RESUMEN
BACKGROUND: Hepatopulmonary syndrome (HPS) is characterized by the triad of abnormal arterial oxygenation caused by intrapulmonary vascular dilatations (IPVD) in the setting of advanced liver disease or portal hypertension, impacting the patient's quality of life and survival. There are still many gaps in the literature on this topic, especially in pediatrics, with practices frequently based on extrapolation of data obtained from adults. OBJECTIVE: Provide a synthesis of the current knowledge about HPS in children. METHODS: The research was carried out through narrative review. The databases used for the search include Medline, Embase, Elsevier, Lilacs and Scielo. The keywords used were "hepatopulmonary syndrome" AND child, children, infant, preschool, pediatric. RESULTS: In cirrhotic children, the prevalence of HPS can reach up to 42.5%, and it is even more common in those whose underlying condition is biliary atresia, reaching up to 63%. Screening with pulse oximetry (O2 saturation <96%), unlike in adults, has low sensitivity in the pediatric age group. Management involves supportive care with oxygen therapy; liver transplantation is the only definitive treatment to reverse the condition and HPS is considered an exceptional criterion for waitlist. The waitlist mortality is similar among children listed by HPS as a special criterion when compared to those listed for other reasons. The reported rates of complete resolution of hypo-xemia after liver transplantation are close to 100% in children. The post-liver transplantation survival is similar or slightly lower in children with HPS when compared to those without HPS. Contrary to findings from adults, no differences were found in post- liver transplantation mortality between children of different hypoxemia ranges, although longer mechanical ventilation time and hospital stay were observed in children with PaO2 <50 mmHg. CONCLUSION: HPS is not an uncommon complication of cirrhosis in children and adolescents, particularly when biliary atresia is the underlying condition. There are still many gaps to be filled regarding the condition, and this article demonstrates that not all data obtained in studies with adults reflects the disease's behavior in pediatrics, especially concerning prognosis.
Asunto(s)
Síndrome Hepatopulmonar , Hipertensión Portal , Humanos , Síndrome Hepatopulmonar/etiología , Síndrome Hepatopulmonar/complicaciones , Hipertensión Portal/complicaciones , Hipertensión Portal/etiología , Niño , Trasplante de Hígado , Cirrosis Hepática/complicacionesRESUMEN
Liver cirrhosis has long been considered a point of no return, with limited hope for recovery. However, recent advancements, particularly the Baveno VII criteria and the utilization of transjugular intrahepatic portosystemic shunt (TIPS), have illuminated the concept of hepatic recompensation. In this editorial we comment on the article by Gao et al published in the recent issue. This editorial provides a comprehensive overview of the evolution of understanding cirrhosis, the criteria for recompensation, and the efficacy of TIPS in achieving recompensation. We discuss key findings from recent studies, including the promising outcomes observed in patients who achieved recompensation post-TIPS insertion. While further research is needed to validate these findings and elucidate the mech-anisms underlying recompensation, the insights presented here offer renewed hope for patients with decompensated cirrhosis and highlight the potential of TIPS as a therapeutic option in their management.
Asunto(s)
Cirrosis Hepática , Derivación Portosistémica Intrahepática Transyugular , Derivación Portosistémica Intrahepática Transyugular/métodos , Derivación Portosistémica Intrahepática Transyugular/efectos adversos , Humanos , Cirrosis Hepática/cirugía , Cirrosis Hepática/complicaciones , Resultado del Tratamiento , Hipertensión Portal/cirugía , Hipertensión Portal/etiología , Hipertensión Portal/diagnóstico , Encefalopatía Hepática/etiología , Encefalopatía Hepática/cirugíaRESUMEN
BACKGROUND: Portal hypertensive enteropathy (PHE) is a small-bowel lesion observed in patients with portal hypertension. The clinical significance of endoscopic findings in PHE remains unclear. We aimed to clarify the clinical significance and predictive factors of capsule endoscopic findings in patients with PHE based on long-term outcomes. METHODS: This retrospective study enrolled 55 patients with PHE (33 males and 22 females; median age, 64 years; range, 23-87) followed for > 3 years using capsule endoscopy (CE) between February 2009 and May 2023. We evaluated the clinical factors affecting PHE exacerbations and the effects of PHE exacerbations on gastrointestinal bleeding by comparing exacerbated and unchanged PHE groups. RESULTS: Overall, 3 (5%) patients showed improvement, 33 (60%) remained unchanged, and 19 (35%) showed exacerbation on follow-up CE. In the exacerbated group, the rates of worsened fibrosis-4 index, exacerbated esophageal varices, and exacerbated portal hypertensive gastropathy were significantly higher than those in the unchanged group (21%, 32%, and 42% vs. 3%, 6%, and 12%, respectively; P < 0.05), and the rate of splenectomy was significantly lower in the exacerbated group than in the unchanged group (5% vs. 39%, respectively; P < 0.01). In multivariate analysis, exacerbation of esophageal varices and absence of splenectomy were significantly associated with PHE exacerbation. The rate of gastrointestinal bleeding after follow-up CE was significantly high in the exacerbated group (log-rank, P = 0.037). CONCLUSIONS: Exacerbation of esophageal varices and splenectomy were significantly associated with exacerbation of PHE. Exacerbated PHE requires specific attention to prevent gastrointestinal bleeding.
