Fabian Doktor, Emily Lo, Victoria Fortuna, Kasra Khalaj, Miguel Garcia, Rebeca Lopes Figueira, Martin Lacher, Lina Antounians, Augusto Zani
{"title":"Sex-specific differences in the severity of pulmonary hypoplasia in experimental congenital diaphragmatic hernia and implications for extracellular vesicle-based therapy.","authors":"Fabian Doktor, Emily Lo, Victoria Fortuna, Kasra Khalaj, Miguel Garcia, Rebeca Lopes Figueira, Martin Lacher, Lina Antounians, Augusto Zani","doi":"10.1007/s00383-024-05856-0","DOIUrl":"https://doi.org/10.1007/s00383-024-05856-0","url":null,"abstract":"<p><strong>Purpose: </strong>Amniotic fluid stem cell extracellular vesicles (AFSC-EVs) hold regenerative potential to treat hypoplastic lungs secondary to congenital diaphragmatic hernia (CDH). This study aims to investigate sex-specific differences in pulmonary hypoplasia severity and responses to AFSC-EV administration in an experimental CDH mouse model.</p><p><strong>Methods: </strong>C57BL/6J dams were fed with nitrofen + bisdiamine (left-sided CDH) or olive oil only (control) at embryonic day (E) 8.5. Lungs were dissected (E18.5), grown ex vivo and treated with medium ± AFSC-EVs that were collected via ultracentrifugation and characterized (nanoparticle tracking analysis, electron microscopy, Western blotting). Pulmonary hypoplasia was assessed via mean linear intercept (MLI). Gene and protein expression changes (Cd31, Enos, Il1b, TNFa) were measured via RT-qPCR and immunofluorescence. Pups were genotyped for Sry.</p><p><strong>Results: </strong>Experimental CDH showed a male predominance without sex differences for pulmonary hypoplasia severity, fetal lung vascularization, and inflammation. AFSC-EV administration led to improved lung growth (decreased MLI), improved fetal lung vascularization (increased Cd31 and Enos), and decreased fetal lung inflammation (Il1b, TNFa). There was no sex-specific response to AFSC-EV administration.</p><p><strong>Conclusion: </strong>This study shows sex-independent impaired lung growth, vascularization and fetal lung inflammation in a CDH mouse model. Antenatal administration of AFSC-EVs reverses aspects of pulmonary hypoplasia secondary to CDH independent of the biological sex.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"278"},"PeriodicalIF":1.5,"publicationDate":"2024-10-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142522653","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Angiopoietin-1 attenuates lipopolysaccharide-induced endotoxemia in a Hirschsprung's disease murine model by improving intestinal vascular integrity: implications for treating postoperative Hirschsprung-associated enterocolitis.","authors":"Asuka Ishiyama, Kazuto Suda, Xuxuan Rao, Minhua Sun, Geoffrey J Lane, Atsuyuki Yamataka, Hiroyuki Koga","doi":"10.1007/s00383-024-05867-x","DOIUrl":"https://doi.org/10.1007/s00383-024-05867-x","url":null,"abstract":"<p><strong>Purpose: </strong>Angiopoietin-1 (Ang1) mitigates inflammation as a proangiogenic growth factor. Action of Ang1 on lipopolysaccharide (LPS)-induced endotoxemic inflammation was investigated in endothelin receptor-B null Hirschsprung's disease mice (KO).</p><p><strong>Methods: </strong>LPS or saline was injected intraperitoneally in KO (KO-LPS; n = 9, KO-sal; n = 5) and wild-type (WT) (WT-LPS; n = 6, WT-sal; n = 6) pups obtained within 24 h of birth. Normoganglionic terminal ileum harvested 6 h after LPS was used for RNA extraction and histology. IL-1β, SELE, VEGFA, Ang1, Angiopoietin-2 (Ang2), and TIE2 expression analyzed by quantitative polymerase chain reaction (qPCR), vascular permeability assessed by the Miles assay, severity of inflammation, and immunofluorescence for phospho-TIE2 and VE-cadherin were used to assess endothelial cell contact integrity and compared with KO pups pretreated with intraperitoneal Ang1 [Ang1(KO-LPS); n = 5] or saline [sal(KO-LPS); n = 6] 2 h before LPS.</p><p><strong>Results: </strong>KO-LPS pups showed significantly increased inflammation (p < 0.05) and expression of IL-1β, SELE, VEGFA, and Ang2 (p = 0.019, 0.003, 0.008 and < 0.0001, respectively); expression of Ang1 and TIE2 remained unchanged when compared with KO-saline. In Ang1(KO-LPS) ileum, changes seen in sal(KO-LPS) were eliminated and phospho-TIE2 and VE-cadherin fluorescence increased.