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1.
J Surg Res ; 295: 423-430, 2024 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-38070256

RESUMEN

INTRODUCTION: Surveillance following sacrococcygeal teratoma (SCT) resection varies. The purpose of this study was to describe the clinical characteristics and outcomes of patients undergoing SCT resection and examine current institutional practices to detect recurrence. METHODS: A single-institution retrospective review of children who underwent resection of an SCT from January 1, 2010 to December 31, 2020 was performed. Data were summarized and surveillance strategies compared between histopathologic subtypes using nonparametric methods. RESULTS: Thirty six patients (75.0% female) underwent SCT removal at a median age of 8 d. Histopathology revealed 27 mature teratomas (75.0%), eight immature teratomas (22.2%), and one malignant germ cell tumor (2.8%). Median postoperative follow-up was 3.17 y (interquartile range [IQR]: 2.31-4.38 y). Patients had a median of 2.32 clinic visits per year (IQR: 2.00-2.70), alpha-fetoprotein levels were obtained at a median of 2.01 times per year (IQR: 0-1.66), and surveillance imaging was performed at a median of 2.31 times per year (IQR: 0-2.84). Patients with immature teratomas had alpha-fetoprotein laboratories obtained more frequently than patients with mature teratomas (3.10 times/year versus 0.93 times/year, P = 0.001). There was no significant difference in the number of imaging studies obtained between groups. Two patients (5.6%) developed recurrence, which were identified on magnetic resonance imaging at 191 and 104 d postresection, respectively. CONCLUSIONS: Postoperative surveillance practices varied widely. Recurrence was noted in a single malignant case in the first year following resection. Multi-institutional studies are needed to determine the optimal surveillance strategy to detect recurrence of SCT.


Asunto(s)
Neoplasias de Células Germinales y Embrionarias , Neoplasias Pélvicas , Teratoma , Niño , Humanos , Femenino , Masculino , alfa-Fetoproteínas , Región Sacrococcígea/patología , Región Sacrococcígea/cirugía , Teratoma/diagnóstico por imagen , Teratoma/cirugía , Neoplasias de Células Germinales y Embrionarias/patología , Neoplasias Pélvicas/patología
2.
Artículo en Inglés | MEDLINE | ID: mdl-38937991

RESUMEN

BACKGROUND: For children with constipation and fecal incontinence treated with antegrade continence enemas (ACE), a fluoroscopic study with contrast administered via appendicostomy/cecostomy can define the anatomy of the colon and simulate the flush to investigate associated symptoms or inadequate response. These studies can at times show retrograde flow into the small intestine. Our objective was to investigate the significance of this finding. METHODS: We reviewed studies at our institution with contrast administered via appendicostomy/cecostomy in children treated with ACE, identifying those demonstrating retrograde flow of contrast. We recorded demographics, medical history, interventions, and outcomes. RESULTS: We identified 162 studies (52% male, median age 10.7 years) with contrast via appendicostomy (76%) or cecostomy (24%). Diagnoses included anorectal malformation (38%), spinal cord anomaly (26%), functional constipation (24%), colonic dysmotility (18%), and Hirschsprung disease (12%). Fifty-nine (36%) studies showed retrograde flow: 28/59 children (48%) were not responding adequately and 21/59 (36%) had symptoms with ACE. Children with retrograde flow were more likely to have symptoms with ACE than those without (36% vs. 15%, p < 0.01). Fourteen children underwent interventions for this finding, including administering flushes more distally (4/8 responded), changing positioning of the child during flush administration, (1/2 responded), and slowing administration (1/1 responded). Retrograde flow was associated with younger age (p < 0.01), not sex or underlying diagnosis. CONCLUSION: Identifying retrograde flow during studies with contrast administered via appendicostomy/cecostomy can be useful for children with a poor response or symptoms associated with ACE, as adjustments to the mechanics of the flush can alleviate those symptoms. LEVEL OF EVIDENCE: Prognostic study, Level III.

3.
J Pediatr Gastroenterol Nutr ; 78(4): 810-816, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38284650

RESUMEN

BACKGROUND: Treatment of functional constipation (FC) in children with autism spectrum disorder (ASD) is challenging due to sensory and behavioral issues. We aimed to understand whether antegrade continence enemas (ACEs) are successful in the treatment of FC in children with ASD. METHODS: A single-institution retrospective review was performed in children diagnosed with ASD and FC who underwent appendicostomy or cecostomy placement from 2007 to 2019. Descriptive statistics regarding soiling and complications were calculated. RESULTS: There were 33 patients included, with a median age of 9.7 years at the time of ACE initiation. The average intelligence quotient was 63.6 (SD = 18.0, n = 12), the average behavioral adaptive score was 59.9 (SD = 11.1, n = 13), and the average total Child Behavioral Checklist score was 72.5 (SD = 7.1, n = 10). Soiling rates were significantly lower following ACE initiation (42.3% vs. 14.8%, p = 0.04). Behavioral issues only prevented 1 patient (3.0%) from proper ACE use. Eleven patients (36.6%) were able to transition to laxatives. There were significant improvements in patient-reported outcomes measures and quality of life. CONCLUSION: Placement of an appendicostomy or cecostomy for management of FC in children with severe ASD was successful in treating constipation and improving quality of life.


