Early onset stage III diabetic nephropathy in a child with Prader-Willi syndrome treated with dulaglutide: a case report.

Background: Prader-Willi syndrome (PWS) is a multisystem genetic disorder caused by chromosomal imprinting gene defects, with approximately 70% of cases resulting from paternal deletion of the chromosomal region 15. The main clinical features include severe infantile hypotonia, early-onset childhood obesity, hyperphagia, and underdeveloped external genitalia. As individuals with PWS age, they may exhibit irritability, social dysfunction, impaired gonadal development, and metabolic syndrome. Previous literature places the prevalence of type 2 diabetes mellitus (T2DM) in PWS at approximately 7-24%. Oxytocin is a neuropeptide secreted by the paraventricular (PVN) and supraoptic (SON) nuclei of the hypothalamus and regulates energy metabolism, which is involved in PWS. Due to age limitations, very few patients progress to diabetic nephropathy during childhood, and reports of typical diabetic nephropathy in PWS during childhood are extremely rare. Dulaglutide is a glucagon-like peptide-1 (GLP-1) receptor agonist which can be used in the treatment of T2DM. Case Description: This article reports a case of a child with PWS complicated by stage III diabetic nephropathy, providing a retrospective analysis of the diagnosis and treatment process, as well as a review of domestic and international literature, to enhance understanding of this condition. And this article provides a treatment idea for PWS patients with diabetic nephropathy. Conclusions: It is very important to enhance understanding of PWS. And we offer new diagnostic and possible therapeutic approaches for pediatric patients with diabetic nephropathy.

Mutations in the NUP93, NUP107 and NUP160 genes cause steroid-resistant nephrotic syndrome in Chinese children.

BACKGROUND: The variants of nucleoporins are extremely rare in hereditary steroid-resistant nephrotic syndrome (SRNS). Most of the patients carrying such variants progress to end stage kidney disease (ESKD) in their childhood. More clinical and genetic data from these patients are needed to characterize their genotype-phenotype relationships and elucidate the role of nucleoporins in SRNS. METHODS: Four patients of SRNS carrying biallelic variants in the NUP93, NUP107 and NUP160 genes were presented. The clinical and molecular genetic characteristics of these patients were summarized, and relevant literature was reviewed. RESULTS: All four patients in this study were female and initially presented with SRNS. The median age at the onset of the disease was 5.08 years, ranging from 1 to 10.5 years. Among the four patients, three progressed to ESKD at a median age of 7 years, ranging from 1.5 to 10.5 years, while one patient reached stage 3 chronic kidney disease (CKD3). Kidney biopsies revealed focal segmental glomerulosclerosis in three patients. Biallelic variants were detected in NUP93 in one patient, NUP107 in two patients, as well as NUP160 in one patient respectively. Among these variants, five yielded single amino acid substitutions, one led to nonsense mutation causing premature termination of NUP107 translation, one caused a single nucleotide deletion resulting in frameshift and truncation of NUP107. Furthermore, one splicing donor mutation was observed in NUP160. None of these variants had been reported previously. CONCLUSION: This report indicates that biallelic variants in NUP93, NUP107 and NUP160 can cause severe early-onset SRNS, which rapidly progresses to ESKD. Moreover, these findings expand the spectrum of phenotypes and genotypes and highlight the importance of next-generation sequencing in elucidating the molecular basis of SRNS and allowing rational treatment for affected individuals.

Clinical, histological and molecular characteristics of Alport syndrome in Chinese children.

BACKGROUND: Alport syndrome is caused by COL4A3, COL4A4, or COL4A5 gene mutations. The present study aims to compare the clinicopathological features, gene mutations, and outcome of Chinese children with different forms of Alport syndrome. METHODS: One hundred twenty-eight children from 126 families diagnosed with Alport syndrome through pathological and genetic examination between 2003 and 2021 were included in this single-center retrospective study. The laboratory and clinicopathological features of the patients with different inheritance patterns were analyzed. The patients were followed-up for disease progression and phenotype-genotype correlation. RESULTS: Of the 126 Alport syndrome families, X-linked forms accounted for 77.0%, autosomal recessive for 11.9%, autosomal dominant for 7.1%, and digenic for 4.0%. Among the patients, 59.4% were males and 40.6% were females. Altogether, 114 different mutations were identified in 101 patients from 99 families by whole-exome sequencing, of which 68 have not been previously reported. The most prevalent type of mutation was glycine substitution, which was identified in 52.1%, 36.7%, and 60% of the patients with X-linked Alport syndrome, autosomal recessive and autosomal dominant Alport syndrome, respectively. At the end of a median follow up of 3.3 (1.8-6.3) years, Kaplan-Meier curves showed kidney survival was significantly lower in autosomal recessive compared to X-linked Alport syndrome (P = 0.004). Pediatric patients with Alport syndrome seldom presented extrarenal involvement. CONCLUSIONS: X-linked Alport syndrome is the most frequent form found in this cohort. Progression was more rapid in autosmal recessive than in X-linked Alport syndrome.

