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1.
Alejandra Vilanova-Sanchez Carlos A. Reck Kate A. McCracken Victoria A. Lane Alessandra C. Gasior Richard J. Wood Marc A. Levitt Geri D. Hewitt 《Journal of pediatric surgery》2018,53(4):698-703
Background/aim
Patients may present with gynecologic concerns after previous posterior sagittal anorectoplasty (PSARP) for repair of an anorectal malformation (ARM). Common findings include an inadequate or shortened perineal body, as well as introital stenosis, retained vaginal septum, and remnant rectovestibular fistula. An inadequate or shortened perineal body may impact fecal continence, sexual function and recommendations regarding obstetrical mode of delivery. We describe our experience with female patients referred to our center for evaluation of their previously repaired ARM, with a specific focus on perineal body anatomy and concomitant gynecologic abnormalities. We outline our collaborative evaluation process and findings as well as subsequent repair and outcomes.Material/methods
A single site retrospective chart review from May 2014 to May 2016 was performed. Female patients with a history of prior ARM repair who required subsequent reoperative surgical repair with perineoplasty were included. The decision for reoperation was made collaboratively after a multidisciplinary evaluation by colorectal surgery, urology, and gynecology which included examination under anesthesia (EUA) with cystoscopy, vaginoscopy, rectal examination, and electrical stimulation of anal sphincters. The type of original malformation, indication for reoperative perineoplasty, findings leading to additional procedures performed at time of perineoplasty, postoperative complications, and the length of follow up were recorded.Results
During the study period 28 patients were referred for evaluation after primary ARM repair elsewhere and 15 patients (60%) met inclusion criteria. Thirteen patients (86.6%) originally had a rectovestibular fistula with prior PSARP and 2 patients (13.4%) originally had a cloacal malformation with prior posterior sagittal anorectovaginourethroplasty. The mean age at the time of the subsequent perineoplasty was 4.6 years (0.5–12). Patients had an inadequate perineal body requiring reoperative perineoplasty due to: anterior mislocation of the anus (n = 11, 73.3%), prior perineal wound dehiscence with perineal body breakdown (n = 2, 13.4%), acquired rectovaginal fistula (n = 1, 6.6%), and posterior mislocated introitus with invasion of the perineal body (n = 1, 6.6%). During the preoperative evaluation, additional gynecologic abnormalities were identified that required concomitant surgical intervention including: introital stenosis (n = 4, 26.6%), retained vaginal septum (n = 3, 20%) and remnant recto vestibular fistula (n = 2, 13.3%).Conclusions
Patients with a previously repaired ARM may present with gynecologic concerns that require subsequent surgical intervention. The most common finding was an inadequate perineal body, but other findings included introital stenosis, retained vaginal septum and remnant recto vestibular fistula. Multidisciplinary evaluation to assess and identify abnormalities and coordinate timing and surgical approach is crucial to assure optimal patient outcomes.Type of study
Case series with no comparison group.Level of evidence
IV. 相似文献2.
Objectives
To estimate the prevalence of urinary incontinence and anal incontinence in Tunisian women and to identify their risk factors.Subjects and methods
A cross-sectional study was conducted among 402 female doctors and nurses randomly selected from 3 large hospitals in the center of Tunisia. The prevalence of urinary incontinence and anal incontinence were measured using validated questionnaires.Results
Overall 45.3% of women experienced incontinence (urinary incontinence or anal incontinence). The overall prevalence of urinary incontinence, anal incontinence and double incontinence were 45%, 6.3% and 6%, respectively. Factors associated with incontinence were postpartum urinary incontinence (OR 11.91, CI 4:72–30:04, P < 0.001), menopausal status (OR 11.72, CI 3:8–36:07, P < 0.001), arterial hypertension (OR 4.17, CI 1:61–10.81, P = 0.003), nurse occupation (OR 3.22, CI 1:62–6:36, P = 0.001) and constipation (OR 1.71, CI 1:02–2:87, P = 0.041). Medical help seeking was taken only by 21% of the incontinent women.Conclusion
Forty five percent of Tunisian women suffered from urinary or anal incontinence. A primary prevention for modifiable risk factors, such as postpartum pelvic floor physiotherapy and hypertension control, should be advised to women in order to optimize their quality of life. 相似文献3.
