[Short-term efficacy of dapagliflozin in children with hereditary proteinuric kidney disease].

Objective: To explore the short-term efficacy and safety of dapagliflozin in children with hereditary proteinuric kidney disease. Methods: This was a prospective cohort study. From August 2020 to December 2021, 23 children with hereditary kidney disease from Children's Hospital of Fudan University were enrolled. Patients received dapagliflozin 5 mg/d (weight≤30 kg) or initial dose 5 mg/d for 1 week, then 10 mg/d (weight>30 kg) and the dose of angiotensin converting enzyme inhibitors was stable during treatment. Clinical data including demographic parameters, primary diagnosis, estimated glomerular filtration rate (eGFR), 24 h proteinuria and characteristics in the follow-up were collected. The primary outcome was the change in 24 h proteinuria at 12 (±2) weeks, secondary outcomes included changes of 24 h proteinuria at 24 (±2) weeks, eGFR at both 12 (±2) and 24 (±2) weeks. The data were analysed by using mixed linear model. Results: Totally 23 patients were enrolled, including 16 males and 7 females. The age was (10.8±2.9) years. The primary diseases were Alport syndrome (12 cases), Dent disease (5 cases), proteinuria (4 cases), and focal segmental glomerulosclerosis (2 cases) respectively. Primary outcome showed that 24 h proteinuria decreased from baseline at 12 (±2) weeks during treatment (1.75 (1.46, 2.20) vs. 1.84 (1.14, 2.54) g/m2, P<0.05). Secondary outcomes showed that there was no significant difference in 24 h urine protein at 24 (±2) weeks (P>0.05). eGFR decreased slightly at 12 (±2) weeks ((107±21) vs. (112±28) ml/(min·1.73m2), P<0.05), and there was no significant difference at 24 (±2) weeks (P>0.05). Serum albumin increased at 12 (±2) and 24 (±2) weeks following the treatment ((39±8) vs. (37±8) g/L, (38±7) vs. (37±8) g/L, both P<0.05). No hypoglycemia event was reported during the treatment. Conclusion: The dapagliflozin had therapeutic effects on decreasing proteinuria and increasing serum albumin in short-term treatment in children with hereditary proteinuric kidney disease, no hypoglycemia or serious adverse events were observed.

[Clinical and prognosis analysis of children with kidney retransplantation].

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 m2) 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.

[Analysis on the status quo and influencing factors of career management of operating room nurses].

Objective: To investigate the status quo of operating room nurse career management in Shandong Province, and to analysis its influencing factors. Methods: In February 2019, 1418 operating room nurses in Shandong province were selected to investigate their general situation and current status of career management and analyze possible influencing factors by using the General Information Questionnaire, the Career Management Questionnaire for Nurses and the Career Planning Questionnaire for Nurses. Results: The total score of organizational career management scale was (48.24±11.12) points, with the score of dimension from high to low as valueing training (12.64±2.54) points, justice in promotion (12.11±3.04) points, providing career information (12.10±3.21) points, promotion in career self-development (11.39±3.46) points. The total score of organizational career planning scale was (43.33±9.00) points. Multiple liner regression analysis showed that career planning (ß=0.742, P<0.001) , hospital grade (ß=-0.068, P<0.001) and age (ß=-0.065, P<0.001) were influencing factors of operating room nurse career management which could explained 57.1% of the variation (F=630.596, P<0.001) . Conclusion: Career management of operating room nurse is at a medium level, the scores of different dimensions are not balanced.Career planning score, hospital level, age and other factors affect the career management score of operating room nurses.

[Introduction of joint model and its applications in medical research].

In medical follow-up studies, longitudinal data and survival data are often accompanied and associated with each other, thus respective analysis of longitudinal and survival data might lead to biased results. Joint model can correct deviations, improve the efficiency of parameter estimation and provide effective inferences by simultaneously processing longitudinal and survival data. It is a popular method in medical research. Joint model has made much progress, whereas the literature about the joint model and its application is limited in China. This paper summarizes the main idea, basic framework, parameter estimation methods of random effect joint model and introduces the analysis on AIDS data set based on the R software package 'JM' to clarify the advantages of the joint model in processing medical follow-up data and promote the use of the joint model in clinical research.

