Complications Of Portal Hypertension Research Articles

Disparities in Cirrhosis Management of Black Patients: Do We Know What to Address?

Disparities in Cirrhosis Management of Black Patients: Do We Know What to Address?

Three year experience of Upper Gastrointestinal Endoscopic procedures at a tertiary care hospital of South Punjab

Introduction: Endoscopy has become a necessity in diagnosing gastrointestinal (GI) disorders. The objective of our study was to evaluate the different indications and findings of upper GI endoscopy. Methods: This retrospective analysis was undertaken at department of Gastroenterology, Nishtar Hospital Multan. Records of all upper GI endoscopic procedures from 1st January 2018 till 31st December 2020 were evaluated. Results: A total 3299 upper GI endoscopic procedures were perfumed during the three-year time period. Mean age was 47 years. Majority of patients were males. Almost 48% of patients belonged to the middle-aged group. The most common indication was upper GI bleeding (57%), followed by dyspepsia (15%). The most common finding was esophageal varices (43%), followed by portal gastropathy (26%) and gastritis (16%). Conclusion: This study concludes that the majority of endoscopies are being undertaken as a result of complications of cirrhosis and portal hypertension. Keywords: Endoscopy, audit, indications, findings

Role of early transjugular intrahepatic portosystemic stent-shunt in acute variceal bleeding: An update of the evidence and future directions.

Variceal bleeding is a serious complication of cirrhosis and portal hypertension. Despite the improvement in management of acute variceal bleed (AVB), it still carries significant mortality. Portal pressure is the main driver of variceal bleeding and also a main predictor of decompensation. Reduction in portal pressure has been the mainstay of management of variceal bleeding. Transjugular intrahepatic porto-systemic stent shunt (TIPSS) is a very effective modality in reducing the portal hypertension and thereby, controlling portal hypertensive bleeding. However, its use in refractory bleeding (rescue/salvage TIPSS) is still associated with high mortality. “Early” use of TIPSS as a “pre-emptive strategy” in patients with AVB at high risk of failure of treatment has shown to be superior to standard treatment in several studies. While patients with Child C cirrhosis (up to 13 points) clearly benefit from early-TIPSS strategy, it’s role in less severe liver disease (Child B) and more severe disease (Child C > 13 points) remains less clear. Moreover, standard of care has improved in the last decade leading to improved 1-year survival in high-risk patients with AVB as compared to earlier “early” TIPSS studies. Lastly in the real world, only a minority of patients with AVB fulfil the stringent criteria for early TIPSS. Therefore, there is unmet need to explore role of early TIPSS in management of AVB in well-designed prospective studies. In this review, we have appraised the role of early TIPSS, patient selection and discussed future directions in the management of patients with AVB.

Long-term results of splenomegaly after surgery for biliary atresia in the native liver

BackgroundBiliary atresia (BA) is a rare disorder characterized by obstructive jaundice in infants, shortly after birth. Postoperatively, some patients exhibit portal hypertension and progressive liver fibrosis. Splenomegaly is a symptom of portal hypertension. We aimed to investigate splenomegaly as a marker for complications of portal hypertension and the relationship between splenomegaly and liver fibrosis in the long-term native liver (NL). MethodsBetween 1977 and 2018, 71 patients underwent hepaticojejunostomy. We included 54 patients (34 NL group, 20 liver transplant (LT) group) who fulfilled the eligibility criteria. Spleen volume (SV), total bile acids, hyaluronic acid, type IV collagen, and aspartate aminotransferase-to-platelet ratio index (APRi) were measured. Data were analyzed using Student's t-test, regression analysis, and receiver operating characteristic (ROC) curve analysis (P < 0.05). ResultsTotal bile acids, hyaluronic acid, type IV collagen, and APRi increased in NL patients with a large SV at >25 years. SV and type IV collagen were correlated with NL for >25 years (r = 0.79 [P = 0.006], y = 1.1 - [0.03 × type IV collagen] [P = 0.008]). In the ROC curve analysis, the cutoff value for type IV collagen was 165 ng/mL (P = 0.07). ConclusionsWe suggest that SV as a prognostic index for End-Stage Liver Disease may be useful in biliary atresia. Long-term follow-up is necessary because the clinical course may be favorable in childhood but worsen during adulthood.

