RESUMO
AIM: Physical exercise training attenuates pulmonary inflammation, but its effects on impaired respiratory function caused by hepatopulmonary syndrome (HPS) have not been evaluated. We determined if the combination of moderate intensity aerobic and resistance training during HPS development modifies exercise capacity, respiratory system mechanics, and lung inflammation responses. MAIN METHODS: Wistar rats were randomly divided into sham, HPS, and HPS + combined exercise training groups. Fifteen days after HPS induction, a moderate intensity aerobic plus resistance exercise training protocol was performed five times a week for 5 weeks on alternate days. Exercise capacity, respiratory system mechanics, lung inflammation, pulmonary morphology, and immunohistochemistry were evaluated. KEY FINDINGS: Overall, our findings indicated that combined exercise training efficiently increased the maximal running and resistance capacity of HPS animals. The training regimen reduced the expression of P2X7 in parenchymal leukocytes (P < 0.01), partially restored the expression of interleukin-10 in airway epithelium (P < 0.01), and increased the expression of TFPI in the airway epithelium (P < 0.01) as well as reduced its expression in parenchymal leukocytes (P < 0.01). However, exercise training did not attenuate HPS-induced respiratory mechanical derangements or lung tissue remodeling. SIGNIFICANCE: Combined exercise training can elicit adaptation with regard to both maximal running capacity and maximum strength and modify the expression of P2X7 and TFPI in parenchymal leukocytes and that of IL-10 in airway epithelium.
Assuntos
Teste de Esforço/métodos , Tolerância ao Exercício/fisiologia , Síndrome Hepatopulmonar/terapia , Condicionamento Físico Animal/métodos , Condicionamento Físico Animal/fisiologia , Pneumonia/terapia , Animais , Síndrome Hepatopulmonar/patologia , Síndrome Hepatopulmonar/fisiopatologia , Masculino , Pneumonia/patologia , Pneumonia/fisiopatologia , Ratos , Ratos Wistar , Mecânica Respiratória/fisiologiaRESUMO
BACKGROUND: The treatment of choice for patients with cirrhosis and HPS is LT. The clinical manifestations associated with hypoxemia result in limitations and a poor health-related quality of life of affected patients. The present report aims to study the differences in outcomes between patients with PaO2 < 50 mm Hg and those with PaO2 ≥ 50 mm Hg. METHODS: This was a retrospective study of 21 patients under 18 years of age conducted from 2001 to 2018; the patients were divided into 2 groups: G1-PaO2 ≥ 50 mm Hg, 11 patients, and G2-PaO2 < 50 mm Hg, 10 patients. Demographic, clinical, laboratory, and perioperative data; outcome variables; and post-transplant survival were compared between the groups. RESULTS: In total, 2/11 (18.2%) patients in G1 and 8/10 (80%) patients in G2 required supplemental oxygen therapy at home (P = .005). Patients in G2 required prolonged MV (median 8.5 days in G2 vs 1 day in G1, P = .015) and prolonged ICU and hospital stays (P = .002 and P = .001, respectively). Oxygen weaning time was longer in G2 (median 127.5 days) than in G1 (median 3 days; P = .004). One (9.1%) patient in G1 and three (30%) patients in G2 died (P = .22). The survival at 90 months was 90.9% in G1 and 70% in G2 (P = .22). CONCLUSION: The survival between groups was similar. Patients with very severe HPS required a longer MV time, longer ICU and hospital stays, and a longer O2 weaning time than those with mild, moderate, or severe HPS.
Assuntos
Síndrome Hepatopulmonar/cirurgia , Hipóxia/etiologia , Cirrose Hepática/cirurgia , Transplante de Fígado , Adolescente , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Seguimentos , Síndrome Hepatopulmonar/fisiopatologia , Humanos , Hipóxia/diagnóstico , Lactente , Tempo de Internação/estatística & dados numéricos , Cirrose Hepática/fisiopatologia , Masculino , Gravidade do Paciente , Cuidados Pós-Operatórios/estatística & dados numéricos , Estudos Retrospectivos , Análise de Sobrevida , Resultado do TratamentoRESUMO
OBJECTIVE: To evaluate the pulmonary alterations of animals with Hepatopulmonary Syndrome (HPS) submitted to Biliary Duct Ligature (BDL), as well as the antioxidant effect of Melatonin (MEL). METHODS: Sixteen male Wistar rats, divided into four Sham groups: BDL group, Sham + MEL group and BDL + MEL. The pulmonary and hepatic histology, lipoperoxidation and antioxidant activity of lung tissue, alveolar-arterial O2 difference and lung / body weight ratio (%) were evaluated. RESULTS: When comparing the groups, could be observed an increase of vasodilation and pulmonary fibrosis in the BDL group and the reduction of this in relation to the BDL + MEL group. It was also observed significant changes in the activity of catalase, ApCO2, ApO2 in the LBD group when compared to the other groups. CONCLUSION: The use of MEL has been shown to be effective in reducing vasodilation, fibrosis levels and oxidative stress as well as gas exchange in an experimental HPS model.
