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Lymphatic interventions in congenital heart disease 先天性心脏病的淋巴管介入治疗
IF 1.7 3区 医学 Q3 PEDIATRICS Pub Date : 2024-06-01 DOI: 10.1016/j.sempedsurg.2024.151419
Christopher L. Smith , Ganesh Krishnamurthy , Abhay Srinivasan , Yoav Dori

Congenital heart disease affects 1/100 live births and is one of the most common congenital abnormalities. The relationship between congenital heart disease and lymphatic abnormalities and/or dysfunction is well documented and can be grossly divided into syndromic and non-syndromic etiologies. In patients with genetic syndromes (as examples listed above), there are known primary abnormal lymphatic development leading to a large pleiotropic manifestation of lymphatic dysfunction. Non-syndromic patients, or those without clear genetic etiologies for their lymphatic dysfunction, are often thought to be secondary to physiologic abnormalities as sequelae of congenital heart disease and palliative surgeries. Patients with congenital heart disease and lymphatic dysfunction have a wide variety of clinical manifestations for which there were not many therapeutic interventions available. The development of new imaging techniques allows us to understand better the pathophysiology of these problems and to develop different percutaneous interventions aiming to restore normal lymphatic function.

每 100 个活产婴儿中就有 1 个患有先天性心脏病,这是最常见的先天性畸形之一。先天性心脏病与淋巴异常和/或淋巴功能障碍之间的关系有据可查,大致可分为综合征病因和非综合征病因。在遗传综合征患者中(如上文列举的例子),已知原发性淋巴发育异常会导致淋巴功能障碍的多种表现。非综合征患者或没有明确遗传病因的淋巴功能障碍患者,通常被认为是继发于先天性心脏病和姑息性手术后的生理异常。先天性心脏病和淋巴功能障碍患者的临床表现多种多样,但可用于治疗的干预措施并不多。新成像技术的发展使我们能够更好地了解这些问题的病理生理学,并开发出不同的经皮干预措施,以恢复正常的淋巴功能。
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引用次数: 0
Surgical management of chronic lymphatic pleural effusions and chronic lymphatic ascites 慢性淋巴性胸腔积液和慢性淋巴性腹水的手术治疗
IF 1.7 3区 医学 Q3 PEDIATRICS Pub Date : 2024-05-23 DOI: 10.1016/j.sempedsurg.2024.151420
Pablo Laje, Yoav Dori, Christopher Smith, Katsuhide Maeda

Patients with central lymphatic conduction disorders commonly have recalcitrant pleural effusions and or ascites. These conditions cause a profound deterioration in the patient's quality of life. Support measures such as low-fat diet and diuretics alone hardly ever provide meaningful improvement. New understanding of the pathophysiology of these disorders has opened the door in recent years to the development of several surgical procedures that have remarkable success rates. However, these patients must be managed by expert multidisciplinary teams.

中枢淋巴传导障碍患者通常会出现顽固性胸腔积液和腹水。这些症状会严重影响患者的生活质量。仅靠低脂饮食和利尿剂等辅助措施很难带来有意义的改善。近年来,人们对这些疾病的病理生理学有了新的认识,从而开发出了几种成功率极高的外科手术。然而,这些患者必须由多学科专家团队进行管理。
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引用次数: 0
Critical care management of patients with lymphatic conduction disorders 淋巴传导障碍患者的重症监护管理
IF 1.7 3区 医学 Q3 PEDIATRICS Pub Date : 2024-05-23 DOI: 10.1016/j.sempedsurg.2024.151423
Aaron DeWitt, Erin Pinto, Lauren Biroc, Meghan Scott, Felina Mille, Emmanuelle Favilla, Danish Vaiyani, Samuel Rosenblatt

Lymphatic dysfunction in critical illness is complex. Primary complex lymphatic anomalies can lead to profound organ dysfunction, particularly respiratory failure and shock. Critical illness, the complications of critical illness, and the procedures and therapies used to treat critical illness, can lead to secondary lymphatic dysfunction. This is most often seen with congenital and acquired cardiovascular disease and respiratory disease. The critical care management of these patients requires an expert multidisciplinary team.