Ultrasonography in the diagnosis of complications in patients with portal hypertension.

This review focuses on ultrasonography (US) to diagnose patients with complications in portal hypertension. Clinicians first use US to evaluate patients with suspected portal hypertension, because US is quick, simple, and radiation free. US is necessary for grading and performing paracentesis for ascites. Doppler US-based detection of reverse splanchnic vein flow or the presence of a spontaneous portosystemic shunt is highly specific in patients with cirrhosis. Since it is important to estimate spleen size in patients with portal hypertension, spleen size is usually measured by US. Spleen volume can be more accurately measured with 3D-US. Estimation of viable residual splenic volume after partial splenic embolization should be limited to cases with total splenic volume less than 1000ml. Portal vein thrombosis is often detected during the US examination performed when symptoms first appear or during the follow-up. Two-dimensional transthoracic echocardiography is an excellent noninvasive screening test in patients with pulmonary portal hypertension who can undergo it. By measuring the maximum and minimum diastolic blood flow velocities in the renal arteries using renal color Doppler US, the pulsatility index (PI) and resistive index (RI) can be calculated. The PI and RI in cirrhotic patients were significantly higher than those in healthy subjects and patients with chronic hepatitis, and showed a significant positive correlation with the Child-Pugh Score. In conclusion, US is an essential tool for the diagnosis and treatment of patients with portal hypertension.

Accuracy of International Classification of Diseases-10 Codes for Cirrhosis and Portal Hypertensive Complications.

International Classification of Diseases (ICD)-10 codes may correspond to cirrhosis diagnosis. However, these codes have not been as well studied as ICD-9 codes. We aimed to evaluate the positive predictive value (PPV) and specificity of ICD-10 codes for cirrhosis. We conducted a single-center retrospective study of patients in Michigan Medicine (Ann Arbor, MI, USA). We evaluated patients with at least one of 28 ICD-10 codes for cirrhosis and randomly selected controls for the presence of cirrhosis and/or portal hypertensive complications. Among 1317 patients with at least one ICD-10 code consistent with cirrhosis and/or portal hypertension, 796 had confirmed cirrhosis. After excluding ICD-10 codes found in < 10 patients, we evaluated the PPV of the 19 remaining codes. Of these, 15 had a high PPV for cirrhosis (> 80%), including codes for cirrhosis itself, gastroesophageal varices, hepatic encephalopathy, and other portal hypertensive complications. Specificity of ICD codes for cirrhosis for these 15 codes was high (> 94% for all). PPV and specificity were high across liver disease etiologies. Among patients without portal hypertension, PPVs of ICD-10 codes for cirrhosis were lower but still > 80% for the most common codes. PPVs of most codes for portal hypertensive complications were > 70%. Defining cirrhosis based on the presence of any of the 15 codes resulted in a PPV of 86% and by two different codes, a PPV 94%. ICD-10 codes for cirrhosis can accurately identify patients with cirrhosis with or without portal hypertensive complications.

Hepatopulmonary Syndrome and Portopulmonary Hypertension: Pulmonary Vascular Complications of Liver Disease.

Pulmonary vascular disease is a frequent complication of chronic liver disease and portal hypertension, affecting up to 30% of patients. There are two distinct pulmonary vascular complications of liver disease: hepatopulmonary syndrome (HPS) and portopulmonary hypertension (POPH). HPS affects 25% of patients with chronic liver disease and is characterized by intrapulmonary vasodilatation and abnormal arterial oxygenation. HPS negatively impacts quality of life and is associated with a 2-fold increased risk of death compared to controls with liver disease without HPS. Angiogenesis, endothelin-1 mediated endothelial dysfunction, monocyte influx, and alveolar type 2 cell dysfunction seem to play important roles in disease pathogenesis but there are currently no effective medical therapies. Fortunately, HPS resolves following liver transplant (LT) with improvements in hypoxemia. POPH is a subtype of pulmonary arterial hypertension (PAH) characterized by an elevated mean pulmonary arterial pressure and pulmonary vascular resistance in the setting of normal left-sided filling pressures. POPH affects 5% to 6% of patients with chronic liver disease. Although the pathogenesis has not been fully elucidated, endothelial dysfunction, inflammation, and estrogen signaling have been identified as key pathways involved in disease pathogenesis. POPH is typically treated with PAH targeted therapy and may also improve with liver transplantation in selected patients. This article highlights what is currently known regarding the diagnosis, management, pathobiology, and outcomes of HPS and POPH. Ongoing research is needed to improve understanding of the pathophysiology and outcomes of these distinct and often misunderstood pulmonary vascular complications of liver disease. © 2021 American Physiological Society. Compr Physiol 11:1-22, 2021.

