短肠综合征
Short bowel syndrome
定义
短肠综合征是一种由先天性缺陷、肠梗死或广泛手术切除引起的肠功能衰竭，其特征为腹泻、营养物质吸收不良、肠道扩张和运动障碍。
基本事实
- 患病率
- 1-9 / 100 000（Europe）
近两年的全球研究 1,632L2
2024/08 起在 Europe PMC 检索所得，按发表时间倒序显示最近 20 篇。标题未译成中文——自动翻译需要接入 LLM 服务，尚未引入。
- 2026-07综述Glucagon-like peptide-1 analogues in the management of high output stoma in adult patients: A narrative review
- 2026-07Long-term antithrombotic therapy in patients with short bowel syndrome: A European Society for Clinical Nutrition and Metabolism (ESPEN) position paper
- 2026-07Vascular Preservation for Long-term Central Venous Access in Infants: The Facial Vein as an Underutilized Option
- 2026-07Short Bowel Syndrome with Intestinal Failure: Healthcare Resource Utilization and Cost of Care in a Large US Claims Database
- 2026-07综述病例报告[Myxedematous coma due to severe hypothyroidism associated with adrenal insufficiency and a history of short small intestine]
- 2026-07Extracellular vesicles in perinatal conditions: A minimally invasive approach to regenerative medicine
- 2026-07Biopsy-Proven Intestinal Failure-Associated Liver Disease in Pediatric Short Bowel Syndrome: Histopathological Findings and Clinical Outcomes
- 2026-07综述Venous thromboembolism in pediatrics: Management of special clinical scenarios
- 2026-07Filling the gaps: Information sources and needs of patients with intestinal failure and their caregivers
- 2026-07Acute mesenteric ischemia complicated by short bowel syndrome: a rare case report from Syria
- 2026-07病例报告Intestinal failure-associated liver disease in a child with short bowel syndrome: management issues
- 2026-07Transition of care for children with complex congenital surgical conditions as they age into adulthood
- 2026-07病例报告Multistage Bowel Salvage Through Surgical Resection and Superior Mesenteric Artery Revascularization in Acute-on-Chronic Mesenteric Ischemia: A Case Report
- 2026-07A memorial tribute to Dr Theresa Byrne, PhD, RD
- 2026-07Assessing the health-related quality of life and clinical effect in children with short bowel syndrome receiving teduglutide treatment
- 2026-07Signal detection and analysis of adverse events associated with sevelamer post-marketing based on the FAERS database
- 2026-07Decreased bone mineral density in adults with type 2 intestinal failure: A descriptive cross-sectional study
- 2026-07Cognitive assessment in children with intestinal failure on and weaned off parenteral nutrition
- 2026-06Development of Teduglutide purity certified reference material (GBW09342) through amino acid-based isotope dilution mass spectrometry and sulfur-based isotope dilution inductively coupled plasma mass spectrometry
- 2026-06Association between teduglutide treatment and long-term reductions in parenteral support: An observational cohort study of adults with short bowel syndrome
境外已获批用于本病的药物 4L2
欧盟 1 项、美国 3 项。同一药物在两地各批一次的，会分别列出。
「境外已获批」不等于「在中国能用」。中间隔着进口注册、临床急需境外新药通道、海南博鳌乐城国际医疗旅游先行区等几条路径，各有各的条件与费用。这一节能确定地告诉你的只有一件事：这个病在世界范围内已经有获得批准的药物，它叫什么名字。拿这个名字去问主治医生，是下一步最省力的做法。
药名一律保留英文原文，不作翻译——中国的药品通用名与英文名的音译经常不一致，译错会让人去找一个不存在的药。
