A child at 6 years and 9 months old with thalassemia was admitted to our hospital in September,2020 due to more than 2months of edema and over 1 moth of albuminuria,hematuria at 2 years and 10 months atter hematopoietic stem cell transplantation.After completing examination in our hospital,hypoalbuminemia and massive albuminuria were observed. Renal biopsy pathology indicated stage II membranous nephropathy（MN）,which was diagnosed as secondary MN. Complete remission of urinary protein was achieved after treatment with glucocorticoid combined with tacrolimus.

Aim IgA nephropathy is virtually known as the most common glomerulopathy to end-stage renal failure in the world. Mycophenolate mofetil is a selective immunosuppressant widely used in organ transplantation, yet its tolerance and effectiveness in IgAN is controversial. Methods This is a systematic review and random-effects meta-analysis, searching PubMed, Embase, Te Cochrane Library, Science Citation Index, Ovid evidence-based medicine, Chinese Biomedical Literature and Chinese Science and Technology Periodicals. Screen out randomized controlled trials on patients with biopsy-proven IgA nephropathy and analysis mycophenolate mofetil treatment regimens used for therapy of IgA nephropathy. Complete remission and partial remission, doubling of creatinine level, proteinuria, incidence of end-stage kidney disease, infection, Cushing syndrome, diabetes, hepatic dysfunction or gastrointestinal symptoms, neurologic or visual ambiguity, acne, and alopecia were observed. Results Nine relevant trials were conducted with 587 patients enrolled. In Mycophenolate mofetil or plus medium/low-dose steroid comparing full-dose steroid alone or placebo, there was no significant difference. The risk of Cushing syndrome and diabetes had been significantly lowered with Mycophenolate mofetil-treated patients, while the risk of infection had been increased. Conclusions Mycophenolate mofetil therapy did not differ in reducing proteinuria and Scr in patients with IgAN who had persistent proteinuria, while having fewer Cushing syndrome and diabetes risk and more infection risk. However, larger randomized studies are needed to reveal these results.

Objective:To investigate the correlation among clinical manifestations, pathological changes and immunofluorescence in children with primary IgA nephropathy (IgAN).Methods:The data of a total of 222 cases diagnosed with IgAN by the Department of Nephrology, Jiangxi Children′s Hospital in recent 10 years were collected for the analysis of clinical and pathological features and their correlation.Results:(1)Immunofluorescence showed that 115 cases (51.8%) IgA patients had only mesangial deposition, and 107 cases (48.2%) IgA patients had both mesangial deposition and capillary loop deposition.Most IgA patients (122 cases, 55.0%) had immunofluorescence intensity+ + .In IgA patients, IgM deposition was the most common [168 cases (75.7%)], followed by C 3 deposition [160 cases (72.1%)]. Patients with C 4 deposition were the least[7 cases (3.2%)]. (2) Hypertension were positively correlated with IgM, C 3 deposition, IgA deposition intensity and IgA with vascular loop deposition(all P<0.05). Hyperuricemia was positively correlated with IgM deposition, IgG deposition, C 3 deposition and IgA with vascular loop deposition(all P<0.05); hypoalbuminemia was negatively correlated with IgM deposition, C 3 deposition, IgA deposition intensity and IgA with vascular loop deposition(all P<0.05). Hypercholesterolemia were positively correlated with C 3 deposition and IgA with vascular loop deposition(all P<0.05). Urine protein quantification were positively related to IgM, IgG and IgA with vascular loop deposition (all P<0.05). The estimated glomerular filtration rate(eGFR)was negatively related to IgA with vascular loop deposition( P<0.05). (3) Lee′s grade were positively correlated with IgM and C 3 deposition, IgA deposition intensity and IgA with capillary loop deposition(all P<0.05). (4) Oxford type mesangial hypercellularity(M1)were positively correlated with C 3 deposition and IgA deposition intensity(all P<0.001). Endocapillary hypercellularity(E1)lesions were positively correlated with IgA deposition intensity and IgA with capilla-ry loop deposition(all P<0.05). Segmental glomerulosclerosis(S1)lesions had no correlation with immunofluorescence pathology.Tubular atrophy and interstitial fibrosis(T1)was positively correlated with IgG and C 3 deposition(all P<0.05). (5) There was no correlation between glomerulosclerosis and immunofluorescence pathology.Crescent was positively correlated with IgM deposition, IgG deposition, C 3 deposition, IgA deposition intensity and IgA capillary loop deposition(all P<0.05). Renal artery thickening was positively correlated with IgG deposition and IgA capillary loop deposition(all P<0.05). Conclusions:IgA intensity of + + and IgM deposition are most commonly found in immunofluorescence of children with primary IgAN.IgA patients with capillary loops or C 3 deposition have more severe clinical manifestations and light microscopy results.

>肺炎支原体肺炎(MPP)是儿童临床常见病,多伴肺内、肺外系统的损害,但并发血栓的病例较少,苏州大学附属儿童医院收治1例MPP并左侧上肢动脉血栓。现报道如下。1临床资料患儿,男,5岁4个月。因"发热9 d、咳嗽3 d"入院。患儿于9 d前出现发热,热峰40.2℃;当地医院查胸片:右下肺炎症;血常规:白细胞5.76×10～9/L,中性粒细胞分类0.589,血红蛋白

目的 探讨儿童寡肌病性皮肌炎并发肺间质病变、纵隔气肿的临床特点及诊断和治疗.方法 回顾性分析1例寡肌病性皮肌炎并发肺间质病变、纵隔气肿患儿的临床资料,并复习相关文献.结果 患儿女性,10岁11个月,因面部红斑1个月入院,有活动后气促的表现,肌力Ⅴ级,肌张力正常;有典型皮疹;肌酸激酶稍高;肌电图提示肌源性损害;膝关节磁共振提示双侧股骨后方软组织少许条状STIR序列高信号;高分辨CT (HRCT)示肺间质病变、纵隔气肿.临床诊断为寡肌病性皮肌炎并发肺间质病变、纵隔气肿.予糖皮质激素、环磷酰胺、环孢素、吡菲尼酮及丙种球蛋白冲击等综合治疗,患儿最终死亡.结论 对于合并肌酸激酶正常的寡肌病性皮肌炎患儿,需警惕并发肺间质病变、纵隔气肿可能,后者病死率高,应早期完善肺部CT检查,及早治疗.