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1.
J Pediatr Urol ; 2024 Jul 10.
Artigo em Inglês | MEDLINE | ID: mdl-39033033

RESUMO

BACKGROUND: Girls with cloacal malformation are at risk of bladder dysfunction, with nearly 90% exhibiting some degree of dysfunction. Surgical dissection, particularly with total urogenital mobilization (TUM), has been hypothesized as a cause of worsening bladder function despite this population commonly having associated vertebral and spinal cord abnormalities that may also explain bladder dysfunction. More recently there has been great effort to select the appropriate surgical technique for cloacal repair in each patient in order to minimize dissection and potential damage to the bladder. We aimed to evaluate the effect of surgical cloacal repair on bladder function based on pre and post-surgery urodynamics (UDS) testing. METHODS: A prospectively collected database of patients with anorectal malformation at a single center was queried for girls with cloacal malformations who had undergone surgical repair from 2015 to 2022. It is our current protocol to perform UDS before and after cloacal repair. Only patients who completed both pre and post-surgery UDS were included. UDS were evaluated and classified using the UMPIRE protocol. RESULTS: A total of 48 patients were included in the cohort. The majority of patients (79.2%) had stable or improved UDS post-op leaving 10 patients (20.8%) who had worsening UDS. Long common channel (≥3 cm) was the only factor significantly associated with worsening UDS. (p = 0.03) Nearly 30% (n = 8) of those undergoing UGS had worse post-op UDS compared to 9.5% (n = 2) with TUM. All patients who worsened UDS initially had safe UDS that changed to intermediate, except for one who worsened to hostile in the setting of significant social challenges and non-compliance. Only common channel length was predictive of worsening UDS, while the type of surgical approach and spine status were not. While the overall risk of worsening UDS after TUM is only 9.5%, patients with normal spines undergoing TUM had the lowest risk, seen in only one in 15 patients (6.6%). CONCLUSIONS: Common channel length was the most significant predictor of worsening UDS, while spine status and surgical technique (TUM vs UGS) did not significantly impact this finding. By following this established surgical protocol based on common channel and urethral lengths, is rare for the surgical cloacal repair to result in worsening post-op UDS, particularly in those undergoing TUM for short common channel and normal spine.

2.
J Pediatr Surg ; 2024 Jun 19.
Artigo em Inglês | MEDLINE | ID: mdl-38981833

RESUMO

BACKGROUND: Children with Hirschsprung disease (HSCR) proximal to the splenic flexure or those needing a redo pull-through (PT) are at risk for tension and ischemia of the PT which could result in leak, stricture, or loss of ganglionated bowel. Colonic derotation is a technique used to minimize tension and avoid duodenal obstruction. The aim of this study was to describe this technique and outcomes in a series of patients requiring this intervention. METHODS: All patients underwent initial diversion and colonic mapping. The derotation procedure involves mobilization of the remaining colon, counterclockwise rotation via the stoma closure site, placement of the pull through (the right colon) lying on the right of the pelvis, and ligation of the middle colic artery with preservation of the marginal branch running from the ileocolic artery. This maneuver prevents compression of the duodenum by the mesenteric vessels and allows for an isoperistaltic, tension-free anastomosis. Intraoperative indocyanine green fluorescence angiography (ICG-FA) was utilized in many of the cases to map the blood supply of the pull-through colon. We reviewed outcomes for all children with HSCR who underwent colonic derotation from 2014 to 2023. Descriptive statistics were performed. RESULTS: There were 37 children included. Most were male (67.5%) with the original transition zone proximal to the rectosigmoid (81.1%). The median age at PT was 9.3 months [6.1-39.7]. Median operative time was 6.6 h [4.9-7.4] and 19 cases (51.4%) used ICG-FA. Most children had no 30-day postoperative complications (67.6%); in those who did develop complications, readmissions for electrolyte imbalance was most common (50.0%). There were zero cases of anastomotic leak at PT anastomosis. At long-term follow up, median 4.4 years [2.3-7.0], three children (8.1%) developed an anastomotic stricture, all were amenable to anal dilation, and five experienced episodes of enterocolitis (14.7%). Most children had between 1 and 4 stools per day (58.8%). CONCLUSION: Colonic derotation is a useful strategy to ensure well-perfused colonic length, protect the marginal artery blood supply, avoid duodenal compression, and ensure a tension-free anastomosis with minimal complications. TYPE OF STUDY: Original research, retrospective cohort. LEVEL OF EVIDENCE: III.

