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闪电会再次击中吗?与罕见肝脏疾病相关的野生型转甲状腺素蛋白心脏淀粉样变性。

Can lightning strike twice? Wild-type transthyretin cardiac amyloidosis associated with rare liver disease.

作者信息

Bhattacharya Priyanka T, Fox Alyson N, Marboe Charles C, Lagana Stephen M, Remotti Helen E, D'Armiento Jeanine M, Goldklang Monica P, Eisenberger Andrew B, Griffin Jan M, Maurer Mathew S

机构信息

Department of Medicine, Columbia University Irving Medical Center, New York, NY, USA.

Department of Pathology & Cell Biology, Columbia University Irving Medical Center, New York, NY, USA.

出版信息

Oxf Med Case Reports. 2021 Nov 25;2021(11):omab113. doi: 10.1093/omcr/omab113. eCollection 2021 Nov.

Abstract

Wild-type ATTR cardiac amyloidosis (ATTRwt-CA) is not as rare as previously thought to be. Patients with infiltrative cardiac amyloidosis often present with right-sided heart failure (HF) symptomatology. Clinically significant liver disease and cirrhosis has not been reported in ATTRwt-CA. We present two cases of ATTRwt-CA with right-sided HF and abnormal liver function tests initially thought to be secondary to congestive hepatopathy but found to have rare and unrelated liver disease. These cases highlight the importance of developing a broad differential diagnosis and leveraging a multidisciplinary team approach in evaluating patients for unusual causes of cirrhosis/other chronic liver diseases when ATTR cardiac amyloidosis patients present with congestive hepatopathy.

摘要

野生型转甲状腺素蛋白淀粉样变心肌病(ATTRwt-CA)并不像之前认为的那么罕见。浸润性心脏淀粉样变患者常表现为右心衰竭症状。临床上,尚未有ATTRwt-CA合并显著肝病和肝硬化的报道。我们报告了两例ATTRwt-CA合并右心衰竭及肝功能检查异常的病例,最初认为这是由充血性肝病继发,但后来发现是罕见且不相关的肝病。这些病例凸显了在评估ATTR心脏淀粉样变患者出现充血性肝病时,对于肝硬化/其他慢性肝病的不寻常病因进行广泛鉴别诊断并采用多学科团队方法的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2a00/8633595/6441f3516e67/omab113f1.jpg

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