S2907 Portal Vein Thrombosis in the COVID-19 Era: A Rare Presentation

Introduction: An increased incidence of venous thromboembolism (VTE) has been reported with SARS-COV2 (severe acute respiratory syndrome coronavirus 2) infections. VTE in unusual sites like the portal vein is limited to case reports. This report adds to the growing body of literature about thrombotic complications with SARS-COV2 infections. Case Description/Methods: A female aged 39 with a medical history of diabetes, hypertension and cholecystectomy presented with three days of right upper quadrant (RUQ) pain. She denied substance use, cirrhosis, autoimmune disease or previous VTE. On presentation, vitals were stable. She had RUQ tenderness. Blood work revealed elevated alkaline phosphatase and transaminases. White cell count, bilirubin and coagulation were normal. Imaging revealed occlusive thrombus of the right portal vein proximal to the bifurcation with peripheral extension (images 1 and 2). Extensive hypercoagulable and infectious workup was negative. She was immediately started on enoxaparin. A chest x ray was suggestive of SARS-COV2 infection and confirmatory testing was positive. Her symptoms improved and lab abnormalities resolved. She was discharged home with oral anticoagulants and instructions to self-isolate. Discussion: Most reported cases of Portal vein thrombosis (PVT) with SARS-COV2 are incidental. Our patient presented with RUQ abdominal pain, highlighting an unusual presentation. PVT is rare and occurs with cirrhosis, connective tissue disease, malignancies, pancreatitis and thrombophilia. These were absent in our patient and other causes of hypercoagulability such as antiphospholipid syndrome, proteins C, S and antithrombin deficiencies, Factor V mutations were ruled out. SARS-COV2 infected patients experience a hypercoagulable state with the incidence of thrombotic complications as high as 31%. Some have suggested that systemic inflammation leads to endothelial dysfunction, causing Von-Willebrand Factor and toll-like receptor activation, leading to platelet aggregation and coagulation cascade initiation. Others have speculated that inflammatory cytokines bind to endothelial surface angiotensin converting enzyme receptor leading to lymphocytic endotheliitis and prothrombotic gene overexpression. Anticoagulation should be used in PVT to reduce complications of intestinal infarction and portal hypertension. Recanalization occurs in 60% of cases with anticoagulation in the first week. We used therapeutic enoxaparin and switched to an oral anticoagulant for a 6-month course.Figure 1.: Image 1 a and b: CT scan showing right portal vein thrombosis, just at its bifurcation (blue arrows) c: chest x ray showing bilateral patchy opacities.

Management of portal hypertensive upper gastrointestinal bleeding: Report of the Coorg Consensus workshop of the Indian Society of Gastroenterology Task Force on Upper Gastrointestinal Bleeding.

Portal hypertensive bleeding is a major complication of portal hypertension (PHT) with high morbidity and mortality. A lot of advances have been made in our understanding of screening, risk stratification, and management strategies for portal hypertensive bleeding including acute variceal bleeding leading to improved overall outcomes in patients with PHT. A number of guidelines on variceal bleeding have been published by various societies in the past few years. The Indian Society of Gastroenterology (ISG) Task Force on Upper Gastrointestinal Bleeding (UGIB) felt that it was necessary to bring out a standard practice guidance document for the use of Indian health care providers especially physicians, gastroenterologists, and hepatologists. For this purpose, an expert group meeting was convened by the ISG Task Force to deliberate on this matter and write a consensus guidance document for Indian practice. The delegates including gastroenterologists, hepatologists, radiologists, and surgeons from different parts of the country participated in the consensus development meeting at Coorg in 2018. A core group was constituted which reviewed all published literature on portal hypertensive UGIB with special reference to the Indian scenario and prepared unambiguous statements on different aspects for voting and consensus in the whole group. This consensus was produced through a modified Delphi process and reflects our current understanding and recommendations for the diagnosis and management of portal hypertensive UGIB in Indians. Intended for use by the health care providers especially gastroenterologists and hepatologists, these consensus statements provide an evidence-based approach to risk stratification, diagnosis, and management of patients with portal hypertensive bleeding.

