Esophago-gastric junction findings on high resolution impedance manometry in children with esophageal atresia.

OBJECTIVES: Using high resolution impedance manometry (HRIM), this study characterized the esophago-gastric junction (EGJ) dynamics in children with esophageal atresia (EA). METHOD: Esophageal HRIM was performed in patients with EA aged less than 18 years. Objective motility patterns were analyzed, and EGJ data reported. Controls were pediatric patients without EA undergoing investigations for consideration of fundoplication surgery. RESULTS: Seventy-five patients (M:F = 43:32, median age 1 year 3 months [3 months-17 years 4 months]) completed 133 HRIM studies. The majority (64/75, 85.3%) had EA with distal tracheo-esophageal fistula. Compared with controls, liquid swallows were poorer in patients with EA, as evident by significant differences in distension pressure emptying and bolus flow time (BFT). The integrated relaxation pressure for thin liquid swallows was significantly different between EA types, as well as when comparing patients with EA with and without previous esophageal dilatations. The BFT for solid swallows was significantly different when compared with EA types. CONCLUSIONS: We have utilized HRIM in patients with EA to demonstrate abnormalities in their long-term EGJ function. These abnormalities correlate with poorer esophageal compliance and reduced esophageal peristalsis across the EGJ. Understanding the EGJ function in patients with EA will allow us to tailor long-term management to specific patients.

Psychosocial Outcomes of Parents of Children with Hirschsprung Disease Beyond Early Childhood.

PURPOSE: The lifelong impact of Hirschsprung disease (HD) upon children and their families is increasingly well recognized. Parental psychosocial wellbeing and family functioning are determinants of psychological and health-related outcomes in children with chronic conditions. We performed a cross-sectional cohort study to evaluate the psychosocial functioning of parents/caregivers of children with HD, beyond early childhood. METHODS: Parents/caregivers of children with HD, aged 4-14 years, managed at a tertiary pediatric surgical center were surveyed. Parent psychosocial outcomes, including adjustment to illness and family response, were assessed using four validated measures: Family Management Measure (FaMM); Parent Experience of Child Illness (PECI); Patient Reported Outcomes Measurement Information System (PROMISR) anxiety; and PROMISR depression. The Pediatric Quality of Life Inventory (PedsQL) was administered to assess child quality of life (proxy-report). RESULTS: Forty parents (mean age 38.7 ± 5.6 years) of children with HD (mean age 8.0 ± 2.5) participated. Parents expressed greater long-term uncertainty (PECI) and poorer perceived condition management ability (FaMM) than comparator chronic disease cohorts. Other scores for parental adjustment to their child's condition (PECI) and family response (FaMM) were comparable to reference cohorts. Symptoms of anxiety and depression were prevalent in our cohort (52.5 % and 42.5 % respectively); however, the proportion with moderate - severe PROMISR anxiety (χ2 = 2.50, p = 0.114) and depression (χ2 = 0.156, p = 0.693) scores did not significantly differ from the expected population distribution. Proxy-reported child quality of life (PedsQL) was significantly reduced relative to healthy children (p = 0.0003), but comparable to those with physical health problems with special healthcare needs (p = 0.624). CONCLUSIONS: Parents of children with HD experience long-term uncertainty and have poorer perceived condition management ability than parents of children with other chronic childhood illnesses. This work highlights the importance of targeted parental education and support beyond primary surgical management, and provides a benchmark for this cohort, against which subsequent intervention-based studies may be assessed. LEVEL OF EVIDENCE: II.

The role of individual differences in resistance to persuasion on memory for political advertisements.

