Compartment syndrome of multiple limbs: An unusual presentation

Milner, Chris S.; Wagstaff, Marcus J. D.; Rose, Gabriel

doi:10.1016/j.bjps.2006.03.061

Cited by 7 publications

(5 citation statements)

References 7 publications

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“…Acute renal failure, noted in nearly half of patients, is a common complication and a consequence of hypotension-related reduced kidney perfusion, acute tubular necrosis, and rhabdomyolysis-related pigmenturia. [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

Kapoor

Greipp

Schaefer

et al. 2010

Mayo Clinic Proceedings

138

135

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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.

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Section: Discussionmentioning

confidence: 99%

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

Kapoor

Greipp

Schaefer

et al. 2010

Mayo Clinic Proceedings

138

135

View full text Add to dashboard Cite

show abstract

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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“…It was also found to be associated with rhabdomyolysis and compartment syndrome [53]. Compartment syndrome of all four limbs has been described which was effectively treated by fasciotomies, but the syndrome itself has a poor prognosis and predisposition to multiple myeloma and leukemia [54]. SCLS presents in three phases: prodromal, extravasation, and recovery [55].…”

Section: Systemic Diseasesmentioning

confidence: 99%