Características clínicas y hallazgos endoscópicos en niños con hipertensión portal atendidos en un hospital pediátrico / Clinical characteristics and endoscopic findings in children with portal hypertension seen in a pediatric hospital

Antecedentes: El término hipertensión portal (HP) se define como el aumento patológico en el gradiente de presión porto-sistémico en cualquier segmento del sistema venoso portal. Objetivo: Determinar las características clínicas y hallazgos endoscópicos de pacientes con HP atendidos en el Hospital María, Especialidades Pediátricas (HMEP). Métodos: Se realizó un estudio observacional, descriptivo y retrospectivo. El universo fueron todos los pacientes de 18 años o menos con diagnóstico de HP que asistieron al servicio de gastroenterología pediátrica del HMEP entre 2015-2022. Fue tomado todo el universo para aná- lisis. Se realizó análisis de datos descriptivo univariado utilizando el programa STATA 15.1. Resultados: Se analizó un total de 38 pacientes, 55.3% (21/38) de edad preescolar. El 57.9% (22/38) fue masculino y el nivel de escolaridad más frecuente fue primaria incompleta en 55.3%. La procedencia en 79% (30/38) de la zona centro y oriente del país. El Sangrado Digestivo Alto (SDA) se en- contró en 42% de los pacientes (16/38) y la etiología pre-hepática fue la más frecuente en 65.8 % (25). Discusión: se encontró que el inicio de la enfermedad fue en pre-escolares con predominio del sexo masculino; las causas pre-hepáticas fueron la etiología más frecuente de SDA coincidiendo con lo publicado por otros autores. El SDA fue el síntoma inicial predominante, evidenciado en hallazgos endoscópicos como várices esofágicas y gástricas. Conclusión: La HP es poco frecuente en edad pediátrica tiene consecuencias severas en la calidad de vida y sobrevida del paciente...(AU)

Guías para el diagnóstico y seguimiento de niños y adolescentes portadores de hipertensión portal / Guidelines for diagnosis and follow-up of children and adolescents with portal hypertension

La hipertensión portal es un síndrome complejo producido por un aumento de la resistencia al flujo venoso esplácnico a nivel de la vena porta o sus ramas, con una circulación sistémica hiperdinámica caracterizada por vasodilatación periférica y aumento del gasto cardíaco. El sitio de obstrucción al flujo portal puede ser prehepático (hígado normal), intrahepático (como en la cirrosis) o posthepático (síndrome de BuddChiari). En los pacientes pediátricos, las causas prehepáticas e intrahepáticas se reparten en proporciones casi iguales (aproximadamente el 50 % cada una). La expresión clínica y el impacto individual son muy variados, pero en todos los casos expresan un deterioro en la salud de los pacientes y la necesidad de corregir el problema, tanto en sus consecuencias como, idealmente, en sus causas.

Portal hypertension is a complex syndrome caused by increased resistance to the splachnic venous flow at the portal vein level, with a hyperdynamic systemic circulation characterized by peripheral vasodilation and high cardiac output. Portal flow can be obstructed at prehepatic (¨normal liver¨), intrahepatic (as in cirrhosis), or post-hepatic level (as in Budd-Chiari syndrome). In pediatric patients, prehepatic and intrahepatic causes are almost equally distributed (nearly 50% each). Clinical presentation and individual impact are heterogeneous, but in each case, it is the expression of a worsening condition and the need to solve the problem, either by treating its consequences or (ideally) its causes.

Cavernomatosis de la porta: presentación de un caso / Portal cavernomatosis: a case report

