Mukta V, Panicker Lakshmi C, Sivamani Kalaimani, Goel Amit, Basu Debdutta, Dhanapathi Halnayak
Ex-Associate Professor, Associate Professor, Department of Medicine JIPMER, Puducherry, India
Ex-Assistant Professor, Department of Gastroenterology JIPMER, Puducherry, India.
Trop Doct. 2017 Jan;47(1):26-30. doi: 10.1177/0049475516636982. Epub 2016 Mar 16.
Non-cirrhotic portal fibrosis (NCPF) is one of the important causes of upper gastrointestinal haemorrhage in patients in tropical countries. The aim of this study was to describe the clinical and laboratory profile of 68 patients with NCPF.
NCPF is defined as liver disease with: (1) evidence of portal hypertension; (2) a liver biopsy showing no cirrhosis or a Tc-labelled sulphur colloid scan showing a pattern suggestive of NCPF; and (3) a patent splenoportal axis. The clinical, laboratory and demographic features of 68 patients with such criteria were studied and analysed.
NCPF was common in women (73.5%) in the fourth decade of life. The median duration of illness was 24 months (range, 1 month-28 years). Patients presented to hospital with the sensation of a mass in the abdomen (50%) or with haematemesis (26.5%). They had splenomegaly (95.6%) and thrombocytopenia (88.2%). The majority of patients had normal liver function tests. Abdominal ultrasonography showed increased periportal and peri gallbladder echoes (72%), spontaneous collaterals (41.2%) and ascites (19.1%). Liver biopsy revealed portal venous sclerosis (76.3%) and periportal fibrosis (55.3%). Tc-labelled sulphur colloid scan was suggestive of NCPF in the remaining 30 cases.
NCPF is common in South India. Transient ascites occurs due to decompensation of liver function after variceal bleeding and in long standing cases of NCPF. Our study used Tc-sulphur scan for diagnosing NCPF in patients where liver biopsy was contraindicated in view of severe thrombocytopenia; however, the diagnostic utility of Tc-sulphur nuclear scan to diagnose NCPF in patients with severe hypersplenism needs to be further evaluated in future studies.
非肝硬化性门脉纤维化(NCPF)是热带国家患者上消化道出血的重要原因之一。本研究旨在描述68例NCPF患者的临床和实验室特征。
NCPF定义为具有以下特征的肝脏疾病:(1)门静脉高压的证据;(2)肝活检显示无肝硬化或锝标记硫胶体扫描显示提示NCPF的模式;(3)脾门静脉轴通畅。对符合这些标准的68例患者的临床、实验室和人口统计学特征进行了研究和分析。
NCPF在40岁女性中常见(73.5%)。疾病的中位持续时间为24个月(范围1个月至28年)。患者因腹部肿块感(50%)或呕血(26.5%)入院。他们有脾肿大(95.6%)和血小板减少(88.2%)。大多数患者肝功能检查正常。腹部超声显示门静脉周围和胆囊周围回声增强(72%)、自发性侧支循环(41.2%)和腹水(19.1%)。肝活检显示门静脉硬化(76.3%)和门静脉周围纤维化(55.3%)。其余30例经锝标记硫胶体扫描提示为NCPF。
NCPF在印度南部常见。由于静脉曲张破裂出血后肝功能失代偿以及NCPF的长期病例会出现短暂性腹水。我们的研究使用锝硫扫描对因严重血小板减少而禁忌肝活检的患者诊断NCPF;然而,锝硫核素扫描在诊断严重脾功能亢进患者NCPF中的诊断效用需要在未来研究中进一步评估。