achalasia

失语症
  • 文章类型: Journal Article
    背景:在过去的30年里,腹腔镜Heller肌切开术(LHM)代表了食管门失弛缓症的治疗选择,解决大多数患者的症状。对LHM或其治疗后复发的患者的命运知之甚少。在这项研究中,我们旨在评估LHM无效后的辅助气动扩张(CPD)的结果。
    方法:我们评估了1992年至2022年接受LHM伴Dor胃底折叠术(LHD)并因持续或复发症状而接受CPD治疗的患者。对患者进行了临床和测压随访,钡燕子,必要时进行内窥镜检查。使用>3的Eckardt评分(ES)作为失败的阈值。
    结果:在1420例LHD患者中,120(8.4%)被认为是失败的,并提供了CPD。10例患者拒绝进一步治疗;在5个CPD中,未显示严重食管炎;1例患者因胃底折叠畸形而手术,1例患者在LHD后2年发展为癌症;这使得103例患者接受了中位数2个CPD(IQR,1-3),中位数为15(IQR,手术后8-36)个月,带3.0至4.0厘米Rigiflex扩张器(波士顿科学,马萨诸塞州,美国)。没有记录到穿孔。只有6例患者失访。因此,97例随访,中位数为37个月(IQR,6-112)在最后一次CPD后:70(72%)无症状,而27例(28%)有显著的持续性吞咽困难(ES>3)。两组之间的唯一差异是手术后的ES(P<0.01)和所需的CPD数量。总的来说,LHD+CPD的组合在96.5%的患者中提供了令人满意的结果.
    结论:CPDs是治疗LHD失败后患者的一种有效和安全的选择:当术后ES持续保持较高且控制症状所需的CPDs数量超过2时,这可能表明需要进一步的侵入性治疗。
    BACKGROUND: In the last 3 decades, laparoscopic Heller myotomy (LHM) has represented the treatment of choice for esophageal achalasia, solving symptoms in most patients. Little is known about the fate of patients relapsing after LHM or their treatment. In this study, we aimed at evaluating the results of complementary pneumatic dilations (CPDs) after ineffective LHM.
    METHODS: We evaluated the patients who underwent LHM with Dor fundoplication (LHD) from 1992 to 2022 and were submitted to CPD for persistent or recurrent symptoms. The patients were followed clinically and with manometry, barium swallow, and endoscopy when necessary. An Eckardt score (ES) of > 3 was used as threshold for failure.
    RESULTS: Of 1420 patients undergoing LHD, 120 (8.4%) were considered failures and were offered CPD. Ten patients refused further treatment; in 5 CPD was not indicated for severe esophagitis; 1 patient had surgery for a misshaped fundoplication and 1 patient developed cancer 2 years after LHD; that leaves 103 patients who underwent a median 2 CPDs (IQR, 1-3), at a median of 15 (IQR, 8-36) months after surgery, with 3.0- to 4.0-cm Rigiflex dilator (Boston Scientific, Massachusetts, USA). No perforations were recorded. Only 6 patients were lost to follow-up. Thus, 97 were followed for a median of 37 months (IQR, 6-112) after the last CPD: 70 (72%) were asymptomatic, whereas 27 (28%) had significant persistent dysphagia (ES > 3). The only differences between the 2 groups were the ES after surgery (P < .01) and the number of required CPD. Overall, the combination of LHD + CPD provided a satisfactory outcome in 96.5% of the patients.
    CONCLUSIONS: CPDs represent an effective and safe option to treat patients after a failed LHD: when the postsurgery ES consistently remains high and the number of CPDs required to control symptoms exceeds 2, this may suggest the need for further invasive treatments.
