Objective:To investigate the short-term efficacy and safety of rituximab (RTX) in children with calcineurin inhibitor (CNI) resistant steroid resistant nephrotic syndrome (SRNS).Methods:A retrospective case analysis was conducted. Thirteen children with CNI resistant SRNS who were regularly treated with RTX (375 mg/m 2 per dose (maximum dose 500 mg), 1 dose per week, a total of 4 doses) in Department of Nephrology, Children′s Hospital of Fudan University from January 2016 to December 2023 were enrolled. The general data, disease related information, urinary protein/creatinine, serum albumin, blood creatinine before RTX treatment, immunosuppressants, adverse events, and monthly urinary protein/creatinine, serum albumin, and blood creatinine indexes within 6 months after RTX treatment were collected. The changes of urinary protein/creatinine, serum albumin and estimated glomerular filtration rate (eGFR) before and after RTX at 3 and 6 months were analyzed by using paired sample t test and Wilcoxon signed-rank test. Results:Among the 13 patients, 8 were male and 5 were female. The age of disease onset was 4.0 (2.9, 6.8) years and the age of RTX treatment was 9.8 (5.9, 13.6) years. There were 8 cases of focal segmental glomerulosclerosis, 3 cases of minimal change disease and 2 cases of mesangial proliferative glomerulonephritis. No clinically significant gene variation was detected in 12 cases and the other one did not receive gene test. Before RTX treatment, 11 cases were in chronic kidney disease stage G1, and 1 case each was in stage G2 and stage G3. Ten children completed 4 doses of RTX treatment, 1 patient completed 3 doses, and 2 patients completed 2 doses. Urinary protein/creatinine in 13 children at 3 and 6 months after RTX treatment was significantly lower than baseline (0.60 (0.13, 2.04), 0.49 (0.28, 1.10) vs. 1.44 (0.76, 4.11) mg/mg, Z=-2.34, -2.34, both P<0.05), and serum albumin was significantly higher than baseline ((35±8), (34±7) vs. (30±6) g/L, t=2.30, 2.60, both P<0.05). The eGFR at 6 months after RTX treatment was not significantly different from the baseline ((110±32) vs. (113±35) ml/(min·1.73 m 2), t=-0.76, P>0.05)). No serious adverse reactions occurred in this study. Conclusion:RTX could reduce urinary protein and increase serum albumin in short-term treatment in children with CNI resistant SRNS without significant side effects.

Objective:To investigate the short-term efficacy and safety of rituximab (RTX) in children with calcineurin inhibitor (CNI) resistant steroid resistant nephrotic syndrome (SRNS).Methods:A retrospective case analysis was conducted. Thirteen children with CNI resistant SRNS who were regularly treated with RTX (375 mg/m 2 per dose (maximum dose 500 mg), 1 dose per week, a total of 4 doses) in Department of Nephrology, Children′s Hospital of Fudan University from January 2016 to December 2023 were enrolled. The general data, disease related information, urinary protein/creatinine, serum albumin, blood creatinine before RTX treatment, immunosuppressants, adverse events, and monthly urinary protein/creatinine, serum albumin, and blood creatinine indexes within 6 months after RTX treatment were collected. The changes of urinary protein/creatinine, serum albumin and estimated glomerular filtration rate (eGFR) before and after RTX at 3 and 6 months were analyzed by using paired sample t test and Wilcoxon signed-rank test. Results:Among the 13 patients, 8 were male and 5 were female. The age of disease onset was 4.0 (2.9, 6.8) years and the age of RTX treatment was 9.8 (5.9, 13.6) years. There were 8 cases of focal segmental glomerulosclerosis, 3 cases of minimal change disease and 2 cases of mesangial proliferative glomerulonephritis. No clinically significant gene variation was detected in 12 cases and the other one did not receive gene test. Before RTX treatment, 11 cases were in chronic kidney disease stage G1, and 1 case each was in stage G2 and stage G3. Ten children completed 4 doses of RTX treatment, 1 patient completed 3 doses, and 2 patients completed 2 doses. Urinary protein/creatinine in 13 children at 3 and 6 months after RTX treatment was significantly lower than baseline (0.60 (0.13, 2.04), 0.49 (0.28, 1.10) vs. 1.44 (0.76, 4.11) mg/mg, Z=-2.34, -2.34, both P<0.05), and serum albumin was significantly higher than baseline ((35±8), (34±7) vs. (30±6) g/L, t=2.30, 2.60, both P<0.05). The eGFR at 6 months after RTX treatment was not significantly different from the baseline ((110±32) vs. (113±35) ml/(min·1.73 m 2), t=-0.76, P>0.05)). No serious adverse reactions occurred in this study. Conclusion:RTX could reduce urinary protein and increase serum albumin in short-term treatment in children with CNI resistant SRNS without significant side effects.

The paper summarizes the clinical and follow-up data of percutaneous endoscopic gastrostomy (PEG) in three infants with chronic kidney disease to explore the safety and reliability of using PEG to improve the growth and development, and nutritional status. During follow-up, the weight and height of case 1 and 3 were obviously improved. Case 2 was followed up for 3 months, due to dying of cardiac arrest, and the infant's height and weight were not significantly improved. Serum albumin and prealbumin improved in 3 cases after PEG. No PEG-related infection occurred in 3 infants.

