Severe combined immunodeficiency (SCID) represents a group of genetically heterogeneous disorders characterized by mutations that lead to profound defects in both humoral and cellular immunity. Transplacental maternal engraftment (TME) is a frequently observed complication in SCID. While most cases of SCID with TME exhibit no substantial impact on disease progression, a subset of patients may encounter diagnostic delays or therapeutic challenges due to TME interference. Furthermore, TME may predispose these individuals to graft-versus-host disease (GVHD) prior to hematopoietic stem cell transplantation, thereby increasing diagnostic complexity and treatment risks. This review systematically examines the etiology and clinical manifestations of SCID associated with TME, analyzes its implications for disease management, and evaluates current detection methodologies. The synthesized evidence provides a theoretical foundation for future research and offers potential insights into the clinical diagnosis and management of SCID associated with TME.
Humans ; Severe Combined Immunodeficiency/diagnosis* ; Pregnancy ; Female ; Hematopoietic Stem Cell Transplantation/adverse effects* ; Maternal-Fetal Exchange/immunology* ; Graft vs Host Disease/etiology* ; Animals ; Placenta/immunology*

This report described one human leukocyte antigen pre-sensitized recipient undergoing preoperative plasmapheresis (PP), intravenous immunoglobulins (IVIG) desensitization and immune induction therapy before kidney transplantation with a donor kidney. Early postoperative clinical diagnosis was acute antibody-mediated rejection (AMR). A marked elevation of donor-specific antibodies (DSA) was accompanied by a decline of renal function. PP/IVIG dosing failed to lower the level of DSA. After low-dose splenic irradiation, DSA level dropped steadily and transplanted kidney function normalized. Thus adjuvant low-dose splenic irradiation may eliminate DSA immediately without a rebound.

Objective:To explore the efficacy and safety of daratumumab in late antibody-mediated rejection (late AMR) after kidney transplantation (KT).Methods:From December 2020 to December 2021, the relevant clinical data were reviewed for 8 patients with late AMR after receiving daratumumab at Affiliated Tongji Hospital. In intensive phase, the combination of plasma exchange (PP)/intravenous immunoglobulin (IVIG) and daratumumab were dosed once a week; in maintenance phase, once every 2 to 4 weeks. The levels of donor-specific antibody (DSA) and renal function were compared pre-treatment and Month 3/12 post-treatment. The treatment-related toxicities were observed. Independent sample T test was utilized for inter-group comparison.Results:The median treatment course during intensive period was 9(4-17) sessions. Maintenance treatment lasted for 5 to 19 months and 2 cases withdrew after 5 to 6 treatments for achieving antibody clearance. A total of 11 DSAs were detected in 8 recipients. At Month 3/12, mean fluorescent intensity (MFI) of DSA was 6 016±4 775 and 6 438±3 668. Both were significantly lower than 11 944±5 237 pre-treatment and the difference was statistically significant ( P=0.012, 0.004). Seven recipients achieved stable renal function during treatment and one recipient resumed hemodialysis at Month 18 due to acute rejection. Glomerular filtration rate of 7 recipients was (40.6±20.1), (53.6±20.9) and (49.0±17.2) ml·min -1· (1.73 m 2) -1 pre-treatment and Month 3/12 and no significant differences existed among different timepoints. During follow-ups, 2 cases developed mild nasal congestion during an early stage of daratumumab infusion while the remainders had no obvious discomfort during infusion and tolerance was decent. Conclusion:Early combination of daratumumab with PP/IVIG, followed by a course of daratumumab has demonstrated an excellent antibody reduction effect on late AMR. During treatment, renal function remains generally stable.