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  • 文章类型: Journal Article
    贲门失弛缓症是一种罕见的食管运动障碍,其特征是食管下括约肌不松弛。腹腔镜Heller肌切开术(LHM)是贲门失弛缓症的金标准治疗方法。经口内镜肌切开术(POEM),一种侵入性较小的治疗方法,进行了广泛的表演,迄今为止,干预方法的选择仍有争议。除了对短期结果的广泛研究之外,最近关于LHM和POEM的长期结局的研究显示,经过5年的随访,其临床效果相似.然而,胃食管反流病(GERD)在接受POEM治疗的患者中比在接受LHM治疗的患者中更常见.此外,现有研究比较了各种疾病状态下的治疗结果.一些研究表明,对于III型门失弛缓症患者,POEM优于LHM,因为POEM允许更长的肌切开术。关于乙状结肠型的治疗研究目前正在进行中。然而,LHD和POEM的长期比较结果不足,最好的治疗方法仍然存在争议。需要进一步的研究,应与患者讨论治疗方案,并根据他们的个人需求和病理情况进行调整。
    Achalasia is a rare esophageal motility disorder characterized by nonrelaxation of the lower esophageal sphincter. Laparoscopic Heller myotomy (LHM) is the gold standard treatment for achalasia. Peroral endoscopic myotomy (POEM), a less invasive treatment, is performed extensively, and the selection of the intervention method remains debatable to date. In addition to the availability of extensive studies on short-term outcomes, recent studies on the long-term outcomes of LHM and POEM have shown similar clinical success after 5 y of follow-up. However, gastroesophageal reflux disease (GERD) was more common in patients who had undergone POEM than in those who had undergone LHM. Moreover, existing studies have compared treatment outcomes in various disease states. Some studies have suggested that POEM is superior to LHM for patients with type III achalasia because POEM allows for a longer myotomy. Research on treatment for sigmoid types is currently in progress. However, the long-term results comparing LHD and POEM are insufficient, and the best treatment remains controversial. Further research is needed, and treatment options should be discussed with patients and tailored to their individual needs and pathologies.
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  • 文章类型: Case Reports
    贲门失弛缓症是一种影响食管运动的罕见疾病。偶尔,经胸超声心动图观察时,贲门失弛缓症食管扩张的外观可能类似于心外肿瘤。由于广泛的食管扩张引起的左心房压迫也很少见,可能导致血液动力学受损。这里,我们介绍了一例罕见的病例,涉及贲门失弛缓症食管扩张引起的左心房压迫,超声心动图检查结果模仿心外肿瘤。
    Achalasia is an uncommon disorder affecting esophageal motility. Occasionally, the appearance of a dilated esophagus in achalasia may resemble an extracardiac tumor when observed through transthoracic echocardiography. Left atrial compression due to extensive esophageal dilation is also rare, potentially leading to hemodynamic compromise. Here, we present a rare case involving left atrial compression caused by esophageal dilation in achalasia, with echocardiographic findings mimicking those of an extracardiac tumor.
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  • 文章类型: Journal Article
    背景:功能腔成像探针(FLIP)扫描法评估食管运动对受控容积扩张的反应。这项研究旨在描述食管下括约肌(LES)的生理力学状态,以响应食管运动障碍的连续填充/排空方案。
    方法:45名患者在FLIP上没有收缩反应,并且诊断为正常运动(n=6),无效食管运动(IEM;n=8),硬皮病(SSc;n=10),包括非痉挛性门失弛缓症(n=21),与20名患者对照一样,在FLIP和测压法上运动正常。在60mL逐步填充FLIP后测量LES直径和压力,70毫升,并排空至60mL,并进行相对变化以定义生理机械状态。
    结果:在所有诊断中有63/65(97%)的患者发生FLIP填充后被动扩张。翻转清空后,被动缩短发生在12/14(86%)正常运动/IEM,10/10(100%)SSc,9/21(43%)贲门失弛缓症,和16/20(80%)控制,在2/14(14%)正常运动性/IEM中观察到营养全张松弛,12/21(57%)贲门失弛缓症,和4/20(20%)控制。门失弛缓症治疗后(LES肌切开术),21/21(100%)的贲门失弛缓症在FLIP排空后出现被动缩短。
    结论:LES的生理机械状态可以通过对FLIP填充和排空方案的响应来确定。虽然被动缩短是对FLIP排空的一般反应,在贲门失弛缓中观察到营养全张松弛,被LES肌切开术打断。需要进一步研究对食管运动障碍的诊断和治疗的临床影响。
    BACKGROUND: Functional lumen imaging probe (FLIP) panometry assesses esophageal motility in response to controlled volumetric distension. This study aimed to describe the physiomechanical states of the lower esophageal sphincter (LES) in response to serial filling/emptying regimes for esophageal motility disorders.