Fundamento: La cavernomatosis portal es una enfermedad poco frecuente causada por la trombosis de la vena porta, que provoca hipertensión portal (HP). Se ha relacionado con la realización de cateterismo umbilical, traumatismos abdominales e infecciones del período neonatal. La presentación clínica más frecuente es la hemorragia digestiva alta, con o sin melena, esplenomegalia, red venosa colateral y en etapas tardías puede observarse pancitopenia. Los métodos diagnósticos son ecografía abdominal, endoscopía digestiva y la angiotomografía. El diagnóstico definitivo es anatomopatológico. La literatura internacional y nacional es escasa para esta enfermedad, predominando el reporte de casos referidos a la edad pediátrica. Objetivo: presentar las características que definen esta enfermedad, en ocasión de darle seguimiento terapéutico a un paciente. Presentación de caso: se presenta un paciente de 20 años de edad, cuyo diagnóstico fue eventual por hallazgo ultrasonográfico en el periodo neonatal, con retraso madurativo y malnutrición proteico-energética. Conclusiones: la cavernomatosis portal o transformación cavernomatosa de la porta se define como la dilatación de las venas paracoledocianas y epicoledocianas generalmente secundaria a una trombosis portal, con una escasa prevalencia, fundamentalmente en edades pediátricas, que constituye la primera causa de hipertensión portal en este grupo etario. Provoca retardo del desarrollo pondoestatural, malnutrición proteicoenergética y sangramientos digestivos.

Background: Portal cavernomatosis is a rare disease caused by portal vein thrombosis, causing portal hypertension. It has been associated with performing umbilical catheterization, abdominal trauma and infections in the neonatal period. The most frequent clinical presentation is bleeding upper digestive, with or without melena, splenomegaly, collateral venous network and pancytopenia can be observed in late stages. Diagnostic methods are abdominal ultrasound, digestive endoscopy, and angiotomography. The definitive diagnosis is pathological. The international and national literature is scarce for this disease, with the predominant reporting of cases referring to pediatric age. Objective: to present the characteristics that define this disease, on the occasion of giving therapeutic follow-up to a patient. Case presentation: a 20-year-old patient is presented, whose diagnosis was eventual by ultrasound finding in the neonatal period, with maturational delay and protein-energy malnutrition. Conclusions: portal cavernomatosis or cavernomatous transformation of the Porta is defined as the dilation of the paracholedocian and epicoledocian veins generally secondary to portal thrombosis, with a low prevalence, mainly at pediatric ages, which is the leading cause of portal hypertension in this group. etareo. It causes delayed development of the body, protein-energy malnutrition and digestive bleeding.

Fibrose hepática congênita e venopatia portal obliterativa sem hipertensão portal - revisão da literatura com base em um caso assintomático / Congenital hepatic fibrosis and obliterative portal venopathy without portal hypertension - a review of literature based on an asymptomatic case

ABSTRACT The disease and the case reported here are relevant especially because of their varied clinical presentation, possibility of being associated with other disorders affecting several organs and possible differential diagnoses. Congenital Hepatic Fibrosis is an autosomal recessive disease due to mutation in the PKHD1 gene, which encodes the fibrocystin/polyductine protein. It is a cholangiopathy, characterized by varying degrees of periportal fibrosis and irregular proliferation of bile ducts. Affected patients are typically diagnosed in childhood, but in some cases the disease may remain asymptomatic for many years. The exact prevalence and incidence of the disease are not known, but it is consider a rare disease, with a few hundred cases described worldwide. It can affect all ethnic groups and occur associated with various hereditary and non-hereditary disorders. The clinical presentation is quite variable, with melena and hematemesis being initial symptoms in 30%-70% of the cases. More rarely, they may present episodes of cholangitis. The disease has been classified into four types: portal hypertension, cholestasis / cholangitis, mixed and latent. Diagnosis begins with imaging tests, but the definition is made by the histopathological sample. So far, there is no specific therapy that can stop or reverse the pathological process. Currently, the therapeutic strategy is to treat the complications of the disease.