3.
J Biol Chem ; 300(7): 107461, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38876299

RESUMO

Theta-mediated end joining (TMEJ) is critical for survival of cancer cells when other DNA double-stranded break repair pathways are impaired. Human DNA polymerase theta (Pol θ) can extend ssDNA oligonucleotides, but little is known about preferred substrates and mechanism. We show that Pol θ can extend both ssDNA and RNA substrates by unimolecular stem-loop synthesis initiated by only two 3' terminal base pairs. Given sufficient time, Pol θ uses alternative pairing configurations that greatly expand the repertoire of sequence outcomes. Further primer-template adjustments yield low-fidelity outcomes when the nucleotide pool is imbalanced. Unimolecular stem-loop synthesis competes with bimolecular end joining, even when a longer terminal microhomology for end joining is available. Both reactions are partially suppressed by the ssDNA-binding protein replication protein A. Protein-primer grasp residues that are specific to Pol θ are needed for rapid stem-loop synthesis. The ability to perform stem-loop synthesis from a minimally paired primer is rare among human DNA polymerases, but we show that human DNA polymerases Pol η and Pol λ can catalyze related reactions. Using purified human Pol θ, we reconstituted in vitro TMEJ incorporating an insertion arising from a stem-loop extension. These activities may help explain TMEJ repair events that include inverted repeat sequences.


Assuntos
DNA Polimerase teta , DNA Polimerase Dirigida por DNA , Humanos , DNA Polimerase Dirigida por DNA/metabolismo , DNA Polimerase Dirigida por DNA/genética , DNA de Cadeia Simples/metabolismo , DNA de Cadeia Simples/genética , DNA de Cadeia Simples/química , Reparo do DNA por Junção de Extremidades , Reparo do DNA , Proteína de Replicação A/metabolismo , Proteína de Replicação A/genética , DNA Polimerase beta/metabolismo , DNA Polimerase beta/genética , DNA Polimerase beta/química
4.
J Pediatr Surg ; 59(8): 1652-1656, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38735806

RESUMO

PURPOSE: Congenital anorectal stenosis is managed by dilations or operative repair. Recent studies now propose use of dilations as the primary treatment modality to potentially defer or eliminate the need for surgical repair. We aim to characterize the management and outcomes of these patients via a multi-institutional review using the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) registry. METHODS: A retrospective database review was performed using the PCPLC registry. The patients were evaluated for demographics, co-morbidities, diagnostic work-up, surgical intervention, current bowel management, and complications. RESULTS: 64 patients with anal or rectal stenosis were identified (57 anal, 7 rectal) from a total of 14 hospital centers. 59.6% (anal) and 42.9% (rectal) were male. The median age was 3.2 (anal) and 1.9 years (rectal). 11 patients with anal stenosis also had Currarino Syndrome with 10 of the 11 patients diagnosed with a presacral mass compared to only one rectal stenosis with Currarino Syndrome and a presacral mass. 13 patients (22.8%, anal) and one (14.3%, rectal) underwent surgical correction. Nine patients (8 anal, 1 rectal) underwent PSARP. Other procedures performed were cutback anoplasty and anterior anorectoplasty. The median age at repair was 8.4 months (anal) and 10 days old (rectal). One patient had a wound complication in the anal stenosis group. Bowel management at last visit showed little differences between groups or treatment approach. CONCLUSION: The PCPLC registry demonstrated that these patients can often be managed successfully with dilations alone. PSARP is the most common surgical repair chosen for those who undergo surgical repair. LEVEL OF EVIDENCE: III.


