结节性硬化症患者巨大肾错构瘤破裂出血1例救治体会并文献复习  

Treatment experience of giant renal hamartoma rupture and hemorrhage in a patient with tuberous sclerosis complex and literature review

作　　者：贾敏楠 刘琼[2] 徐若云 牛凯[2] Jia Minnan;Liu Qiong;Xu Ruoyun;Niu Kai(Graduate School of Hebei Medical University,Shijiazhuang 050017,China;Department of Nephrology,Hebei General Hospital,Shijiazhuang 050051,China)

机构地区：[1]河北医科大学研究生学院,河北石家庄050017 [2]河北省人民医院肾内科,河北石家庄050051

出　　处：《临床荟萃》2024年第12期1111-1114,共4页Clinical Focus

摘　　要：目的探讨巨大错构瘤成因及有效治疗手段。方法回顾性分析1例巨大肾错构瘤破裂出血的结节性硬化患者的诊疗经过,并复习国内外相关文献。结果该患者巨大肾错构瘤破裂出血,行动脉栓塞术,术后发热合并急性肾损伤,评估再出血风险较高,行肾脏切除手术治疗。结论结节性硬化症合并肾错构瘤不易引起肾功能异常,针对破裂出血患者,主要治疗手段为保守治疗或介入栓塞治疗。该疾病具有家族遗传性,早期诊断及监测至关重要。Objective To explore the causes and effective treatment of giant hamartomas.Methods The diagnosis and treatment of 1 case of nodular sclerosis with giant renal angiomyolipoma rupture and hemorrhage were retrospectively analyzed,and the relevant literatures at home and abroad were reviewed.Results The patient was treated with arterial embolization due to rupture and bleeding.After surgery,the patient developed fever and acute kidney injury,with a high risk of rebleeding that requires a renal resection.Conclusion Tuberous sclerosis combined with renal hamartoma is not likely to cause abnormal renal function.For patients with rupture and bleeding,conservative or interventional embolization is preferred.The disease is hereditary in families,and early diagnosis and monitoring are essential.

关 键 词：结节性硬化症 肾错构瘤 介入栓塞治疗 

分 类 号：R742.89[医药卫生—神经病学与精神病学]