When people see political advertisements on a polarized issue they take a stance on, what factors influence how they respond to and remember the adverts contents? Across three studies, we tested competing hypotheses about how individual differences in social vigilantism (i.e., attitude superiority) and need for cognition relate to intentions to resist attitude change and memory for political advertisements concerning abortion. In Experiments 1 and 2, we examined participants' intentions to use resistance strategies to preserve their pre-existing attitudes about abortion, by either engaging against opposing opinions or disengaging from them. In Experiment 3, we examined participants' memory for information about both sides of the controversy presented in political advertisements. Our results suggest higher levels of social vigilantism are related to greater intentions to counterargue and better memory for attitude-incongruent information. These findings extend our understanding of individual differences in how people process and respond to controversial social and political discourse.

Post-Operative Anorectal Manometry in Children following Anorectal Malformation Repair: A Systematic Review.

Despite surgical correction, children with anorectal malformations may experience long-term bowel dysfunction, including fecal incontinence and/or disorders of evacuation. Anorectal manometry is the most widely used test of anorectal function. Although considerable attention has been devoted to its application in the anorectal malformation cohort, there have been few attempts to consolidate the findings obtained. This systematic review aimed to (1) synthesize and evaluate the existing data regarding anorectal manometry results in children following anorectal malformation repair, and (2) evaluate the manometry protocols utilized, including equipment, assessment approach, and interpretation. We reviewed four databases (Embase, MEDLINE, the Cochrane Library, and PubMed) for relevant articles published between 1 January 1985 and 10 March 2022. Studies reporting post-operative anorectal manometry in children (<18 years) following anorectal malformation repair were evaluated for eligibility. Sixty-three studies were eligible for inclusion. Of the combined total cohort of 2155 patients, anorectal manometry results were reported for 1755 children following repair of anorectal malformations. Reduced resting pressure was consistently identified in children with anorectal malformations, particularly in those with more complex malformation types and/or fecal incontinence. Significant variability was identified in relation to manometry equipment, protocols, and interpretation. Few studies provided adequate cohort medical characteristics to facilitate interpretation of anorectal manometry findings within the context of the broader continence mechanism. This review highlights a widespread lack of standardization in the anorectal manometry procedure used to assess anorectal function in children following anorectal malformation repair. Consequently, interpretation and comparison of findings, both within and between institutions, is exceedingly challenging, if not impossible. Standardized manometry protocols, accompanied by a consistent approach to analysis, including definitions of normality and abnormality, are essential to enhance the comparability and clinical relevance of results.

Screening for VACTERL Anomalies in Children with Anorectal Malformations: Outcomes of a Standardized Approach.

PURPOSE: The majority of patients with an anorectal malformation (ARM) have associated congenital anomalies. It is well established that all patients diagnosed with an ARM should undergo systematic screening, including renal, spinal, and cardiac imaging. This study aimed to evaluate the findings and completeness of screening, following local implementation of standardized protocols. METHODS: A retrospective cohort study was performed assessing all patients with an ARM managed at our tertiary pediatric surgical center, following a standardized protocol implementation for VACTERL screening (January 2016-December 2021). Cohort demographics, medical characteristics, and screening investigations were analyzed. Findings were compared with our previously published data (2000-2015), conducted prior to protocol implementation. RESULTS: One hundred twenty-seven (64 male, 50.4%) children were eligible for inclusion. Complete screening was performed in 107/127 (84.3%) children. Of these, one or more associated anomalies were diagnosed in 85/107 (79.4%), whilst the VACTERL association was demonstrated in 57/107 (53.3%). The proportion of children that underwent complete screening increased significantly in comparison with those assessed prior to protocol implementation (RR 0.43 [CI 0.27-0.66]; p < 0.001). Children with less complex ARM types were significantly less likely to receive complete screening (p = 0.028). Neither presence of an associated anomaly, nor prevalence of the VACTERL association, differed significantly by ARM type complexity. CONCLUSION: Screening for associated VACTERL anomalies in children with ARM was significantly improved following standardized protocol implementation. The prevalence of associated anomalies in our cohort supports the value of routine VACTERL screening in all children with ARM, regardless of malformation type. LEVEL OF EVIDENCE: II.

Model-based identification of vadose zone controls on PFAS mobility under semi-arid climate conditions.

