生长抑素类似物在遗传性出血性毛细血管扩张症慢性胃肠道出血中的长期应用。
Long-term use of somatostatin analogs for chronic gastrointestinal bleeding in hereditary hemorrhagic telangiectasia.
作者信息
Torres-Iglesias Raquel, Mora-Luján José María, Iriarte Adriana, Cerdà Pau, Alba Esther, Sánchez-Corral Miguel Ángel, Berrozpe Ana, Cruellas Francesc, Gamundí Enric, Ribas Jesús, Castellote Jose, Riera-Mestre Antoni
机构信息
HHT Unit, Hospital Universitari de Bellvitge, Barcelona, Spain.
Internal Medicine Department, Hospital Universitari de Bellvitge, Barcelona, Spain.
出版信息
Front Med (Lausanne). 2023 May 12;10:1146080. doi: 10.3389/fmed.2023.1146080. eCollection 2023.
BACKGROUND
Chronic bleeding due to gastrointestinal (GI) involvement in patients with hemorrhagic hereditary telangiectasia (HHT) can provoke severe anemia with high red blood cells (RBC) transfusion requirements. However, the evidence about how to deal with these patients is scarce. We aimed to assess the long-term efficacy and safety of somatostatin analogs (SA) for anemia management in HHT patients with GI involvement.
METHODS
This is a prospective observational study including patients with HHT and GI involvement attended at a referral center. SA were considered for those patients with chronic anemia. Anemia-related variables were compared in patients receiving SA before and during treatment. Patients receiving SA were divided into responders (patients with minimal hemoglobin levels improvement >10 g/L and maintaining hemoglobin levels ≥80 g/L during treatment), and non-responders. Adverse effects during follow-up were collected.
RESULTS
Among 119 HHT patients with GI involvement, 67 (56.3%) received SA. These patients showed lower minimal hemoglobin levels (73 [60-87] vs. 99 [70.2-122.5], < 0.001), and more RBC transfusion requirements (61.2% vs. 38.5%, = 0.014) than patients without SA therapy. Median treatment period was 20.9 ± 15.2 months. During treatment, there was a statistically significant improvement in minimum hemoglobin levels (94.7 ± 29.8 g/L vs. 74.7 ± 19.7, < 0.001) and a reduction of patients with minimal hemoglobin levels <80 g/L (39 vs. 61%, = 0.007) and RBC transfusions requirement (33.9% vs. 59.3%, < 0.001). Sixteen (23.9%) patients showed mild adverse effects, mostly diarrhea or abdominal pain, leading to treatment discontinuation in 12 (17.9%) patients. Fifty-nine patients were eligible for efficacy assessment and 32 (54.2%) of them were considered responders. Age was associated with non-responder patients, OR 95% CI; 1.070 (1.014-1.130), = 0.015.
CONCLUSION
SA can be considered a long-term effective and safe option for anemia management in HHT patients with GI bleeding. Older age is associated with poorer response.
背景
出血性遗传性毛细血管扩张症（HHT）患者因胃肠道（GI）受累导致的慢性出血可引发严重贫血，需要大量输注红细胞（RBC）。然而，关于如何治疗这些患者的证据很少。我们旨在评估生长抑素类似物（SA）对伴有GI受累的HHT患者贫血治疗的长期疗效和安全性。
方法
这是一项前瞻性观察性研究，纳入了在一家转诊中心就诊的伴有GI受累的HHT患者。对于那些患有慢性贫血的患者给予SA治疗。比较接受SA治疗的患者治疗前和治疗期间与贫血相关的变量。接受SA治疗的患者分为反应者（血红蛋白水平最低改善>10 g/L且治疗期间血红蛋白水平维持≥80 g/L的患者）和无反应者。收集随访期间的不良反应。
结果
在119例伴有GI受累的HHT患者中，67例（56.3%）接受了SA治疗。与未接受SA治疗的患者相比，这些患者的最低血红蛋白水平较低（73[60 - 87] vs. 99[70.2 - 122.5]，<0.001），且需要更多的RBC输血（61.2% vs. 38.5%，=0.014）。中位治疗期为20.9±15.2个月。治疗期间，最低血红蛋白水平有统计学显著改善（94.7±29.8 g/L vs. 74.7±19.7，<0.001），最低血红蛋白水平<80 g/L的患者比例降低（39% vs. 61%，=0.007），RBC输血需求降低（33.9% vs. 59.3%，<0.001）。16例（23.9%）患者出现轻度不良反应，主要为腹泻或腹痛，导致12例（17.9%）患者停药。59例患者符合疗效评估标准，其中32例（54.2%）被视为反应者。年龄与无反应患者相关，OR 95%CI：1.070（1.014 - 1.130），=0.015。
结论
对于伴有GI出血的HHT患者，SA可被视为一种长期有效且安全的贫血治疗选择。年龄较大与反应较差相关。
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