Shireen Anne Nah Caroline C.P. Ong Seyed Ehsan Saffari Lin Yin Ong Te-Lu Yap Yee Low Anette S. Jacobsen 《Journal of pediatric surgery》2018,53(8):1550-1554
Background
Patients with anorectal malformation (ARM) and Hirschsprung’s disease (HD) face long-term disturbance in bowel function even after definitive surgery. This study evaluates the quality of life (QOL) of patients with ARM and HD, and compares them to healthy controls using self-report questionnaires.Methodology
A prospective study was performed recruiting patients with ARM or HD from September 2013 to December 2014 who had primary surgery done in our institution at least 2 years prior to participation. Age-matched and gender-matched controls were enrolled from our patients with minor outpatient complaints. All participants completed the following PedsQL? scales (maximum score 100): 4.0 Generic Core Scales, 3.0 General Well-Being (GWB) Scale and 2.0 Family Impact (FI) Module. All were also scored on bowel function (BFS), with a maximum score 20. Appropriate statistical analysis was performed, with significance level < 0.05.Results
There were 193 participants: 87 controls, 62 ARM, 44 HD. When comparing Core, GWB and FI scores, there were no significant differences between groups although controls had best scores indicating best QOL and general wellbeing, with least impact of the child’s health on the family. BFS was significantly different with controls having best and ARM worst scores. There were no significant differences in scores between parent and child indicating intradyad consistency. There was significant positive correlation between BFS and Core (p < 0.0001), and between BFS and GWB scores (p < 0.005); and significant negative correlation between BFS and FI scores (p < 0.0001).Conclusions
Bowel function impacts quality of life. Those with ARM and HD can achieve good quality of life comparable to controls, based on patient and caregiver self-reported outcomes.Type of study
Prospective comparative studyLevel of evidence
Level II. 相似文献4.
Katerina Dukleska Loren Berman Allison A. Aka Charles D. Vinocur Erin A. Teeple 《Journal of pediatric surgery》2018,53(6):1154-1159
Introduction
Patients with familial adenomatous polyposis (FAP) and ulcerative colitis (UC) commonly undergo restorative proctocolectomy with ileal-pouch anal anastomosis (RP-IPAA). We sought to describe patient characteristics and postoperative outcomes in this patient population.Methods
Using the National Surgical Quality Improvement Program-Pediatric Participant Use Files from 2012 to 2015, children who were 6–18 years old who underwent RP-IPAA for FAP or UC were identified. Postoperative morbidity, including reoperation and readmission were quantified. Associations between preoperative characteristics and postoperative outcomes were analyzed.Results
A total of 260 children met the inclusion criteria, of which 56.2% had UC. Most cases were performed laparoscopically (58.1%), and the operative time was longer with a laparoscopic versus open approach (326 [257–408] versus 281 [216–391] minutes, p = 0.02). The overall morbidity was 11.5%, and there were high reoperation and readmission rates (12.7% and 21.5%, respectively). On bivariate analysis, preoperative steroid use was associated with reoperation (22.5% versus 10.9%, p = 0.04). On multivariable regression analysis, obesity was independently associated with reoperation (odds ratio: 3.34 [95% confidence intervals: 1.08–10.38], p = 0.04).Conclusions
Children who undergo RP-IPAA have high rates of overall morbidity, reoperation, and readmission. Obesity was independently associated with reoperation. This data can be used by practitioners in the preoperative setting to better counsel families and establish expectations for the postoperative setting.Type of Study
Retrospective Comparative Study.Level of Evidence
Level III. 相似文献5.
Background
Childhood constipation is common. Previously, internal anal sphincterotomy has been used for hypertensive/non-relaxing sphincters; however, recent benefit has been shown with Botulinum Toxin (BT) injections. The aim is to investigate BT, including response duration, symptom association and effectiveness in relation to sphincter dynamics.Methods
Retrospective study of 164 children receiving sphincter BT for severe constipation unresponsive to medication management. Charts reviewed for symptoms, anorectal manometry (ARM) findings and response defined by decreased pain or increased defecation. Patients were grouped: normal sphincter pressure (≤ 50 mmHg), elevated (> 50 mmHg), normal and abnormal rectoanal inhibitory reflex (RAIR).Results
There were 142 analyzed and 124 completed ARMs; 98 (70%) had positive response with 57% lasting greater than 6 months. 36 had normal sphincter pressure with 24 (69%) responding. 88 had elevated pressure with 60 (68%) responding (p = 0.87). 90 normal RAIRs with 64 (71%) responding. 34 abnormal RAIRs with 22 (64%) responding (p = 0.41). With logistic regression, fecal incontinence prior to BT was a predictor of poor response (p = 0.02). The most common side effect was fecal incontinence typically resolving within week with equal frequency regardless of sphincter dynamics.Conclusions
BT is effective for children with chronic constipation. Patients with fecal incontinence are less likely to respond. More than half had prolonged beneficial response. Those with normal and abnormal sphincter dynamics had similar responses and without differences in side effects. Therefore, injection may be considered in patients with intractable constipation unresponsive to medication, regardless of anal sphincter dynamics.Level of Evidence
Level III (Treatment Study: Retrospective comparative study). 相似文献6.