Assuntos
Dilatação , Humanos , Masculino , Estudos Retrospectivos , Feminino , Lactente , Pré-Escolar , Constrição Patológica/cirurgia , Dilatação/métodos , Sistema de Registros , Malformações Anorretais/cirurgia , Malformações Anorretais/complicações , Criança , Resultado do Tratamento , Reto/anormalidades , Reto/cirurgia , Doenças Retais/terapia , Doenças Retais/cirurgia , Doenças Retais/congênito , Doenças Retais/diagnóstico , Canal Anal/anormalidades , Canal Anal/cirurgia , Anus Imperfurado/cirurgia , Anus Imperfurado/complicações , Anus Imperfurado/diagnóstico
5.
J Pediatr Surg ; 59(8): 1647-1651, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38734497

RESUMO

BACKGROUND: Multiple factors impact ability to achieve urinary continence in cloacal malformation including common channel (CC) and urethral length and presence of spinal cord abnormalities. Few publications describe continence rates and bladder management in this population. We evaluated our cohort of patients with cloacal malformation to describe the bladder management and continence outcomes. METHODS: We reviewed a prospectively collected database of patients with cloacal malformation managed at our institution. We included girls ≥3 years (y) of age and evaluated their bladder management methods and continence. Dryness was defined as <1 daytime accident per week. Incontinent diversions with both vesicostomy and enterovesicostomy were considered wet. RESULTS: A total of 152 patients were included. Overall, 93 (61.2%) are dry. Nearly half (47%) voided via urethra, 65% of whom were dry. Twenty patients (13.1%) had incontinent diversions. Over 40% of the cohort performed clean intermittent catheterization (CIC), approximately half via urethra and half via abdominal channel. Over 80% of those performing CIC were dry. In total, 12.5% (n = 19) required bladder augmentation (BA). CC length was not associated with dryness (p = 0.076), need for CIC (p = 0.253), or need for abdominal channel (p = 0.497). The presence of a spinal cord abnormality was associated with need for CIC (p = 0.0117) and normal spine associated with ability to void and be dry (p = 0.004) CONCLUSIONS: In girls ≥ 3 y of age with cloacal malformation, 61.2% are dry, 65% by voiding via urethra and 82% with CIC. 12.5% require BA. Further investigation is needed to determine anatomic findings associated with urinary outcomes. LEVEL OF EVIDENCE: IV.


Assuntos
Cloaca , Bexiga Urinária , Incontinência Urinária , Humanos , Feminino , Pré-Escolar , Cloaca/anormalidades , Cloaca/cirurgia , Incontinência Urinária/etiologia , Bexiga Urinária/anormalidades , Bexiga Urinária/cirurgia , Estudos Retrospectivos , Criança , Uretra/anormalidades , Uretra/cirurgia , Derivação Urinária/métodos , Cateterismo Uretral Intermitente , Resultado do Tratamento
6.
J Pediatr Surg ; 59(8): 1643-1646, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38749777

RESUMO

BACKGROUND: As pediatric patients with colorectal diseases grow, it is important to address transition to adult practice. We aim to describe our center's transition process and early outcomes. METHODS: We developed a standardized process for transition to adult practice. An annual survey is given to parents and caregivers starting at age 12 that assesses knowledge of disease, independence with healthcare tasks, and confidence and interest regarding transition. After multidisciplinary review, those eligible are recommended for transition. Those not referred are provided with tools to help with areas of weakness. Outcomes were analyzed with descriptive and regression analyses (significance at p ≤ 0.05). RESULTS: A total of 116 patients were evaluated, with 80 patients (69.0%) recommended for transition. Median age at survey was 15.5 years [IQR: 13.7-18.1], and those recommended were older (16.6 years [IQR: 14.7-19.4] vs 13.5 years [IQR: 12.5-14.9], p < 0.001)). Primary diagnosis and gender were not associated with recommendation for transition. Overall, a minority (18.1%) were able to complete healthcare tasks; this correlated strongly with transition recommendation (26.3% vs 0.0%, p < 0.0001). On regression controlling for age, diagnosis, knowledge, and confidence, age (aOR 1.98, 95% CI 1.44-2.71) and confidence (aOR 3.78, 95% CI 1.29-11.11) independently predicted transition recommendation. CONCLUSION: A standardized approach may be effective in transitioning patients from pediatric to adult colorectal surgery practice. Patients who transition are more confident and can perform healthcare tasks independently; however, these skills are not essential prior to a recommendation of transition. LEVEL OF EVIDENCE: III.