Assuntos
Antioxidantes/farmacologia , Síndrome Hepatopulmonar/tratamento farmacológico , Pulmão/efeitos dos fármacos , Melatonina/farmacologia , Animais , Pressão Arterial/efeitos dos fármacos , Ductos Biliares/cirurgia , Gasometria , Catalase/análise , Modelos Animais de Doenças , Glutationa Transferase/análise , Síndrome Hepatopulmonar/patologia , Síndrome Hepatopulmonar/fisiopatologia , Ligadura , Peroxidação de Lipídeos/efeitos dos fármacos , Fígado/efeitos dos fármacos , Fígado/patologia , Fígado/fisiopatologia , Cirrose Hepática/tratamento farmacológico , Cirrose Hepática/patologia , Cirrose Hepática/fisiopatologia , Pulmão/patologia , Pulmão/fisiopatologia , Masculino , Tamanho do Órgão/efeitos dos fármacos , Estresse Oxidativo/efeitos dos fármacos , Ratos Wistar , Reprodutibilidade dos Testes , Substâncias Reativas com Ácido Tiobarbitúrico/análise , Resultado do TratamentoRESUMO
BACKGROUND: Hepatopulmonary syndrome (HPS) is defined as a triad characterized by arterial deoxygenation, intrapulmonary vascular dilatations (IPVDs), and liver disorder. The aims of this study were to assess the prevalence of HPS in children with cirrhosis, the clinical characteristics of patients with HPS, and the tests used for the diagnosis of IPVD. PATIENTS AND METHODS: This was a prospective, cross-sectional study of 40 children with cirrhosis (median age: 44 months). Investigations of HPS included arterial blood gas analysis, contrast-enhanced transthoracic echocardiography (CE-TTE), and perfusion lung scanning using technetium-99m-labeled macroaggregated albumin (Tc-MMA). Patients' clinical characteristics (age, etiology of cirrhosis, and severity of hepatopathy) were assessed. HPS was defined as liver disease; alveolar-arterial oxygen gradient of at least 15 mmHg and/or partial pressure of arterial oxygen less than 80 mmHg; and detection of IPVD by CE-TTE or Tc-MMA scanning. Statistical significance was indicated by a P value less than 0.05. RESULTS: The prevalence of HPS was 42.5% (17/40). Eight patients had moderate HPS (47%) and two patients had severe HPS (12%). In bivariate analysis, biliary atresia (P=0.033) and median age (10 months; P=0.005) were associated with HPS. In multivariate analysis, only age remained statistically significant (prevalence ratio=0.99; 95% confidence interval=0.98-0.99; P=0.010). Sixteen patients with HPS had IPVD detected by CE-TTE (94.1%) and six patients had IPVD detected by Tc-MMA scanning (35.3%), with no significant agreement between these methods (κ=-0.12; P=0.163). CONCLUSION: HPS is a common complication of cirrhosis in children. A combination of clinical and imaging criteria should be used to diagnose HPS.
Assuntos
Síndrome Hepatopulmonar/epidemiologia , Cirrose Hepática/epidemiologia , Adolescente , Fatores Etários , Gasometria , Brasil/epidemiologia , Criança , Pré-Escolar , Estudos Transversais , Ecocardiografia , Feminino , Síndrome Hepatopulmonar/diagnóstico por imagem , Síndrome Hepatopulmonar/fisiopatologia , Humanos , Lactente , Cirrose Hepática/diagnóstico , Cirrose Hepática/fisiopatologia , Masculino , Imagem de Perfusão/métodos , Valor Preditivo dos Testes , Prevalência , Prognóstico , Estudos Prospectivos , Compostos Radiofarmacêuticos/administração & dosagem , Fatores de Risco , Índice de Gravidade de Doença , Agregado de Albumina Marcado com Tecnécio Tc 99m/administração & dosagemRESUMO
ABSTRACT Objective: To evaluate the pulmonary alterations of animals with Hepatopulmonary Syndrome (HPS) submitted to Biliary Duct Ligature (BDL), as well as the antioxidant effect of Melatonin (MEL). Methods: Sixteen male Wistar rats, divided into four Sham groups: BDL group, Sham + MEL group and BDL + MEL. The pulmonary and hepatic histology, lipoperoxidation and antioxidant activity of lung tissue, alveolar-arterial O2 difference and lung / body weight ratio (%) were evaluated. Results: When comparing the groups, could be observed an increase of vasodilation and pulmonary fibrosis in the BDL group and the reduction of this in relation to the BDL + MEL group. It was also observed significant changes in the activity of catalase, ApCO2, ApO2 in the LBD group when compared to the other groups. Conclusion: The use of MEL has been shown to be effective in reducing vasodilation, fibrosis levels and oxidative stress as well as gas exchange in an experimental HPS model.
RESUMO Objetivo: Avaliar as alterações pulmonares de animais com Síndrome Hepatopulmonar (SHP), submetidos à ligadura de ducto biliar (LDB), bem como o efeito antioxidante da Melatonina (MEL). Métodos: Dezesseis ratos machos da espécie Wistar, divididos em quatro grupos: Sham, Grupo LDB, Grupo Sham + MEL e LDB + MEL. Foram avaliadas a histologia pulmonar e hepática, a lipoperoxidação e atividade antioxidante do tecido pulmonar, diferença álveolo-arterial de O2 e relação peso pulmonar/peso corporal (%). Resultados: Quando comparados os grupos, observamos um aumento da vasodilatação e fibrose pulmonar no grupo LDB e a redução deste em relação ao grupo LDB+MEL. Observamos ainda alterações significativas na atividade da catalase, PaCO2, PaO2 no grupo LBD quando comparado aos demais grupos. Conclusões: A utilização da MEL demonstrou-se eficaz na redução da vasodilatação, níveis de fibrose e estresse oxidativo assim como na troca gasosa em modelo experimental de SHP.