Steroid-resistant nephrotic syndrome associated with certain SGPL1 variants in a family: Case report and literature review.

Objectives: Steroid-resistant nephrotic syndrome (SRNS) is a clinical syndrome characterized by the lack of response to standard steroid therapy, usually progressing to end-stage renal disease. We reported two cases of female identical twins with SRNS caused by SGPL1 variants in one family, reviewed the relevant literature, and summarized their clinical phenotypes, pathological types, and genotypic characteristics. Methods: Two cases of nephrotic syndrome caused by SGPL1 variants were admitted to Tongji Hospital, affiliated with Tongji Medical College of Huazhong University of Science and Technology. Their clinical data were retrospectively collected, and the peripheral blood genomic DNA was captured and sequenced by whole exome sequencing. Related literature published in PubMed, CNKI, and Wan fang databases was reviewed. Results: We described two Chinese identical twin girls with isolated SRNS due to compound heterozygous variants in the SGPL1 (intron4 c.261 + 1G > A and intron12 c.1298 + 6T > C). The patients were followed up for 60.0 months and 53.0 months, respectively, having no extra-renal manifestations. They all died due to renal failure. A total of 31 children with SGPL1 variants causing nephrotic syndrome (including the reported two cases) were identified through a literature review. Conclusions: These two female identical twins were the first reported cases of isolated SRNS caused by SGPL1 variants. Almost all homozygous and compound heterozygous variants of SGPL1 had extra-renal manifestations, but compound heterozygous variants in the intron of SGPL1 may have no obvious extra-renal manifestations. Additionally, a negative genetic testing result does not completely rule out genetic SRNS because the Human Gene Mutation Database or ClinVar is constantly being updated.

Case Report: The Application of Dupilumab in Atopic Dermatitis Children Complicated With Nephrotic Syndrome.

Nephrotic syndrome (NS) tends to be more common in patients with history of allergies. Atopic dermatitis (AD) is one of the most common allergic diseases in children. Dupilumab, a dual IL-4 and IL-13 inhibitor, has been widely used to treat AD patients. However, the efficacy and safety of Dupilumab in NS is unclear. We reported two AD patients with NS comorbidities treated with Dupilumab. The outcomes showed the good control of NS and less systemic steroids and/or immunosuppressive agents use during the Dupilumab treatment period, accompanied by significant relief of AD symptoms. We suggest prospective pilot studies and randomized controlled trials could be carried out to validate the efficacy and safety of Dupilumab in the treatment of NS patients.

A novel variant site of Alstrom syndrome in a Chinese child: a case report.

Background: Alstrom syndrome (ALMS) is an ultra-rare multisystem genetic disorder caused by autosomal recessive inheritance of the ALMS1 gene. It manifests as multisystem dysfunction, displaying unique clinical signs and symptoms and various severity, which may lead to delayed prognosis or misdiagnosis in medical practice. Although almost 300 pathogenic variants have been reported, there are some variant sites that have not been recognized yet. Case Description: We report a case of a 14-year-old boy with manifestations, including binocular vision loss, acanthosis nigricans, type 2 diabetes, insulin resistance, elevated transaminase, hepatic fibrosis, and proteinuria. Compound heterozygous variants in the ALMS1 gene have been discovered by whole exon sequencing. One of his variant sites was C. 8158C>T, which was from his father. And the other variant site was C. 3575C>A, which was from his mother. To the great of our knowledge, this site has not been reported before. Both of the variants make the synthesis of the peptide chain terminated in advance and an incomplete polypeptide chain is formed. Conclusions: The clinical presentations of ALMS are complicated and varied. Although early diagnosis can be made according to typical clinical symptoms, whole exon sequencing is necessary for the diagnosis of ALMS, as indicated by our study.

Clinical and Renal Histology Findings and Different Responses to Induction Treatment Affecting the Long-Term Renal Outcomes of Children With ANCA-Associated Vasculitis: a Single-Center Cohort Analysis.

