Objective

To summarize the clinical and pathological features, diagnosis, and treatment of recurrent allograft nephropathy in children, and to provide experience for early diagnosis and treatment.

Methods

The clinical and pathological data of children ≤18 years old with recurrent immune glomerular disease after kidney transplantation diagnosed and treated in the First Affiliated Hospital of Sun Yat-sen University from January 2014 to October 2024 were retrospectively analyzed and summarized.

Results

There were 11 males and 7 females. Of the 18 patients, 9 cases of focal segmental glomerulosclerosis (FSGS), 5 cases of IgA nephropathy (IgAN), 2 cases of anti-neutrophil cytoplasmic antibody associated glomerulonephritis (AAGN), and 2 cases of IgA vasculitis associated glomerulonephritis (IgAVN). Among the 9 cases of FSGS, 4 cases achieved complete remission after treatment, 6 cases had normal renal function 3 months to 7 years after operation, and 5 cases had no proteinuria. Among them, 2 cases had graft failure, 1 case had graft insufficiency, and 2 cases had normal renal function. Of the 5 IgAN patients, 2 cases showed massive proteinuria and entered ESRD at 2 and 7 years after operation. Two cases of IgAVN responded to glucocorticoid therapy. One case was treated with rituximab and the symptoms disappeared. The other case experienced two relapses and maintained partial remission. Two patients with AAGN achieved complete remission with normal renal function after treatment.

Conclusions

The common recurrent allograft nephropathy in children includes FSGS, IgAN, AAGN, and IgAVN. Active renal biopsy and effective intervention can achieve good curative effects.

The process of kidney transplantation surgery inevitably involves a phase of kidney ischemia and reperfusion, resulting in damage to the kidney's ischemia-reperfusion injury (IRI),which is intimately linked to the initial functional recuperation and prolonged survival of the transplanted organ. Presently, definitive and efficacious methods for clinical prevention and treatment remain elusive. A thorough examination of how IRI functions in transplanted kidneys offers both theoretical and scientific foundations for creating novel protective approaches. This review discusses the mechanisms of IRI and its impact on renal allograft.

Objective

To investigate the effect of denovo donor-specific antibody (dnDSA)and complement (C1q and C3d)-binding dnDSA on the long-term survival of stable kidney transplant recipients.

Methods

A total of 48 kidney recipients who underwent kidney transplantation in the Second People's Hospital of Shanxi Province from December 2009 to May 2015 were included, whose kidney function was stable at the time of admission. The HLA antibody test was completed in 2015.According to the HLA antibody test results and the HLA alleles of the donors, the 48 recipients were divided into the dnDSA group, the non-DSA (NDSA) group, and the anti-HLA antibody-negative group. The recipients in the dnDSA group and the NDSA group were further divided into the C1q⁺group, the C1q⁺C3d⁺

group, and the C1q^-C3dgroup based on the complement test results. The kidney function, recipient/graft survival rate, graft failure, and acute rejection were analyzed. The comparison of quantitative data among multiple groups was conducted using one-way analysis of variance or Kruskal-Wallis H test； the comparison of count data between groups was conducted using the chi-square test or Fisher's exact probability method. The survival curve was plotted using the Kaplan-Meier method, and the log-rank test was used for comparison. P＜0.05 was considered statistically significant.

Results

For the recipients in the dnDSA group (n=9), NDSA group (n=15 ), and the anti-HLA antibody-negative group (n=24), the transplantation time was 108.0 (69.0-130.5),42.0 (2.0-78.0), and 40.0 (5.3-77.3) months. The MFI values of HLA-Ⅱ were 29 534 (11 761-23 961), 3 239 (1 699-12 277), and 411 (185-1 297) respectively. The differences were all statistically significant (H=7.88 and 35.78, all P＜0.05). The proportion of C1q⁺C3d⁺recipients in the dnDSA group was higher than that in the NDSA group (P＜0.05)； the proportion of C1q^-C3drecipients in the NDSA group was higher than that in the dnDSA group (P＜0.05). Up to June 2024,the proportions of graft loss in the dnDSA group, NDSA group, and anti-HLA antibody negative group were 3/9, 1/15 and 2/24, with no statistical difference (P＞0.05). The 15-year graft/recipient survival rates were 62.2%/88.9% (dnDSA group), 75.0%/100% (NDSA group), and 91.5%/100%(anti-HLA antibody-negative group), with no statistical significance (χ²=2.07 and 1.67, all P＞0.05). The incidence of acute rejection in C1q⁺group, C1q⁺C3d⁺ group and C1q^-C3d^-group was 0,4/11 and 0. The difference was also statistically significant (P＜0.05). The incidence of graft loss were 0, 4/11 and 0, and the difference was statistically significant (P＜0.05).

