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Systemic capillary leak syndrome. Case report
D D Sabbadini1, R Peressutti, A Di Silvestre
1Second Division of Anesthesia Resuscitation and Antalgic Therapy, Santa Maria della Misericordia Hospital, Udine, Italy. dariodavide.sabbadini@poste.it
Minerva Anestesiologica
|February 15, 2003
Summary
Systemic capillary leak syndrome (SCLS) is a rare condition causing shock, fluid shifts, and organ failure. Early diagnosis and treatment with theophylline and terbutaline may improve patient outcomes.
Area of Science:
- Internal Medicine
- Critical Care Medicine
- Rare Diseases
Background:
- Systemic capillary leak syndrome (SCLS) is a rare disorder characterized by recurrent episodes of hypotension, hemoconcentration, and hypoproteinemia.
- It involves unexplained episodic capillary hyperpermeability, leading to fluid and protein shifting from the intravascular to the interstitial space.
Observation:
- A 59-year-old male presented with unexplained shock, hemoconcentration, renal failure, and metabolic acidosis.
- Initial resuscitation attempts with crystalloids and dopamine were unsuccessful.
- Diagnostic imaging (CT, TEE, X-ray) and absence of infection/anaphylaxis were noted.
Findings:
- The patient was resuscitated with aggressive fluid infusion and norepinephrine.
- Diagnosis was based on prior shock episodes, hemoconcentration, and negative cardiopulmonary findings.
- Monoclonal immunoglobulin G, kappa chain, in serum confirmed the SCLS diagnosis.
Implications:
- SCLS must be considered in the differential diagnosis of idiopathic and anaphylactic shock.
- Prophylactic treatment with theophylline and terbutaline may benefit SCLS patients.
- This case highlights the diagnostic challenges and potential therapeutic strategies for SCLS.