Peter C. Minneci Rashmi S. Kabre Grace Z. Mak Devin R. Halleran Jennifer N. Cooper Amin Afrazi Casey M. Calkins Cynthia D. Downard Peter Ehrlich Jason Fraser Samir K. Gadepalli Michael A. Helmrath Jonathan E. Kohler Rachel Landisch Matthew P. Landman Constance Lee Charles M Leys Daniel L. Lodwick Katherine J. Deans 《Journal of pediatric surgery》2018,53(6):1163-1167
Background
This study evaluates screening practices and the incidence of associated anomalies in infants with anorectal malformations (ARM).Methods
We performed a multi-institutional retrospective cohort study of children born between 2007 and 2011 who underwent surgery for ARM at 10 children's hospitals. ARM type was classified based on the location of the distal rectum, and all screening studies were reviewed.Results
Among 506 patients, the most common ARM subtypes were perineal fistula (40.7%), no fistula (11.5%), and vestibular fistula (10.1%). At least 1 screening test was performed in 96.6% of patients, and 11.3% of patients underwent all. The proportion of patients with ≥ 1 abnormal finding on any screening test varied by type of ARM (p < 0.001). Screening rates varied from 15.2% for limb anomalies to 89.7% for renal anomalies. The most commonly identified anomalies by screening category were: spinal: tethered cord (20.6%); vertebral: sacral dysplasia/hemisacrum (17.8%); cardiac: patent foramen ovale (58.0%); renal: hydronephrosis (22.7%); limb: absent radius (7.9%).Conclusion
Screening practices and the incidence of associated anomalies varied by type of ARM. The rate of identifying at least one associated anomaly was high across all ARM subtypes. Screening for associated anomalies should be considered standard of care for all ARM patients.Type of study
Multi-institutional retrospective cohort study.Level of evidence
III 相似文献7.
Mirjam Stahl Simon Y. Graeber Cornelia Joachim Sandra Barth Isabell Ricklefs Gesa Diekmann Matthias V. Kopp Lutz Naehrlich Marcus A. Mall 《Journal of cystic fibrosis》2018,17(2):249-255
Background
Lung clearance index (LCI) detects early ventilation inhomogeneity and has been suggested as sensitive endpoint in multicenter intervention trials in infants and preschoolers with cystic fibrosis (CF). However, the feasibility of multicenter LCI in this age group has not been determined. We, therefore, investigated the feasibility of LCI in infants and preschoolers with and without CF in a three-center setting.Methods
Following central training, standardized SF6-MBW measurements were performed in 73 sedated children (10 controls, 49 with CF and 14 with other lung diseases), mean age 2.3 ± 1.2 years across three centers, and data were analyzed centrally.Results
Overall success rate of LCI measurements was 91.8% ranging from 78.9% to 100% across study sites. LCI was increased in patients with CF (P < 0.05) and with other lung diseases (P < 0.05) compared to controls.Conclusion
Our results support feasibility of LCI as multicenter endpoint in clinical trials in infants and preschoolers with CF. 相似文献8.