Conclusions

dnDSA affects the prognosis of kidney transplant recipients in a stable condition. The combined detection of DSA with C1q and C3d may have important value in predicting the long-term survival of transplanted kidney and guiding the clinical intervention.

Objective

To evaluate the efficacy in pediatric kidney transplant recipients combined with bladder dysfunction.

Methods

Retroactively review the clinical data of pediatric recipients who underwent kidney transplantation operation at the Children's Hospital of Fudan University from December 1, 2022 to July 15th, 2024. Children with bladder dysfunction (neurogenic bladder and posterior urethral valve) were included in the case group (n=8), while those without bladder dysfunction were included in the control group (n=97). Collect data and compare the basic information, surgical and hospitalization duration, as well as the incidence of delayed graft function(DGF), acute rejection, urinary tract infection, hydronephrosis of the transplanted kidney, and ureterovesical anastomotic obstruction after transplantation between the two groups. The survival rates of the children and the transplanted kidneys were also compared. The preoperative bladder dysfunction and postoperative follow-up of the children in the case group were evaluated. The Mann-Whitney U test was used for inter group comparison of non-normal distribution quantitative data, and Fisher's exact probability method was used for inter group comparison of count data. P value ＜0.05 was considered statistically significant.

Results

Among the 8 cases in the case group, 3 cases were neurogenic bladder and 5 cases were posterior urethral valve. The average follow-up time for the case group and the control group until February 2025 was 417(291, 543) days and 434(295, 670) days, respectively.8 cases of pediatric patients and transplanted kidneys in the case group all survived； All 97 children in the control group survived, while 5 cases experienced loss of transplanted kidney function. There was no statistically significant difference in the survival rate of pediatric patients and transplanted kidneys between the case group and the control group (χ²=0.00 and 0.43, all P＞0.05). The incidence of postoperative urinary tract infection in the case group (6/8) was higher than that in the control group(8/97), and the difference was statistically significant (P＜0.001). None of the patients in the case group experienced surgical complications such as anastomotic obstruction after surgery, while the 4 patients in the control group underwent reoperation due to ureteral and bladder anastomotic obstruction after removing the double-J tube. There were no statistically significant difference in surgical duration,hospitalization duration, DGF and acute rejection of transplanted kidneys, transplanted kidney hydronephrosis, and reoperation for anastomotic obstruction between the case and the control groups (all P＞0.05).

Conclusions

The herapeutic effect in pediatric kidney transplant patients combined with bladder dysfunction is satisfactory, but the incidence of postoperative urinary tract infection is relatively high.

The occurrence of postoperative lung transplantaion complications severely affect the patient's quality of life and prognosis. Accurate diagnosis of allograft rejection and pulmonary infection is crucial for the treatment and outcome of patients. Traditional histopathological examination by lung biopsies is the gold standard for the diagnosis of rejection, but it has drawbacks such as invasiveness,low positivity rate, high operational difficulty, and delayed results. Cell-free DNA in plasma,especially donor-derived cell-free DNA (dd-cfDNA), as a sensitive biomarker, has been increasingly used for monitoring rejection after solid organ transplantation such as kidney and liver transplantation.This article reviews application value of dd-cfDNA as an potential tool for post-transplant management,in the management of complications after lung transplantation.

Objective

To summarize the clinical characteristics and efficacy of kidney transplantation in children with end-stage renal disease (ESRD).

Methods

The clinical features and follow-up results of children with ESRD after kidney transplantation in Children's Hospital of Chongqing Medical University were analyzed retrospectively. The primary diseases, clinical manifestations,dialysis state, donor features, operation and follow-up results were summarized and analyzed. The dynamic changes of renal allograft function within 2 weeks after operation and prognosis were observed.

Results

A total of 31 pediatric kidney transplantat recipients were included, 15 males and 16 females,with a median age of 13.5 years. The clinical presentation was atypical, and 67.7% (21/31) of the children were emaciated. The primary disease was primary urological abnormalities in 41.9% (13/31)and nonurological abnormalities in 58.1% (18/31). The median waiting time from the diagnosis of ESRD to renal transplantation was 10 months, and only 12.9% obtained preemptive renal transplantation. The median warm ischemia time of donor kidney was 6 min, the median cold ischemia time was 5 h, and the median operation time of renal transplantation was 162.5 min. Stable dialysis creatinine levels were achieved in 25.0%, 59.3%, 86.2%, and 92.0% of children at 1, 2, 3, and 4 days after surgery, respectively, and renal allograft function recovered close to normal in most children within 1 week. As of August 2024, the median postoperative follow-up time was 8 months. During follow-up, there were 2 cases of renal allograft failure, 2 cases of antibody-mediated acute rejection,and 2 cases of BK virus infection.

