The value of bioimpedance analysis in the assessment of hydration and nutritional status in children on chronic peritoneal dialysis.

Bioimpedance analysis (BIA)-body composition monitoring (BCM) has been used to evaluate the hydration and nutritional status of adults and children on dialysis. However, its clinical application still has challenges, so further exploration is valuable. We used BIA-BCM to evaluate the hydration and nutritional status of children undergoing chronic peritoneal dialysis from 1 July 2021 to 31 December 2022 in the Children's Hospital of Fudan University to explore the clinical value of this method. A total of 84 children on chronic peritoneal dialysis (PD) were included. In the PD group, 16 (19.05%) and 31 (36.90%) had mild and severe overhydration (OH), respectively; 41.27% (26/63) had a low lean tissue index (LTI). In the PD group, patients with relative OH (Re-OH) > 5.6% had significantly higher systolic blood pressure (SBP) and SBP z score (SBPz). Patients with LTI > 12% had significantly higher body mass index (BMI) and BMI z score (BMIz). Canonical correlation analysis indicated a linear relationship (ρ = 0.708) between BIA-BCM hydration and the clinical hydration indicator and a linear relationship (ρ = 0.995) between the BIA-BCM nutritional indicator and the clinical nutritional indicator. A total of 56% of children on chronic peritoneal dialysis had OH, and 41% had a low LTI. In PD patients, SBP and SBPz were correlated with BIA-BCM Re-OH, and BMI and BMIz were correlated with BIA-BCM LTI. BIA-BCM indicators have good clinical value in evaluating hydration and nutrition.

Risk factors for breakthrough urinary tract infection in children with vesicoureteral reflux receiving continuous antibiotic prophylaxis.

BACKGROUND: To investigate the risk factors for breakthrough urinary tract infection (BT-UTI) in children with vesicoureteral reflux (VUR) receiving continuous antibiotic prophylaxis (CAP). METHODS: This was a single-centre cohort study (January 2016 to December 2019). The clinical data of 256 children with grade I-V VUR receiving CAP were analysed. In this study, exposure variables were sex, younger age at the initial diagnosis of UTI ≤12 months, high-grade VUR, bilateral VUR, aetiology, presence of renal scarring at the initial diagnosis, presence of renal function impairment at the initial diagnosis, ultrasound abnormalities, antibiotic used and bladder and bowel dysfunction (BBD). Outcome was BT-UTI. RESULTS: BT-UTI occurred in 81 out of 256 children with grade I-V VUR who received CAP, an incidence of 31.64%. Univariate analysis showed that younger age at the initial diagnosis of UTI (≤12 months), bilateral VUR, renal scarring on the dimercaptosuccinic acid (DMSA) scan at the initial diagnosis of UTI and BBD were correlated with the occurrence of BT-UTI. Multivariate analysis showed that younger age at the initial diagnosis of UTI (≤12 months) [hazard ratio (HR): 4.629; 95% confidence interval (CI): 1.302-16.462], bilateral VUR (HR: 2.078; 95% CI: 1.084-4.022) and BBD (HR: 3.194; 95% CI: 1.243-8.206) were independent risk factors for the occurrence of BT-UTI. CONCLUSIONS: For VUR children receiving CAP, younger age at the initial diagnosis of UTI (≤12 months), bilateral VUR, and BBD were independent risk factors for the occurrence of BT-UTI.

Reduction in peritonitis rates: 18-year results from the most active pediatric peritoneal dialysis center in China.

BACKGROUND: Most of the available epidemiological data on peritonitis have been derived from developed countries. Limited data from China have been reported. METHODS: An 18-year (2001-2018) peritoneal dialysis (PD) program at the Children's Hospital of Fudan University was described, and data on peritonitis were retrospectively analyzed. RESULTS: Since 2001, a program with a comprehensive PD care bundle has been developed, and 283 patients (53.7% male, median age 9.3 years) were enrolled between 2001 and 2018. Among these patients, 117 peritonitis episodes occurred in 68 (24.0%) patients over 4896 patient-months. The peritonitis rate decreased 20-fold from 2.2 episodes per patient-year in 2003 to 0.11 episodes in 2018. The culture-negative rate decreased from 68.7% during 2001-2006 to 18.5% during 2013-2018, and the proportion of gram-negative and fungal infections increased significantly from 6.6 to 33.8% and 0 to 9.2%, respectively (p < 0.001). Short stature as height ≤ - 2 SD (OR 2.35, 95% CI 1.30-4.24, p = 0.005) and PD duration ≥ 1 year (OR 3.38, 95% CI 1.76-6.49, p < 0.001) were independently associated with a higher risk of developing peritonitis. Of the 117 peritonitis episodes, 9.4% required permanent removal of the catheter, among which half were fungal infections. Patients with peritonitis had a higher risk for PD technique failure (p = 0.006), but there was no difference in estimated patient survival rates and no patient death due to peritonitis. CONCLUSIONS: With the successful development of the PD program and care bundles per the International Society of Pediatric Dialysis (ISPD) guidelines, peritonitis rates have been tremendously reduced in the most active pediatric PD center in China. Growth deficits and a long PD duration were risk factors for developing peritonitis, requiring further close monitoring for a better outcome. A higher resolution version of the Graphical abstract is available as Supplementary information.