Objective:To analyze the clinical and prognosis of children with kidney retransplantation.Methods:Clinical data of 11 children who underwent kidney retransplantation from January 2011 to December 2020 in Department of Nephrology, Children′s Hospital of Fudan University were retrospectilely analyzed. The clinical data including demographic parameters, primary diagnosis, characteristics in the follow-up of renal allograft were analyzed.Results:Totally 11 cases received secondary renal transplantation (male 6, female 5). They were initially diagnosed with chronic kidney disease at the age of 11.9 (7.4, 13.3) years. The median duration of dialysis was 22.1 (3.5, 36.5) months. In the first transplantation, recipient age was 13.9 (11.1, 15.2) years. Ten cases received donation from cardiac death donor (DCD) (9 cases received donors aged less than one year, 5 of them received whole kidney transplantation and one case received donor aged one to three years) and 1 case with living-related donor. Ten graft failures occurred within 1 month after renal transplantation and the other one occurred at the fifth month after transplantation. The causes included vascular factors (9 cases), rejection (1 case) and primary non-function (1 case). In the second transplantation, recipient age was 14.7 (11.7, 16.2) years. All the 11 children received dialysis (7 with PD and 4 with HD) and successfully completed the second transplantation. The median time between the two transplants was 210 (16, 1 041) days. Donors were all DCD donors from 3 years of age or older. The mean follow-up duration was (42±15) months. The estimated glomerular filtration rate was (85±34)ml/(min·1.73 m 2) when the last investigation after kidney retransplantation with the kidney and patient all survived. Conclusions:Kidney retransplantation may have better prognosis in children. Dialysis transition during waiting period and DCD donor from 3 years of age or older can effectively ensure the success of kidney retransplantation.

Objective: To evaluate the quality of life (QOL) of children with uremia who underwent renal replacement therapy (RRT) and identify the influencing factors for QOL in order to improve the QOL of children with uremia. Methods: Children with ESRD who underwent dialysis or kidney transplantation (KT) at Children's Hospital of Fudan University between November 2016 and October 2017 were enrolled. The children and/or their parents completed and returned the Pediatric QOL Inventory Measurement Models (PedsQL^TM) 4.0 questionnaire. Moreover, the clinical data of these children were collected. According to the way of RRT, children were divided into dialysis group and KT group. The differences of scores between two groups were compared. Multiple linear regression analysis was used to analyze the factors affecting the QOL of children. Results: A total of 79 children undergoing RRT were enrolled. Among them, 48 cases in the dialysis group and 31 cases in the KT group. For children in KT group, the total PedsQL scores of child-self and parent-proxy assessment were higher than those in dialysis group (P<0.05). The total scores for the QOL of child-self and parent-proxy assessment were roughly the same for KT children (P>0.05). The total scores for the QOL of child-self and parent-proxy assessment were different for dialysis children (P=0.05). Short stature (height<3th percentile) and elevated left ventricular mass index (LVMI) were the independent influencing factors for the QOL of child-self and parent-proxy assessment in children undergoing KT, respectively (B=12.162, t=2.681, P<0.05; B=-0.240, t=-4.276, P<0.01). Conclusions QOL was higher in children undergoing KT than those on dialysis. Short stature and elevated LVMI were the independent influencing factors for QOL in children undergoing KT.

Anticonvulsants ; therapeutic use ; Child ; Consensus ; Epilepsy ; therapy ; Humans ; Pediatrics ; Practice Guidelines as Topic

OBJECTIVETo study and summarize the etiology of children patients with chronic kidney disease (CKD) stage 2 to 5 seen in Children's Hospital of Fudan University from Jan. 2004 to Dec. 2013.

METHODBy complying with the NKF-K/DOQI guidelines, we collected data of 264 cases of children patients with CKD stage 2-5 from Jan. 2004 to Dec. 2013 in the medical record system of Children's Hospital of Fudan University. And we retrospectively analyzed their age and CKD stage at first diagnosis, primary diseases, complications, etc.

RESULTIn the collected 264 cases, 52 cases (19.7%) were diagnosed at stage 2, 67 (25.4%) at stage 3, 52 (19.7%) at stage 4 and 93 (35.2%) at stage 5. For disease causes, 116 cases (43.9%) had congenital anomalies of the kidney and urinary tract (CAKUT), 61 cases (23.1%) had glomerular disease, 15 (5.7%) had hereditary kidney disease, 14 (5.3%) had other diseases and in 58 cases (22.0%) the causes of disease were unknown. In the group with age between 0 and 3.0 and 3.1 and 6.0 years, 57.1% (24 cases) and 60.0% (30 cases) had primary disease with CAKUT. In the group with age older than 10 years, 49.2% (30 cases) had primary disease with glomerular disease and 32.0% (32 cases) with unknown causes.

CONCLUSIONThe major cause of CKD stage 2-5 in children in our hospital during the last ten years was CAKUT (43.9%), followed by glomerular disease (23.1%). The primary diseases of CKD were significantly different between the 2 age groups. CAKUT was more common in infants and preschool children while for adolescents, glomerular disease was the major cause.

Child ; Child, Preschool ; China ; Humans ; Infant ; Infant, Newborn ; Kidney ; physiopathology ; Renal Insufficiency, Chronic ; etiology ; Retrospective Studies