RESUMO A patologia e o caso aqui reportados são relevantes especialmente devido sua variada apresentação clínica, possibilidade de estar associada com outras desordens acometendo diversos órgãos e pelos possíveis diagnósticos diferenciais. A fibrose hepática congênita é uma doença autossômica recessiva, devido mutação no gene PKHD1, que codifica a proteína fibrocistina/poliductina. É uma colangiopatia, caracterizada por variados graus de fibrose periportal e proliferação irregular de ductos biliares. Os pacientes acometidos são tipicamente diagnosticados na infância, mas em alguns casos a doença pode permanecer assintomática por muitos anos. Exatas prevalência e incidência da doença não são conhecidas, mas sabe-se que é uma doença bastante rara, com algumas centenas de casos descritos no mundo. Pode afetar todos grupos étnicos e ocorrer associada com diversas desordens hereditárias e não-hereditárias. A apresentação clínica é bastante variável, com melena e hematêmese sendo sintomas iniciais em 30%-70% dos casos. Mais raramente, podem apresentar episódios de colangite. A doença tem sido classificada em quatro tipos: hipertensão portal, colestática/colangite, mista e latente. O diagnóstico inicia com exames de imagem, mas a definição é feita pela amostra histopatológica. Até o momento, não há terapia específica que possa parar ou reverter o processo patológico e a estratégia terapêutica atual é tratar as complicações da doença.

Hipertensión portopulmonar: Revisión actualizada / Portopulmonary hypertension: Updated review

Resumen: La hipertensión portopulmonar (HPP) es una entidad poco frecuente a nivel mundial, aunque se desconocen los datos epidemiológicos en México. Sin embargo, las enfermedades crónicas del hígado son muy prevalentes en mexicanos. La HPP es el 4.◦ subtipo en frecuencia del grupo de la hipertensión arterial pulmonar. Su diagnóstico está dentro de 2 escenarios: los pacientes con sospecha de hipertensión pulmonar y los candidatos a trasplante hepático ortotópico (THO). Tanto el ecocardiograma como el cateterismo cardiaco derecho son determinantes para el diagnóstico en ambos escenarios. La HPP es un reto para el THO, pues aumenta la mortalidad perioperatoria de manera importante. El uso de terapia específica es la piedra angular de este padecimiento, como una medida para poder mejorar el desenlace de los que llegan a ser candidatos a un THO con HPP moderada a grave. Es importante reconocer que la HPP puede llegar a ser una contraindicación para el THO. Hasta el momento el papel del trasplante combinado pulmón-hígado o corazón-pulmón-hígado como una medida de curación de la enfermedad vascular pulmonar en pacientes con HPP es incierto. © 2016 Instituto Nacional de Cardiolog´ıa Ignacio Cha´vez. Publicado por Masson Doyma Me´xico S.A. Este es un art´ıculo Open Access bajo la licencia CC BY-NC-ND (https://creativecommons.org/licenses/by-nc-nd/4.0/).

Abstract: Portopulmonary hypertension (PPH) is a rare condition worldwide, although epidemiological data are unknown in Mexico. However, chronic liver diseases are very prevalent in Mexico. PPH is the 4th subtype in frequency in the group of pulmonary arterial hypertension. Its diagnosis is made within 2 scenarios: patients with suspected pulmonary hypertension and candidates for orthotopic liver transplantation (OLT). Both echocardiogram and a right cardiac catheterisation are crucial for diagnosis in both cases. PPH is a challenge for OLT, since it can significantly increase perioperative mortality. The use of specific therapy is the cornerstone of this disease, as a measure to improve the outcome of those who become candidates for OLT with moderate to severe PPH. It is important to recognise that PPH can be a contraindication to OLT. The role of lung-liver transplantation or heart-lung-liver transplantation as a measure to heal pulmonary vascular disease in patients with PPH is still uncertain.© 2016 Instituto Nacional de Cardiolog´ıa Ignacio Cha´vez. Published by Masson Doyma Me´xico S.A. This is an open access article under the CC BY-NC-ND license (http://creativecommons. org/licenses/by-nc-nd/4.0/).

