A multi-center epidemiological investigation on therapeutic plasma exchange for the children with hemolytic uremic syndrome

Abstract

Objective To study the current status of therapeutic plasma exchange(TPE) for the children with hemolytic uremic syndrome(HUS) in China, and to provide evidence for further standardizing the consensus of diagnosis and treatment. Methods The questionaires were designed after discussion by Pediatric Blood Purification Specialist Committee to investigate the data of 146 hospitalized HUS children with TPE, which were collected and analyzed from January 2012 to December 2017 in 22 units. Results (1)Among the 146 cases, there were 91 males and 55 females, with an average age of 5.68 years old (2 months to 18 years old). The causes included infection(41.1%, 60 cases), complement defects(12.3%, 18 cases), drugs(10.3%, 15 cases), rheumatic diseases(6.2%, 9 cases)and hereditary metabolic diseases(4.1%, 6 cases). (2)Before TPE, the main manifestations of the sick children were oliguria or anuria(101 cases), and disturbance of consciousness(45 cases), hypertension(52 cases), and gastrointestinal bleeding(42 cases), which were significantly relieved after TPE.(3)Before TPE, hemoglobin[(68.26±18.17) g/L], red blood cell count[(2.43±0.70) ×109/L], platelet count[(77.49±72.93) ×109/L], C3[(0.70±0.26) g/L], glomerular filtration rate(GFR)[(26.98±17.16) mL/1.73 m2]were all decreased, and total bilirubin[(44.33±42.07) μmol/L], unconjugated bilirubin [(30.78±26.68) μmol/L], fibrin breakdown products(FDP)[(12.52±14.97) mg/L], D-dimer[(2 306.20±2 354.97) μg/L], creatinine[(305.45±290.19) μmol/L], 24-hour urinary protein [(2.17±2.55) g/d] all increased, the above indicators were significantly improved after the TPE[ (85.08±18.98) g/L, (2.80±0.98) ×109/L, (183.80±131.95) ×109/L, (0.85±0.25) g/L, (98.58±66.02) mL/1.73 m2, (19.71±33.24) μmol/L, (9.60±10.00) μmol/L, (7.33±9.81) mg/L, (1 322.05±1 595.23) μg/L, (139.28±163.75) μmol/L, (1.46±2.03) g/d, respectively], and the differences were all statistically significant (all P<0.05). (4)Complications occurred in 5 cases, including hypotension shock (2 cases), blockage of pipe (1 case), thrombocytopenia (1 case), anemia (1 case). (5)After TPE, 130 cases (89%) were significantly effective, but 16 cases (11%) died. Conclusions TPE for children with HUS has been widely carried out in China, and its effectiveness and safety had been confirmed.The timing of treatment is further confirmed by evidence-based medicine. Key words: Hemolytic uremic syndrome; Therapeutic plasma exchange; Child; Epidemiology