Outcomes in patients with Hirschsprung disease following definitive surgery.
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ABSTRACT:
SUBMITTER: Gunadi
PROVIDER: S-EPMC6123951 | biostudies-literature | 2018 Sep
REPOSITORIES: biostudies-literature
Publications
Outcomes in patients with Hirschsprung disease following definitive surgery.
Gunadi Karina Stefani Melisa SM Dwihantoro Andi A
BMC research notes 20180904 1
<h4>Objective</h4>Several pull-through procedures have been described for Hirschsprung disease (HSCR) with varying outcomes. We aimed to describe the outcomes in HSCR patients < 18 year of age who underwent surgical procedures at Dr. Sardjito Hospital, Yogyakarta, Indonesia from January 2013 to December 2014.<h4>Results</h4>We utilized 67 HSCR patients, of whom 49 (73%) were males and 18 (27%) females. Neonatal presentation was seen in 57 cases (85%) and most patients (98.5%) had short-segment H ...[more]
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Project description:BackgroundThe growth of children with Hirschsprung disease (HSCR) can be affected by many factors, including the environment, nutrient intake, and surgery. Our study compared the long-term (i.e., at least 3 years of follow-up) growth outcomes in HSCR children after transabdominal Soave and Duhamel and transanal endorectal pull-through (TEPT) surgeries.MethodsA cross-sectional study was conducted in children <18 years of age diagnosed histopathologically with HSCR who underwent pull-through between January 1, 2012-December 31, 2015 in our institution. The postoperative anthropometric data were obtained prospectively through interviews during the outpatient clinic appointment or by telephone.ResultsWe recruited 21 patients (Soave: 7 vs. Duhamel: 4 vs. TEPT: 10; p = 0.06). There were no significant differences between the three surgical methods in terms of preoperative and postoperative nutritional status categories (p = 0.52). Concerning the changes in nutritional status, after Soave surgery, it was improved, steady, and worsened in 28.6%, 57.1%, and 14.3% of the children, respectively. The nutritional status of the Duhamel group was worsened and steady in 25% and 75% of the children, respectively, while in the TEPT group, it was improved and steady in 40% and 60% of the children, respectively. However, these differences were not statistically significant (p = 0.42).ConclusionsWhile some HSCR children show an improvement in their nutritional status after Soave and TEPT procedures, the overall nutritional status is similar among different procedures. Further multicenter studies with a larger sample size are important to clarify our findings.
| S-EPMC7554195 | biostudies-literature
Project description:to determine the efficacy of 3 periodontal treatments used in the treatment of chronic periodontitis
| PRJEB10230 | ENA
Project description:ObjectivesKnowledge on long-term outcomes in patients with Hirschsprung disease is progressing. Nevertheless, differences in outcomes according to aganglionic lengths are unclear. We compared long-term bowel function and generic quality of life in Hirschsprung patients with total colonic or long-segment versus rectosigmoid aganglionosis.MethodsIn this nationwide, cross-sectional study participants with proven Hirschsprung disease received the Defecation and Fecal Continence questionnaire, and the Child Health Questionnaire Child Form-87, or the WHO Quality of Life-100. We excluded deceased patients, patients who were younger than 8 years, lived abroad, had a permanent enterostomy, or were intellectually impaired.ResultsThe study population (n = 334) was operated for rectosigmoid (83.9%), long-segment (8.7%), or total colonic aganglionosis (7.5%). Fecal incontinence in general was not significantly different between the three groups, but liquid fecal incontinence was significantly associated with total colonic aganglionosis (odds ratio [OR] = 6.00, 95% confidence interval [CI] 2.07-17.38, P = 0.001). Regarding constipation, patients with total colonic or long-segment aganglionosis were less likely to suffer from constipation than the rectosigmoid group (OR = 0.21, 95% CI, 0.05-0.91, P = 0.038 and OR = 0.11, 95% CI, 0.01-0.83, P = 0.032). Quality of life was comparable between the three groups, except for a lower physical score in children with total colonic aganglionosis (P = 0.016).ConclusionsOver time Hirschsprung patients with total colonic or long-segment aganglionosis do not suffer from worse fecal incontinence in general. A difference in stool consistency may underlie the association between liquid fecal incontinence and total colonic aganglionosis and constipation in patients with rectosigmoid aganglionosis. Despite these differences, generic quality of life is comparable on reaching adulthood.