Assuntos
Animais , Masculino , Síndrome Hepatopulmonar/tratamento farmacológico , Pulmão/efeitos dos fármacos , Melatonina/farmacologia , Antioxidantes/farmacologia , Ductos Biliares/cirurgia , Gasometria , Peroxidação de Lipídeos/efeitos dos fármacos , Catalase/análise , Síndrome Hepatopulmonar/fisiopatologia , Síndrome Hepatopulmonar/patologia , Modelos Animais de Doenças , Pressão Arterial/efeitos dos fármacos , Glutationa Transferase/análise , Ligadura , Fígado/efeitos dos fármacos , Fígado/patologiaRESUMO
BACKGROUND AND STUDY AIMS: Chronic liver disease (CLD) can cause hepatopulmonary syndrome (HPS), defined as triad of liver disease, hypoxemia, and intrapulmonary vascular dilation (IPVD). The aim of this study was to determine the evidence of IPVD in a cohort of pediatric patients with CLD pre- and post-liver transplantation (LT). MATERIAL AND METHODS: All pediatric patients with CLD listed for LT were studied. Pulse oxygen saturation (SpO(2)), technetium-99m-labeled macroaggregated albumin ((99m)Tc- MAA) perfusión scan (positive test: uptake of the isotope ≥ 6% in the brain), and echocardiography with saline bubble test (SBT) were performed. SBT was re-evaluated at 3-6 months after LT. Grading of SBT included grade 0 (no bubble), I (1-9 bubbles), grade II (10-20 bubbles), and grade III (> 20 bubbles). RESULTS: Eighteen patients, median age 22.5 months (8-108), were enrolled. Most had biliary atresia (77.8%). Pre-LT, all patients had SpO(2) of 100% and none had positive (99)mTc- MAA perfusion scan. Two patients (11%) had negative SBT (grade 0), 1 (5.5%) had grade I, 3 (16.5%) had grade II, and 12 (67%) had grade III, respectively. Post-LT SBT became negative in all survivors (n = 16), (p = 0.0001). CONCLUSIONS: Most cirrhotic children in this cohort study had evidence of IPVD by positive SBT. However, none of these met the criteria for diagnosis of HPS. This evidence of IPVD subsided after LT.
Assuntos
Síndrome Hepatopulmonar/etiologia , Cirrose Hepática/cirurgia , Transplante de Fígado , Artéria Pulmonar/fisiopatologia , Vasodilatação , Fatores Etários , Criança , Pré-Escolar , Doença Crônica , Ecocardiografia , Feminino , Síndrome Hepatopulmonar/diagnóstico , Síndrome Hepatopulmonar/fisiopatologia , Humanos , Lactente , Cirrose Hepática/complicações , Cirrose Hepática/diagnóstico , Masculino , Oximetria , Imagem de Perfusão/métodos , Valor Preditivo dos Testes , Circulação Pulmonar , Compostos Radiofarmacêuticos/administração & dosagem , Fatores de Risco , Cloreto de Sódio/administração & dosagem , Agregado de Albumina Marcado com Tecnécio Tc 99m/administração & dosagem , Resultado do TratamentoRESUMO
INTRODUCTION: Hepatopulmonary syndrome (HPS) is characterized by a clinical triad of liver disease and/or portal hypertension, intrapulmonary vascular dilatation and abnormal arterial oxygenation. These conditions can worsen muscle strength, exercise capacity and functionality in the affected population. OBJECTIVE: The objective of this study was to compare exercise capacity, functional condition and respiratory muscle strength in cirrhotic patients diagnosed with HPS and cirrhotic patients without this diagnosis. MATERIAL AND METHODS: This cross-sectional study used a convenience sample consisting of 178 patients (92 patients with HPS and 86 patients without HPS) with a diagnosis of liver cirrhosis caused by either alcohol consumption or the hepatitis C virus (HCV). Peak oxygen consumption (VO2 peak) was used to verify exercise capacity, the six-minute walk test (6MWT) was used to test functionality, and manovacuometry was used to evaluate the strength of the respiratory muscles. The Kolmogorov-Smirnov test and Student's t-test were used for the statistical analysis. The data were analyzed using SPSS 16.00, and p < 0.05 was considered significant. RESULTS: The group of patients with the diagnosis of HPS exhibited a lower VO2 peak (14.2 ± 2.3 vs. 17.6 ± 2.6, p < 0.001), shorter distance walked in the 6MWT (340.8 ± 50.9 vs. 416.5 ± 91.4, p < 0.001), lower maximal inspiratory pressure (-49.1 ± 9.8 vs. -74.2 ± 13.9, p = 0.001) and lower maximum expiratory pressure (60.1 ± 12.2 vs. 76.8 ± 14.7, p = 0.001). CONCLUSION: The group of cirrhotic patients diagnosed with HPS exhibited lower values for VO2 peak, distance walked in the 6MWT and respiratory muscle strength than the cirrhotic patients not diagnosed with HPS.
