Manometric findings in relation to functional outcomes in different types of anorectal malformations

Aims: To compare anorectal manometry (AM) in patients with different types of anorectal malformations (ARMs) in relation to functional outcomes. Methods: A single-institution, cross-sectional study. After ethical approval, all patients >= 7 years old treated for anterior anus (AA), perineal fistula (PF), vestibular fistula (VF), or rectourethral fistula (RUF) from 1983 onwards were invited to answer the Rintala bowel function score (BFS) questionnaire and to attend anorectal manometry (AM). Patients with mild ARMs (AA females and PF males) had been treated with minimally invasive perineal procedures. Females with VF/PF and males with RUF had undergone internal-sphincter saving sagittal repairs. Results: 55 of 132 respondents (42%; median age 12 (7-29) years; 42% male) underwent AM. Patients with mild ARMs displayed good anorectal function after minimally invasive treatments. The median anal resting and squeeze pressures among patients with mild ARMs(60 cm H2O and 116 cm H2O respectively) were significantly higher than among patients with more severe ARMs (50 cm H2O, and 80 cm H2O respectively; p Conclusions: Our findings support the appropriateness of our minimally invasive approaches to the management of mild ARMs, and IAS-saving anatomical repairs for patients with more severe malformations. (C) 2017 Elsevier Inc. All rights reserved.Peer reviewe

Anorektumin epämuodostumien hoito ja myöhäisongelmat

Vertaisarvioitu. English summaryAnorektumin epämuodostumat ovat tavallisimpia suoliston synnynnäisiä epämuodostumia, jotka vaihtelevat peräaukon lievästä virhesijainnista vaikeisiin monianomalioihin. Niiden yleisyys Suomessa on 1/2 500 elävänä syntynyttä, ja 65 %:lla potilaista todetaan liitännäisanomalioita. Lievät anorektumin kehityshäiriöt hoidetaan ensisijaisesti laajentamalla tai yksinkertaisella anoplastialla, mutta vaikeimmissa epämuodostumissa potilaille joudutaan tekemään monimutkaisia rekonstruktioita avannesuojassa. Lievissä tapauksissa suolen toiminnallinen ennuste on erinomainen. Vaikeammissa tapauksissa merkittävälle osalle potilaista jää suolen pysyvä toimintahäiriö, johon voi liittyä virtsateiden ja sukuelinten toiminnallisia ongelmia sekä heikentynyt elämänlaatu. Potilaiden onnistunut hoito edellyttää asiantuntevaa seurantaa ja tukitoimia läpi koko lapsuusiän, ja niiden tulisi jatkua aikuislääketieteen piiriin siirtymisen jälkeenkin.Peer reviewe

Combined management of perianal rhabdomyosarcoma with chemotherapy, radical surgery, and irradiation : A series of three consecutive children

Background and aim: We describe a series of three successive patients with perianal rhabdomyosarcoma (PRMS) from 2014 to 2017 managed with combined chemotherapy, radical surgery, and radiotherapy. Methods: Ethical consent was obtained. Data including tumor presentation, treatment, and survival was collected from hospital reports. Results: Two girls aged 15 and 16 years (patient #1 and #2) and one boy aged five years (patient #3) were referred because of a suspected perianal abscess. MRI showed large perianal tumors from 7 to 12 cm in diameter that surrounded or infiltrated the anal sphincters and were inconsistent with abscess. Tumor biopsies showed RMS of alveolar (#1 and #2) and embryonal (#3) types. Patient #1 had lymph node and bone metastases, patient #2 lymph node metastases, and patient # 3 no metastases. Pretreatment staging, IRS Clinical Group, and Risk Groups were: Stage 4, II, high; Stage 3, GII, intermediate; and Stage 3, I, low, respectively. All underwent colostomy before neoadjuvant chemotherapy (CWS-RMS 2009 program). Neoadjuvant chemotherapy failed to clear the tumors from anal sphincters preventing anus-saving surgery, and all patients underwent abdominoperineal excision. All removed specimens had freemargins with negative lymph nodes. After adjuvant chemotherapy and local radiation, the patients were tumor free after 48, 13, and 18 months. Conclusion: In PRMS local surgical control required abdominoperineal excision. Confusion between PRMS and abscess may cause unnecessary delay in management. (C) 2018 Elsevier Inc. All rights reserved.Peer reviewe

Outcomes of fundoplication in oesophageal atresia associated gastrooesophageal reflux disease