Introduction: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is relatively rare in children. This article aimed to analyze clinical and renal histology findings and different responses to induction treatment associated with the long-term renal outcomes in children with AAV in a single center. Methods: All pediatric patients with AAV admitted to Tongji Hospital from January 2002 to January 2021 were included in the study. The demographic, clinical, pathological, laboratory, and treatment data and outcomes were collected and analyzed to identify predictors associated with response to induction treatment and progression to end-stage renal disease (ESRD). Results: In total, 48 children with AAV were included in this cohort; 81.25% of them were women, and 91.7% were microscopic polyangiitis (MPA). Kidney involvement was found in 45 patients (93.75%). The most common histopathological subtype was crescentic form in this cohort according to Berden's classification. In total, 34 patients (70.8%) showed eGFR <60 ml/min/1.73 m2 at the time of diagnosis. Complete and partial remission was achieved in 8 patients (16.7%) and 19 patients (39.6%), respectively, following 6-month induction treatment. Half of the patients eventually progressed to ESRD at a mean time of (13.04 ± 15.83) months after diagnosis. The independent predictors of nonremission following induction treatment and progression to ESRD were baseline eGFR <60 ml/min/1.73 m2 and hypertension at diagnosis. Renal survival significantly decreased over time in patients with renal sclerotic subtypes or those with nonremission following induction treatment by Kaplan-Meier curve estimation. Conclusions: Our study demonstrates that women, MPA, and crescentic subtypes are predominant in pediatric AAV in China. Initial renal failure (eGFR <60 ml/min/1.73 m2), hypertension, sclerotic pathological subtype, and nonremission following induction treatment are predictive of long-term renal outcomes.

CUBN gene mutations may cause focal segmental glomerulosclerosis (FSGS) in children.

BACKGROUND: Imerslund-Gräsbeck Syndrome (IGS) is mainly caused by CUBN gene biallelic mutations. Proteinuria accompanies IGS specific symptoms in about half of the patients, isolated proteinuria is rarely reported. Here we present 3 patients with isolated proteinuria and focal segmental glomerulosclerosis (FSGS) caused by CUBN gene biallelic pathogenic variants. METHOD: Whole exome sequencing was performed on three children with isolated proteinuria. CUBN gene biallelic pathogenic variants were found and then verified by sanger sequencing. Their clinical, pathological and molecular genetic characteristics were analyzed and correlated accordingly. RESULTS: All three children presented with isolated proteinuria, no megaloblastic anemia. Their urine levels of ß2 microglobulin were normal or slightly higher. Renal biopsies showed focal segmental glomerulosclerosis with mild glomerular mesangial hypercellularity, partial effacement of foot processes and podocyte microvillation. Two of them were found to carry compound heterozygous mutations and one homozygous mutation of CUBN gene. Totally four CUBN gene biallelic pathogenic variants were identified, including c.9287 T > C (p.L3096P), c.122 + 1G > A, c.7906C > T (p.R2636*), c.10233G > A (p.W3411*). Except for intron splice-site mutation, all other variants are located in highly conserved sites of CUB domain for binding to albumin. CONCLUSION: The results demonstrate that CUBN gene mutations may cause isolated proteinuria pathologically presented as FSGS. Our cases extend the spectrum of renal manifestation and genotype of CUBN gene mutations.

Zero-contrast PTRA and stenting for child with TRAS: A case report.

BACKGROUND: Kidney transplantation is often complicated by TRAS, and PTRA can effectively treat it. However, PTRA is not appropriate for patients with CKD because iodinated contrast agent may induce nephropathy. METHODS: This article reports about a 14-year-old boy with a history of kidney transplantation complicated by RAS. He initially underwent percutaneous balloon dilation to relieve the stenosis. One and half a year after dilation, he received zero-contrast PTRA and stenting under the guidance of external and IVUS with reference to previous PAG image. RESULTS: After successful stent implantation, the cross-sectional area of the renal artery lumen was significantly increased.The blood pressure and level of creatinine also decreased and kept stable during follow-up. CONCLUSION: This report suggests the feasibility of external ultrasound and IVUS guided, zero-contrast PTRA in patients with poor renal function or iodinated contrast allergy.

Long-Term Clinical and Immunological Impact of Severe COVID-19 on a Living Kidney Transplant Recipient - A Case Report.

