 67 well-documented episodes of Clarkson syndrome. The condition affected more frequently girls (21, 66%) than boys, presented throughout childhood, and was preceded by a mostly viral illness in 75% of cases. A monoclonal gammopathy was never reported. Uncompensated circulatory shock, muscle compartment syndrome, acute kidney injury, pulmonary edema, and either pleural or pericardial effusion were, in decreasing order of frequency, the most common complications. Four patients died.

Conclusion: Clarkson syndrome develops not only in adulthood but also in childhood. In this age group, this condition is not linked to a monoclonal gammopathy.

What is Known:
• Clarkson syndrome is a rare condition that has been diagnosed mainly in middle-aged adults and is mostly linked to a monoclonal gammopathy.
What is New:
• In subjects ≤ 18 years of age, Clarkson syndrome is not linked to a monoclonal gammopathy.

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Acknowledgments

The authors would like to thank Dr. Alec Villa for his assistance in the linguistic revision.

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Authors

Contributions

- Concept of the manuscript: G.P.M., M.G.B.

- Literature search and analysis: M.A.B., M.G.B., S.A.G.L.

- Statistical analysis: S.A.G.L.

- Drafting of the manuscript: M.A.B.

- Critical revision of the manuscript: G.P.M., E.F.F., S.A.G.L.

- Final manuscript: M.A.B, G.P.M., M.G.B., E.F.F., S.A.G.L.

Corresponding author

Correspondence to Gregorio P. Milani.

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The study has been performed in accordance with the ethical standards as laid down in the 1964 Declaration of Helsinki and its later amendments.

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The authors declare that they have no conflict of interest.

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Communicated by Peter de Winter

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Bozzini, MA., Milani, G.P., Bianchetti, M.G. et al. Idiopathic systemic capillary leak syndrome (Clarkson syndrome) in childhood: systematic literature review. Eur J Pediatr 177, 1149–1154 (2018). https://doi.org/10.1007/s00431-018-3189-8

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Keywords

  • Acute kidney injury
  • Capillary leak syndrome
  • Capillary permeability
  • Compartment syndrome