Budd-Chiari syndrome with hepatopulmonary syndrome: a case report and literature review / 中华儿科杂志

Objective: To summarize the clinical features and prognosis of Budd-Chiari syndrome with hepatopulmonary syndrome (HPS) in children. Methods: The clinical data of a child who had Budd-Chiari syndrome with HPS treated at the Department of Pediatrics of the First Affiliated Hospital of Zhengzhou University in December 2016 was analyzed retrospectively. Taking "Budd-Chiari syndrome" and "hepatopulmonary syndrome" in Chinese or English as the keywords, literature was searched at CNKI, Wanfang, China Biomedical Literature Database and PubMed up to July 2023. Combined with this case, the clinical characteristics, diagnosis, treatment and prognosis of Budd-Chiari syndrome with HPS in children under the age of 18 were summarized. Results: A 13-year-old boy, presented with cyanosis and chest tightness after activities for 6 months, and yellow staining of the skin for 1 week. Physical examination at admission not only found mild yellow staining of the skin and sclera, but also found cyanosis of the lips, periocular skin, and extremities. Laboratory examination showed abnormal liver function with total bilirubin 53 μmol/L, direct bilirubin 14 μmol/L, and indirect bilirubin 39 μmol/L, and abnormal blood gas analysis with the partial pressure of oxygen of 54 mmHg (1 mmHg=0.133 kPa), the partial pressure of carbon dioxide of 31 mmHg, and the alveolar-arterial oxygen gradient of 57 mmHg. Hepatic vein-type Budd-Chiari syndrome, cirrhosis, and portal hypertension were indicated by abdominal CT venography. Contrast-enhanced transthoracic echocardiography (CE-TTE) was positive. After symptomatic and supportive treatment, this patient was discharged and received oxygen therapy outside the hospital. At follow-up until March 2023, there was no significant improvement in hypoxemia, accompanied by limited daily activities. Based on the literature, there were 3 reports in English while none in Chinese, 3 cases were reported. Among a total of 4 children, the chief complaints were dyspnea, cyanosis, or hypoxemia in 3 cases, and unknown in 1 case. There were 2 cases diagnosed with Budd-Chiari syndrome with HPS at the same time due to respiratory symptoms, and 2 cases developed HPS 1.5 years and 8.0 years after the diagnosis of Budd-Chiari syndrome respectively. CE-TTE was positive in 2 cases and pulmonary perfusion imaging was positive in 2 cases. Liver transplantation was performed in 2 cases and their respiratory function recovered well; 1 case received oxygen therapy, with no improvement in hypoxemia; 1 case was waiting for liver transplantation. Conclusions: The onset of Budd-Chiari syndrome with HPS is insidious. The most common clinical manifestations are dyspnea and cyanosis. It can reduce misdiagnosis to confirm intrapulmonary vascular dilatations with CE-TTE at an early stage. Liver transplantation is helpful in improving the prognosis.

Frecuencia de síndrome hepatopulmonar en pacientes pediátricos trasplantados en un hospital de alta complejidad en Medellín, Colombia / Frequency of hepato-pulmonary syndrome in pediatric transplanted patients in a high complexity hospital in Medellín, Colombia

Introducción. El síndrome hepatopulmonar (SHP) es una complicación grave en los pacientes con enfermedad hepática crónica y/o hipertensión portal. La frecuencia descrita en adultos es entre 4 % y 47 %. En pediatría, los reportes también son muy variables y van desde el 3 % hasta el 40 %, desconociendo la real incidencia de este. El objetivo de esta descripción es conocer la frecuencia del SHP en pacientes pediátricos en un hospital de alta complejidad, por medio de una búsqueda activa del SHP en los estudios pretrasplante hepático hallados en las historias clínicas. Metodolo-gía. Estudio retrospectivo de 5 años, en un hospital de alta complejidad en Colombia, en menores de 15 años con un primer trasplante hepático. Resultados. Se contó con la información de 24 pacientes, se analizaron variables demográficas y se confirmó el SHP en 18 pacientes (75 %), encontrando una gravedad leve o moderada en el 33 % y 44 %, respectivamente, siendo en este grupo la cirrosis con complicaciones por hipertensión portal la indicación más frecuente para el trasplante, y como etiología de base, la atresia de vías biliares en un 61 %. Conclusión. El SHP en nuestra población se encontró con una alta frecuencia de presentación, por encima de lo reportado en la literatura, llevando a recomendar una búsqueda activa, con el objetivo de brindar un manejo integral y oportuno.

