ABSTRACT
Objective:To analyze the clinical characteristics of children diagnosed with systemic lupus erythematosus(SLE)complicated with thrombotic microangiopathy(TMA)for early recognition.Methods:We retrospectively reviewed the clinical records of 14 SLE patients with TMA hospitalized at Shanghai Children′s Medical Center, Shanghai Jiaotong University School of Medicine from December 2005 to October 2020.Results:The incidence of TMA was 5.65%(14/248)of the hospitalized patients with SLE and 7.87%(14/178)of the hospitalized patients with lupus nephritis.Four patients were boys while ten patients were girls.One boy was six years old and other 13 patients were from 11 to 18 years old.Their SLEDAI scores ranged from 14 to 31, and all of them were severe activity.Renal biopsy of 11 patients during TMA course all revealed lupus nephritis(type Ⅳ, n=8; type Ⅳ+ Ⅴ, n=3). These 14 SLE children were diagnosed with TMA within 3 days to 2 months after admission.At the beginning of the hospitalization, only six patients had both anemia and thrombocytopenia, while eight patients only had moderate anemia.All of the patients had obvious hypocomplementemia.Especially in the patients with first onset of SLE without treatment, their serum levels of C3 were less than 0.17 g/L and C4 were less than 0.07 g/L.Moreover, glomerular filtration rates of these patients were lower than that in normal range.The follow-up time were 0.2-11.3 years(median time was 2.6 years). After treatment, six patients obtained complete remission, and five patients obtained partial remission.One patient had sudden death during the 4th plasmapheresis, and the other two patients deteriorated. Conclusion:Children with SLE and TMA are mostly in severe disease activity, and renal pathology is type Ⅳ lupus nephritis.The SLE children with anemia should be paid special attention to the level of serum complement whether they have thrombocytopenia or not.If the level of serum complements decrease obviously, glomerular filtration rates should be monitored closely and schistocytes should be searched repeatedly in the blood smears of the peripheral blood to facilitate the early recognition of TMA.
ABSTRACT
Objective To explore the diagnosis and treatment of childhood primary Sjogren syndrome (pSS) with central nervous system symptoms as the first manifestation. Methods The clinical data of an 11-year-old male with pSS which had onset with fever and headache was retrospectively analyzed. The related literatures were reviewed. Results The subject was diagnosed with pSS by autoantibody detection, lip biopsy, and ophthalmologic examination. The symptoms were improved after immunosuppressive therapy. According to the literature, the incidence of childhood pSS was low, in which the incidence with involvment of the nervous system as primanry manifestation was even lower, and headache is the most common symptom of central nervous system. The application of corticosteroids and immunosuppressants may help improve the conditions. Conclusions Involvement of central nervous system in childhood pSS is not typical. Detection of autoantibodies and lip biopsy are helpful for diagnosis.
ABSTRACT
Objective To explore the diagnosis and treatment of childhood primary Sjogren syndrome (pSS) with central nervous system symptoms as the first manifestation. Methods The clinical data of an 11-year-old male with pSS which had onset with fever and headache was retrospectively analyzed. The related literatures were reviewed. Results The subject was diagnosed with pSS by autoantibody detection, lip biopsy, and ophthalmologic examination. The symptoms were improved after immunosuppressive therapy. According to the literature, the incidence of childhood pSS was low, in which the incidence with involvment of the nervous system as primanry manifestation was even lower, and headache is the most common symptom of central nervous system. The application of corticosteroids and immunosuppressants may help improve the conditions. Conclusions Involvement of central nervous system in childhood pSS is not typical. Detection of autoantibodies and lip biopsy are helpful for diagnosis.
ABSTRACT
Objective To study the role of urinary kidney injury molecule-1 (KIM-1) in pediatric Henoch-Sch?nlein purpura (HSP). Methods Urinary levels of KIM-1 were examined using ELISA in 48 children with HSP including 23 HSPN children (HSPN group) and 25 non-HSPN children (HSP group), and 20 healthy children. The levels of urinary creatinine and 24-hour urine protein were also detected. The results were analyzed and compared among groups. Results The ratio of urinary KIM-1/creatinine (Cr) in HSPN children was signiifcantly higher than that in the other two groups (P0.05). The ratio of urinary KIM-1/Cr had no correlation with 24-hour urine protein in all HSP children (r=0.239, P=0.590). Conclusions Urinary KIM-1 may play a role in the pathogenesis of pediatric HSPN.
ABSTRACT
Objective To evaluate the value of immune cell functional assay (ImmuKnow CD4+ T cell ATP assay) in monitoring immune status in renal recipients.Methods A total of 131 adult renal transplant recipients who received transplantation for the first time were under investigation.According to the dynamic monitoring ATP concentration before operation,2 week,1,3,6 months after operation and during infect or rejection,samples were divided into the following groups:health control group (HC),pretransplant (Pre-Tx) group,stable (Tx) group,infect group,acute rejection (AR) group,acute kidney injury (AKI) group.Immune cell functions were detected by ImmuKnow CD4+ T cell ATP assay.Lymphocyte subsets (CD4+/CD8+) were analysed and serum concentrations of FK506 were tested.Mixed lymphocyte reaction(MLR) was analysed.Results The ATP concentration was no significant difference between Pre-Tx and HC group.The ATP concentration of 2 weeks,1 months after operation were significantly higher than Pre-Tx group (P ＜ 0.01).After 3 months,6 months follow-up,the ATP concentration stabilized with time.The ATP concentration of AR group was significantly higher than other three groups (Tx,infect and AKI group,all P ＜ 0.05).The correlation coefficient between the ATP concentration and MLR,CD4+/CD8+,FK506 level were R2=0.0072,R2=10-6,R2=0.004 respectively (all P ＞ 0.05).Conclusions The cell-mediated immunity of recipients is relatively strongger during the first month after transplantation.The ATP concentration is not related to the levels of MLR,CD4+/CD8+,FK506.ImmuKnow ATP assay is a valuable predictor in acute rejection diagnosis.
ABSTRACT
Objective To explore the efficacy of mycophenolate mofetil-based induction or maintenance therapy for children with lupus nephritis combined with Calcineurin inhibitors (cyclosporine A/tacrolimus) for resistant cases.Methods the clinical data were analyzed for 10 patients treated with multi-target therapy (Glucocorticoid + Mycophenolate mofetile+ Cyclosporine A/Tacrolimus) in Department of Nephrology and Rheumatology from January 2005 to December 2011.Calcineurin inhibitor (Cyclosporine A/Tacrolimus) was added when Mycophenolate mofetil-based induction or maintenance protocol was not satisfactory.The data was analyzed by repeated measures data of ANOVA.Results The SLEDAI score (10.9±3.8 vs 7.9±2.6),24 hours urinary protein quantification [(4.0±2.0) vs (2.2±1.8) g] and ESR [(78±10) vs (54±8) mm/l h] were decreased significantly (P＜0.01),while serum albumin [(29±8) vs (34±8) g/L] and complement C3 level [(0.41±0.20) vs (0.70±0.30) g/L] were increased significantly (P＜0.05).The complete remission rate (CR) was 70％ and the response rate was 90％ in the first 6 month,and the CR was 90％ in the last month.Except 1 case withdrawal,the follow-up period for 9 cases was 24 months.Adverse reactions were rare,1 case had lung in-fection,1 case had hyperglycemia and 1 case had transaminase elevation,which disappeared after mana-gement.Conclusion Mycophenolate mofetil combined with Calcineurin inhibitors (cyclosporine A/tacrolimus) is effective for treating lupus nephritis,the dose of induction or maintenance period is low,and adverse reactions are rare.