Anna Svenningsson Anna Gunnarsdottir Tomas Wester 《Journal of pediatric surgery》2018,53(11):2183-2188
Purpose
The aim of this study was to assess maternal risk factors and perinatal characteristics of patients with anorectal malformations (ARM) in Sweden.Methods
Population-based case–control study including all patients with ARM born in Sweden 1973–2014. Patients were identified from the Swedish National Patient Register, and data on possible maternal risk factors and perinatal characteristics were obtained from the Swedish Medical Birth Register. The association between study variables and ARM was analyzed using conditional logistic regression.Results
1167 patients and 5835 controls were analyzed. Patients with ARM were more often prematurely born (< 35?weeks: OR 4.81 95% CI 3.42–6.75, 35–36?weeks OR 2.96 95% CI 2.13–4.11) or small for gestational age (SGA) (OR 3.82 95% CI 2.66–5.50). Maternal BMI ≥ 30 was associated with an increased risk for ARM (OR 1.42; 95% CI 1.08–1.86). Maternal smoking ≥ 10 cigarettes daily was associated with ARM only in patients without associated malformations (OR 1.67 95% CI 1.08–2.58).Conclusions
Maternal obesity is a possible risk factor for ARM. Prematurity and SGA was more common among ARM patients. Maternal smoking may be a risk factor of ARM in patients without associated malformations.Level of evidence
III. 相似文献9.
Abdullah Sarkar Saud Al Shanafey Mohammad Mourad Anas Abudan 《Journal of pediatric surgery》2018,53(9):1734-1736
Introduction
Anorectal malformation (ARM) is a congenital defect that exists in varying presentations and no-fistula type (NFT) ARM is a rare high-type category. We aim to report our experience with management of this anomaly and its outcome, compared to the more common fistula-type (FT) ARM.Methods
A retrospective review of medical records of patients diagnosed with high ARM and 3 years and older, for the period between September 2000 and January 2015 was conducted. Demographic, anatomic, and outcome data were obtained for each group and compared. Quality of life data were collected using the Krickenbeck classification and assessed as documented at clinic visits as well as phone interviews.Results
There were 100 patients managed for ARM during that period and were 3 years or older. Sixteen of them were NFT (16%). For comparison purposes each NFT patient was matched with 3 FT patients.We have analyzed data on 44 patients with FT (44 males), and 16 with NFT (12 males and 4 females). The occurrence of Down syndrome in NFT patients was 56.2% compared to 0% in the FT patients (p = < 0.0001). Quality of Life data showed no significant differences between the two groups with p-values of 0.39, 1.0, and 1.0 for Voluntary Bowel Movement, Soiling, and Constipation respectively.Conclusion
NFT ARM represents a significant number at our population (16%). There is a strong association between NFT ARM and Down syndrome. NFT ARM has similar outcomes compared to the FT ARM.Type of study
Clinical research paper.Level of evidence
Level III. 相似文献10.
Joana Barbosa Sequeira Ana Coelho Ana Sofia Marinho Berta Bonet Fátima Carvalho João Moreira-Pinto 《Journal of pediatric surgery》2018,53(10):2003-2007
Purpose
To evaluate the effectiveness and safety of Endoscopic Pilonidal Sinus Treatment (EPSiT) in the pediatric population and compare it with excision followed by primary closure (EPC) regarding intra- and postoperative outcomes.Methods
A retrospective analysis of all patients with chronic sacrococcygeal pilonidal sinus submitted to EPSiT and EPC during a 12-month period in our institution was performed. Data concerning patients' demographics and surgical outcomes were collected and compared between the two groups.Results
We analyzed a total of 21 cases that underwent EPSiT and 63 cases of EPC, both groups with similar demographic characteristics. Operative time was similar for both groups (30 vs. 38 min; p > 0.05). No major intraoperative complications were reported. Wound infection rate was lower for EPSiT ((5.2% [n = 1] vs. 20.0% [n = 12]); p > 0.05). Healing time was similar for both groups (28 vs. 37.5 days). Recurrence occurred in 18,9% (n = 15), with 2 cases (10.5%) reported in the EPSiT group versus 13 (21.6%) in EPC. There were no differences between groups regarding postoperative complications, complete wound healing and recurrence rates or healing time (p > 0.05).Conclusions
Our results suggest that EPSiT is as viable as excision followed by primary closure in the management of sacrococcygeal pilonidal sinus in the pediatric population.Level of evidence
Therapeutic study – level III. 相似文献11.
