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.

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.

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.

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.

Transport and retention of graphene oxide nanoparticles in sandy and carbonaceous aquifer sediments: Effect of physicochemical factors and natural biofilm.

There is a paucity of information regarding the interaction between GONPs and natural aquifer sediments. Therefore, batch and column experiments were carried out to determine the transport, retention and attachment behavior of GONPs with the surfaces of native aquifer sediments. The experiments were performed with sediments comprising contrasting mineralogical features (sand grains, quartz and limestone sediments), at different temperatures, ionic strength and compositions. Uniquely, this research also investigated the effect of natural biofilm on the retention behavior of nanoparticles in porous media. The retention rate of GONPs at 22 °C was higher than at 4 °C. Moreover, there was greater retention of GONPs onto the surfaces of collectors at higher ionic strengths and cation valence. The retention profiles (RPs) of GONPs in pristine porous media at low ionic strength were linear, which contrasted with hyper-exponential shape of RPs at high ionic strength. The size-distribution analysis of retained GONPs showed decreasing particle diameter with increasing distance from the column inlet at high ionic strength and equal diameter at low ionic strengths. The GONP retention rate was higher for natural porous media than for sand, due to the presence of metal oxides heterogeneities. The presence of biofilm on porous media increased the retention rate of GONPs when compared to the porous media in the absence of biofilm.

Predictors of Mortality after Primary Discharge from Hospital in Patients with Esophageal Atresia.

OBJECTIVE: To describe esophageal atresia mortality rates and their associations in our cohort. STUDY DESIGN: Patients with esophageal atresia, managed at The Royal Children's Hospital, Melbourne (1980-2018), who subsequently died, were retrospectively identified from the prospective Nate Myers Oesophageal Atresia database. Data collected included patient and maternal demographics, vertebral anomalies, anorectal malformations, cardiovascular anomalies, tracheoesophageal fistula, renal anomalies, and limb defects (VACTERL) associations, mortality risk factors, and preoperative, operative, and postoperative findings. Mortality before discharge was defined as death during the initial admission. RESULTS: A total of 88 of the 650 patients (13.5%) died during the study period; mortality before discharge occurred in 66 of the 88 (75.0%); mortality after discharge occurred in 22 of the 88 (25.0%). Common causes of mortality before discharge were palliation for respiratory anomalies (15/66 [22.7%]), associated syndromes (11/66 [16.7%]), and neurologic anomalies (10/66 [15.2%]). The most common syndrome leading to palliation was trisomy 18 (7/66 [10.6%]). Causes of mortality after discharge had available documentation for 17 of 22 patients (77.3%). Common causes were respiratory compromise (6/17 [35.3%]), sudden unexplained deaths (6/17 [35.3%]), and Fanconi anemia (2/17 [11.8%]). Of the patients discharged from hospital, 22 of 584 (3.8%) subsequently died. There was no statistical difference in VACTERL association between mortality before discharge (31/61 [50.8%]) and mortality after discharge (11/20 [55.0%]), nor in incidence of twins between mortality before discharge (8/56 [14.3%]) and mortality after discharge (2/18 [11.1%]). CONCLUSIONS: We identified predictors of mortality in patients with esophageal atresia in a large prospective cohort. Parents of children with esophageal atresia must be counselled appropriately as to the likelihood of death after discharge from hospital.

Polyethylene Glycol Dosing for Constipation in Children Younger Than 24 Months: A Systematic Review.