</p><p><strong>Conclusion: </strong>Ang1 successfully attenuated LPS-induced normoganglionic intestinal inflammation, downregulated pro-inflammatory genes, and improved vascular barrier integrity in KO pups.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"277"},"PeriodicalIF":1.5,"publicationDate":"2024-10-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142522651","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Features of recurrence and contralateral metachronous inguinal hernia (CMIH) in the patients underwent laparoscopic percutaneous extraperitoneal closure (LPEC) for pediatric inguinal hernia.","authors":"Hiromu Miyake, Masaya Yamoto, Akiyoshi Nomura, Yu Sugai, Yousuke Gohda, Yutaro Yamashiro, Koji Fukumoto","doi":"10.1007/s00383-024-05859-x","DOIUrl":"https://doi.org/10.1007/s00383-024-05859-x","url":null,"abstract":"<p><strong>Purpose: </strong>Recurrence and contralateral metachronous inguinal hernia (CMIH) are important postoperative complications in patients with inguinal hernia (IH) who undergo laparoscopic percutaneous extraperitoneal closure (LPEC). This study aimed to evaluate the incidence and causes of recurrence and CMIH after LPEC.</p><p><strong>Methods: </strong>Among the included patients, 2484 hernias were included in the analysis of recurrence. CMIH was analyzed in 2094 cases with unilateral IH without a history of contralateral IH.</p><p><strong>Results: </strong>Overall, 21 hernias (0.85%) recurred and seven patients (0.34%) experienced CMIH. Based on the video inspection, injury and skipping of the peritoneum were suspected as the cause of recurrence in eight cases, while loose ligation was suspected in five cases. The cause of recurrence could not be predicted in the remaining eight patients. Of the seven patients with CMIH, three had a mild depression around the internal inguinal ring at the first surgery. In the remaining four CMIH cases, no noteworthy findings were noted around the internal ring.</p><p><strong>Conclusions: </strong>More than half of the recurrences, in which skipping of the peritoneum and loose ligation were suspected, could have been prevented by secure ligation during the first surgery. Extending the indications of prophylactic surgery for shallow depressions may reduce CMIH.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"276"},"PeriodicalIF":1.5,"publicationDate":"2024-10-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142522652","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Naho Fujiwara, Dorothy Lee, Bo Li, Agostino Pierro, Atsuyuki Yamataka
{"title":"Enhancement of enteric neural stem cell neurogenesis by glial cell-derived neurotrophic factor in experimental Hirschsprung's disease.","authors":"Naho Fujiwara, Dorothy Lee, Bo Li, Agostino Pierro, Atsuyuki Yamataka","doi":"10.1007/s00383-024-05861-3","DOIUrl":"10.1007/s00383-024-05861-3","url":null,"abstract":"<p><strong>Purpose: </strong>Stem cell therapy offers a promising solution for congenital diseases like Hirschsprung's disease (HSCR). Optimizing stem cell efficacy by modifying the cells and their environment is crucial, but in vitro culture conditions need to be further improved. Glial cell-derived neurotrophic factor (GDNF) plays an important role in neuronal survival, proliferation, migration and differentiation during enteric nervous system (ENS) development. In this study, the effects of GDNF on neurites derived from an Ednrb knockout model were investigated with the aim of enhancing the neurogenic potential of enteric neural crest cells (ENCCs).</p><p><strong>Methods: </strong>Neurospheres were generated form Ednrb<sup>+/+</sup> (control) and Ednrb<sup>-/-</sup> mice at embryonic day13.5 (E13.5) with Sox10-green fluorescent protein (Venus) transgenic expression. These neurospheres were cultured in control media and neurospheres from Ednrb<sup>-/-</sup> were cultured with either control media or media supplemented with GDNF. ENCCs differentiation was assessed using immunofluorescence staining after 18 days.</p><p><strong>Results: </strong>GDNF-treated Ednrb<sup>-/-</sup> neurospheres showed increased size and higher density of Sox10-positive ENCCs compared to untreated Ednrb<sup>-/-</sup> neurospheres. GDNF also enhanced the distribution of both TUJ1-positive neurons and S100-positive glial cells.</p><p><strong>Conclusion: </strong>GDNF effectively enhanced the neurogenic potential of ENCCs from HSCR animal model. This finding is crucial for the development of cell therapy in HSCR.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"274"},"PeriodicalIF":1.5,"publicationDate":"2024-10-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142505545","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ryosuke Satake, Hiroshi Yamakawa, Nozomi Aoki, Rina Tanaka, Sakiko Yoshimoto, Tokiko Okunobo, Hiroki Nakamura, Takashi Doi
