Porto-sinusoidal vascular disease: proposal and description of a novel entity.

Portal hypertension in the absence of portal vein thrombosis and without cirrhosis, but with mild or moderate alterations of liver histology (eg, obliterative venopathy, nodular regenerative hyperplasia, or incomplete septal cirrhosis) is being increasingly recognised. Owing to the heterogeneity of causes and histological findings, a substantial number of terms have been used to describe such idiopathic non-cirrhotic portal hypertension. Patients with the same clinical and histological features exist, but without portal hypertension at the time of diagnosis. Therefore, improved criteria are needed to define this form of liver disease. Here, we propose the term porto-sinusoidal vascular disease, since all lesions found involve the portal venules or sinusoids. The definition of this entity is based on the characteristic absence of cirrhosis with or without signs of portal hypertension or histological lesions. The presence of known causes of liver disease does not rule out porto-sinusoidal vascular disease, but specific causes of vascular liver disease are excluded from its definition. The diagnosis of porto-sinusoidal vascular disease is based on liver biopsy and might include signs specific for portal hypertension with normal or mildly elevated liver stiffness values and no complete portal vein thrombosis. We provide simple diagnostic criteria, because agreement on a uniform nomenclature is an essential requirement for future collaborative studies.

Clinically Evident Portal Hypertension: An Operational Research Definition for Future Investigations in the Pediatric Population.

Portal hypertension (PHT) is a major cause of morbidity and mortality in pediatric liver diseases. Thus, research into causes and disease modifiers in PHT in these conditions is vitally important. PHT is rarely directly or indirectly measured in the assessment of children with chronic liver disease. A straightforward, reproducible definition of PHT could be invaluable for consistently identifying patients with PHT and for grouping these patients according to their risk of complications from their disease. We propose the term Clinically Evident Portal Hypertension (CEPH) to denote clinical findings that demonstrate evidence of elevated portal pressure. When CEPH criteria are met, PHT is highly likely to be present, although it is likely that PHT exists for variable periods of time before meeting CEPH criteria. Use of this research definition of CEPH will allow for consistent identification of these patients by clinicians in nearly any clinical setting and serve as a clinical milepost that may dictate future prognosis in pediatric patients with cirrhosis.

Canine and feline abdominal arterioportal communications can be classified based on branching patterns in computed tomographic angiography.

Arterioportal vascular anomalies are communications between the splanchnic arteries and the portal system that represent a rare cause of presinusoidal portal hypertension in small animals. There is little information concerning the imaging findings of arterioportal communications in small animals and no classification could be found for radiologists and surgeons. The aims of this retrospective descriptive multicentric study were to describe the computed tomographic characteristics of arterioportal communications in a group of cats and dogs, and to propose a classification based on computed tomography (CT) angiographic anatomy. Computed tomography databases from multiple veterinary hospitals were searched for cats and dogs with a diagnosis of arterioportal communication. A total of 36 animals (33 dogs, three cats) met the inclusion criteria. There were 32 intrahepatic arterioportal malformations and four extrahepatic fistulae. The intrahepatic arterioportal malformations were classified as right divisional (11/32) and left divisional (21/32), and the left divisional were subclassified as left medial (16/21) and left lateral (4/21). One patient showed multiple intrahepatic arterioportal communications with concomitant left medial and left lateral conformations. Two patients with intrahepatic arteriovenous malformation showed concomitant congenital intrahepatic shunts. The proposed anatomical classification based on CT angiography could allow veterinary radiologists to have a more systematic approach and help improve the radiologist-surgeon communication.