Asunto(s)
Endoscopía Capsular , Progresión de la Enfermedad , Várices Esofágicas y Gástricas , Hemorragia Gastrointestinal , Hipertensión Portal , Humanos , Hipertensión Portal/etiología , Hipertensión Portal/complicaciones , Masculino , Femenino , Persona de Mediana Edad , Anciano , Estudios Retrospectivos , Adulto , Hemorragia Gastrointestinal/etiología , Anciano de 80 o más Años , Várices Esofágicas y Gástricas/etiología , Adulto Joven , Esplenectomía , Enfermedades Intestinales/etiología , Enfermedades Intestinales/complicaciones , Intestino Delgado/patología , Intestino Delgado/diagnóstico por imagenRESUMEN
Portal cavernoma thrombosis is a complication of portal cavernoma. We describe the case of a 74-year-old patient who presented to the emergency department with abdominal pain. The computed tomography scan showed a mass from the head of the pancreas to the hepatic hilum not enhanced after injection of iodinated contrast. There was no dilatation of the bile ducts. Abdominal magnetic resonance ruled out a tumour and confirmed a portal cavernoma thrombosis. In 50 % of cases the etiology of the portal cavernoma is unknown. It is often asymptomatic. It may be discovered in case of complications of portal hypertension. In rare cases the portal cavernoma can compress the bile ducts. To our knowledge, portal cavernoma thrombosis has only been described in one article. It is important to search for a thrombophilic disorder when such a complication is found. We share this case report in order to raise awareness in the medical community about this rare complication.
La thrombose de cavernome portal est une complication du cavernome porte. Nous décrivons le cas d'un patient de 74 ans qui s'est présenté aux urgences pour des douleurs abdominales. La tomodensitométrie montrait un syndrome de masse de la tête du pancréas jusqu'au hile hépatique non rehaussé après injection de produit de contraste iodé. Il n'y avait pas de dilatation des voies biliaires. Une imagerie par résonance magnétique abdominale a permis d'infirmer l'hypothèse d'une masse tumorale et d'affirmer une thrombose du cavernome porte. Dans 50 % des cas, l'étiologie du cavernome portal est inconnue. Il est souvent asymptomatique. Il peut être découvert en cas de complications à la suite d'une hypertension portale. Dans de rares cas, le cavernome portal peut comprimer les voies biliaires. À notre connaissance, la thrombose de cavernome portal n'a été décrite que dans un seul article. Il est important de rechercher un désordre thrombophilique quand une telle complication est retrouvée. Nous partageons ce cas clinique afin de sensibiliser la communauté médicale à cette rare complication.
Asunto(s)
Vena Porta , Humanos , Anciano , Vena Porta/diagnóstico por imagen , Masculino , Hemangioma Cavernoso/complicaciones , Hemangioma Cavernoso/diagnóstico , Trombosis de la Vena/etiología , Trombosis de la Vena/diagnóstico , Trombosis de la Vena/diagnóstico por imagen , Hipertensión Portal/etiología , Hipertensión Portal/complicaciones , Trombosis/etiología , Trombosis/diagnóstico por imagen , Trombosis/diagnóstico , Imagen por Resonancia Magnética , Tomografía Computarizada por Rayos XAsunto(s)
Hipertensión Portal , Humanos , Embarazo , Femenino , Hipertensión Portal/diagnóstico , Hipertensión Portal/complicaciones , Hipertensión Portal/etiología , Adulto , Policitemia/diagnóstico , Policitemia/etiología , Policitemia/complicaciones , Complicaciones Hematológicas del Embarazo/diagnóstico , Complicaciones Cardiovasculares del Embarazo/diagnóstico , Trombocitemia Esencial/complicaciones , Trombocitemia Esencial/diagnósticoRESUMEN
Bleeding events are feared complications in patients with advanced liver diseases and are associated with morbidity and mortality. In this context, gastrointestinal bleeding, particularly upper gastrointestinal bleeding, has a special clinical importance. In addition to endoscopic measures for hemostasis, reducing portal pressure in particular is a key component of treatment. Although the standard coagulation parameters are often altered in patients with liver diseases, optimizing coagulation plays a secondary role. Typically, a bundle of measures are employed in patients with portal hypertensive bleeding, which nowadays in most cases can halt the bleeding and stabilize the situation. The measures include endoscopy, antibiotic treatment, vasopressor treatment and, if necessary, shunt placement (transjugular intrahepatic portosystemic shunt).
