Clinical Diagnosis and Pathological Spectrum of Porto-sinusoidal Vascular Disease in India
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 发起方
- 入组人数
- 210
- 试验地点
- 1
- 主要终点
- To describe the clinical presentation of patients with a diagnosis of PSVD.
研究概览
简要总结
There is a need to re-evaluate the patients classified as NCPH and determine whether the new histological classification proposed by the VALDIG applies to the Indian scenario. We intend to identify the patient cohorts who have been diagnosed as NCPH, NCPF, EHPVO, hepatic venous outlet tract obstruction (HVOTO), Veno-occlusive disease (VOD) and sinusoidal obstruction syndrome (SOS) based on their liver biopsy, endoscopy, HVPG, and radiology reports. These patients will be screened to find the patients who fit the diagnosis of PSVD. It is important to establish whether the new definition of PSVD is relevant to the Indian population and establish the usefulness of invasive tests like liver biopsy in diagnosing the disease. The patient cohorts meeting diagnosis of INCPH will be compared with those meeting the new diagnosis of PSVD. The investigators will describe the clinical (demographic, clinical risk factors, socioeconomic status), etiological (associated conditions, coagulation disorders medication use, genetic risk factors), imaging (based on ultrasound Doppler imaging or cross- sectional imaging), endoscopic, fibrosis tests (using non-invasive tests), and the histopathology of the patients who fulfil the criteria of PSVD.
详细描述
Portal hypertension is clinically characterized by a portal venous pressure gradient between the inferior vena cava and the portal vein is more than 5 mm. Liver cirrhosis remains the leading cause of portal hypertension in the western world. However, there are conditions where portal hypertension can even occur without liver cirrhosis, referred to as Noncirrhotic portal hypertension (NCPH). The term NCPH has been derived from a variety of histopathological entities such as hepatoportal sclerosis, noncirrhotic portal fibrosis, nodular regenerative hyperplasia, or incomplete septal fibrosis. In the absence of other causes of cirrhosis and portal venous thrombosis, the condition is termed idiopathic noncirrhotic portal hypertension. The histopathological findings of INCPH are not entirely specific to portal hypertension, similar changes can be seen in patients without portal hypertension. Therefore the term Porto-sinusoidal vascular disorder(PSVD) has been recently introduced to define a group of liver vessel disorders affecting the portal venules and sinusoids irrespective of the absence or presence of liver cirrhosis. This disease entity can have a varied clinical presentation and histological findings and has been associated with several immunological and systemic disorders.
Porto-sinusoidal vascular disease - Currently, for clinical practice, the VALDIG group has given a working definition for PSVD.
"Liver biopsy ≥20mm without cirrhosis with either 1 specific sign of portal hypertension OR 1 specific histological finding for PSVD" OR "Liver biopsy ≥20mm without cirrhosis with 1 sign not specific for portal hypertension AND 1 sign not specific for PSVD"
研究设计
- 研究类型
- Observational
- 观察模型
- Case Only
- 时间视角
- Prospective
入排标准
- 年龄范围
- 12 Years 至 70 Years（Child, Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Patients of 12-70 years, either gender with clinical, pathological radiological diagnosis of NCPH
排除标准
- •Any patients having Cirrhosis based on clinical, pathological, or radiological diagnosis
- •Any patients having active malignancy
结局指标
主要结局
To describe the clinical presentation of patients with a diagnosis of PSVD.
时间窗: Day 0
Listing clinical presentation (signs and symptoms) of patients with PSVD (variceal bleeding, pain, ascites, sensation of lump, etc)
次要结局
- Radiological description of patients with PSVD based on Doppler Ultrasound(Day 0)
- Radiological description of patients with PSVD based on CT venography(Day 0)
- Presence of varices and other endoscopic findings in patients with PSVD(Day 0)
- Histopathological features and subtypes of the PSVD spectrum in patients from India with assessment of parenchyma, vessels, portal triad and fibrosis(Day 0)
- Ratio of liver stiffness and splenic stiffness using transient elastography in diagnosis of PSVD(Day 0)
- Change in biomarkers like Factor VIII Ag or vWF in diagnosis of PSVD(Day 0)
研究者
Madhumita Premkumar
ASSOCIATE PROFESSOR
Post Graduate Institute of Medical Education and Research, Chandigarh