遗传性出血性毛细血管扩张症并发肝性脑病的弥漫性脑水肿和迫在眉睫的疝

IF 0.8 Q3 MEDICINE, GENERAL & INTERNAL Case Reports in Medicine Pub Date : 2022-02-18 DOI:10.1155/2022/2612544
Charisma Mylavarapu, Alexander J. Lu, E. Burns, Justin Samorajski, D. Gotur, K. Baker
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引用次数: 1

摘要

遗传性出血性毛细血管扩张症(HHT)是一种常染色体显性的血管疾病,其特征是皮肤和内脏毛细血管扩张和动静脉畸形(AVM)的形成。多个器官可能受到影响,包括鼻黏膜、皮肤、肺、胃肠道和大脑。以下病例强调了HHT在胃肠道出血和鼻出血患者中的独特表现,导致高氨血症和弥漫性脑水肿和疝出。临床医生应该意识到这类患者的潜在并发症,并尽早使用氨还原剂以避免这种毁灭性的后果。
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Diffuse Cerebral Edema and Impending Herniation Complicating Hepatic Encephalopathy in Hereditary Hemorrhagic Telangiectasia
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular disease characterized by the formation of cutaneous and visceral telangiectasias and arteriovenous malformations (AVM). Multiple organs may be affected, including the nasal mucosa, skin, lungs, gastrointestinal tract, and brain. The following case highlights a unique manifestation of HHT in a patient with a gastrointestinal hemorrhage and epistaxis, resulting in hyperammonemia and diffuse cerebral edema and herniation. Clinicians should be aware of this potential complication in such patients and initiate ammonia-reducing agents early to avoid this devastating consequence.
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来源期刊
Case Reports in Medicine
Case Reports in Medicine MEDICINE, GENERAL & INTERNAL-
CiteScore
1.70
自引率
0.00%
发文量
53
审稿时长
13 weeks
期刊最新文献
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