Clinical profile and pathogenesis of idiopathic non-cirrhotic intra-hepatic portal hypertension (NCIPH)

Goel Ashish Anilkumar, (2018) Clinical profile and pathogenesis of idiopathic non-cirrhotic intra-hepatic portal hypertension (NCIPH). Doctoral thesis, The Tamilnadu Dr. M.G.R. Medical University, Chennai.


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INTRODUCTION: Idiopathic non-cirrhotic intra-hepatic portal hypertension (NCIPH) is a disorder characterised by occlusion of small intra-hepatic portal venous radicles (3rd/ 4th order branches). It is thus a cause of pre-sinusoidal intra-hepatic portal hypertension. NCIPH is clinically characterised by portal hypertension (gastro-oesophageal varices and subsequent bleeding), hypersplenism (low blood counts secondary to peripheral destruction) and liver functions that deteriorate, albeit much later in comparison to cirrhosis1, 2. A proportion of NCIPH patients develop liver failure and require liver transplant. Even the explant liver at the time of transplant grossly gives the appearance of cirrhosis1. AIM OF THE STUDY: The studies will explore the natural history and pathogenesis of idiopathic non-cirrhotic intra-hepatic portal hypertension (NCIPH). We also plan to study the associated disorders in patients with NCIPH. Case definition of NCIPH : For all the studies, diagnosis of NCIPH will be based on the following published criteria : 1. Portal hypertension – evidenced by gastro-oesophageal varices on upper gastrointestinal endoscopy. 2. Patent portal vein (inflow) and hepatic venous outflow tract on Doppler of the intra-abdominal vessels. 3. Absence of known aetiology of chronic liver disease – e.g. Hepatitis B/ C virus, alcohol intake etc. 4. Absence of cirrhosis or advanced fibrosis (bridging fibrosis) on liver biopsy. 5. Absence of any aetiology known to cause portal venous lesions similar to NCIPH – e.g. sarcoidosis, congenital hepatic fibrosis etc. OBJECTIVES: 1. To study the clinical profile, natural history and prognostic factors of patients with NCIPH. 2. To explore pathogenic mechanisms involved in NCIPH. 3. To study the associated disorders in patients with NCIPH. SUMMARY AND CONCLUSION: 1. Non-cirrhotic intra-hepatic portal hypertension (NCIPH) remains a common cause of portal hypertension in 21st century India. 2. The overall prognosis in these patients remains good, but these patients are prone to compensation, heptocellular carcinoma and portal vein thrombosis. 3. Imbalance of von-Willebrand factor and its cleaving protease (ADAMTS-13), despite normal liver function, suggestsa possibility of micro-angiopathy involving small portal venous radicles as the pathogenetic mechanism involved in NCIPH causation. 4. Over-representation for gut disorders, often non-specific inflammaton which is clinically silent, suggests the important role of GI tract in the pathogenesis of NCIPH. This may also partly explain the increased prevalence of NCIPH in India, esp. in the lower socio-economic strata. 5. Continued ingestion of arsenic contaminated groundwater may be one of the pathogenic mechanisms in NCIPH, esp. in patients hailing from regions near ganges.

Item Type: Thesis (Doctoral)
Uncontrolled Keywords: non-cirrhotic intra-hepatic portal hypertension (NCIPH), Clinical profile, pathogenesis, idiopathic.
Subjects: Respiratory Medicine > Hepatology
> Respiratory Medicine > Hepatology
Depositing User: Subramani R
Date Deposited: 12 Nov 2019 05:46
Last Modified: 24 Oct 2022 13:48

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