NON-CIRRHOTIC PORTAL FIBROSIS CAUSING PORTAL HYPERTENSION

2021 ◽  
pp. 9-11
Author(s):  
Praveen. J ◽  
Tumbanatham. A

Portal hypertension is more prevalent in patients with liver cirrhosis and occurs infrequently in those without liver cirrhosis. Non-cirrhotic portal brosis (NCPF) and extrahepatic portal venous obstruction (EHPVO) are the two most common causes of non-cirrhotic portal hypertension. Unlike EHPVO, NCPF does not cause thrombosis of the extrahepatic portal vein. Sclerosis of the portal vein's medium and small branches occurs in NCPF. In NCPF, the hepatic venous pressure gradient (HVPG) is normal, in contrast to cirrhosis, where it is increased. Additionally, NCPF is referred to as non-cirrhotic intrahepatic portal hypertension (NCIPH), idiopathic portal hypertension, hepatoportal sclerosis, and benign intrahepatic portal hypertension. It is a disease with an unknown etiology that primarily affects middle-aged males and females and manifests as hematemesis and massive splenomegaly

2020 ◽  
Vol 4 (7) ◽  
pp. 1296-1306
Author(s):  
Ankur Varma ◽  
Susan C. Abraham ◽  
Rohtesh S. Mehta ◽  
Neeraj Y. Saini ◽  
Medhavi Honhar ◽  
...  

Abstract At our center, we observed a series of patients who developed transudative refractory ascites secondary to noncirrhotic, non-veno-occlusive disease (VOD)–related portal hypertension after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Patients were considered to have idiopathic portal hypertension-related refractory ascites (IRA) if they developed ascites secondary to intrahepatic portal hypertension (serum ascites albumin gradient ≥1.1 g/dL or hepatic venous pressure gradient [HVPG] >5 mm Hg), but did not meet the clinical criteria for classical VOD/sinusoidal obstructive syndrome (SOS) and did not have any alternate etiology of portal hypertension. From our institutional database, we identified 40 patients who developed IRA after allo-HSCT between 2004 and 2018. The patients’ median age at the time of allo-HSCT was 54 years (range, 21-73 years). The median time to development of IRA after allo-HSCT was 80 days (range, 16-576 days). The median number of paracentesis was 3 (range, 1-11), and 15 (38%) patients had an intraperitoneal catheter placed for continued drainage of the rapidly accumulating ascites. Portal pressures were measured in 19 patients; 6 (15%) had moderate portal hypertension (HVPG 6-9 mm Hg), and 13 (33%) had severe portal hypertension (HVPG ≥ 10 mm Hg). Liver biopsy was performed in 24 patients. None of the patients met the criteria for classical VOD/SOS (clinical/histological) or cirrhosis (histological). The cumulative incidence of nonrelapse mortality was 63%, and the median survival duration after the development of the IRA was 7 months (range, 0.8-125.6 months). IRA is a poorly understood and often fatal complication of allo-HSCT.


2002 ◽  
Vol 17 (1) ◽  
pp. 6-16 ◽  
Author(s):  
RADHA KRISHAN DHIMAN ◽  
YOGESH CHAWLA ◽  
RAKESH KUMAR VASISHTA ◽  
NANDITA KAKKAR ◽  
JANG BAHADUR DILAWARI ◽  
...  