Asunto(s)
Trastorno del Espectro Autista , Incontinencia Fecal , Niño , Humanos , Calidad de Vida , Trastorno del Espectro Autista/complicaciones , Trastorno del Espectro Autista/terapia , Estreñimiento/terapia , Estreñimiento/complicaciones , Cecostomía/efectos adversos , Enema/efectos adversos , Estudios Retrospectivos , Incontinencia Fecal/etiología , Incontinencia Fecal/terapia , Resultado del Tratamiento
4.
J Pediatr Gastroenterol Nutr ; 77(2): 191-197, 2023 08 01.
Artículo en Inglés | MEDLINE | ID: mdl-37195883

RESUMEN

OBJECTIVES: The objective of this study is to investigate long-term outcomes of antegrade continence enema (ACE) treatment in children with constipation or fecal incontinence. METHODS: Prospective cohort study including pediatric patients with organic or functional defecation disorders who started ACE treatment. Data were collected at baseline and at follow-up (FU) from 6 weeks until 60 months. We assessed parent and patient-reported gastrointestinal health-related quality of life (HRQoL) using the Pediatric Quality of Life Inventory Gastrointestinal Symptoms Module (PedsQL-GI), gastrointestinal symptoms, adverse events, and patient satisfaction. RESULTS: Thirty-eight children were included (61% male, median age 7.7 years, interquartile range 5.5-12.2). Twenty-two children (58%) were diagnosed with functional constipation (FC), 10 (26%) with an anorectal malformation, and 6 (16%) with Hirschsprung disease. FU questionnaires were completed by 22 children (58%) at 6 months, 16 children (42%) at 12 months, 20 children (53%) at 24 months, and 10 children (26%) at 36 months. PedsQL-GI scores improved overall with a significant increase at 12- and 24-month FU for children with FC and a significant increase in parent reported PedsQL-GI score at 36-month FU for children with organic causes. Minor adverse events, such as granulation tissue, were reported in one-third of children, and 10% of children needed a surgical revision of their ACE. The majority of all parents and children reported that they would "probably" or "definitely" choose ACE again. CONCLUSION: ACE treatment is perceived positively by patients and parents and can lead to long-term improvement in gastrointestinal HRQoL in children with organic or functional defecation disorders.


Asunto(s)
Incontinencia Fecal , Humanos , Niño , Masculino , Femenino , Incontinencia Fecal/terapia , Calidad de Vida , Estudios Prospectivos , Resultado del Tratamiento , Estreñimiento/terapia , Enema/efectos adversos , Estudios Retrospectivos
5.
Pediatr Surg Int ; 39(1): 157, 2023 Mar 23.
Artículo en Inglés | MEDLINE | ID: mdl-36952009

RESUMEN

PURPOSE: Fecal incontinence is a problem for many patients born with an anorectal malformation (ARM) that can impact quality of life. It is unknown if racial, ethnic, and socioeconomic disparities relate to fecal continence in these children. We sought to examine outcomes and potential disparities in care. METHODS: We performed a multicenter retrospective study of children > 3y with ARM evaluated at sites participating in the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC). The primary outcome was fecal continence. We evaluated for associations between fecal continence and race, sex, age, and insurance status. RESULTS: 509 patients with ARM from 11 institutions were included. Overall, 24% reported complete fecal continence, and fecal continence was associated with older age (p < .001). For school-aged children, 27% reported complete continence, while 53% reported none. On univariate analysis, patients with combined private and public insurance showed lower rates of continence when compared to those with private insurance (23 vs. 12%; p = 0.02). Age was associated with continence on univariate and multivariable analyses. CONCLUSION: Rates of complete fecal continence in this population are low. Differences based on payor status may exist. There were no observed disparities related to sex and race. Further investigation is warranted to improve care for this patient population. LEVEL OF EVIDENCE: III. TYPE OF STUDY: Multi-institutional retrospective comparative study.