- Teduglutide Viatris欧盟2026-01-08teduglutide官方记录
- Zorbtive美国2003-12-01Somatropin (r-DNA)官方记录
- NutreStore美国2004-06-10Glutamine官方记录
- GATTEX美国2012-12-21teduglutide [rDNA origin]官方记录
已获孤儿药资格、尚未获批的在研药物（19 项）
孤儿药资格只是一种监管身份——它意味着监管机构认可这是罕见病用药并给予研发激励，不代表这个药已被证明有效，也不代表将来一定能上市。绝大多数最终不会成药。列在这里是为了看清有哪些方向正在被尝试。
- [Gly2]-recombinant human glucagon-like peptide欧盟2001-12-12Treatment of short bowel syndrome官方记录
- oxalobacter formigenes strain HC-1欧盟2014-10-15Treatment of short bowel syndrome官方记录
- insulin human欧盟2015-08-10Treatment of short bowel syndrome官方记录
- apraglutide欧盟2018-11-19Treatment of short bowel syndrome官方记录
- human glucagon-like peptide-2 analogue linked to a human immunoglobuli欧盟2019-01-11Treatment of short bowel syndrome官方记录
- crofelemer欧盟2021-12-10Treatment of short bowel syndrome官方记录
- Recombinant human insulin-like growth factor-I美国2000-02-16Treatment of short-bowel syndrome as a result of resection of the small bowel or as a result of congenital dysfunction of the intestines.官方记录
- conjugated bile acids美国2003-07-18Treatment of steatorrhea in patients with short bowel syndrome官方记录
- opium tincture美国2011-12-02Treatment of chronic diarrhea in short bowel syndrome patients with an inadequate response to anti-diarrheal treatment官方记录
- human insulin (rDNA)美国2015-04-06Treatment of short bowel syndrome (SBS).官方记录
- docosahexaenoic acid美国2015-06-01Treatment of short bowel syndrome官方记录
- recombinant fusion protein comprising exenatide and XTEN美国2015-10-07Treatment of short bowel syndrome.官方记录
- melatonin美国2017-02-14Treatment of short bowel syndrome官方记录
- crofelemer美国2017-08-02Treatment of short bowel syndrome官方记录
- glepaglutide美国2017-10-20Treatment of short bowel syndrome官方记录
- Glucagon-Like Peptide 2 fused to hyFc美国2018-10-11Treatment of short bowel syndrome官方记录
- apraglutide美国2018-12-20Treatment of short bowel syndrome (SBS)官方记录
- human glucagon-like peptide-2 analogue linked to a human immunoglobuli美国2019-05-01Treatment of short bowel syndrome官方记录
- Arginine butyrate美国2023-03-09Treatment of Short Bowel Syndrome官方记录
数据来自欧洲药品管理局（EMA）的药品与孤儿药资格公开导出表，以及美国 FDA 孤儿药资格数据库。两边口径不同：欧盟一侧取的是当前状态仍为「已授权」的药品；美国一侧记录的是「曾获批准」这一事实，FDA 的公开表不追踪药物此后是否退市（例如 Relyvrio 于 2024 年撤市，表中仍记为已获批）。请以官方记录页为准。
在中国开展的临床试验 13L2
按病名在 ClinicalTrials.gov 检索、并校验研究中心含中国大陆而来。登记状态不等于现在真的能入组——务必按 NCT 号到原站核实，并与主治医生商量。
登记为可入组 5
- 招募中NCT07691125Phase IV Study of rhGH Injection in Adult Short Bowel Syndrome中国研究中心 2 个：Nanjing、Xiamen
- 招募中NCT06512584Efficacy and Safety of PJ009 in Patients With Short Bowel Syndrome Requiring Parenteral Nutrition中国研究中心 8 个：Beijing、Changsha、Chongqing、Guangzhou、Nanjing、Qingdao 等 8 地
- 招募中NCT06973304A Study of Teduglutide in Chinese Adults With Short Bowel Syndrome中国研究中心 4 个：Changping、Dongcheng、Nanjing、Siming
- 招募中NCT07319832A Study of Teduglutide in Chinese Children and Teenagers With Short Bowel Syndrome中国研究中心 4 个：Guangzhou、Hangzhou、Shanghai
- 招募中NCT07704047A Clinical Study of FT1 in Patients With Short Bowel Syndrome中国研究中心 1 个：Nanjing
其他状态的试验（8 项）
- 已完成NCT01974336The Preventative and Therapeutical Effect of Ursodeoxycholic Acid(UDCA) to Short Bowel Syndrome Patients中国研究中心 1 个：Nanjing