The long-term impact of COVID-19 on transplant recipients remains unknown. We describe the case of a 30-year-old male kidney transplant recipient from Wuhan, China that was treated for severe COVID-19 in February 2020. He suffered an acute lung and renal injury and required systemic treatment including adjustment of his immunosuppressant regime. He was followed up to 1-year after discharge. No chronic lung fibrosis or deterioration of his pulmonary function was observed. Despite COVID-19 mediated damage to his renal tubular cells, no transplant rejection occurred. His immunological profile demonstrated both cellular anti-SARS-CoV-2 reactivity and specific humoral immunity, indicating that it is beneficial for the transplanted patients to be immunized with SARS-CoV-2 virus vaccine. This case will help guide clinical decision making for immunocompromised individuals that become infected with SARS-CoV-2.

CCL2 regulation of MST1-mTOR-STAT1 signaling axis controls BCR signaling and B-cell differentiation.

Chemokines are important regulators of the immune system, inducing specific cellular responses by binding to receptors on immune cells. In SLE patients, decreased expression of CCL2 on mesenchymal stem cells (MSC) prevents inhibition of B-cell proliferation, causing the characteristic autoimmune phenotype. Nevertheless, the intrinsic role of CCL2 on B-cell autoimmunity is unknown. In this study using Ccl2 KO mice, we found that CCL2 deficiency enhanced BCR signaling by upregulating the phosphorylation of the MST1-mTORC1-STAT1 axis, which led to reduced marginal zone (MZ) B cells and increased germinal center (GC) B cells. The abnormal differentiation of MZ and GC B cells were rescued by in vivo inhibition of mTORC1. Additionally, the inhibition of MST1-mTORC1-STAT1 with specific inhibitors in vitro also rescued the BCR signaling upon antigenic stimulation. The deficiency of CCL2 also enhanced the early activation of B cells including B-cell spreading, clustering and signalosome recruitment by upregulating the DOCK8-WASP-actin axis. Our study has revealed the intrinsic role and underlying molecular mechanism of CCL2 in BCR signaling, B-cell differentiation, and humoral response.

Recommendations for the Diagnosis, Prevention, and Control of Coronavirus Disease-19 in Children-The Chinese Perspectives.

Ever since SARS-CoV-2 began infecting people by the end of 2019, of whom some developed severe pneumonia (about 5%), which could be fatal (case fatality ~3.5%), the extent and speed of the COVID-19 outbreak has been phenomenal. Within 2.5 months (by March 18, 2020) over 191,127 COVID-19 patients have been identified in 161 countries. By then, over 700 pediatric patients were confirmed to have COVID-19 in China, with only about 58 diagnosed elsewhere. By now, there are thousands of children and adolescents infected. Chinese pediatricians would like to share their experience on how these patients were managed in China and the key recommendations that had guided them in meeting the evolving challenges. A group of experts were summoned by the Chinese Pediatric Society and Editorial Board of Chinese Journal of Pediatrics to extract informative data from a survey on confirmed COVID-19 pediatric patients in China. Consensus on diagnosis, management, and prevention of pediatric COVID-19 were drawn up based on the analysis of such data plus insights gained from the past SARS and MERS coronavirus outbreaks. Relevant cumulating experiences from physicians managing adult patients, expedited reports on clinical and scientific COVID-19 and SARS-CoV-2 data, and the National Health Committee guidelines on COVID-19 management were integrated into this proposal.

Kindergartens Reopening in the Period of Regular Epidemic Prevention and Control, Benefitial or Harmful?

In the period of regular epidemic prevention and control of Coronavirus disease 2019 (COVID-19) in our country, work resumption has been fully advanced. But there are still new sporadic local cases and imported cases across the country. In this situation, whether kindergartens reopening will increase the risk of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) spread still remains uncertain. We reviewed two pediatric patients with moderate COVID-19, collected the epidemiologic information and monitored the cycle threshold value of rectal specimen and the viral loads, and discussed the transmission of SARS-CoV-2 in pediatric patients and the virulence of feces in children with moderate COVID-19, in order to analyze the risk of kindergartens reopening.

Possible PKHD1 Hot-spot Mutations Related to Early Kidney Function Failure or Hepatofibrosis in Chinese Children with ARPKD: A Retrospective Single Center Cohort Study and Literature Review.