Contamination through per-and poly-fluoroalkyl substances (PFAS) have occurred globally in soil and groundwater systems at military, airport and industrial sites due to the often decades-long periodic application of firefighting foams. At PFAS contaminated sites, the unsaturated soil horizon often serves as a long-term source for sustained PFAS contamination for both groundwater and surface water runoff. An understanding of the processes controlling future mass loading rates to the saturated zone from these source zones is imperative to design efficient remediation measures. In the present study, hydrochemical data from a site where PFAS transport was observed as a result of the decades-long application of AFFF were used to develop and evaluate conceptual and numerical models that determine PFAS mobility across the vadose zone under realistic field-scale conditions. The simulation results demonstrate that the climate-driven physical flow processes within the vadose zone exert a dominating control on the retention of PFAS. Prolonged periods of evapotranspiration exceeding rainfall under the semi-arid conditions trigger periods of upward flux and evapoconcentration, leading to the observed persistence of PFAS compounds in the upper ca. 2 metres of the vadose zone, despite cessation of AFFF application to soils since more than a decade. Physico-chemical retention mechanisms, namely sorption to the air-water interface (AWI) and sediment surfaces, contribute further to PFAS retention. The simulations demonstrate how PFAS downward transport is effectively confined to short periods following discrete rain events when soils display a high degree of saturation. During these periods, AWI sorption is at a minimum. In addition, high PFAS concentrations measured and simulated below the source zone reduce the effect of the AWI further due to a decrease in surface tension associated with elevated PFAS concentrations. Consequently, time-integrated PFAS migration and retardation illuminates that the field-relevant PFAS transport rates are predominantly controlled by the physical flow processes with a lower relative importance of AWI and sediment sorption adding to PFAS retention.

Post-operative colonic manometry in children with anorectal malformations: A systematic review.

BACKGROUND: Children with anorectal malformations may experience constipation and fecal incontinence following repair. The contribution of altered anorectal function to these persistent symptoms is relatively intuitive; however, colonic motility in this cohort is less well understood. Manometry may be used to directly assess colonic motility. PURPOSE: The purpose of this systematic review was to synthesize the available evidence regarding post-operative colonic motility in children with anorectal malformations and evaluate the reported equipment and protocols used to perform colonic manometry in this cohort. This systematic review was conducted in compliance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). We conducted a systematic review of four databases: Embase, MEDLINE, PubMed, and the Cochrane Library (1st January 1985-22nd July 2021). Studies reporting colonic manometry performed in children following anorectal malformation repair were assessed for eligibility. Data were extracted independently by two authors. Four studies were eligible for inclusion. Of the combined total cohort of 151 children, post-operative colonic manometry was conducted in 35. Insufficient reporting of medical characteristics, bowel function, and manometric outcomes restricted comparison between studies, and limited clinical applicability. No results from high-resolution colonic manometry were identified. Despite the prevalence of post-operative bowel dysfunction in children with repaired anorectal malformations, this systematic review highlighted the markedly limited evidence regarding post-operative colonic motility. This cohort may benefit from assessment with high-resolution techniques; however, future work must emphasize adherence to standardized manometry protocols, and include robust reporting of surgical characteristics, bowel function, and manometric outcomes.

Narrative Comprehension Guides Eye Movements in the Absence of Motion.

Viewers' attentional selection while looking at scenes is affected by both top-down and bottom-up factors. However, when watching film, viewers typically attend to the movie similarly irrespective of top-down factors-a phenomenon we call the tyranny of film. A key difference between still pictures and film is that film contains motion, which is a strong attractor of attention and highly predictive of gaze during film viewing. The goal of the present study was to test if the tyranny of film is driven by motion. To do this, we created a slideshow presentation of the opening scene of Touch of Evil. Context condition participants watched the full slideshow. No-context condition participants did not see the opening portion of the scene, which showed someone placing a time bomb into the trunk of a car. In prior research, we showed that despite producing very different understandings of the clip, this manipulation did not affect viewers' attention (i.e., the tyranny of film), as both context and no-context participants were equally likely to fixate on the car with the bomb when the scene was presented as a film. The current study found that when the scene was shown as a slideshow, the context manipulation produced differences in attentional selection (i.e., it attenuated attentional synchrony). We discuss these results in the context of the Scene Perception and Event Comprehension Theory, which specifies the relationship between event comprehension and attentional selection in the context of visual narratives.