Asunto(s)
Hemorragia Gastrointestinal , Hipertensión Portal , Derivación Portosistémica Intrahepática Transyugular , Humanos , Hemorragia Gastrointestinal/terapia , Hemorragia Gastrointestinal/etiología , Hemorragia Gastrointestinal/diagnóstico , Hipertensión Portal/diagnóstico , Hipertensión Portal/terapia , Hipertensión Portal/etiología , Terapia Combinada , Hepatopatías/diagnóstico , Hepatopatías/terapia , Vasoconstrictores/uso terapéutico , Antibacterianos/uso terapéutico , Cirrosis Hepática/complicaciones , Cirrosis Hepática/diagnóstico , Várices Esofágicas y Gástricas/terapia , Várices Esofágicas y Gástricas/diagnósticoRESUMEN
BACKGROUND: Gut microbiota (GM) affects the progression and response to treatment in liver diseases. The GM composition is diverse and associated with different etiologies of liver diseases. Notably, alterations in GM alterations are observed in patients with portal hypertension (PH) secondary to cirrhosis, with hepatitis B virus (HBV) infection being a major cause of cirrhosis in China. Thus, understanding the role of GM alterations in patients with HBV infection-related PH is essential. AIM: To evaluate GM alterations in patients with HBV-related PH after transjugular intrahepatic portosystemic shunt (TIPS) placement. METHODS: This was a prospective, observational clinical study. There were 30 patients (with a 100% technical success rate) recruited in the present study. Patients with esophagogastric variceal bleeding due to HBV infection-associated PH who underwent TIPS were enrolled. Stool samples were obtained before and one month after TIPS treatment, and GM was analyzed using 16S ribosomal RNA amplicon sequencing. RESULTS: One month after TIPS placement, 8 patients developed hepatic encephalopathy (HE) and were assigned to the HE group; the other 22 patients were assigned to the non-HE group. There was no substantial disparity in the abundance of GM at the phylum level between the two groups, regardless of TIPS treatment (all, P > 0.05). However, following TIPS placement, the following results were observed: (1) The abundance of Haemophilus and Eggerthella increased, whereas that of Anaerostipes, Dialister, Butyricicoccus, and Oscillospira declined in the HE group; (2) The richness of Eggerthella, Streptococcus, and Bilophila increased, whereas that of Roseburia and Ruminococcus decreased in the non-HE group; and (3) Members from the pathogenic genus Morganella appeared in the HE group but not in the non-HE group. CONCLUSION: Intestinal microbiota-related synergism may predict the risk of HE following TIPS placement in patients with HBV-related PH. Prophylactic microbiome therapies may be useful for preventing and treating HE after TIPS placement.
Asunto(s)
Microbioma Gastrointestinal , Encefalopatía Hepática , Virus de la Hepatitis B , Hipertensión Portal , Derivación Portosistémica Intrahepática Transyugular , Humanos , Derivación Portosistémica Intrahepática Transyugular/efectos adversos , Masculino , Femenino , Persona de Mediana Edad , Hipertensión Portal/etiología , Hipertensión Portal/diagnóstico , Hipertensión Portal/microbiología , Estudios Prospectivos , Encefalopatía Hepática/etiología , Adulto , Virus de la Hepatitis B/aislamiento & purificación , Heces/microbiología , Cirrosis Hepática/virología , Cirrosis Hepática/microbiología , Cirrosis Hepática/cirugía , China/epidemiología , Resultado del Tratamiento , Hepatitis B/complicaciones , Hepatitis B/diagnóstico , Hepatitis B/virología , Várices Esofágicas y Gástricas/etiología , Várices Esofágicas y Gástricas/diagnóstico , Várices Esofágicas y Gástricas/microbiología , Várices Esofágicas y Gástricas/virología , Hemorragia Gastrointestinal/etiología , ARN Ribosómico 16S/genética , Disbiosis/etiología , Anciano , Bacterias/aislamiento & purificación , Bacterias/genéticaRESUMEN
BACKGROUND AND AIMS: Transjugular intrahepatic portosystemic shunt (TIPS) is an established procedure for the treatment of several complications of portal hypertension (PH), including non-neoplastic portal vein thrombosis (PVT). Selection criteria for TIPS in PVT are not yet well established. Despite anecdotal, cases of thromboembolic events from paradoxical embolism due to the presence of patent foramen ovale (PFO) after TIPS placement have been reported in the literature. Therefore, we aimed at describing our experience in patients with non-neoplastic splanchnic vein thrombosis (SVT) who underwent TIPS following PFO screening. METHODS: We conducted a single-centre retrospective study, including consecutive patients who underwent TIPS for the complications of cirrhotic and non-cirrhotic portal hypertension (NCPH) and having SVT. RESULTS: Of 100 TIPS placed in patients with SVT, 85 patients were screened for PFO by bubble-contrast transthoracic echocardiography (TTE) with PFO being detected in 22 (26%) cases. PFO was more frequently detected in patients with non-cirrhotic portal hypertension (NCPH) (23% in the PFO group vs. 6% in those without PFO, p = .04) and cavernomatosis (46% in the PFO group vs. 19% in those without PFO, p = .008). Percutaneous closure was effectively performed in 11 (50%) after multidisciplinary evaluation of anatomical and clinical features. No major complications were observed following closure. CONCLUSIONS: PFO screening and treatment may be considered feasible for patients with SVT who undergo TIPS placement.