2020 ◽  
Vol 3 (31) ◽  
pp. 53-61
Author(s):  
T. Bentsa ◽  

Introduction. Liver cirrhosis (LC) is an important medical and socio-economic problem not only in Ukraine, but throughout the world. The urgency of this disease is due to its significant spread, increase of the number of etiological factors, as well as the occurrence of severe complications, which often leads to death. The prognosis depends on several factors, such as etiology, the severity of liver damage, the presence of complications and concomitant diseases. The aim of the study. To review the scientific literature and summarize the published studies devoted to the study of the etiology, classification, clinical picture and diagnosis of liver cirrhosis. Materials and methods. The content analysis, the method of systemic and comparative analysis, the bibliosemantic method of studying the current scientific research on the etiology, classification, clinical picture and diagnosis of LC were used. The search for sources was carried out in scientometric databases: PubMed-NCBI, Medline, Research Gate, Cochrane Database of Systematic Reviews for the keywords: liver cirrhosis, diagnosis, treatment. 37 literary sources were selected and analyzed. Results. LC is currently ranked 11th among the most common causes of death. The common causes of LC are chronic alcohol intoxication and viral hepatitis B, C, and D. LC is represented by an increase in severity, which is characterized by the lesions of the liver parenchyma with necrosis, dystrophy of hepatocytes, their nodular regeneration, as well as its interstitium with diffuse proliferation of connective tissue, leading to liver failure and portal hypertension. Most patients with cirrhosis remain asymptomatic until they develop decompensated LC. Despite the existence of a number of LC classifications – by morphology, etiology, severity, course, hepatocellular insufficiency stage, the severity of the disease is usually assessed by evaluation of the hepatic functional reserve (according to the C. G. Child – R. N. Pugh classification). Patients with LC often have life-threatening conditions such as variceal hemorrhages, ascites, spontaneous bacterial peritonitis, hepatic encephalopathy, hepatorenal syndrome. Variceal bleeding is a major complication of portal hypertension, which is associated with significant mortality. Ascites represents the most common decompensating event in patients with LC. The appearance of ascites is strongly related to portal hypertension, which leads to splanchnic arterial vasodilation, reduction of the effective circulating volume, activation of endogenous vasoconstrictor systems, and avid sodium and water retention in the kidneys. Bacterial translocation further worsens hemodynamic alterations of patients with cirrhosis and ascites. Ascites is also associated with a high risk of developing the further complications of cirrhosis such as dilutional hyponatremia, spontaneous bacterial peritonitis and/or other bacterial infections and acute kidney injury. Pharmacotherapy for LC should be implemented in accordance with up-to-date guidelines and in conjunction with etiology management, nutritional optimization and patients’ education. The main treatment of uncomplicated ascites is diuretics such as spironolactone in combination with a loop one. Vasoconstrictors and albumin are recommended for the treatment of refractory ascites. In its turn antibiotics play a well-established role in the treatment and prevention of spontaneous bacterial peritonitis. The administration of vasopressor terlipressin and albumin is recommended for the treatment of hepatorenal syndrome. Pharmacological therapy of variceal bleeding aims to decrease the portal pressure by acting on its pathophysiological mechanisms such as increased hepatic vascular tone and splanchnic vasodilatation. Propranolol blocks the β-1 in the heart and the peripheral β-2 adrenergic receptors. β-1 blockade of cardiac receptors reduces heart rate, cardiac output and subsequently decreases flow into splanchnic circulation. β-2 blockade leads to unopposed α-1 adrenergic activity that causes splanchnic vasoconstriction and reduction of portal inflow. Both effects contribute to reduction in portal pressure. Carvedilol is more powerful in reducing hepatic venous pressure gradient than traditional nonselective β-blockers. Endoscopic treatment in many cases is used for the variceal bleeding (eg., ligation of the esophageal varices and tissue glue usage for the gastric varices). A shunt (transjugular intrahepatic portosystemic shunting – TIPS) is used to treat severe and often repeat variceal hemorrhage or refractory ascites. Non-selective β-blockers effectively reduce variceal re-bleeding risk in LC patients with moderate/large varices. Conclusions. Liver cirrhosis is one of the most dangerous multi-organ diseases of a human with multiple pathogenetic links, the causes of which invariably remain hepatitis viruses, alcohol, toxic substances, drugs, ultraviolet radiation, genetic factors, some chronic diseases of the internal organs. There are a number of classifications of liver cirrhosis – by morphology, etiology, severity, course, severity of hepatocellular insufficiency etc. Examination of this category of patients requires timeliness, scrupulousness, compliance with a comprehensive approach using modern clinical, laboratory and instrumental methods. During the objective examination of a patient a doctor traditionally draws attention to the presence of telangiectasia, palmar erythema, jaundice, “raspberry” tongue, scratching marks, gynecomastia in men, ascites and “caput medusae”, during the palpation the liver is enlarged, dense, with a sharp lower edge, spleen is enlarged. Among the laboratory methods, in addition to routine ones, the immunological tests are used, among the main instrumental examination – ultrasound, computed tomography, indirect elastometry of the liver or Fibroscan, esophagogastrofibroscopy, puncture biopsy of the liver, in particular modern ones – vibrational transient elastography and magnetic resonance elastography. Although liver cirrhosis is the final stage of liver disease, this diagnosis cannot be considered a verdict for a patient, because today there are quite effective treatments using the principles of differentiation – the impact on the etiological factor, liver state and comorbid lesions and their complications often allows if not to cure the patient, then to prevent the negative disease course. Among them, there are diet, the use of etiotropic drugs, intestinal sanitation, correction of clinical and laboratory syndromes, portal hypertension syndrome, endothelial and autonomic dysfunction as causes of comorbid lesions and their complications.


2018 ◽  
Vol 84 (6) ◽  
pp. 1033-1038 ◽  
Author(s):  
Guangjin Tian ◽  
Deyu Li ◽  
Haibo Yu ◽  
Yadong Dong ◽  
Huanzhou Xue

This study was performed to evaluate the feasibility of the splenic bed laparoscopic splenectomy approach (SBLS) for massive splenomegaly (≥30 cm) in patients with hypersplenism secondary to portal hypertension and liver cirrhosis. Patients who underwent laparoscopic splenectomy (LS) from January 2012 to December 2016 were retrospectively reviewed. We performed LS in 83 patients with massive splenomegaly (≥30 cm) secondary to portal hypertension and liver cirrhosis. Of these patients, 37 underwent the SBLS and 46 underwent anterior LS (ALS). Five patients in the ALS group and none in the SBLS group underwent conversion to open surgery. The operation time, intraoperative blood loss volume, transfusion volume, frequency of transfusion, hemorrhage of short gastric vessels, conversion rate, postoperative hospital stay, and incidence of pancreatic fistula were all significantly lower in the SBLS than ALS group (all P < 0.05). No death or postoperative bleeding occurred in the two groups, and there were no significant differences in age, gender, spleen size, hemoglobin level, platelet count, prothrombin time, Child-Pugh class, hypoproteinemia, or ascites (all P > 0.05). The SBLS is more feasible and effective than ALS in patients with massive splenomegaly (≥30 cm) secondary to portal hypertension and liver cirrhosis.


Medicina ◽  
2013 ◽  
Vol 49 (11) ◽  
pp. 73 ◽  
Author(s):  
Vilma Silkauskaite ◽  
Jouzas Kupčinskas ◽  
Andrius Pranculis ◽  
Laimas Jonaitis ◽  
Vitalija Petrenkiene ◽  
...  

Background and Objective: Alternative drug therapies are needed for the treatment of portal hypertension.[...]


Radiology ◽  
2009 ◽  
Vol 252 (2) ◽  
pp. 587-594 ◽  
Author(s):  
Hitoshi Maruyama ◽  
Hiroyuki Ishibashi ◽  
Masanori Takahashi ◽  
Fumio Imazeki ◽  
Osamu Yokosuka

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