Clinical analysis of children having primary nephrotic syndrome complicated with posterior reversible encephalopathy syndrome on treatment of immunosuppressants

Abstract

Objective To explore the relationship between posterior reversible encephalopathy syndrome (PRES) and the treatment of immunosuppressants such as cyclosporine A (CsA) and tacrolimus (FK506) in children with nephrotic syndrome. Methods The clinical data of nephrotic syndrome children with PRES caused by immunosuppressants who were hospitalized in Guangzhou First People's Hospital from June 2014 to May 2017 were collected. Their clinical characteristics, imaging features, treatments and prognosis were analyzed. Results A total of 23 children were enrolled, including 13 children with CsA and 10 children with FK506. In the concurrent of PRES 20 cases were in the activity stage of nephrotic syndrome, with large amounts of urinary protein, obvious edema, hypoalbuminemia and hyperlipidemia; while 3 cases were in the remission of nephrotic syndrome. The main clinical symptoms of PRES were hypertension, headache, epileptic attack, consciousness disorder, visual disorder and so on. Sixty-nine point six percent of children were using high dose immunosuppressive agents, and 78.3% had high drug concentration. The cranial magnetic resonance imaging (MRI) results of 17 patients showed that they had T1 weighted (T1WI) hypointense, T2 weighted (T2WI) and fluid-attenuated inversion recovery (FLAIR) images hyperintense, as well as iso-and slight hypointense of diffusion-weighted image (DWI) in parietal-occipital regions or complicated with frontal lobes or basal nuclei region. Computer tomography (CT) examinations of 6 cases showed low-density focus of the occipital lobes. Children were relieved muscular spasm, debased intracranial hypertension, improved circulation, discontinued or reduced immunosuppressants at the onset of PRES. After these treatments, 21 patients' symptoms and signs disappeared within one week; two patients suffered convulsions 2 times in one week, but recovered after one month. After three months 5 children had MRI and CT re-examination and it showed that their brain lesions disappeared. Conclusions PRES may be related to the dose and blood concentration of immunosuppressive agents. The immunosuppressants for nephrotic syndrome children should be increased gradually with low initiating doses. Physicians need to be precautious to prevent the occurrence of PRES once neurological symptoms occur. Key words: Nephrotic syndrome; Posterior leukoencephalopathy syndrome; Immunosuppressive agents; Children