Assuntos
Tolerância ao Exercício/fisiologia , Síndrome Hepatopulmonar/fisiopatologia , Cirrose Hepática/fisiopatologia , Estudos Transversais , Teste de Esforço , Feminino , Síndrome Hepatopulmonar/complicações , Humanos , Cirrose Hepática/etiologia , Masculino , Pessoa de Meia-Idade , Força Muscular/fisiologia , Consumo de Oxigênio , Músculos Respiratórios/fisiopatologiaRESUMO
Chronic liver disease and/or portal hypertension may be associated with one of the two pulmonary vascular complications: portopulmonary hypertension and hepatopulmonary syndrome. These pulmonary vascular disorders are notoriously underdiagnosed; however, they have a substantial negative impact on survival and require special attention in order to understand their diagnostic approach and to select the best therapeutic options. Portopulmonary hypertension results from excessive vasoconstriction, vascular remodeling, and proliferative and thrombotic events within the pulmonary circulation that lead to progressive right ventricular failure and ultimately to death. On the other hand, abnormal intrapulmonary vascular dilations, profound hypoxemia, and a wide alveolar-arterial gradient are the hallmarks of the hepatopulmonary syndrome, resulting in difficult-to-treat hypoxemia. The aim of this review is to summarize the latest pathophysiologic concepts, diagnostic approach, therapy, and prognosis of portopulmonary hypertension and hepatopulmonary syndrome, as well as to discuss the role of liver transplantation as a definitive therapy in selected patients with these conditions.
Assuntos
Hipertensão Portal/complicações , Hepatopatias/complicações , Pneumopatias/etiologia , Circulação Pulmonar/fisiologia , Síndrome Hepatopulmonar/fisiopatologia , Síndrome Hepatopulmonar/terapia , Humanos , Hipertensão Portal/diagnóstico , Hipertensão Portal/tratamento farmacológico , Hipertensão Portal/epidemiologia , Hipertensão Portal/fisiopatologia , Hepatopatias/diagnóstico , Hepatopatias/epidemiologia , Hepatopatias/fisiopatologia , Hepatopatias/terapia , Transplante de Fígado , Pneumopatias/diagnóstico , Pneumopatias/epidemiologia , Pneumopatias/fisiopatologia , Pneumopatias/terapiaRESUMO
Intrapulmonary vasodilation is a hallmark of the hepatopulmonary syndrome (HPS). However, its effects on respiratory mechanical properties and lung morphology are unknown. To determine these effects, 28 rats were randomly divided to control and experimental HPS groups (eHPS). The spontaneous breathing pattern, gas exchange, respiratory system mechanical properties, and lung and liver morphology of the rats were evaluated. Tidal volume, minute ventilation and mean inspiratory flow were significantly reduced in the eHPS group. Chest wall pressure dissipation against the resistive and viscoelastic components and elastic elastance were increased in the eHPS group. The lung resistive pressure dissipation was lower but the viscoelastic pressure was higher in the eHPS group. The airway volume proportion of collagen and elastic fibers was increased in the eHPS animals (16% and 51.7%; P<0.05 and P<0.001, respectively). The proportion of collagen volume in the vasculature increased 29% in the eHPS animals (P<0.01). HPS presents with respiratory system mechanical disarray as well as airway and vascular remodeling.
Assuntos
Remodelação das Vias Aéreas/fisiologia , Síndrome Hepatopulmonar/fisiopatologia , Mecânica Respiratória/fisiologia , Animais , Modelos Animais de Doenças , Síndrome Hepatopulmonar/patologia , Fígado/patologia , Fígado/fisiopatologia , Masculino , Ratos , Ratos WistarRESUMO
OBJECTIVE: To determine the occurrence of hepatopulmonary syndrome (HPS) in patients with cirrhosis who are candidates for liver transplantation; to compare demographic, clinical, laboratory, and spirometric characteristics, as well as echocardiography results, arterial blood gas analysis, and severity of liver disease between the groups of patients with and without HPS; and to describe the occurrence of HPS in the subgroup of patients with cirrhosis and schistosomiasis mansoni (mixed liver disease). METHODS: Between January and November of 2007, we evaluated 44 patients under treatment at the Liver Transplant Outpatient Clinic of the Federal University of Pernambuco Hospital das Clínicas, in the city of Recife, Brazil. The diagnostic criteria for HPS were intrapulmonary vascular dilatation, identified by transthoracic echocardiography, and an alveolar-arterial oxygen tension difference >or= 15 mmHg or a PaO2 < 80 mmHg. RESULTS: The mean age of the patients was 52 years, and 31 patients (70%) were male. The most common cause of cirrhosis was alcohol use. Schistosomiasis was present in 28 patients (64%). Of the 44 patients, 20 (45.5%) were diagnosed with HPS. No significant differences were found between those patients and the patients without HPS in terms of any of the characteristics studied. Of the 28 patients with cirrhosis and schistosomiasis, 10 (35.7%) were diagnosed with HPS. CONCLUSIONS: In the population studied, HPS was highly prevalent and did not correlate with any of the variables analyzed.