Observational Cohort Study of Hepatic Encephalopathy After Transjugular Intrahepatic Portosystemic Shunt (TIPS)

Abstract: Introduction and aim. Hepatic encephalopathy (HE) is a common complication of transjugular intrahepatic portosystemic shunting (TIPS). It is associated with a reduced quality of life and poor prognosis. The aim of this study was to compare two groups of patients who did and did not develop overt HE after TIPS. We looked for differences between these groups before TIPS. Material and methods. A study of 895 patients was conducted based on a retrospective analysis of clinical data. Data was analyzed using Fisher’s exact test, χ2, Mann Whitney test, unpaired t-test and logistic regression. After the initial analyses, we have looked at a regression models for the factors associated with development of HE after TIPS. Results. 257 (37.9%) patients developed HE after TIPS. Patients’ age, pre-TIPS portal venous pressure, serum creatinine, aspartate transaminase, albumin, presence of diabetes mellitus and etiology of portal hypertension were statistically significantly associated with the occurrence of HE after TIPS (p < 0.01). However, only the age, pre-TIPS portal venous pressure, serum creatinine, presence of diabetes mellitus and etiology of portal hypertension contributed to the regression model. Patients age, serum creatinine, presence of diabetes mellitus and portal vein pressure formed the model describing development of HE after TIPS for a subgroup of patients with refractory ascites. Conclusion. We have identified, using a substantial sample, several factors associated with the development of HE after TIPS. This could be helpful in further research.

Hipertensión portal no cirrótica por didanosina: Un caso infrecuente / Non-cirrhotic portal hypertension due to didanosina: A rare case

El compromiso hepático es usualmente visto en pacientes con infección por el virus de inmunodeficiencia humana (VIH), sobretodo en pacientes coinfectados con el virus de la hepatitis B o C, con el abuso de alcohol, etc. Sin embargo, existe un grupo de pacientes que desarrolla compromiso hepático e hipertensión portal de causa no específica. La hipertensión portal no cirrótica (HPNC) es un desorden hepático descrito recientemente, potencialmente grave, que ha sido reportado en pacientes infectados por el VIH con terapia antirretroviral de gran actividad (TARGA), específicamente didanosina (DDI). La fisiopatología involucra al agente infeccioso (VIH) y a su tratamiento (TARGA), pues ambas generan una venulopatía prehepática portal. Además, la infección por el VIH genera un estado protrombótico por deficiencia de proteína S conllevando a la obliteración de pequeñas vénulas hepáticas. Se ha postulado a la didanosina como un cofactor en la patogénesis del HPNC. Todo ello conlleva a que en muchas de las biopsias hepáticas se evidencie una hiperplasia nodular regenerativa. Se reporta el caso de una paciente con infección del VIH y en tratamiento con DDI de larga data que debuta con hemorragia digestiva alta (HDA) y ascitis como consecuencia de la HPNC, cuyo diagnóstico fue corroborado por biopsia. No existe reporte de casos del tema en nuestro país

Liver involvement is usually seen in patients infected with the human immunodeficiency virus (HIV), especially in patients coinfected with hepatitis B or C, in alcohol abuse, etc. However, there is a group of patients who develop liver involvement and portal hypertension of unspecified cause. Non-cirrhotic portal hypertension (NCPH) is a liver disorder recently described, but potentially serious. It has been reported in HIV-infected patients with highly active antiretroviral therapy (HAART), specifically didanosine (DDI). The pathophysiology involves the infectious agent (HIV) and its treatment (HAART), since both generate a pre-hepatic portal venulopathy. Similarly, HIV infection produces a prothrombotic state by protein S deficiency leading to the obliteration of small hepatic venules. It has been postulated that DDI as a cofactor in the pathogenesis of NCPH. All this leads that many of the liver biopsies show nodular regenerative hyperplasia. We present the case of a HIV-infected patient who was treated with a longstanding DDI. She developed upper gastrointestinal bleeding (UGB) and ascites due to NCPH, whose diagnosis was confirmed by biopsy. However, there is no similar study in our country

Caput Medusae due to portal hypertension in schistosomiasis mansoni / Cabeza de Medusa debida a la hipertensión portal en esquistosomiasis mansoni

A 62-year-old Brazilian man who lived in endemic areas of tropical diseases had an episode of hematemesis associated with portal hypertension. He used to swim in natural ponds during childhood and developed the hepatosplenic form of schistossomiasis with moderate ascites, in addition to the characteristic features of abdominal Caput Medusae. The aim of the report is highlight the role of chronic liver disease and schistossomiasis