[Coenzyme Q(10) treatment for one child with COQ6 gene mutation induced nephrotic syndrome and literature review].

Objective: To summarize the clinical manifestation and molecular characteristics of COQ6 mutation induced nephrotic syndrome, and to evaluate efficacy of CoQ(10) therapy. Method: Clinical data of the case with infantile nephrotic syndrome was summarized, including clinical manifestations, laboratory findings and family investigation. The patient received CoQ(10) 30 mg/(kg·d) therapy. Urine protein/creatinine ratio, serum albumin and creatinine were detected to assess the efficacy of the therapy. Result: (1) The 10 months old boy was presented with nephrotic level proteinuria and hypoalbuminemia. Extra-renal manifestations included cardiovascular abnormality, motor and mental retardation and unilateral ptosis. The patient had no consanguinity. A novel homozygous p. R360W mutation in COQ6 gene was identified and confirmed by next-generation sequencing and Sanger sequencing, respectively. Family analysis showed that homozygous p. R360W mutation in COQ6 gene was inherited from his parents. Missense p. R360W mutation was damaging by prediction online PolyPhen and SIFT software. After 2 months of CoQ(10) complementary therapy, the patient's urine protein/creatinine ratio declined from 7.2 to 1.3, and decreased further to 0.01 mg/mg with normal albumin level and renal function within 3 months. Nephropathy remission was maintained and growth retardation improved significantly during the last follow-up. Nevertheless, the patient manifested with sensorineural deafness at the age of 2 years. (2) There were 6 different mutations in coenzyme Q(10) biosynthesis monooxygenase 6 (COQ6) in 13 individuals from 7 families by homozygosity mapping in the whole world. Each mutation was linked to early-onset SRNS with sensorineural deafness. Renal biopsy revealed FSGS in 7 cases and DMS in 1 case. Other manifestations included ataxia, seizures, facial dysmorphism, nephrolithiasis and growth retardation. Four patients received CoQ(10) supplementation and responded to the treatment. Conclusion: Renal disease caused by recessive COQ6 gene mutation was nephrotic syndrome. The patient benefited from early CoQ(10) complement and reached nephropathy remission.

[Clinical analysis of 39 cases who received transplantation of kidney from pediatric organ donors].

OBJECTIVE: To explore the feasibility and clinical significance of pediatric renal transplantation using organ donations from pediatric patients donors. METHOD: Clinical data of children who underwent renal transplantation using organ donations from pediatric patients between September 2011 and December 2014 were retrospectively analyzed, including diagnosis and treatment before transplant, transplant operation, immunosuppression, complication and prognosis. RESULT: Totally 39 cases received pediatric organ donation-pediatric kidney transplantation. The median age at transplantation was 10.5 years. Before transplantation, 37 cases received chronic dialysis. The median waiting time for transplantation was 15 months. The 39 cases received kidneys from 29 donors who were aged from 9 days to 7 years (median 13 months). The median duration of follow-up after the transplantation was 19 months. At 3 month after transplantation and latest follow-up, the length of graft increased (11.3±6.4) mm and (17.5±10.8) mm, respectively. At 6 month and 12 month after transplantation, the height of the recipients increased (5.8±3.5) cm and (15.0±3.5) cm, respectively. At latest follow-up, the serum creatinine level was (80.3±31.9) µmol/L and the eGFR was (94.4±32.9) ml/(min·1.73 m(2)). The recipient survival rate was 100% and grafts survived in 34 cases; 3 cases lost graft function due to thrombosis of renal vessels and the other 2 cases due to bleeding and chronic allograft nephropathy. Donor age of less than 12 months carried higher risk of thrombosis/hemorrhagic complication (P=0.042) and graft dysfunction (P=0.017). CONCLUSION: Favorable outcome can be obtained from pediatric organ donation-pediatric kidney transplantation. Patient survival rate reached 100% at one-year and three-year graft survival rate was 87%. The size of the allograft would increase and the height of the recipients would improve after transplantation.