    METHODS: Fourty-five patients with absent contractile response on FLIP and diagnoses of normal motility (n = 6), ineffective esophageal motility (IEM; n = 8), scleroderma (SSc; n = 10), or nonspastic achalasia (n = 21) were included, as were 20 patient controls with normal motility on FLIP and manometry. LES diameter and pressure were measured after stepwise FLIP filling at 60 mL, 70 mL, and emptying to 60 mL with relative changes used to define physiomechanical states.
    RESULTS: Passive dilatation after FLIP filling occurred in 63/65 (97%) patients among all diagnoses. After FLIP emptying, passive shortening occurred in 12/14 (86%) normal motility/IEM, 10/10 (100%) SSc, 9/21(43%) achalasia, and 16/20 (80%) controls, with auxotonic relaxation seen in 2/14 (14%) normal motility/IEM, 12/21 (57%) achalasia, and 4/20 (20%) controls. After achalasia treatment (LES myotomy), 21/21 (100%) achalasia had passive shortening after FLIP emptying.
    CONCLUSIONS: Physiomechanical states of the LES can be determined via response to FLIP filling and emptying regimes. While passive shortening was the general response to FLIP emptying, auxotonic relaxation was observed in achalasia, which was disrupted by LES myotomy. Further investigation is warranted into the clinical impact on diagnosis and treatment of esophageal motility disorders.
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  • 文章类型: Journal Article
    经口内镜肌切开术(POEM)是一种已建立的贲门失弛缓症治疗方法,然而,仍然缺乏技术标准化。没有明确的\'长\'定义,\'标准\',或\'短\'POEM存在到目前为止。我们用荟萃分析进行了系统评价,以分析当前的POEM长度标准。我们包括报告POEM技术细节的研究,其中未有意采用明确或比较的肌切开术长度(标准肌切开术)。主要结果是合并平均总肌切开术长度。进行亚组分析以探索研究之间的异质性。从最初的7172条记录中,纳入了31项研究,包括3023例患者。总肌切开术长度的汇总平均值为10.39cm(95%CI10.06-10.71;I299.3%)。食管和胃肌切开术长度的汇总平均值,由17项研究提供,分别为7.11厘米(95%CI6.51-7.71;I299.8%)和2.81厘米(95%CI2.41-3-22;I299.8%),分别。关于贲门失弛缓亚型的亚组分析,非痉挛性门失弛缓症(I型和II型)的合并平均长度为10.17cm(95%CI9.91-10.43;I294.2%),而在III型中,它是14.02cm(95%CI10.59-17.44;I298.9%)。2014-2020年期间进行的研究的合并平均肌切开术长度为10.53cm(95%CI,10.22-10.84;I299.1%)和2021-2022年的9.74cm(95%CI,7.95-11.54;I299.7%)。标准POEM期间的肌切开术长度为10.4厘米,在非痉挛性贲门失弛缓症剩余超过10厘米。跨研究的高度异质性证实了POEM技术需要进一步标准化。我们没有发现采用短POEM的显著时间趋势,尽管最近有证据支持它的使用。
    Peroral endoscopic myotomy (POEM) is an established treatment for achalasia, yet there is still a lack of technical standardization. No clear definition of \'long\', \'standard\', or \'short\' POEM exists to date. We conducted a systematic review with meta-analysis to analyze current POEM length standards. We included studies reporting technical details of POEM, in which no definite or comparative myotomy length was intentionally adopted (standard myotomy). The primary outcome was the pooled mean total myotomy length. Sub-group analyses were performed to explore heterogeneity across studies. From the initial 7172 records, 31 studies with 3023 patients were included. Pooled mean of total myotomy length was 10.39 cm (95% CI 10.06-10.71; I2 99.3%). Pooled mean of esophageal and gastric myotomy length, provided by 17 studies, was 7.11 cm (95% CI 6.51-7.71; I2 99.8%) and 2.81 cm (95% CI 2.41-3-22; I2 99.8%), respectively. On subgroup analysis for achalasia subtypes, pooled mean length in non-spastic achalasia (type I and II) was 10.17 cm (95% CI 9.91-10.43; I2 94.2%), while in type III it was 14.02 cm (95% CI 10.59-17.44; I2 98.9%). Pooled mean myotomy length for studies conducted between 2014-2020 was 10.53 cm (95% CI, 10.22-10.84; I2 99.1%) and 9.74 cm (95% CI, 7.95-11.54; I2 99.7%) in 2021-2022. Myotomy length during a \'standard\' POEM is 10.4 cm, remaining over 10 cm in non-spastic achalasia. The high heterogeneity across studies confirms that the POEM technique needs further standardization. We found no significant time trend towards adopting short POEM, despite recent evidence supporting its use.