| S-EPMC8860201 | biostudies-literature
Project description:Several pull-through procedures have been described for Hirschsprung disease (HSCR) with varying functional outcomes. The voluntary bowel movement (VBM) and the absence of soiling or constipation after pull-through remain the most important markers of good outcome. We aimed to compare the functional outcomes in HSCR patients following Soave and Duhamel procedures.Krickenbeck classification was utilized to determine VBM, soiling and constipation for patients who underwent Soave and Duhamel pull-through at Dr. Sardjito Hospital, Indonesia from 2013 to 2016.Fifty-three patients were ascertained (Soave: 23 males and 2 females vs. Duhamel: 22 males and 6 females, p =?0.26). Ninety-three and 88% patients had a VBM following Duhamel and Soave pull-through, respectively (p =?0.66). Constipation frequency was significantly higher in Soave than Duhamel groups (24% vs. 4%; p =?0.04) with OR of 8.5 (95% CI?=?1.0-76.7), whereas soiling rate was similar between Duhamel (21%) and Soave (8%) groups (p =?0.26). Furthermore, the risk of constipation was increased ~?21.7-fold in female patients after Soave procedure and was almost statistically significant (p =?0.05).The constipation rate is higher in patients who underwent Soave than Duhamel procedure, but the VBM and soiling frequencies are similar. The constipation risk following Soave pull-through might be increased by the female gender. Furthermore, a multicenter study with a larger sample of patients is necessary to clarify and confirm our findings.
| S-EPMC5923197 | biostudies-other
Project description:BackgroundAlthough most patients with Hirschsprung disease (HSCR) improve after pull-through, some patients still have persistent obstructive symptoms. Most previous studies reported persistent obstructive symptoms after pull-through in HSCR patients from developed countries. Our study determined the prognostic factors of persistent obstructive symptoms in patients with HSCR following pull-through from a particular developing country.MethodsA cross-sectional study was conducted using medical records of patients with HSCR at our institution from January 2017 to January 2022.ResultsWe ascertained 114 patients with HSCR: 79 males and 35 females. Most of them (90.4%) showed a short aganglionosis and underwent transanal endorectal pull-through (55.3%). Twenty-two percent of patients showed persistent obstructive symptoms following pull-through. Operative technique and age at definitive surgery were significantly associated with the persistent obstructive symptoms after pull-through (p = 0.011 and 0.019, respectively), while sex, aganglionic segment length, presence of global developmental delay, and Down syndrome were not (p = 0.873, 0.525, 0.647, and 0.301, respectively). Multivariate analysis revealed that age at pull-through was a significant independent factor for persistent obstructive symptoms after pull-through, with an odds ratio of 3.41 (95% CI = 1.18-9.91; p = 0.02).ConclusionsOur study shows a moderate frequency of persistent obstructive symptoms after pull-through in our institution. In addition, patients who underwent pull-throughs at a younger age might have persistent obstructive symptoms following a definitive surgery. Our study provides new data on persistent obstructive symptoms after pull-through from a particular population that might be beneficial for pediatric surgeons' consideration before performing definitive surgery on patients with HSCR.