Indian College of Radiology and Imaging Evidence-Based Guidelines for Interventions in Portal Hypertension and Its Complications.

Portal hypertension is a complication of chronic liver disease. Various radiological interventions are being done to aid in the diagnosis of portal hypertension; further, an interventional radiologist can offer various treatments for the complications of portal hypertension. Diagnosis of portal hypertension in its early stage may require hepatic venous pressure gradient measurement. Measurement of gradient also guides in diagnosing the type of portal hypertension, measuring response to treatment and prognostication. This article attempts to provide evidence-based guidelines on the management of portal hypertension and treatment of its complications.

Impact of Different Modalities of Treatment of Hepatorenal Syndrome as Midodrine, Somatostatin analogue, Albumin infusion and Tapping of Ascites on Renal Artery Resistive Index by Doppler ultrasound

Abstract Background Liver cirrhosis is a major cause of morbidity and mortality worldwide, mainly due to complications of portal hypertension. Ascites formation in patients with Kristeen E. cirrhosis, portal hypertension, or both usually results from hyperdynamic circulatory Zaki dysfunction. Aim of the Work to investigate the effect and prognostic value of treatment of hepatorenal syndrome in patients with liver cirrhosis and massive ascites by LVP, somatostatin analogue and midodrine intake and its effect on renal resistive index. Patients and Methods This study was carried on 40 patients divided into 4 groups where 10 patients (G1) received midodrine therapy and albumin infusion, 10 patients (G2) had tapping of ascites with albumin infusion, 10 patients (G3) received octreotide therapy and albumin infusion and the last 10 patients (G4) received octreotide, midodrine and albumin infusion. All patients were subjected to careful history taking and thorough clinical examination. Before and after intervention, patients had laboratory investigation including complete blood picture, liver function tests, renal function test and ascitic fluid analysis in addition to renal artery Doppler imaging. Results The study revealed considerable decline in RI in G1 (midodrine group), the change fell short of statistical significance. However, our study showed significant effect of midodrine administration on renal functions as expressed by serum creatinine, blood urea and creatinine clearance. In G3 (octreotide group), the effect of treatment on renal artery RI was minimal. The other group that perceived significant decline in renal artery resistive index was G4 (midodrine and octreotide group). Conclusions LVP and midodrine combination administration resulted in significant decrease in creatinine clearance. Renal artery resistive index has significant correlation with creatinine clearance in studied group and can be used as non-invasive tool.

S2610 Perihepatic Biloma: A Rare Complication After Transjugular Intrahepatic Portosystemic Shunt (TIPS)

Introduction: Transjugular intrahepatic portosystemic shunt (TIPS) is a minimally invasive procedure and an effective therapeutic option for managing portal hypertension complications. Biliary complications after TIPS are uncommon. Here we present a case of recurrent perihepatic biloma in a woman who underwent TIPS. Case Description/Methods: A 40-year-old woman was admitted for epigastric abdominal pain for five days. She reported taking oral contraceptive pills (OCPs) for one month before presentation. She had epigastric tenderness on examination and normocytic anemia (Hgb 10 g/L) on admission labs. CT scan of the abdomen showed a large thrombus in the main portal vein extending into the right and left portal venous branches. The patient was started on heparin drip and underwent TIPS with thrombectomy. Hypercoagulability workup was unremarkable. The post-procedure course was complicated by elevation of alkaline phosphatase (552 U/L), AST (293 U/L), and ALT (324 U/L). Abdomen MRI showed patent TIPS and persistent complete thrombosis of all intrahepatic portal venous branches. She underwent repeat thrombolysis. However, liver enzymes continued to rise, and a repeat MRI showed intrahepatic biloma at the mid aspect of TIPS (Figure 1A, B). IR-guided drainage of the biloma was performed. The hospital course was complicated by fever and leukocytosis. A follow-up CT scan showed a 13 cm complex fluid collection at the dome of the liver (Figure 2A). She underwent drainage of the new collection and received IV Cefoxotin with an interval decrease in the collection size (Figure 2B). Fever resolved, and liver enzymes normalized. Discussion: Bilomas are either intra- or extrahepatic collections of bile that can occur spontaneously, or as a result of trauma to the biliary tree, including iatrogenic injury. Bilomas are usually asymptomatic but may present with abdominal pain due to symptomatic bile peritonitis or secondary bacterial infection leading to abscess formation and sepsis. A biloma may wall off or may continue to have active bile leakage. CT intravenous cholangiography, MRI with gadolinium, and Tc99 scintigraphy can identify bile leak. Differential diagnoses include hepatic cyst and hematoma, liver abscess, loculated ascites, and postoperative fluid collections, including seroma and lymphocele. Treatment options include endoscopic drainage, IR guided, or surgical drainage with bile leak repair.Figure 1.: 1A, and 1B: MRI coronal T2W, MRI axial T2W and MRI coronal post contrast, respectively, showing intrahepatic biloma (white arrow) abutting a portion of the TIPS (yellow arrow) with upstream intrahepatic biliary ductal dilatation (blue arrow). 2A and 2B: CT coronal image demonstrates a new peri-hepatic collection surrounding the hepatic dome (black arrow) and interval placement of a percutaneous drainage catheter and decrease in size of the peri-hepatic collection (black arrow).