Asunto(s)
Malformaciones Anorrectales , Cirugía Colorrectal , Incontinencia Fecal , Humanos , Niño , Malformaciones Anorrectales/cirugía , Malformaciones Anorrectales/complicaciones , Estudios Retrospectivos , Recto/cirugía , Calidad de Vida , Canal Anal/anomalías , Incontinencia Fecal/epidemiología , Incontinencia Fecal/complicaciones
6.
J Pediatr Gastroenterol Nutr ; 75(3): 286-292, 2022 09 01.
Artículo en Inglés | MEDLINE | ID: mdl-35687612

RESUMEN

OBJECTIVES: Patients experiencing functional constipation (FC) can participate in structured bowel management programs (BMPs) to manage constipation or fecal incontinence when standard management fails. We sought to evaluate the efficacy of BMPs for children with FC with and without neurodevelopmental disorders. METHODS: We performed a retrospective review of children with FC who participated in our BMP from 2014 to 2021. Stool/urinary continence, bowel regimen, surgical history, parent-reported outcomes measures (PROMs: Cleveland Clinic Constipation Score, Baylor Continence Scale, Vancouver Symptom Score for Dysfunctional Elimination), and Pediatric Quality of Life Inventory (PedsQL) were assessed pre- and at least 9 months post-BMP. RESULTS: The cohort included 156 patients with a median age of 9 years and follow-up of 627 days (IQR: 389-808 days). Two sub-cohorts included patients with FC only (69%) and FC plus a neurodevelopmental disorder (31%): 59% attention-deficit/hyperactivity disorder, 33% autism spectrum disorder, and 8% obsessive-compulsive disorder. Both groups had significantly improved follow-up bowel movement frequency and continence (39%-90% neurodevelopmental, 44%-82% FC only, P < 0.001) and urinary continence (65%-90% neurodevelopmental, 69%-91% FC only, P < 0.02). There was a significant improvement in most of the PROMs at follow-up. Both groups experienced a clinically meaningful improvement in overall PedsQL scores (pre- and postBMP difference of >4.5). CONCLUSIONS: Patients with FC with and without a neurodevelopmental disorder had significant improvement in stool and urinary continence after undergoing a BMP. Further studies are needed to see if this improvement is durable over a longer period of time in this challenging cohort.


Asunto(s)
Trastorno del Espectro Autista , Incontinencia Fecal , Niño , Estreñimiento/diagnóstico , Estreñimiento/terapia , Defecación , Incontinencia Fecal/etiología , Incontinencia Fecal/terapia , Humanos , Calidad de Vida
7.
Pediatr Surg Int ; 38(10): 1473-1479, 2022 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-35930047

RESUMEN

PURPOSE: We sought to determine if children with functional constipation (FC) would have an improvement in bladder function with treatment of constipation with a bowel management program (BMP). METHODS: A single-institution review was performed in children aged 3-18 with FC who underwent a BMP from 2014 to 2020. Clinical characteristics, bowel management details, and the Vancouver Symptom Score for Dysfunctional Elimination Syndrome (VSS), Baylor Continence Scale (BCS), and Cleveland Clinic Constipation Score (CCCS) were collected. Data were analyzed using linear mixed effect modeling with random intercept. RESULTS: 241 patients were included with a median age of 9 years. Most were White 81 and 47% were female. Univariate tests showed improvement in VSS (- 3.6, P < 0.0001), BCS (- 11.96, P < 0.0001), and CCCS (- 1.9, P < 0.0001) among patients having undergone one BMP. Improvement was noted in VSS and CCCS among those with more than one BMP (VSS: - 1.66, P = 0.023; CCCS: - 2.69, P < 0.0001). Multivariate tests indicated undergoing a BMP does result in significant improvement in VSS, BCS, and CCCS (P < 0.0001). CONCLUSIONS: There is significant improvement in bladder function in children with FC who undergo a BMP. For patients with bowel and bladder dysfunction and FC, a BMP is a reasonable treatment strategy for lower urinary tract symptoms.


Asunto(s)
Síntomas del Sistema Urinario Inferior , Vejiga Urinaria , Niño , Estreñimiento/terapia , Femenino , Humanos , Intestinos , Masculino , Síndrome
8.
J Surg Res ; 257: 379-388, 2021 01.
Artículo en Inglés | MEDLINE | ID: mdl-32892134

RESUMEN

BACKGROUND: The impact of surgical volume on outcomes in Hirschsprung's disease (HD) remains uninvestigated. We aimed to determine whether higher surgeon and hospital-level HD operative volumes are associated with improved surgical outcomes following primary surgery for neonatal HD. MATERIALS AND METHODS: Neonates who underwent either an ostomy or pull-through (PT) procedure for HD before 60 d of life and a PT procedure by age 1 y were identified in the Pediatric Health Information System (PHIS). Index admissions from January 2000 to September 2012 across 41 tertiary childrens hospitals were included. Surgeon and hospital-level HD operative volume were defined as the average annual number of PT procedures performed for HD in the 2 y preceding each included case. We examined the relationship between operative volumes and all-cause readmission, readmission for Hirschsprung's associated enterocolitis (HAEC), and rates of reoperation within 30 d and 2 y. RESULTS: A total of 1268 infants were included. There were 218 patients (17.2%) readmitted to the hospital within 30 d and 540 (42.6%) within 2 y. A total of 119 patients (9.4%) had HAEC-related readmission within 30 d, and 271 (21.4%) had HAEC-related readmission within 2 y. A total of 57 patients (4.5%) had a reoperation within 30 d and 129 (10.2%) within 2 y. In risk-adjusted analyses, there were no significant associations between either surgeon or hospital HD operative volumes and readmission/reoperation rates within 30 d or 2 y. CONCLUSIONS: Neither surgeon nor hospital PT volumes were significantly associated with readmission or reoperation rates for infants with Hirschsprung's disease. Future work is needed to evaluate whether operative volumes are associated with functional outcomes following PT for HD.