Clinical Features and Risk Factors of Fungal Peritonitis in Children on Peritoneal Dialysis.

Objective: To analyse the clinical manifestations, aetiology, prognosis, and risk factors of fungal peritonitis (FP) in children on peritoneal dialysis (PD). Methods: Among 322 children undergoing PD at Children's Hospital of Fudan University, between January 2001 and December 2019, FP cases were retrospectively analysed and compared with those of bacterial peritonitis (BP) to analyse the risk factors of FP. Results: A total of 124 cases of peritonitis were treated, including 11 FP cases in 11 children (0.0019 episodes/patient*month) and 113 BP cases in 64 children (0.02 episodes/patient*month). Among the 11 FP cases, 7 cases (63.64%) were caused by Candida and Candida parapsilosis (5/7) was the most common pathogen of Candida. All FP patients were converted to haemodialysis (HD) and did not resume PD during follow-up. Two patients (18.2%) died after 6 months of HD due to heart failure, 2 patients underwent kidney transplant after 2 years of infection, and the other 7 patients were still on HD. The univariate analysis showed the usage rate of antibiotics in the month before the onset of peritonitis was higher (45.45 vs. 15.93%) and the mean serum albumin was lower (31.4 vs. 34.4 g/L) in the FP group when compared with BP group (P < 0.05), while multivariate analysis showed that serum albumin ≤ 30 g/L was an independent risk factor for FP (odds ratio 4.896, 95% confidence interval 1.335-17.961). Conclusion: FP is a rare complication of PD in children, but it is associated with high technique failure. Attention should be paid to hypoproteinaemia and antibiotic use in children on PD.

Risk factors for loss of residual renal function in children with end-stage renal disease undergoing automatic peritoneal dialysis.

BACKGROUND: This study analysed children with end-stage renal disease treated with automated peritoneal dialysis (APD) in our centre to explore the risk factors associated with residual renal function (RRF) loss. METHODS: Children treated with APD as the initial renal replacement therapy regimen from January 2008 to December 2016 were included. All the children had a daily urine volume of ≥100 ml/m2 when APD was initiated and a dialysis follow-up time of ≥12 months. A daily urine volume of <100 ml/m2 after 12 months of APD treatment was defined as loss of RRF. Possible risk factors that may be associated with RRF loss were analysed. RESULTS: A total of 66 children were included in the study. After 12 months of APD treatment, the daily urine volume decreased by 377.45 ± 348.80 ml/m2, the residual glomerular filtration rate decreased by 6.39 ± 3.69 ml/min/1.73 m2 and 29 of the patients (43.9%) developed RRF loss. The higher risk of RRF loss after 1 year of APD treatment was most pronounced in patients with daily urine volume of ≤400 ml/m2 before treatment, higher glucose exposure and higher ultrafiltration volume, while the lower risk of RRF loss was in patients with administration of diuretics. Each increase of 1 g/m2/day glucose exposure was associated with a 5% increase in RRF loss (odds ratio (OR) 1.05, p = 0.023) and each increase of 1 ml/m2/day ultrafiltration volume was associated with a 1% increase in RRF loss (OR 1.01, p = 0.013). CONCLUSION: In children undergoing APD, the risk for loss of RRF is associated with low urine volume at the start of APD, high glucose loading and high peritoneal ultrafiltration volume, while preservation of RRF is associated with the usage of diuretics.

Exploration of the Optimal Desmopressin Treatment in Children With Monosymptomatic Nocturnal Enuresis: Evidence From a Chinese Cohort.