{"title":"A biologically transparent illumination device is more useful in children for detecting the position of the nasogastric tube in the stomach.","authors":"Ryosuke Satake, Hiroshi Yamakawa, Nozomi Aoki, Rina Tanaka, Sakiko Yoshimoto, Tokiko Okunobo, Hiroki Nakamura, Takashi Doi","doi":"10.1007/s00383-024-05854-2","DOIUrl":"10.1007/s00383-024-05854-2","url":null,"abstract":"<p><strong>Purpose: </strong>The study aimed to evaluate the efficacy of the Biologically Transparent Illumination (BTI) device for confirming the correct placement of nasogastric (NG) tubes in children, as an alternative to X-ray, which exposes patients to radiation.</p><p><strong>Methods: </strong>In this prospective observational study, 106 pediatric patients (ages 0-16) undergoing NG-tube insertion after general anesthesia were evaluated. The BTI catheter was used to emit bio-permeable red light from the NG tube, which was then visually confirmed in the cervical, thoracic, and epigastric regions. X-ray confirmed NG-tube placement in all patients. The ethics committee approved the study.</p><p><strong>Results: </strong>The average patient age was 3.8 years, with a male-to-female ratio of 72:34. BTI was successfully detected in the epigastric area in 105 of 106 patients, with one 9-year-old patient having unclear BTI visibility. X-ray confirmed NG-tube placement in the stomach for all patients, resulting in a BTI sensitivity of 99%. The mean NG-tube insertion time was 38 s, and the mean abdominal thickness was 9.8 mm.</p><p><strong>Conclusions: </strong>The BTI device proved to be a safe and effective method for NG-tube placement in children, offering a radiation-free alternative with 100% successful placement when BTI was detected in the epigastric area.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"275"},"PeriodicalIF":1.5,"publicationDate":"2024-10-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142505544","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mario O'Connor, Andrew Well, Joshua Morgan, Michael Y Liu, Michael D Josephs, Neil M Venardos, Charles D Fraser, Carlos M Mery
{"title":"Management and outcomes of acute appendicitis in children with congenital heart disease.","authors":"Mario O'Connor, Andrew Well, Joshua Morgan, Michael Y Liu, Michael D Josephs, Neil M Venardos, Charles D Fraser, Carlos M Mery","doi":"10.1007/s00383-024-05864-0","DOIUrl":"https://doi.org/10.1007/s00383-024-05864-0","url":null,"abstract":"<p><strong>Background: </strong>Congenital heart disease (CHD) care has evolved during the past decades. Advances in care have contributed to improved survival among CHD patients. Children with CHD are requiring interventions for non-CHD related medical issues that occur in the general pediatric population.</p><p><strong>Methods: </strong>A retrospective review of the Pediatric Health Information System (PHIS) database from January 1, 2004, to July 31, 2023. Discharges of patients with an admitting/principal diagnosis of appendicitis were evaluated and categorized as CHD or non-CHD.</p><p><strong>Results: </strong>A total of 319,228 patients were identified with 1,25,858(39.4%) female, 1,38,966(43.5%) white, and median age of 11[IQR:8-14] years. 708(0.2%) had CHD with 85(12%) of them having a diagnosis consistent with single-ventricle CHD (SV-CHD). In univariate analysis, CHD patients were more likely to undergo conservative treatment (n = 172(24.2%)vs n = 59,358(18.6%)) and less likely to undergo laparoscopic appendectomy (n = 483(68.2%) vs n = 2,35,324(73.8%))(p < 0.001) compared to non-CHD. After adjustment, CHD patients had increased odds of undergoing open appendectomy compared to non-CHD. CHD patients were more likely to have an ICU admission (OR:8.36(95%CI 6.35-10.00),p < 0.001) and had a 77.6%(95%CI 40.89-123.93) increase in length of stay (LOS) (p < 0.001).