Percutaneous Anorectoplasty (PARP)-An Adaptable, Minimal-Invasive Technique for Anorectal Malformation Repair

Background: Anorectal malformations comprise a broad spectrum of disease. We developed a percutaneous anorectoplasty (PARP) technique as a minimal-invasive option for repair of amenable types of lesions. Methods: Patients who underwent PARP at five institutions from 2008 through 2021 were retrospectively analyzed. Demographic information, details of the operative procedure, and perioperative complications and outcomes were collected. Results: A total of 10 patients underwent the PARP procedure during the study interval. Patients either had low perineal malformations or no appreciable fistula. Most procedures were guided by ultrasound, fluoroscopy, or endoscopy. Median age at PARP was 3 days (range 1 to 311) days;eight patients were male. Only one intraoperative complication occurred, prompting conversion to posterior sagittal anorectoplasty. Functional outcomes in most children were highly satisfactory in terms of continence and functionality. Conclusions: The PARP technique is an excellent minimal-invasive alternative for boys born with perineal fistulae, as well as patients of both sexes without fistulae. The optimal type of guidance (ultrasound, fluoroscopy, or endoscopy) depends on the anatomy of the lesion and the presence of a colostomy at the time of repair

Habitual physical activity in patients born with oesophageal atresia: a multicenter cross-sectional study and comparison to a healthy reference cohort matched for gender and age

Oesophageal atresia (EA) is associated with life-long gastrointestinal and respiratory morbidity and other associated malformations. The aim of this study is to compare physical activity (PA) levels of children and adolescents with and without EA. A validated questionnaire (MoMo-PAQ) was used to evaluate PA in EA patients EA (4–17 years), who were randomly matched for gender and age (1:5) with a representative sample of the Motorik-Modul Longitudinal Study (n = 6233). Sports activity per week (sports index) and minutes of moderate to vigorous physical activity per week (MVPA minutes) were calculated. Correlations between PA and medical factors were analysed. In total, 104 patients and 520 controls were included. Children with EA were significantly less active at higher intensities (mean MPVA minutes 462; 95% confidence interval (CI): 370–554) compared to controls (626; 95% CI: 576–676), although there was no statistically significant difference in the sports index (187; 95% CI: 156–220 versus 220; 95% CI: 203–237). A lower mean weight-for-age and height-for-age, additional urogenital (r =  − 0.20, p = 0.04) or anorectal malformation (r =  − 0.24, p = 0.01) were associated with fewer MVPA minutes. For other medical factors (prematurity, type of repair, congenital heart disease, skeletal malformation or symptom load), no statistically significant association with PA was found. Conclusion: EA patients participated in PA at a similar level but lower intensities compared to the reference cohort. PA in EA patients was largely independent of medical factors

Identification of a PA-Binding Peptide with Inhibitory Activity against Influenza A and B Virus Replication

There is an urgent need for new drugs against influenza type A and B viruses due to incomplete protection by vaccines and the emergence of resistance to current antivirals. The influenza virus polymerase complex, consisting of the PB1, PB2 and PA subunits, represents a promising target for the development of new drugs. We have previously demonstrated the feasibility of targeting the protein-protein interaction domain between the PB1 and PA subunits of the polymerase complex of influenza A virus using a small peptide derived from the PA-binding domain of PB1. However, this influenza A virus-derived peptide did not affect influenza B virus polymerase activity. Here we report that the PA-binding domain of the polymerase subunit PB1 of influenza A and B viruses is highly conserved and that mutual amino acid exchange shows that they cannot be functionally exchanged with each other. Based on phylogenetic analysis and a novel biochemical ELISA-based screening approach, we were able to identify an influenza A-derived peptide with a single influenza B-specific amino acid substitution which efficiently binds to PA of both virus types. This dual-binding peptide blocked the viral polymerase activity and growth of both virus types. Our findings provide proof of principle that protein-protein interaction inhibitors can be generated against influenza A and B viruses. Furthermore, this dual-binding peptide, combined with our novel screening method, is a promising platform to identify new antiviral lead compounds

Esophageal Magnetic Compression Anastomosis in Esophageal Atresia Repair: A PRISMA-Compliant Systematic Review and Comparison with a Novel Approach.