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  • 文章类型: Journal Article
    腹胀症,以食管下括约肌(LES)松弛受损和蠕动失败为特征,突出是最广泛认可的原发性食管运动障碍。它表现为吞咽困难,固体和液体食物,胸痛,返流,和减肥,导致严重的发病率和医疗负担。传统上,手术Heller肌切开术和充气扩张术是贲门失弛缓症的主要治疗方法。然而,2009年,Inoue和他的同事介绍了一种开创性的内镜技术,称为经口内镜肌切开术(POEM),彻底改变了这种状况的管理。这篇综述旨在全面研究POEM技术在诊断为门失弛缓症患者中的最新进展。深入研究关键方面,比如肌肉切开术的剪裁,预防术中不良事件(AE),对长期结果的评估,以及在治疗失败的情况下再治疗的可行性。
    Achalasia, characterized by impaired lower esophageal sphincter (LES) relaxation and failed peristalsis, stands out as the most widely recognized primary esophageal motility disorder. It manifests with dysphagia to solid and liquid foods, chest pain, regurgitation, and weight loss, leading to significant morbidity and healthcare burden. Traditionally, surgical Heller myotomy and pneumatic dilation were the primary therapeutic approaches for achalasia. However, in 2009, Inoue and colleagues introduced a groundbreaking endoscopic technique called peroral endoscopic myotomy (POEM), revolutionizing the management of this condition. This review aims to comprehensively examine the recent advancements in the POEM technique for patients diagnosed with achalasia, delving into critical aspects, such as the tailoring of the myotomy, the prevention of intraprocedural adverse events (AEs), the evaluation of long-term outcomes, and the feasibility of retreatment in cases of therapeutic failure.
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  • 文章类型: Journal Article
    经口内镜肌切开术(POEM)后的反流可以说是与该手术相关的最大问题之一。确切的发病率很难确定为反流症状,食管炎,和异常酸暴露相关性差,保证进行彻底的诊断调查。发病率是,然而,在所有这三个参数中,高于海勒肌切开术或气动扩张术后。尽管PPI对食管炎的症状和愈合有效,难治性患者存在。食管超敏反应和酸性发酵/食管淤滞是最可能的原因,可以通过手动分析pH测量轨迹来诊断。消化性狭窄和Barrett食管等长期并发症很少见,偶尔有报道。旨在减少POEM后回流的POEM程序的修改导致没有结论性的首选技术。诸如腔内功能性管腔成像探针之类的现代研究可能有助于将肌切开术个性化为食管下括约肌的所需扩张性并减少反流。
    Reflux after peroral endoscopic myotomy (POEM) is arguably one of the greatest concerns related to the procedure. The exact incidence is difficult to establish as reflux symptoms, esophagitis, and abnormal acid exposure correlate poorly, warranting thorough diagnostic investigation. The incidence is, however, higher than after Heller myotomy or pneumatic dilatation across all these three parameters. Although PPI are effective in the resolution of symptoms and healing of esophagitis, refractory patients exist. Esophageal hypersensitivity and acidic fermentation/esophageal stasis are most likely causes and could be diagnosed by manual analysis of pH metry tracings. Long-term complications like peptic stricture and Barrett\'s esophagus are rare and reported sporadically. Modifications of POEM procedure aiming to decrease post-POEM reflux led to no conclusive preferred technique. Modern investigations like endoluminal functional lumen imaging probe might help to personalize myotomy to the desired distensibility of the lower esophageal sphincter and decrease reflux.