危重病人的淋巴功能障碍非常复杂。原发性复杂淋巴异常可导致严重的器官功能障碍,尤其是呼吸衰竭和休克。危重病、危重病并发症以及用于治疗危重病的程序和疗法都可能导致继发性淋巴功能障碍。这种情况多见于先天性和后天性心血管疾病以及呼吸系统疾病。对这些病人的重症监护管理需要一个多学科专家团队。
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引用次数: 0
Surgical management of thoracic duct anomalies 胸导管异常的手术治疗
IF 1.7 3区 医学 Q3 PEDIATRICS Pub Date : 2024-05-23 DOI: 10.1016/j.sempedsurg.2024.151421
Pablo Laje, Yoav Dori, Christopher Smith, Katsuhide Maeda

The development of new imaging techniques for the study of the central lymphatic system allows us to understand the anatomy and pathophysiology of all the disorders of the thoracic duct. With the help of catheters placed percutaneously in the thoracic duct, we can do now complex operations on the thoracic duct to restore its functionality. Advance imaging, expert percutaneous skills, and expert microsurgical skills are critical to the success of these interventions.

研究中央淋巴系统的新成像技术的发展,使我们能够了解胸导管所有疾病的解剖和病理生理学。借助经皮置入胸导管的导管,我们现在可以对胸导管进行复杂的手术,以恢复其功能。先进的成像技术、专业的经皮技术和专业的显微外科技术是这些干预措施取得成功的关键。
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引用次数: 0
Perianal disease: Updates and controversies in closing the gaps 肛周疾病:缩小差距的最新进展与争议
IF 1.7 3区 医学 Q3 PEDIATRICS Pub Date : 2024-04-01 DOI: 10.1016/j.sempedsurg.2024.151402
Samir K. Gadepalli , Jeremy Adler

Perianal complications are common and morbid in children with Crohn’s disease. In this review, we describe the epidemiology, the presentation and diagnosis, evaluation and management. We focus on updates such as the increasing frequency of biologic medications and MRI for evaluation. We also highlight controversies on the timing and approaches to surgical techniques. Finally, perianal disease requires the coordination of multidisciplinary care with nursing, radiology, gastroenterology, and surgery to optimize outcomes – both medical and patient-centered.

肛周并发症是克罗恩病患儿的常见病和多发病。在这篇综述中,我们描述了流行病学、表现和诊断、评估和管理。我们重点关注最新进展,如生物药物和核磁共振成像评估的使用频率不断增加。我们还强调了手术时机和方法方面的争议。最后,肛周疾病需要护理、放射科、消化内科和外科等多学科的协调护理,以优化医疗和患者为中心的治疗效果。
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引用次数: 0
Inflammatory bowel disease – evolving therapeutic approaches to a hidden epidemic 炎症性肠病--针对隐性流行病的不断演变的治疗方法
IF 1.7 3区 医学 Q3 PEDIATRICS Pub Date : 2024-04-01 DOI: 10.1016/j.sempedsurg.2024.151407
Lisa McMahon , Samir K. Gadepalli
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引用次数: 0
fm i -- Contents FM I -- 目录
IF 1.7 3区 医学 Q3 PEDIATRICS Pub Date : 2024-04-01 DOI: 10.1053/S1055-8586(24)00032-5
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引用次数: 0
Medical management of pediatric inflammatory bowel disease 小儿炎症性肠病的药物治疗
IF 1.7 3区 医学 Q3 PEDIATRICS Pub Date : 2024-04-01 DOI: 10.1016/j.sempedsurg.2024.151398
Brad Pasternak

Management of inflammatory bowel disease, both Crohn's disease (CD) and ulcerative colitis (UC), has seen a seismic shift over the past decade. Over the past five years, there has been the introduction of many new therapies with differing mechanisms of action and a goal of achieving mucosal healing, as well as clinical and biochemical remission (1,2). In addition, management is aimed at restoring normal growth and normalizing quality of life. The ultimate goal is to individualize medical management and determine the right drug for the right patient by identifying which inflammatory pathway is predominant and avoiding unwarranted lack of efficacy or side effects through biomarkers and risk prognostication. Patient's age, location of disease, behavior (inflammatory vs. penetrating/structuring), severity and growth delay all play into deciding on the best treatment approach. Ultimately, early intervention is key in preventing complications. The therapeutic approaches to management can be broken down to nutritional therapy, biologic agents, immunomodulators (including corticosteroids), aminosalicylates and antibiotics. There are numerous other therapies, such as small molecule agents recently approved in adults, which are garnering a great deal of interest.

