Signs Of Portal Hypertension Research Articles

Early portal vein thrombosis after hepatectomy for perihilar cholangiocarcinoma: Incidence, risk factors, and management

To study the incidence, risk factors and management of portal vein thrombosis (PVT) after hepatectomy for perihilar cholangiocarcinoma (PHCC). Single-center retrospective analysis of 86 consecutive patients who underwent major hepatectomy for PHCC, between 2012 and 2019, with comparison of the characteristics of the groups with (PVT+) and without (PVT-) postoperative portal vein thrombosis. Seven patients (8%) presented with PVT diagnosed during the first postoperative week. Preoperative portal embolization had been performed in 71% of patients in the PVT+ group versus 34% in the PVT- group (P=0.1). Portal reconstruction was performed in 100% and 38% of PVT+ and PVT- patients, respectively (P=0.002). In view of the gravity of the clinical and/or biochemical picture, five (71%) patients underwent urgent re-operation with portal thrombectomy, one of whom died early (hemorrhagic shock after surgical treatment of PVT). Two patients had exclusively medical treatment. Complete recanalization of the portal vein was achieved in the short and medium term in the six survivors. After a mean follow-up of 21 months, there was no statistically significant difference in overall survival between the two groups. Post-hepatectomy PVT for PHCC is a not-infrequent and potentially lethal event. Rapid management, adapted to the extension of the thrombus and the severity of the thrombosis (hepatic function, signs of portal hypertension) makes it possible to limit the impact on postoperative mortality. We did not identify any modifiable risk factor. However, when it is oncologically and anatomically feasible, left±extended hepatectomy (without portal embolization) may be less risky than extended right hepatectomy, and portal vein resection should only be performed if there is strong suspicion of tumor invasion.

The case report of Abernethy malformation type Ib in an adult patient with pulmonary arterial hypertension

This clinical case illustrates the diagnosis of a rare congenital pathology – an adult patient with pulmonary arterial hypertension and Abernethy malformation type Ib.Clinical observation: Patient S., 45 years old, with pulmonary arterial hypertension, was admitted with complaints of shortness of breath during intense physical exertion. Based on the anamnesis morbi, the diagnosis of idiopathic pulmonary arterial hypertension has been verified since 2017. Results of comprehensive examination: functional status (six-minute walk test), NTpro-BNP level, chest x-ray, echocardiography, right heart catheterization were assessed during the hospitalization. According to these there were signs of the high pulmonary arterial hypertension with systolic pressure in the lung arteries 68-70 mm Hg, extension of the trunk, right and left branches of the pulmonary artery. In order to exclude objective evidence of cardiac dysfunction, portal hypertension, an abdominal ultrasound was performed. We didn’t obtaine the information about an increase in central venous pressure, the presence of signs of portal hypertension, such as ascites, increased pressure in the portal system. However, echo signs of congenital pathology were determined - Abernethy malformation type Ib: porto-caval shunt between the superior mesenteric vein and the inferior vena cava. According to multispiral computed tomography of abdominal organs with contrast: the superior mesenteric vein and splenic vein join to form a short extra-hepatic portal vein which drains into a systemic vein (inferior vena cava). Taking into account the results of risk stratification, the patient underwent an escalation of pathogenetic therapy for the treatment of pulmonary arterial hypertension, and dynamic monitoring was recommended.Conclusion: The presented clinical case is unique in terms of diagnosing a rare congenital pathology of the hepatic blood flow – Abernethy malformation type Ib in an adult patient with pulmonary arterial hypertension.

Congenital Hepatic Fibrosis as an Early Sign of Presentation of ADPKD.

Autosomal dominant polycystic kidney disease (ADKPD) is the most frequent type of polycystic kidney disease. It is inherited through family members, with an incidence of approximately 1:400 to1:1000.Typically, individuals with ADKPD are identified between their fourth and fifth decade of life. ADKPD occurs as a results of mutation in one of the two genes, PDK1 and PDK2.Patients with PKD1 experience renal failure at an earlier onset than those with PKD2. We report on a 2 year-old-boy with hepatosplenomegaly and signs of portal hypertension. Both kidneys appeared normal until the age of 8, when multiple cysts developed, this being typical of ADKPD. Suspecting ADKPD, we performed whole exome sequencing, thereby confirming a mutation of c.6730 673del p.(Ser 2244Hisfs*17). The investigations of all family members found other individuals affected by ADKPD.