Asunto(s)
Procedimientos Quirúrgicos del Sistema Digestivo/estadística & datos numéricos , Enfermedad de Hirschsprung/cirugía , Hospitales de Alto Volumen/estadística & datos numéricos , Hospitales de Bajo Volumen/estadística & datos numéricos , Cirujanos/estadística & datos numéricos , Femenino , Humanos , Recién Nacido , Masculino , Estudios Retrospectivos , Resultado del Tratamiento
9.
J Pediatr Gastroenterol Nutr ; 72(1): 168-180, 2021 01 01.
Artículo en Inglés | MEDLINE | ID: mdl-33075010

RESUMEN

ABSTRACT: Neurogastroenterology and motility (NGM) disorders are common in childhood and are often very debilitating. Although pediatric gastroenterology fellows are expected to obtain training in the diagnosis and management of patients with these disorders, there is an ongoing concern for unmet needs and lack of exposure and standardized curriculum. In the context of tailoring training components, outcome and expressed needs of pediatric gastroenterology fellows and programs, members of the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) and American Neurogastroenterology and Motility Society (ANMS) developed guidelines for NGM training in North America in line with specific expectations and goals of training as delineated through already established entrustable professional activities (EPAs). Members of the joint task force applied their expertise to identify the components of knowledge, skills, and management, which are expected of NGM consultants. The clinical knowledge, skills and management elements of the NGM curriculum are divided into domains based on anatomic regions including esophagus, stomach, small bowel, colon and anorectum. In addition, dedicated sections on pediatric functional gastrointestinal (GI) disorders, research and collaborative approach, role of behavioral health and surgical approaches to NGM disorders and transition from pediatric to adult neurogastroenterology are included in this document. Members of the NASPGHAN-ANMS task force anticipate that this document will serve as a resource to break existing barriers to pursuing a career in NGM and provide a framework towards uniform training expectations at 3 hierarchical tiers corresponding to EPA levels.


Asunto(s)
Gastroenterología , Enfermedades Gastrointestinales , Adulto , Niño , Competencia Clínica , Curriculum , Gastroenterología/educación , Enfermedades Gastrointestinales/diagnóstico , Enfermedades Gastrointestinales/terapia , Humanos , América del Norte , Sociedades Médicas , Estados Unidos
10.
Curr Gastroenterol Rep ; 23(11): 18, 2021 Oct 11.
Artículo en Inglés | MEDLINE | ID: mdl-34633517

RESUMEN

PURPOSE OF REVIEW: Ideally, after operative intervention, a child born with Hirschsprung disease (HD) should thrive, achieve fecal continence, and avoid recurrent episodes of abdominal distention and enterocolitis. However, a significant number of patients continue to struggle following their pull-through procedure. The purpose of this review is to present an organized and practical approach to the evaluation and management of the symptomatic patient post pull-through operation for HD. RECENT FINDINGS: Children diagnosed with HD who are not doing well after their initial operation can be categorized in three distinct groups: (1) those that have fecal incontinence, (2) those with obstructive symptoms, and (3) those with recurrent episodes of enterocolitis. It is important to have a systematic diagnostic approach for these patients based on a comprehensive protocol. All three of these patient groups can be treated with a combination of either medical management, reoperation when a specific anatomic or pathologic etiology is identified, or botulinum toxin for non-relaxing sphincters contributing to the obstructive symptoms or recurrent enterocolitis. For patients not doing well after their initial pull-through, a systematic workup should be employed to determine the etiology. Once identified, a multidisciplinary and organized approach to management of the symptomatic patients can alleviate most post pull-through symptoms.