Assuntos
Síndrome Hepatopulmonar/epidemiologia , Cirrose Hepática/diagnóstico , Transplante de Fígado , Esquistossomose/diagnóstico , Brasil/epidemiologia , Feminino , Síndrome Hepatopulmonar/diagnóstico , Síndrome Hepatopulmonar/fisiopatologia , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
OBJETIVO: Verificar a ocorrência da síndrome hepatopulmonar (SHP) em pacientes cirróticos candidatos a transplante de fígado; comparar as características demográficas, clínicas, laboratoriais e espirométricas, resultados de ecocardiografia, análise de gases arteriais e da gravidade da doença hepática nos pacientes com e sem SHP; e descrever a ocorrência de SHP no subgrupo de pacientes com cirrose associada à esquistossomose mansônica (doença hepática mista). MÉTODOS: Entre janeiro e novembro de 2007, foram avaliados 44 pacientes inscritos no Ambulatório de Transplante Hepático do Hospital das Clínicas da Universidade Federal de Pernambuco, em Recife (PE). Os critérios diagnósticos para SHP foram a presença de dilatações vasculares intrapulmonares, identificadas por ecocardiografia transtorácica, assim como diferença alveoloarterial de oxigênio > 15 mmHg ou PaO2 < 80 mmHg. RESULTADOS: A idade média foi 52 anos, e 31 pacientes (70 por cento) eram do sexo masculino. A causa mais frequente de cirrose foi uso de etanol. A esquistossomose esteve presente em 28 pacientes (64 por cento). Dos 44 pacientes, 20 (45,5 por cento) foram diagnosticados com SHP. Não foram observadas diferenças significativas em relação às características estudadas. No subgrupo de pacientes com cirrose associada à esquistossomose, 10/28 (35,7 por cento) receberam o diagnóstico de SHP. CONCLUSÕES: A SHP apresentou elevada prevalência nesta população estudada, não sendo observadas associações entre a sua ocorrência e as variáveis analisadas.
OBJECTIVE: To determine the occurrence of hepatopulmonary syndrome (HPS) in patients with cirrhosis who are candidates for liver transplantation; to compare demographic, clinical, laboratory, and spirometric characteristics, as well as echocardiography results, arterial blood gas analysis, and severity of liver disease between the groups of patients with and without HPS; and to describe the occurrence of HPS in the subgroup of patients with cirrhosis and schistosomiasis mansoni (mixed liver disease). METHODS: Between January and November of 2007, we evaluated 44 patients under treatment at the Liver Transplant Outpatient Clinic of the Federal University of Pernambuco Hospital das Clínicas, in the city of Recife, Brazil. The diagnostic criteria for HPS were intrapulmonary vascular dilatation, identified by transthoracic echocardiography, and an alveolar-arterial oxygen tension difference > 15 mmHg or a PaO2 < 80 mmHg. RESULTS: The mean age of the patients was 52 years, and 31 patients (70 percent) were male. The most common cause of cirrhosis was alcohol use. Schistosomiasis was present in 28 patients (64 percent). Of the 44 patients, 20 (45.5 percent) were diagnosed with HPS. No significant differences were found between those patients and the patients without HPS in terms of any of the characteristics studied. Of the 28 patients with cirrhosis and schistosomiasis, 10 (35.7 percent) were diagnosed with HPS. CONCLUSIONS: In the population studied, HPS was highly prevalent and did not correlate with any of the variables analyzed.
Assuntos
Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Síndrome Hepatopulmonar/epidemiologia , Transplante de Fígado , Cirrose Hepática/diagnóstico , Esquistossomose/diagnóstico , Brasil/epidemiologia , Síndrome Hepatopulmonar/diagnóstico , Síndrome Hepatopulmonar/fisiopatologiaRESUMO
The clinical course of patients with portal hypertension or liver disease may be complicated by two low prevalence entities with high morbimortality: the hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHT). Each one is a consequence ofan impaired hepatic clearance of several vascular mediators, triggering vasodilation of the pulmonary vascular territory in HPS and vasoconstriction with vessel remodelation in PPHT. Both disorders have some similar clinical findings, but useful findings for differential diagnosis are the presence of platypnoea and orthodeoxia in HPS, and echocardiographic extracardiac and intrapulmonary shunt in HPS or pulmonary hypertension in PPHT. Currently, liver transplantation is the only effective treatment for both entities provided that indication and timing must be accurately evaluated. We present a review and three cases of both entities.
El curso clínico de los pacientes con cirrosis y/o hipertensión portal puede verse complicado por dos entidades de baja prevalencia pero de elevada morbimortalidad, que corresponden al síndrome hepatopulmonar (SHP) y la hipertensión portopulmonar (HPP). Ambas se presentan a consecuencia de un déficit en la depuración hepática de diversos mediadores vasculares, provocando en el territorio pulmonar una vasodilatación en el SHP y una vasoconstricción con remodelación vascular en la HPP. Si bien estas entidades comparten algunos aspectos clínicos, resulta útil en su diferenciación la presencia de platipnea y ortodeoxia y el hallazgo ecocardiográfico de un shunt extracardíaco e intrapulmonar en el SHP, o de hipertensión pulmonar en HPP. Hasta el momento la única terapia efectiva para ambas entidades es el trasplante hepático, cuya indicación exige una evaluación rigurosa y oportuna. Se presenta una revisión y tres casos clínicos de ambas entidades.
