title: [Above-standard proceeding in nephrotic syndrome - case report].
abstract: The nephrotic syndrome is characterized by the loss of many proteins, via the urinary system. It exceeds the bodies compensatory abilities and results in abnormalities in blood clotting system, particularly due to antithrombin deficiency. It significantly increases the risk of thromboembolic complications. A loss of erythropoietin and transferrin leads to anemia. Polycythemia is a rarely reported phenomenon. The case describes a 20-years old patient with massive nephrotic syndrome and polycythemia, complicated by a pulmonary embolism. The patient had a steroid-dependent submicroscopic glomerulonephritis with a severe episode of nephrotic syndrome associated with centralization of circulation, proteinuria 40.9 g/day, deep hypoproteinemia (albumin=1.2 g/dl), hyperlipidemia, hypercoagulable state (antithrombin activity 29%), polycythemia (Hb=21.1 g/dl, HTC=60%). Kidney function parameters were normal. We started the immunosupression (glycocorticosteroids i.v., continuated p.o. and cyclosporine A) and intensive symptomatic treatment. To reverse hypovolemia and polycythemia, 20% albumin solutions, intravenous infusions and diuretics were used. There was no effect. Due to intensive polycythemia the erythroapheresis procedure was performed. It resulted in normalization of the red blood cell count (Hb=13.4 g/dl, HCT=37%) and the improvement of blood circulation. To prevent the patient from thromboembolism, the prophylactic dose of low molecular weight heparin (LMWH) was administered (dalteparin 5000 IU subcutaneously, once a day). Despite the prophylaxis, an episode of dyspnea with tachycardia occured. It was connected with elevated Ddimer and troponin levels and a right ventricle overload in echocardiographic imaging. The pulmonary embolism was suspected. Perfusion lung scintigraphy confirmed this diagnosis. We supposed that the heparin was ineffective due to an antithrombin deficiency. Therefore, apart from a therapeutic dose of LMWH, intravenous antithrombin concentrate was given to the patient (1500 IU twice). The dyspnea resolving was observed. The D-dimer and troponin level reversion to normal was noticed. Heparin injections, connected with antithrombin infusion, was an effective treatment of the pulmonary embolism. Due to the lack of antithrombin in nephrotic syndrome, using only heparin may be insufficient. The erythroapheresis is an effective treatment of polycythemia.
authors.affiliation: Department of Internal Diseases, Nephrology and Dialysis, Central Clinical Hospital of the Ministry of National Defense, Military Institute of Medicine in Warsaw, Poland.
authors.forename: Anna
authors.initials: A
authors.lastname: Matyjek
authors.affiliation: Department of Internal Diseases, Nephrology and Dialysis, Central Clinical Hospital of the Ministry of National Defense, Military Institute of Medicine in Warsaw, Poland.
authors.forename: Dorota
authors.initials: D
authors.lastname: Brodowska-Kania
authors.affiliation: Department of Internal Diseases, Nephrology and Dialysis, Central Clinical Hospital of the Ministry of National Defense, Military Institute of Medicine in Warsaw, Poland.
authors.forename: Stanisław
authors.initials: S
authors.lastname: Niemczyk
mesh_terms: D000991:Antithrombins / Q000627:therapeutic use*; D001781:Blood Component Removal; D017707:Erythrocyte Transfusion; D006495:Heparin, Low-Molecular-Weight / Q000627:therapeutic use*; D006801:Humans; D008297:Male; D009404:Nephrotic Syndrome / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D011086:Polycythemia / Q000209:etiology; D011655:Pulmonary Embolism / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D013923:Thromboembolism / Q000517:prevention & control; D055815:Young Adult
chemical_list: D000991:Antithrombins; D006495:Heparin, Low-Molecular-Weight
keywords: antithrombin III; erythroapheresis; polycythemia; pulmonary embolism; submicroscopic glomerulonephritis