Millie Garg Steven T. Leach Michael J. Coffey Tamarah Katz Roxanne Strachan Tamara Pang Bronwen Needham Kei Lui Fathalla Ali Andrew S. Day Laura Appleton Vesal Moeeni Adam Jaffe Chee Y. Ooi 《Journal of cystic fibrosis》2017,16(5):631-636
Background
Fecal calprotectin may be used as a non-invasive method to assess the effect of novel therapies on the gut in cystic fibrosis (CF).Method
Stools from CF patients and healthy controls (HC) (0–10 years old) were prospectively collected for evaluation of temporal trends.Results
130 CF samples (64 subjects) and 114 HC samples (101 subjects) were collected. Overall, fecal calprotectin levels were different in CF patients and HC from 0 to 10 years (P = 0.0002). Fecal calprotectin in CF was significantly lower than HC from 0 to 1 years (P = 0.03) and demonstrated an upward trajectory until 4 years. From > 4 to 10 years calprotectin was consistently higher in CF patients compared with HC (P = 0.007).Conclusions
Fecal calprotectin levels in children with CF and HC were age-dependent and had distinct trajectories. Careful interpretation of calprotectin is required if used in drug trials for CF, particularly in children less than 4 years old. 相似文献12.
Patrick A. Flume Claire E. Wainwright D. Elizabeth Tullis Sally Rodriguez Minoo Niknian Mark Higgins Jane C. Davies Jeffrey S. Wagener 《Journal of cystic fibrosis》2018,17(1):83-88
Background
Pulmonary exacerbations (PEx) are associated with acute loss of lung function that is often not recovered after treatment. We investigated lung function recovery following PEx for ivacaftor- and placebo-treated subjects.Methods
Short- and long-term pulmonary function recovery data after PEx were summarized from a placebo-controlled trial in 161 cystic fibrosis patients ≥ 12 years old with the G551D-CFTR mutation (NCT00909532). Short-term recovery was measured 2 to 8 weeks after treatment, and long-term recovery was determined at the end-of-study, both compared with baseline measured just prior to the PEx.Results
Fewer patients receiving ivacaftor experienced a PEx than patients receiving placebo (33.7% vs. 56.4%; P = 0.004) and had a lower adjusted incidence rate of PEx (0.589 vs. 1.382; P < 0.001). The proportion of PEx followed by full short-term recovery of percent predicted forced expiratory volume in 1 s was similar (ivacaftor vs. placebo, 57.1% vs. 53.7), as was the proportion of patients having long-term recovery (46.4% vs. 47.7%).Conclusions
Ivacaftor treatment reduces the frequency of PEx but does not improve on the rate of complete lung function recovery after PEx when compared with placebo. 相似文献13.
Matthew A. Tucker Breana Berry Nichole Seigler Gareth W. Davison John C. Quindry Dabney Eidson Kathleen T. McKie Ryan A. Harris 《Journal of cystic fibrosis》2018,17(2):256-263
Background
The impact of blood flow regulation and oxidative stress during exercise in cystic fibrosis (CF) has yet to be investigated.Methods
A maximal graded exercise test was conducted to determine exercise capacity (VO2 peak) and peak workload in 14 pediatric patients with mild CF (age 14 ± 3 y, FEV1 93 ± 16 % predicted) and 14 demographically-matched controls. On a separate visit, participants performed submaximal cycling up to 60% of peak workload where brachial artery blood velocity was determined using Doppler ultrasound. Retrograde and antegrade components were further analyzed as indices of blood flow regulation.Results
The cumulative AUC for retrograde velocity was lower in patients versus controls (1770 ± 554 vs. 3440 ± 522 cm, P = 0.038). In addition, an exaggerated oxidative stress response during exercise occurred in patients only (P = 0.004).Conclusion
These data suggest that patients with mild CF exhibit impaired blood flow regulation and an exaggerated oxidative stress response to submaximal exercise. 相似文献14.
Suna Sun Weihua Pan Wenjie Wu Yiming Gong Jia Shi Jun Wang 《Journal of pediatric surgery》2018,53(8):1584-1587
Objectives
The treatment of long gap esophageal atresia (LGEA) is one of the most challenging congenital malformations in neonatal surgery. A preoperative bougienage stretching technique for elongation of the two segments of esophagus is applied to achieve utilizing the native esophagus to establish esophageal continuity by open or thoracoscopic approach.Methods
From January 2015 to May 2017, 12 neonates who suffered from LGEA were admitted to our department. They were divided into 2 groups (A and B) according to their admission time. They all accepted bougienage stretching technique before esophageal anastomosis.Results
Initially the lengths of esophageal gap in 12 infants ranged from 4 to 7.5 vertebral bodies (M = 5.8 ± 1.1). The gap lengths became –1 to 2.5 vertebral bodies after bougienage stretching technique and tension-free anastomosis were performed successfully for all 12 cases: Group A (n = 5) by thoracotomy and group B (n = 7) by thoracoscopic approach. 12 cases have been followed up for 1–25 months (M = 12.4 ± 8.5) after definitive surgery.Conclusions
Bougienage stretching technique for LGEA is feasible with satisfactory clinical results. Thoracoscopic approach is a good choice for primary anastomosis in LGEA.Levels of evidence
Treatment Study Level IV 相似文献15.