Objective:To investigate the diagnostic value of urine pH, serum uric acid and related clinical indicators in the diagnosis of urinary infection stones, and to construct a prediction nomogram.Methods:The clinical data of 432 patients with urinary calculi admitted to Xuzhou Central Hospital from August 2018 to November 2023 were retrospectively analyzed. The study included 289 males and 143 females, with an average age of (52.72±13.46) years old. Among the patients, there were 98 cases of hypertension, 67 cases of diabetes, and 100 cases of recurrent calculi. Kidney stones were present in 152 cases, ureteral stones in 242 cases, and bladder stones in 38 cases. Urine bacterial culture yielded positive results in 97 cases. According to the results of postoperative stone composition analysis, the two groups were categorized as infection and no-infection stone groups, and the differences in general data between the two groups were compared. Univariate and multivariate logistic regression analysis were conducted to assess the diagnostic value of urine pH, serum uric acid, and related clinical indicators for urinary infection stones. Receiver operating characteristic (ROC) curve and area under curve (AUC) were utilized to evaluate the clinical significance of urine pH, serum uric acid, and combined indexes in preoperatively diagnosing urinary infection stones, as well as constructing a nomogram prediction model.Results:There were 127 cases of infection stones and 305 cases of no-infection stones. The infection stone group exhibited a higher urine pH value [7.0(6.5, 7.5) vs. 6.0(5.5, 6.5), P<0.001], lower serum uric acid levels [(301.38±70.12) vs. (358.88±88.99) μmol/L, P<0.001], a higher proportion of females [55.1%(70/127) vs. 23.9%(73/305), P<0.001], younger age [(48.36±14.83)vs. (53.12±12.61)years old, P<0.001], a higher proportion of recurrence stones [34.6 %(44/127) vs. 18.4%(56/305), P<0.001], and a higher rate of positive urine bacteria culture[29.9%(38/127)vs. 19.3%(59/305), P=0.016]and nitrite test results [18.9%(24/127)vs. 6.3%(19/305), P <0.001]. Univariate logistic regression analysis revealed that urine pH value, serum uric acid levels, gender, age, recurrent stones, urine bacterial culture, and urine nitrite were associated with urinary infection stones ( P< 0.05). Multivariate logistic regression analysis revealed that urine pH value ( OR= 4.836, 95% CI 3.342-6.997), female gender( OR=2.320, 95% CI 1.286-4.186), recurrent stones ( OR=2.225, 95% CI 1.208-4.101), positive urine bacterial culture ( OR=2.061, 95% CI 1.094-3.883), serum uric acid ( OR=0.992, 95% CI 0.949-0.989), age ( OR=0.969, 95% CI 0.949-0.990) were independent risk factors for urinary infection stones ( P<0.05). The combined diagnostic value of six indicators was the highest, with an AUC of 0.874 (95% CI 0.837-0.911). Following this, urine pH exhibited an AUC of 0.818 (95% CI 0.778-0.858), while serum uric acid had an AUC of 0.704 (95% CI 0.652-0.756). The nomogram model was successfully constructed based on the six indicators. The mean AUC of the ROC curve after 1 000 resamples of the Bootstrap method was 0.864 (95% CI 0.828-0.900), and the calibration curve showed that the predicted curve fit the ideal curve well, with a mean absolute error of 0.005 and a Hosmer-Lemeshow test of P>0.05. Clinical decision curve analysis (DCA) showed that the model had a higher net clinical benefit when the model had a threshold probability value≥0.01. Conclusions:Urine pH and serum uric acid are closely related to urinary infection stones. A nomogram model combining these factors with gender, age, recurrent stones, and urine culture results can effectively predict the probability of infection-related stones, providing significant clinical value.

