SynthesisEuropean journal of pediatrics2018
Idiopathic systemic capillary leak syndrome (Clarkson syndrome) in childhood: systematic literature review.
Synthesis in European journal of pediatrics, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers.
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Who cites it
17 citing papers in PubMed, 34 citations in OpenAlex.
- Idiopathic systemic capillary leak syndrome and related shock in PICU: an underdiagnosed disease?Journal of anesthesia, analgesia and critical care · 2026Article
- Clarkson disease in critically and non-critically ill patients: insights from the Italian IRIS-CLS registry.Internal and emergency medicine · 2025Article
- Characteristics of SARS-CoV-2-associated severe episodes of monoclonal gammopathy-associated capillary leak syndrome (Clarkson disease).Annals of intensive care · 2025Article
- Systemic capillary leak syndrome.Nature reviews. Disease primers · 2024Review
- 22q11.2 Deletion-Associated Blood-Brain Barrier Permeability Potentiates Systemic Capillary Leak Syndrome Neurologic Features.Journal of clinical immunology · 2024Article
- Ultrasound findings and specific intrinsic blood volume expansion therapy for neonatal capillary leak syndrome: report from a multicenter prospective self-control study.European journal of medical research · 2024Article
- Myocardial Edema: A Rare but Specific Manifestation of Neonatal Capillary Leak Syndrome.Diagnostics (Basel, Switzerland) · 2023Article
- Capillary hyperpermeability syndrome: A fatal complication of acute leukaemia: Case report and review.Annals of medicine and surgery (2012) · 2022Article
- Risk factors for anesthesia-associated postoperative capillary leakage after thoracoscopic surgery in neonates: A single-center observational study.Frontiers in pediatrics · 2022Article
- A Rare Case of Idiopathic Systemic Capillary Leak Syndrome.Journal of community hospital internal medicine perspectives · 2022Article
- Extensive leukoencephalopathy associated with idiopathic capillary leak syndrome: report of a case with neuropathology.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2021Article
- A preliminary study of influences of hydroxyethyl starch combined with ulinastatin on degree of edema in newborns with capillary leak syndrome.American journal of translational research · 2021Article
- [Neonatal capillary leak syndrome].Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics · 2020Article
- A case report of capillary leak syndrome with recurrent pericardial and pleural effusions.European heart journal. Case reports · 2020Article
- A Remarkable Coexistence of Systemic Capillary Leak Syndrome and Diabetes in an 11-Year-Old Boy: A Case Report and Review of the Literature.Case reports in immunology · 2020Article
- Myocardial oedema in an 8-year-old Chinese boy with Idiopathic systemic capillary leak syndrome.BMC pediatrics · 2019Article
- Beyond the Syndrome: Extensive Congenital Abnormalities in an Infant With Trisomy 21.Clinical pathology (Thousand Oaks, Ventura County, Calif.)Article
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Authors and funding
5 authors at 5 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
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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