Hiperplasia nodular regenerativa hepática asociada a inmunodeficiencia común variable y a otras comorbilidades / Nodular regenerative hyperplasia associated with common variable immunodeficiency and other comorbidities

Fundamento y objetivo: Actualmente no se dispone de muchos datos sobre la evolución de la hiperplasia nodular regenerativa (HNR) asociada o no a enfermedades de base, y en concreto de la asociada a inmunodeficiencia común variable (IDCV). Se presentan 20 casos de HNR y se analizan las diferencias entre los casos asociados a IDCV y los relacionados con otras enfermedades. Métodos: Estudio retrospectivo y descriptivo durante un período de 14 años. Resultados: De los 20 pacientes, 12 eran hombres; la mediana de edad fue de 51 años. La IDCV fue la principal enfermedad asociada con HNR. En los pacientes con IDCV y HNR la hemorragia gastrointestinal fue más común, todos tenían elevación de FA y GGT y ninguno tenía valores de albúmina y bilirrubina alterados comparados con los pacientes sin IDCV. De los pacientes con HNR en seguimiento han fallecido el 50% de los asociados a IDCV (2/4) frente al 33,3% (5/15) sin IDCV. Conclusiones: La HNR en los pacientes con IDCV parece manifestarse más con datos bioquímicos de colestasis anictérica e hipertensión portal y podría asociar una supervivencia menor (AU)

Background and objective: Currently, there are not many data on the evolution of nodular regenerative hyperplasia (NRH) associated or not with underlying diseases and in particular that associated with common variable inmunodeficiency (CVID). Twenty cases of NRH are presented, and the differences between the cases associated with CVID and those related to other diseases are analysed. Methods: Retrospective and descriptive study over a period of 14 years. Results: Twelve out of the 20 patients were men; the median age was 51 years. CVID was the main illness associated with NRH. In patients with CVID and NRH, gastrointestinal haemorrhage was more common, all the patients had high gamma glutamyl transferase and alkaline phosphatase and none had altered albumin and bilirubin levels compared to the patients without CVID. On follow-up, 50% of patients with CVID (2/4) had died compared to 33.3% (5/15) without CVID. Conclusions: NRH in patients with CVID seems to have more biochemical data of anicteric cholestasis and portal hypertension and could be associated with lower survival (AU)

[DETERMINING THE DEGREE OF DISRUPTION OF THE STRUCTURE OF THE LIVER AND THE SEVERITY OF PORTAL HYPERTENSION IN CHILDREN].

AIM: To develop a system to define the degree of liver disruption and severity of portal hypertension in children based on the International Classification of Functioning, Disability and Health (ICF). PATIENTS AND METHODS: Studied the results of laboratory and instrumental methods 382 children: 267 patients with various liver diseases, including 49 patients who underwent liver transplantation, and 115 children without liver disease. RESULTS: Based on analysis of statistical data obtained were identified 10 indicators, a set of changes which can be used to assess the degree of disruption of the structure of the liver and the severity of portal hypertension: indicators that reflect the severity of fibrosis and cirrhosis of the liver (METAVIR score on a scale at fibroelastometrii, scores are Desmet at morphological study of the liver) and indicators that reflect the severity of portal hypertension (the diameter of the portal vein, splenic vein diameter, the length of the spleen, recanalization of the umbilical vein, esophageal varices, ascites, hydropericardium, hydrothorax). Each of the indicators was assessed on a 5-point system. Number of points reflects the sum of the changes of these parameters. Decrease the number of points on 0-4% (38-40 points) is regarded as a lack of structural failure of the liver and the severity of portal hypertension by 5-24% (30-37 points)--minor violations on 25-49% (20-29 points) -moderation disorders, 50-95% (3-12 points)--severe handicaps, 96-100% (0-2 points)--absolute violation. Studied the dynamics of children with autoimmune hepatitis, Wilson's disease and chronic hepatitis C. CONCLUSION: The proposed scoring system for assessing the degree of disruption of the structure of the liver and the severity of portal hypertension can be used as an objective criterion of the severity of the pathological process, to estimate the dynamics of defeat against the background of the therapy, determining the prognosis of the disease and as a criterion of the indications for liver transplantation.

Clinically severe portal hypertension: role of multi-detector row CT features in diagnosis.