Asunto(s)
Foramen Oval Permeable , Hipertensión Portal , Vena Porta , Derivación Portosistémica Intrahepática Transyugular , Trombosis de la Vena , Humanos , Derivación Portosistémica Intrahepática Transyugular/efectos adversos , Foramen Oval Permeable/complicaciones , Foramen Oval Permeable/cirugía , Foramen Oval Permeable/diagnóstico por imagen , Estudios Retrospectivos , Hipertensión Portal/cirugía , Hipertensión Portal/etiología , Hipertensión Portal/complicaciones , Femenino , Masculino , Persona de Mediana Edad , Trombosis de la Vena/etiología , Trombosis de la Vena/diagnóstico por imagen , Trombosis de la Vena/cirugía , Vena Porta/cirugía , Adulto , Prevalencia , Anciano , Ecocardiografía , Circulación Esplácnica , Cirrosis Hepática/complicaciones , Cirrosis Hepática/cirugía , Resultado del TratamientoRESUMEN
BACKGROUND: Portal hypertension is a common diagnosis in Sub-Saharan African countries, with the majority of patients presenting late. This study aimed to understand Clinical characteristics, aetiology, the treatment offered in our setting, and factors associated with portal hypertension at a tertiary-level hospital, in Tanzania. METHODOLOGY: A prospective cross-sectional observational single hospital-based study was conducted at MNH, from May 2021 to April 2022. A minimum of 152 subjects were required with an error of less than 5% and a study power of 80% at a 95% confidence interval. A structured questionnaire was used to collect data. Ethical clearance was obtained from the MUHAS/MNH IRB. RESULTS: A total of 154 eligible participants consented and participated in this study. The mean age of participants was 42 ± 15.8 years (range 2-87). Most of the study participants were males 64.9% with a male-to-female (M: F) ratio of 1.8:1. Vomiting blood was the common symptom among the study participants 51.3%. Schistosomiasis 53.9% and viral infection 26.6% were the common etiologies followed by alcohol abuse 7.8%. Most were medically treated at 89.61% followed by radiological treatment at 8.44% while only 1.95% of patients received surgical treatment. There was a significant association between the grade of oesophagal varices and bleeding consequences (p-value < 0.01). CONCLUSION: The majority of patients were medically treated while patients who require surgical care are unable to assess it. We recommend the establishment of a transplant services program to counteract the unmet need and more retrospective research toward policy establishment.
Asunto(s)
Hipertensión Portal , Humanos , Masculino , Femenino , Hipertensión Portal/diagnóstico , Hipertensión Portal/terapia , Hipertensión Portal/fisiopatología , Hipertensión Portal/epidemiología , Hipertensión Portal/etiología , Tanzanía/epidemiología , Adulto , Estudios Transversales , Persona de Mediana Edad , Estudios Prospectivos , Adulto Joven , Anciano , Adolescente , Anciano de 80 o más Años , Niño , Preescolar , Factores de Riesgo , Várices Esofágicas y Gástricas/terapia , Várices Esofágicas y Gástricas/diagnóstico , Várices Esofágicas y Gástricas/etiología , Resultado del Tratamiento , Centros de Atención Terciaria , Hemorragia Gastrointestinal/terapia , Hemorragia Gastrointestinal/etiología , Hemorragia Gastrointestinal/diagnóstico , Hemorragia Gastrointestinal/epidemiologíaRESUMEN
Portal vein thrombosis (PVT) refers to the development of a non-malignant obstruction of the portal vein, its branches, its radicles, or a combination. This Review first provides a comprehensive overview of all aspects of PVT, namely the specifics of the portal venous system, the risk factors for PVT, the pathophysiology of portal hypertension in PVT, the interest in non-invasive tests, as well as therapeutic approaches including the effect of treating risk factors for PVT or cause of cirrhosis, anticoagulation, portal vein recanalisation by interventional radiology, and prevention and management of variceal bleeding in patients with PVT. Specific issues are also addressed including portal cholangiopathy, mesenteric ischaemia and intestinal necrosis, quality of life, fertility, contraception and pregnancy, and PVT in children. This Review will then present endpoints for future clinical studies in PVT, both in patients with and without cirrhosis, agreed by a large panel of experts through a Delphi consensus process. These endpoints include classification of portal vein thrombus extension, classification of PVT evolution, timing of assessment of PVT, and global endpoints for studies on PVT including clinical outcomes. These endpoints will help homogenise studies on PVT and thus facilitate reporting, comparison between studies, and validation of future studies and trials on PVT.
