Nie Qilong, Liang Qiuyan, Li Mingyang, Zhu Ronghuo, Ren Jian, Jiang Kaiping, Li Jianhong
The Eighth Clinical Medical College, Guangzhou University of Chinese Medicine, Foshan, Guangdong, China.
Foshan Hospital of Traditional Chinese Medicine, Guangzhou University of Chinese Medicine, Foshan, Guangdong, China.
Medicine (Baltimore). 2024 Dec 20;103(51):e40642. doi: 10.1097/MD.0000000000040642.
Idiopathic noncirrhotic portal hypertension (INCPH) is a rare liver disorder with elevated portal pressure without cirrhosis, making diagnosis challenging. This case report presents a 46-year-old Chinese male with INCPH, highlighting the crucial role of liver biopsy.
A 46-year-old male presented with persistent fatigue that lasted for 2 months and significantly worsened over the last 3 days. The patient described his fatigue as a profound lack of energy that persisted throughout the day, which progressively impaired his ability to perform daily activities and maintain his usual work responsibilities. He reported feeling exhausted even after light physical exertion, such as walking or standing for short periods. The severity of the fatigue also led to frequent short rests during the day, and he experienced difficulty concentrating and carrying out routine tasks. In addition, he noted a loss of appetite and mild discomfort in the upper abdomen. Given his previous history of abnormal liver function tests and a liver biopsy showing mild chronic liver damage, the patient was initially diagnosed with cirrhosis at a local hospital. This initial diagnosis caused significant emotional distress, as the patient experienced a state of panic and anxiety over the implications of having a progressive liver disease. The psychological burden was evident in his reported difficulty sleeping and persistent worry about his health and future.
Initial imaging suggested portal hypertension and cirrhosis, but a liver biopsy ruled out cirrhotic changes, confirming INCPH by excluding other causes such as chronic hepatitis.
The patient received symptomatic treatment (acid suppression, gastric and liver protection) and underwent a liver biopsy. Histological analysis confirmed INCPH, ruling out cirrhosis.
After the definitive diagnosis, the patient's anxiety lessened. Fatigue and weakness improved with ongoing symptomatic treatment, and psychological support enhanced his overall well-being. His follow-up plan includes regular liver function monitoring, imaging for portal pressure changes, and potential anticoagulation therapy for thrombosis risks.
This case highlights the diagnostic difficulty of INCPH and underscores the importance of liver biopsy. Further research is needed to develop specific diagnostic tools and treatments for INCPH.
特发性非肝硬化性门静脉高压症(INCPH)是一种罕见的肝脏疾病,门静脉压力升高但无肝硬化,这使得诊断具有挑战性。本病例报告介绍了一名46岁的中国男性INCPH患者,强调了肝活检的关键作用。
一名46岁男性出现持续疲劳2个月,在过去3天中明显加重。患者将其疲劳描述为一整天都深感精力不足,这逐渐损害了他进行日常活动和履行日常工作职责的能力。他报告说,即使进行轻度体力活动,如短时间行走或站立后也感到疲惫不堪。疲劳的严重程度还导致他白天频繁短暂休息,并且他难以集中注意力和执行日常任务。此外,他注意到食欲不振和上腹部轻度不适。鉴于他以前肝功能检查异常的病史以及肝活检显示轻度慢性肝损伤,该患者最初在当地医院被诊断为肝硬化。这一初步诊断引起了严重的情绪困扰,因为患者对患有进行性肝病的影响感到恐慌和焦虑。他报告睡眠困难以及对自己的健康和未来持续担忧,这明显体现了心理负担。
初步影像学检查提示门静脉高压和肝硬化,但肝活检排除了肝硬化改变,通过排除慢性肝炎等其他原因确诊为INCPH。
患者接受了对症治疗(抑酸、胃和肝脏保护)并进行了肝活检。组织学分析确诊为INCPH,排除了肝硬化。
明确诊断后,患者的焦虑减轻。通过持续的对症治疗,疲劳和虚弱有所改善,心理支持提高了他的整体幸福感。他的随访计划包括定期监测肝功能、门静脉压力变化的影像学检查以及针对血栓形成风险的潜在抗凝治疗。
本病例突出了INCPH的诊断困难,并强调了肝活检的重要性。需要进一步研究以开发针对INCPH的特定诊断工具和治疗方法。