Short-term seawater inundation induces metal mobilisation in freshwater and acid sulfate soil environments.

Climate change is leading to global sea level rise. Storm surges and higher tides will generate short-term 'pulses' of seawater into freshwater systems, often for the first time in over 3000 years. The effect of increased seawater inundation upon soil geochemistry is poorly understood. We identified 12 sites in South Australia which are predicted to be inundated by seawater storm surges in the next 20 years. Within these 12 sites are three distinct environments; fresh water streams and lakes, hypersaline saltmarsh and mangroves, and acid sulfate soils. Soils were inundated with seawater under laboratory conditions to replicate a short-term (two weeks) inundation by a storm surge. Lowering of redox potential and dissolution of high concentrations of reactive Mn and Fe in freshwater environments lead to the release of dissolved Fe and Mn in the soils from freshwater environments. Soils also released As, Cu, Ni, Cd and Co, while Zn and Pb were less mobilised. Concentrations of metals released exceeded water quality guidelines to protect freshwater aquatic ecosystems in most cases. By comparison, hypersaline soils only released minor amounts of Mn, Fe, Cd and Ni, and only in some of the soils. The moderately acidic acid sulfate soil (pH 5.41) reductively dissolved Mn and Fe releasing significant amount of Fe and Mn as well as As, Cu, Ni, Cd and Co, whereas almost all metal species decreased in the porewaters of the strongly acidic acid sulfate soil (pH 2.77). The response to short-term seawater inundation in acid sulfate soils was dependent upon the baseline soil acidification status. This study highlights the need for further research on seawater inundation of coastal soils as sea levels rise and storm surges penetrate further inland.

Testicular descent: A review of a complex, multistaged process to identify potential hidden causes of UDT.

BACKGROUND/PURPOSE: What causes normal descent of the testis in a fetus, and what goes wrong with this complex process to cause undescended testes (UDT), or cryptorchidism? Over the last 2 decades, most authors searching for the cause(s) of UDT have looked at the 2 main hormones involved, insulin-like hormone 3 (Insl3) and testosterone (T)/ dihydrotestosterone (DHT), and their known upstream (hypothalamic-pituitary axis) and intracellular 'downstream' pathways. Despite these detailed searches, the genetic causes of UDT remain elusive, which suggest the aetiology is multifactorial, and/or we are looking in the wrong place. METHODS: In this review we highlight the intricate morphological steps involved in testicular descent, which we propose may contain the currently 'idiopathic' causes of UDT. By integrating decades of research, we have underlined many areas that have been overlooked in the search for causes of UDT. RESULTS: It is quite likely that the common causes of UDT are still hidden in these areas, and we suggest examining these processes is worthwhile in the hope of finding the common genetic anomalies that lead to cryptorchidism. Given the fact that a fibrous barrier preventing descent is often described at orchidopexy, examination of the extracellular matrix enzymes needed to allow gubernacular migration may be a fruitful place to start. CONCLUSION: This review of the complex anatomical steps and hormonal regulation of testicular descent highlights many areas of morphology and signalling pathways that have been overlooked in the search for causes of UDT.

BDNF is upregulated by androgen in the inguinal fat pad of immature mice and may regulate inguinoscrotal testicular descent.