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Vena Porta , Trombosis de la Vena , Humanos , Trombosis de la Vena/diagnóstico , Trombosis de la Vena/terapia , Hipertensión Portal/diagnóstico , Hipertensión Portal/terapia , Hipertensión Portal/etiología , Hipertensión Portal/complicaciones , Factores de Riesgo , Anticoagulantes/uso terapéutico , Cirrosis Hepática/complicaciones , Cirrosis Hepática/diagnóstico , Hemorragia Gastrointestinal/etiología , Hemorragia Gastrointestinal/terapia , Hemorragia Gastrointestinal/diagnóstico , Embarazo , Femenino , Calidad de VidaRESUMEN
The Baveno VII criteria redefine the management of decompensated liver cirrhosis, introducing the concept of hepatic recompensation marking a significant departure from the conventional view of irreversible decline. Central to this concept is addressing the underlying cause of cirrhosis through tailored therapies, including antivirals and lifestyle modifications. Studies on alcohol, hepatitis C virus, and hepatitis B virus-related cirrhosis demonstrate the efficacy of these interventions in improving liver function and patient outcomes. Transjugular intrahepatic portosystemic shunt (TIPS) emerges as a promising intervention, effectively resolving complications of portal hypertension and facilitating recompensation. However, optimal timing and patient selection for TIPS remain unresolved. Despite challenges, TIPS offers renewed hope for hepatic recompensation, marking a significant advancement in cirrhosis management. Further research is needed to refine its implementation and maximize its benefits. In conclusion, TIPS stands as a promising avenue for improving hepatic function and patient outcomes in decompensated liver cirrhosis within the framework of the Baveno VII criteria.
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Hipertensión Portal , Cirrosis Hepática , Selección de Paciente , Derivación Portosistémica Intrahepática Transyugular , Humanos , Cirrosis Hepática/virología , Cirrosis Hepática/diagnóstico , Cirrosis Hepática/complicaciones , Cirrosis Hepática/terapia , Derivación Portosistémica Intrahepática Transyugular/métodos , Hipertensión Portal/etiología , Hipertensión Portal/diagnóstico , Hipertensión Portal/terapia , Resultado del Tratamiento , Antivirales/uso terapéutico , Hígado/cirugíaAsunto(s)
Várices Esofágicas y Gástricas , Hemorragia Gastrointestinal , Derivación Portosistémica Intrahepática Transyugular , Humanos , Derivación Portosistémica Intrahepática Transyugular/efectos adversos , Hemorragia Gastrointestinal/etiología , Várices Esofágicas y Gástricas/etiología , Várices Esofágicas y Gástricas/cirugía , Hipertensión Portal/complicaciones , Hipertensión Portal/etiología , Cirrosis Hepática/complicacionesRESUMEN
INTRODUCTION: Cystic fibrosis (CF)-associated liver disease can significantly affect the quality of life and survival of people with CF. The hepatobiliary manifestations in CF are various, with focal/multilobular biliary cirrhosis more common in children and porto-sinusoidal vascular disease (PSVD) in young adults. Portal hypertensive complications, particularly bleeding from esophagogastric varices and hypersplenism are common, while liver failure is rarer and mainly linked to biliary disease. AREAS COVERED: This review explores current therapeutic options for CF-associated liver disease, presenting ongoing studies and new insights into parthenogenesis for potential future therapies. EXPERT OPINION: Monitoring for signs of portal hypertension is essential. Limited evidence supports ursodeoxycholic acid (UDCA) efficacy in halting CF liver disease progression. The effect of cystic fibrosis transmembrane conductance regulator (CFTR) modulators on liver outcomes lacks definitive data, since patients with CF-related liver disease were excluded from trials due to potential hepatotoxicity. A proposed approach involves using UDCA and modulators in early stages, along with anti-inflammatory agents, with further therapeutic strategies awaiting randomized trials. Prevention of portal hypertensive bleeding includes endoscopic sclerotherapy or ligation of esophageal varices. Nonselective beta-blockers may also prevent bleeding and could be cautiously implemented. Other non-etiological treatments require investigation.