The Power of Share Wave Elastography in Evaluating Portal Hypertension Signs in Children

The Power of Share Wave Elastography in Evaluating Portal Hypertension Signs in Children

Recall patterns and risk of primary liver cancer for subcentimeter ultrasound liver observations: a multicenter study

Patients with cirrhosis and subcentimeter lesions on liver ultrasound are recommended to undergo short-interval follow-up ultrasound because of the presumed low risk of primary liver cancer (PLC). The aim of this study is to characterize recall patterns and risk of PLC in patients with subcentimeter liver lesions on ultrasound. We conducted a multicenter retrospective cohort study among patients with cirrhosis or chronic hepatitis B infection who had subcentimeter ultrasound lesions between January 2017 and December 2019. We excluded patients with a history of PLC or concomitant lesions ≥1cm in diameter. We used Kaplan Meier and multivariable Cox regression analyses to characterize time-to-PLC and factors associated with PLC, respectively. Of 746 eligible patients, most (66.0%) had a single observation, and the median diameter was 0.7cm (interquartile range: 0.5-0.8cm). Recall strategies varied, with only 27.8% of patients undergoing guideline-concordant ultrasound within 3-6 months. Over a median follow-up of 26 months, 42 patients developed PLC (39 HCC and 3 cholangiocarcinoma), yielding an incidence of 25.7 cases (95% CI, 6.2-47.0) per 1000 person-years, with 3.9% and 6.7% developing PLC at 2 and 3 years, respectively. Factors associated with time-to-PLC were baseline alpha-fetoprotein >10ng/mL (HR: 4.01, 95% CI, 1.85-8.71), platelet count ≤150 (HR: 4.90, 95% CI, 1.95-12.28), and Child-Pugh B cirrhosis (vs. Child-Pugh A: HR: 2.54, 95% CI, 1.27-5.08). Recall patterns for patients with subcentimeter liver lesions on ultrasound varied widely. The low risk of PLC in these patients supports short-interval ultrasound in 3-6 months, although diagnostic CT/MRI may be warranted for high-risk subgroups such as those with elevated alpha-fetoprotein levels.

Relationship of cytokine gene polymorphism and the development of liver fibrosis in patients with chronic hepatitis B&C

One of the features of the spread of infectious diseases is an increase in the number of mixed infections caused by various types of microorganisms: bacteria, viruses, protozoa, fungi, mycoplasmas, chlamydia, etc. In mixed infections, the simultaneous presence of several pathogens leads to not only to the complex interaction of pathological processes, but also complicates the diagnosis and choice of treatment. Mixed hepatitis is characterized by high biochemical activity and histological changes, the presence of signs of portal hypertension. Infection with two viruses increases the risk of developing hepatocellular carcinoma. It should be noted that in patients with HBV infection, the development of hepatocellular carcinoma is possible at the stage of chronic hepatitis before the onset of liver cirrhosis. The aim of the study: to investigate a comprehensive assessment of the allelic polymorphism of the IL-4 (rs2243250), IL-10 (rs1800896), TNFα (rs1800620), SMAD family member 7 (rs4939827) and eIF3h (rs16892766) genes with the degree of fibrosis in patients with chronic hepatitis B&C. Materials and methods. In the course of the study, 62 patients with chronic hepatitis B&C were examined before the start of treatment and after the end of the course of antiviral therapy. The degree of liver fibrosis was established by FibroScan. Molecular genetic studies included the determination of polymorphic variants of the IL-4 (rs2243250), IL10 (rs1800896), TNFα (rs1800620), SMAD family member 7 (rs4939827), eIF3h (rs16892766) genes. Polymorphism was studied by amplification of the corresponding regions of the genome by PCR. Results and discussions. Genotypes CC IL-4 (rs2243250), GG IL-10 (rs1800896), GG TNFα (rs1800620), AA SMAD family member 7 (rs4939827), TT eIF3h (rs16892766) have a protective effect on the course of chronic hepatitis B&C, as they are established in patients with chronic hepatitis B&C with a lower degree of fibrosis. Heterozygous CT IL-4 (rs2243250), GA IL-10 (rs1800896), GA TNFα (rs1800620), CC SMAD family member 7 (rs4939827), CC eIF3h (rs16892766) have a profibrotic effect on the course of chronic hepatitis B&C, therefore that they are established in patients with chronic hepatitis B&C with a greater degree of fibrosis.In patients with a low stage of fibrosis, the genotypes CC IL-4 (rs2243250), GG IL-10 (rs1800896), GG TNFα (rs1800620), AA SMAD family member 7 (rs4939827), TT eIF3h (rs16892766), are most often determined. In patients with a degree of fibrosis, other genotypes were detected, namely CT IL-4 (rs2243250), GA IL-10 (rs1800896), GA TNFα (rs1800620), CC SMAD family member 7 (rs4939827), CC eIF3h (rs16892766). Сonclusions. The revealed differences in the frequency of occurrence of various gene polymorphisms in healthy subjects and patients with chronic hepatitis indicate a certain predisposition to the development of a chronic inflammatory process in the liver.