BACKGROUND: Androgens control rodent inguinoscrotal testicular descent during a "programming window" (E12-17). It is proposed that androgen masculinises the genitofemoral nerve, but the mechanism remains unknown. We investigate androgen receptor (AR)-containing target organs: inguinal fat pad (IFP) and mammary bud (MB), supplied by the genitofemoral nerve, hypothesizing that neurotrophic factors may retrogradely masculinise the GFN. METHODS: The IFP, MB and bulbocavernosus (BC) muscle were collected at E12.5/E17.5 from androgen receptor knockout (ARKO) mice and wild-type (WT) littermates. Immunofluorescence and gene expression (RT-qPCR; n = 8/group) for Bdnf, active (TrkB) and inactive (truncated TrkB) receptors, Cntf and Cntf receptor were performed. RESULTS: In the IFP at E12.5, ARKO TrkB mRNA expression was significantly downregulated compared to WT males (p < 0.0026). By E17.5, there was increased Bdnf expression (p < 0.0233). The MB had no differences at E12.5 and had regressed in WT males by E17.5. The BC had no differences at E12.5, but at E17.5 had significant upregulation of Bdnf expression in ARKO, compared to WT males. There were no differences in CNTF or CNTF receptor expression. CONCLUSIONS: Androgen alters active TrkB and Bdnf expression in the IFP. IFP Bdnf signalling may regulate "masculinisation" of the GFN sensory nerves to indirectly control inguinoscrotal testicular descent. IMPACT: Androgen mediates neurotrophin release in the inguinal fat pad in mice, which may facilitate normal testicular descent by masculinising the GFN by peripheral uptake of neurotrophin. This is the first study to examine the role of neurotrophins in testicular descent. This suggests novel steps in the mechanical process of normal testicular descent that may be abnormal in some children with undescended testes.

Post-operative anorectal manometry in children with Hirschsprung disease: A systematic review.

BACKGROUND: Hirschsprung disease is commonly encountered by pediatric surgeons. Despite advances in the surgical management, these children may experience symptoms of bowel dysfunction throughout adulthood. Anorectal manometry may be used to assess post-operative anorectal structure and function. This review aimed to consolidate and evaluate the literature pertaining to post-operative findings of anorectal manometry in children with Hirschsprung disease. PURPOSE: (1) Synthesize the available data regarding anorectal motility patterns in children following repair of Hirschsprung disease. (2) Evaluate the reported anorectal manometry protocols. DATA SOURCES: We performed a systematic review of four databases: Embase, MEDLINE, the Cochrane Library, and PubMed. STUDY SELECTION: This systematic review was performed in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Studies reporting results of post-operative anorectal manometry in children with Hirschsprung disease were evaluated for inclusion. RESULTS: Twenty-three studies satisfied inclusion criteria, with a combined cohort of 939 patients. Post-operative anorectal manometry results were reported for 682 children. The majority of included studies were assessed as "poor quality." Disparate manometry protocols, heterogeneous cohorts, and lack of standardized outcome assessments introduced a risk of outcome reporting bias, limited the comparability of results, and impeded clinical translation of findings. CONCLUSIONS: This systematic review demonstrated the lack of high-quality evidence underlying the current understanding of post-operative anorectal motility in children with HD. There was little consistency in reported manometry outcomes between studies. In future work, emphasis must be placed on the application of standardized manometry protocols, cohort reporting, and patient outcome assessments.

The Majority of Boys Having Orchidopexy for Congenital Nonsyndromic Cryptorchidism during Minipuberty Exhibited Normal Reproductive Hormonal Profiles.