Un hombre natural de Brasil de 62 años de edad que vivía en zonas endémicas de enfermedades tropicales presentó un episodio de hematemesis asociada con hipertensión portal. Frecuentemente se bañaba en los estanques naturales durante la infancia y desarrolló la forma hepatosplénica de la esquistosomiasis con ascitis moderada, además de los rasgos abdominales característicos de la Cabeza de Medusa. El objetivo del informe es poner de relieve el papel de la enfermedad hepática crónica y de la esquistosomiasis

Supervivencia del paciente cirrótico con gastropatía portal hipertensiva / Survival of the Cirrhotic Patient with Hypertensive Portal Gastropathy

Introducción: la gastropatía portal hipertensiva constituye una complicación de la hipertensión portal que ocurre en pacientes cirróticos. Objetivo: determinar la probabilidad de supervivencia en un grupo de pacientes cirróticos con gastropatía portal hipertensiva. Métodos: seguimiento longitudinal, prospectivo, para estudiar la supervivencia de 34 pacientes con cirrosis hepática atendidos en el servicio de Gastroenterología del Hospital Militar Central Dr. Luis Díaz Soto desde octubre de 2012 hasta octubre del 2015. Se construyeron las curvas de sobrevida total y por estratos, según clasificación de Child-Pugh-Turcotte, etiología de la cirrosis, gravedad de la gastropatía y ocurrencia de sangrado agudo durante el período de observación. Se utilizó para ello el método de Kaplan-Meier y la comparación de las curvas por el logaritmo de rangos. Resultados: se apreció una probabilidad de supervivencia global de 94,1 por ciento a los dos meses y 61,7 por ciento a los 30 meses. La media de la supervivencia para pacientes con gastropatía leve y grave fue de 29,1 y 29,7 meses, respectivamente; esta fue de 28,3 meses para casos con cirrosis por virus C y de 30,1 meses para los de otras etiologías. Hubo predominio de casos en estadios de Child A (41,2 percent) y B (47,1 percent), con una media de supervivencia de 33,5 y 30,1 meses; para los del Child C (12 por ciento) fue de 12 meses; (p= 0,05). De los pacientes, el 35,3 por ciento de los casos sangraron, con una media de supervivencia de 25,5 meses inferior respecto a los que no sangraron (p= 0,35). Conclusiones: la presencia de gastropatía portal hipertensiva se relaciona con una mayor probabilidad de fallecer cuando hay un mayor deterioro de la función hepática o ha ocurrido un sangrado agudo, a partir de los 30 meses de haberse realizado su diagnóstico(AU)

Introduction: Hypertensive portal gastropathy is a complication of portal hypertension that occurs in cirrhotic patients. Objective: Determine the probability of survival in a group of cirrhotic patients with hypertensive portal gastropathy. Methods: a longitudinal, prospective follow-up was conducted to study the survival of thirty four (34) patients with liver cirrhosis and they were treated at the Gastroenterology Service of Dr. Luis Díaz Soto Central Military Hospital from October 2012 to October 2015. Full-length and strata survival curves were constructed, according to Child-Pugh-Turcotte classification, as well as etiology of cirrhosis, severity of gastropathy, and occurrence of acute bleeding during the observation period. Kaplan-Meier method was used and the comparison of the curves by the logarithm of ranges. Results: A global survival probability of 94.1 was observed at two months and 61.7 percent at 30 months. The mean survival for patients with mild and severe gastropathy was 29.1 and 29.7 months, respectively; this was 28.3 months for cases with C virus cirrhosis and 30.1 months for those of other etiologies. There were predominant cases in Child A (41.2 percent) and B (47.1 percent), with an average survival of 33.5 and 30.1 months; For Child C (12 percent) was 12 months; (P = 0.05). 35.3 percent of the cases bled, with an average survival of 25.5 months lower than those who did not bleed (p = 0.35). Conclusions: The presence of hypertensive portal gastropathy is associated with a greater probability of dying when there is a greater deterioration of the liver function or acute bleeding has occurred, as of 30 months after having been diagnosed(AU)