Assuntos
Humanos , Adolescente , Feminino , Pessoa de Meia-Idade , Hipertensão Portal/diagnóstico , Hipertensão Pulmonar/diagnóstico , Síndrome Hepatopulmonar/diagnóstico , Cirrose Hepática/complicações , Diagnóstico Diferencial , Hipertensão Portal/etiologia , Hipertensão Portal/terapia , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/terapia , Insuficiência Hepática/complicações , Prognóstico , Síndrome Hepatopulmonar/etiologia , Síndrome Hepatopulmonar/fisiopatologia , Síndrome Hepatopulmonar/terapiaRESUMO
OBJECTIVE: The aim of this study was to identify the best experimental model in which to observe the pulmonary alterations characterizing hepatopulmonary syndrome (HPS). METHODS: Male Wistar rats, with mean weight of 250 g, were used in four experimental models: inhaled carbon tetrachloride; intraperitoneal carbon tetrachloride; partial portal vein ligation; and bile duct ligation (BDL). The animals in all groups were divided into control and experimental subgroups. The following variables were measured: transaminase levels; blood gases; lipoperoxidation, using thiobarbituric acid reactive substances (TBARS) and chemiluminescence; and levels of superoxide dismutase (SOD) anti-oxidant activity. Anatomopathological examination of the lung was also performed. RESULTS: There were statistically significant differences between the BDL control and BDL experimental groups: aspartate aminotransferase (105.3 +/- 43 vs. 500.5 +/- 90.3 IU/L); alanine aminotransferase (78.75 +/- 37.7 vs. 162.75 +/- 35.4 IU/L); alkaline phosphatase (160 +/- 20.45 vs. 373.25 +/- 45.44 IU/L); arterial oxygen tension (85.25 +/- 8.1 vs. 49.9 +/- 22.5 mmHg); and oxygen saturation (95 +/- 0.7 vs. 73.3 +/- 12.07%). Lipoperoxidation and antioxidant activity also differed significantly between the two BDL groups (control vs. experimental): TBARS (0.87 +/- 0.3 vs. 2.01 +/- 0.9 nmol/mg protein); chemiluminescence (16008.41 +/- 1171.45 vs. 20250.36 +/- 827.82 cps/mg protein); and SOD (6.66 +/- 1.34 vs. 16.06 +/- 2.67 IU/mg protein). The anatomopathological examination confirmed pulmonary vasodilatation in the BDL model. In the other models, there were no alterations that were characteristic of HPS. CONCLUSIONS: The data obtained suggest that the BDL model can be used in future studies involving hepatic alterations related to oxidative stress and HPS.
Assuntos
Síndrome Hepatopulmonar/complicações , Hipertensão Pulmonar/etiologia , Cirrose Hepática Experimental/patologia , Pulmão/patologia , Estresse Oxidativo , Análise de Variância , Animais , Antioxidantes/metabolismo , Peso Corporal , Ducto Colédoco/cirurgia , Síndrome Hepatopulmonar/fisiopatologia , Hipertensão Pulmonar/patologia , Hipertensão Pulmonar/fisiopatologia , Fígado/patologia , Fígado/fisiopatologia , Cirrose Hepática Experimental/induzido quimicamente , Cirrose Hepática Experimental/fisiopatologia , Testes de Função Hepática , Pulmão/fisiopatologia , Masculino , Tamanho do Órgão , Veia Porta/fisiopatologia , Troca Gasosa Pulmonar , Ratos , Ratos Wistar , Superóxido Dismutase/metabolismoRESUMO
OBJETIVO: O objetivo deste trabalho foi avaliar o melhor modelo experimental para observar alterações pulmonares que caracterizam a síndrome hepatopulmonar (SHP). MÉTODOS: Ratos machos Wistar, com peso médio de 250 g foram usados em quatro modelos experimentais: tetracloreto de carbono inalatório; tetracloreto de carbono intraperitoneal; ligadura parcial de veia porta; e ligadura de ducto biliar (LDB). Em todos os grupos os animais foram divididos em controle e experimental. Foram avaliadas as seguintes variáveis: transaminases; gasometria; lipoperoxidação por substâncias que reagem ao ácido tiobarbitúrico (TBARS) e por quimiluminescência; e atividade antioxidante da enzima superóxido dismutase (SOD). Foi feito também o exame anatomopatológico do pulmão. RESULTADOS: Observou-se diferenças significativas entre os grupos LDB controle e experimental: aspartato amino transferase (105,3 ± 43 vs. 500,5 ± 90,3 UI/L); alanino aminotransferase (78,75 ± 37,7 vs. 162,75 ± 35,4 UI/L); fosfatase alcalina (160 ± 20,45 vs. 373,25 ± 45,44 UI/L); pressão parcial de oxigênio (85,25 ± 8,1 vs. 49,9 ± 22,5 mmHg); e saturação de hemoglobina (95 ± 0,7 vs. 73,3 ± 12,07 por cento). A lipoperoxidação e a atividade antioxidante também demonstrou diferenças entre os dois grupos LDB (controle vs. experimental): TBARS (0,87 ± 0,3 vs. 2,01 ± 0,9 nmol/mg proteína); quimiluminescência (16008,41 ± 1171,45 vs. 20250,36 ± 827,82 cps/mg proteína); e SOD (6,66 ± 1,34 vs. 16,06 ± 2,67 UI/mg proteína). No exame anatomopatológico observou-se vasodilatação pulmonar no modelo de LDB. CONCLUSÕES: Os dados sugerem que o modelo de LDB pode ser usado para outros estudos envolvendo alterações hepáticas e suas relações com o estresse oxidativo e a SHP.