INTRODUCTION: The activation of the hypothalamic-pituitary-gonadal axis that occurs in male minipuberty during the first 5 months of life is important for early germ cell development. Orchidopexy during minipuberty may improve fertility potential as the germinative epithelium may benefit from the naturally occurring gonadotropin stimulation. We hypothesize that most boys with congenital nonsyndromic cryptorchidism display normal reproductive hormonal profiles and histological findings during minipuberty. METHODS: We included boys with congenital nonsyndromic cryptorchidism who underwent orchidopexy at less than 160 days of age, having no potential for spontaneous resolution clinically. At surgery, testicular biopsies and reproductive hormones were collected and compared with normal reference values. We measured the germ cells (G/T) and type A dark spermatogonia number per tubule. RESULTS: Thirty-five boys aged 37 to 159 (median age: 124) days at orchidopexy were included, five were bilateral. G/T was below the normal lower range in 26% (9/35) of the cases. In six of these cases, luteinizing hormone (LH) and follicle-stimulating hormone (FSH) were above 97.5 percentile, whereas one case had FSH below 2.5 percentile. Totally, 97% (33/34, one FSH was missing) exhibited a normal LH/FSH ratio. LH was more often above 97.5 percentile than FSH: 34% (12/35) versus 3% (1/34, p < 0.001). Inhibin B was below 2.5 percentile in 17% (6/35) of cases who all proved FSH above normal mean and four had LH above normal mean. CONCLUSION: Generally, reproductive hormonal profiles of the cryptorchid boys exhibited normal minipubertal pattern. Thus, 26% of the boys had reduced germ cell number, and transient hypogonadotropic hypogonadism could be suspected in few cases.

A simple and rapid ICP-MS/MS determination of sulfur isotope ratios (34S/32S) in complex natural waters: A new tool for tracing seawater intrusion in coastal systems.

Conventional sulfur isotope measurements in complex natural liquid or solid samples via GS-IRMS are complicated, time consuming and relatively expensive. Here we assessed a novel 'collision cell' based ICP-MS/MS approach which can determine the sulfur isotope abundances (i.e., 34S/32S ratios, expressed as δ34S) in complex coastal waters rapidly, accurately and with minimal sample preparation. The approach was validated via repeated ICP-MS/MS measurement of S isotope certified reference materials (CRM) providing accurate and reproducible results, with a typical uncertainty on δ34S of around 1.1-1.5‰ (1SD). This novel approach is suitable for water samples with sulfur concentrations at or above 2 µg/mL (ppm). Matrix matching between samples and the CRM was necessary when seawater-like solutions were analysed addressing common matrix related errors. The ICP-MS/MS approach was used to investigate δ34S signature of porewaters from a variety of coastal systems in South Australia (including acid sulfate soils), and how they responded to progressive seawater inundation. Importantly, inundation induced a shift in S isotope ratio in affected porewaters in which δ34S approached that of seawater. The simple sample preparation, with rapid and accurate δ34S determination of complex natural waters using the ICP MS/MS approach, greatly increases the applicability of sulfur isotope tracing studies to identify and monitor sources and bio-geochemical pathways of S in coastal and near-surface environments.

Post-operative colonic manometry in children with Hirschsprung disease: A systematic review.

BACKGROUND: A significant proportion of children experience bowel dysfunction (including constipation and fecal incontinence) following surgical repair of Hirschsprung disease (HD). Persistent symptoms are thought to relate to underlying colonic and/or anorectal dysmotility. Manometry may be used to investigate the gastrointestinal motility patterns of this population. PURPOSE: To (1) evaluate the colonic manometry equipment and protocols used in the assessment of the post-operative HD population and (2) summarize the available evidence regarding colonic motility patterns in children with HD following surgical repair. DATA SOURCES: We performed a systematic review of the Cochrane Library, Embase, MEDLINE, and PubMed databases (January 1, 1980 and March 9, 2020). Data were extracted independently by two authors. STUDY SELECTION: This systematic review was performed in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). Studies reporting the post-operative assessment of children with HD using colonic manometry were considered for inclusion. RESULTS: Five studies satisfied selection criteria, providing a combined total of 496 children. Of these, 184 children with repaired HD underwent colonic manometry. Studies assessed heterogeneous populations, utilized variable manometry equipment and protocols, and reported limited baseline symptom characteristics, thus restricting comparability. All studies used low-resolution colonic manometry. CONCLUSIONS: This systematic review highlighted the paucity of evidence informing the understanding of colonic dysmotility in the post-operative HD cohort. Current literature is limited by variable methodologies, heterogeneous cohorts, and the lack of high-resolution manometry.