Asunto(s)
Procedimientos Quirúrgicos del Sistema Digestivo , Enterocolitis , Incontinencia Fecal , Enfermedad de Hirschsprung , Niño , Enterocolitis/diagnóstico , Enterocolitis/etiología , Enterocolitis/terapia , Incontinencia Fecal/etiología , Incontinencia Fecal/terapia , Enfermedad de Hirschsprung/complicaciones , Enfermedad de Hirschsprung/diagnóstico , Enfermedad de Hirschsprung/cirugía , Humanos , Complicaciones Posoperatorias/diagnóstico , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/terapia , Estudios Retrospectivos , Resultado del Tratamiento
11.
Pediatr Radiol ; 51(10): 1867-1872, 2021 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-33991195

RESUMEN

BACKGROUND: The sacral ratio has been used as a tool for evaluating sacral development in patients with anorectal malformations. Sacral ratios can be calculated by obtaining sacral radiographs in the anteroposterior (AP) and lateral planes. OBJECTIVE: The objective of the study was to determine the correlation and agreement in sacral ratio calculations. MATERIALS AND METHODS: In this single institution retrospective cohort study, we reviewed medical charts of all pediatric anorectal malformation patients treated between March 2014 and September 2018 who had both AP and lateral images of their sacrum. All sacral ratios were measured by three radiologists. Pearson's correlation coefficients and corresponding 95% confidence intervals (CIs) were used to assess the correlation between the AP and lateral radiographs. A weighted Kappa statistic was used to measure the agreement between how the AP and lateral sacral ratios categorized observations into risk groups. RESULTS: Our initial cohort consisted of 646 observations from patients with anorectal malformations who had radiographs obtained in both AP and lateral planes. We excluded all observations (n=76) where the radiographs were deemed to be inadequate or not appropriately centered to measure sacral ratio. For a given pair of measurements, the mean lateral sacral ratio was 0.07 units greater than the AP plane (95% CI 0.06-0.09, paired t-test P-value <0.0001). AP and lateral images had a moderate positive correlation (Pearson's r=0.76, 95% CI 0.73-0.79, P<0.0001) and moderate agreement in risk categorization (unweighted kappa = 0.60, P<0.0001). AP and lateral readings conducted by all three radiologists had excellent inter-rater reliability with intraclass correlations for AP and lateral sacral ratios of 0.88 and 0.84, respectively. CONCLUSION: Even though the AP and lateral sacral ratios had moderate positive correlation, the mean sacral ratio determined by images in the lateral plane was 0.07 units greater than the AP plane. AP and lateral sacral ratios concluded different risk categories relatively often. Future studies are needed to determine whether AP or lateral sacral ratios correlate better with continence in patients with anorectal malformations.


Asunto(s)
Malformaciones Anorrectales , Malformaciones Anorrectales/diagnóstico por imagen , Niño , Humanos , Radiografía , Reproducibilidad de los Resultados , Estudios Retrospectivos , Sacro/diagnóstico por imagen
12.
J Sci Food Agric ; 101(5): 1833-1843, 2021 Mar 30.
Artículo en Inglés | MEDLINE | ID: mdl-32964464

RESUMEN

BACKGROUND: It is relatively unknown as to how dietary bioactive compound sulforaphane (SFN) and vitamin D regulate gene expression in colorectal cancer. We hypothesized that a combination of SFN with vitamin D would prove beneficial in colorectal cancer. A combinatorial chemo-preventive strategy was employed to investigate the impact of SFN on chromatin remodeling in colorectal carcinoma. To understand the epigenetics-mediated changes in gene expression in response to SFN and vitamin D, Caco-2 cells were exposed for 24 h to vitamin D (100 nmol L-1 ) either alone or in combination with SFN and trichostatin A (20 and 1 µmol L-1 , respectively) at 70% confluency (proliferating) and after 13 days post-confluency (fully differentiated). Changes to VDR, CYP24A1, CYP27B1 and TRPV6 gene expressions were quantified using real-time PCR-based assays. Histone deacetylase (HDAC) inhibitor activity was assessed using HDAC I/II assay that measured global changes in acetylation status. RESULTS: In differentiated Caco-2 cells, none of the genes had significant changes from D alone group. D + SFN (P = 0.99) demonstrated an opposing effect from D alone and decreased VDR expression. However, in proliferating Caco-2 cells, D + SFN (P < 0.04) increased VDR expression and decreased CYP27B1 (P < 0.01) more than D alone (P = 0.38 and 0.07, respectively). Although statistically significant, D + SFN (P = 0.01) effect on HDAC inhibitor activity was less than trichostatin A alone group (P < 0.0004) or SFN alone group (P < 0.0014). CONCLUSIONS: The data suggest that colon cancer cells respond to dietary components differently under different conditions. The effect of vitamin D and SFN is selective and gene-specific in the complex multistep process of colorectal carcinogenesis in vitro. © 2020 Society of Chemical Industry.