Objectives: Nocturnal enuresis (NE) is a common pediatric condition, and desmopressin (dDAVP) is a first-line therapy for NE. The standard initial dosage of dDAVP is 0. 2 mg/day, and most guidelines recommend that the dose should be increased at 0.2 mg increments until dryness is achieved or to the maximal recommended dose. However, previous evidence has shown that this strategy seems insufficient to further improve efficacy and results in unnecessarily high doses for some patients. Our study aimed to assess the efficacy of our modified dDAVP treatment regimen in children with MNE in China and evaluate predictive factors associated with the dDAVP response. Methods: All MNE patients at the Department of Nephrology at Children's Hospital of Fudan University from January to December 2019 were prospectively and consecutively enrolled. dDAVP treatment comprised a dose titration period and a 3-month maintenance period. The efficacy of dDAVP was assessed according to the latest International Children's Continence Society criteria at the end of the study. Predictive factors were evaluated by logistic regression analysis. Results: Overall, 322 MNE patients were enrolled in our study, and 225 (69.9%) completed the study. The intention to treat analysis showed that the overall dDAVP response rate was 69.9%: among these patients 32.3% were complete responders, and 37.6% were partial responders. At the end of the study, 194/225 (86.2%) patients received a final dose of 0.2 mg, 24/225 (10.7%) patients received a final dose of 0.3 mg, and 7/225 (3.1%) patients received a final dose of 0.4 mg. Multivariate analysis showed that patients requiring lower doses to achieve responses were significantly more likely to experience complete response during the maintenance period [odds ratio (OR)=9.683; 95% confidence interval (CI), 2.770-33.846]. Conclusions: Our results indicate that the dDAVP treatment regimen provides a comparable efficacy to the international conventional treatment regimen with a lower overall dose. Low-dose responders were likely to achieve a complete response without increasing the dose; in these cases, the maximum dose might not be necessary.

Gene mutation and clinical analysis of nephronophthisis diagnosed using whole exome sequencing: Experience from China
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OBJECTIVE: Nephronophthisis (NPH) is an autosomal recessive cystic kidney disease. Its onset is obscure, and its early clinical manifestations and pathological changes lack specificity, which makes clinical diagnosis difficult. At present, as many as 90 genetic alterations can result in NPH, which exhibits significant genetic heterogeneity. Therefore, high-throughput sequencing technology provides an effective method to identify and characterize novel NPH pathogenic genes when compared to Sanger sequencing. This study summarizes the gene mutations and clinical data of whole exome sequencing, which was used to diagnose 5 NPH patients to improve the understanding of the causative genes and clinical phenotypes of NPH. MATERIALS AND METHODS: The clinical manifestations, laboratory examination indexes, and imaging data of 5 patients of NPH were reported. Whole exome sequencing was performed in 5 children, and the causative genes and mutation sites were analyzed by bioinformatics and genetics. The mutation sites were verified in children and their parents using Sanger direct sequencing. RESULTS: Among the 5 patients (3 male and 2 female), 2 patients had infantile NPH, and 3 patients had juvenile NPH. The 2 infantile NPH patients were characterized by the onset of liver dysfunction accompanied by hypertension and left ventricular change, and the renal function progressed to end-stage renal disease (ESRD) after 7 months and 9 months, respectively. The 2 cases of infantile NPH had NPHP3 mutations, with one carrying compound heterozygous mutations (c.1358A>G, c.2369A>G) and the other simultaneously carrying a c.1174C>T IVS26-3A>G cleavage site mutation from the father and a nonsense mutation (p.392R>X, 939) from the mother. The 2 juvenile NPH children had entered ESRD at the onset of the disease, including 1 patient with Joubert syndrome. The 2 patients with juvenile NPH had frameshift mutations (c.1583 to 1596: deletion) and homozygous point mutations (7 c.640G>T) of the NPHP1 gene. In addition, another patient with frequent urination and nocturia resulting in stage CKD3 renal function had a complex heterozygous mutation of the NPHP2 gene (c.2686G>A, c.1943A>G). The urine A1MU/creatinine and urinary transferrin increased in all 5 patients without hematuria. CONCLUSION: Whole exome sequencing identified the causative genes of NPH in 5 children. In NPH children with NPHP3 gene mutations, renal functional damage was characterized by early onset and rapid progression to ESRD, often accompanied by liver dysfunction and hypertension.

[Etiological analysis of 264 cases with chronic kidney disease stage 2 to 5 in children].

OBJECTIVE: To 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. METHOD: By 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. RESULT: In 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. CONCLUSION: The 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.