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The given text provides information on an article, a study, and a document related to anorectal malformations (ARM). The article discusses the establishment of a patient registry to collect data on ARM patients, providing insights into demographics, diagnostics, surgeries, complications, and outcomes. The study examines the clinical and surgical characteristics of ARM patients, highlighting the prevalence of associated anomalies and the need for improved postoperative management. The document is a list of references and citations on various aspects of ARM, offering a comprehensive overview of current knowledge and research in the field. [Extracted from the article]

Background Esophageal achalasia (EA) is a rare primary motility disorder in any age group, and particularly rare in the pediatric population, with a reported incidence of 0.18 per 100,000 children a year. EA in pediatric age is currently treated in the same way as in adults, but this approach is based on only a few studies on small case series. The aim of this retrospective study was to assess the long-term outcome of the laparoscopic Heller–Dor (LHD) procedure when performed in pediatric patients with EA at our university hospital. Materials and Methods We considered children and adolescents younger than 16 years old diagnosed with EA and treated with LHD between 1996 and 2022. Clinical data were prospectively collected in an ongoing database. Symptoms were recorded and their severity was calculated using the Eckardt score. Barium swallow, esophageal manometry (conventional or high-resolution), and endoscopy were performed before and after the surgical procedure. Results During the study period, 40 children with a median age of 14 years (interquartile range [IQR]: 11–15) underwent LHD. At a median follow-up of 10.5 years (IQR: 4.5–13.9), a good outcome was achieved in 36/40 patients (90%). Two of the four patients whose surgical procedure failed underwent complementary pneumatic dilations successfully, thus increasing the overall success rate to 95%. A previous endoscopic treatment (in five patients) did not affect the final outcome (p = 0.49). An intraoperative mucosal lesion was detected in only one patient (2.5%) and was repaired at the time without further consequences. During the follow-up, 22 patients underwent endoscopy, and 17 had pH monitoring as well: only 2 of these patients showed reflux esophagitis at endoscopy (one of them with abnormal findings on pH monitoring), amounting to a 9.1% rate of instrumentally confirmed postoperative reflux. Conclusion LHD is a safe and persistently effective treatment for EA in pediatric age, with a success rate comparable with what is usually obtained in adults, and better than what has been reported to date in the pediatric literature. Adding a fundoplication certainly helps ensure an optimal long-term control of any gastroesophageal reflux induced by the myotomy. [ABSTRACT FROM AUTHOR]

This review focuses on the crucial role of the intestinal epithelium in maintaining intestinal homeostasis and its significance in the pathogenesis of necrotizing enterocolitis (NEC) and inflammatory bowel diseases (IBD). NEC is a devastating neonatal disease, while IBD represents a global healthcare problem with increasing incidence. The breakdown of the intestinal barrier in neonates is considered pivotal in the development and progression of both disorders. This review provides an overview of the current state of in vitro, ex vivo, and animal models to study epithelial injury in NEC and IBD, addressing pertinent questions that engage clinicians and researchers alike. Despite significant advancements in early recognition and aggressive treatment, no single therapy has been conclusively proven effective in reducing the severity of these disorders. Although early interventions have improved clinical outcomes, NEC and IBD continue to impose substantial morbidity, mortality, and economic burdens on affected individuals and society. Consequently, exploring alternative therapeutic options capable of preventing and treating the sequelae of NEC and IBD has become a pressing necessity. In recent decades, extracellular vehicles (EVs) have emerged as a potential solution to modulate the pathogenic mechanism in these multifactorial and complex disorders. Despite the diverse array of proposed models, a comprehensive model to investigate and decelerate the progression of NEC and IBD remains to be established. To bridge the translational gap between preclinical studies and clinical applications, enhancements in the technical development of gut-on-a-chip models and EVs hold considerable promise. Graphical Abstract [ABSTRACT FROM AUTHOR]

Purpose: To assess the number, characteristics, and functional short-, and midterm outcomes of patients with rectal atresia (RA) and stenosis (RS) in the ARM-Net registry. Methods: Patients with RA/RS were retrieved from the ARM-Net registry. Patient characteristics, associated anomalies, surgical approach, and functional bowel outcomes at 1 and 5-year follow-up were assessed. Results: The ARM-Net registry included 2619 patients, of whom 36 (1.3%) had RA/RS. Median age at follow-up was 7.0 years (IQR 2.3–9.0). Twenty-three patients (63.9%, RA n = 13, RS n = 10) had additional anomalies. PSARP was the most performed reconstructive surgery for both RA (n = 9) and RS (n = 6) patients. At 1-year follow-up, 11/24 patients with known data (45.8%, RA n = 5, RS n = 6) were constipated, of whom 9 required stool softeners and/or laxatives. At 5-year follow-up, 8/9 patients with known data (88.9%, RA n = 4, RS n = 4) were constipated, all requiring laxatives and/or enema. Conclusion: RA and RS are rare types of ARM, representing 1.3% of patients in the ARM-Net registry. Additional anomalies were present in majority of patients. Different surgical approaches were performed as reconstructive treatment, with constipation occurring in 46% and 89% of the patients at 1 and 5-year follow-up. However, accurate evaluation of long-term functional outcomes remains challenging. [ABSTRACT FROM AUTHOR]

