Effect of therapeutic plasma exchange on myasthenia gravis patients in children / 中国小儿急救医学

ABSTRACT

Objective:To investigate the effects of therapeutic plasma exchange(TPE)as adjuvant therapy in children with myasthenia gravis(MG)in pediatric intensive care unit(PICU).Methods:A retrospective study was conducted in 7 children with MG admitted to PICU at Shanghai Children′s Hospital from January 2016 to December 2019.TPE was performed on unsatisfactory effect of acetylcholinesterase inhibitors, glucocorticoids or IVIG.The TPE dose was 50-70 mL/kg for 2 to 3 times for each case.The clinical symptoms, anti-acetylcholine antibody(AChR-Ab)level and prognosis were measured before and after TPE.Results:Seven children with myasthenia gravis admitted to PICU from January 2016 to December 2019, including 4 cases of systemic myasthenia gravis(1 case of myasthenia crisis with respiratory failure)and 3 cases of ocular myasthenia gravis.The AChR-Ab level decreased from 1.66(0.99, 3.33)nmol/L before TPE to 0.66(0.40, 10.97)nmol/L after TPE( Z=-2.545, P=0.011). The symptoms of muscle weakness and blepharoptosis were partially or completely relieved in 7 cases.There were no significantly changes in the levels of circulating immune complex, complement C3, CD4 + , CD8 + and NK cells before and after TPE(all P>0.05). During the process of TPE, 2 cases had mild rash, and 1 case had hypotensive shock, which were recovered after timely treatment.After TPE, the fibrin levelsdecreased from 1.90(1.40, 2.40)g/L to 1.10(1.00, 1.30)g/L( Z=-3.092, P=0.002). Conclusion:TPE reduce the AChR-Ab levels and improve the short-term symptoms in children with myasthenia gravis who have failed conventional treatment.TPE may be an optional therapy for pediatric severe MG.