PRIMARY SCLEROSING CHOLANGITIS AND AUTOIMMUNE HEPATITIS: OVERLAPPING CHALLENGES IN DIAGNOSIS AND TREATMENT

Abstract Overlap syndromes (OS) describe the simultaneous presentation of more than one immune-mediated liver disease in a singular patient, and present unique challenges both in diagnosis and treatment. Here we report a case of primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) OS, ultimately treated with Vancomycin. A 30-year-old female with known ulcerative pancolitis (UC) and alcoholic cirrhosis in the setting of AIH presented to the ED with severe abdominal pain, bloody diarrhea, and bilious emesis. Initial labs showed ESR 48 mm/hr, CRP 32.97 mg/L, fecal calprotectin 1131.2 µg/mg, AST 45 IU/L, and total protein 9.1 g/dL. Clostridium Difficile and stool studies were negative. Patient was admitted with concern for acute UC flare. CT scan showed mild worsening of intrahepatic and extrahepatic biliary ductal dilatation suspicious for PSC, and a cirrhotic liver with signs of portal hypertension and splenomegaly (Fig. 1a). Endoscopy revealed multiple non-bleeding duodenal ulcers and grade II esophageal varices. Colonoscopy found moderately active UC Mayo score 2, worsened from prior exam. MRCP was significant for cirrhotic liver morphology with nodular surface contour, and intrahepatic and extrahepatic biliary dilatation with beading and irregularity of the intrahepatic biliary tree and cystic duct, consistent with PSC (Fig. 1b). She was diagnosed with PSC-AIH OS and underwent treatment with Prednisone, Azathioprine, and Ustekinumab with minimal improvement. Only after the addition of Vancomycin was there normalization of biochemical markers and clinical status. Autoimmune liver disease is generally categorized into hepatic (AIH) or cholestatic (PSC and PBC) predominance. PSC is known to be associated with IBD, specifically UC. It is hypothesized that PSC evolves in UC patients due to uptake into the enterohepatic circulation of proinflammatory cells from the colon; these agents typically concentrate in the biliary system where they ultimately cause the biliary ductal damage frequently seen with PSC. By targeting enteric bacteria, Vancomycin decreases the absorption of these inflammatory biliary toxins. For PSC patients with UC and co-occurring AIH, the addition of Vancomycin could significantly reduce mortality and disease burden. Several case reports and one pilot study have investigated Vancomycin’s role in the treatment of PSC. However, it remains a vastly underutilized therapy, and its role specifically in PSC-AIH OS has not been previously reported. There remains an evident need for a large double blind randomized placebo-controlled clinical trial to further support the addition of Vancomycin to the standard treatment regimen for PSC, and to establish clear parameters on effective dosage and length of treatment. In the meantime, clinicians should be aware of the possible use of Vancomycin to treat patients with PSC +/- AIH and IBD. Figure 1a: CT abdomen pelvis with IV contrast highlighting stigmata of cirrhosis with portal hypertension and splenomegaly. Image also shows worsening of intrahepatic and extrahepatic biliary ductal dilatation in the setting of PSC given the patient’s underlying history of ulcerative colitis. Figure 1b: MRCP showing beading and irregularity of the intrahepatic biliary tree and cystic duct, in keeping with known history of primary sclerosing cholangitis. This image also highlights the dilation of the intrahepatic and extrahepatic biliary system.