Assuntos
Transição para Assistência do Adulto , Humanos , Transição para Assistência do Adulto/normas , Adolescente , Feminino , Masculino , Criança , Cirurgia Colorretal/normas , Adulto Jovem , Doenças Retais/cirurgia
7.
J Pediatr Surg ; 2024 May 09.
Artigo em Inglês | MEDLINE | ID: mdl-38816305

RESUMO

BACKGROUND: Children with colorectal diseases such as anorectal malformations (ARM), Hirschsprung disease (HD), and functional constipation (FC) undergo bowel management programs (BMPs) to achieve cleanliness. While patient outcomes, such as cleanliness and quality of life, are well understood, patient experience, such as relationships, ability to participate in sports, and independence and self-confidence is less well understood. We aimed to assess the relationship between BMP and patient experience. METHODS: A cross-sectional survey was administered to 295 patients ≥3 years old with ARM, HD, and FC completing BMP. The survey contains 22 questions regarding patient-reported experience measures (PREMs) and 11 regarding patient-reported outcomes measures (PROMs). Each was graded on a Likert scale, with higher scores meaning better experience. Scores were compared by demographics and clinical characteristics and logistic regression was performed controlling for clinically significant variables. A p-value of ≤0.05 was significant. RESULTS: There were 205 eligible respondents (69.5%) with a median age of 8.9 years [IQR: 6.1-12.4]. ARM was most common (51.2%) and most achieved cleanliness on BMP (69.3%). There were no differences in experience scores by age, diagnosis, or bowel regimen. Patients that were clean had significantly higher PREM scores (67.7 [IQR: 64.0-83.0] vs. 64.8 [IQR: 55.0-70.1], p = 0.0002) and PROM scores (36.8 [IQR: 33.0-41.0] vs. 34.0 [31.0-38.5], p = 0.005). On regression analysis, cleanliness remained a strongly significant predictor of positive experience scores (ß 7.37, SE 1.86, p < 0.0001). CONCLUSIONS: Achieving cleanliness was associated with positive patient experience of bowel management programs. This finding suggests that achieving cleanliness, regardless of regimen, may allow patients the best functional and experiential outcomes.

8.
Artigo em Inglês | MEDLINE | ID: mdl-38768703

RESUMO

STUDY OBJECTIVE: Mullerian duct anomalies are common in females with anorectal malformations (ARMs), although there are no universally recommended screening protocols for identification. Historically, at our institution, we have recommended a screening pelvic ultrasound (PUS) 6 months after thelarche and menarche. We aimed to evaluate outcomes associated with our post-thelarche screening PUS in females with ARMs. METHODS: An institutional review board-approved retrospective chart review was performed for all female patients 8 years old or older with ARMs and documented thelarche. Data were collected on demographic characteristics and clinical course. The primary outcome was adherence to the recommended PUS. Secondary outcomes included imaging correlation with suspected Mullerian anatomy and need for intervention on the basis of imaging findings. RESULTS: A total of 112 patients met the inclusion criteria. Of them, 87 (77.7%) completed a recommended post-thelarche screening PUS. There were no differences in completion on the basis of age, race, establishment with a primary care provider, insurance status, or type of ARM. Nine patients (10.3%) had findings on their PUS that did not correlate with their suspected Mullerian anatomy; five (5.7%) required intervention, with two requiring menstrual suppression, two requiring surgical intervention, and one requiring further imaging. CONCLUSION: Most patients completed the recommended post-thelarche screening PUS. In a small subset of patients, PUS did not correlate with suspected Mullerian anatomy and generated a need for intervention. Post-thelarche PUS can be a useful adjunct in patients with ARMs to identify gynecologic abnormalities.

