先天性肝血管瘤(CHHs)是良性血管肿瘤,组织学,和遗传相关性最近在长期生存的患者中得到了描述,尽管迄今为止尚未发现死亡危险因素.这项研究的目的是分析CHH患者死亡率的预测因素。对1991年至2021年在我们机构诊断的连续CHH患者进行了回顾性单中心病例对照研究,根据生存率分为两组。人口统计,妊娠,成像,收集诊断时的实验室数据,并比较两组间的差异.共纳入29例患者(男性12例,女性17例),其中5例因心力衰竭和凝血障碍导致CHH演变而死亡。直到死亡的平均年龄为11天。人口统计学或妊娠数据没有差异。在比较影像学检查时,没有任何差异,也不在位置上,受累肝段的数量,或CHH估计体积。根据诊断时的实验室数据,死亡患者的中位肝酶显着升高[谷氨酸-草酰乙酸转氨酶(359u/Lvs.45u/L;p<0.01)和谷氨酸-丙酮酸转氨酶(313u/Lvs.20u/L;p<0。01)],以及降低的中位血小板计数(85,250/µLvs.337,000/微升;p<0.01),凝血酶原活性(54%vs.93%;p<0.01),和纤维蛋白原(131mg/dLvs.284mg/dL;p<0.01),血细胞计数或生化数据没有差异。
结论:CHH的临床行为可以是无害的或危及生命的。血小板减少症,凝血障碍,诊断时肝酶升高似乎是死亡率的主要预测因素。
背景:•先天性肝血管瘤(CHHs)是良性血管肿瘤,其临床行为可以是无害的或危及生命的。
背景:•血小板减少症,凝血障碍和诊断时肝酶升高似乎是这些患者死亡率的主要预测因素。
Congenital hepatic hemangiomas (CHHs) are benign vascular tumors whose clinical, histological, and genetic correlation has recently been described in patients with long-term survival, although no mortality risk factors have been identified to date. The aim of this
study is to analyze predictors of mortality in patients with CHH. A retrospective single-center case-control
study of consecutive CHH patients diagnosed in our institution between 1991 and 2021 was performed, who were classified into two groups according to their survival. Demographic, gestational, imaging, and laboratory data at diagnosis were collected and compared between both groups. A total of 29 patients were included (12 males; 17 females) of whom 5 died as a result of CHH evolution due to cardiac failure and coagulopathy, with a median age of 11 days until death. No differences in demographic or gestational data were reported. There were neither differences when comparing imaging tests, nor in location, number of affected liver segments, or CHH estimated volume. Upon laboratory data at diagnosis, deceased patients had a significant elevation of median liver enzymes [glutamic-oxaloacetic transaminase (359 u/L vs. 45 u/L; p < 0.01) and glutamic-pyruvic transaminase (313 u/L vs. 20 u/L; p < 0. 01)], as well as a decreased median platelet count (85,250/µL vs. 337,000/µL; p < 0.01), prothrombin activity (54% vs. 93%; p < 0.01), and fibrinogen (131 mg/dL vs. 284 mg/dL; p < 0.01), with no differences in blood count or biochemistry data.
CONCLUSIONS: CHH clinical behavior can be innocuous or life-threatening. Thrombocytopenia, coagulation disorders, and increased liver enzymes at diagnosis seem to be the main predictors of mortality.
BACKGROUND: • Congenital Hepatic Hemangiomas (CHHs) are benign vascular tumors whose clinical behavior can be innocuous or life-threatening.
BACKGROUND: • Thrombocytopenia, coagulation disorders and increased liver enzymes at diagnosis seem to be the main predictors of mortality in these patients.