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We present a patient with spontaneous compartment syndrome of the upper extremity in association with nephrotic syndrome and transient bacteremia, of which the exact cause of compartment syndrome was unclear. This is an extremely rare occurrence, and index of suspicion should be maintained even in the absence of trauma when a patient presents with acute swelling of the upper or lower extremity together with nephrotic syndrome.

Spontaneous compartment syndrome of the forearm in association with nephrotic syndrome and transient bacteremia