Asunto(s)
Neoplasias del Colon/genética , Histona Desacetilasas/metabolismo , Isotiocianatos/farmacología , Sulfóxidos/farmacología , Vitamina D/farmacología , Acetilación/efectos de los fármacos , Células CACO-2 , Canales de Calcio/genética , Canales de Calcio/metabolismo , Neoplasias del Colon/metabolismo , Neoplasias del Colon/prevención & control , Regulación Neoplásica de la Expresión Génica/efectos de los fármacos , Histona Desacetilasas/genética , Humanos , Ácidos Hidroxámicos/farmacología , Receptores de Calcitriol/genética , Receptores de Calcitriol/metabolismo , Canales Catiónicos TRPV/genética , Canales Catiónicos TRPV/metabolismo , Vitamina D3 24-Hidroxilasa/genética , Vitamina D3 24-Hidroxilasa/metabolismo
13.
J Surg Res ; 245: 564-568, 2020 01.
Artículo en Inglés | MEDLINE | ID: mdl-31476645

RESUMEN

BACKGROUND: Children with Hirschsprung disease (HD) and Down syndrome (DS) are reported to experience a higher incidence of Hirschsprung-associated enterocolitis (HAEC) than those without DS, although whether their individual episodes are more severe is unknown. METHODS: A single-institution retrospective cohort study was performed on all patients with HD admitted with HAEC between 2009 and 2017. Patients were divided into two groups based on an associated diagnosis of DS, and demographics, clinical characteristics at presentation, and clinical outcomes were compared. RESULTS: Of 86 admissions for HAEC over the study period, 12 (14%) were for patients with DS. Patients admitted with and without DS were similar in terms of age (3.0 versus 2.6 y, P = 0.72), male gender (58% versus 77%, P = 0.17), and proportion with disease proximal to the left colon (9% versus 12%, P = 0.70). Patients admitted with DS were more likely to be tachycardic (75% versus 19%, P ≤ 0.001) and hypotensive (33% versus 7%, P = 0.01) at presentation and presented for treatment after a longer duration of symptoms than those without DS (84 versus 24 h, P = 0.001). Patients admitted with DS were more likely to require intensive care unit admission (58% versus 12%, P = 0.006) and received a longer course of antibiotics (13 d versus 7 d, P = 0.02). The length of stay was similar (6 versus 4 d, P = 0.34). CONCLUSIONS: The results of our study suggest that patients with DS experience more severe episodes of HAEC. Ongoing study is needed to identify preventive strategies to decrease the morbidity of HAEC in children with HD and DS.


Asunto(s)
Síndrome de Down/complicaciones , Enterocolitis/epidemiología , Enfermedad de Hirschsprung/complicaciones , Adolescente , Estudios de Casos y Controles , Niño , Preescolar , Enterocolitis/diagnóstico , Enterocolitis/etiología , Femenino , Humanos , Incidencia , Lactante , Masculino , Estudios Retrospectivos , Índice de Severidad de la Enfermedad
14.
J Surg Res ; 256: 272-281, 2020 12.
Artículo en Inglés | MEDLINE | ID: mdl-32712441

RESUMEN

BACKGROUND: Anorectal malformations (ARMs) are a spectrum of congenital anomalies with varying prognosis for fecal continence. The sacral ratio (SR) is a measure of sacral development that has been proposed as a method to predict future fecal continence in children with ARM. The aim of this study was to quantify the inter-rater reliability (IRR) of SR calculations by radiologists at different institutions. MATERIALS AND METHODS: x-Rays in the anteroposterior (AP) and lateral planes were reviewed by a pediatric radiologist at each of six different institutions. Subsequently, images were reviewed by a single, central radiologist. The IRR was assessed by calculating Pearson correlation coefficients and intraclass correlation coefficients from linear mixed models with patient and rater-level random intercepts. RESULTS: Imaging from 263 patients was included in the study. The mean inter-rater absolute difference in the AP SR was 0.05 (interquartile range, 0.02-0.10), and in the lateral SR was 0.16 (interquartile range, 0.06-0.25). Overall, the IRR was excellent for AP SRs (intraclass correlation coefficient [ICC], 81.5%; 95% confidence interval, 75.1%-86.0%) and poor for lateral SRs (ICC, 44.0%; 95% CI, 29.5%-59.2%). For both AP and lateral SRs, ICCs were similar when examined by the type of radiograph used for calculation, severity of the ARM, presence of sacral or spinal anomalies, and age at imaging. CONCLUSIONS: Across radiologists, the reliability of SR calculations was excellent for the AP plane but poor for the lateral plane. These results suggest that better standardization of lateral SR measurements is needed if they are going to be used to counsel families of children with ARM.