Targeting integrin \u03b1v\u03b23 by a rationally designed protein for chronic liver disease treatment

Chronic Liver Diseases (CLD) are characterized by abnormal accumulation of collagen fibrils, neo-angiogenesis, and sinusoidal remodeling. Collagen deposition along with intrahepatic angiogenesis and sinusoidal remodeling alters sinusoid structure resulting in portal hypertension, liver failure, and other complications. Efforts were made to develop treatments for CLDs. However, the success of such treatments is limited and unpredictable. We report a strategy for CLD treatment by induction of integrin αvβ3 mediated cell apoptosis using a rationally designed protein (ProAgio). ProAgio is designed to target integrin αvβ3 at a novel site. Integrin αvβ3 is highly expressed in activated Hepatic Stellate Cells (HSC), angiogenic endothelium, and capillarized Liver Sinusoidal Endothelial Cells (LSEC). ProAgio induces apoptosis of these disease causative cells. Tests with liver fibrosis mouse models demonstrate that ProAgio reverses liver fibrosis and relieves blood flow resistance by depleting activated HSC and capillarized LSEC. Our studies demonstrate an effective approach for CLD treatment.

Portosystemic shunting procedures for complicated portal hypertension: modern opportunities of mini-invasive technique

Aim. To improve the results of treatment for patients with complicated portal hypertension of cirrhotic genesis using the transjugular intahepatic portosystemic shunt with endovascular obliteration of the inflow pathways to the esophageal-gastric varicose veins.Material and methods. Transjugular intahepatic portosystemic shunt was performed in 172 patients with gastroesophageal variceal bleeding. The patients were divided into 3 clinical groups. The shunting procedure was applied to 62 patients. Another 110 patients underwent transjugular intahepatic portosystemic shunt and selective obliteration of the esophageal-gastric vein inflow pathways. The short-term and long-term results (up to 140 months), the incidence of thrombosis, recurrent bleeding and mortality, as well as their relationship with the established risk factors for complications were traced.Results. All patients achieved an effective reduction in the portosystemic pressure gradient and a reduction in the manifestations of portal hypertension. Recurrence of bleeding caused by shunt thrombosis occurred in 23 (13.3%) patients. In clinical group I (n = 62), this complication was noted in 9 (14.5%) patients, in II (n = 54) – 11 (20.4%) cases, in III (n = 56) – only in 3 (5.4%) cases. Bleeding-free survival decreased from 1.0 to 0.82 in 83.9 months, then plateaued. A sharp decrease in the Kaplan–Meier curve from 1.0 to 0.88 was observed within 24.5 months after the intervention. The largest number of deaths was in group I (30.6%), the smallest in group III (7.1%).Conclusion. Transjugular intahepatic portosystemic shunt supplemented by obliteration the inflows of the esophagealgastric varicose veins provides complete eradication of varicose veins, helps to reduce the frequency of recurrent bleeding and death.