OBJECTIVES: Evaluate safety and effectiveness of Polyethylene glycol (PEG) for chronic constipation in children aged younger than 24 months. Identify the optimum dose of PEG to manage chronic constipation in children aged younger than 24 months. METHODS: In this systematic review, Embase, Medline Ovid, Pubmed, and the Cochrane Library were searched between January 1, 2000 and February 1, 2019. Studies investigating functional constipation, in which patients younger than 24 months of age were treated with PEG, were considered as potentially eligible for review. Two authors screened the studies against inclusion/exclusion criteria. Study quality was assessed with the PEDro quality assessment, Cochrane risk of bias tool, and/or the Newcastle-Ottawa Scale. RESULTS: Five studies (2 randomized controlled trials, 3 retrospective chart reviews) satisfied selection criteria (n = 459). All studies employed different dosage categories: mean effective maintenance dose, mean initial dose, mean short-term and long-term dose, and mean daily dose. Dosage regimens were variable, with 0.45 to 1.1 g ·â€Škg ·â€Šday for PEG3350 and 0.48 to 0.65 g ·â€Škg ·â€Šday for PEG4000. Adverse effects were transient across all studies for all types of PEG; these included diarrhea and abdominal pain. CONCLUSIONS: This systematic review provided evidence for a lack of reported side effects from PEG for children aged younger than 24 months. Evidence to establish appropriate dosage regimens does not exist.An infographic accompanying this article can be found at http://links.lww.com/MPG/B839.

The role of gonadotrophins in gonocyte transformation during minipuberty.

PURPOSE: Postnatal surge of gonadotrophins, Luteinizing hormone (LH) and Follicle-Stimulating hormone (FSH) known as minipuberty, is critical for gonocyte maturation into spermatogonial stem cells (SSC) in the testis. Gonadotrophins are essential for optimum fertility in men, but very little is known how they regulate germ cells during minipuberty. This study examined whether gonadotrophins play a role on gonocyte transformation in vivo. METHODS: Testes from hypogonadal (hpg) mice and their wild type (WT) littermates (n = 6/group) were weighed, and processed in paraffin at postnatal days (D) 0, 3, 6 and 9. Mouse VASA homologue (germ cell marker), anti-Müllerian hormone (Sertoli cell marker) antibodies and DAPI (nuclei marker) were used for immunofluorescence followed by confocal imaging. Germ cells on or off basement membrane (BM) and Sertoli cells/tubule were counted using Image J and analyzed with GraphPad. RESULTS: Comparing to WT littermates, there were significantly fewer germ cells on BM/tubule (p < 0.05) in D9 hpg mice, whereas there was no significant difference for germ cells off BM/tubule and Sertoli cells/tubule between littermates. However, testicular weight was significantly reduced in D3-D9 hpg mice comparing to WT littermates. CONCLUSION: Gonadotrophin deficiency reduced D9 germ cells on BM indicating impaired gonocyte transformation into SSC. This suggests that gonadotrophins may mediate gonocyte transformation during minipuberty.

Frequency of inguinal herniotomy in Australia (1998-2017).

BACKGROUND/AIM: Closure of the processus vaginalis (PV) is considered as the last step of testicular descent. Therefore, patent processus vaginalis (PV), and inguinal hernias are linked to cryptorchidism. As the National Australian incidence of orchidopexy has decreased over the previous 20 years, we aimed to explore the incidence of inguinal herniotomy (including hydrocele) over time in Australia. METHODS: The National Department of Human Services (DHS) database, and Bureau of Statistics database were obtained for the years 1998-2017. The numbers of inguinal herniotomies in patients aged 0-4, 5-14 and 15-24 yearswere examined with ethical approval. RESULTS: Over the 20-year period, over 87,000 inguinal herniotomy procedures were performed in males. The incidence per year in males decreased across all ages over the 20-year period, but was most pronounced in infants and toddlers. Similar to males, the incidence in females decreased over time, with the ratio of procedures per head of population decreasing in children under 5 years of age. The ratio of male: females varied according to ages, and was between 2.8 and 6.2 males: 1 female. CONCLUSION: This study suggests that fewer 0-4-year olds are undergoing inguinal herniotomy, compared with 20 years ago. This is likely due to a change in practice for the management of unilateral symptomatic hernias, from routine bilateral herniotomies, to unilateral surgery. As well as less aggressive surgical intervention for hydroceles in boys. LEVEL OF EVIDENCE: III.

Gonocyte transformation in congenital undescended testes: what is the role of inhibin-B in cell death?

