PDF(Pubmed)
Abstract：
UNASSIGNED: Porto-sinusoidal vascular syndrome is characterised by specific histological changes that do not include cirrhosis, with or without portal hypertension. Patients are usually asymptomatic until development of portal hypertension complications.
UNASSIGNED: A 69-year-old female with history of JAK2 positive essential thrombocythemia (ET) was referred to internal medicine consultation due to elevated liver enzymes. The patient had no previous history of liver disease. Seven months earlier, she had an ischaemic stroke and started treatment with atorvastatin. After discontinuing medication, liver enzymes returned to normal and atorvastatin-related drug-induced liver disease (DILI) was presumed.During a follow-up visit, iron deficiency anaemia was detected and an endoscopic study was performed. It revealed a gastric varix actively bleeding, which was successfully treated with cyanoacrylate.Two months later, the patient was admitted due to a new episode of variceal bleeding, and a portal hypertension complementary study was made.
UNASSIGNED: Although the pathogenesis of porto-sinusoidal vascular disease (PSVD) remains poorly understood, vascular changes within the liver have been associated with several predisposing conditions, such as hypercoagulable states. Patients with ET, especially those with JAK2 mutation, are known to be at increased risk of non-cirrhotic vein thrombosis. Concerning PSVD, the association is not clear but it is believed that both PSVD and myeloproliferative neoplasms share a common denominator: a state characterised by hypercoagulability, inflammation, endothelial dysfunction and, in some cases, portal hypertension.
UNASSIGNED: Portal hypertension without cirrhosis is a rare condition, presenting diagnostic challenges and significant impact on the patient\'s prognosis.
CONCLUSIONS: The suspicion of PSVD should be raised when signs of portal hypertension are present with normal or mildly elevated liver enzymes and normal liver stiffness measurement. A liver biopsy should be performed in this situation.Although the pathogenesis of PSVD is not clearly understood, it is based on the development of vascular changes within the liver and there might be several predisposing conditions such as coagulation disorders.
UNASSIGNED: A 69-year-old female with history of JAK2 positive essential thrombocythemia (ET) was referred to internal medicine consultation due to elevated liver enzymes. The patient had no previous history of liver disease. Seven months earlier, she had an ischaemic stroke and started treatment with atorvastatin. After discontinuing medication, liver enzymes returned to normal and atorvastatin-related drug-induced liver disease (DILI) was presumed.During a follow-up visit, iron deficiency anaemia was detected and an endoscopic study was performed. It revealed a gastric varix actively bleeding, which was successfully treated with cyanoacrylate.Two months later, the patient was admitted due to a new episode of variceal bleeding, and a portal hypertension complementary study was made.
UNASSIGNED: Although the pathogenesis of porto-sinusoidal vascular disease (PSVD) remains poorly understood, vascular changes within the liver have been associated with several predisposing conditions, such as hypercoagulable states. Patients with ET, especially those with JAK2 mutation, are known to be at increased risk of non-cirrhotic vein thrombosis. Concerning PSVD, the association is not clear but it is believed that both PSVD and myeloproliferative neoplasms share a common denominator: a state characterised by hypercoagulability, inflammation, endothelial dysfunction and, in some cases, portal hypertension.
UNASSIGNED: Portal hypertension without cirrhosis is a rare condition, presenting diagnostic challenges and significant impact on the patient\'s prognosis.
CONCLUSIONS: The suspicion of PSVD should be raised when signs of portal hypertension are present with normal or mildly elevated liver enzymes and normal liver stiffness measurement. A liver biopsy should be performed in this situation.Although the pathogenesis of PSVD is not clearly understood, it is based on the development of vascular changes within the liver and there might be several predisposing conditions such as coagulation disorders.
摘要：
■窦口血管综合征的特征是不包括肝硬化的特定组织学变化，有或没有门静脉高压症。患者通常无症状，直到门静脉高压并发症发展。
■一名69岁女性，有JAK2阳性原发性血小板增多症（ET）病史，由于肝酶升高，被转诊至内科咨询。患者既往无肝病史。七个月前,她患有缺血性卒中,开始接受阿托伐他汀治疗.停药后，肝酶恢复正常,推测与阿托伐他汀相关的药物性肝病(DILI).在后续访问中，检测到缺铁性贫血,并进行了内镜研究.它显示胃静脉曲张积极出血，成功用氰基丙烯酸酯处理。两个月后,该患者因静脉曲张破裂出血而入院，并进行了门静脉高压症的补充研究。
■尽管对门窦血管疾病（PSVD）的发病机制知之甚少，肝脏内的血管变化与几种易感疾病有关，如高凝状态。ET患者,尤其是那些有JAK2突变的人，已知非肝硬化静脉血栓形成的风险增加。关于PSVD，这种关联尚不清楚，但人们认为PSVD和骨髓增殖性肿瘤有一个共同点：一种以高凝状态为特征的状态，炎症,内皮功能障碍和,在某些情况下,门静脉高压症。
■没有肝硬化的门脉高压是一种罕见的疾病，提出诊断挑战和对患者预后的重大影响。
结论：当门脉高压征象出现正常或轻度升高的肝酶和正常的肝硬度测量时，应增加对PSVD的怀疑。在这种情况下应进行肝活检。虽然PSVD的发病机制尚不清楚,它是基于肝脏内血管变化的发展，可能有几种诱发疾病，如凝血障碍。
■一名69岁女性，有JAK2阳性原发性血小板增多症（ET）病史，由于肝酶升高，被转诊至内科咨询。患者既往无肝病史。七个月前,她患有缺血性卒中,开始接受阿托伐他汀治疗.停药后，肝酶恢复正常,推测与阿托伐他汀相关的药物性肝病(DILI).在后续访问中，检测到缺铁性贫血,并进行了内镜研究.它显示胃静脉曲张积极出血，成功用氰基丙烯酸酯处理。两个月后,该患者因静脉曲张破裂出血而入院，并进行了门静脉高压症的补充研究。
■尽管对门窦血管疾病（PSVD）的发病机制知之甚少，肝脏内的血管变化与几种易感疾病有关，如高凝状态。ET患者,尤其是那些有JAK2突变的人，已知非肝硬化静脉血栓形成的风险增加。关于PSVD，这种关联尚不清楚，但人们认为PSVD和骨髓增殖性肿瘤有一个共同点：一种以高凝状态为特征的状态，炎症,内皮功能障碍和,在某些情况下,门静脉高压症。
■没有肝硬化的门脉高压是一种罕见的疾病，提出诊断挑战和对患者预后的重大影响。
结论：当门脉高压征象出现正常或轻度升高的肝酶和正常的肝硬度测量时，应增加对PSVD的怀疑。在这种情况下应进行肝活检。虽然PSVD的发病机制尚不清楚,它是基于肝脏内血管变化的发展，可能有几种诱发疾病，如凝血障碍。