</p><p><strong>Conclusion: </strong>CHD patients are more likely to have an open appendectomy than non-CHD patients. These findings suggest a distinctive pattern in the care of CHD patients compared to non-CHD. Overall, CHD patients had a more intense level of care with longer LOS and increased ICU admissions. Further work is needed to evaluate drivers of management decisions, the role of conservative treatment with antibiotics alone in the CHD population, and the potential impacts and safety of a laparoscopic approach.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"273"},"PeriodicalIF":1.5,"publicationDate":"2024-10-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142505546","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Alejandra Castrillo, Soichi Shibuya, Eri Ueda, Manuel López, Geoffrey J Lane, Ryohei Kuwatsuru, Atsuyuki Yamataka, Hiroyuki Koga
{"title":"Role of percutaneous transhepatic biliary drainage for managing bile lake formation after Kasai portoenterostomy.","authors":"Alejandra Castrillo, Soichi Shibuya, Eri Ueda, Manuel López, Geoffrey J Lane, Ryohei Kuwatsuru, Atsuyuki Yamataka, Hiroyuki Koga","doi":"10.1007/s00383-024-05857-z","DOIUrl":"https://doi.org/10.1007/s00383-024-05857-z","url":null,"abstract":"<p><strong>Purpose: </strong>Bile lake (BL) formation following Kasai portoenterostomy (KPE) can complicate the prognosis of biliary atresia (BA). Percutaneous transhepatic biliary drainage (PTBD) performed under fluoroscopic/ultrasonographic (US) guidance is discussed for the management of BL.</p><p><strong>Methods: </strong>A retrospective review of 64 BA patients treated by KPE (open = 31, laparoscopic = 33) at a single center (2004-2023) identified 9 BL cases (9/64; 14.1%). PTBD was indicated for jaundice or cholangitis refractory to antibiotic therapy.</p><p><strong>Results: </strong>All BL were asymptomatic, diagnosed after an episode of postoperative cholangitis. KPE type was not correlated with BL incidence; 2/31 (6.5%) for open and 7/33 (21.2%) for laparoscopic; p = 0.15. Median onset was postoperative day 273 (IQR: 170-920). One case resolved with antibiotics while another case required early liver transplantation (LTx) due to advanced hepatic dysfunction unrelated to BL. All remaining cases (7/9) had PTBD at a median of 14.3 months (IQR: 7.3-34.7) post-KPE, with successful resolution in 6/7; one case required two PTBD procedures. Post-PTBD biliary peritonitis (n = 3) was resolved by abdominal lavage (laparoscopic = 2; open = 1).</p><p><strong>Conclusion: </strong>Screening for BL is advisable in all postoperative BA patients especially when cholangitis occurs. Although the risk for biliary peritonitis warrants caution, PTBD seems a viable option for managing BL formation after KPE.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"272"},"PeriodicalIF":1.5,"publicationDate":"2024-10-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142505547","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The 37th international symposium on pediatric surgical research.","authors":"Prem Puri","doi":"10.1007/s00383-024-05852-4","DOIUrl":"https://doi.org/10.1007/s00383-024-05852-4","url":null,"abstract":"","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"271"},"PeriodicalIF":1.5,"publicationDate":"2024-10-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142472096","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Norhafiza Ab Rahman, Mohd Yusof Abdullah, Muhammad 'Adil Zainal Abidin, Shireen Anne Nah
{"title":"Burden and mortality of congenital gastrointestinal anomalies: insights from a nationwide cohort study.","authors":"Norhafiza Ab Rahman, Mohd Yusof Abdullah, Muhammad 'Adil Zainal Abidin, Shireen Anne Nah","doi":"10.1007/s00383-024-05844-4","DOIUrl":"https://doi.org/10.1007/s00383-024-05844-4","url":null,"abstract":"<p><strong>Purpose: </strong>Improved perioperative care has enhanced survival in children with congenital gastrointestinal conditions and abdominal wall defects (AWD). However, epidemiological and surgical outcomes in developing nations are still scarce. Our aim was to assess the burden and mortality of common congenital gastrointestinal anomalies and AWD in Malaysia, and their influencing factors.