Nodular Regenerative Hyperplasia Is Not a Rare Condition After Liver Transplantation: Incidence, Predictive Factors, and Impact on Survival.

The objectives of this study were to evaluate incidence and to identify the risk factors of occurrence and the predictive factors of symptomatic forms of nodular regenerative hyperplasia (NRH) after liver transplantation (LT). To identify risk factors of NRH following LT, we included 1648 patients transplanted from 2004 to 2018 and compared the patients developing NRH after LT to those who did not. To identify predictive factors of symptomatic NRH, we selected 115 biopsies displaying NRH and compared symptomatic to asymptomatic forms. Symptomatic NRH was defined as the presence of ascites, esophageal varices, hepatic encephalopathy, portal thrombosis, retransplantation, or death related to NRH. The incidence of NRH following LT was 5.1%. In multivariate analysis, the independent factor of developing NRH after LT was the donor's age (odds ratio [OR] = 1.02; confidence interval, 1.01-1.03; P = 0.02). Symptomatic forms occurred in 29 (25.2%) patients: 19 (16.5%) patients presented with ascites, 13 (11.3%) with esophageal varices, 4 (3.5%) with hepatic encephalopathy, and 8 (7%) with portal thrombosis. The median period before the onset of symptoms was 8.4 (1.5-11.3) y after LT. The spleen size at diagnosis/before LT ratio (OR = 12.5; 114.17-1.37; P = 0.0252) and thrombectomy during transplantation (OR = 11.17; 1.48-84.11; P = 0.0192) were associated with symptomatic NRH in multivariate analysis. NRH following LT is frequent (5.1%) and leads to symptomatic portal hypertension in 25.2% of patients. Using older grafts increases the risk of developing NRH after LT. Clinicians should screen for signs of portal hypertension, particularly in measuring spleen size.

Right-sided heart failure as a first presentation of portopulmonary hypertension

Introduction. Pulmonary artery hypertension and right ventricular failure are potentially fatal complications that can develop in patients with portal hypertension. The objective of this case report was to report a patient with end-stage liver disease, and portal and pulmonary artery hypertension and right heart failure. Case outline. A 57-year-old man was admitted to the Cardiology Department of a tertiary referral hospital due to signs of right-sided heart failure, ascites, pleural effusions, and pretibial edema. The patient had the history of alcohol abuse, arterial hypertension, and gout. Just prior to the admission, abdominal ultrasound revealed granular liver structure, as well as ascites. Laboratory tests showed microcytic anemia, values of transaminases below referent, hypoalbuminemia, low creatinine clearance. Echocardiography revealed pulmonary hypertension, and right ventricle failure. Right heart catheterization unraveled precapillary pulmonary hypertension, but thoracic CT scan and thoracocentesis excluded underlying pulmonary illness. Treatment continued at the Gastroenterology Department of the tertiary hospital. Abdominal CT scan diagnosed cirrhotic liver, and signs of portal hypertension. The patient was treated with symptomatic therapy, but developed acute-on-chronic renal failure and eventually died. Conclusion. Multidisciplinary approach is very important to distinguish portopulmonary hypertension early in the course of liver disease, because evolution of right sided heart failure precludes these patients from adequate lifesaving therapy.

REX SHUNT IN CHILDREN: COULD DYSFUNCTION BE PROGNOSED? A RETROSPECTIVE ANALYSIS.