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Fibrosis Quística , Hipertensión Portal , Humanos , Hipertensión Portal/fisiopatología , Hipertensión Portal/tratamiento farmacológico , Hipertensión Portal/etiología , Fibrosis Quística/complicaciones , Fibrosis Quística/fisiopatología , Fibrosis Quística/tratamiento farmacológico , Ácido Ursodesoxicólico/uso terapéutico , Hepatopatías/fisiopatología , Hepatopatías/terapia , Hepatopatías/tratamiento farmacológico , Várices Esofágicas y Gástricas/etiología , Várices Esofágicas y Gástricas/terapia , Várices Esofágicas y Gástricas/fisiopatología , Regulador de Conductancia de Transmembrana de Fibrosis Quística/genética , Regulador de Conductancia de Transmembrana de Fibrosis Quística/metabolismo , Calidad de Vida , Progresión de la EnfermedadRESUMEN
PURPOSE: This study evaluated portal hypertension (PHT) and its predictors among native liver survivors (NLS) of biliary atresia (BA) after Kasai portoenterostomy (KPE). METHODS: This was a multicenter study using prospectively collected data. The subjects were patients who remained transplant-free for 5 years after KPE. Their status of PHT was evaluated and variables that predicted PHT were determined by regression analysis and receiver operating characteristic (ROC) curve. RESULTS: Six centers from East Asia participated in this study and 320 subjects with KPE between 1980 to 2018 were analyzed. The mean follow-up period was 10.6 ± 6.2 years. At the 5th year after KPE, PHT was found in 37.8% of the subjects (n = 121). Patients with KPE done before day 41 of life had the lowest percentage of PHT compared to operation at older age. At 12 months after KPE, PHT + ve subjects had a higher bilirubin level (27.1 ± 11.7 vs 12.3 ± 7.9 µmol/L, p = 0.000) and persistent jaundice conferred a higher risk for PHT (OR = 12.9 [9.2-15.4], p = 0.000). ROC analysis demonstrated that a bilirubin level above 38 µmol/L at 12 months after KPE predicted PHT development (sensitivity: 78%, specificity: 60%, AUROC: 0.75). CONCLUSIONS: In BA, early KPE protects against the development of PHT among NLSs. Patients with persistent cholestasis at one year after KPE are at a higher risk of this complication. They should receive a more vigilant follow-up. LEVEL OF EVIDENCE: Level III.
Asunto(s)
Atresia Biliar , Colestasis , Hipertensión Portal , Portoenterostomía Hepática , Humanos , Atresia Biliar/cirugía , Atresia Biliar/complicaciones , Portoenterostomía Hepática/métodos , Masculino , Femenino , Hipertensión Portal/etiología , Lactante , Colestasis/etiología , Complicaciones Posoperatorias/epidemiología , Estudios Prospectivos , Estudios de Seguimiento , Sobrevivientes/estadística & datos numéricos , Recién Nacido , PreescolarRESUMEN
Portal hypertension (PHT) is defined as an increase in pressure at the level of the portal vein above 5 mmHg, the most common cause being liver cirrhosis. Among the presinusoidal intrahepatic causes of PHT with portal venular involvement, what was traditionally known as idiopathic non-cirrhotic portal hypertension (NCIH) is described, with the requirements of excluding those patients who did not present PHT, as well as those with the presence of liver cirrhosis and thrombosis. portal venous vein (PVT). Currently, the diagnostic criteria for this entity have been reconsidered, and its name, being known as porto-sinusoidal vascular disease (PSVD), also does not exclude patients with PHT or the presence of underlying liver disease. Liver biopsy continues to be the gold standard for diagnosis. The clinical manifestations are derived from PHT and the management is similar to the complications that occur in patients with liver cirrhosis. The case of a male patient is presented who presents with symptoms of digestive bleeding, with findings of esophageal varices in upper endoscopy in addition to a study of viral, autoimmune liver disease and negative deposits, with a conclusive liver biopsy of porto-sinusoidal vascular disease.
Asunto(s)
Hemorragia Gastrointestinal , Hipertensión Portal , Humanos , Masculino , Hemorragia Gastrointestinal/etiología , Hemorragia Gastrointestinal/diagnóstico , Hipertensión Portal/complicaciones , Hipertensión Portal/etiología , Hipertensión Portal/diagnóstico , Vena Porta , Persona de Mediana Edad , Enfermedad Veno-Oclusiva Hepática/diagnóstico , Enfermedad Veno-Oclusiva Hepática/complicacionesRESUMEN
OBJECTIVE: Several studies have investigated the correlation between the effects of different surgical treatments and laboratory exams for schistosomal portal hypertension, especially concerning portal system thrombosis. The etiopathogenic factors of this thrombosis are not fully understood. In this study, the correlation between surgical treatment for schistosomal portal hypertension and the occurrence of postoperative portal system thrombosis was investigated. METHODS: A total of 61 patients who underwent surgical treatment for schistosomal portal hypertension were distributed into four groups: Patients in Group 1 (n=12) underwent portal variceal disconnection associated with splenic artery ligation and spleen preservation. Patients in Group 2 (n=20) underwent portal variceal disconnection and total splenectomy. Patients in Group 3 (n=20) underwent portal variceal disconnection with subtotal splenectomy, preserving the upper splenic pole supplied by the splenogastric vessels. Patients in Group 4 (n=9) underwent portal variceal disconnection with total splenectomy and autogenous splenic implants on the greater omentum. Late postoperative portal vein thrombosis was diagnosed using Doppler ultrasound. RESULTS: Over the 10-year follow-up, portal vein thrombosis occurred in 26 operated patients (42.6%), with no significant difference observed among the four surgical groups (p=0.217). Most of the thrombi only partially occluded the portal system veins. All the patients presented with a thrombus inside the portal vein. There was no difference in hematological and biochemical tests between groups with or without portal vein thrombosis. CONCLUSIONS: Portal vein thrombosis is often observed in the late postoperative period, irrespective of the surgical treatment employed, and is not associated with patient characteristics or any hematological and biochemical tests.