</p><p><strong>Methods: </strong>Using the Global PaedSurg study protocol with permission, we performed a prospective cohort study on children presenting for the first time between October 2021 and April 2022 with these conditions: Esophageal atresia (EA), congenital diaphragmatic hernia (CDH), intestinal atresia, gastroschisis, exomphalos, anorectal malformation (ARM) and Hirschsprung's disease. We compared mortality and 30-day outcome data across different geographical regions in Malaysia.</p><p><strong>Results: </strong>There were 228 patients with 242 study conditions (EA n = 28, CDH n = 36, intestinal atresia n = 49, gastroschisis n = 12, exomphalos n = 8, ARM n = 77, Hirschsprung's disease n = 32). Our mortality rate was 8.8%; 60% of these were CDH patients. Factors significantly associated with mortality were CDH diagnosis, central venous access requirement, higher American Society of Anesthesiologists (ASA) score, blood transfusion and ventilation requirement.</p><p><strong>Conclusion: </strong>Diagnosis of CDH is the most important predictor for sepsis on arrival and mortality, therefore measures should be taken for early recognition and aggressive management.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"270"},"PeriodicalIF":1.5,"publicationDate":"2024-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142472095","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Joshua Gertler, Jenny Oddsberg, Anna Gunnarsdóttir, Anna Svenningsson, Tomas Wester, Lisa Örtqvist
{"title":"Sexual function and fertility in young female adults surgically treated for anorectal malformations.","authors":"Joshua Gertler, Jenny Oddsberg, Anna Gunnarsdóttir, Anna Svenningsson, Tomas Wester, Lisa Örtqvist","doi":"10.1007/s00383-024-05847-1","DOIUrl":"10.1007/s00383-024-05847-1","url":null,"abstract":"<p><strong>Purpose: </strong>The aim was to investigate sexual function and fertility in female adults operated on for anorectal malformations (ARM).</p><p><strong>Methods: </strong>This was a cross-sectional questionnaire-based study including female adult patients treated for ARM at our institution between 1994 and 2003. Sexual function in females was assessed using the Profile of Sexual Function (PFSF). Additional questions regarding fertility were answered by the participants. Patient characteristics were retrospectively retrieved from the medical records and descriptive statistics were used for analysis. Sexual function outcomes were compared to a control group from a previously published group of females. Composite outcome analysis was performed using previously published data to determine the potential impact of bowel function and health-related quality of life on sexual function. The ethics review authorities approved the study.</p><p><strong>Results: </strong>A total of 14 of 30 (46.7%) females responded to the questionnaires and had a mean age of 21.1 years (range 18-26). No association was found between PFSF and age or bowel function (Bowel Function Score), however, a strong correlation was found between PFSF and health-related quality of life (HRQoL) with a Spearman correlation of ρ 0.82 (p = 0.0011). The general satisfaction question was strongly associated with their total PFSF score (ρ = 0.71, p = 0.0092). Except for the \"desire\" item, the females in this cohort did not have significantly worse sexual function than the control population(p = 0.015). Ten of fourteen (71.4%) females had had their sexual debut at a mean age of 16.3 years and two of these women (20%) have been pregnant. All females had had menarche at a mean age of 12.7 years.</p><p><strong>Conclusion: </strong>Sexual function in adult females was comparable to healthy controls except for the \"desire\" item where the cohort reported poorer outcomes. The cohort's sexual function had a direct association with their reported HRQoL where individuals with worse HRQoL also reported poorer sexual function.</p><p><strong>Level of evidence: </strong>III.</p>","PeriodicalId":19832,"journal":{"name":"Pediatric Surgery International","volume":"40 1","pages":"269"},"PeriodicalIF":1.5,"publicationDate":"2024-10-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11467109/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142400935","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}