Naho Fujiwara Katsumi Miyahara Nana Nakazawa-Tanaka Chihiro Akazawa Atsuyuki Yamataka 《Journal of pediatric surgery》2018,53(2):326-329
Purpose
Semaphorins are guidance cues for developing neurons, implicated in the determination of the migratory pathway of neural crest-derived neural precursors during enteric nervous system development. Recently, it has been reported that Semaphorin 3A (SEMA3A) expression is up-regulated in the aganglionic colon in Hirschsprung disease (HD) patients, suggesting that increased SEMA3A expression may be a risk factor for HD. Thus, the aim of our study was to determine the expression of SEMA3A using Sox10-Venus mice gut.Methods
We harvested the gut on postnatal day 2 (P2). SOX10-Venus +/EDNRB?/? mice were compared with SOX10-Venus +/EDNRB+/+ mice as controls. QRT-PCR was performed to determine gene expression of SEMA3A (n = 8). Fluorescent immunohistochemistry was performed to assess protein distribution.Results
On P2, gene expression levels of SEMA3A were significantly increased in the HD group compared to controls in the proximal and distal colon (p < 0.05). Laser scanning microscopy revealed SEMA3A expression was localized within the submucosa and muscle layer of the gut in both HD and controls. In HD, SEMA3A was highly expressed in the proximal and distal colon.Conclusions
In the present study, we demonstrated that SEMA3A expression is increased in the EDNRB ?/? HD model on P2, suggesting that SEMA3A may interfere with ENCC migration, resulting in an absence of enteric neurons. 相似文献16.
Toshiharu Matsuura Yusuke Yanagi Makoto Hayashida Yoshiaki Takahashi Koichiro Yoshimaru Tomoaki Taguchi 《Journal of pediatric surgery》2018,53(4):671-675
Background
No protocol has been established for the diagnosis and management of chylous ascites after liver transplantation (LT). In this study, we retrospectively reviewed our cases of posttransplant chylous ascites (PTCA) and aimed to propose a diagnostic and management protocol.Patients and methods
We retrospectively reviewed the clinical records of 96 LT recipients who underwent LT at our department. The incidence of PTCA and the associated risk factors were analyzed and our protocol for chylous ascites was evaluated.Results
PTCA occurred in 6 (6.3%) patients (mean age: 10.7 ± 11.0 years) at a mean of 10.8 ± 3.6 days after LT. The primary disease in all of PTCA cases was biliary atresia (BA). The periportal lymphadnopathy was an independent risk factor for PTCA. In all cases PTCA successfully resolved according to our protocol. Octreotide was administered in 4 of our 6 PTCA cases. The mean postoperative hospital stay was 40.2 ± 8.4 days, which was similar to that of cases without PTCA.Conclusions
The incidence of PTCA in LT patients, especially in those with BA, is relatively high. Our diagnostic criteria and our management protocol were helpful for patients with refractory ascites after LT.Type of study
Diagnostic test: Level II. Treatment study: Level III. 相似文献17.
Jeffrey S. Wagener Stefanie J. Millar Nicole Mayer-Hamblett Gregory S. Sawicki Edward F. McKone Christopher H. Goss Michael W. Konstan Wayne J. Morgan David J. Pasta Richard B. Moss 《Journal of cystic fibrosis》2018,17(4):503-510
Background
Patients with cystic fibrosis (CF) experience variable lung disease phenotypes. The R117H mutation is often associated with preserved lung function. Our objective was to compare the rate of lung function decline in patients with the R117H mutation and patients homozygous for the F508del mutation.Methods
Rate of decline in percentage-of-predicted FEV1 (ppFEV1) was analyzed using the 2006–2010 US CF Foundation Patient Registry.Results
4-year rate of decline was slower in 156 R117H patients compared with 6251 F508del patients (? 0.61 vs ? 2.03 ppFEV1/year, P < 0.001). Rates of decline in children were slower in R117H vs F508del patients (6–12-year-olds: + 0.73 vs ? 1.91 ppFEV1/year, P < 0.001 and 13–17-year-olds: ? 1.55 vs ? 2.66 ppFEV1/year, P = 0.046), whereas rates in adults were not significantly different (18–24-year-olds: ? 1.52 vs ? 2.12, P = 0.26 and ≥ 25-year-olds: ? 1.17 vs ? 1.40, P = 0.33).Conclusions
These findings are consistent with a delayed onset, but ultimately similar progression, of lung disease in R117H compared with homozygous F508del patients. 相似文献18.