OBJECTIVE: The aim of this study was to identify the best experimental model in which to observe the pulmonary alterations characterizing hepatopulmonary syndrome (HPS). METHODS: Male Wistar rats, with mean weight of 250 g, were used in four experimental models: inhaled carbon tetrachloride; intraperitoneal carbon tetrachloride; partial portal vein ligation; and bile duct ligation (BDL). The animals in all groups were divided into control and experimental subgroups. The following variables were measured: transaminase levels; blood gases; lipoperoxidation, using thiobarbituric acid reactive substances (TBARS) and chemiluminescence; and levels of superoxide dismutase (SOD) anti-oxidant activity. Anatomopathological examination of the lung was also performed. RESULTS: There were statistically significant differences between the BDL control and BDL experimental groups: aspartate aminotransferase (105.3 ± 43 vs. 500.5 ± 90.3 IU/L); alanine aminotransferase (78.75 ± 37.7 vs. 162.75 ± 35.4 IU/L); alkaline phosphatase (160 ± 20.45 vs. 373.25 ± 45.44 IU/L); arterial oxygen tension (85.25 ± 8.1 vs. 49.9 ± 22.5 mmHg); and oxygen saturation (95 ± 0.7 vs. 73.3 ± 12.07 percent). Lipoperoxidation and antioxidant activity also differed significantly between the two BDL groups (control vs. experimental): TBARS (0.87 ± 0.3 vs. 2.01 ± 0.9 nmol/mg protein); chemiluminescence (16008.41 ± 1171.45 vs. 20250.36 ± 827.82 cps/mg protein); and SOD (6.66 ± 1.34 vs. 16.06 ± 2.67 IU/mg protein). The anatomopathological examination confirmed pulmonary vasodilatation in the BDL model. In the other models, there were no alterations that were characteristic of HPS. CONCLUSIONS: The data obtained suggest that the BDL model can be used in future studies involving hepatic alterations related to oxidative stress and HPS.
Assuntos
Animais , Masculino , Ratos , Síndrome Hepatopulmonar/complicações , Hipertensão Pulmonar/etiologia , Cirrose Hepática Experimental/patologia , Pulmão/patologia , Estresse Oxidativo , Análise de Variância , Antioxidantes/metabolismo , Peso Corporal , Ducto Colédoco/cirurgia , Síndrome Hepatopulmonar/fisiopatologia , Hipertensão Pulmonar/patologia , Hipertensão Pulmonar/fisiopatologia , Testes de Função Hepática , Cirrose Hepática Experimental/induzido quimicamente , Cirrose Hepática Experimental/fisiopatologia , Fígado/patologia , Fígado/fisiopatologia , Pulmão/fisiopatologia , Tamanho do Órgão , Troca Gasosa Pulmonar , Veia Porta/fisiopatologia , Ratos Wistar , Superóxido Dismutase/metabolismoAssuntos
Humanos , Masculino , Feminino , Diagnóstico , Transplante de Fígado , Oxigenoterapia , Síndrome Hepatopulmonar/epidemiologia , Síndrome Hepatopulmonar/fisiopatologia , Diagnóstico Biopatográfico , Diagnóstico Clínico , Síndrome Hepatopulmonar/terapia , Técnicas e Procedimentos DiagnósticosRESUMO
Cardiopulmonar complications in chronic liver diseases were described 100 years ago. Altough both hepatopulmonary sindrome and portopulmonary hypertension originates from liver damage, clinical findings and diagnosis are very different. These complications are important due to the highly deleterous impact on disease evolution and prognosis. Currently, there is not an ideal treatment for these diseases and liver transplantation should be adequately evaluated. In this review we analyze the most important issues on hepatopulmonary sindrome and portopulmonary hypertension. These complications,under the cornerstone of portal hypertension are characterized by pulmonary constriction and dilatation, respectively.
Assuntos
Síndrome Hepatopulmonar , Hipertensão Portal , Hipertensão Pulmonar , Cirrose Hepática/complicações , Adulto , Algoritmos , Criança , Ensaios Clínicos como Assunto , Ecocardiografia , Feminino , Síndrome Hepatopulmonar/diagnóstico , Síndrome Hepatopulmonar/diagnóstico por imagem , Síndrome Hepatopulmonar/tratamento farmacológico , Síndrome Hepatopulmonar/epidemiologia , Síndrome Hepatopulmonar/etiologia , Síndrome Hepatopulmonar/fisiopatologia , Síndrome Hepatopulmonar/terapia , Humanos , Hipertensão Portal/diagnóstico , Hipertensão Portal/tratamento farmacológico , Hipertensão Portal/epidemiologia , Hipertensão Portal/etiologia , Hipertensão Portal/fisiopatologia , Hipertensão Portal/terapia , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/tratamento farmacológico , Hipertensão Pulmonar/epidemiologia , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/fisiopatologia , Hipertensão Pulmonar/terapia , Cirrose Hepática/fisiopatologia , Transplante de Fígado , Masculino , Derivação Portossistêmica Transjugular Intra-Hepática , Prevalência , Prognóstico , Artéria Pulmonar/fisiopatologia , Circulação Pulmonar , Vasoconstrição/fisiologia , Vasodilatação/fisiologiaRESUMO
Las complicaciones pulmonares de las enfermedades crónicas del hígado han sido descritas desde hace más de un siglo. Aunque originadas por una lesión común y vías fisiopatológicas similares, sus manifestaciones clínicas y el diagnóstico son divergentes. Su impacto clínico es importante ya que afectan de forma deletérea el pronóstico de los pacientes. Hasta el momento no existe un tratamiento efectivo para el manejo de estas enfermedades y el trasplante hepático debe ser evaluado de forma muy cuidadosa. En esta revisión se analizan los aspectos más importantes de la hipertensión portopulmonar y del síndrome hepatopulmonar, entidades que bajo el entorno de la hipertensión portal se caracterizan respectivamente por procesos de vasoconstricción y vasodilatación pulmonar.