Introduction Vascular complications are severe complications of pediatric kidney transplantation (KT). We aimed to investigate whether a complex bench surgery (BS) affects the outcomes. Methods All pediatric KT performed at the University Hospital of Padua from 2015 to 2019 were analyzed, comparing those in which a standard BS was possible to those that necessitated a complex BS. The rates of vascular complications, patients' outcome, and graft survival were compared in the two groups. Results Eighty KTs were performed in 78 patients with a median age of 11 years (interquartile range [IQR] 4.3–14) and a median body weight of 24 kg (IQR 13–37). Thirty-nine donor kidneys (49%) needed a complex BS due to anomalies of renal veins in 12 (31%) and renal arteries in 16 (41%). The remaining 11 grafts (28%) underwent an elongation of the vein. There was no difference in the rate of primary graft non function (p = 0.97), delayed graft function (p = 0.72), and overall survival (p = 0.27). The rates of vascular complications, bleedings, and venous graft thrombosis were similar (p = 0.51, p = 0.59, p = 0.78, respectively). No arterial thrombosis or stenosis was reported. Conclusion Complex BS did not compromise survival of the graft and did not put the allograft at risk of vascular complications, such as bleedings or thrombosis. [ABSTRACT FROM AUTHOR]

Introduction:  To investigate the current experience of the ARM-Net Consortium in the management of epididymo-orchitis (EO) in patients with anorectal malformations (ARMs), and to identify specific risk factors and the need for urological care involvement.Materials and Methods:  We retrospectively collected data of EO in patients with ARM between 2015 and 2019. Data on urological aspects, ARM type, surgical approach, associated anomalies, diagnosis, and treatment of EO were collected and analyzed.Results:  Twenty-nine patients were reported by 12 centers. Twenty-six patients with EO (90%) had ARM with a rectourinary fistula. Median age at first EO was 2 years (range: 15 days-27 years). Twenty patients (69%) experienced multiple EO, and 60% of recurrences were ipsilateral. Associated urological anomalies included vesicoureteral reflux (48%), urethral anomalies (41%), neurogenic bladder (41%), and ectopic vas (10%). A positive urine culture during EO was present in 69%. EO was treated with antibiotics (90%), limiting surgical exploration to 14%. Prevention of recurrences included surgery (bulking agents 15%, vasectomy 15%, and orchiectomy 5%) and antibiotic prophylaxis (20%).Conclusion:  Urologists may encounter patients with EO in ARM patients, frequently with positive urine culture. An appropriate urologic work-up for most ARM patients is necessary to identify and treat underlying risk factors. A practical scheme for the work-up is suggested for a close collaboration between pediatric surgeons and urologists. [ABSTRACT FROM AUTHOR]

Background: A prompt detection of graft venous thrombosis might preserve the transplanted organ. A real‐time near‐infrared spectroscopy (NIRS) monitoring of the allograft perfusion could fulfill this goal. The aim of this work was to report the trend of allograft perfusion (rSO2) after pediatric kidney transplantation (KT) complicated by graft venous thrombosis. Case presentation. An infant, affected by end‐stage kidney disease due to posterior urethral valves, underwent non‐living donor KT. The allograft presented both arterial and venous variants and required a complex bench surgery reconstruction. The perfusion of the allograft was monitored by real‐time transcutaneous NIRS. The early post‐operative was conditioned by worsening clinical conditions, and a graft venous thrombosis was detected after four hours since the transplantation. NIRS monitoring lasted for 348 minutes. Median lower pole rSO2‐value was 65% (IQR 62–66%), while the median upper pole rSO2‐value was 70% (IQR 70–71%). These data reflected the congestion of the lower pole, observed at the end of the transplantation. The lower pole showed inferior rSO2 values (p<0.0001). At the end of the monitoring, the decrease in peripheral rSO2, measured in left lower limb as a benchmark, was consistent with the ongoing hypovolemic shock and severe acute anemia. Conclusion: Transcutaneous NIRS might be a reliable device for monitoring allograft and peripheric perfusion after pediatric KT. The modifications of rSO2 values helped the clinicians manage the patients in the post‐operative and early detect acute complications. [ABSTRACT FROM AUTHOR]