Clinical characteristics and influence of postoperative Hirschsprung-associated enterocolitis: retrospective study at a tertiary children's hospital.

PURPOSE: To explore the influence of postoperative Hirschsprung-associated enterocolitis (post-HAEC) on long-term outcomes and to identify risk factors of post-HAEC. METHODS: The medical records of 304 eligible patients diagnosed with Hirschsprung's disease (HSCR) were reviewed. We analyzed the clinical characteristics of post-HAEC and its influence on long-term outcomes. Furthermore, risk factors for early and recurrent HAEC were identified separately. RESULTS: The overall incidence of post-HAEC was 29.9% (91/304). We categorized early HAEC as occurring within postoperative 3 months (n = 39) and recurrent HAEC as occurring ≥ 3 episodes within postoperative 6 months (n = 25). Patients with early HAEC were more likely to experience worse nutritional status, defecation function, and quality of life compared to those with late or no episodes (P < 0.05). Similarly, the adverse influences of recurrent HAEC on these outcomes were also significant (P < 0.05). The risk factors for early HAEC included preoperative undernutrition, long-segment HSCR, and postoperative Grade 3-4 complications within 30 days. For recurrent HAEC, risk factors were preoperative malnutrition, non-parental caregivers, long-segment HSCR, and postoperative Grade 3-4 complications within 30 days. CONCLUSION: Classification of post-HAEC based on the first episode time and frequency was necessary. The earlier or more frequent episodes of post-HAEC have detrimental influences on long-term outcomes. Furthermore, risk factors for early and recurrent HAEC were different.

Transanal fistula repair for the treatment of rectovestibular fistula with normal anus in female children: a 5-year single-center experience.

PURPOSE: The purpose of this study was to review a 5-year operative experience of transanal fistula repair for the treatment of rectovestibular fistula with a normal anus in female children. METHODS: In this study, we conducted a retrospective review of children diagnosed with rectovestibular fistula with normal anus who underwent transanal fistula repair in the department of General Surgery, Children's Hospital of Chongqing Medical University. Clinical data were retrospectively analyzed. RESULTS: A total of 56 female children were included in the study. The patients' ages ranged from 1 year 10 months to 15 years 11 months, with an average age of 5 years 1 month. These children had a clear history of gas or loose stool leakage through the vestibular area, with or without a history of vestibular infection. All patients had a normal anus and underwent transanal fistula repair. Follow-up was conducted through telephone or outpatient visits for a duration of 10 months to 5 years (average follow-up duration 19 months). Three patients experienced minimal secretion from the external orifice of the vestibular fistula within two weeks after the operation, but were successfully treated with sitting bath therapy without any relapse. Another three cases had a recurrence of the fistula, and two of them underwent transanal fistula repair at our center again, resulting in a successful cure after reoperation. The remaining case has not yet undergone reoperation. In the long-term follow-up, all the children had satisfactory anal appearance, with no fecal incontinence, anorectal stenosis, or fistula infection. CONCLUSION: Transanal fistula repair is a simple, safe, and effective surgical method to treat female children with rectovestibular fistula with a normal anus.

Clinical characteristics of congenital heart defects in mild congenital anorectal malformation: single-centre experience.

OBJECTIVE: To analyze the clinical characteristics and types of congenital heart defect (CHD) in mild congenital anorectal malformation (CARM), namely the rectoperineal and rectovestibular fistulas. METHODS: The retrospective study of 183 patients with mild CARM was conducted with assessments of demographic information, color Doppler echocardiography results, and follow-up data. We performed an analysis of the clinical characteristics of CHD, grouping them based on sex and type of mild CARM. RESULTS: Of the 183 patients, rectoperineal fistula occurred in 133 patients (72.7%), while the frequency of CHD was 79.8% (146/183). Ventricular septal defects (VSDs) occur more frequently in patients with rectoperineal fistula compared to those with rectovestibular fistula (1.5% vs. 10%), while the opposite trend was observed for patent ductus arteriosus (PDAs) (39.8% vs. 22.0%). Additionally, males presented higher frequency of PDA (42.7% vs. 26.4%) and self-healing (6 months: 87.2% vs. 42.6%; 12 months: 91.0% vs. 63.2%) than females. However, males had a lower rate of undergoing cardiac surgery (6.4% vs. 17.6%) and a younger median diagnosis age (1 day vs. 9 days). CONCLUSION: Our study indicates that there is a necessity for meticulous cardiac assessment and follow-up in neonates diagnosed with mild CARM.

