Liver involvement is often observed in hematological disorders, resulting in liver abnormality, including unconjugated hyperbilirubinemia, monoclonal hyperglobulinemia, portal vein, or hepatic vein thrombosis or portal hypertension, hepatosplenomegaly, or iron accumulation in the liver. Here we summarize the major hematological diseases that often affect the liver: hemolytic anemia, defect in coagulation or anti-coagulation factors, myeloproliferative neoplasm, hemophagocytic lymphohistiocytosis, multiple myeloma, leukemia, and lymphoma. We hope this review will help clinicians diagnose and manage the patients with liver involvement by hematological disorders.
Hematologic Diseases ; Humans ; Hypertension, Portal ; Myeloproliferative Disorders/diagnosis* ; Portal Vein/pathology*

Chronic liver disease is a major disorder worldwide. A better understanding of anatomy, blood flow, and pathophysiology may be a key issue for their proper management. Ultrasound (US) is a simple and non-invasive diagnostic tool in the abdominal field. Doppler mode offers real-time hemodynamic evaluation, and the contrast-enhanced US is one of the most frequently used modalities for the detailed assessment. Further development in digital technology enables three-dimensional (3D) visualization of target images with high resolution. This article reviews the wide ranges of application in the abdominal US and describes the recent progress in the diagnosis of chronic liver diseases.
Diagnosis ; Fibrosis ; Hemodynamics ; Hypertension, Portal ; Liver Diseases ; Liver ; Ultrasonography

Ascites is the most common cause of decompensation in cirrhosis, and 5% to 10% of patients with compensated cirrhosis develop ascites each year. The main factor of ascites formation is renal sodium retention due to activation of the renin-angiotensin-aldosterone system and sympathetic nervous system by the reduced effective volume secondary to splanchnic arterial vasodilation. Diagnostic paracentesis is indicated in all patients with a new onset of grade 2 or 3 ascites and in those admitted to hospital for any complication of cirrhosis. A serum-ascites albumin gradient of ≥1.1 g/dL indicates portal hypertension with an accuracy of approximately 97%. Sodium restriction, diuretics, and large volume paracentesis are the mainstay of treatment in grade 1 to 3 ascites. The refractoriness of ascites is associated with a poor prognosis with a median survival of approximately six months. Repeated large volume paracentesis plus albumin is the first line treatment, and liver transplantation is recommended in patients with refractory ascites. A careful selection of patients is also important to obtain the beneficial effects of transjugular intrahepatic portosystemic shunts in refractory ascites. This review details the recent diagnosis and treatment of cirrhotic ascites.
Ascites* ; Diagnosis ; Diuretics ; Fibrosis ; Humans ; Hypertension, Portal ; Liver Cirrhosis ; Liver Transplantation ; Paracentesis ; Portasystemic Shunt, Surgical ; Prognosis ; Renin-Angiotensin System ; Sodium ; Sympathetic Nervous System ; Vasodilation

Accessory hepatic lobe is noted as and considered a rare disease in children. It can manifest with various symptoms and complications depending on the location, volume, type and position of the disease as presented on a child. The patient presented as a 14-month-old girl who was seen with a notable hepatosplenomegaly and portal hypertension. A diagnosis was made after taking an extensive medical history, observation and radiological examinations. The formal diagnosis was a prehepatic portal hypertension associated with accessory hepatic lobe.
Child* ; Diagnosis ; Female ; Humans ; Hypertension, Portal* ; Infant ; Rare Diseases

A 2-year-old, spayed female, Korean domestic short-hair cat was presented with depression and vomiting. The patient had history of weight loss lasting seven months. Physical examination revealed icterus in the pinna, oral mucosa, and sclera. Based on ultrasonography and computed tomography, tentative diagnosis was extrahepatic biliary tract obstruction with acquired portosystemic shunt (PSS). Tumor or inflammation of hepatobiliary system was suspected as the cause of obstruction of the common bile duct. But it could not be determined without biopsy. The severely dilated cystic duct was considered to cause portal hypertension and secondary multiple PSS. The patient expired without histopathologic examination.
Animals ; Biliary Tract ; Biopsy ; Cats ; Child, Preschool ; Cholestasis, Extrahepatic ; Common Bile Duct ; Cystic Duct ; Depression ; Diagnosis ; Female ; Humans ; Hypertension, Portal ; Inflammation ; Jaundice ; Mouth Mucosa ; Physical Examination ; Portasystemic Shunt, Surgical ; Sclera ; Ultrasonography ; Vomiting ; Weight Loss

Arteriovenous fistula (AVF) involving the inferior mesenteric artery and vein is very rare with only 33 cases described in the literature and may be of congenital or acquired (iatrogenic or traumatic) or idiopathic etiology. The pathophysiology of AVF that acts as a left-to-right shunt has accounted for clinical signs and symptoms associated with ischemic colitis, portal hypertension, and heart failure. A low incidence and nonspecific clinical signs and symptoms such as abdominal pain, thrill and mass, lower and upper gastrointestinal bleeding make it difficult to establish a diagnosis of inferior mesenteric AVF. Diagnosis of inferior mesenteric AVF is usually established by radiological or intraoperative examination. We report a case of idiopathic inferior mesenteric AVF causing ischemic colitis in a 56-year-old man that was diagnosed preoperatively by multidetector computed tomography and angiography and successfully treated by surgical resection.
Abdominal Pain ; Angiography ; Arteriovenous Fistula* ; Colitis, Ischemic ; Diagnosis ; Heart Failure ; Hemorrhage ; Humans ; Hypertension, Portal ; Incidence ; Mesenteric Artery, Inferior ; Middle Aged ; Multidetector Computed Tomography ; Veins