William Bonadio Syeda Shahid Lior Vardi Carly Buckingham Allison Kornblatt Chloe Free Peter Homel 《Journal of pediatric surgery》2018,53(3):441-445
Importance
Appendicitis is a common, potentially serious pediatric disease. An important factor in determining management strategy [whether/when to perform appendectomy, duration of antibiotic therapy/hospitalization, etc.] and predicting outcome is distinguishing whether perforation is present.Objective
The objective was to determine efficacy of commonly assessed pre-operative variables in stratifying perforation risk in children with appendicitis.Design
A retrospective analysis of consecutive cases was performed.Setting
The setting was a large urban hospital pediatric emergency department.Participants
Four hundred forty-eight consecutive cases of CT [computerized tomography]-confirmed pediatric appendicitis during a 6-year period in an urban pediatric ED [emergency department]: 162 with perforation and 286 non-perforated.Main outcome(s) and measure(s)
To determine efficacy of clinical and laboratory variables with distinguishing perforation outcome in children with appendicitis.Results
Regression analysis identified 3 independently significant variables associated with perforation outcome – and determined their ideal threshold values: duration of symptoms > 1 day; ED-measured fever [body temperature > 38.0 °C]; CBC WBC absolute neutrophil count > 13,000/mm3. The resulting multivariate ROC [receiver operating characteristic] curve after applying these threshold values gave an AUC [area under curve] of 89% for perforation outcome [p < 0.001]. Risk for perforation was additive with each additional predictive variable exceeding its threshold value, linearly increasing from 7% with no variable present to 85% when all 3 variables are present.Conclusions
A pre-operative scoring system comprised of 3 commonly assessed clinical/laboratory variables is useful in stratifying perforation risk in children with appendicitis.Physicians can utilize these factors to gauge pre-operative risk for perforation in children with appendicitis, which can potentially aid in planning subsequent management strategy.Level of evidence
III. 相似文献19.
Cristen N. Litz Jessica B. Asuncion Paul D. Danielson Nicole M. Chandler 《Journal of pediatric surgery》2018,53(3):449-451
Purpose
Antibiotic administration within one hour prior to incision is a common quality metric; however, antibiotics are typically started at the time of diagnosis in pediatric patients with acute appendicitis. The purpose was to determine if antibiotic administration within one hour prior to incision reduces the incidence of surgical site infections (SSI) in pediatric patients with acute appendicitis started on parenteral antibiotics upon diagnosis.Methods
A retrospective review was performed of 478 patients aged 0–18 years who underwent appendectomy for acute appendicitis from 7/2013 to 4/2015. Patients were categorized based on timing of antibiotic administration; there were 198 patients in Group A (< 60 min before) and 280 in Group B (> 60 min before).Results
Demographics and operative time (A: 30.5 ± 9.9 vs B: 30.8 ± 12.2 min, p = 0.51) were similar. Procedures were performed laparoscopically and the groups had similar proportions of single-incision operations (A: 53% vs B: 55%, p = 0.64). There was no difference in the incidence of superficial SSI (A: 2.0% vs B: 2.1%, p = 1.0) or intraabdominal abscess (A: 4.0% vs B: 3.6%, p = 0.81) and this remained true when stratified by intraoperative classification.Conclusion
Antibiotic administration within one hour of appendectomy in pediatric patients with acute appendicitis who receive antibiotics at diagnosis did not change the incidence of postoperative infectious complications.Type of study
Treatment study.Level of evidence
III. 相似文献20.
C. Lehoux Dubois V. Boudreau F. Tremblay A. Lavoie Y. Berthiaume R. Rabasa-Lhoret A. Coriati 《Journal of cystic fibrosis》2017,16(3):418-424