Quality of Life Outcomes in Primary Caregivers of Children with Esophageal Atresia.

OBJECTIVE: To investigate the quality of life (QoL) impact on primary caregivers of children with esophageal atresia. STUDY DESIGN: We used a prospective cohort study design, inviting primary caregivers of children with esophageal atresia to complete the following questionnaires: Parent Experience of Child Illness (PECI), Patient-Reported Outcomes Measurement Information System (PROMIS) Anxiety, PROMIS Depression, 12-Item Short Form Survey (SF-12), and Pediatric Quality of Life Inventory (PedsQL). The PECI, PROMIS Anxiety and Depression, and SF-12 assessed caregiver QoL, and the PedsQL assessed patient QoL. Patients with Gross type E esophageal atresia served as controls. RESULTS: The primary caregivers of 100 patients (64 males, 36 females; median age, 4.6 years; range, 3.5 months to 19.0 years) completed questionnaires. The majority (76 of 100) of patients had Gross type C esophageal atresia. A VACTERL (vertebral anomalies, anorectal malformation, cardiac anomalies, tracheoesophageal fistula, renal anomalies, limb anomalies) association was found in 30, ≥1 esophageal dilatation was performed in 57, and fundoplication was performed in 11/100. When stratified by esophageal atresia types, significant differences were found in 2 PECI subscales (unresolved sorrow/anger, P = .02; uncertainty, P = .02), in PROMIS Anxiety (P = .02), and in SF-12 mental health (P = .02) and mental component summary scores (P = .02). No significant differences were found for VACTERL association, nor esophageal dilatation. Requirement for fundoplication resulted in lower SF-12 general health score, and lower PedsQL social and physical functioning scores. CONCLUSIONS: We have demonstrated that caring for a child with esophageal atresia and a previous requirement for fundoplication impacts caregiver QoL.

Complication profile of augmentation cystoplasty in contemporary paediatric urology: a 20-year review.

BACKGROUND: The aim of this study was to describe the complication profile of augmentation cystoplasty in contemporary paediatric urology as well as its effect on bladder metrics. METHODS: Consecutive operative cases were retrospectively reviewed at a single institution over 20 years (1999-2019). Short- and long-term outcomes and complications following augmentation cystoplasty were defined. RESULTS: Of the 71 operative cases; the most common underlying diagnoses were neurogenic bladder (34%), exstrophy-epispadias complex (30%) and posterior urethral valves (23%). The most common tissue-type utilized was ileal (58%) and ureteric (30%). Peri-operative urine leak affected nine (13%) children but reservoir perforations were less common (4%). Mean end-of-study detrusor pressure improved significantly following bladder augmentation (38-17 cmH2 O, P < 0.001). Bladder capacity improved significantly (67-89%, P = 0.041). The median follow-up was 4.5 years (interquartile range: 1.9-10 years). Bladder urolithiasis affected 13 (18%) patients, and symptomatic urinary tract infections 36 (51%) patients. Formation of a continent catheterisable channel contributed a number of complications relating predominantly to stenosis (50%). Repeat augmentation cystoplasty was necessary in three (4%) cases. CONCLUSION: Augmentation cystoplasty is a surgical intervention that improves bladder metrics. Given the potential complications, careful patient selection and appropriate pre-operative counselling are essential. Furthermore, pro-active post-operative management and transitional care are vital in the surgical care of children following augmentation cystoplasty.

Radiation burden in patients with esophageal atresia: a systematic review.