Caracterización clínica de pacientes cirróticos con gastropatía portal hipertensiva / Clinical Characterization of Cirrhotic Patients with Hypertensive Portal Gastropathy

Introducción: la gastropatía portal hipertensiva (GPH) constituye una complicación de la hipertensión portal de relevancia clínica, que aparece en pacientes con cirrosis hepática. Objetivo: caracterizar los diferentes tipos de gastropatía portal hipertensiva (GPH) según la presencia de manifestaciones de hipertensión portal, el estado de la función hepática y la ocurrencia de eventos de sangrado digestivo. Método: estudio descriptivo, de corte transversal que incluyó 46 pacientes con diagnóstico de cirrosis y evidencia endoscópica de gastropatía portal hipertensiva (GPH), atendidos entre 2011-2013 en el servicio de Gastroenterología del Hospital Militar Central Dr. Luis Díaz Soto. Se clasificaron según su forma leve o grave y se hicieron comparaciones entre ambos grupos. Resultados: el 78,2 por ciento de los pacientes presentó una gastropatía leve y la localización más frecuente resultó ser el fondo gástrico (78,2 por ciento). Todos los casos con la forma grave fueron hombres (p=0,008). Entre los que tuvieron una gastropatía grave, predominaron los que tuvieron un mayor diámetro de la porta (18,3 mm vs 13,5 mm, p=0,001) y del bazo (137,4mm vs130,03 mm, (p=0,0002), así como los que también tenían várices esofágicas (100 por ciento, p=0,007), por lo que el sangrado agudo también fue más frecuente entre ellos (60 por ciento, p=0,01). No se manifestaron diferencias con los parámetros hematológicos de hipertensión portal ni con los grados de función hepática, aun cuando el 63 por ciento de los pacientes se encontró en los grupos B y C de la clasificación de Child-Pughs-Turcotte. Conclusiones: la forma grave de la gastropatía portal hipertensiva (GPH) es la menos frecuente, pero se acompaña de alteraciones clínicas, humorales e imaginológicas relevantes, que requieren una atención personalizada para este tipo de enfermos(AU)

Introduction: Hypertensive portal gastropathy (HPG) is a complication of portal hypertension of clinical relevance, which appears in patients with liver cirrhosis. Objective: Characterize the different types of hypertensive portal gastropathy (HPG) according to the presence of manifestations of portal hypertension, the state of liver function and the occurrence of digestive bleeding events. Method: A descriptive, cross-sectional study was conducted in 46 patients diagnosed with cirrhosis and endoscopic evidence of portal hypertensive gastropathy (HPG), treated from 2011 to 2013 in the Gastroenterology Department of Dr. Luis Díaz Soto Central Military Hospital. They were classified according to mild or severe form and comparisons were made between both groups. Results: 78.2 percent of the patients presented mild gastropathy and the most frequent location was the gastric fundus (78.2 percent). All cases with the severe form were men (p = 0.008). Among those who had severe gastropathy, those with larger diameter of the portal (18.3 mm vs 13.5 mm, p = 0.001) and spleen (137.4 mm vs 130.03 mm, p = 0, 0002), as well as those who also had esophageal varices (100 percent, p = 0.007), so that acute bleeding was also more frequent among them (60 percent, p = 0.01). Hematologic factors of portal hypertension or with degrees of liver function, although 63 percent of patients were found in groups B and C of the Child-Pughs-Turcotte classification. Conclusions: The severe form of hypertensive portal gastropathy (GPH) is the least frequent, but it is accompanied by relevant clinical, humoral and imaging alterations, which require a personalized attention for this type of patients(AU)

Portal biliopathy treated with endoscopic biliary stenting

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

A case of portal hypertension by presumed as plexiform neurofibroma at the hepatic hilum

Neurofibromas can occur anywhere in the body, but they usually involve the head, neck, pelvis, and extremities. Abdominal visceral involvement is rare, and intrahepatic involvement is even less common. We describe a patient who suffered from plexiform neurofibromatosis with liver involvement. A 49-year-old man, who had previously been diagnosed with neurofibromatosis, underwent esophagogastroduodenoscopy and abdominal ultrasonography for screening purposes. Esophagogastroduodenoscopy showed grade 2 esophageal varices and abdominal ultrasonography showed conglomerated nodules with echogenic appearances in the perihepatic space. Magnetic resonance imaging showed presumed plexiform neurofibroma involving the lesser sac and hepatic hilum and encasing the common hepatic artery celiac trunk and superior mesenteric artery left portal triad. We report an unusual case of portal hypertension attributed to the compressive narrowing of the portal vein by presumed as plexiform neurofibroma at the lesser sac and hepatic hilum.