Asunto(s)
Hipertensión Portal , Vena Porta , Esplenectomía , Trombosis de la Vena , Humanos , Hipertensión Portal/cirugía , Hipertensión Portal/etiología , Femenino , Masculino , Esplenectomía/efectos adversos , Esplenectomía/métodos , Vena Porta/cirugía , Vena Porta/diagnóstico por imagen , Adulto , Persona de Mediana Edad , Trombosis de la Vena/etiología , Trombosis de la Vena/cirugía , Trombosis de la Vena/diagnóstico por imagen , Resultado del Tratamiento , Adulto Joven , Esquistosomiasis/cirugía , Esquistosomiasis/complicaciones , Estudios de Seguimiento , Complicaciones Posoperatorias , Ligadura/métodos , Várices Esofágicas y Gástricas/cirugía , Várices Esofágicas y Gástricas/etiología , Adolescente , Ultrasonografía DopplerAsunto(s)
Ascitis , Hipertensión Portal , Miositis , Humanos , Ascitis/etiología , Ascitis/diagnóstico , Hipertensión Portal/diagnóstico , Hipertensión Portal/etiología , Hipertensión Portal/fisiopatología , Miositis/diagnóstico , Miositis/complicaciones , Miositis/inmunología , Resultado del Tratamiento , Femenino , Persona de Mediana Edad , Masculino , AdultoRESUMEN
INTRODUCTION: Patients with clinically significant portal hypertension (CSPH) are recommended to be treated with non-selective beta-blockers (ie, carvedilol) to prevent the first hepatic decompensation event by the renewing Baveno VII consensus. CSPH is defined by hepatic venous pressure gradient (HVPG)≥10 mm Hg; however, the HVPG measurement is not widely adopted due to its invasiveness. Liver stiffness (LS)≥25 kPa can be used as a surrogate of HVPG≥10 mm Hg to rule in CSPH with 90% of the positive predicting value in majority aetiologies of patients. A compelling argument is existing for using LS≥25 kPa to diagnose CSPH and then to initiate carvedilol in patients with compensated cirrhosis, and about 5%-6% of patients under this diagnosis criteria may not be benefited from carvedilol and are at risk of lower heart rate and mean arterial pressure. Randomised controlled trial on the use of carvedilol to prevent liver decompensation in CSPH diagnosed by LS remains to elucidate. Therefore, we aimed to investigate if compensated cirrhosis patients with LS≥25 kPa may benefit from carvedilol therapy. METHODS AND ANALYSIS: This study is a randomised, double-blind, placebo-controlled, multicentre trial. We will randomly assign 446 adult compensated cirrhosis patients with LS≥25 kPa and without any previous decompensated event and without high-risk gastro-oesophageal varices. Patients are randomly divided into two groups, with 223 subjects in group A and 223 subjects in group B. Group A is a carvedilol intervention group, while group B is a placebo group. All patients in both groups will receive aetiology therapies and are followed up at an interval of 6 months. The 3-year incidences of decompensated events of cirrhosis-related and liver-related death are the primary outcome. The secondary outcomes include development of each complication of portal hypertension individually (ascites, variceal bleeding or overt hepatic encephalopathy), development of spontaneous bacterial peritonitis and other bacterial infections, development of new varices, growth of small varices to large varices, delta changes in LS and spleen stiffness, change in hepatic dysfunction assessed by Child-Pugh and model for end-stage liver disease score, change in platelet count, development of hepatocellular carcinoma, development of portal vein thrombosis and adverse events with a 3-year follow-up. A predefined interim analysis will be performed to ensure that the calculation is reasonable. ETHICS AND DISSEMINATION: The study protocol has been approved by the ethics committees of the Sixth People's Hospital of Shenyang (2023-05-003-01) and independent ethics committee for clinical research of Zhongda Hospital, affiliated to Southeast University (2023ZDSYLL433-P01). The results from this trial will be submitted for publication in peer-reviewed journals and will be presented at international conferences. TRIAL REGISTRATION NUMBER: ChiCTR2300073864.
