Fatal diffuse pulmonary arterial thrombosis as a complication of nephrotic syndrome

Abstract

A 21-year-old man was admitted to our hospital because of leg edema. Because laboratory findings revealed massive proteinuria and hypoproteinemia, he was diagnosed as having nephritic syndrome caused by minimal change disease. He was given a continuous heparin infusion and intravenous steroid therapy, at a prednisolone dose of 1 mg/kg per day, and his condition gradually improved. Five months after discharge, the patient's proteinuria relapsed. He was readmitted to our hospital and we restarted anticoagulant treatment with intravenous heparin and 60 mg prednisolone. On the third hospital day, he complained of chest pain with sudden onset and dyspnea. He quickly developed shock and died. The findings of an autopsy confirmed the presence of diffuse fibrin thrombi in bilateral pulmonary arteries, and we diagnosed the cause of death as diffuse pulmonary artery thrombosis. A coagulation test for activated partial thromboplastin time (aPTT) had already shown that aPTT was prolonged before the initiation of treatment. There may have been a deficit of antithrombin III (ATIII) - a cofactor of heparin - because of the proteinuria; thus, the continuous heparin treatment might not have been effective for the prevention of thrombosis. Alternatives to heparin treatment that do not suppress AT III, such as nafamostat mesilate or argatroban, which do not require the presence of AT III for their anticoagulant action, should be considered in cases similar to the that in the patient reported here. In patients with nephrotic syndrome who exhibit altered coagulation test results, the choice of anticoagulation therapy for treatment of the hypercoagulabilty status associated with nephrotic syndrome should be carefully considered.

Popliteal arterial thrombosis in nephrotic syndrome: a case report

“…Diuretics used to decrease the edema due to NS may lead to hemoconcentration and increased risk of thrombosis. [5] Similarly, steroids used in the management of NS, as in our patient, can contribute to thrombosis by altering the level of coagulation factors, thereby aggravating the hypercoagulability. [1] Hypertension, hyperlipidemia, diabetes mellitus, tobacco, and obesity are additional risk factors.…”

“…[1,5] Hypercoagulable state occurs due to changes in the blood level and function of prothrombotic and antithrombotic factors. [1] There is decreased level of factors IX, XI, XII and antithrombin III due to urinary loss.…”

“…[1] There is decreased level of factors IX, XI, XII and antithrombin III due to urinary loss. [5] Similarly, there is increased level of fibrinogen, factors II, V, VII, VIII, and X due to increased synthesis in the liver in response to hypoalbuminemia. [5] Hypoalbuminemia in NS causes intravascular dehydration.…”

“…[5] Similarly, there is increased level of fibrinogen, factors II, V, VII, VIII, and X due to increased synthesis in the liver in response to hypoalbuminemia. [5] Hypoalbuminemia in NS causes intravascular dehydration. Diuretics used to decrease the edema due to NS may lead to hemoconcentration and increased risk of thrombosis.…”

Hereditary hyperhomocysteinemia associated with nephrotic syndrome complicated by artery thrombosis and chronic thromboembolic pulmonary hypertension: A case report

“…In particular, the presence of pulmonary artery thrombosis is often fatal [2]. Hyperhomocysteinemia is another accepted risk factor for pulmonary thromboembolism [3].…”