Systemic Capillary Leak Syndrome associated with hypovolemic shock and compartment syndrome. Use of transpulmonary thermodilution technique for volume management

Saugel, Bernd; Umgelter, Andreas; Fortin, Martin; Phillip, Veit; Schmid, Roland M.; Huber, Wolfgang

doi:10.1186/1757-7241-18-38

Cited by 14 publications

(14 citation statements)

References 21 publications

(18 reference statements)

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“…1 Histopathology of the peripheral microvasculature from patient biopsies has not shown gross anomalies, evidenced of disrupted angiogenesis, or the presence of inflammatory cells, complement, or immunoglobulin to suggest a vasculitis. 3, 26, 49 The absence of persistent structural abnormalities is consistent with a defective, but reversible, barrier dysfunction response of the blood vessels. Several results suggest the presence of humoral factors promoting VEH during disease flares: 1) transient spikes in monocyte/macrophage-associated inflammatory mediators during flares; 2) transient elevation in circulating angiogenic proteins during flares; 51, 61 and 3) impairment of in vitro microvascular endothelial cell (EC) barrier function provoked by acute but not convalescent SCLS sera.…”

Section: Pathophysiology Of Scls: the Role Of Vascular Endothelial Hymentioning

confidence: 73%

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Idiopathic systemic capillary leak syndrome (Clarkson disease)

Druey

Parikh²

2017

Journal of Allergy and Clinical Immunology

140

172

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In 1960, Dr. Bayard Clarkson described a woman experiencing sporadic, recurrent episodes of shock and anasarca. Plasma from an acute attack induced a “shock”-like syndrome when injected into rats. The enigmatic “Systemic Capillary Leak Syndrome” (SCLS) named for Dr. Clarkson is characterized by transient and severe, but reversible, hemoconcentration and hypoalbuminemia due to leakage of fluids and macromolecules into tissues. Although < 500 cases of SCLS have been reported in the literature since 1960, the condition is probably under-diagnosed due to lack of awareness and a high mortality without treatment. Allergists should be vigilant of this diagnosis since its presentation can resemble more common plasma leakage syndromes including angioedema or systemic anaphylaxis. Although the precise molecular etiology of SCLS remains unknown, substantial advances over the last five years have increased our understanding of SCLS pathogenesis.

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Section: Pathophysiology Of Scls: the Role Of Vascular Endothelial Hymentioning

confidence: 73%

“…3 Over the last six years, 134 new cases of SCLS have been identified, 4–49 including a European registry of 28 cases 50 and our NIH cohort of 58 patients. 51 The demographics of these patients are described in Table 1.…”

Section: Incidence Of Idiopathic Sclsmentioning

confidence: 99%

Idiopathic systemic capillary leak syndrome (Clarkson disease)

Druey

Parikh²

2017

Journal of Allergy and Clinical Immunology

140

172

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“…Similar to the course in patients with sepsis, a significant drop in leukocyte and platelet counts was observed in both genotypes 6 h after sepsis induction. With the progression of systemic inflammation and infection, leukopenia and thrombocytopenia, which may function as indicators of disseminated intravascular coagulation, occur (50, 51). Further studies are needed to elucidate the proposed mechanism and to understand the role of aSMase in platelet activation and thrombocytopenia during sepsis.…”

Section: Discussionmentioning

confidence: 99%

Hyperresponsiveness of mice deficient in plasma-secreted sphingomyelinase reveals its pivotal role in early phase of host response

Jbeily

Suckert

Gonnert

et al. 2013

Journal of Lipid Research

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Plasma secretion of acid sphingomyelinase is a hallmark of cellular stress response resulting in the formation of membrane embedded ceramide-enriched lipid rafts and the reorganization of receptor complexes. Consistently, decompartmentalization of ceramide formation from inert sphingomyelin has been associated with signaling events and regulation of the cellular phenotype. Herein, we addressed the question of whether the secretion of acid sphingomyelinase is involved in host response during sepsis. We found an exaggerated clinical course in mice genetically deficient in acid sphingomyelinase characterized by an increased bacterial burden, an increased phagocytotic activity, and a more pronounced cytokine storm. Moreover, on a functional level, leukocyte-endothelial interaction was found diminished in sphingomyelinase-deficient animals corresponding to a distinct leukocytes’ phenotype with respect to rolling and sticking as well as expression of cellular surface proteins. We conclude that hydrolysis of membrane-embedded sphingomyelin, triggered by circulating sphingomyelinase, plays a pivotal role in the first line of defense against invading microorganisms. This function might be essential during the early phase of infection leading to an adaptive response of remote cells and tissues.

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“…The principle of treatment is to control its progression, maintain organ function, maintain effective blood volume, and improve capillary permeability. [ 9 ] For liquid expansion, hydroxyethyl starch is preferred as it can improve endothelial function, reduce inflammation, effectively promote the flow of liquids and thus is often used in the clinic. Glucocorticoids can improve capillary permeability, which can be used to suppress inflammation, and are combined with diuretics to reduce volume load.…”

Section: Discussionmentioning

confidence: 99%

Capillary leak syndrome and aseptic meningitis in a patient with Kawasaki disease

Zhang

wan²,

et al. 2018

Medicine

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Rationale:Kawasaki disease (KD) is an acute vasculitis of childhood, coronary complications are the most serious and classic complications of this disease. However, simultaneous complications such as systemic capillary leak syndrome (CLS) and aseptic meningitis are rarely reported.Patient concerns:A 19-month-old boy had continuous fever for 6 days, rash for 3 days, and somnolence for 1 day.Diagnoses:The boy was diagnosed with KD presenting with SCLS and aseptic meningitis.Interventions:He was treated with gamma globulin (2 g/kg) for 1 day, mannitol and furosemide to reduce intracranial pressure, human albumin to correct hypoproteinemia, methylprednisolone to control inflammation, and both aspirin and dipyridamole for anticoagulation.Outcomes:After treatment, the patient recovered well. At one year follow-up, the patient was asymptomatic and showed no recurrence of skin rash.Lessons:The incidence of KD has recently increased and cardiovascular complications are frequently reported. This may be combined with systemic damage, however, the combination of SCLS and aseptic meningitis is rarely reported, therefor, children who have SCLS, aseptic meningitis and unexplained fever >5 days, KD should be taken into account. Early diagnosis and timely treatment can reduce complications induced by KD.

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Systemic Capillary Leak Syndrome associated with hypovolemic shock and compartment syndrome. Use of transpulmonary thermodilution technique for volume management

Cited by 14 publications

References 21 publications

Idiopathic systemic capillary leak syndrome (Clarkson disease)

Idiopathic systemic capillary leak syndrome (Clarkson disease)

Hyperresponsiveness of mice deficient in plasma-secreted sphingomyelinase reveals its pivotal role in early phase of host response

Capillary leak syndrome and aseptic meningitis in a patient with Kawasaki disease

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