Asunto(s)
Carvedilol , Hipertensión Portal , Cirrosis Hepática , Carvedilol/uso terapéutico , Carvedilol/farmacología , Humanos , Hipertensión Portal/tratamiento farmacológico , Hipertensión Portal/etiología , Cirrosis Hepática/complicaciones , Método Doble Ciego , China/epidemiología , Estudios Multicéntricos como Asunto , Ensayos Clínicos Controlados Aleatorios como Asunto , Antagonistas Adrenérgicos beta/uso terapéutico , Femenino , Hígado/efectos de los fármacos , Hígado/fisiopatología , Presión Portal/efectos de los fármacos , Várices Esofágicas y Gástricas/etiología , Várices Esofágicas y Gástricas/prevención & control , Diagnóstico por Imagen de Elasticidad , Adulto , MasculinoRESUMEN
BACKGROUND: Portal hypertension affects hepatic, splanchnic and portosystemic collateral systems. Although alcohol is a well-known risk factor for liver cirrhosis, it also affects vascular contractility. However, the relevant effects on portal hypertension have not been evaluated in non-alcoholic cirrhosis. The present study aimed to investigate the impacts of low-dose alcohol on portal hypertension-related derangements in non-alcoholic cirrhotic rats. METHODS: Sprague-Dawley rats received bile duct ligation to induce cirrhosis or sham operation as controls. The chronic or acute effects of low-dose alcohol (2.4 g/kg/day, oral gavage, approximately 1.3 drinks/day in humans) were evaluated. RESULTS: The chronic administration of low-dose alcohol did not precipitate liver fibrosis in the sham or cirrhotic rats; however, it significantly increased splanchnic blood inflow (P=0.034) and portosystemic collaterals (P=0.001). Mesenteric angiogenesis and pro-angiogenic proteins were up-regulated in the alcohol-treated cirrhotic rats, and poorer collateral vasoresponsiveness to vasoconstrictors (P<0.001) was noted. Consistently, acute alcohol administration reduced splenorenal shunt resistance. Collateral vasoresponsiveness to vasoconstrictors also significantly decreased (P=0.003). CONCLUSIONS: In non-alcoholic cirrhosis rats, a single dose of alcohol adversely affected portosystemic collateral vessels due to vasodilatation. Long-term alcohol use precipitated splanchnic hyperdynamic circulation, in which mesenteric angiogenesis played a role. Further studies are warranted to evaluate the benefits of avoiding low-dose alcohol consumption in patients with non-alcoholic cirrhosis.
Asunto(s)
Etanol , Hipertensión Portal , Cirrosis Hepática , Ratas Sprague-Dawley , Circulación Esplácnica , Animales , Etanol/administración & dosificación , Masculino , Ratas , Circulación Esplácnica/efectos de los fármacos , Cirrosis Hepática/fisiopatología , Cirrosis Hepática/inducido químicamente , Cirrosis Hepática/patología , Hipertensión Portal/fisiopatología , Hipertensión Portal/etiología , Hipertensión Portal/inducido químicamente , Hipertensión Portal/patología , Circulación Colateral/efectos de los fármacos , Vasoconstricción/efectos de los fármacosRESUMEN
BACKGROUND AND AIMS: Porto-sinusoidal vascular disease (PSVD) is an under-recognized and under-diagnosed disease. The purpose of this study was to investigate the clinical features and prognosis of PSVD. METHODS: The patients who underwent liver biopsies were analyzed retrospectively. The clinical and pathological data were reviewed and screened according to the latest diagnostic criteria of PSVD. RESULTS: A total of 234 patients were diagnosed as PSVD, including 103 patients presented with portal hypertension (PH) and 131 patients without PH. At baseline, the alanine aminotransferase (ALT) and γ-glutamyl transpeptidase (GGT) levels were higher in the no-PH group. The liver stiffness increased in the PH group. In histological review, obliterative portal venopathy, sinusoidal dilatation and architectural disturbance were more common in the PH group, while portal tract abnormalities were more widely distributed in the no-PH group. After a median follow-up of 43.6 months, the survival rate of patients with baseline liver decompensation was 76.0%, and that of patients at a liver compensated stage in the PH group was 98.7%. First variceal bleeding occurred in 13.8% of patients with gastric-oesophageal varices. None of the patients in the no-PH group developed portal hypertension during follow-up. CONCLUSIONS: PSVD can manifest as PH or mild liver enzyme abnormalities. There are significant differences in pathological features among patients with different clinical manifestations. Recurrent ascites are the main cause of death in PSVD patients. However, patients without PH have a slow disease progression, with recurrent elevated GGT levels being their main clinical feature.