Objective:To investigate the diagnostic value of urine pH, serum uric acid and related clinical indicators in the diagnosis of urinary infection stones, and to construct a prediction nomogram.Methods:The clinical data of 432 patients with urinary calculi admitted to Xuzhou Central Hospital from August 2018 to November 2023 were retrospectively analyzed. The study included 289 males and 143 females, with an average age of (52.72±13.46) years old. Among the patients, there were 98 cases of hypertension, 67 cases of diabetes, and 100 cases of recurrent calculi. Kidney stones were present in 152 cases, ureteral stones in 242 cases, and bladder stones in 38 cases. Urine bacterial culture yielded positive results in 97 cases. According to the results of postoperative stone composition analysis, the two groups were categorized as infection and no-infection stone groups, and the differences in general data between the two groups were compared. Univariate and multivariate logistic regression analysis were conducted to assess the diagnostic value of urine pH, serum uric acid, and related clinical indicators for urinary infection stones. Receiver operating characteristic (ROC) curve and area under curve (AUC) were utilized to evaluate the clinical significance of urine pH, serum uric acid, and combined indexes in preoperatively diagnosing urinary infection stones, as well as constructing a nomogram prediction model.Results:There were 127 cases of infection stones and 305 cases of no-infection stones. The infection stone group exhibited a higher urine pH value [7.0(6.5, 7.5) vs. 6.0(5.5, 6.5), P<0.001], lower serum uric acid levels [(301.38±70.12) vs. (358.88±88.99) μmol/L, P<0.001], a higher proportion of females [55.1%(70/127) vs. 23.9%(73/305), P<0.001], younger age [(48.36±14.83)vs. (53.12±12.61)years old, P<0.001], a higher proportion of recurrence stones [34.6 %(44/127) vs. 18.4%(56/305), P<0.001], and a higher rate of positive urine bacteria culture[29.9%(38/127)vs. 19.3%(59/305), P=0.016]and nitrite test results [18.9%(24/127)vs. 6.3%(19/305), P <0.001]. Univariate logistic regression analysis revealed that urine pH value, serum uric acid levels, gender, age, recurrent stones, urine bacterial culture, and urine nitrite were associated with urinary infection stones ( P< 0.05). Multivariate logistic regression analysis revealed that urine pH value ( OR= 4.836, 95% CI 3.342-6.997), female gender( OR=2.320, 95% CI 1.286-4.186), recurrent stones ( OR=2.225, 95% CI 1.208-4.101), positive urine bacterial culture ( OR=2.061, 95% CI 1.094-3.883), serum uric acid ( OR=0.992, 95% CI 0.949-0.989), age ( OR=0.969, 95% CI 0.949-0.990) were independent risk factors for urinary infection stones ( P<0.05). The combined diagnostic value of six indicators was the highest, with an AUC of 0.874 (95% CI 0.837-0.911). Following this, urine pH exhibited an AUC of 0.818 (95% CI 0.778-0.858), while serum uric acid had an AUC of 0.704 (95% CI 0.652-0.756). The nomogram model was successfully constructed based on the six indicators. The mean AUC of the ROC curve after 1 000 resamples of the Bootstrap method was 0.864 (95% CI 0.828-0.900), and the calibration curve showed that the predicted curve fit the ideal curve well, with a mean absolute error of 0.005 and a Hosmer-Lemeshow test of P>0.05. Clinical decision curve analysis (DCA) showed that the model had a higher net clinical benefit when the model had a threshold probability value≥0.01. Conclusions:Urine pH and serum uric acid are closely related to urinary infection stones. A nomogram model combining these factors with gender, age, recurrent stones, and urine culture results can effectively predict the probability of infection-related stones, providing significant clinical value.

Objective:To summarize the incidence of acute rejection (AR) after pediatric kidney transplantation (KT) at a single center and examine its impact on graft/patient survival and risk factors for AR.Methods:This is a retrospective cohort study including pediatric recipients who underwent kidney transplantation in past 8 years.After excluding recipients of graft thrombosis within a week post-transplant and lost to follow-ups, a total of 143 cases were ultimately recruited and assigned into two groups of AR (n=29) and non-AR (n=114).Basic profiles of both donors and recipients and graft/patient survival rate were compared between two groups.Relative risk factors for AR episodes were also examined by Logistic regression.Results:Renal grafts for 130/143 cases (90.9%) were harvested from deceased donors and 120(83.9%) cases from children.Twenty-seven transplants (18.9%) were performed in infants and young recipients aged < 3 years.During a median follow-up of 33 months, 34 AR episodes occurred in 29(20.3%) patients.Rate of re-transplantation (27.6% vs. 7.9%), pediatric donor (96.5% vs. 80.7%) and rabbit anti-human thymocyte globulin (rATG) induction (79.3% vs. 36%) were significantly higher in AR group than non-AR group ( P=0.007, P=0.046, P<0.001).Multivariate regression analysis indicated that basiliximab induction caused a significant reduction in the risk of AR incidence as compared with rATG induction (odds ratio 0.13, 95% confidence interval 0.04-0.43, P<0.001).The median time of AR incidence was 1.3 months post-transplantation and 23 episodes (67.6%) were confirmed by biopsy.After anti-rejection treatment, 52.9%(n=18) of the cases achieved a full recovery and 38.3% (n=13) had improved graft function.However, 3 cases (8.8%) developed irreversible graft failure.The 1/3-year graft survival rates were significantly lower in AR group than those in non-AR group (75.3% vs. 95.2%, 68.4% vs. 90.4%, P=0.01), and there was no significant difference in 1-and 3-year patient survival rates between two groups. Conclusions:The incidence of AR is relatively high in pediatric renal transplantation, which has an impact on graft survival.Basiliximab induction can effectively reduce the risk of AR.