9.
BMC Health Serv Res ; 24(1): 556, 2024 Apr 30.
Artigo em Inglês | MEDLINE | ID: mdl-38693557

RESUMO

OBJECTIVE: Long waiting times for elective hospital treatments are common in many countries. This study seeks to address a deficit in the literature concerning the effect of long waits on the wider consumption of healthcare resources. METHODS: We carried out a retrospective treatment-control study in a healthcare system in South West England from 15 June 2021 to 15 December 2021. We compared weekly contacts with health services of patients waiting over 18 weeks for treatment ('Treatments') and people not on a waiting list ('Controls'). Controls were matched to Treatments based on age, sex, deprivation and multimorbidity. Treatments were stratified by the clinical specialty of the awaited hospital treatment, with healthcare usage assessed over various healthcare settings. Wilcoxon signed-rank tests assessed whether there was an increase in healthcare utilisation and bootstrap resampling was used to estimate the magnitude of any differences. RESULTS: A total of 44,616 patients were waiting over 18 weeks (the constitutional target in England) for treatment during the study period. There was an increase (p < 0.0004) in healthcare utilisation for all specialties. Patients in the Cardiothoracic Surgery specialty had the largest increase, with 17.9 [interquartile-range: 4.3, 33.8] additional contacts with secondary care and 17.3 [-1.1, 34.1] additional prescriptions per year. CONCLUSION: People waiting for treatment consume higher levels of healthcare than comparable individuals not on a waiting list. These findings are relevant for clinicians and managers in better understanding patient need and reducing harm. Results also highlight the possible 'false economy' in failing to promptly resolve long elective waits.


Assuntos
Procedimentos Cirúrgicos Eletivos , Aceitação pelo Paciente de Cuidados de Saúde , Listas de Espera , Humanos , Estudos Retrospectivos , Masculino , Feminino , Pessoa de Meia-Idade , Procedimentos Cirúrgicos Eletivos/estatística & dados numéricos , Procedimentos Cirúrgicos Eletivos/economia , Idoso , Aceitação pelo Paciente de Cuidados de Saúde/estatística & dados numéricos , Inglaterra , Adulto , Estudos de Casos e Controles , Reino Unido
10.
J Pediatr Surg ; 59(8): 1633-1637, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38760307

RESUMO

BACKGROUND: Children with functional constipation require prolonged laxative administration for proper emptying. Whether these laxatives can be weaned after better functioning is achieved is unknown. We aim to describe a standardized protocol for stimulant laxative weaning and its early outcomes. METHODS: Patients were candidates for weaning if they had been on a stable laxative dose for six months, defined as one bowel movement per day with no soiling, impaction, or enemas. Laxative dose was decreased by 10-25% with re-evaluation every two weeks. If patients remained well without constipation, dose was weaned further by 10-25%. If there were worsening of symptoms, lower dose was maintained for 3-6 months until re-evaluation. RESULTS: There were a total of sixteen patients evaluated. Median age was 12.7 years [IQR: 11.7-15.3] with laxative duration of 8.0 years [IQR: 5.4-10.7]. All patients were on senna; some were on fiber. Median starting senna dose was 71.3 mg [IQR: 54.3-75.0] and median fiber dose was 5.5 g [IQR: 4.0-6.0]. As of most recent follow up, nine patients (56.3%) had weaned off laxatives in 3.7 months [IQR: 1.3-11.6]. For those still on laxatives, median reduction in dose was 41.4 mg [30.0-75.0], and over half weaned their dose by >50%. Almost all (90.9%) of those on high doses were able to wean. CONCLUSION: A standardized laxative weaning process can be successful in patients with functional constipation, especially on high doses. Further prospective studies will be necessary to confirm the success of this protocol. LEVEL OF EVIDENCE: III.