РЕЗУЛЬТАТЫ ПРИМЕНЕНИЯ МОДИФИЦИРОВАННОЙ ЛАПАРОСКОПИЧЕСКОЙ ЭЗОФАГОГАСТРАЛЬНОЙ ДЕВАСКУЛЯРИЗАЦИИ

Background. Transabdominal gastroesophageal devascularization is a technically feasible backup operation for most general surgeons in case of failure of endoscopic prophylaxis of bleeding from esophageal varices. There is an emerging trend in the development of laparoscopic technologies in the surgical correction of complications of portal hypertension. Purpose. Assessment of the clinical efficacy of laparoscopic esophagogastric devascularization in its own modification. Material and methods. In the period from 2009 to 2020, the results of treatment of 31 patients were studied. The patients were divided into 2 groups by the method of simple randomization. In the control group, surgery was performed in a standard way, in the main group - in the modification we proposed. Results. The use of our proposed technological methods when performing the stage of transection of the abdominal esophagus during laparoscopic esophagogastric devascularization in its own modification reduced the frequency of technical difficulties at this stage, which caused conversions, anastomotic leakage and other intra and postoperative complications. In the long-term period, there was a decrease in the degree of esophageal varices, a decrease in the frequency of recurrent bleeding and mortality, as well as an improvement in the quality of life in patients after a modified operation in comparison with the standard technique. Laparoscopic esophagogastric devascularization in our modification shows better results as a method of secondary prevention of bleeding from esophageal varices in cirrhosis of the liver in patients with a high risk of recurrence than the use of endoscopic sclerosis of esophageal varices, is characterized by a lower incidence of repeated episodes of bleeding from esophageal varices and less lethality. Conclusions. Based on the analysis of the immediate and long-term results of the use of laparoscopic esophagogastric devascularization in its own modification in the clinic, it should be noted the reproducibility of the surgical intervention, its effectiveness and safety.

Lower Ratio of Liver Volume and Body Weight Is a Negative Predictor of Survival after Transjugular Intrahepatic Portosystemic Shunt.

Transjugular intrahepatic portosystemic shunt (TIPS) is the most effective measure to treat complications of portal hypertension. However, liver function may deteriorate after TIPS. Predictors of liver function and outcome after TIPS are therefore important for management of TIPS patients. The study aimed to evaluate the impact of liver volume on transplant-free survival (TFS) after TIPS, as well as the evolution of liver volume and its relationship with liver function after TIPS. A retrospective analysis of all consecutive patients who underwent TIPS in a tertiary care university liver center between 2012 and 2017 (n = 216) was performed; n = 72 patients with complete prior and follow-up (FU) computed tomography (CT) imaging studies were included in the study. Volumetry of the liver was performed by a semi-automatic 9-lobe image segmentation algorithm at baseline and FU (FU 1: 90–180 d; FU 2: 180–365 d; FU 3: 365–545 d; FU 4: 545–730 d; FU 5: >730 d). Output variables were total liver volume (TLV, cm3), left liver volume (LLV, cm3), right liver volume (RLV, cm3) and TLV/body weight ratio. CT derived liver volumes were correlated with liver function tests, portosystemic pressure gradient (PPG) measurements and survival. To assess predictors of liver volume change over time we fitted linear mixed models. Kaplan–Meier analysis was performed and validated by matched pair analysis followed by Cox regression to determine independent prognostic factors for survival. The median TLV at baseline was 1507.5 cm3 (773.7–3686.0 cm3). Livers with higher baseline liver volumes and larger TLV/weight ratios retained their volume after an initial loss while smaller livers continuously lost volume after TIPS. At the first follow-up period (90–180 d post-TIPS) lower liver volumes and TLV/weight ratios were associated with higher bilirubin levels. Within the final multivariable model containing time (days since TIPS), baseline INR and baseline TLV, the average loss of liver volume was 0.74 mL per day after TIPS. Twelve-month overall transplant-free survival was 89% and median overall TFS was 33 months. The median TFS for a baseline TLV/body weight ratio > 20 was significantly higher compared with ≤20 (40.0 vs. 27.0 months, p = 0.010) while there were no differences regarding the indication for TIPS or etiology of liver disease in the matched pair analysis. Lower TLV/weight ratios before TIPS were associated with shorter TFS and should therefore be critically considered when selecting patients for TIPS. In addition, this study provides first evidence of an effect of TIPS on subsequent liver volume change and associated liver function.