Aim of the study: Conservative management of gastrooesophageal reflux (GORD) in oesophageal atresia (OA) is sometimes inefficient, and fundoplication is required. We assessed the outcomes of fundoplication among OA patients from 1980 to 2016. Methods: After ethical consent, hospital records of 290 patients, including 22 referred patients, were reviewed. Included were 262 patients with end-to-end repair. Excluded were patients who underwent oesophageal reconstruction (n = 23) or no repair (n = 5). Primary outcome measures included survival, retaining the native oesophagus, resolution of GGORD symptoms, failure of fundoplication, and long-term endoscopic results. Main results: Gross types of OA in 262 patients were A (n = 12), B (n = 2), C (n = 217), D (n = 10), E (n = 19), and F (n = 2). Eighty-six (33%) patients, type A (n = 12, 100%), B (n = 2, 100%), C (n = 69, 31%), D (n = 3, 30%), and F (n = 1, 50%), underwent fundoplication at the median age of 5.4 (IQR 3.1-16) months. Main indications included recalcitrant anastomotic stenosis (RAS) in 41 (48%), respiratory symptoms in 16 (19%), and acute life threatening events (ALTE) in 15 (17%) of patients. Associated tracheomalacia in 25 (29%) patients were treated with aortopexy. Median follow-up was 7.5 (IQR 1.8-15) years. RAS resolved in 30 (73%) patients, whereas 11 (27%) with unresolved RAS underwent oesophageal resection (n = 8) or replacement (n = 3). Six (7%) patients died of heart failure (n = 4), bolus impaction (n = 1), and ALTE (n = 1). Fundoplication failed in 27 (31%) patients, and 13 (15%) underwent redo fundoplication. Fundoplication failure was predicted by long-gap OA RR = 3.8 (95% CI = 1.1-13), P = 0.04. In total GORD associated symptoms persisted in 7 (8%) patients, including one with permanent feeding jejunostomy. Latest endoscopy showed moderate or severe oesophagitis in 7% of fundoplicated and in 3% nonfundoplicated patients and intestinal metaplasia in 3% and 1% (p = 0.20-0.29). Conclusion: Fundoplication provided a safe and relatively effective control of OA associated symptomatic GORD and oesophagitis. The failure rate of fundoplication was high in those with long-gap OA. Type of study: Treatment study. Level of evidence: IV (C) 2017 Elsevier Inc. All rights reserved.Peer reviewe

Sexual Function, Fertility and Quality of Life after Modern Treatment of Anorectal Malformations

Purpose: Sexual dysfunction and impaired quality of life due to fecal incontinence are common after classic operations for anorectal malformations. We hypothesized that modern repairs may result in improved outcomes. Materials and Methods: Following ethical approval for this single institution cross-sectional study, all patients 16 years or older treated for rectourethral, vestibular or perineal fistula from 1983 onward were sent detailed postal questionnaires on sexual function and quality of life. Each respondent was age and gender matched to 3 controls randomly selected from the general population. Penoscrotal/gynecologic abnormalities were obtained from the records. Results: A total of 41 patients (62%) with a median age of 22 years participated in the study. Of the patients 20 were males with rectourethral fistula (prostatic in 60%), 10 were females with vestibular/perineal fistula and 11 were males with low malformations. Although experience of sexual relationships and orgasmic function were reported in comparable proportions to controls, age at coital debut was significantly delayed in all groups of patients (p Conclusions: While erectile and orgasmic function appear preserved after sagittal repair, further evaluation of fertility issues in males with rectourethral fistula is indicated. Larger multicenter studies are needed to confirm our findings.Peer reviewe

Lower urinary tract symptoms and sexual functions after endorectal pull-through for Hirschsprung disease : controlled long-term outcomes

Background/purpose: To define the prevalence of lower urinary tract symptoms (LUTS) and outcomes for sexual function after endorectal pull-through (EPT) for Hirschsprung disease (HD) compared to controls. To date, similar controlled studies are lacking. Methods: Patients aged = 4 years (n= 123) operated on forHDat our center between 1987 and 2011were invited to answer questionnaires on LUTS and sexual function (aged = 16 years). Patients with an intellectual disability and patients with a definitive endostomy were excluded. Patients were matched to three controls and also invited to a clinical follow-up for urological investigations including urine flow measurement, renal tract ultrasound, and urinalysis. Results: Altogether, 59 responses concerning LUTS and 24 responses concerning sexual functions were analyzed. No significant differences were demonstrated in the overall prevalence of LUTS between patients (67%) and controls (80%), nor in the prevalence of frequent LUTS (14% vs. 16%; P = NS for both). One patient (2%) had a urethral stricture after laparotomy-assisted EPT. Male patients reported sexual satisfaction and erectile function similar to controls (P N 0.10). Female patients were currently less in stable relationships compared to controls (25% vs. 83%, P= 0.005). Conclusions: Our results support the safety of EPT in patientswith HDwith regard to preservation of the integrity and functioning of the genitourinary tract. (C) 2017 Elsevier Inc. All rights reserved.Peer reviewe