The aim: To analyze retrospectively our experience of Rex shunt in children with symptomatic portal hypertension, its effect on hypersplenism regression and varices eradication, assess shunt survival and investigate risk factors, that could lead to shunt dysfunction and thrombosis. Materials and methods: 24 children (16 males, 8 females) ,with portal hypertension included into the study. All surgeries were performed within single center in a period from January 2010 to March 2022. Follow up period was 6.75±1.19 years. Results: Age at diagnosis was 5.39±0.64 years. 5 (20.8%) had umbilical catheter in anamnesis. 16 (66.7%) manifested bleeding episodes as the first sign of portal hypertension. 9 (37.5%) of children manifested severe hypersplenism. Age at Rex shunting was 7.5±0.7 years. In 7 (31.8%) cases Rex shunt thrombosis occurred. 1 successful thrombectomy and 6 splenorenal shunting were performed. Kaplan-Meyer analysis showed Rex shunt survival 0.670 (95%CI 0.420-0.831). Logistic regression model indicated thrombocytes count (p=0.0423) and cytopenia (p=0.0272) as factors that could influence shunt thrombosis. Follow-up group included 18 patients. Spleen volume regression became significant by 1 p/o year p<0,05, thrombocytes significant increasement reached in 1 p/o months (p<0.01), varices involution was achieved by 1 p/o year (p<0,001). Conclusions: Rex shunt effectiveness in study group was 70.9%., shunt survival assessed 0.670 (95%CI 0.420-0.831). Rex shunt was effective in bleeding prophylaxis in all patients of follow up group. Preoperative thrombocytes count (p=0.0423) and cytopenia (p=0.0272) were detected as factors that could influence shunt thrombosis, that is to be considered in RS preoperative period and require following studies.

Congenital porto-pulmonary shunt in a dog

Congenital extra-hepatic porto-systemic shunts (CEPS) are a non-rare vascular anomaly observed in dogs, most commonly in small and toy pure breeds. Computed tomographic angiography (CTA) examination is considered the gold standard imaging modality for the diagnosis of anomalous vascular connections. An anomalous congenital porto-pulmonary shunt was incidentally diagnosed in a 5-year-old French Bulldog. The anomalous vessel originated from the ventral aspect of the portal vein and went cranially towards the esophageal hiatus, entering the lobar vein of the caudal left pulmonary lobe. The dog did not show any significant clinical or computed tomography angiography-perceived hepatic abnormalities and no signs of portal hypertension were evidenced. No case of porto-pulmonary shunt in veterinary medicine have ever been reported, while in humans it was rarely described secondarily to portal hypertension, severe hepatopathies or complex cardiac malformations. CTA must be considered the best imaging modality for the diagnosis also of unusual CEPS and in the author's opinion the congenital porto-pulmonary shunt described in the patient was of little or no clinical relevance.

Spleen Stiffness Measurement Across the Spectrum of Liver Disease Patients in Real-World Practice

Spleen stiffness measurement (SSM) provides a non-invasive surrogate marker for clinical significant portal hypertension (CSPH). Results obtained in highly selected populations were promisingbut require validation across the spectrum of liver disease. We aimed to investigate the clinical applicability of SSM in a real-world setting. We prospectively enrolled patients referred for liver ultrasound (January-May 2021). Patients with a portosystemic shunt, liver transplant, or extrahepatic etiology of portal hypertension were excluded. We performed liver ultrasound, liver stiffness measurement (LSM) and SSM (dedicated software, 100Hz-probe). Probable CSPHwas established if ≥1 of the following items occurred: ascites, varices, encephalopathy, splenomegaly, recanalized umbilical vein, collaterals, dilated portal veins, hypertensive gastropathy, or LSM ≥25kPa. We enrolled 185 patients (53% male; age 53years [37-64], 33% viral hepatitis, 21% fatty liver disease). Of them, 31% of patients had cirrhosis (68% Child-Pugh A) and 38% of patients had signs of portal hypertension. SSM (23.8kPa [16.2-42.3]) and LSM (6.7kPa [4.6-12.0]) were successful and met reliability criteria in 70% and 95%, respectively. Spleen size was inversely associated with SSM failure (odds ratio: 0.66 increment/cm, 95% confidence interval: 0.52-0.82). Optimal spleen stiffness cut-off to detect probable CSPH was >26.5kPa (likelihood ratio: 4.5, sensitivity: 83%; specificity: 82%). Spleen stiffness did not outperform liver stiffness in detecting probable CSPH (P= 1.0). In real-world practice, reliable SSM were obtained in 70%and could potentially stratify patients between high- and low-risk of probable CSPH. However, cut-offs for CSPH might be substantially lower than previously reported. Future studies validating these results are required. Netherlands Trial Register (Registration number: NL9369).