| S-EPMC10490837 | biostudies-literature
Project description:PurposeExisting definitions of high-risk prostate cancer consist of men who experience significant heterogeneity in outcomes. As such, criteria that identify a subpopulation of National Comprehensive Cancer Network (NCCN) high-risk prostate cancer patients who are at very high risk (VHR) for poor survival outcomes following prostatectomy were recently developed at our institution and include the presence of any of the following disease characteristics: multiple NCCN high-risk factors, primary Gleason pattern 5 disease and/or ≥5 biopsy cores with Gleason sums of 8 to 10. Whether these criteria also apply to men undergoing definitive radiation is unclear, as is the optimal treatment regimen in these patients.Methods and materialsAll men consecutively treated with definitive radiation by a single provider from 1993 to 2006 and who fulfilled criteria for NCCN high-risk disease were identified (n=288), including 99 patients (34%) with VHR disease. Multivariate-adjusted competing risk regression models were constructed to assess associations between the VHR definition and biochemical failure (BF), distant metastasis (DM), and prostate cancer-specific mortality (PCSM). Multivariate-adjusted Cox regression analysis assessed the association of the VHR definition with overall mortality (OM). Cumulative incidences of failure endpoints were compared between VHR men and other NCCN high-risk men.ResultsMen with VHR disease compared to other NCCN high-risk men experienced a higher 10-year incidence of BF (54.0% vs 35.4%, respectively, P<.001), DM (34.9% vs 13.4%, respectively, P<.001), PCSM (18.5% vs 5.9%, respectively, P<.001), and OM (36.4% vs 27.0%, respectively, P=.04). VHR men with a detectable prostate-specific antigen (PSA) concentration at the end of radiation (EOR) remained at high risk of 10-year PCSM compared to VHR men with an undetectable EOR PSA (31.0% vs 13.7%, respectively, P=.05).ConclusionsNCCN high-risk prostate cancer patients who meet VHR criteria experience distinctly worse outcomes following definitive radiation and long-term androgen deprivation therapy, particularly if an EOR PSA is detectable. Optimal use of local therapies for VHR patients should be explored further, as should novel agents.
| S-EPMC5065713 | biostudies-literature
Project description:BACKGROUND:Recently, pathogenic alleles within ubiquitin N-recognin domain-containing E3 ligase 4 (UBR4) gene have been shown to be associated with Hirschsprung disease (HSCR). We determined the UBR4 expressions in Indonesian HSCR patients. METHODS:We analyzed the UBR4 expressions in the colons of HSCR patient and anorectal malformation (ARM) patient as control by real-time polymerase chain reaction (qPCR). RESULTS:Thirty-seven patients with non-syndromic HSCR and eighteen controls were involved in this study. qPCR revealed that the UBR4 expression was strongly decreased (0.77-fold) in the ganglionic group of patients with HSCR compared to the control group with ARM (?CT 2.43?±?0.36 vs. 2.05?±?0.69; p?=?0.009), whereas the UBR4 expression was also significantly reduced (0.79-fold) in the aganglionic group of patients with HSCR compared to the control group with ARM (?CT 2.39?±?0.46 vs. 2.05?±?0.69; p?=?0.044). However, the UBR4 expression change was not associated with gender (p?=?0.35 and 0.80), nor with degree of aganglionosis both in ganglionic and aganglionic colons (p?=?0.72 and 0.73), respectively. CONCLUSION:Our study demonstrates that expression of UBR4 is decreased in both aganglionic and ganglionic colon of HSCR patients.