Hipertensão portopulmonar / Portopulmonary hypertension

A hipertensão portopulmonar (POPH) é definida como hipertensão pulmonar na presença de hipertensão portal. Acomete cerca de 5% dos pacientes com hipertensão portal, cirróticos ou não, em avaliação para transplante hepático. A fisiopatologia dessa doença não está completamente elucidada. O hiperfluxo pulmonar e a formação conexões porto-sistêmicas, que permitem o acesso de bactérias e de substâncias inflamatórias aos pulmões, são mecanismos prováveis. Variações genéticas possivelmente estão implicadas na variabilidade do comprometimento vascular pulmonar dos pacientes com hipertensão portal. O ecocardiograma tem papel importante como rastreamento, sendo o diagnóstico definitivo realizado através do cateterismo cardíaco direito. A POPH pode ser considerada contraindicação ao transplante hepático, nos casos moderados e graves, no entanto, o tratamento com vasodilatadores pulmonares, indicado para esses pacientes, pode ser capaz de melhorar o perfil hemodinâmico e permitir que mais pacientes atinjam os critérios de segurança para o transplante. Com as novas abordagens terapêuticas, observou-se melhora no prognóstico desses pacientes nos últimos anos...

Portopulmonary hypertension (POPH) is defined as pulmonary hypertension in the presence of portal hypertension. It affects about 5% of patients with portal hypertension, cirrhotic or not, under evaluation for liver transplantation. The pathophysiology of this disease is not completely understood, but hyperdynamic state in pulmonary circulation and formation of porto-systemic connections, which allow access of intestinal bacteria and inflammatory substances to the lungs, are probably involved. Genetic variations possibly play a role in the variability of the pulmonary vascular impairment of patients with portal hypertension. Echocardiography is important for screening, but the definitive diagnosis is made by right heart catheterization. The POPH can be considered a contraindication for liver transplantation in moderate to severe cases, however, treatment with pulmonary vasodilators, indicated for these patients, may be able to improve hemodynamic profile and allow more patients meet the criteria for transplantation. With new therapeutic approaches, there was an improvement in the prognosis of these patients in recent years...

The Accuracy of Ultrasonography for the Evaluation of Portal Hypertension in Patients with Cirrhosis: A Systematic Review

OBJECTIVE: Studies have presented conflicting results regarding the accuracy of ultrasonography (US) for diagnosing portal hypertension (PH). We sought to identify evidence in the literature regarding the accuracy of US for assessing PH in patients with liver cirrhosis. MATERIALS AND METHODS: We conducted a systematic review by searching databases, including MEDLINE, EMBASE, and the Cochrane Library, for relevant studies. RESULTS: A total of 14 studies met our inclusion criteria. The US indices were obtained in the portal vein (n = 9), hepatic artery (n = 6), hepatic vein (HV) (n = 4) and other vessels. Using hepatic venous pressure gradient (HVPG) as the reference, the sensitivity (Se) and specificity (Sp) of the portal venous indices were 69-88% and 67-75%, respectively. The correlation coefficients between HVPG and the portal venous indices were approximately 0.296-0.8. No studies assess the Se and Sp of the hepatic arterial indices. The correlation between HVPG and the hepatic arterial indices ranged from 0.01 to 0.83. The Se and Sp of the hepatic venous indices were 75.9-77.8% and 81.8-100%, respectively. In particular, the Se and Sp of HV arrival time for clinically significant PH were 92.7% and 86.7%, respectively. A statistically significant correlation between HVPG and the hepatic venous indices was observed (0.545-0.649). CONCLUSION: Some US indices, such as HV, exhibited an increased accuracy for diagnosing PH. These indices may be useful in clinical practice for the detection of significant PH.