Assuntos
Protocolos Clínicos , Constipação Intestinal , Laxantes , Humanos , Constipação Intestinal/tratamento farmacológico , Projetos Piloto , Laxantes/uso terapêutico , Laxantes/administração & dosagem , Criança , Masculino , Feminino , Adolescente , Extrato de Senna/uso terapêutico , Extrato de Senna/administração & dosagem , Fibras na Dieta/administração & dosagem , Fibras na Dieta/uso terapêutico , Esquema de Medicação , Resultado do Tratamento
11.
J Pediatr Surg ; 59(8): 1638-1642, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38760309

RESUMO

PURPOSE: Antegrade colonic enemas (ACE) can be an effective management option for defecation disorders and improve quality of life. Best practice regarding channel placement is unclear and variation may exist around preferred initial type of channel, age at placement, and underlying diagnoses. We aimed to describe practice patterns and patient characteristics around ACE channel placement. METHODS: We conducted a multicenter retrospective study of children with an ACE channel cared for at sites participating in the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) from 2017 to 2022. Kruskal-Wallis test was utilized to test the age at surgery by site with significance level of 0.05. RESULTS: 500 patients with ACE channel were included. 293 (58.6%) patients had their ACE procedure at a PCPLC center. The median age at surgery was 7.6 [IQR 5.3-11.0] years for the overall cohort and 8.1 [IQR 5.3-11.5] years for placement at PCPLC centers. For PCPLC centers, median age at placement varied significantly across centers (p = 0.009). 371 (74.2%) patients received Malone appendicostomy, 116 (23.2%) received cecostomy, and 13 (2.6%) received Neo-Malone appendicostomy. Median age of patients by channel type was 7.7 [IQR 5.3-11.0], 7.5 [IQR 5.7-11.0], and 9.8 [IQR 4.2-11.6] years, respectively. The most common indication for cecostomy was idiopathic/refractory constipation (52.6%), whereas anorectal malformation was the most common indication for Malone (47.2%) and Neo-Malone (61.5%). Among ACE channels placed at PCPLC centers, there was variation across institutions in preferred initial channel type. The 4 highest volume centers favored Malone appendicostomy over cecostomy. CONCLUSION: There is variation in practice of ACE channel placement. At specialty pediatric colorectal centers, age at time of placement and type of channel placed varied across institutions. Further work is needed to better characterize diagnosis- and age-focused patient centered outcomes to clarify recommendations for our patients who benefit from these procedures. TYPE OF STUDY: Retrospective comparative study. LEVEL OF EVIDENCE: Level III.


Assuntos
Constipação Intestinal , Enema , Padrões de Prática Médica , Humanos , Estudos Retrospectivos , Enema/métodos , Criança , Feminino , Masculino , Pré-Escolar , Constipação Intestinal/terapia , Padrões de Prática Médica/estatística & dados numéricos , Cecostomia/métodos
12.
J Pediatr Surg ; 59(7): 1240-1244, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38584009

RESUMO

BACKGROUND: Hirschsprung-associated enterocolitis (HAEC) is the most common cause of morbidity and mortality in patients with Hirschsprung disease (HD). There is a correlation between social determinants of health (SDOH) and outcomes in children with HD. The Child Opportunity Index (COI) is a publicly available dataset that stratifies patients by address into levels of opportunity. We aimed to understand if a relationship exists between COI and HAEC. METHODS: A single-institution, IRB-approved, retrospective cohort study was performed of children with HD. Census tract information was used to obtain COI scores, which were stratified into categories (very low, low, medium, high, very high). Subgroups with and without history of HAEC were compared. RESULTS: The cohort had 100 patients, of which 93 had a COI score. There were 27 patients (29.0%) with HAEC. There were no differences in demographics or clinical factors, including length of aganglionic colon, operative approach, and age at pull-through. As child opportunity score increased from very low to very high, there was a statistically significant decrease in the incidence of HAEC (p = 0.04). CONCLUSION: We demonstrate a significant association between increasing opportunity and decreasing incidence of HAEC. This suggests an opportunity for targeted intervention in populations with low opportunity. LEVEL OF EVIDENCE: III. IRB NUMBER: IRB14-00232.