- 状态未知NCT02452177Preventive and Reversional Effect of Vitamin D on Parenteral Nutrition Associated Liver Disease中国研究中心 1 个：Nanjing
- 已完成NCT02699320Intestinal Microbial Dysbiosis in Chinese Infants With Short Bowel Syndrome With Different Complications中国研究中心 1 个：Shanghai
- 状态未知NCT03590418Microbial Diversity of Small Bowel Stoma Effluent and Colonic Faeces中国研究中心 1 个：Shanghai
- 状态未知NCT06427642Efficacy Evaluation of UCB-MNCs in the Treatment of Refractory Neonatal Diseases中国研究中心 1 个：Jinan
- 已完成NCT06523101A Study of PJ009 in Healthy Adult Volunteers中国研究中心 1 个：Taiyuan
- 已完成NCT07186608Specific Dietary Fibers May Enhance Colonic Adaptation in Short Bowel Syndrome Through Microbial and Metabolic Mechanisms That Drive Functional Compensation.中国研究中心 1 个：Nanjing
- 已完成NCT07297238Effect of GLP-1 on Intestinal Barrier Function in SBS-IF Patients: A Preliminary Exploration.中国研究中心 2 个：Nanjing、Nanning
中国境外的在招试验 27L2
这些试验在中国没有研究中心，通常无法直接报名——入组一般要求在当地居住并接受随访。列在这里是因为它另有用处：看清楚全世界正在试哪些药、做到了哪一期、由谁在做。把药名和 NCT 编号记下来去问主治医生，或据此进一步查该药是否已在境外获批、是否有拓展性用药（expanded access）通道。
美国11法国6比利时4丹麦4德国4意大利4波兰3英国3澳大利亚2奥地利2加拿大2匈牙利2荷兰2韩国2另有 13 个国家/地区
CT.gov 报告命中 27 项，此处取回并展示最近的 15 项。
- 招募中NCT07742735A Study to Investigate the Efficacy and Safety of Apraglutide Compared With Placebo in Adult Participants With Short Bowel Syndrome Associated With Intestinal Failure (SBS-IF)阿根廷、澳大利亚、奥地利、比利时、巴西、加拿大、捷克、丹麦 等 22 国
- 招募中NCT07392801AGE Accumulation in Patients on Long-term Parenteral Nutrition法国
- 招募中NCT07703371Kodo Millet Porridge and Its Effects on Gut Health and Metabolic Syndrome印度
- 尚未开始招募NCT07530055Role of the Microbiota in Intestinal Adaptation During Short Bowel Syndrome
- 招募中NCT07197944Efficacy And Safety Evaluation of Glepaglutide in Treatment of SBS奥地利、比利时、丹麦、芬兰、法国、德国、匈牙利、意大利 等 14 国
- 尚未开始招募NCT07206004Effect of Parenteral Support on FibroScan in Short Bowel Syndrome丹麦
- 招募中NCT07713641Short-Chain Fatty Acids in Lypopolysaccharide-Induced Inflammation and Executive Function比利时
- 招募中NCT07714499Resting Energy Expenditure by Gastrointestinal Functional Status斯洛文尼亚
- 招募中NCT06904872Safety and Efficacy of Crofelemer in Adult Patients With Short Bowel Syndrome and Intestinal Failure (SBS-IF) Without Colon-in-continuity (CIC)德国、意大利
- 招募中NCT06877923Elemental 028 Extra Case Studies英国
- 招募中NCT06326645Open-Label Pilot Study With Crofelemer in Patients With Short Bowel Syndrome美国
- 招募中NCT06771505SBS DISK- Creation of a Quality of Life Tool for Short Bowel Patients Compared With a Validated Quality of Life Questionnaire (SBS QoL)法国
- 招募中NCT06668922Irritable Bowel Syndrome and Control Volunteers: Diet Challenge美国
- 招募中NCT07400783TED_ORG: Study on Short Bowel Syndrome意大利
- 招募中NCT05535361A Feasibility Study to Evaluate Safety and Probable Benefit of the Eclipse XL1 System for Distraction Enterogenesis in Adult and Pediatric Patients With Short Bowel Syndrome美国
外部标识与链接
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本页数据来源
- 疾病定义、同义词、基因、表型、流行病学：Orphanet（CC BY 4.0）
- 中文病名：Orphanet 中文包，冻结于 2020-06-01
- 表型中文标签：HPO 简体中文翻译（CHPO 上游成果）