Negative emotions and quality of life of parents with children diagnosed with anorectal malformations.

PURPOSE: To investigate the anxiety, depression, stress, and quality of life of parents with children diagnosed with anorectal malformations. METHODS: 68 parents participated in the study and completed the unified Self-Rating Anxiety Scale, Self-Rating Depression Scale, Perceived Stress Scale, and the World Health Organization Quality of Life-BREF. RESULTS: Compared with the Chinese reference values, parents in our study had higher scores on anxiety and depression and lower scores on the psychological and environmental domain of the World Health Organization Quality of Life-BREF. Parents who live in rural areas, pay for medical treatment out of pocket, and have multiple children are prone to feel anxious. Parents with multiple children also scored worse in the domains of physiology, psychology, social relationships, and general quality of life assessment. They scored significantly lower in the domains of psychology and social relationships when parents had a low education level. Parents of children who had undergone staged operations got lower scores on the general quality of life assessment. CONCLUSIONS: Parents of children with anorectal malformations have varying degrees of psychological and emotional problems that require attention in the clinic.

Correlation of systemic immune-inflammation Index with surgical necrotizing enterocolitis.

Background: Systemic Immune-Inflammation Index (SII), known as an easy, economical and useful marker, correlates with the severity of inflammatory response. However, the usefulness of SII in necrotizing enterocolitis (NEC) remains unclear. Therefore, we evaluated the correlation of SII at NEC diagnosis and subsequent surgery. Methods: Retrospective review of 131 neonates with NEC in a tertiary-level pediatric referral hospital was conducted with assessments of demographic data, general blood examination results at NEC diagnosis, treatment strategies and clinical outcomes. The receiver operating characteristic (ROC) curve determined the optimal cut-off values of SII, platelet-to-lymphocyte ratio (PLR), and neutrophil-to-lymphocyte ratio. Univariate/multivariate logistic regression analysis and ROC curve were conducted to evaluate the predictive significance of SII in identifying the patients who eventually received surgery. Additionally, NEC-related deaths were assessed. Results: Overall, 49 (37.4%) cases received surgical intervention and mortality was 12.3% (14/131). The area under ROC curve of SII at NEC diagnosis to predict subsequent surgery was 0.833 (optimal cut-off value: 235.85). The SII value in surgical intervention group was significantly higher than that in medical treatment group (332.92 ± 158.52 vs. 158.84 ± 106.82, P < 0.001). Independent influencing factors for surgical NEC were SII (95% confidence interval [CI]: 4.568∼36.449, odds ratio [OR]:12.904, P < 0.001) and PLR (95% CI: 1.071∼7.356, OR:2.807, P = 0.036). SII ≤ 235.85 could identify patients at high risk for surgery, with 87.76% sensitivity, 73.17% specificity, outperformed PLR. Furthermore, mortality was significantly higher in patients with SII ≤ 235.85 than those with SII > 235.85 (20.0% vs. 1.5%, P < 0.001). Conclusion: SII and PLR at NEC diagnosis were independent influencing factors for subsequent surgery. SII ≤ 235.85 may be a useful predictive marker for the identification of surgical NEC and mortality.

Application of coagulation parameters at the time of necrotizing enterocolitis diagnosis in surgical intervention and prognosis.