Conclusions

The clinical manifestations of children with ESRD are atypical and non-specific. The overall effect of pediatric kidney transplantation is promising, and most recipients gradually tend to have stable renal allograft function 4 days after surgery.

Objective

To analyze the clinical phenotypes and renal transplantation outcomes in children with Papillorenal syndrome (PAPRS) caused by PAX2 gene variations, and to propose strategies for pre-transplant risk assessment and post-transplant management strategies.

Methods

A retrospective analysis was performed on the clinical data of four pediatric patients diagnosed with PAPRS at 900th Hospital of PLA Joint Logistic Support Force between 2018 and 2024. These patients underwent kidney transplantation at other medical institutions and were subsequently followed up at our hospital. Clinical data, including genetic variant types, clinical manifestations, postoperative complications, and multi-system abnormalities, were systematically evaluated.

Results

All patients carried novel heterozygous variations in the PAX2 gene (NM_000278.5), including c.221_226dup(p.E74_T75dup), c.185A＞G (p.H62R) (not previously reported in global or domestic databases),and c.76dup (p.V26Gfs*28) (two cases). These variations were located within a hotspot mutation domain. All patients were female and were diagnosed with abnormal renal function during school age,progressing to end-stage renal disease, leading to renal transplantation during adolescence.Postoperatively, all patients experienced varying degrees of infection and exhibited extrarenal manifestations, including ocular lesions such as refractive errors, visual field abnormalities,reproductive system abnormalities such as fallopian tube or ovarian lesions, and central nervous system abnormalities such as intellectual disability, mood disorders.

Conclusions

Variations in the PAX2 gene not only impair renal function, but also result in multi-systemic involvement, with a particular focus on neuropsychological assessment. Early psychological intervention is conducive to improving adherence to long-term follow-up management after renal transplantation.

Objective

To analyze the clinical characteristics and treatment of post-transplantation lymphoproliferative disorder (PTLD) in pediatric renal transplant recipients.

Methods

A retrospective analysis was conducted on the clinical data of children who developed PTLD during the follow-up after kidney transplantation at the Children's Hospital of Fudan University from January 2011 to June 2024,including the onset time, clinical manifestations, treatment plans, and prognosis.

Results

Eight recipients (2.04%) developed PTLD during follow-up, with a median onset interval of 4 months posttransplantation (range 3-14 months). Early-onset PTLD occurred in 7 patients, while 1 presented with late-onset PTLD. The most common initial manifestations were fever (n=7) and gastrointestinal symptoms (n=5). All 7 patients were serologically negative for epstein-barr virus (EBV) before surgery, and all 8 patients were associated with EBV infection when PTLD was diagnosed.Histopathological evaluation identified monomorphic B-cell PTLD in 6 patients and polymorphic PTLD in 1 case (1 patient undiagnosed due to missing biopsy). Initial management with rituximab (RTX)plus chemotherapy in two early-diagnosed patients achieved hematological remission； however, one patient succumbed to a fatal intracranial infection. Subsequent treatment protocol modification incorporating RTX with prophylactic antimicrobial therapy (sulfamethoxazole and posaconazole) in 6 patients resulted in complete PTLD remission with preserved graft function in all patients.

Conclusions

PTLD should be highly vigilant when EBV infection, fever and digestive tract symptoms occur in renal transplant children during follow-up. Monomorphic B-cell and polymorphic PTLD responded well to RTX combined with infection prevention, achieving satisfactory therapeutic outcomes.

Solid organ dysfunction leads to structural brain lesions and dysfunctions, of which cognitive impairment is the most obvious, seriously affecting the quality of life. With the widespread development of solid organ transplantation, the brain structure and function of post-transplant recipients can be improved as the organ function is restored. Improving the monitoring level of brain structure and function in post-transplantation recipients, timely detecting the structural lesions and dysfunction of the brain, and providing scientific and reasonable treatments can improve the metabolism of brain tissues,promote the functional recovery of the damaged nervous system, and minimize the occurrence of complications, thereby improving the prognosis. In this paper, we review the research progress of solid organ transplantation affecting brain structure and function changes, aiming to provide reference for the transplantation-related brain structure and function changes.