PURPOSE: Undescended testes (UDT) are subjected to heat stress, which can disturb gonocyte transformation as well as apoptosis. This study aims to describe the apoptosis pathway occurring during minipuberty of children with unilateral (UDT), and to investigate the role of inhibin-B. METHODS: Testicular biopsies at unilateral orchidopexy of 10 boys (6-9 months old) with normal inhibin-B (n = 5) or low inhibin-B (n = 5) were selected for immunohistochemistry and TUNEL (Terminal deoxynucleotidyl transferase dUTP nick end labelling) assay. Testicular tubules were labelled with antibodies against Anti-Müllerian hormone (AMH, Sertoli cell marker), mouse Vasa Homolog (MVH) and placental alkaline phosphatase (PLAP) (both germ cell markers), cleaved caspase3 (apoptotic marker), and followed by confocal imaging and cell counting with Fiji/ImageJ. Data were analyzed with GraphPad Prism. RESULTS: In males with low and normal inhibin-B, there was no statistical difference (p > 0.05) in the percentage of testicular tubules containing TUNEL + cells, number of cleaved caspase3 ± germ cells/tubule, total number of germ cells/tubule, and the percentage of fibrotic tubules or number of Sertoli cells/tubule. CONCLUSIONS: These results suggest that inhibin-B does not regulate cell death of gonocytes and further studies are required to uncover any role of inhibin-B in gonocyte transformation.

Impact of Esophageal Atresia on the Success of Fundoplication for Gastroesophageal Reflux.

OBJECTIVES: Fundoplication is commonly performed in patients with a history of esophageal atresia (EA), however, the success of this surgery is reduced, as reflected by an increased rate of redo fundoplication. We aimed to determine whether EA impacts the prevalence of fundoplication, its timing, and performance of a redo operation. STUDY DESIGN: A single-center, retrospective review of all patients undergoing fundoplication over a 20-year period (1994-2013) was performed. Redo fundoplication was used as a surrogate for surgical failure. RESULTS: A total of 767 patients (patients with EA 85, those who did not have EA 682) underwent fundoplication during the study period. Median age (months) at primary fundoplication was lower in patients with EA (7.2 vs those who did not have EA 23.3; P < .001). Redo fundoplication rates between groups were not significantly different (EA 11/85 vs 53/682; P = .14). Median time (months) between primary and redo fundoplication was greater in patients with EA (36.2 vs 11.7; P = .03). CONCLUSIONS: Contrary to popular belief, the incidence of redo fundoplication was not significantly increased in patients with a history of EA. However, patients with EA underwent fundoplication at younger ages, which may be related to early life-threatening events in these patients. These results inform perioperative counseling, and highlight the importance of sustained surgical follow-up in patients with EA.

Presence of viable germ cells in testicular regression syndrome remnants: Is routine excision indicated? A systematic review.

There is no consensus in the literature about the necessity for excision of testicular remnants in the context of surgery for an impalpable testis and testicular regression syndrome (TRS). The incidence of germ cells (GCs) within these nubbins varies between 0 and 16% in previously published series. There is a hypothetical potential future malignancy risk, although there has been only one previously described isolated report of intratubular germ-cell neoplasia. Our aim was to ascertain an accurate incidence of GCs and seminiferous tubules (SNTs) within excised nubbins and hence guide evidence-based practice. The systematic review protocol was designed according to the PRISMA guidelines, and subsequently published by the PROSPERO database after review (CRD42013006034). The primary outcome measure was the incidence of GCs and the secondary outcome was the incidence of SNTs. The comprehensive systematic review included articles published between 1980 and 2016 in all the relevant databases using specific search parameters and terms. Strict inclusion and exclusion criteria were ultilised to identify articles relevant to the review questions. Twenty-nine paediatric studies with a total of 1455 specimens were included in the systematic review. The mean age of the patients undergoing nubbin resection was 33 months and the TRS specimen was more commonly excised from the left (68%). The incidence of SNTs was 10.7% (156/1455) and the incidence of GCs, 5.3% (77/1455). Histological analysis excluding the presence of either SNTs or GCs was consistent with TRS, fibrosis, calcification or haemosiderin deposits. There is limited evidence on subset analysis that GCs and SNTs may persist with increasing patient age. This systematic review has identified that 1 in 20 of resected testicular remnants has viable GCs and 1 in 10 has SNTs present. There is insufficiently strong evidence for the persistence of GCs and SNTs with time or future malignant potential. Intra-abdominal TRS specimens may contain more elements and, therefore, require excision, although this is based on limited evidence. However, there is no available strong evidence to determine that a TRS specimen requires routine excision in an inguinal or scrotal position.