PURPOSE: It has been shown that abnormalities of coagulation and fibrinolysis system are involved in the pathogenesis of necrotizing enterocolitis (NEC), but not well studied challenge in the context of early detection of disease progression. The present study mainly explores the predictive significance of coagulation parameters at the time of NEC diagnosis in identifying the patients who eventually received surgery and/or NEC-related deaths. METHODS: The retrospective study of 114 neonates with NEC was conducted with assessments of demographic data, laboratory results at the time of NEC diagnosis, treatment methods and prognosis. According to treatment methods, patients were divided into surgical intervention group and medical treatment group. Predictive factors were put forward and determined by receiver operating characteristic (ROC) curve analysis. An analysis of the surgical intervention and prognosis was performed. RESULTS: Of 114 patients, 46 (40.4%) cases received surgical intervention and 14 (12.3%) deaths. prothrombin time (PT), PT international normalized ratio, activated partial thromboplastin time (APTT), fibrinogen and platelet count at the time of NEC diagnosis were independently associated with surgical NEC. The APTT could identify patients at high risk for surgical NEC, with 67.39% sensitivity, 86.76% specificity, better than that of other serological parameters. Coagulopathy was found in 38.6% of all patients. For surgical intervention, the area under the ROC curve (AUC) of coagulopathy was 0.869 (95% confidence interval [CI]: 0.794 ~ 0.944, P < 0.001), with 82.61% sensitivity and 91.18% specificity, outperformed APTT (95% CI: 0.236 ~ 0.173, P = 0.001). Furthermore, the AUC for coagulopathy to predict mortality was 0.809 (95% CI: 0.725 ~ 0.877, P < 0.001), with 92.86% sensitivity and 69.0% specificity. CONCLUSION: Coagulation parameters at the time of NEC diagnosis were conducive to early prediction of surgical NEC and -related deaths, which should be closely monitored in neonates at high risk of NEC and validated as a clinical decision-making tool.

Impact of overweight/obesity on clinical outcomes after reduction for intussusception in children.

PURPOSE: Childhood obesity is a worsening epidemic. Little is known about the impact of overweight and obesity (OV-OB) on clinical outcomes after reduction for intussusception in children. The aim of this study was to compare clinical outcomes after primarily air enema reduction for intussusception in grouped OV-OB (body mass index-for-age percentile ≥ 85) pediatric patients compared with no OV-OB patients. METHODS: A retrospective study of 564 children who had undergone intussusception reduction via pneumatic reduction (PR) from April 2018 to January 2020 was conducted with assessments of demographic data, clinical symptoms, pre-reduction examination, and reduction results. One-to-one propensity score matching (PSM) was performed to compare clinical outcomes between patients with and without OV-OB, and the risk factors affecting recurrence and surgical reduction were analyzed in the PSM population. RESULTS: Of the 564 patients, 132 cases (23.4%) were OV-OB (overweight: 95 cases; obesity: 37 cases). In the propensity-matched analysis, the OV-OB group showed a significant increase in surgical reduction (10.2% versus 0.9%, P = 0.005) and recurrence (47.2% versus 10.2%, P < 0.001), excretion time of carbon powder after PR (median: 11.2 h versus 8.4 h, P < 0.001), higher maximum pressure of PR (median: 10.2 kPa versus 7.8 kPa, P < 0.001), and number of PR attempts (mean: 2.0 versus 1.4, P < 0.001). There were no significant differences in the reasons for surgery (PR failure or bowel perforation), time to recurrence (early or late), and the times of recurrences ≥ 2. After applying the multivariate logistic regression analysis, we found that OV-OB and white blood cell count ≥ 20 × 109/L were risk factors for both surgical reduction and the recurrence of intussusception. CONCLUSION: This study suggested that childhood OV-OB was associated with the failure of PR and recurrence of intussusception after reduction, which should be paid more attention in clinical practice.

Chimeric Antigen receptor-T (CAR-T) cells targeting Epithelial cell adhesion molecule (EpCAM) can inhibit tumor growth in ovarian cancer mouse model.