Asunto(s)
Malformaciones Anorrectales/cirugía , Antropometría/métodos , Incontinencia Fecal/epidemiología , Complicaciones Posoperatorias/epidemiología , Sacro/diagnóstico por imagen , Malformaciones Anorrectales/complicaciones , Malformaciones Anorrectales/diagnóstico , Incontinencia Fecal/etiología , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Variaciones Dependientes del Observador , Complicaciones Posoperatorias/etiología , Pronóstico , Radiografía , Reproducibilidad de los Resultados , Estudios Retrospectivos , Medición de Riesgo/métodos , Sacro/anomalías , Sacro/crecimiento & desarrollo , Índice de Severidad de la Enfermedad , Resultado del Tratamiento
15.
J Pediatr Gastroenterol Nutr ; 69(2): e34-e38, 2019 08.
Artículo en Inglés | MEDLINE | ID: mdl-30921256

RESUMEN

AIMS AND OBJECTIVES: The aim of this review is develop a reliable and valid questionnaire that assesses patient-reported experience measures (PREMs) and patient-reported outcome measures (PROMs) of caregivers, families, and patients with severe constipation and fecal incontinence who failed conservative treatments and require a formal bowel management program (BMP). METHODS: A 5-step iterative process was utilized to ensure the reliability and validity of the final instrument. Parents or guardians of patients undergoing our week-long BMP were first asked 20 open-ended questions aimed at understanding the challenges in managing their child's condition. Responses were transformed into an initial 41-item survey constituted of 2 constructs relating to PREMs (24 items) and PROMs (17 items). Survey items were then administered to 359 parents and guardians undergoing BMP. Cognitive interviews were performed with 20 families to assess face validity and to further refine the survey. Instrument readability and reliability was assessed by Flesch-Kincaid and Crohnbach α analyses. Items that weakly correlated were deleted to yield a final instrument that was both valid and reliable. RESULTS: A 33-statement survey was developed that encompasses important physical and emotional health concerns, quality of life, treatment success, financial considerations, schooling, parental employment, and social concerns. The final instrument, the Patient-Reported Experience and Outcome Measure in a Bowel Management Program (PREOM-BMP), is divided into 2 separate constructs that assess important patient-reported experience (22 items) and outcome (11 items) measures. Reliability analyses on the final instrument yielded a Crohnbach α of 0.925. CONCLUSIONS: The PREOM-BMP offers physicians and nursing professionals a brief and valid tool to measure the impact of a bowel management program on both the child and the family unit.


Asunto(s)
Estreñimiento/prevención & control , Incontinencia Fecal/prevención & control , Medición de Resultados Informados por el Paciente , Encuestas y Cuestionarios , Adolescente , Niño , Preescolar , Femenino , Humanos , Masculino , Reproducibilidad de los Resultados , Adulto Joven
16.
J Am Soc Nephrol ; 29(1): 250-259, 2018 01.
Artículo en Inglés | MEDLINE | ID: mdl-29038285

RESUMEN

CKD is steadily increasing along with obesity worldwide. Furthermore, obesity is a proinflammatory risk factor for progression of CKD and cardiovascular disease. We tested the hypothesis that implementation of caloric restriction and aerobic exercise is feasible and can improve the proinflammatory metabolic milieu in patients with moderate to severe CKD through a pilot, randomized, 2×2 factorial design trial. Of 122 participants consented, 111 were randomized to receive caloric restriction and aerobic exercise, caloric restriction alone, aerobic exercise alone, or usual care. Of those randomized, 42% were women, 25% were diabetic, and 91% were hypertensive; 104 started intervention, and 92 completed the 4-month study. Primary outcomes were a change from baseline in absolute fat mass, body weight, plasma F2-isoprostane concentrations, and peak oxygen uptake (VO2 peak). Compared with usual care, the combined intervention led to statistically significant decreases in body weight and body fat percentage. Caloric restriction alone also led to significant decreases in these measures, but aerobic exercise alone did not. The combined intervention and each independent intervention also led to significant decreases in F2-isoprostane and IL-6 concentrations. No intervention produced significant changes in VO2 peak, kidney function, or urine albumin-to-creatinine ratio. In conclusion, 4-month dietary calorie restriction and aerobic exercise had significant, albeit clinically modest, benefits on body weight, fat mass, and markers of oxidative stress and inflammatory response in patients with moderate to severe CKD. These results suggest healthy lifestyle interventions as a nonpharmacologic strategy to improve markers of metabolic health in these patients.


Asunto(s)
Restricción Calórica , Ejercicio Físico/fisiología , Insuficiencia Renal Crónica/fisiopatología , Insuficiencia Renal Crónica/terapia , Adiposidad , Anciano , Albuminuria/orina , Peso Corporal , Creatinina/orina , F2-Isoprostanos/sangre , Femenino , Tasa de Filtración Glomerular , Humanos , Interleucina-6/sangre , Masculino , Persona de Mediana Edad , Estrés Oxidativo , Consumo de Oxígeno , Proyectos Piloto
17.
J Digit Imaging ; 32(5): 761-765, 2019 10.
Artículo en Inglés | MEDLINE | ID: mdl-30350007