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  • 文章类型: Journal Article
    非贲门失弛缓症食管运动障碍(NAOMD)代表一组异质性的罕见疾病,包括食管胃结合部流出道梗阻,远端食管痉挛,和食管过度收缩。尽管病因不同,测压和病理生理特征,这些疾病由相似的临床表现统一,包括吞咽困难和胸痛.这些疾病的管理仍然是临床医生的挑战。药物治疗,肉毒杆菌毒素注射,内窥镜扩张术,采用了腹腔镜Heller肌切开术,在大多数患者中疗效有限。目前,文献中没有对照研究表明哪一种是这些疾病的最佳治疗方法.自从引入临床实践以来,经口内镜肌切开术(POEM)已经成为一种非常有前途的,食管贲门失弛缓症的微创有效治疗.不再在第一次使用后,POEM也已成功用于选定的NAOMD患者的管理,然而,目前可用的数据受到研究样本量小和短期随访的限制.
    Non-achalasia oesophageal motility disorders (NAOMD) represent a heterogeneous group of rare diseases, including oesophagogastric junction outflow obstruction, distal oesophageal spasm, and hypercontractile oesophagus. Despite the differing aetiological, manometric and pathophysiological characteristics, these disorders are unified by similar clinical presentation, including dysphagia and chest pain. The management of these disorders remain a challenge for the clinician. Pharmacotherapy, botulinum toxin injection, endoscopic dilation, and laparoscopic Heller myotomy have been employed, with limited efficacy in the majority of patients. Currently, there are no controlled studies in literature that suggest which is the best management of these diseases. Since its introduction in clinical practice, PerOral Endoscopic Myotomy (POEM) has emerged as a very promising, minimally invasive and effective treatment for oesophageal achalasia. No longer after the first uses, POEM has been successfully used also for the management of selected patients with NAOMD, However, currently available data are limited by small study sample sizes and short-term follow-up.
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  • 文章类型: Journal Article
    背景:腔内功能性管腔成像探头(EndoFLIP)是一种测量胃食管交界处(GEJ)扩张性的装置。然而,在经口内镜肌切开术(POEM)中,没有发现GEJ扩张性随胃肌切开术长度的变化而增加.本研究旨在探讨POEM胃肌切开术长度与术中EndoFLIP结果之间的关系。
    方法:这种单中心,回顾性队列研究纳入了2019年12月至2023年1月期间接受POEM术中EndoFLIP治疗的患者.使用EndoFLIP,在肌切开术前后测量最小球囊直径及其扩张指数(DI).主要和次要结果是30ml和40ml体积填充的肌切开术后EndoFLIP发现。
    结果:该研究包括44名患者(平均年龄53.1岁,50%女性)。芝加哥分类包括I型门失弛缓症(39%),II(41%),III(9%),食管过度收缩(2%),和EGJOO(9%)。平均食管肌切开术长度为7.5±2.2cm,胃肌切开术为2.1±0.6cm。简单的线性回归分析表明,胃肌切开术长度每增加1厘米,在30毫升体积填充时,DI估计增加2.0mm2/mmHg(p<0.05,R2=0.41),30毫升体积填充时的最小直径估计增加2.4毫米(p<0.05,R2=0.48),并且在40ml体积填充时的最小直径估计增加1.3mm(p<0.05,R2=0.09)。
    结论:这项研究表明,在POEM过程中,胃肌切开术长度与EndoFLIP测量的GEJ扩张性之间存在显著的线性关系。通过使EndoFLIP能够帮助校准所需的胃肌切开术长度以实现最佳DI和最小直径,这些发现可能在临床实践中很有用。
    BACKGROUND: Endoluminal Functional Lumen Imaging Probe (EndoFLIP) is a device that measures gastro-esophageal junction (GEJ) distensibility. However, it is not demonstrated that GEJ distensibility increases proportionally with varying gastric myotomy length in peroral endoscopic myotomy (POEM). This study aimed to investigate the association between gastric myotomy length in POEM and intraoperative EndoFLIP findings.
    METHODS: This single-center, retrospective cohort study included patients who underwent POEM with intraoperative EndoFLIP from December 2019 to January 2023. Using EndoFLIP, minimal balloon diameter and its distensibility index (DI) were measured pre- and post-myotomy. Primary and secondary outcomes were the post-myotomy EndoFLIP findings at 30 ml and 40 ml volume fills.