Antiphospholipid Syndrome as a Cause of Recurrent Portal Vein Thrombosis in a Man with a Medical History of other Thrombosis

Aim: to present a clinical case of antiphospholipid syndrome (APS) manifested as recurrent portal vein thrombosis in a man with a medical history of other thromboses.Key points. APS is a syndrome that includes venous and/or arterial thrombosis, various forms of obstetric pathology, thrombocytopenia, as well as a variety of neurological, skin, cardiovascular and hematological disorders. The article presents a clinical case of a male patient with several episodes of vascular thrombosis, two of which (thrombophlebitis of the veins of the lower extremities and stroke) developed at the age of 39 years, and recurrent thrombosis of the portal vein since 2018. For several years, the patient had the diagnosis of cirrhosis based on the presence of signs of portal hypertension. However, the patient's liver function remained practically intact all the time. During the current examination, APS was suspected, and the result of a blood test for antibodies against phospholipids was positive.Conclusion. This clinical case of APS reflects the difficulties in diagnosing this disease. It should be remembered that cases of thrombosis at a young age may be due to APS.

Features of Surgical Treatment of Patients with Splenic Artery Aneurysms (Case Report and Literature Review)

Despite the insigniϐicant prevalence, aneurysms of the splenicartery take a dominant share among all visceral aneurysms with a predominance of morbidity in women of childbearing age. Taking into account the mostly asymptomatic course and the high risk of mortality due to its rupture, timely diagnosis and correct treatment strategy remain extremely important. Questions regarding the choice of surgical treatment tactics for patients with this pathology remain debatable.&#x0D; The aim. Demonstration of a clinical case, improvement of diagnosis andsurgical treatment of patients with splenic artery aneurysms.&#x0D; Clinical case. Female patient G., 32 years old, with complaints of periodic pain in the left hypochondrium, signs of portal hypertension against the background of cavernous transformationof the portal vein, history of recurrent bleeding from the esophageal veins, hypersplenism admitted with a pre-diagnosed aneurysm of the splenic artery of giant dimensions (diameter 8x5 cm). The patient was qualified for open surgery and aneurysm resection with splenectomy and spleen autotransplantation by N. Roth. The postoperative period was complicated by the occurrence of reactive pancreatitis on day 7, which was successfully treated conservatively. In the future, the course was unremarkable. The remote follow-up lasted two years, and no recurrence of the aneurysm was detected. The performed immunogram showed normal results.&#x0D; Conclusion. The timely diagnosis makes it possible to establish the correct diagnosis, justify the treatment tactics and prevent fatal complications. Open surgical intervention in somecases remains the method of choice in the treatment of this pathology. Autotransplantation of the spleen has shown good long-term results and can be performed in patients of this category for the prevention of immunodeficiency states.

Pseudocirrhosis and portal hypertension in patients with metastatic cancers: a systematic review and meta-analysis

Pseudocirrhosis is a clinical and radiological entity mimicking liver cirrhosis in patients without a history of chronic liver disease. We performed a systematic review and meta-analysis of the current literature to evaluate the state-of-the-art and investigate the epidemiology and clinical features of pseudocirrhosis. We searched PubMed, Web of Science and Scopus for literature published until February 28, 2022. We included in the final analysis 62 articles (N = 389 patients): 51 case reports (N = 64 patients), 5 case series (N = 35 patients) and 6 observational studies (N = 290 patients). About 80% of patients included in the case reports and case series had breast cancer. Most patients had at least one clinical sign of portal hypertension and ascites was the most common clinical manifestation of portal hypertension. The median time from pseudocirrhosis to death was 2 months (IQR 1–7 months). Alkylating agents and antimitotics were the most common classes of anticancer drugs reported in our study population. Notably, about 70% of patients received three or more anticancer drugs. Finally, pseudocirrhosis is a condition that occurs in patients with hepatic metastases and may have a negative impact on survival and clinical management of patients because of the potential development of portal hypertension and its complications.