Acute kidney injury (AKI) is one of the most common manifestations encountered in clinical practice. It is associated with high morbidity and mortality in cirrhotic pre- and post-transplantation patients. Hepatorenal syndrome (HRS), a special form of AKI in cirrhotic patients, was recognized as a consequence of renal vasoconstriction from systemic/renal hemodynamic alterations developed in advanced cirrhosis with portal hypertension. Recently, multiple factors—such as infection/inflammation, underlying glomerulonephritis, bile cast, or increased abdominal pressure—have been considered to contribute to renal dysfunction in cirrhotic patients, which were presumed to induce HRS. Moreover, in addition to changing the definition of AKI in the nephrologic guidelines, the new AKI definition for early diagnosis and intervention based on characteristics of liver cirrhosis has been proposed in an international meeting. This article provides a comprehensive and recent review of AKI definition, laying out the topics in accordance with the pathophysiologic mechanisms and therapeutic interventions of AKI in cirrhotic patients with portal hypertension.
Acute Kidney Injury* ; Bile ; Early Diagnosis ; Fibrosis ; Glomerulonephritis ; Hemodynamics ; Hepatorenal Syndrome ; Humans ; Hypertension, Portal* ; Liver Cirrhosis ; Mortality ; Vasoconstriction

BACKGROUNDBalanced adjustment of the portal vein shunt volume during a transjugular intrahepatic portosystemic shunt (TIPS) is critical for maintaining liver perfusion and decreasing the incidence of liver insufficiency. A stent-graft is proved to be superior to a bare metal stent (BMS) for the construction of a TIPS. However, the clinical results of the combination application of stents and stent-grafts have not been determined. This study aimed to compare the technique of using a combination of stents and stent-grafts with using a single stent-graft to construct a TIPS.

METHODSFrom April 2011 to November 2014, a total of fifty patients were randomly assigned to a stents-combination group (Group I, n = 28) or a stent-graft group (Group II, n = 22). Primary patency rates were calculated. Clinical data, including the technical success rate, bleeding control results, incidence of encephalopathy, liver function preservation, and survival rate, were assessed.

RESULTSTechnically, the success rate was 100% for both groups. The primary patency rates at 1, 2, and 3 years for Group I were 96%, 84%, and 77%, respectively; for Group II, they were 90%, 90%, and 78%, respectively. The survival rates at 1, 2, and 3 years for Group I were 79%, 74%, and 68%, respectively; for Group II, they were 82%, 82%, and 74%, respectively. The incidence of hepatic encephalopathy was 14.3% for Group I and 13.6% for Group II. The Child-Pugh score in Group I was stable at the end of the follow-up but had significantly increased in Group II (t = -2.474, P = 0.022).

CONCLUSIONSThe construction of a TIPS with either the single stent-graft or BMS/stent-graft combination is effective for controlling variceal bleeding. The BMS/stent-graft combination technique is superior to the stent-graft technique in terms of hepatic function preservation indicated by the Child-Pugh score. However, considering the clinical results of the TIPS, the two techniques are comparable in their primary shunt patency, incidence of encephalopathy and patient survival during the long-term follow-up.

Aged ; Female ; Hepatic Encephalopathy ; diagnosis ; etiology ; Humans ; Hypertension, Portal ; complications ; mortality ; surgery ; Male ; Middle Aged ; Polytetrafluoroethylene ; Portal Vein ; surgery ; Portasystemic Shunt, Transjugular Intrahepatic ; adverse effects ; methods ; Postoperative Complications ; Prospective Studies ; Stents ; Treatment Outcome

Recent advances in the noninvasive imaging of chronic liver disease have led to improvements in diagnosis, particularly with magnetic resonance imaging (MRI). A comprehensive evaluation of the liver may be performed with the quantification of the degree of hepatic steatosis, liver iron concentration, and liver fibrosis. In addition, MRI of the liver may be used to identify complications of cirrhosis, including portal hypertension, ascites, and the development of hepatocellular carcinoma. In this review article, we discuss the state of the art techniques in liver MRI, namely, magnetic resonance elastography, hepatobiliary phase MRI, and liver fat and iron quantification MRI. The use of these advanced techniques in the management of chronic liver diseases, including non-alcoholic fatty liver disease, will be elaborated.
Ascites ; Carcinoma, Hepatocellular ; Diagnosis ; Elasticity Imaging Techniques* ; Fatty Liver ; Fibrosis ; Hypertension, Portal ; Iron ; Liver Cirrhosis ; Liver Diseases* ; Liver* ; Magnetic Resonance Imaging* ; Non-alcoholic Fatty Liver Disease

Portal biliopathy is defined as abnormalities in the extra- and intrahepatic ducts and gallbladder of patients with portal hypertension. This condition is associated with extrahepatic venous obstruction and dilatation of the venous plexus of the common bile duct, resulting in mural irregularities and compression of the biliary tree. Most patients with portal biliopathy remain asymptomatic, but approximately 10% of them advance to symptomatic abdominal pain, jaundice, and fever. Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography are currently used as diagnostic tools because they are noninvasive and can be used to assess the regularity, length, and degree of bile duct narrowing. Management of portal biliopathy is aimed at biliary decompression and reducing the portal pressure. Portal biliopathy has rarely been reported in Korea. We present a symptomatic case of portal biliopathy that was complicated by cholangitis and successfully treated with biliary endoscopic procedures.
Abdomen/diagnostic imaging ; Cholangiopancreatography, Endoscopic Retrograde ; Cholestasis/*diagnosis ; Humans ; Hypertension, Portal/*diagnosis ; Male ; Middle Aged ; Portal Vein ; *Stents ; Tomography, X-Ray Computed