ABSTRACT
Objectives To detect the level of soluble programmed death 1 (sPD-1) and soluble programmed death ligand 1 (sPD-L1) in serum and urine of children with primary nephrotic syndrome (PNS),and explore its clinical significance.Methods From July 2017 to November 2017,children with PNS admitted to the Children's Hospital Affiliated to Soochow University were divided into onset group (36 cases) and remission group (33 cases).Thirty healthy children who underwent medical examination for enrollment,undersize or overweight in the outpatient department of pediatric health care and inpatient department of Endocrinology were selected as healthy control group.Serum and urine samples were collected,in which the levels of sPD-1 and sPD-L1 were detected by enzyme-linked immunosorbent assay (ELISA).The correlation between serum and urine sPD-1,sPD-L1 levels and lymphocyte subsets,urinary protein were analyzed by Pearson and Spearman correlation analysis.Results The level of sPD-1 in serum was lower in remission group than those in healthy controlgroup [1.60(0.48,8.15) ng/ml vs 7.38(2.15,19.02) ng/ml,P ＜ 0.01].The level of urinary sPD-1 in onset group was higher than that in remission group [1.21(0.61,2.56) pg/μg vs 0.51(0.31,0.97) pg/μg,P ＜0.001] and healthy control group [1.21(0.61,2.56) pg/μg vs 0.82(0.34,1.15) pg/μg,P ＜ 0.01].The levels of sPD-L1 in serum and urine were higher in onset and remission group than those in healthy control group (P ＜ 0.001).The level of sPD-1 in the serum was positive correlated with the numbers of CD3+,CD3+CD4+,CD3+ CD8+ T lymphocytes and CD3-CD19+,CD19+CD23+ B lymphocytes (r=0.537,0.478,0.454,0.429 and 0.374;P=0.002,0.008,0.012,0.018 and 0.042).The level of sPD-1 in the urine had positive relation with the ratio of 24 hours urinary albumin and weight (24 h UmAlb/Wt),N-acetylglucosaminidase and urinary creatinine (UNAG/Cr) and β2 microglobulin and urinary creatinine (Uβ2MG/Cr) (r=0.409,0.588 and 0.276;P=0.016,0.000 and 0.032).Conclusions The dynamic changes of sPD-1 and sPD-L1 in serum and urine suggested that PD-1/PD-L1 signaling pathway is involved in the development process of childhood primary nephrotic syndrome.
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Objective To discusses the effectiveness of tocilizumab in the treatment of hypomyopathic dermatomysositis (HDM) combined with interstitial lung disease (ILD) in children. Methods The clinical characteristic, treatment, and prognosis of HDM combined with ILD were analyzed in 2 patients. The related literatures were reviewed. Results Both ten-year-old girl and 8-year-old boy had shortness of breath after activities, but had no clinical manifestations of muscle damage; both of them had typical rash, but had nornal muscle strength and muscular tension. Laboratory tests showed the elevation of serum ferritin, lactate dehydrogenase, glutamate aminotransferase, and aspartate aminotransferase. Creatine kinase slightly increased in the initial test, and then was in the normal range in the following tests. The high resolution computed tomography showed that pulmonary interstitial lesions. HDM combined ILD was diagnosed clinically. The girl died after treatment with high-dose hormones, cyclophosphamide, cyclosporine, pirfenidone, and gamma globulin failed. The boy was stabled after conventional hormone treatment plus tocilizumab (240 mg twice). His laboratory indicators were in the normal range in the follow-up. Conclusions The clinical manifestations and laboratory indicators aren't typical in childhood HDM. The mortality is high. Combined with tocilizumab treatment is effective in one case.
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Objectives To investigate the etiology, renal pathology, treatment, and prognosis of children's urinary system injury after hematopoietic stem cell transplantation (HSCT). Methods Clinical data of 81 children with urinary dysfunction after HSCT admitted to the Hematology Department in Children's Hospital of Soochow University were analyzed, and relevant literatures were reviewed. Results In 81 cases (50 males and 31 females), the age ranges from 8 months to 17 years old. Thirty cases (37%) with prerenal injury were recovered after active rehydration and other symptom specific treatment. There were 9 (11.1%) children with renal injury, four cases were given up therapy or transferred to other hospitals, thus lead to an unknown prognosis. Kidney biopsy was performed in the remaining five cases for pathological investigation. After active symptom-speific and etiology-based treatment, serum creatinine and glomerular filtration rate of four cases return to normal. But in the long-term follow-up,one case died of recurrence of primary disease, reinfusion of hematopoietic stem cell combined with renal failure. The remaining 3 patients were with chronic kidney disease (CKD). One case with renal thrombotic microangiopathy was in the chronic dialysis. Postrenal renal injuries were mainly hemorrhagic cystitis (28.4%) and urinary tract infection (16%). After a large dose of rehydration, urine alkalization and anti-infection therapy, they were recovered in the short term with a good prognosis. Conclusions Urinary injury after HSCT is mainly divided into three categories: prerenal, renal and postrenal, in which renal injury is prone to frequent recurrence.