Home-Based Transabdominal Interferential Electrical Stimulation for Six Months Improves Paediatric Slow Transit Constipation (STC).

BACKGROUND: Transcutaneous electrical stimulation (TES) for one to two months has produced some improvement in treatment-resistant slow-transit constipation (STC) in children. Optimal parameters for treatment are not known. It is possible that more improvement would occur with stimulation for longer. This study examined the effectiveness of stimulation for six months. METHODS: Children with STC confirmed by nuclear transit study (NTS) were enrolled prospectively. All had chronic constipation for greater than two years and had failed medical treatment. TES was performed for one hour/day for six months using the INF 4160 (Fuji Dynamics) portable stimulator and 4 cm × 4 cm electrodes near the belly button and on the back. Families kept bowel diaries and completed PEDSQLCore QOL (4.0) questionnaires before and at end of treatment. RESULTS: Sixty-two children (34 females; seven years, 2-16 year) with STC were studied. Defecation frequency increased in 57/62 (91%, mean ± SEM pre- 1.49 ± 0.20 vs. post- 3.25 ± 0.25 defecation/week, p < 0.0001) with the number with ≥3BA increasing from 6 to 37 (10-59%). Soiling frequency decreased from 4.8 to 1.1 days/week (p <0.001). Abdominal pain decreased from 1.7 to 0.3 days/week (<0.0001), and spontaneous urge to defecate improved. Quality of life (p < 0.01), mean transit index and gastric emptying on NTS improved (p < 0.005). CONCLUSION: Treatment-resistant STC responds to TES using interferential current across the abdomen when given daily for many months. Battery operated stimulators allowed stimulation at home for an hour each day. Stimulation for six months produced clinically significant improvement in defecation frequency, soiling, abdominal pain, urge to defecate, and quality of life in half of these chronic patients.

Short-Term Interferential Transabdominal Electrical Stimulation Did Not Change Oral-Rectal Transit Time in Piglets.

BACKGROUND: Transcutaneous electrical stimulation (TES) using interferential current (IFC) is a new therapeutic treatment for constipation. Clinical studies show that TES-IFC for 3-6 months improves colonic transit, but it is not clear if short-term stimulation affects transit or the effect requires longer to develop. The aim of this study was to determine if TES-IFC for only four days affects oral-rectal transit time in healthy pigs. METHODS: Twenty-two 4-5-week old large white female piglets had transit studies during week 4 and week 5 by placing a capsule containing 18 radiopaque plastic markers in the esophagus under anesthetic followed by x-rays at 6, 30, 54, and 78 hours. Animals were randomly assigned to active or control groups. The active group received TES for 30 min daily for four days. Interferential current was applied through four electrodes (4 × 4 cm), with two para-spinal just below the last rib and two on the belly at the same level. Stimulation was at 4000 Hz and 4080-4160 Hz with currents crossing through the abdominal cavity. RESULTS: Whole bowel transit times ranged from 7.7 to 72.2 hours, stomach transit from <1 to 63 hours, and bowel with rectum transit time from 5 to 53 hours. Transit times were the same for the control (median 28.4 hours) and TES-IFC (23.0 hours) groups in the prestimulation and stimulation weeks (control 23.0, TES-IFC 19.8 hours) with no change within or between groups. CONCLUSION: Four days of half-hour TES-IFC daily in healthy 5-week-old piglets did not change oral-rectal transit time.