Assuntos
Enterocolite , Doença de Hirschsprung , Humanos , Doença de Hirschsprung/cirurgia , Doença de Hirschsprung/complicações , Estudos Retrospectivos , Enterocolite/epidemiologia , Enterocolite/etiologia , Masculino , Feminino , Lactente , Incidência , Pré-Escolar , Determinantes Sociais da Saúde , Recém-Nascido
13.
J Pediatr Surg ; 59(7): 1250-1255, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38677965

RESUMO

BACKGROUND: Hirschsprung Disease (HD) is a rare cause of functional bowel obstruction in children. Patients are typically diagnosed in the neonatal period and undergo pull-through (PT) soon after diagnosis. The optimal management and post-operative outcomes of children who present in a delayed fashion are unknown. METHODS: A multi-center retrospective review of children with HD was performed at participating Pediatric Colorectal and Pelvic Learning Consortium sites. Children were stratified by age at diagnosis (neonates <29 days; infants 29 days-12 months; toddler 1 year-5 years and child >5 years). RESULTS: 679 patients with HD from 14 sites were included; Most (69%) were diagnosed in the neonatal period. Age at diagnosis was not associated with differences in 30-day complication rates or need for PT revision. Older age at diagnosis was associated with a greater likelihood of undergoing fecal diversion after PT (neonate 10%, infant 12%, toddler 26%, child 28%, P < 0.001) and a greater need for intervention for constipation or incontinence postoperatively (neonate 56%, infant 62%, toddler 78%, child 69%, P < 0.001). CONCLUSION: Delayed diagnosis of HD does not impact 30-day post-operative outcomes or need for revision surgery but, delayed diagnosis is associated with increased need for fecal diversion after pull-through. LEVEL OF EVIDENCE: III.


Assuntos
Diagnóstico Tardio , Doença de Hirschsprung , Complicações Pós-Operatórias , Humanos , Doença de Hirschsprung/cirurgia , Doença de Hirschsprung/diagnóstico , Estudos Retrospectivos , Lactente , Diagnóstico Tardio/estatística & dados numéricos , Pré-Escolar , Feminino , Masculino , Recém-Nascido , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/diagnóstico , Resultado do Tratamento , Criança , Reoperação/estatística & dados numéricos
14.
J Pediatr Surg ; 59(5): 997-1002, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38365475

RESUMO

BACKGROUND: Anorectal malformations (ARM) are rare and heterogenous which creates a challenge in conducting research and offering recommendations for best practice. The Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) was formed in 2016 to address this challenge and created a shared national data registry to collect information about pediatric colorectal patients. There has been no external validation of the data collected. We sought to evaluate the database by performing a patient matched analysis comparing 30-day outcomes identified in the PCPLC registry with the NSQIP-P database for patients undergoing surgical repair of ARM. METHODS: Patients captured in the PCPLC database from 2016 to 2021 at institutions also participating in NSQIP-P who underwent ARM repair younger than 12 months old were reviewed for 30-day complications. These patients were matched to their NSQIP-P record using their hospital identification number, and records were compared for concordance in identified complications. RESULTS: A total of 591 patient records met inclusion criteria in the PCPLC database. Of these, 180 patients were also reviewed by NSQIP-P. One hundred and fifty-six patient records had no complications recorded. Twenty-four patient records had a complication listed in one or both databases. There was a 91 % concordance rate between databases. When excluding complications not tracked in the PCPLC registry, this agreement improved to 93 %. CONCLUSION: Including all patients evaluated for this subpopulation, a 91 % concordance rate was observed when comparing PCPLC collected complications to NSQIP-P. Future efforts can focus on further validating the data within the PCPLC for other patient populations. LEVEL OF EVIDENCE: V.


Assuntos
Malformações Anorretais , Neoplasias Colorretais , Humanos , Criança , Lactente , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Malformações Anorretais/cirurgia , Malformações Anorretais/complicações , Sistema de Registros , Bases de Dados Factuais , Neoplasias Colorretais/complicações , Melhoria de Qualidade , Estudos Retrospectivos
15.
Eur J Pediatr Surg ; 2024 Feb 12.
Artigo em Inglês | MEDLINE | ID: mdl-38262474