Attenuation of right gastrocaval extrahepatic shunt with cellophane band in a dog: Case report

Congenital extrahepatic portosystemic shunt is a vascular anomaly that consists in the communication between the portal and systemic circulation, causing substances normally metabolized and excreted by the liver to be diverted directly to the systemic circulation and triggering changes in neurological, gastrointestinal and urinary systems. Diagnosis is through imaging tests, such as ultrasound and computed tomography. For patient stabilization, initial clinical treatment is highly indicated, especially for those who manifest signs of hepatic encephalopathy, and the definitive treatment is gradual surgical attenuation. Currently, the most indicated techniques for shunt occlusion are the ameroid constrictor ring and the cellophane band, which have good long-term results. The prognosis of patients undergoing the surgical procedure varies according to the general condition of the patient at the time of diagnosis and the complications that may occur in the trans and postoperative periods, such as portal hypertension, seizures, hypoglycemia and failure to close the shunt. This report aims to describe a case of surgical attenuation of a right extrahepatic gastrocaval shunt using the cellophane band technique. The canine patient, pug, aged eight months, was treated at the Veterinary Hospital of the Federal University of Paraná, presenting neurological clinical signs and a previous diagnosis of portosystemic shunt. To determine the origin of the anomalous vessel and optimize surgical planning, computed angiotomography was indicated. Clinical treatment was instituted and, after 15 days, surgical attenuation of the shunt was performed using the cellophane band fixed with titanium clips. The patient showed signs of portal hypertension, ascites and postoperative hypoglycemia, which were treated during hospitalization. After 15 days of hospital discharge, the patient returned to the Veterinary Hospital with good general condition, absence of ascites and neurological signs and no significant changes were observed in laboratory tests, demonstrating the effectiveness of the chosen surgical technique.

Porto-sinusoidal vascular disorder.

It is well established that portal hypertension can occur in the absence of cirrhosis, as reported in patients with immune disorders, infections and thrombophilia. However, similar histological abnormalities primarily affecting the hepatic sinusoidal and (peri)portal vasculature have also been observed in patients without portal hypertension. Thus, the term porto-sinusoidal vascular disorder (PSVD) has recently been introduced to describe a group of vascular diseases of the liver featuring lesions encompassing the portal venules and sinusoids, irrespective of the presence/absence of portal hypertension. Liver biopsy is fundamental for PSVD diagnosis. Specific histology findings include nodular regenerative hyperplasia, obliterative portal venopathy/portal vein stenosis and incomplete septal fibrosis/cirrhosis. Since other conditions including alcohol-related and non-alcoholic fatty liver disease, or viral hepatitis, or the presence of portal vein thrombosis may occur in patients with PSVD, their relative contribution to liver damage should be carefully assessed. In addition to histology and clinical diagnostic criteria, imaging and non-invasive tests such as liver and spleen stiffness measurements could aid in the diagnostic workup. The introduction of PSVD as a novel clinical entity will facilitate collaborative studies and investigations into the underlying molecular pathomechanisms encompassed by this term.

S3091 Noncirrhotic Portal Hypertension in Turner’s Syndrome: A Case Report

Introduction: Turner's syndrome is one the most common chromosomal aneuploidies in humans. Although it can affect multiple organs, involvement of gastrointestinal (GI) organs is rare. Case Description/Methods: A 20-year-old female with history of Turner's syndrome with premature ovarian failure and mild mitral regurgitation was evaluated for easy fatigability and bruising. Laboratory assessment indicated pancytopenia. Peripheral blood smear showed a variety of red cell shape abnormalities and bone marrow biopsy showed normal cellular marrow. Computed tomography of the chest, abdomen and pelvis with and without contrast showed severe splenomegaly, tiny varices in the anterior mediastinum along the gastrohepatic ligament, and no portal vein thrombosis. Abdominal ultrasound examination revealed severe diffuse heterogeneity of the liver parenchyma with a slightly nodular hepatic contour and Fibroscan indicated mild steatosis S1 with advanced fibrosis F3. She underwent liver biopsy which showed focal mature fibrous expansion of some portal tracts, with delicate bridging fibrosis, as well as patchy increase in the number of small portal arteries with occasional increased thickness of arterial wall, subtle lobular parenchymal changes, suggestive of porto-sinusoidal vascular disease with no evidence of cirrhosis. Upper GI endoscopy revealed grade 2 esophageal varices and erythematous mucosa in stomach, suggestive of portal hypertension. Considering the definitive clinical signs of portal hypertension (gastroesophageal varices), the absence of cirrhosis on liver biopsy and histologic signs of porto-sinusoidal vascular disease, she was diagnosed with non-cirrhotic portal hypertension due to porto-sinusoidal vascular disease. She was started on propranolol for gastroesophageal varices and her heart rate is currently well-controlled. Follow-up abdominal ultrasound is negative for further changes in the liver size, morphology, or masses. Discussion: Turner’s syndrome might be associated with non-cirrhotic portal hypertension. High index of clinical suspicion can lead to early diagnosis and treatment of portal hypertension in individuals with Turner’s syndrome, reducing the burden of complications of portal hypertension such as cytopenia or bleeding.