Mouse minipuberty coincides with gonocyte transformation into spermatogonial stem cells: a model for human minipuberty.

As the transient postnatal hormone surge in humans, known as 'minipuberty', occurs simultaneously with key steps in germ-cell development, we investigated whether similar changes occur in the hypothalamic-pituitary-testicular axis of neonatal mice at a time that would coincide with gonocyte transformation into spermatogonial stem cells (SSC). Serum and testes were collected from C57Bl/6 mice at embryonic Day 17 (E17), birth (postnatal Day 0; P0) and daily until P10. Serum FSH and testosterone levels in both serum and testes were analysed and gene expression of FSH receptor (Fshr), luteinising hormone receptor (Lhr), anti-Müllerian hormone (Amh), octamer-binding transcription factor 4 (Oct-4), membrane type 1 metalloprotease (Mt1-mmp), proto-oncogene C-kit and promyelocytic leukaemia zinc finger (Plzf ) was quantified by real-time polymerase chain reaction. We found a transient surge of serum and testicular testosterone levels between P1 and P3 and a gradual increase in FSH from P1 to P10. Testis Lhr expression remained low from P0 until P10 but Fshr expression peaked between P3 and P6 (P<0.01). The same was found for Oct-4 expression (a gonocyte marker), which surged between P3 and P6 (P<0.01). Mt1-mmp expression peaked at P3 (P<0.05). The expression pattern of both C-kit and Plzf (SSC markers) was similar with a steady increase from P1 to P10. These results show a transient activation of the hypothalamic-pituitary-testicular axis postnatally with increases in serum and testicular testosterone at P1-P3 and testicular Fshr (but not Lhr) at P3-P6. These changes coincide with increases in gene expression of Oct4, Mt1-mmp, Plzf and C-kit, reflecting gonocyte activation, migration and transformation into SSC. In conclusion, these findings suggest that 'minipuberty' does occur in mice and that gonocyte transformation may be driven by a transient FSH signalling pathway.

Undescended testis: What paediatricians need to know.

Undescended testis (UDT) occurs when something goes wrong with testicular descent from high in the abdominal cavity to the scrotum. Normal descent occurs in two steps, with the transabdominal phase controlled by a new testicular hormone, insulin-like hormone 3, and the inguinoscrotal phase controlled by androgens. The latter phase requires a complex process of migration from the inguinal abdominal wall to the scrotum and is commonly defective, leading to the high incidence (2-4%) of UDT at birth. The clinical examination of babies and infants aims to confirm the persistence of congenital UDT by 3-6 months, so surgery can be optimally timed at 6-12 months. For those boys who develop acquired UDT later in childhood, the 'ascending' testis often needs surgery between 5 years and 10 years, so all boys should be screened again for UDT at school entry.

Retrograde continence enema in children with spina bifida: Not as effective as first thought.

AIM: The aim of the study is to investigate the effectiveness of Peristeen retrograde continence enema (RCE) in the management of faecal incontinence in children with spina bifida. METHODS: We identified a homogenous group of spina bifida patients in whom RCE was initiated (Jan 2006-July 2013). Confidential assessments included (i) Fecal Incontinence Quality Of Life (FIQOL), (ii) St Marks Faecal Incontinence score, (iii) Cleveland Clinic Constipation score and (iv) Neurogenic Bowel Dysfunction score. RESULTS: Of 20 patients, 11 (mean age 14.5 ± 5.3 years) were male. Of 20 patients, nine were still using RCE (mean follow-up 4.1 years). Three patients ceased RCE within 10 days, six after 4-12 months and two after 36-48 months. Reasons for cessation included balloon difficulties (n = 4), procedure deemed too difficult (n = 4) and pain (n = 3). There were no differences between the groups in length of training time for technique, instillate fluid/volume used and time taken to perform RCE. There were no differences between the groups for quality of life, faecal incontinence or constipation scores. CONCLUSIONS: We demonstrated a high rate of cessation with RCE in patients with spina bifida. This could not be explained by associated conditions, or by enema-related parameters. One possible explanation is the lack of ongoing outpatient support for the children and their families.