RESUMEN

Surgeons have a steep learning capacity to understand 2-D images provided by conventional cloacagrams. Imaging advances now allow for 3-D reconstruction and 3-D models; but no evaluation of the value of these techniques exists in the literature. Therefore, we sought to determine if advances in 3-D imaging would benefit surgeons, lead to accelerated learning, and improve understanding for operative planning of a cloaca reconstruction. Questionnaires were used to assess the understanding of 2-D and 3-D images by pediatric surgical faculty and trainees. For the same case of a cloacal malformation, a 2D contrast study cloacagram, a 3D model rotatable CT scan reconstruction, a software enhanced 3D video animation (which allowed the observer to manipulate the structure in any orientation), and a printed physical 3D cloaca model that could be held in the observer's hand were employed. Logistic mixed effect models assessed whether the proportion of questions about the case that were answered correctly differed by imaging modality, and whether the proportion answered correctly differed between trainee and attending surgeons for any particular modality. Twenty-nine pediatric surgery trainees (27 pediatric general surgery and 2 pediatric urology surgery trainees) and 30 pediatric surgery and urology faculty participated. For trainees, the percentage of questions answered correctly was: 2-D 10.5%, 3-D PACS 46.7%, 3-D Enhanced 67.1%, and 3-D Printed 73.8%. For faculty, the total percentage of questions answered correctly was: 2-D 22.2%, 3-D PACS 54.8%, 3D Enhanced 66.2%, and 3-D printed 74.0%. The differences in rates of correctness across all four modalities were significant in both fellows and attendings (p < 0.001), with performance being lowest for the 2-D modality, and with increasing percentage of correct answers with each subsequent modality. The difference between trainees and attendings in correctness rate was significant only for the 2-D modality, with attendings answering correctly more often. The 2-D cloacagram, as the least complex model, was the most difficult to interpret. The more complex the modality, the more correct were the responses obtained from both groups. Trainees and attendings had similar levels of correct answers and understanding of the cloacagram for the more advanced modalities. Mental visualization skills of anatomy and complex 3-D spatial arrangements traditionally have taken years of experience to master. Now with novel surgical education resources of a 3-D cloacagram, a more quickly advancing skill is possible.


Asunto(s)
Cloaca/anatomía & histología , Imagenología Tridimensional/métodos , Modelos Anatómicos , Pediatría/métodos , Cirujanos , Cloaca/diagnóstico por imagen , Fluoroscopía , Humanos
18.
Clin Colon Rectal Surg ; 31(2): 61-70, 2018 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-29487488

RESUMEN

Anorectal malformation are common congenital problems occurring in 1 in 5,000 births and have a spectrum of anatomical presentations, requiring individualized treatments for the newborn, sophisticated approaches to the definitive reconstruction, and management of long-term treatments and outcomes. Associated anomalies related to the cardiac, renal, gynecologic, orthopedic, spinal, and sacral systems impact care and prognosis. Long-term results are good provided there is an accurate anatomical reconstruction and a focus on maximizing of functional results.

20.
Mediators Inflamm ; 2017: 9474896, 2017.
Artículo en Inglés | MEDLINE | ID: mdl-28316379

RESUMEN

Obesity is an established risk factor for many diseases including intestinal cancer. One of the responsible mechanisms is the chronic inflammation driven by obesity. However, it remains to be defined whether diet-induced obesity exacerbates the intestinal inflammatory status by cytokines produced in adipose tissue or the high fat diet first alters the gut microbiota and then drives intestinal inflammation. To address this question, we fed C57BL/6 mice with a high fat diet (HF, 60%) and sacrificed them sequentially after 8, 12, and 16 weeks, and then compositions of gut microbiota and expressions of antimicrobial peptides were determined. The compositions of gut microbiota were altered at 8 wk HF feeding, followed with reduced Paneth antimicrobial peptides lysozyme and Reg IIIγ after 12 and 16 wk HF feeding (p < 0.05), whereas elevations of circulating inflammatory cytokines IFNγ and TNF-α were observed until feeding a HF diet for 16 weeks (p < 0.05). These results indicated that high fat diet may stimulate intestinal inflammation via altering gut microbiota, and it occurs prior to the potential influence by circulating inflammatory cytokines. These findings emphasized the importance of microbiota, in addition to adipose tissue per se, in driving intestinal inflammation, which may thereafter promote intestinal tumorigenesis.


Asunto(s)
Citocinas/metabolismo , Dieta Alta en Grasa/efectos adversos , Microbioma Gastrointestinal/fisiología , Células de Paneth/metabolismo , Péptidos/metabolismo , Animales , Western Blotting , Microbioma Gastrointestinal/genética , Inmunohistoquímica , Inflamación/inmunología , Inflamación/metabolismo , Masculino , Ratones , Ratones Endogámicos C57BL , Muramidasa/genética , Muramidasa/metabolismo , Obesidad/inmunología , Obesidad/metabolismo , Péptidos/genética , Ribonucleasa Pancreática/genética , Ribonucleasa Pancreática/metabolismo
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