过去十年间,克罗恩病(CD)和溃疡性结肠炎(UC)这两种炎症性肠病的治疗方法发生了翻天覆地的变化。在过去五年中,许多新疗法相继问世,它们的作用机制各不相同,目标都是实现粘膜愈合以及临床和生化缓解(1,2)。此外,治疗的目的还包括恢复正常生长和正常生活质量。最终目标是通过生物标志物和风险预后,确定哪种炎症途径占主导地位,避免不必要的疗效缺乏或副作用,从而实现个体化医疗管理,并为合适的患者确定合适的药物。患者的年龄、发病部位、行为(炎症性与穿透性/结构性)、严重程度和生长延迟都是决定最佳治疗方法的因素。归根结底,早期干预是预防并发症的关键。治疗方法可分为营养疗法、生物制剂、免疫调节剂(包括皮质类固醇)、氨基水杨酸盐和抗生素。还有许多其他疗法,如最近获准用于成人的小分子药物,正引起人们的极大兴趣。
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引用次数: 0
Evolution of enhanced recovery for children undergoing elective intestinal surgery 为接受择期肠道手术的儿童加强康复的发展历程
IF 1.7 3区 医学 Q3 PEDIATRICS Pub Date : 2024-04-01 DOI: 10.1016/j.sempedsurg.2024.151400
Mallory N. Perez, Mehul V. Raval

Enhanced recovery protocols (ERP) have been widely adopted in adult populations, with over 30 years of experience demonstrating the effectiveness of these protocols in patients undergoing gastrointestinal (GI) surgery. In the last decade, ERPs have been applied to pediatric populations across multiple subspecialties. The objective of this manuscript is to explore the evolution of how ERPs have been implemented and adapted specifically for pediatric populations undergoing GI surgery, predominantly for inflammatory bowel disease. The reported findings reflect a thorough exploration of the literature, including initial surveys of practice/readiness assessments, consensus recommendations of expert panels, and data from a rapidly growing number of single center studies. These efforts have culminated in a national prospective, multicenter trial evaluating clinical and implementation outcomes for enhanced recovery in children undergoing GI surgery. In short, this historical and clinical review reflects on the evolution of ERPs in pediatric surgery and expounds upon the next steps needed to apply ERPs to future pediatric populations.

强化恢复方案(ERP)已在成人群体中广泛采用,30 多年的经验证明了这些方案对胃肠道(GI)手术患者的有效性。近十年来,ERP 在多个亚专科的儿科人群中得到了应用。本手稿旨在探讨ERP在小儿消化道手术(主要是炎症性肠病)中的应用和调整的演变过程。所报告的研究结果反映了对文献的深入探讨,包括对实践/准备评估的初步调查、专家小组的共识建议以及数量迅速增加的单中心研究数据。这些努力最终促成了一项全国性的前瞻性多中心试验,对加强儿童消化道手术恢复的临床和实施效果进行评估。总之,这篇历史和临床综述反映了ERP在儿科手术中的发展,并阐述了将ERP应用于未来儿科人群所需的下一步措施。
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引用次数: 0
Surgical management of complicated Crohn's disease 并发克罗恩病的手术治疗
IF 1.7 3区 医学 Q3 PEDIATRICS Pub Date : 2024-04-01 DOI: 10.1016/j.sempedsurg.2024.151399
Sarah J. Ullrich, Jason S. Frischer

Surgical management of pediatric Crohn's disease is fundamentally palliative, aiming to treat the sequalae of complicated disease while preserving intestinal length. Multidisciplinary discussion of risk factors and quality of life should take place prior to operative intervention. Though the surgical management of pediatric Crohn's disease is largely based on the adult literature, there are considerations specific to the pediatric population – notably disease and treatment effects on growth and development. Intrabdominal abscess is approached with percutaneous drainage when feasible, reserving surgical intervention for the patient who is unstable or failing medical therapy. Pediatric patients with fibrostenotic disease should be considered for strictureplasty when possible, for maximum preservation of bowel length. Patients with medically refractory Crohn's proctocolitis should be treated initially with fecal diversion without proctocolectomy.

小儿克罗恩病的手术治疗从根本上说是姑息性的,旨在治疗复杂疾病的后遗症,同时保留肠道长度。在进行手术干预之前,应就风险因素和生活质量进行多学科讨论。虽然小儿克罗恩病的手术治疗主要以成人文献为基础,但也要考虑到小儿的特殊情况,特别是疾病和治疗对生长发育的影响。腹腔内脓肿在可行的情况下采用经皮引流术,对于病情不稳定或药物治疗无效的患者保留手术治疗。患有纤维狭窄性疾病的小儿患者应尽可能考虑进行狭窄成形术,以最大限度地保留肠道长度。药物难治性克罗恩直肠结肠炎患者应首先接受粪便转流治疗,而不进行直肠切除术。
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Seminars in Pediatric Surgery
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