BACKGROUND AND AIM: To explore the CT signs which permit estimation of clinically severe portal hypertension (PH) [≥ 12 of hepatic vein pressure gradient (HVPG)]. METHODS: One-hundred and seven consecutive patients who underwent HVPG measurement in the PH group and 52 controls were included. The diameters of main portal vein (øMPV), superior mesenteric vein (øSMV), splenic vein (øSV), and left gastric vein, øMPV/øSV, øSMV/øSV, as well as estimated spleen volumes were evaluated on the CT scan. The grade of varix and ascites were also evaluated semi-quantitatively. We explored the statistically significant CT features related to severe PH and performed a logistic regression analysis for an estimation model for severe PH. RESULTS: øMPV/øSV and øSMV/øSV tended to gradually increase as the PH became severer, and the difference between severe and not severe groups was statistically significant (p = 0.015 and 0.038, respectively). According to the regression analysis, øSMV/øSV and the grade of esophageal varix and ascites were finally included as related variables for predicting severe PH. The odds ratio (OR) of øSMV/øSV was 4.596, and large esophageal varix (OR 4.135) and mild (OR 3.051) and large amount of ascites (OR 21.781) were statistically significantly related to severe PH. CONCLUSION: Changing diameters of portal system, the grades of esophageal varices and ascites on multi-detector row computed tomography might be indicative features for clinically severe PH.

[Pharmacotherapy of portal hypertension and its complications: analysis of efficacy of preparations for clinical practice and discussion of promising methods of treatment].

Bleeding from oesophageal varicose veins is the terminal stage of a sequence of complications of liver cirrhosis caused by progressive fibrosis, circulation blockade, and development of portal hypertension syndrome followed by collateral shunt. It leads to progressive vein dilation and their rupture. The main issue of today is to prevent the development of successive stages of portal hypertension, to search for therapeutic and surgical methods for marked reduction of pressure in the portal system, and to prevent the risk of hemorrhage from varicose veins. Another approach is to use local endoscopic treatment of varicose veins for prevention of their rupture. The authors analyse the efficacy of pharmacotherapy in patients with liver cirrhosis and portal hypertension and discuss the existing recommendations on the prevention of hemorrhage with special reference to the yet unsolved problems and prospects for the improvement of therapy.

Surgical management of portal hypertension in children.

The management of children with portal hypertension has dramatically changed during the past decade, with an improvement in outcome. This has been achieved by improved efficiency of endoscopic variceal control and the success of liver transplantation. Emergency surgical shunt procedures are rarely required, with acute bleeding episodes generally controlled endoscopically or, occasionally in adults, by interventional radiological procedures. Portosystemic shunts may be considered as a bridge to transplant in adults but are rarely used in this context in children. Nontransplant surgery or radiological interventions may still be indicated for noncirrhotic portal hypertension when the primary cause can be cured and to allow normalization of portal pressure before liver parenchyma is damaged by chronic secondary changes in some specific diseases. The meso-Rex bypass shunt is used widely but is limited to those with a favorable anatomy and can even be performed preemptively. Elective portosystemic shunt surgery is reserved for failure to respond to conservative management in the absence of alternative therapies.

[Portal gastropathy: diagnosis, classification].

Portal hypertensive gastropathy (PHG) is a complex of secondary macroscopic and microscopic changes in the mucous layer of the stomach, resulting from portal hypertension of any origin. The overall prevalence of PHG ranges from 9.1 to 80%. PHG is a potential cause of an acute or chronic gastric bleeding. The presence of PHG is associated with prognosis deterioration that dictates the need for improved diagnosis and treatment strategy. The article summarizes literature on the pathological changes, diagnosis and classification of portal hypertensive gastropathy.

Etiología de la hipertensión porta: segunda parte / Etiology of hypertension carries: part two

De 130 pacientes estudiados, 73 eran del género masculino. La mayoría de los pacientes tenían de 2 a 11 años. Entre las causas de hipertensión porta (HTP)prehepática, la muestra quedó constituida por 62 niños, la causa más frecuente del bloqueo sanguíneo correspondió a la trombosis de la vena porta en 34 de ellos y la degeneración cavernomatosa en 26. Entre las causas de HTP posthepática, se encontró en 10 niños, de los cuales 7 presentaron un síndrome de Budd-Chiari, 3 pacientes con trombosis de la vena cava en su porción poshepática, 1 con estenosis de las venas suprahepáticas y 2 hermanos con trombosis de las venas suprahepáticas.