Recurrence of Primary Sclerosing Cholangitis After Liver Transplant in Children: An International Observational Study.

Recurrent primary sclerosing cholangitis (rPSC) following liver transplant (LT) has a negative impact on graft and patient survival; little is known about risk factors for rPSC or disease course in children. We retrospectively evaluated risk factors for rPSC in 140 children from the Pediatric PSC Consortium, a multicenter international registry. Recipients underwent LT for PSC and had >90days of follow-up. The primary outcome, rPSC, was defined using Graziadei criteria. Median follow-up after LT was 3years (interquartile range 1.1-6.1). rPSC occurred in 36 children, representing 10% and 27% of the subjects at 2years and 5years following LT, respectively. Subjects with rPSC were younger at LT (12.9 vs. 16.2years), had faster progression from PSC diagnosis to LT (2.5 vs. 4.1years), and had higher alanine aminotransferase (112 vs. 66IU/L) at LT (all P<0.01). Inflammatory bowel disease was more prevalent in the rPSC group (86% vs. 66%; P=0.025). After LT, rPSC subjects had more episodes of biopsy-proved acute rejection (mean 3 vs. 1; P<0.001), and higher prevalence of steroid-refractory rejection (41% vs. 20%; P=0.04). In those with rPSC, 43% developed complications of portal hypertension, were relisted for LT, or died within 2years of the diagnosis. Mortality was higher in the rPSC group (11.1% vs. 2.9%; P=0.05). The incidence of rPSC in this cohort was higher than previously reported, and was associated with increased morbidity and mortality. Patients with rPSC appeared to have a more aggressive, immune-reactive phenotype. These findings underscore the need to understand the immune mechanisms of rPSC, to lay the foundation for developing new therapies and improve outcomes in this challenging population.

Идиопатическая нецирротическая портальная гипертензия и ее причины

Идиопатическая нецирротическая портальная гипертензия представляет собой плохо диагностируемое заболевание неясной этиологии. Заболевание прогрессирует медленно, является доброкачественным, прогноз более оптимистичный, чем при лечении осложнений портальной гипертензии. Данное заболевание фиксируют в различных странах мира и в разнообразных этнических группах с различными факторами риска, чем обусловлены существование разнообразной терминологии и отсутствие стандартизированных критериев диагностики, хотя для постановки диагноза «идиопатическая нецир­ротическая портальная гипертензия» требуется клинически и гистологически исключить другую патологию.