The use of magnet compression to endoscopically create an esophageal anastomosis is an intriguing approach to esophageal atresia repair, but published cases with an existing available device have demonstrated mixed success. One major shortcoming has been the formation of subsequent severe, recalcitrant strictures after primary repair. To address the limitations of the existing device, we recently introduced and reported success with specially designed bi-radial magnets that exhibit a novel geometry and unique tissue compression profile. The aim of this study is to compare the outcomes using our novel device (novel group, NG) with those of previous reports which utilized the historical device (historic group, HG) in a PRISMA-compliant systematic review. Seven studies were eligible for further analysis. Additionally, one of our previously unreported cases was included in the analysis. Esophageal pouch approximation prior to primary repair was performed more frequently in the NG than in the HG (100% NG vs. 21% HG; p = 0.003). There was no difference in the overall postoperative appearance of postoperative stricture (95% HG vs. 100% NG; p = 0.64). The number of postoperative dilatations trended lower in the NG (mean 4.25 NG vs. 9.5 HG; p = 0.051). In summary, magnetic compression anastomosis adds a new promising treatment option for patients with complex esophageal atresia. Prior approximation of pouches and a novel magnet design have the potential to lower the rate of stricture formation

Pterygium axillae as a rare manifestation of Poland syndrome

AbstractPoland syndrome is characterized by a combination of absent pectoralis muscle, abnormalities of the rib cage, the breast, as well as brachy-syndactyly. We report a case of a 3 month old girl who was born with right sided axillary pterygium combined with flattening of the right anterior chest wall. Resection of a sclerotic band with reconstruction by Z-plasty was performed. Intraoperative and histopathological findings confirmed that the axillary pterygium developed on the basis of a scarred, hypoplastic pectoralis major muscle. Our case adds to the body of evidence that Poland syndrome may present in a heterogeneous fashion, including the rare finding of axillary pterygium with associated contracture. In these cases, early intervention prevents functional impairment

Telementoring in Minimally Invasive Esophageal Atresia Repair: Results of a Case-Control Study and Lessons Learned from the TIC-PEA Study (Telemedical Interdisciplinary Care for Patients with Esophageal Atresia)

Minimally invasive esophageal atresia (EA) repair is deemed one of the most demanding procedures in pediatric surgery. Open repair is considered the gold standard and learning opportunities for minimally invasive repairs remain scarce. “Telemedical Interdisciplinary Care for Patients with Esophageal Atresia (TIC-PEA)” offers free access to an interdisciplinary network of experts for telemedical consultation (telementoring). The aim of this study was to determine the frequency of minimally invasive surgery (MIS) in TIC-PEA patients compared to the general population. TIC-PEA patients were matched and compared to controls regarding the use of MIS, patient characteristics, and complications. Patients (n = 31) were included at a mean age of 62.8 days (95%-CI: 41.4–84.3, 77% after the primary esophageal repair). The odds-ratio to have MIS was 4.03 (95%-confidence interval: 0.79–20.55) for esophageal anastomosis and 4.60 (95%-confidence interval: 0.87–24.22) for tracheoesophageal fistula-repair in the TIC-PEA group. Telementoring offered the chance to select the ideal candidate for MIS, plan the procedure, and review intraoperative images and videos with the expert. Telementoring as offered is ideal to promote MIS for EA and helps to address the individual learning curve. In order to maximize benefits, patients need to be included prior to the first esophageal procedure

Esophageal Magnetic Compression Anastomosis in Esophageal Atresia Repair: A PRISMA-Compliant Systematic Review and Comparison with a Novel Approach.

The use of magnet compression to endoscopically create an esophageal anastomosis is an intriguing approach to esophageal atresia repair, but published cases with an existing available device have demonstrated mixed success. One major shortcoming has been the formation of subsequent severe, recalcitrant strictures after primary repair. To address the limitations of the existing device, we recently introduced and reported success with specially designed bi-radial magnets that exhibit a novel geometry and unique tissue compression profile. The aim of this study is to compare the outcomes using our novel device (novel group, NG) with those of previous reports which utilized the historical device (historic group, HG) in a PRISMA-compliant systematic review. Seven studies were eligible for further analysis. Additionally, one of our previously unreported cases was included in the analysis. Esophageal pouch approximation prior to primary repair was performed more frequently in the NG than in the HG (100% NG vs. 21% HG; p = 0.003). There was no difference in the overall postoperative appearance of postoperative stricture (95% HG vs. 100% NG; p = 0.64). The number of postoperative dilatations trended lower in the NG (mean 4.25 NG vs. 9.5 HG; p = 0.051). In summary, magnetic compression anastomosis adds a new promising treatment option for patients with complex esophageal atresia. Prior approximation of pouches and a novel magnet design have the potential to lower the rate of stricture formation

Reply to Peña, A. Comment on “Küppers et al. Percutaneous Anorectoplasty (PARP)—An Adaptable, Minimal-Invasive Technique for Anorectal Malformation Repair. <i>Children</i> 2022, <i>9</i>, 587”

Thank you so much for your thoughtful comments [...

Inhibition of FluA and FluB by the dual binding peptide PB1_1–25A_T6Y.

<p>Plaque reduction assay using PB1_1–25A-Tat; PB1_1–25A_T6Y-Tat; PX-Tat (control peptide) <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0007517#pone.0007517-Ghanem1" target="_blank">[32]</a> with FluA, FluB and VSV (vesicular stomatitis virus). A H₂O control was used to standardize the assay to 100%. Note that PB1_1–25B-Tat could not be tested due to insolubility. Error bars represent S.D.</p