Ovarian cancer (OC) is one of the most lethal solid tumors with poor prognosis. In 2017, two chimeric antigen receptor-T (CAR-T) cell drugs were approved by the U.S. Food and Drug Administration (FDA), and continuously optimized CAR-T cells therapy might be the novel hope for OC patient. EpCAM are known to be over-expressed in OC cells and could be targeted by CAR-T cells. However, the feasibility of using EpCAM-CAR-T cells to treat OC still needs to be verified. We engineered the 3rd-generation EpCAM-CAR containing a single-chain variable fragment (scFv) EpCAM-scFv that targeting EpCAM, a CD8 transmembrane domain, the costimulatory domains from both CD28 and 4-1BB, and activating domain CD3ζ and then transduced the CAR into T-cells via lentivirus. In addition, the cytotoxicity and cytokine releasing ability of the EpCAM-CAR-T cells against OC cell SKOV3 were verified in vitro. The in vivo data also showed that EpCAM-CAR-T cells significantly reduced the tumor size in OC xenograft mouse models. The anti-tumor activity of EpCAM-CAR-T cells against OC in vitro and in vivo indicated that the CAR-T might provide a promising therapeutic approach to OC.

Primary angiosarcoma arising in an angiomyolipoma of the kidney: case report and literature review.

BACKGROUND: Primary angiosarcoma of the kidney is a rare and aggressive malignant tumor presenting with a recognizable vascular differentiation. Its prognosis is fatal and the pathogenesis remains unclear. Renal angiomyolipoma is a relatively infrequent renal cortical neoplasm and is composed of variable proportions of adipose tissue, spindle cells, epithelioid smooth muscle cells and abnormal thick-walled blood vessels. CASE PRESENTATION: Here, we reported a case in which a 64-year-old woman presenting with the chief complaint of a progressively enlarged mass in the left abdomen. Abdominal computed tomography confirmed presence of a tumor measuring 18 cm × 11 cm in the left posterior renal fascia. Microscopic examination and immunohistochemical staining confirmed co-existence of angiomyolipoma and angiosarcoma. Furthermore, the two components interspersed with each other and there were transitional zones between them. CONCLUSIONS: In this case, we described for the first time a primary renal angiosarcoma possibly arising in a pre-existing angiomyolipoma of the kidney.

Lymphangiosarcoma of the jejunum in a 44-year-old man: report of a case and review of literature.

Lymphangiosarcoma is a malignant tumor arising from lymphatic endothelial cells and has largely been abandoned in the current classification of endothelial neoplasms. Recently, lymphangiosarcoma has been resurrected by several novel markers for lymphatic endothelium available for immunohistochemistry. Here, we reported a new case of lymphangiosarcoma that was located in the Jejunum of a 44-year-old man. To our best knowledge, this is the first report of lymphangiosarcoma in small intestine with no known history of lymphedema, lymphaticmalformation or previous radiation.

[SiRNA inhibition of E6AP expression in cervical cancer cells].

OBJECTIVE: To study the effect of RNA interference (RNAi) targeting E6AP on the proliferation and apoptosis of HeLa cells. METHODS: HeLa cells were cultured and divided into 3 groups: blank control group, cells transfected with nonsense siRNA (small interference RNA), and cells transfected with specific E6AP siRNA. The expressions of E6AP mRNA and protein were detected by RT-PCR and Western blot before and after the transfection respectively. Cell proliferation was determined by methylthiazolyl tetrazolium (MTT). The cell apoptosis index was assessed by flow cytometry. RESULTS: Upon treatment with E6AP siRNA for 24, 48 and 72 h, the expression level of E6AP mRNA decreased 33%, 72% and 70% than siRNA treated group. The protein expression levels in 48 h and 72 h E6AP siRNA groups decreased 38%, 59% comparing with those of the nonsense siRNA treated group (P < 0.05). The proliferative capacity of cells transfectd with E6AP siRNA was significantly lower than blank control group (F = 101.38, P < 0.05) and siRNA treated group (F = 38.64, P < 0.05). The apoptosis index of HeLa cells treated with E6AP siRNA was significantly higher than that of the nonsense siRNA (F = 41.48, P < 0.05) and the blank control group (F = 86.36, P < 0.05). CONCLUSION: SiRNA targeting can effectively suppress the expression levels of E6AP mRNA, corresponding with a proliferation inhibition and an enhanced apoptosis of HeLa cells.