    RESULTS: The study included 44 patients (mean age 53.1 years, 50% female). Chicago classification included achalasia type I (39%), II (41%), III (9%), hypercontractile esophagus (2%), and EGJOO (9%). The mean esophageal myotomy length was 7.5 ± 2.2 cm and gastric myotomy was 2.1 ± 0.6 cm. Simple linear regression analyses indicated that for each 1 cm increase in gastric myotomy length, the DI at 30 ml volume fill was estimated to increase by 2.0 mm2/mmHg (p < 0.05, R2 = 0.41), the minimal diameter at 30 ml volume fill was estimated to increase by 2.4 mm (p < 0.05, R2 = 0.48), and the minimal diameter at 40 ml volume fill was estimated to increase by 1.3 mm (p < 0.05, R2 = 0.09).
    CONCLUSIONS: This study demonstrates a significant linear relationship between gastric myotomy length and GEJ distensibility measured by EndoFLIP during POEM. These findings may be useful in clinical practice by enabling EndoFLIP to help calibrate a desired gastric myotomy length to achieve optimal DI and minimal diameter.
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  • 文章类型: Journal Article
    目的:贲门失弛缓症,食道的原发性运动障碍,造成严重的营养不良风险。本研究旨在全面评估未经治疗的门失弛缓症患者的营养状况,将其与功能性胃肠病(FGIDs)病例进行比较,并在一年内进行经口内镜肌切开术(POEM)对营养的影响。
    方法:我们进行了一项前瞻性研究,包括连续的贲门失弛缓症,从2021年12月到2022年4月在三级护理中心。生化参数,人体测量学,使用主观整体评估(SGA)和营养不良通用筛查工具进行营养评估.诊断为FGIDs的病例作为对照。
    结果:多达118例(41.2±13.9年,61%的男性)患有贲门失弛缓症和200名对照(43.4±11.9岁,69%的男性)被纳入研究。贲门失弛缓症的亚型包括I型(16.9%),II(76.3%)和III(6.8%)。总的来说,中,重度营养不良占38.1%和6.8%,分别。与对照组相比,贲门失弛缓症患者的前白蛋白较低(19.4vs.25.2;p=0.001),血清钙(p=0.012),维生素D(p=0.001),血清铁(p=0.001),三头肌褶皱厚度(p=0.002)和握力(p=0.001)。在单变量分析中,I型贲门失弛缓症,身体质量指数,%重量损失,食管下括约肌压和Eckardt评分是营养不良(SGA)的预测因子.在多变量分析中,贲门失弛缓症的类型,中臂围和低体重指数是贲门失弛缓症患者营养不良的重要预测因素。在一年的随访中,POEM后营养状况显着改善。
    结论:贲门失弛缓症患者与FGIDs患者相比,营养不良的风险明显更高。POEM后营养状况明显改善。(NCT05161923)。
    OBJECTIVE: Achalasia cardia, a primary motility disorder of the esophagus, poses significant malnutrition risks. This study aims at comprehensively assessing the nutritional status in untreated achalasia patients, contrasting it with functional gastrointestinal disorders (FGIDs) cases and impact of per-oral endoscopic myotomy (POEM) on nutrition at one-year.
    METHODS: We conducted a prospective study, including consecutive achalasia cases, from December 2021 to April 2022 at a tertiary care centre. Biochemical parameters, anthropometry, subjective global assessment (SGA) and malnutrition universal screening tool were used for nutritional assessment. Cases diagnosed with FGIDs served as controls.
    RESULTS: As many as 118 cases (41.2 ± 13.9 years, 61% males) with achalasia and 200 controls (43.4 ± 11.9 years, 69% males) were included in the study. Sub-types of achalasia included type I (16.9%), II (76.3%) and III (6.8%). Overall, 38.1% and 6.8% cases were moderately and severely malnourished, respectively. As compared to controls, cases with achalasia had lower pre-albumin (19.4 vs. 25.2; p = 0.001), serum calcium (p = 0.012), vitamin D (p = 0.001), serum iron (p = 0.001), triceps fold thickness (p = 0.002) and hand-grip strength (p = 0.001). On univariate analysis, type-I achalasia, body mass index, % weight loss, lower esophageal sphincter pressures and Eckardt scores were predictors of malnourishment (SGA). On multivariate analysis, type of achalasia, mid arm circumference and low body mass index were significant predictors of malnourishment in cases with achalasia. There was significant improvement in the nutritional status after POEM at one-year follow-up.
    CONCLUSIONS: Achalasia patients demonstrate a notably higher risk of malnutrition compared to individuals with FGIDs. Nutritional status significantly improves after POEM. (NCT05161923).
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