赫氏胃肠病的治疗方法:临床病例。

Q3 Medicine Journal of Medicine and Life Pub Date : 2024-06-01 DOI:10.25122/jml-2024-0307
Viorel Țandea, Ionuț Daniel Răducan, Oana Neagu, Silviu Constantinoiu
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引用次数: 0

摘要

赫氏病是一种先天性疾病,其特征是结肠肠肌丛和粘膜下神经节细胞缺失,导致肠蠕动受损和功能性肠梗阻。大约每 5,000 名新生儿中就有 1 人患此病,男孩发病率更高。虽然这种疾病在 17 世纪就被首次发现,但 Harald Hirschsprung 于 1886 年明确了它与慢性便秘的关系。当代的治疗策略强调早期诊断、手术切除激动区段以及术后专业护理,以恢复正常的结肠功能并改善患者的生活质量。本文回顾了当前的治疗策略,重点介绍了先进的手术技术、诊断方法和术后管理。两个临床病例说明了该疾病的影响和治疗方法。第一个病例是一名 5 个月大的男性,患有严重营养不良、脱水和急性小肠结肠炎,他接受了右侧结肠造口术,后来又接受了 Duhamel 型粘膜外括约肌-直肠-肌瘤切除术,并取得了良好的进展。第二个病例是一名 1.5 岁的患者,曾做过结肠造口术和回肠造口术,需要进一步手术治疗。两例病例的组织病理学检查均显示神经节细胞缺失,确诊为赫氏病。
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Therapeutic approaches in Hirschsprung's disease: clinical cases.

Hirschsprung's disease is a congenital disorder characterized by the absence of ganglion cells in the myenteric and submucosal plexuses of the colon, resulting in impaired peristalsis and functional intestinal obstruction. This condition affects approximately 1 in 5,000 newborns, with a higher prevalence in boys. Although first identified in the 17th century, its connection to chronic constipation was clearly established by Harald Hirschsprung in 1886. Contemporary treatment strategies emphasize early diagnosis, surgical excision of the aganglionic segment, and specialized postoperative care to restore normal colonic function and improve the quality of life for affected individuals. This article reviews current therapeutic strategies, highlighting advanced surgical techniques, diagnostic methods, and postoperative management. Two clinical cases illustrate the impact and treatment of the disease. The first case involves a 5-month-old male with severe malnutrition, dehydration, and acute enterocolitis, who underwent a right-side colostomy and later a Duhamel-type extramucosal sphincter-rectal-myotomy, showing favorable progress. The second case is a 1.5-year-old patient with a history of colostomy and ileostomy, requiring further surgery. Histopathological examinations in both cases revealed the absence of ganglion cells, confirming the diagnosis of Hirschsprung's disease.

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来源期刊
Journal of Medicine and Life
Journal of Medicine and Life Medicine-Medicine (all)
CiteScore
1.90
自引率
0.00%
发文量
202
期刊介绍: The Journal of Medicine and Life publishes peer-reviewed articles from various fields of medicine and life sciences, including original research, systematic reviews, special reports, case presentations, major medical breakthroughs and letters to the editor. The Journal focuses on current matters that lie at the intersection of biomedical science and clinical practice and strives to present this information to inform health care delivery and improve patient outcomes. Papers addressing topics such as neuroprotection, neurorehabilitation, neuroplasticity, and neuroregeneration are particularly encouraged, as part of the Journal''s continuous interest in neuroscience research. The Editorial Board of the Journal of Medicine and Life is open to consider manuscripts from all levels of research and areas of biological sciences, including fundamental, experimental or clinical research and matters of public health. As part of our pledge to promote an educational and community-building environment, our issues feature sections designated to informing our readers regarding exciting international congresses, teaching courses and relevant institutional-level events.
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