| S-EPMC6907357 | biostudies-literature
Project description:ObjectiveFew studies have assessed the outcomes of mitral valve surgery in patients with obesity. We sought to study factors that determine the in-hospital outcomes of this population to help clinicians provide optimal care.MethodsA retrospective analysis of adult patients with obesity who underwent open mitral valve replacement or repair between January 1, 2012, and December 31, 2020, was conducted using the National Inpatient Sample. Weighted logistic regression and random forest analyses were performed to assess factors associated with mortality and the interaction of each variable.ResultsOf the 48,775 patients with obesity, 34% had morbid obesity (body mass index ≥40), 55% were women, 66% underwent elective surgery, and 55% received isolated open mitral valve replacement or repair. In-hospital mortality was 5.0% (n = 2430). After adjusting for important covariates, a greater risk of mortality was associated with older patients (adjusted odds ratio [aOR], 1.24; 95% CI, 1.08-1.43), higher Elixhauser comorbidity score (aOR, 2.10; 95% CI, 1.87-2.36), prior valve surgery (aOR, 1.63; 95% CI, 1.01-2.63), and more than 2 concomitant procedures (aOR, 2.83; 95% CI, 2.07-3.85). Lower mortality was associated with elective admissions (aOR, 0.70; 95% CI, 0.56-0.87) and valve repair (aOR, 0.58; 95% CI, 0.46-0.73). Machine learning identified several interactions associated with early mortality, such as Elixhauser score, female sex, body mass index ≥40, and kidney failure.ConclusionsThe complexity of presentation, comorbidities in older and female patients, and morbid obesity are independently associated with an increased risk of mortality in patients undergoing open mitral valve replacement or repair. Morbid obesity and sex disparity should be recognized in this population, and physicians should consider older patients and females with multiple comorbidities for earlier and more opportune treatment windows.
| S-EPMC10556846 | biostudies-literature
Project description:BACKGROUND:Hirschsprung disease (HSCR) is a heterogeneous genetic disorder characterized by absence of ganglion cells along the intestines resulting in functional bowel obstruction. Mutations in neuregulin 1 (NRG1) gene have been implicated in some cases of intestinal aganglionosis. This study aims to investigate the contribution of the NRG1 gene to HSCR development in an Indonesian population. METHODS:We analyzed the entire coding region of the NRG1 gene in 54 histopathologically diagnosed HSCR patients. RESULTS:All patients were sporadic non-syndromic HSCR with 53/54 (98%) short-segment and 1/54 (2%) long-segment patients. NRG1 gene analysis identified one rare variant, c.397G?>?C (p.V133 L), and three common variants, rs7834206, rs3735774, and rs75155858. The p.V133 L variant was predicted to reside within a region of high mammalian conservation, overlapping with the promoter and enhancer histone marks of relevant tissues such as digestive and smooth muscle tissues and potentially altering the AP-4_2, BDP1_disc3, Egr-1_known1, Egr-1_known4, HEN1_2 transcription factor binding motifs. This p.V133 L variant was absent in 92 non-HSCR controls. Furthermore, the rs7834206 polymorphism was associated with HSCR by case-control analysis (p =?0.037). CONCLUSIONS:This study is the first report of a NRG1 rare variant associated with HSCR patients of South-East Asian ancestry and provides further insights into the contribution of NRG1 in the molecular genetic pathogenesis of HSCR.
| S-EPMC6123906 | biostudies-literature
Project description:PurposeTo present a case series of cataract surgery outcomes in choroideremia eyes with an emphasis on the safety of this common operation in advanced stages of the disease.MethodsA single centre retrospective interventional case series comprising six patients with varying degrees of visual loss secondary to choroideremia underwent cataract surgery at a single tertiary eye hospital. Pre- and post-operative best-corrected Snellen visual acuity, spectral domain optical coherence tomography (SD-OCT), and slit lamp examination were performed together with fundus autofluorescence (FAF) and colour fundus photographs.The prevalence of intra- or post-operative complications, post-operative visual outcome, and change in central macular thickness were recorded.ResultsThe pre-operative best-corrected Snellen visual acuity in the operated eyes ranged from 6/12 (20/40) to PL. All but one patient had either an objective or a subjective improvement in visual acuity. There was no evidence of retinal phototoxicity or post-operative cystoid macular oedema (CMO). Three patients developed early capsular fibrosis.ConclusionsAlthough the residual functioning retina in choroideremia patients may be potentially vulnerable, this report finds no evidence of iatrogenic vision loss after uncomplicated cataract surgery. This suggests that cataract surgery may be performed safely in choroideremia patients, although a guarded prognosis for visual improvement should be emphasized in the informed consent.
| S-EPMC4816357 | biostudies-literature