Неинвазивная оценка структурных изменений печени при ее диффузных заболеваниях

Актуальность. К важнейшим факторам, которые влияют на прогноз, тактику и результаты лечения хронических диффузных заболеваний печени (ХДЗП), в частности неалкогольной жировой болезни печени (НАЖБП) и хронического гепатита, ассоциированного с вирусом С (ХГС), относятся скорость прогрессирования фиброзной транс­формации печени и площадь фиброзной ткани. Распространенность фиброза является предиктором портальной гипертензии и таких летальных осложнений, как кровотечение из варикозно расширенных вен пищевода, выраженная печеночная недостаточность и гепатоцеллюлярная карцинома. Материалы и методы. Обследованы 66 пациентов с ХДЗП. Все больные были разделены на две группы. I группу составили пациенты с ХГС и циррозом печени, ассоциированным с вирусом гепатита С, во II группу вошли пациенты с НАЖБП. В зависимости от выраженности фиброза пациенты каждой группы были разделены на три подгруппы: без фиброза (F0), с умеренным (FI + FII) и выраженным фиброзом (FIII + FIV). Вирусная этиология болезни была подтверждена с помощью иммуноферментного анализа и ПЦР-диагностики. I группу составили 24 (57,1 %) мужчины и 18 (42,9 %) женщин, средний возраст пациентов был (45,8 ± 2,2) года. Диагноз НАЖБП подтверждали с помощью объективного обследования (наличие избыточного веса тела, висцерального распределения жировой ткани), выявления инсулинорезистентности и гиперлипидемии. II группу составили 8 мужчин (33,3 %) и 16 женщин (66,7 %), средний возраст (47,3 ± 3,1) года. У всех больных диагноз был подтвержден морфологически. Выполняли ультразвуковое исследование печени в В-режиме, а также сдвиговолновую эластографию (SWE). Результаты. В 13 (19,7 %) случаях (у 7 больных ХГС и у 6 пациентов с НАЖБП) фиброзные изменения печени отсутствовали. В 43,9 % случаев (у 19 больных ХГС и у 10 пациентов с НАЖБП) диагностирована умеренная стадия фиброза печени, что соответствовало 1 и 2 стадии фиброза по шкале METAVIR. В 36,4 % случаев (у 16 больных ХГС и у 8 пациентов с НАЖБП) диагностирована стадия выраженного фиброза печени, что соответствовало 3 и 4 стадии фиброза по шкале METAVIR. По данным ROC-анализа, пороговое значение SWE, выше которого диагностировали умеренную стадию фиброза печени при ХГС, составляло 6,63 кПа (с чувствительностью 94,7 % и специфичностью 85,7 %), при оценке выраженного фиброза — более 8,81 кПа (с чувствительностью 93,7 % и специфичностью 84,2 %). При НАЖБП пороговое значение для умеренного фиброза — 5,56 кПа (AUC = 0,867 (95% ДИ 0,606–0,982; р &lt; 0,001)), для выраженного — более 7,87 кПа (с чувствительностью 87,5 % и специфичностью 90,0 %). Выводы. Умеренный фиброз по данным SWE у больных ХГС соответствует значению 6,63 кПа и 5,56 кПа — для НАЖБП; выраженный фиброз диагностируют при показателях более 8,81 и 7,87 кПа для ХГС и НАЖБП соответственно. Метод SWE обладает высокой чувствительностью и специфичностью для определения степени фиброза печени у пациентов с ХГС и НАЖБП и может быть рекомендован для использования в клинической практике. Внедрение SWE с использованием определенных пороговых значений показателей жесткости позволяет оптимизировать неинвазивную диагностику структурных изменений печени у пациентов с ХГС и НАЖБП.

Transjugular intrahepatic portosystemic shunts in portal vein thrombosis: A review.

The presence of portal vein thrombosis (PVT) has previously been considered a contraindication to the insertion of transjugular intrahepatic portosystemic shunts (TIPSS). However, patients with PVT may have portal hypertension complications and may thus benefit from TIPSS to reduce portal venous pressure. There is an increasing body of literature that discusses the techniques and outcomes of TIPSS in PVT. This review summarizes the techniques, indications and outcomes of TIPSS in PVT in published case reports, case series and comparative trials, especially regarding the reduction in portal hypertensive complications such as variceal bleeding. A comprehensive literature search was conducted using MEDLINE and PubMed databases. Manuscripts published in English between 1 January 1990 and 1 March 2021 were used. Abstracts were screened and data from potentially relevant articles analyzed. TIPSS in PVT has been reported with high levels of technical success, short-term portal vein recanalization and long-term PV patency and TIPSS patency outcomes. Several comparative studies, including randomized controlled trials, have shown favorable outcomes of TIPSS compared with non-TIPSS treatment of PVT complications. Outcomes of TIPSS with PVT appear similar to those in TIPSS without PVT. However, TIPSS may be more technically difficult in the presence of PVT, and such procedures should be performed in expert high-volume centers to mitigate the risk of procedural complications. The presence of PVT should no longer be considered a contraindication to TIPSS. TIPSS for PVT has been acknowledged as a therapeutic strategy in recent international guidelines, although further studies are needed before recommendations can be strengthened. KEY POINTS: Portal vein thrombosis (PVT) is no longer a contraindication to the insertion of transjugular intrahepatic portosystemic shunts (TIPSS) TIPSS often leads to the spontaneous dissolution of PVT, but can be combined with mechanical or pharmacological thrombectomy TIPSS reduces portal hypertensive complications of PVT, such as variceal bleeding, and can also facilitate liver transplantation where PVT may otherwise interfere with vascular anastomoses Studies have shown favorable long-term outcomes of TIPSS compared with TIPSS without PVT; as well as compared with non-TIPSS treatment of PVT complications TIPSS in PVT should be performed in high-volume specialist centers due to technical difficulties.