Liver and heart failure: an ultrasound relationship.

Liver and heart are anatomically and patho-physiologically related. In heart failure (HF) the increased right atrial pressure and volume overload cause histological changes in hepatocytes, leading to a condition known as "congestive hepatopathy" (CH), with consequent variations in liver functioning and ultrasound (US) findings. CH has specifical US findings especially regarding venous vessels aspect, easily detecting by gray-scale study, but many others can be distinguished by Doppler analysis. Usually, hepatic veins look enlarged and hypocollassing, together with signs of portal hypertension (hepatomegaly, ascites, splenomegaly, porto-systemic collaterals). Typically, in CH Doppler findings regard alterations in venous vessel flow and arterial resistance (venous system hyperpulsatility, reduced velocity flow, high resistance index in hepatic arterial Doppler spectrum). Sometimes CH and other primary hepatopathy can coexist, and therefore some of the expected variations may not manifest: it allows suspecting an unknown underlying liver disease. At last, US technologies of more recent applications, even if not routinely used, allow investigating additional aspects such as elastography that detects changes in liver elasticity or contrastographic US, able to show differences in hepatic venous opacification. However, most of these US signs are not pathognomonic, and therefore a multidisciplinary clinical reasoning must not be lacking. The aim of the present review is to easily provide US signs of liver alterations in HF, in particular right heart failure with volume overload, suggesting including liver US in instrumental diagnosis and therapeutic monitoring of HF.

IS HOMEOSTASIS MODEL ASSESSMENT FOR INSULIN RESISTANCE >2.5 A DISTINGUISHED CRITERIA FOR METABOLIC DYSFUNCTION-ASSOCIATED FATTY LIVER DISEASE IDENTIFICATION?

Insulin resistance (IR), assessed by different criteria, is an important factor in the pathogenesis of non-alcoholic fatty liver disease (NAFLD). More recently with the characterization of this metabolic dysfunction-associated fatty liver disease (MAFLD), one of the proposed criteria for this diagnosis has been the determination of the homeostasis model assessment-insulin resistance (HOMA-IR). The purpose of this study was to evaluate the relationship of HOMA-IR>2.5 with clinical, metabolic, biochemical and histological data obtained in non-diabetic patients diagnosed with NAFLD by liver biopsy. Cross-sectional, retrospective study was carried out with data from 174 adult individuals of both genders with non-diabetics NAFLD, without obvious signs of portal hypertension. The body mass index (BMI) was classified according to the World Health Organization (1998), and the metabolic syndrome by the criteria of NCEP-ATP-III. Biochemical tests were evaluated using an automated method and insulinemia through immunofluorometric assay. Histological findings were classified according to Kleiner et al. (2005). The mean age of the studied population was 53.6±11.2 years, with 60.3% being female. The average BMI was 30.3 kg/m2 and 75.9% of the patients had increased waist circumference. Among evaluated metabolic parameters, there was a higher prevalence of metabolic syndrome (MS) in patients with HOMA-IR>2.5, with no statistical difference in relation to BMI between studied groups. Values of liver enzymes and serum ferritin were significantly higher in patients with this marker of IR, who had a higher prevalence of non-alcoholic steatohepatitis (NASH) and advanced liver fibrosis. In the multivariate analysis, the clinical diagnosis of MS, hyperferritinemia and the presence of NASH in the liver biopsy were the factors independently associated with the presence of altered HOMA-IR. HOMA-IR values >2.5 identify patients with NAFLD with distinct clinical and metabolic characteristics and with a greater potential for disease progression, which validates this parameter in the identification of patients with MAFLD.