Severe cerebral involvement due to idiopathic systemic capillary leak syndrome

Günes, Ali Riza; Berlit, Peter; Weber, Ralph

doi:10.1002/ccr3.525

Cited by 7 publications

(3 citation statements)

References 13 publications

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“…Abdominal compartment syndrome has not previously been described in the absence of recent intraabdominal surgery [4]. Unlike our case cerebral oedema is also rarely seen [3,5]. We describe a fulminant SCLS episode in a patient admitted to our institution.…”

Section: Introductionmentioning

confidence: 41%

“…During acute episodes patients with SCLS may require surgical intervention and can prove extremely challenging. Our patient was distinctive in some respects: her course was fulminant and refractory to aggressive therapy; she resembled the archetypal Clarkson patient including the onset of symptoms at time of menses; and the development of presumed cerebral oedema during the course of her acute illness [5,16]. Supportive care in an ICU environment is the only well-established treatment for acute SLCS episodes at present.…”

Section: Discussionmentioning

confidence: 95%

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The challenges in managing a case of refractory and overwhelming shock caused by Systemic Capillary Leak Syndrome

2021

JACCR

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Introduction: Systemic Capillary Leak Syndrome (SCLS) or Clarkson syndrome is a rare condition characterised by vascular hyperpermeability. The frequency and severity of disease flares is highly variable. The disease is associated with significant morbidity and mortality. We describe a fulminant episode in a patient known to have the condition. Case Report: A forty-four-year-old woman self-presented with malaise and dyspnoea. Over twelve hours she developed refractory shock despite aggressive volume resuscitation and vasopressor use. She ultimately succumbed to the condition despite aggressive treatment which included fasciotomies of all four limbs for compartment syndrome and a laparostomy for suspected abdominal compartment syndrome. Conclusion: SCLS should be considered in the context of hypotension, haemoconcentration, and hypoalbuminaemia in the absence of albuminuria. Management of acute SLCS episodes can prove extremely challenging for both the intensivist and the anaesthetist. The underlying pathophysiology is unique and fascinating. Supportive care in an intensive care setting is the only established treatment. There are still no proven pharmacotherapies to modulate the course of acute episodes of this disease. Keywords: Shock states; Capillary leak; Compartment syndrome.

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Section: Introductionmentioning

confidence: 41%

Section: Discussionmentioning

confidence: 95%

The challenges in managing a case of refractory and overwhelming shock caused by Systemic Capillary Leak Syndrome

2021

JACCR

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“…While several hundred cases have been reported in adults, only a handful of SCLS cases in children have ever been documented and none have been described in patients with the 22qDS (17,18). As brain involvement is a rare presentation of SCLS (19), here we explore the clinical and immunological phenotypes in a 22qDS patient with severe SCLS, and identify the BBB as a potential conduit by which SCLS impacted the brain in this patient.…”

Section: Introductionmentioning

confidence: 99%

22q11.2 Deletion-Associated Blood-Brain Barrier Permeability Potentiates Systemic Capillary Leak Syndrome Neurologic Features

Crockett,

Kebir,

Anderson

et al. 2024

J Clin Immunol

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We present a case study of a young male with a history of 22q11.2 deletion syndrome (22qDS), diagnosed with systemic capillary leak syndrome (SCLS) who presented with acute onset of diffuse anasarca and sub-comatose obtundation. We hypothesized that his co-presentation of neurological sequelae might be due to blood-brain barrier (BBB) susceptibility conferred by the 22q11.2 deletion, a phenotype that we have previously identi ed in 22qDS. Using pre-and post-intravenous immunoglobulins (IVIG) patient serum, we studied circulating biomarkers of in ammation and assessed the potential susceptibility of the 22qDS BBB. We employed in vitro cultures of differentiated BBB-like endothelial cells derived from a 22qDS patient and a healthy control. We found evidence of peripheral in ammation and increased serum lipopolysaccharide (LPS) alongside endothelial cells in circulation. We report that the patient's serum signi cantly impairs barrier function of the 22qDS BBB compared to control. Only two other cases of pediatric SCLS with neurologic symptoms have been reported, and genetic risk factors have been suggested in both instances. As the third case to be reported, our ndings are consistent with the hypothesis that genetic susceptibility of the BBB conferred by the 22q11.2 deletion promoted neurologic involvement during SCLS in this patient.

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Extensive leukoencephalopathy associated with idiopathic capillary leak syndrome: report of a case with neuropathology

et al. 2021

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Severe cerebral involvement due to idiopathic systemic capillary leak syndrome

Cited by 7 publications

References 13 publications

The challenges in managing a case of refractory and overwhelming shock caused by Systemic Capillary Leak Syndrome

The challenges in managing a case of refractory and overwhelming shock caused by Systemic Capillary Leak Syndrome

22q11.2 Deletion-Associated Blood-Brain Barrier Permeability Potentiates Systemic Capillary Leak Syndrome Neurologic Features

Extensive leukoencephalopathy associated with idiopathic capillary leak syndrome: report of a case with neuropathology

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