RESUMO

INTRODUCTION: Children with anorectal malformations (ARMs) benefit from bowel management programs (BMPs) to manage constipation or fecal incontinence. We aimed to understand the role of social determinants of health (SDOH) in outcomes following BMPs in this population. MATERIALS AND METHODS: A single-institution, institutional review board (IRB) approved, retrospective review was performed in children with ARM who underwent BMP from 2014 to 2021. Clinical, surgical, and SDOH data were collected. Children were stratified as clean or not clean per the Rome IV criteria at the completion of BMP. Descriptive statistics were computed. Categorical variables were analyzed via Fisher's exact tests and continuous variables with Mood's median tests. RESULTS: In total, 239 patients who underwent BMP were identified; their median age was 6.62 years (interquartile range [IQR]: 4.78-9.83). Of these, 81 (34%) were not clean after completing BMP. Children with prior history of antegrade enema procedures had a higher rate of failure. Children who held public insurance, lived within driving distance, had unmarried parents, lived with extended family, and lacked formal support systems had a significant association with BMP failure (p < 0.05 for all). Type of ARM, age at repair, type of repair, age at BMP, and type of BMP regimen were not significantly associated with failure. CONCLUSIONS: There is a significant correlation of failure of BMPs with several SDOH elements in patients with ARM. Attention to SDOH may help identify high-risk patients in whom additional care may lead improved outcomes following BMP.

16.
Arq. bras. endocrinol. metab ; 53(5): 688-694, jul. 2009. tab
Artigo em Inglês | LILACS | ID: lil-525430

RESUMO

Milk intake is widely recommended for a healthy diet. Recent evidences suggest that milk/dairy products are associated with a lower risk of type 2 diabetes and hypertension. On the other hand, high calcium intake has been associated with a higher risk of prostate cancer. The calcium and vitamin D content in dairy foods could have beneficial effects on glucose metabolism and renin/angiotensin system as well regulates body weight. The association between high dairy/calcium consumption and prostate cancer risk are related to the presence of estrogens and insulin like growth factor (IGF-I) in milk. Based on the current evidence, it is possible that milk/dairy products, when consumed in adequate amounts and mainly with reduced fat content, has a beneficial effect on the prevention of hypertension and diabetes. Its potential role in the pathogenesis of prostate cancer is not well supported and requires additional study.


A ingestão de leite é amplamente recomendada para uma dieta saudável. Evidências recentes sugerem que leite e produtos lácteos estão associados a menor risco de diabetes melito tipo 2 e hipertensão. Por outro lado, a ingestão elevada de cálcio foi associada a maior risco de câncer de próstata. A quantidade de cálcio e de vitamina D presentes em produtos lácteos tem efeito benéfico no metabolismo da glicose e no sistema renina-angiotensina, além de regular o peso corporal. A associação entre o consumo elevado de laticínios/cálcio e o risco de câncer de próstata está relacionada à presença de estrogênios e de fator de crescimento semelhante à insulina (IGF-I) no leite. Com base nas evidências atuais, é possível que o leite e os produtos lácteos, se consumidos em quantidades adequadas e, principalmente, com reduzido teor de gordura, tenham um efeito benéfico na prevenção da hipertensão e do diabetes. Sua função potencial na patogênese do câncer de próstata não é bem sustentada e requer estudos adicionais.


Assuntos
Animais , Humanos , Masculino , Cálcio da Dieta/administração & dosagem , /prevenção & controle , Hipertensão/prevenção & controle , Leite , Neoplasias da Próstata/etiologia , Inquéritos sobre Dietas , Medicina Baseada em Evidências , Fator de Crescimento Insulin-Like I/metabolismo , Leite/efeitos adversos , Leite/química , Neoplasias da Próstata/metabolismo , Fatores de Risco
17.
Washington, D.C; ASM Press; 2nd ed; 2006. 1118 p.
Monografia em Inglês | LILACS, Coleciona SUS | ID: biblio-941243

Assuntos
Masculino , Feminino , Humanos , DNA
18.
Washington, D.C; ASM Press; 2nd ed; 2006. 1118 p.
Monografia em Inglês | LILACS | ID: lil-760863

Assuntos
Humanos , Masculino , Feminino , DNA
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