Cardiopulmonar complications in chronic liver diseases were described 100 years ago. Altough both hepatopulmonary sindrome and portopulmonary hypertension originates from liver damage, clinical findings and diagnosis are very different. These complications are important due to the highly deleterous impact on disease evolution and prognosis. Currently, there is not an ideal treatment for these diseases and liver transplantation should be adequately evaluated. In this review we analyze the most important issues on hepatopulmonary sindrome and portopulmonary hypertension. These complications,under the cornerstone of portal hypertension are characterized by pulmonary constriction and dilatation, respectively.
Assuntos
Humanos , Masculino , Feminino , Criança , Adulto , Cirrose Hepática/complicações , Síndrome Hepatopulmonar , Hipertensão Portal , Hipertensão Pulmonar , Algoritmos , Artéria Pulmonar/fisiopatologia , Cirrose Hepática/fisiopatologia , Ecocardiografia , Hipertensão Portal/diagnóstico , Hipertensão Portal/etiologia , Hipertensão Portal/fisiopatologia , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/etiologia , Hipertensão Pulmonar/fisiopatologia , Transplante de Fígado , Derivação Portossistêmica Transjugular Intra-Hepática , Prevalência , Prognóstico , Circulação Pulmonar , Síndrome Hepatopulmonar/diagnóstico , Síndrome Hepatopulmonar/etiologia , Síndrome Hepatopulmonar/fisiopatologia , Vasoconstrição/fisiologia , Vasodilatação/fisiologiaRESUMO
El síndrome hepatopulmonar constituye una causa conocida de insuficiencia respiratoria en cirrosis hepática. Se define en base a la tríada de enfermedad hepática generalmente con hipertensión portal, hipoxemia arterial y vasodilatación pulmonar capilar y precapilar, que condicionan shunts de derecha a izquierda, alteraciones en la ventilación-perfusión y en la difusión. Su incidencia oscila entre el 15 y 47 por ciento, y aunque se puede presentar en pacientes con hepatopatía aguda, es una complicación característica de pacientes con cirrosis. Clinicamente existe disnea de esfuerzo, platipnea y ortodeoxia, además de cianosis, acropaquia y nevi aracniformes. Su diagnóstico se basa en el estudio de la función pulmonar y el ecocardiograma con contraste de burbujas. La gammagrafía pulmonar de perfusión con albúmina macroagregada marcada con tecnesio-99m permite la estimación de la fracción del shunt. El trasplante hepático es el único tratamiento demostradamente eficaz, excepto en aquellos pacientes con un trastorno ventilatorio más grave, por su mayor morbi-mortalidad.
The Hepatopulmonary syndrome is a know cause of respiratory failure in cirrhosis. It is a clinical triad characterized by liver disease generally with portal hypertension, arterial hypoxaemia and precapillary-capillary intrapulmonary vascular dilatation leading to right and left shunts, ventilation/perfusion defects and diffusion impairment. Its incidence is about 15 to 47 percent in patients with acute liver disease but characteristly in chronic liver disease. Shortness of breath, orthodeoxia and platypnoea, togheter with cyanosis, digital clubbing and spider naevi are common. Its diagnosis on the basis of the pulmonary gas exchange abnormality and contrast-enhanced echocardiography. The perfusion lung scanning using technetium-labelled macro-aggregatesalbumin estimate the shunt fraction. The orthotopic liver transplantation is the only efficacy treatment in patients without several gas exchange abnormality.
Assuntos
Humanos , Masculino , Feminino , Criança , Pessoa de Meia-Idade , Síndrome Hepatopulmonar/epidemiologia , Síndrome Hepatopulmonar/etiologia , Síndrome Hepatopulmonar/fisiopatologia , Síndrome Hepatopulmonar/patologia , Síndrome Hepatopulmonar/terapia , HepatopatiasRESUMO
OBJECTIVES: To determine the frequency and the clinical characteristics of hepatopulmonary syndrome (HPS) in cirrhotic candidates for orthotopic liver transplantation and to identify the major respiratory parameters predictive of the presence of changes in arterial oxygenation. PATIENTS AND METHODS: Patients underwent transthoracic contrast-enhanced echocardiography, pulmonary scintigraphy, pulmonary function test with diffusing capacity of lung for carbon monoxide (DLCO), and measurement of arterial blood gases. RESULTS: Fifty-six patients were studied. Twenty-five patients (45%) presented with intrapulmonary vascular dilatations, but only 9 (16%) fulfilled the criteria for HPS. The clinical or demographic characteristics considered did not differ in the patients with and without HPS. The DLCO value was significantly lower in patients with HPS (P=.01). However, 32 (80%) of 40 patients with low DLCO values did not fulfill the criteria for HPS. An alveolar arterial oxygen gradient (AaPO2) of more than 20 mm Hg showed a higher diagnostic accuracy (91%) in the assessment of HPS than did the DLCO of less than 80% predicted (41%) and the AaPO2 of more than 15 mm Hg (71%). CONCLUSIONS: The AaPO2 proved to be a more reliable index than PaO2 and DLCO for the determination of changes in arterial oxygenation in HPS. The DLCO does not seem to be a good marker for HPS screening. Intrapulmonary vascular dilatations were frequent, even in patients who did not fulfill the criteria for HPS.