Of 130 patients studied, 73 were male. Most patients were aged 2 to 11 years. Among the causes of prehepatic portal hypertension (PHT), the sample wascomposed of 62 children, the most common cause of blood clotting corresponded to the portal veinthrombosis in 34 of them and cavernomatosa degeneration in 26. Causes of PHT posthepatic wasfound in 10 children, of whom 7 had a Budd-Chiari syndrome, 3 patients with thrombosis of the vena cava in its portion posthepatic 1 with hepatic vein stenosis with thrombosis and 2 brothers of the hepatic veins.

Non-cirrhotic portal hypertension with large regenerative nodules: a diagnostic challenge.

Non-cirrhotic portal hypertension is a poorly understood condition characterized by portal hypertension in the absence of conventional hepatic cirrhosis and described in association with blood coagulation disorders, myeloproliferative and immunological diseases and with exposure to toxic drugs. Very recently, precise classification criteria have been proposed in order to define four distinct subcategories. The present case highlights how the clinical presentation, the confounding results from imaging studies, and the difficulties in the histological evaluation often render cases of non-cirrhotic portal hypertension a real diagnostic challenge. It also underscores the classification problems which can be faced once this diagnosis is performed. Indeed, the different subcategories proposed result from the prevalent subtypes in a spectrum of hepatic regenerative responses to a variety of injuries determining microcirculatory disturbances. More flexibility in classification should derive from this etiopathogenic background.

Hipertensión porta / Portal hypertension

La hipertensión porta en niños es un síndrome que resulta del aumento de la presión dentro del sistema venoso porta por encima de 10 mm Hg. Inicialmente puede ser asintomática, luego se puede manifstar con hematemesis, esplenomegalia, anemia, ascitis y circulación colateral. En la hipertensión porta prehepática las pruebas de función del hígado (aminotransferasasy niveles de albúmina) y las hematológicas están mínimamente alteradas. En hipertensión porta losniños se puede complicar con variasmanifestaciones, pero cuando se presenta el sangrado de vías digestivas altas por ruptura de várices esofágicas, puede ser realmente alarmante porque algunas veces es profuso, este generalmente es más frecuente ante un hígado cirrótico porque se suele acompañar con peor función hepática.

Portal hypertension in children is a syndrome that results from increased pressure within the portalvenous system above 10 mm Hg. May be asymptomatic initially, then may manifest with hematemesis, splenomegaly, anemia, ascites and collateral circulation. In prehepatic portal h y p e r t e n s i o n o f l i v e r f u n c t i o n t e s t s (aminotransferase and albumin levels) and hematologic are minimally altered. Portal hypertension in children is complicated by several events, but when presented with upper GI bleeding from ruptured esophageal varices, can be very alarming because sometimes it is heavy, this usually is more common to a cirrhotic liver because it is usually accompanied with poor liverfunction.

Relationship of increased serum brain natriuretic peptide levels with hepatic failure, portal hypertension and treatment in patients with cirrhosis.

BACKGROUND/AIMS: Brain natriuretic peptide is a cardiac neurohormone secreted from ventricles in response to end diastolic pressure and increased volume. It has diuretic, natriuretic and vasodilator effects. In cirrhosis, a hyperdynamic circulation occurs because of hemodynamic and hemostatic alterations. The increase in brain natriuretic peptide concentration shows parallelism with the stage of cirrhosis. The aim of this study is to investigate the relation of increased brain natriuretic peptide level with the pathophysiologic components of cirrhosis and treatment. METHODS: Ninety-five cirrhotic patients in different stages (Child-A: 33; Child-B: 25; Child-C:37) and age and sex matched 86 healthy individuals were recruited for the study. Brain natriuretic peptide concentration was measured with brain natriuretic peptide-Triage test device using fluoresan immune assay method. RESULTS: Brain natriuretic peptide levels of patients with hepatic cirrhosis were significantly higher compared to control group (288.5±329.2/60.2±29.5/p=0.000, respectively). Serum brain natriuretic peptide levels were positively correlated with Child score (Child A-B-C; 201.2±266/258.7±233.6/386.5±407.7, respectively). A negative correlation was observed between brain natriuretic peptide and albumin levels (p=0.002). Brain natriuretic peptide concentration was significantly correlated with the grade of esophagus varices, and presence of ascites and collateral circulation (p=0.006; p=0.001; p=0.002; respectively). Patients receiving with beta-blocker and diuretic treatments had significantly higher brain natriuretic peptide levels. CONCLUSIONS: High brain natriuretic peptide levels in patients with cirrhosis may be due to hepatocellular insufficiency or portal hypertension, but a cardiomyopathy developing insiduously should not be regarded.