Hipertensión portal en el embarazo: presentación de un caso / Portal hypertension in pregnancy: report of a case

La coexistencia de embarazo y enfermedad hepática representa una situación clínica compleja. Durante el embarazo se desarrolla un estado hipervolémico debido a un flujo esplácnico incrementado, que contribuye a mayor presión portal transmitida a las venas colaterales que incrementan el riesgo de hemorragia varicosa en este grupo de pacientes. Se reporta el caso de una paciente de 39 años en el sexto embarazo y sin ningún antecedente médico previo que presenta hipertensión portal pre-sinusoidal y que gracias al manejo multidisciplinario adecuado, tuvo un parto sin complicaciones. Se revisa la literatura pertinente al caso.

The coexistence of pregnancy and liver disease represents a complex clinical situation. Pregnancy develops hypervolemic state due to increased splachnic blood flow, which contributes to increased portal pressure transmitted to collateral veins that increase the risk of variceal bleeding in these patients. We report the case of a 39 years old patient in the sixth pregnancy and without any previous medical history that presented pre-sinusoidal portal hypertension, and thanks to appropriate multidisciplinary management had an uncomplicated delivery. We review the literature relevant to the case.

Do cirrhotic patients with a high MELD score benefit from TIPS?

No abstract available.

Clinical outcomes of transjugular intrahepatic portosystemic shunt for portal hypertension: Korean multicenter real-practice data

BACKGROUND/AIMS: This retrospective study assessed the clinical outcome of a transjugular intrahepatic portosystemic shunt (TIPS) procedure for managing portal hypertension in Koreans with liver cirrhosis. METHODS: Between January 2003 and July 2013, 230 patients received a TIPS in 13 university-based hospitals. RESULTS: Of the 229 (99.6%) patients who successfully underwent TIPS placement, 142 received a TIPS for variceal bleeding, 84 for refractory ascites, and 3 for other indications. The follow-up period was 24.9+/-30.2 months (mean+/-SD), 74.7% of the stents were covered, and the primary patency rate at the 1-year follow-up was 78.7%. Hemorrhage occurred in 30 (21.1%) patients during follow-up; of these, 28 (93.3%) cases of rebleeding were associated with stent dysfunction. Fifty-four (23.6%) patients developed new hepatic encephalopathy, and most of these patients were successfully managed conservatively. The cumulative survival rates at 1, 6, 12, and 24 months were 87.5%, 75.0%, 66.8%, and 57.5%, respectively. A high Model for End-Stage Liver Disease (MELD) score was significantly associated with the risk of death within the first month after receiving a TIPS (P=0.018). Old age (P<0.001), indication for a TIPS (ascites vs. bleeding, P=0.005), low serum albumin (P<0.001), and high MELD score (P=0.006) were associated with overall mortality. CONCLUSIONS: A high MELD score was found to be significantly associated with early and overall mortality rate in TIPS patients. Determining the appropriate indication is warranted to improve survival in these patients.

A Case of Gastric Adenocarcinoma Presenting as Portal Hypertension / 대한소화기학회지

Portal vein thrombus has been detected in patients with liver cirrhosis, pancreatitis, ulcerative colitis, septicemia, myeloproliferative disorder, and neoplasm. The formation of portal tumor thrombus by hepatocellular carcinoma is well recognized, because of its high incidence, and subsequent development of portal hypertension such as rupture of varices, ascites and liver failure indicates the poor prognosis. In gastric cancer, portal hypertension as an initial presentation is extremely rare. Herein we report a case presenting as portal hypertension caused by tumor thrombus without invasion of liver parenchyma. It is presumed to be intraluminal tumor thrombus originating from primary foci of gastric adenocarcinoma. Tumor thrombus in the portal vein is demonstrated on the PET-CT.

Portal Bilopathy / 대한소화기학회지

No abstract available.