ABSTRACT
Background: The nephortic syndrome (NS) is associated with a hypercoagulable state and an increase tendency for thromboembolism. The reported incidence of thromboembolic complications ranged from 1.8% to 6.6% in children with NS. Although thromboembolism occur anywhere, deep vein thrombosis and pulmonary embolism are most frequently encountered in the clinical setting. Sever pulmonary embolism is a critical condition which needs urgent intervention, but minor embolism to the pulmonary arteries may not induce clinical symptoms, and occurs in up to 28% of patients, Recurrent pulmonary embolism may induce increased pulmonary pressure.
Objectives: Assessment of the pulmonary arterial pressure in children with the NS to find out whether it is a problem in such a category of patients.
Methods: Complete blood picture, serum total protein, albumin and cholesterol, total protein in 24 hours urine, coagulation profile and Doppler echocardiography was performed in 40 children with NS (aged 2-14 years). 17 NS responsive patients, 12 NS dependent patients, 11 NS resistant patients and 20 normal controls. Pulmonary pressure was estimated by measuring the systolic the transtricuspid gradient from tricuspid regurgitation.
Results: All patients had edema, heavy proteinuria, 6.98 ± 3.58 g/24 hrs, hypoalbuminaemia, serum albumin was 1.92 ± 0.48 g/dl, and hypercholestrolaemia 462.23 ± 92.84 mg/dl. Serum creatinine was 1.12 ± 0.80 mg/dl. Total Platelet count was 32.6 ± 9.9 x 104/mm³, PT 13.65 ± 1.31, PTT 34.32 ± 3.60 seconds and prothrombin fragments I & II 1.59 ± 0.46. Thirty seven of the 40 patients with NS had measurable tricuspid regurgitation with a pulmonary systolic pressure ranging from 26 to 52 mmHg. Pulmonary systolic pressure was > 40 mmHg in seven patients.
Conclusion: Pulmonary arterial pressure was increased in children 9with NS , so we recommend further studies to evaluate the etiology and clinical effects of this abnormality.