Fecal calprotectin in the prediction of postoperative recurrence of Crohn's disease in children and adolescents

Background: Fecal calprotectin (FC) correlates with endoscopic recurrence of Crohn's disease (CD) in adults but has not been studied among children postoperatively. We aimed to analyze whether FC relates with postoperative CD recurrence in children. Methods: Altogether 51 postoperative endoscopies and FC measurements from 22 patients having undergone surgery for CD at age Results: Ileocecal resection (n = 15), small bowel resection (n = 6), or left hemicolectomy (n = 1) was performed at median age of 15.1 (interquartile range 14.4-17.6) years. Following surgery, FC decreased significantly (659 vs. 103 mu g/g, p = 0.001). During median follow-up of 5.7 (4.2-7.7) years, either endoscopic or histological recurrence occurred in 17 patients (77%). FC > 139 mu g/g at time of endoscopy or FC increase of 79 mu g/g compared to first postoperative value was suggestive of endoscopic recurrence (Rutgeerts score i2-i4), while FC > 101 mu g/g or increase of 21 mu g/g indicated histological recurrence. Best accuracy for prediction of recurrence was obtained by combining FC at endoscopy and the postoperative increase of FC. The corresponding AUROC values were 0.74 (95% 0.58-0.89) for endoscopic recurrence whereas 0.81 (95% CI 0.67-0.95) for histological recurrence. Conclusion: FC is a useful surrogate marker of postoperative recurrence also in pediatric CD patients. (C) 2016 Elsevier Inc. All rights reserved.Peer reviewe

GUNS UNDER FIRE: Initial results of the 2005 firearms amnesty

On 1 January 2005 government launched the largest ever firearms amnesty. By 31 March 50,233 firearms had been surrendered. Due to public demand, the amnesty was extended to 30 June. This article assesses the impact of the first three months of the amnesty. Although media coverage focused on the illegal weapons handed in, the nearly 28,000 licensed guns surrendered represent just over one year’s supply of lost guns that will now not enter the illegal market. And considering the widespread use of handguns in violent crime, the removal of over 43,000 handguns from circulation represents a substantial victory

No Tissue Expression of KRAS or BRAF Mutations in 61 Adult Patients Treated for Esophageal Atresia in Early Childhood

Background Previous studies have reported an association among esophageal atresia (EA), Barrett's esophagus, and esophageal adenocarcinoma later in life. Objective The objective of the article is to evaluate KRAS and BRAF mutations as potential genetic markers for early detection of malignant transformation, we used an ultrasensitive technique to detect tissue expression of KRAS and BRAF mutations in endoscopic biopsies from 61 adult patients under follow-up after treatment for EA. Materials and Methods RNA was extracted from 112 fresh-frozen endoscopic tissue biopsies from 61 adult patients treated for EA in early childhood. RNA was reverse transcribed using the extendable blocking probe reverse transcription method. KRAS codons 12 and 13, as well as BRAF mutations were detected by quantitative polymerase chain reaction. Results No mutations of KRAS codon 12, KRAS codon 13, or BRAF were found in 112 endoscopic biopsy samples from 61 patients. Conclusion Despite the presence of histological findings indicating long-standing gastroesophageal reflux in 25%, as well as symptomatic gastroesophageal reflux in more than 40%, there was no detectable tissue expression of KRAS or BRAF mutations in this cohort of patients.Peer reviewe

Ex-situ biological hydrogen methanation in trickle bed reactors : Integration into biogas production facilities

Biological hydrogen methanation (BHM) is a biocatalytic process for biogas upgrading. Integrating ex-situ BHM processes into biogas facilities has the advantage of using inoculum, CO2, and nutrients directly from anaerobic digestion (AD) processes to enhance CH4 productivity. This study investigated the potential of biowaste digestate as an inoculum and digested biowaste reject water as the trickling liquid and nutrient source in thermophilic trickle bed reactors (TBRs). Use of reject water improved H2 conversion efficiency to up to 99 %, thus achieving a H2 loading rate of 10.8 LH2 L Rv-1 d-1 at a gas retention time (GRT) of 1.8 h and CH4 productivity of 2.6 L LRv-1 d-1 implying that reject water contains macronutrients beneficial to enriching hydrogenotrophic methanogens. However, at high H2 loading rates, a trace element supply was necessary to stabilize process performance. Hydrogenotrophic methanogens Methanothermobacter and Methanobacterium were selectively enriched, mainly due to the increased H2 loading rate.publishedVersionPeer reviewe