Evidence map›Paper›PMID 41514342›Full record

ArticleJournal of anesthesia, analgesia and critical care2026

Idiopathic systemic capillary leak syndrome and related shock in PICU: an underdiagnosed disease?

Marco Piastra, Ivonne Portaccio, Arianna Dondi, Enzo Picconi, Tony Christian Morena, Maria Cristina Mondardini, Donato Rigante, Giorgia Spinazzola, Gabriella De Rosa, Giorgio Conti

Abstract read
In one paragraph

Article in Journal of anesthesia, analgesia and critical care, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

10 authors.

Marco PiastraPediatric Intensive Care Unit and Trauma Center, Fondazione Policlinico Universitario "A. Gemelli" IRCCS , Largo A. Gemelli 8, Rome, 00168, Italy.
Ivonne PortaccioPediatric Intensive Care Unit and Trauma Center, Fondazione Policlinico Universitario "A. Gemelli" IRCCS , Largo A. Gemelli 8, Rome, 00168, Italy. ivonne.portaccio@gmail.com.
Arianna DondiDepartment of Pediatrics and PICU, IRCCS AOUBO, Bologna, Italy.
Enzo PicconiPediatric Intensive Care Unit and Trauma Center, Fondazione Policlinico Universitario "A. Gemelli" IRCCS , Largo A. Gemelli 8, Rome, 00168, Italy.
Tony Christian MorenaPediatric Intensive Care Unit and Trauma Center, Fondazione Policlinico Universitario "A. Gemelli" IRCCS , Largo A. Gemelli 8, Rome, 00168, Italy.
Maria Cristina MondardiniDepartment of Pediatrics and PICU, IRCCS AOUBO, Bologna, Italy.
Donato RiganteDepartment of Pediatrics, Fondazione Policlinico Universitario "A. Gemelli" IRCCS, Rome, Italy.
Giorgia SpinazzolaDepartment of Anesthesia and Intensive Care, Fondazione Policlinico Universitario "A. Gemelli" IRCCS, Largo A. Gemelli 8, Rome, 00168, Italy.
Gabriella De RosaDepartment of Pediatrics, Fondazione Policlinico Universitario "A. Gemelli" IRCCS, Rome, Italy.
Giorgio ContiPediatric Intensive Care Unit and Trauma Center, Fondazione Policlinico Universitario "A. Gemelli" IRCCS , Largo A. Gemelli 8, Rome, 00168, Italy.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundIdiopathic systemic capillary leak syndrome (SCLS), or Clarkson syndrome, is a rare disorder characterized by increased capillary permeability causing fluid leakage into interstitial spaces. SCLS remains underdiagnosed in both adults and children due to its resemblance to septic shock, with particularly limited pediatric data available.

methodsWe conducted a retrospective analysis of six pediatric patients with seven SCLS episodes diagnosed in two Italian PICUs over a ten-year period.

resultsPatients aged 4-10 years presented with hypovolemic shock and the characteristic triad of hypovolemia, hemoconcentration, and hypoalbuminemia without albuminuria. No monoclonal gammopathy was observed. Viral infections (75%, predominantly influenza) were identified as triggers. Common complications included rhabdomyolysis (100%), compartment syndrome (100%), acute kidney injury (71.4%), and cardiac involvement (71.4%). Cardiothoracic index measurements showed significant elevation during acute phases (mean 58% ± 1%) with reduction after treatment (mean 51% ± 1%). Mean hospitalization was 20 days (12 in PICU). One patient died from refractory ventricular fibrillation, another experienced recurrence.

conclusionsPediatric SCLS represents a severe condition with distinctive characteristics compared to adult cases, including absence of monoclonal gammopathy, frequent viral triggers, and universal complications of rhabdomyolysis and compartment syndrome. Early recognition using our proposed diagnostic red flags can facilitate prompt intervention and improve outcomes.

Indexed as

Cardiothoracic IndexClarkson SyndromeCompartment SyndromeEndothelial DysfunctionHypoalbuminemiaInfluenzaPediatric Intensive CareRhabdomyolysisSystemic Capillary Leak SyndromeViral Trigger

Identifiers

PMID41514342
PMCPMC12882570

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.