Capillary leakage complicated by compartment syndrome necessitating surgery

Guidet, Bertrand; Guérin, Bastien; Maury, Éric; Amstutz, P

doi:10.1007/bf01706361

Cited by 31 publications

(12 citation statements)

References 6 publications

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“…Twenty-tivc of these were found to have a paraprotein, whereas only two did not have a paraprotein and in three cases paraproteins were not reported [2][3][4][5]. All paraproteins were IgG, although they differed in subclass and light chains.…”

Section: Discussionmentioning

confidence: 92%

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The paraproteins in systemic capillary leak syndrome

Zhang

Ewan

Lachmann

1993

Clinical and Experimental Immunology

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SUMMARYSystemic capillary leak syndrome (SCI.S) is a rare disease characterized by episodes of collapse due to rapid transfer of considerable volumes of plasma from the intravascular to the extravascular compartment. The pathogenesis of this disease is unknown. The diagnosis is made largely on ehnical grounds, and investigations are unhelpful. The only consistent abnormality is that an IgG paraprotein is found in most patients, raising the possibility that the paraprotein may be invohed in the pathogenesis of the disease. Reduction of the paraprotein level in our patient was associated with remission. Blood samples from three SCLS patients and one probable SCLS have been studied. All patients had monoelonal IgG paraproteins. The pttrified paraproteins were all of IgG 1 subelass and had K light chains. However, they differed in size and charge. Antibodies against each of the paraproteins were raised in rabbits, Alfinity-puritied anti-idiotypie antibodies were tested for crossreaetivity against the other paraproteins using immunoblotting and Ouehterlony assay. These assays showed that the anti-idiotypic antibodies reacted only with the immunizing paraprotein and not with any of the other paraproteins, i,e. thai the paraproteins do not share a common Idiotype. Paraproteins did nol bind t() cultured endothelial cells, either unaetivated or following activation with interferon-gamma (IFN-;-)-lL-2 or IL-6, In addition, we were unable to demonstrate any eytotoxicity towards cultured htmian endothclial cells by paraprotein alone, or in the presence of netJtrophils (pronounced neutrophrlia being ;( fealure of attacks). The relationship betwetn the paraproteins and the disease retnains unclear. It is likely that additional, as yet unidentilied. factors are required for the paraprotein to lead to capillary leak.

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Section: Discussionmentioning

confidence: 92%

“…Systemic capillary leak syndrome (SCLS) is a rare disease, first described in I960 [1], Since then, only 30 cases have been reported [2][3][4][5][6]. SCLS is characterized by episodes of sudden movement of large amounts of plasma, including water, electrolytes and proteins of mol.…”

mentioning

confidence: 99%

The paraproteins in systemic capillary leak syndrome

Zhang

Ewan

Lachmann

1993

Clinical and Experimental Immunology

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“…[43][44][45] Tissue salvage (decompression) therapy was required by 3 of the 5 patients who had developed compartment syndrome, a grave complication of SCLS due to massive fluid leakage into the muscle tissue. [46][47][48] Acute deep venous thrombosis, which occurred in 8% of patients, perhaps resulted from attack-induced activation of the components of the Virchow triad.…”

Section: Discussionmentioning

confidence: 99%

Idiopathic Systemic Capillary Leak Syndrome (Clarkson's Disease): The Mayo Clinic Experience

Jkapoor¹,

Greipp²,

Schaefer³

et al. 2010

SciVee

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Objective: To determine clinical features, natural history, and outcome of a well-defined cohort of 25 consecutive patients with idiopathic systemic capillary leak syndrome (SCLS) evaluated at a tertiary care center. Results: Of the 34 patients whose records were reviewed, 25 fulfilled all diagnostic criteria for SCLS. The median age at diagnosis of SCLS was 44 years. Median follow-up of surviving patients was 4.9 years, and median time to diagnosis from symptom onset was 1.1 years (interquartile range, 0.5-4.1 years). Flulike illness or myalgia was reported by 14 patients (56%) at onset of an acute attack of SCLS, and rhabdomyolysis developed in 9 patients (36%). Patients with a greater decrease in albumin level had a higher likelihood of developing rhabdomyolysis (p=.03). Monoclonal gammopathy, predominantly of the IgG-κ type, was found in 19 patients (76%). The progression rate to multiple myeloma was 0.7% per person-year of follow-up. The overall response rate to the different therapies was 76%, and 24% of patients sustained durable (>2 years) complete remission. The estimated 5-year overall survival rate was 76% (95% confidence interval, 59%-97%).cOnclusiOn: Systemic capillary leak syndrome, a rare disease that occurs in those of middle age, is usually diagnosed after a considerable delay from onset of symptoms. The degree of albumin decrement during an attack correlates with development of rhabdomyolysis. A reduction in the frequency and/or the severity of attacks was seen in nearly three-fourths of patients who were offered empirical therapies. The rate of progression to multiple myeloma appears to be comparable to that of monoclonal gammopathy of undetermined significance. Clin proc. 2010;85(10):905-912 Mayo

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“…The median age for the first SCLS-manifestation is 46 years with no sex-related difference [3]. Hard physical work several days before SCLS-symptoms and flu-like-illness at the beginning of a SCLS-episode has been described in several case reports [3,4]. However the pathogenesis of SCLS is still unknown.…”

Section: Resultsmentioning

confidence: 99%

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

Saugel

Umgelter

Fortin

et al. 2010

Scandinavian Journal of Trauma, Resuscitation and Emergency Med

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Systemic Capillary Leak Syndrome (SCLS) is a rare disorder characterized by increased capillary hyperpermeability leading to hypovolemic shock due to a markedly increased shift of fluid and protein from the intravascular to the interstitial space. Hemoconcentration, hypoalbuminemia and a monoclonal gammopathy are characteristic laboratory findings. Here we present a patient who suffered from SCLS with hypovolemic shock and compartment syndrome of both lower legs and thighs. Volume and catecholamine management was guided using transpulmonary thermodilution. Extended hemodynamic monitoring for volume and catecholamine management as well as monitoring of muscle compartment pressure is of crucial importance in SCLS patients.

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Capillary leakage complicated by compartment syndrome necessitating surgery

Cited by 31 publications

References 6 publications

The paraproteins in systemic capillary leak syndrome

The paraproteins in systemic capillary leak syndrome

Idiopathic Systemic Capillary Leak Syndrome (Clarkson's Disease): The Mayo Clinic Experience

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

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