Zarka Jabra, Jeong Kwonho, Yabes Jonathan G, Ragni Margaret V
Department of Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA.
University of Pittsburgh Center for Research on Health Care Data Center, Pittsburgh, PA.
Blood Adv. 2023 Oct 10;7(19):5843-5850. doi: 10.1182/bloodadvances.2023010743.
Hereditary hemorrhagic telangiectasia (HHT) is a common bleeding disorder, but little is known regarding prevalence and risk factors for bleeding. Adult discharges with HHT and bleeding were identified by International Classification of Disease, 10th edition (ICD-10) codes in the National Inpatient Sample (NIS), 2016-2018. Prevalence estimates were weighted using NIS discharge-level weights to reflect national estimates. Risk factors for bleeding were determined by weighted multivariable logistic regression. Among 18 170 849 discharges, 2528 (0.01%) had HHT, of whom 648 (25.6%) had bleeding. Arteriovenous malformation (AVM) (31.9% vs 1.3%), angiodysplasia (23.5% vs 2.3%), telangiectasia (2.3% vs 0.2%), and epistaxis (17.9% vs 0.6%) were more common in HHT than in non-HHT patients (non-HHT), each P < .001. In contrast, menstrual (HMB) and postpartum bleeding (PPH) were less common in reproductive-age HHT than non-HHT, each P < .001. Anemia associated with iron deficiency (IDA), was equally common in HHT with or without bleeding (15.7% vs 16.0%), but more common than in non-HHT (7.5%), P < .001. Comorbidities, including gastroesophageal reflux (25.9% vs 20.0%) and cirrhosis (10.0% vs 3.6%) were greater in HHT than non-HHT, each P < .001. In multivariable logistic regression, peptic ulcer disease (OR, 8.86; P < .001), portal vein thrombosis (OR, 3.68; P = .006), and hepatitis C, (OR, 2.13; P = .017) were significantly associated with bleeding in HHT. In conclusion, AVM and angiodysplasia are more common and HMB and PPH less common in patients in those with HHT than non-HHT. IDA deficiency is as common in HHT with and without bleeding, suggesting ongoing blood loss and need for universal iron screening.
遗传性出血性毛细血管扩张症(HHT)是一种常见的出血性疾病,但关于其患病率及出血风险因素的了解却很少。通过2016 - 2018年全国住院患者样本(NIS)中的国际疾病分类第10版(ICD - 10)编码识别出患有HHT且有出血症状的成年出院患者。患病率估计值采用NIS出院水平权重进行加权,以反映全国估计情况。出血风险因素通过加权多变量逻辑回归确定。在18170849例出院患者中,2528例(0.01%)患有HHT,其中648例(25.6%)有出血症状。动静脉畸形(AVM)(31.9%对1.3%)、血管发育异常(23.5%对2.3%)、毛细血管扩张(2.3%对0.2%)和鼻出血(17.9%对0.6%)在HHT患者中比非HHT患者更常见,P值均<0.001。相比之下,育龄期HHT患者的月经过多(HMB)和产后出血(PPH)比非HHT患者少见,P值均<0.001。缺铁性贫血(IDA)在有出血和无出血的HHT患者中同样常见(15.7%对16.0%),但比非HHT患者更常见(占7.5%),P<0.001。包括胃食管反流(25.9%对20.0%)和肝硬化(10.0%对3.6%)在内的合并症在HHT患者中比非HHT患者更常见,P值均<0.001。在多变量逻辑回归中,消化性溃疡病(OR,8.86;P<0.001)、门静脉血栓形成(OR,3.68;P = 0.006)和丙型肝炎(OR,2.13;P = 0.017)与HHT患者的出血显著相关。总之,与非HHT患者相比,AVM和血管发育异常在HHT患者中更常见,而HMB和PPH则较少见。IDA在有出血和无出血的HHT患者中同样常见,提示存在持续失血,需要进行普遍的铁筛查。