Objective:To observe the effect of Tuina(Chinese therapeutic massage)combined with functional training in improving clinical symptoms and balance function in patients with meniscus injury. Methods:A total of 93 patients with grade Ⅰ-Ⅱ meniscus injury were randomly divided into a functional training group,a Tuina group,and a combination group,with 31 cases in each group.The Tuina group received Tuina manipulation.The functional training group underwent functional training under the supervision of physicians.The combination group received Tuina manipulation in addition to the functional training.The treatment course of all three groups was 8 weeks.The clinical symptoms were observed before the intervention and after 4 and 8 weeks of interventions,and the changes in knee injury and osteoarthritis outcome score(KOOS)and Y-balance test(YBT)were compared. Results:There was no statistically significant difference in the total response rate among the three groups after treatment(P>0.05).The total and subscale KOOS in the three groups were significantly higher than those before treatment after 4 and 8 weeks of interventions(P<0.05).There was a statistically significant difference in the total KOOS between the combination group and the functional training group after 4 and 8 weeks of interventions(P<0.05);there was a statistically significant difference in the score of daily living ability and sports and recreation function between the two groups after 8 weeks of interventions(P<0.05).The YBT balance coefficients of the affected and non-affected knees of the three groups were significantly improved after 4 and 8 weeks of interventions(P<0.05),and there was no statistically significant difference among the groups(P>0.05). Conclusion:Tuina,functional training,and their combination can improve clinical symptoms and balance function in patients with grade Ⅰ-Ⅱ meniscus injury.Tuina combined with functional training is significantly effective in improving daily living ability and sports and recreation function.

Humans ; Mitral Valve/surgery* ; Heart Valve Prosthesis ; Cardiac Surgical Procedures

Humans ; Mitral Valve/surgery* ; Heart Valve Prosthesis ; Cardiac Surgical Procedures

Objective:To explore the clinical features of transplanted renal artery stenosis after pediatric donor kidneys in children.Methods:We retrospectively summarized the clinical data in five cases of transplanted renal artery stenosis undergoing deceased pediatric donor kidney transplantation from May 1, 2014 to June 30, 2021.Donor/receptor characteristics, diagnosis, treatment and prognosis were recorded.The median follow-up period was 29 months.The median age of five donors and recipients was 9 and 132 months respectively.En bloc renal allograft( n=2)and single kidney transplantation( n=3)were performed.End-to-side anastomosis was performed for renal arteries.The median diagnostic time of renal artery stenosis was 10(3-60)months post-transplantation.Except for one 3-year-old recipient with an earlier onset of stenosis, four stenotic cases during a rapid growth period had a maximal height increase of 30 cm post-transplantation.Three of them had a history of surgery at graft site, including previous kidney transplantation( n=1)and transplant urinary tract reconstruction( n=2). All five cases had hypertension and two showed an elevated serum level of creatinine.Ultrasound indicated a significantly elevated flow rate of >300 cm/s( n=4)and CTA/MRA indicated that the degrees of stenosis varied from 50% to 95%( n=5). Results:After balloon dilatation, stenosis either improved( n=2)or relapsed( n=2). Further stenting succeed( n=1)or failed( n=1). One case of stenosis was relieved partially after 6-month observation without any invasive treatment. Conclusions:As a serious complication, transplant renal artery stenosis is common after pediatric donor kidney transplantation.Too small size in donor kidney and rapid recipient growth may be specific risk factors.After diagnosis, balloon dilation is a preferred treatment.Stent placement should be cautiously employed.