PKHD1 mutations are generally considered to cause autosomal recessive polycystic kidney disease (ARPKD). ARPKD is a rare disorder and one of the most severe conditions leading to end-stage renal disease in childhood. With the biallelic deletion mutation, patients have difficulty in surviving the perinatal period, resulting in perinatal or neonatal death. This study retrospectively analyzed patient characteristics, imaging characteristics, laboratory examinations and family surveys from 7 Chinese children with different PKHD1 gene mutations diagnosed by high-throughput sequencing from January 2014 to February 2018. Of the 7 children, there were 3 males and 4 females. Eight missense mutations, two frameshift mutations, two deletion mutations, and two intronic slicing mutations were identified. Six of the mutations have not previously been identified. In the literature search, we identified a total of 29 Chinese children with PKHD1 mutations. The missense mutation c.2507T>C in exon 24 was found in one patient in our study, and five patients with liver fibrosis but normal renal function were reported in the literature. The missense mutation c.5935G>A in exon 37 was found in two patients in our study and three cases in the literature. Four patients had renal failure at an age as young as 1 year of those five patients with the missense mutation c.5935G>A in exon 37. It was concluded that: (1) Kidney length more than 2-3 SDs above the mean and early-onset hypertension might be associated with PKHD1-associated ARPKD; (2) The more enlarged the kidney size is, the lower the renal function is likely to be; (3) c.5935G>A may be a hot spot that leads to early renal failure in Chinese children with PKHD1 mutations; (4) c.2507T>C may be a hot-spot mutation associated with hepatic lesions in Chinese children with PKHD1.

Relationship between Vesicoureteral Reflux and Glomerular Filtration Rate in Children.

Vesicoureteral reflux (VUR) is one of the most common urinary tract anomalies in children and causes renal damage and studies focusing on the effect of VUR on renal function are rare. We recruited 35 primary VUR patients with recurrent urinary tract infection (UTI) and 10 non-VUR patients with recurrent UTI. Contrast-enhanced voiding urosonography (ceVUS) was performed for VUR grading, and renal dynamic imaging was used for evaluating glomerular filtration rate (GFR, mL/min). Standardized GFR (sGFR), namely GFR/BSA (mL·min-1·m-2), was calculated based on the body surface area (BSA). Total sGFR (tsGFR, mL·min-1·m-2) was obtained from the sum of sGFR on the left and right sides of all the children. The risk of renal regurgitation was equal in the unilateral reflux group. The sGFR of children with grade IV (45.74±18.05 mL·min-1·m-2) and grade V (49.67±23.63 mL·min-1·m-2) reflux was significantly lower than that in children with grade III (77.69 ±22.21 mL·min-1·m-2). The renal function compensation of contralateral non-reflux kidney increased in unilateral reflux group, which was higher than that in the control group and level II, IV and V of reflux group respectively. In VUR group of the same grade, sGFR decreased with the age at diagnosis. In unilateral grade V reflux group, the tsGFR was lower than that in the unilateral grade III reflux group (133.51±48.21 vs. 186.87±53.49 mL·min-1·m-2). The patients with VUR of unilateral grade II were significantly older than those with VUR of unilateral grades III and IV. This study indicates that severe VUR is significantly associated with decreased renal function. Therefore, VUR should be diagnosed early and managed individually.

Are They Just Two Children COVID-19 Cases Confused With Flu?

COVID-19, an emerging infectious disease, has quickly spread all over the world. All human populations are susceptible to this disease. Here we present two pediatric COVID-19 cases, both of whom exhibited negative SARS-CoV-2 nucleic acid tests upon nasopharyngeal swab and were initially diagnosed with influenza A infection. COVID-19 was later confirmed in both patients by serum antibodies of SARS-CoV-2 and nucleic acid test on stool samples. Because children are susceptible to many respiratory pathogens, especially influenza, we concluded that children can be coinfected with multiple pathogens, and more attention should be paid to the exploration of SARS-CoV-2 during the pandemic of COVID-19. This report shows the possibility of misdiagnosis or missed diagnosis of children with COVID-19. We suggest that highly suspected pediatric COVID-19 cases with negative nucleic acid tests on nasopharyngeal swabs should be further checked by performing a nucleic acid test on stool samples and testing serum for antibodies against SARS-CoV-2.

A Case of Critically Ill Infant of Coronavirus Disease 2019 With Persistent Reduction of T Lymphocytes.