Disorders of sex development (DSD): not only babies with ambiguous genitalia. A practical guide for surgeons.

INTRODUCTION AND METHODS: In this review, we describe the common clinical scenarios that may be present to a paediatric surgeon when a patient has a disorder of sex development (DSD). Our aim was to prepare surgeons so that they can respond with correct approaches to diagnose and manage the given situations. RESULTS: DSD present in three distinct clinical situations: in the neonate with some abnormality of the external genitalia; in the child undergoing surgical treatment for inguinal hernia or during open or laparoscopic orchidopexy or during hypospadias correction; and at or after puberty, which may be precocious or delayed or in an adolescent girl with masculinisation at puberty. We describe the clinical features, likely diagnoses and the recommended management pathway in these scenarios.

Foreskin reconstruction vs circumcision in distal hypospadias.

PURPOSE: The purpose of the study was to determine if there were differences in the complication rates between foreskin reconstruction (FR) and circumcision (CIRC) in distal hypospadias repairs. The primary outcomes were urethrocutaneous fistula (UF) and dehiscence. METHODS: The data of distal hypospadias operated between 2005 and 2013 were retrospectively reviewed. The inclusion criteria were any distal hypospadias repair that required an urethroplasty. The exclusion criteria were follow-up <1 year, redo procedures, chordee greater than 20°, and incomplete data. Univariate and multivariate analysis was performed on the results. RESULTS: 213 patients were included (95 FR and 118 CIRC). The 2 groups were comparable for age at surgery 19.32 months in FR and 14.25 months in CIRC. Mathieu repair was more common in FR (47/95-49.47%) than in CIRC (45/118-38.14%). The total subsequent procedures required were 23 in FR and 57 in CIRC. The incidence of UF was 6.3% (6/95) in FR and 27.1% (32/118) in CIRC (p < 0.001, OR 5.52, 95% CI 2.2-13.9). Complete dehiscence rates were 3.16% (3/95) FR vs 11.02% (13/118) for CIRC (p = 0.037, OR 3.8, 95% CI 1.05-13.74). The incidence of patients requiring reoperation was 18.9% (18/95) in FR versus 45.8% (54/118) in CIRC (p < 0.001, OR 3.61, 95% CI 1.93-6.76). CONCLUSIONS: Foreskin Reconstruction conferred a significantly lower rate of complications, particularly the UF rate, dehiscence rate, and number of patients that required reoperation. Our rate of complications in the CIRC group is much higher than other published data.

Drainage and Nitrate Leaching from Artificially Drained Maize Fields Simulated by the Precision Nitrogen Management Model.

Environmental nitrogen (N) losses (e.g., nitrate leaching, denitrification, and ammonia volatilization) frequently occur in maize ( L.) agroecosystems. Decision support systems, designed to optimize the application of N fertilizer in these systems, have been developed using physically based models such as the Precision Nitrogen Management (PNM) model of soil and crop processes, which is an integral component of Adapt-N, a decision support tool providing N fertilizer recommendations for maize production. Such models can also be used to estimate N losses associated with particular management practices and over a range of current climates and future climate projections. The objectives of this study were to update the PNM model to include an option for simulating soil-water processes in artificially drained soils, and to calibrate the revised PNM model and test it against multiyear field studies in New York and Minnesota with different soils and management practices. Minimal calibration was required for the model. Denitrification rate constants were calibrated by minimizing the error between simulated and observed nitrate leaching for each study site. The normalized root mean squared error of cumulative daily drainage for the validation sets ranged from 10 to 23%. For cumulative daily nitrate leaching, the normalized root mean squared error ranged from 11 to 28% for the validation sets. The minimal calibration required and relatively simple data inputs make the PNM model a broadly applicable tool for simulating water and N flows in maize systems.