Hipertensión portal / Portal hypertension

Portal hypertension (PH) defined as an increase of hydrostatic pressure in the portal vessels is a relevant clinical condition. Pathogenesis of PH, and classification according to anatomical level of increased resistance, as well as the different etiological conditions are analyzed. Mechanisms involved in the development and rupture of gastro-esophageal varices and the consequent variceal hemorrhage are reviewed. The different choice of treatment available in the prevention of variceal bleed or when hemorrhage occurs are discussed.

La hipertensión portal (HP) definida como un aumento de la presión hidrostática en el sistema venoso portal es una condición clínica muy relevante. La fisiopatología de la HP y la clasificación de acuerdo al nivel de las estructuras anatómicas donde se genera el aumento de resistencia así como las diferentes etiologías relacionadas, son analizados. Se revisan los mecanismos que intervienen en el desarrollo y rupturas de las várices gastroesofágicas y de la consecuente hemorragia variceal. Se discuten los diferentes tratamientos incluyendo la prevención de la hemorragia variceal.

Selection criteria for patients undergoing transjugular intrahepatic portosystemic shunt procedures: current status.

The transjugular intrahepatic portosystemic shunt (TIPS) procedure has a well-established role in the management of patients with complications of portal hypertension such as variceal bleeding or refractory ascites. Several clinical variables have been described to be associated with a poor prognosis after a TIPS procedure, including the presence of uncontrollable ascites, the number of sclerotherapy sessions to control a bleeding episode, the use of drugs for hemodynamic support, the use of balloon tamponade to control bleeding, the need for an emergency TIPS procedure, the need for mechanical ventilation, prothrombin time, increased serum creatinine, increased serum bilirubin, encephalopathy, and sepsis. In addition, several scoring systems have been developed and applied to patients undergoing TIPS procedures in an attempt to improve patient selection criteria for this invasive procedure. This article reviews the most important scoring systems that have been developed and applied to patients undergoing emergency or elective TIPS procedures, with particular emphasis on the prognostic index designed for patients undergoing emergency TIPS procedures and the Model for End-stage Liver Disease score designed for patients undergoing elective TIPS procedures. The most practical application of these scoring systems is probably that, with the information provided, the operator is able to discuss with referring physicians, patients, and family members the expected outcomes of this challenging procedure.

Hipertensão portal / Portal hypertension

A hipertensão portal é uma síndrome clínica freqüente em nosso meio e acompanha-se de manifestações clínicas graves. Aqui são abordados alguns aspectos da hipertensão portal, com particular ênfase na fisiopatologia da síndrome e em suas manifestações clínicas. São listadas causas de hipertensão portal e discute-se a importância de informações obtidas durante a história clínica e no exame físico, que permitem o diagnóstico da síndrome e que auxiliam no esclarecimento da possível etiologia do processo

Pulmonary hypertension.

Pulmonary hypertension, in its simplest sense, is elevation of the pulmonary artery pressure above normal. A multitude of diseases may increase the pulmonary artery pressure and result in right ventricular dysfunction. The treatments of pulmonary hypertension are as varied as its causes. The past decade has realized remarkable growth in knowledge of the mechanisms of pulmonary arterial hypertension and, concurrently, therapies for this once uniformly fatal disease. In addition to continuous intravenous epoprostenol, subcutaneous treprostinil and oral bosentan are now FDA approved for the treatment of pulmonary arterial hypertension. Other forms of pulmonary hypertension, such as pulmonary venous hypertension, pulmonary hypertension related to diseases of the respiratory system, and thromboembolic pulmonary hypertension will be discussed.