BACKGROUND: The outbreak of coronavirus disease 2019 (COVID-19) is becoming a global threat. However, our understanding of the clinical characteristics and treatment of critically ill pediatric patients and their ability of transmitting the coronavirus that causes COVID-19 still remains inadequate because only a handful pediatric cases of COVID-19 have been reported. METHODS: Epidemiology, clinical characteristics, treatment, laboratory data and follow-up information and the treatment of critically ill infant were recorded. RESULTS: The infant had life-threatening clinical features including high fever, septic shock, recurrent apnea, petechiae and acute kidney injury and persistent declined CD3+, CD4+ and CD8+ T cells. The duration of nasopharyngeal virus shedding lasted for 49 days even with the administration of lopinavir/ritonavir for 8 days. The CD3+, CD4+ and CD8+ T cells was partially recovered 68 days post onset of the disease. Accumulating of effector memory CD4+ T cells (CD4+TEM) was observed among T-cell compartment. The nucleic acid tests and serum antibody for the severe acute respiratory syndrome coronavirus 2 of the infant's mother who kept intimate contact with the infant were negative despite no strict personal protection. CONCLUSIONS: The persistent reduction of CD4+ and CD8+ T cells was the typical feature of critically ill infant with COVID-19. CD4+ and CD8+ T cells might play a key role in aggravating COVID-19 and predicts a more critical course in children. The prolonged nasopharyngeal virus shedding was related with the severity of respiratory injury. The transmission of SARS-CoV-2 from infant (even very critical cases) to adult might be unlikely.

A quantitative grading system of vesicoureteral reflux by contrastenhanced voiding urosonography.

AIMS: Contrast-enhanced voiding urosonography (ceVUS) is a well-established imaging modality for the diagnosis of vesicoureteral reflux (VUR). However, discrepancies of grading diagnosis of VUR exist due to the qualitative grading criteria currently used in clinics. This study aimed to evaluate numerical markers for a quantitative VUR grading system. MATERIAL AND METHODS: CeVUS images of grade II-VVUR were analysed. A quantitative indicator, i.e. sectional area ratio (SAR), on the imaging section with maximum cross-section area and the presence of kidney hilum was calculated to distinguish different grades of VUR. The diagnostic performance of SAR was evaluated using receiver operating characteristic curve (ROC) analysis, and the maximum Youden Index was used to determine the optimal cut-off values. RESULTS: A total of 63 patients with 126 PelviUreteral Units were enrolled. The SAR value increased significantly along with the increase of VUR grade. SAR had an excellent diagnostic performance in grading VUR. For differentiating VUR of grade II vs III, III vs IV and IV vs V, the area under the ROC curve values of SAR were 0.967, 0.943 and 0.865, respectively, while the optimal SAR cut-off values were 14.3%, 34.9% and 51.0%, respectively. The quantitative grading system based on the optimal SAR cut-off values showed excellent consistency with the qualitative grading system of VUR currently used in clinic. CONCLUSIONS: The numerical indicator SAR calculated from ceVUS may be used to establish a quantitative VUR grading system with excellent diagnostic performance and can potentially serve as a reliable tool for the evaluation and follow-up of VUR.

Contrast-enhanced voiding urosonography with intravesical administration of ultrasound contrast agent for the diagnosis of pediatric vesicoureteral reflux.

Vesicoureteral reflux (VUR) is one of the most common urinary tract anomalies in children. It has been reported that VUR may be associated with reflux nephropathy. Ultrasound contrast-enhanced voiding urosonography (CeVUS) has become a routine diagnostic method for VUR in a number of European countries; however, it is not widely used in China. The aim of the present study was to analyze the clinical application and evaluate the safety of CeVUS as a diagnostic tool for VUR in children in order to establish a standardized operating procedure for CeVUS in pediatric VUR in China. Between August 2016 and October 2017, 90 children who were susceptible to VUR were admitted into the Pediatric Nephrology Department of Tongji Hospital and underwent CeVUS. The SonoVue second-generation USA contrast agent was administered intravesically via a transurethral bladder catheter at a dose of 1 ml. The occurrence of adverse events was monitored. Urine analysis and culture were performed. A total of 90 children (47 female, 43 male; mean age, 36.6 months) with 178 Pelvi-Ureteral Units (PUUs) underwent CeVUS to screen for VUR. VUR was detected in 44/90 pediatric patients (48.89%) and 65/178 PUUs (36.52%) by CeVUS. The grade distribution of the 65 PUUs with VUS was as follows: Grade I, 3; Grade II, 9; Grade III, 14; Grade IV, 22; and Grade V, 17. The accuracy of CeVUS in the present study were consistent with previous reports. No urethral anomalies were detected and there were no adverse events. CeVUS was demonstrated to be a safe, accurate and reliable imaging technique for detecting VUR in high-risk children, including neonates. Results of the present study indicated that CeVUS can be adopted as the primary screening and follow-up method for pediatric VUR diagnoses in China.