Esophageal atresia (EA) is the most common congenital esophageal disorder. Radiological imaging facilitates diagnosis, surgical interventions, and follow-up. Despite this, standardized monitoring guidelines are lacking. We aimed to: (1) review the literature regarding radiation burden in children with EA; (2) establish the presence of guidelines for diagnosis and follow-up in children with EA. The systematic review was performed according to PRISMA protocol. Two investigators conducted independent searches (PubMed, Ovid, Cochrane Review) and data extraction. Analysis focused on pre- and post-operative imaging type and frequency to determine the radiation burden. Seven studies met the inclusion criteria (337 patients). All authors agreed upon the need to minimize radiation burden, recommending symptoms-guided management, use of dosimeters, and non-radiating imaging. One study identified a median 130-fold increase in cumulative lifetime cancer risk in children with EA compared with other babies in the special care unit. The most common investigations were X-ray and CT (pre-operatively), and X-ray and contrast swallow (post-operatively). Standardized guidelines focused upon the frequency and type of radiological imaging for children with EA are lacking. Children with EA are subjected to more radiation exposure than the general population. Implementation of non-radiating imaging (ultrasonography, manometry) is recommended.

Survival Trends and Syndromic Esophageal Atresia.

BACKGROUND AND OBJECTIVES: Presence of a syndrome (or association) is predictive of poor survival in esophageal atresia (EA). However, most reports rely on historical patient outcomes, limiting their usefulness when estimating risk for neonates born today. We hypothesized improved syndromic EA survival due to advances in neonatal care. METHODS: A retrospective single-center review of survival in 626 consecutive patients with EA from 1980 to 2017 was performed. Data were collected for recognized risk factors: preterm delivery; birth weight <1500 g; major cardiac disease; vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, and limb abnormalities (VACTERL); and non-VACTERL syndromes. Cox proportional hazards regression models were used to evaluate temporal trends in survival with respect to year of birth and syndromic EA. RESULTS: Overall, 87% of 626 patients with EA survived, ranging from 82% in the 1980s to 91% in the 2010s. After adjusting for confounders, syndromic EA survival did not improve during the study, with no association found between year of birth and survival (hazard ratio [HR] 0.98, 95% confidence interval [CI]: 0.95-1.01). Aside from lethal non-VACTERL syndromes, patients with nonlethal non-VACTERL syndromes (HR 6.85, 95% CI: 3.50-13.41) and VACTERL syndrome (HR 3.02, 95% CI: 1.66-5.49) had a higher risk of death than those with nonsyndromic EA. CONCLUSIONS: Survival of patients with syndromic EA has not improved, and patients with non-VACTERL syndromes have the highest risk of death. Importantly, this is independent of syndrome lethality, birth weight, and cardiac disease. This contemporary survival assessment will enable more accurate perinatal counseling of parents of patients with syndromic EA.

During infancy low levels of follicle-stimulating hormone may result in high rate of germ cell apoptosis.

PURPOSE: It has been suggested that follicle-stimulating hormone (FSH) plays a role in preventing germ cell apoptosis. We aimed to compare apoptotic rate of boys with cryptorchidism having different levels of FSH in order to investigate its role in apoptosis. METHODS: Hormonal profiles and testicular biopsies from 30 boys with unilateral cryptorchidism (age range: 4-14 months) were included. Based on FSH level, the boys were grouped into three (3 × 10) having high (>97.5percentile), low (<2.5percentile), or within normal range. Sections underwent immunohistochemical staining to analyze the number of germ cells and type A dark spermatogonia per cross-sectional tubule. One section was co-stained with immunofluorescent antibodies against an apoptotic marker (cleaved caspase-3), proliferation marker (Ki67), Sertoli cell marker (anti-Müllerian hormone) and processed by confocal imaging for analysis. Germ cell apoptosis was calculated as the apoptosis index (percentage caspase-3+ germ cells/total germ cell number). RESULTS: Fifty percent (5/10) of the boys with low FSH had an apoptosis index above 90% compared with 15% (3/20) of the boys with normal or high FSH (p = 0.04). Caspase-3+ germ cells were most likely to be located on the basement membrane (p<0.05). CONCLUSION: Our findings lead to trends proposing that FSH may play a role in preventing apoptosis. TYPE OF STUDY: Prognosis Study LEVEL OF EVIDENCE: III.