Introduction. Hepatopulmonary syndrome (HPS) is a serious complication in patients with chronic liver disease and/or portal hypertension. The frequency described in adults is between 4% and 47%. In pediatrics, reports are also highly variable and range from 3% to 40%, with the real incidence not clear yet. The objective of this study is to know the frequency of HPS in our population through an active search for HPS in pre-liver transplant studies in clinical records. Methodology. This is a 5-year retrospective study, in a reference hospital in Colombia, that included children under 15 years of age with a first liver transplant. Results. In 24 patients, the information was available, demographic variables were analyzed, and HPS was confirmed in 18 patients (75%), finding mild and moderate severity in 33% and 44%, respectively. In this group, cirrhosis with complications due to portal hyper-tension was the most frequent indication for transplantation and biliary atresia was the main etiology in 61%. Conclusion. HPS in our population was found with a high frequency, higher than is described in the literature, leading to the recommendation of an active search, with the aim of providing com-prehensive and timely management.

Correlation between hepatopulmonary syndrome and oxygen saturation pulse oximetry in cirrhotic patients

SUMMARY Hepatopulmonary Syndrome (HPS) is a complication of cirrhosis that worsens the disease's prognosis, pre and post liver transplant. The objective of this study is to analyze the prevalence of HPS in cirrhotic patients at our service and to correlate it with oxygen saturation (SatO2) using a pulse oximeter to evaluate if this is useful as a screening test for HPS. A prospective study was conducted in patients with hepatic cirrhosis conventionally selected from 2014 to 2016. All the patients underwent an echocardiogram with microbubbles and oxygen saturation measurement by pulse oximetry. Those with intrapulmonary shunt were submitted to arterial blood gas analysis. The relationship between oxygen saturation and HPS was assessed by the multivariate model of binary logistic regression. We analyzed 77 patients, and 23.3% (18 patients) had all criteria for HPS. The relationship between HPS and SatO2 did not show statistical significance, even after the variables were adjusted for sex, age, and smoking. Oxygen saturation alone was not able to detect HPS in the sample of cirrhotic patients. More accurate methods for screening and diagnosis of the syndrome should be used.

RESUMO A Síndrome Hepatopulmonar (SHP) é uma complicação da cirrose que piora o prognóstico da doença pré e pós-transplante hepático. O objetivo do trabalho é analisar a prevalência de SHP em pacientes cirróticos de nosso serviço e correlacioná-la com a saturação de oxigênio (SatO2) pelo oxímetro de pulso, e avaliar se este seria útil como um exame de triagem no diagnóstico de SHP. Foi realizado um estudo prospectivo em pacientes portadores de cirrose hepática no período de 2014 a 2016. Todos os pacientes foram submetidos a um ecocardiogama com microbolhas e a saturação de oxigênio pela oximetria de pulso. Aqueles com shunt intrapulmonar foram submetidos a gasometria arterial. A relação entre a saturação de oxigênio e SHP foi avaliada pelo modelo multivariado de regressão logística binário. Foram analisados 77 pacientes, destes 23,3% (18 pacientes) apresentaram todos os critérios para SHP. A relação entre a SHP com a SatO2 não obteve significância estatística, mesmo após as variáveis terem sido ajustadas pelo sexo, idade e tabagismo atual ou passado. A saturação de oxigênio, de forma isolada, não foi capaz de distinguir a SHP na amostra de pacientes cirróticos em nosso estudo. Deve-se utilizar métodos mais acurados para a triagem e diagnóstico dessa síndrome.

Capacidade de exercício e sobrevida de pacientes cirróticos com e sem síndrome hepatopulmonar após o transplante hepático / Capacity of exercise and survival in patients with cirrhosis with and without hepatopulmonary syndrome after liver transplantation

ABSTRACT BACKGROUND: Hepatopulmonary syndrome (HPS) is a complication associated with cirrhosis that may contribute to worsening exercise capacity and reduced survival after liver transplantation (LT). OBJECTIVE: To evaluate exercise capacity, complications and survival after LT in patients with cirrhosis and HPS and to compare these results with the results of patients with cirrhosis without HPS. METHODS: A prospective cohort study, consisting initially of 178 patients, of whom 90 underwent LT (42 with HPS and 48 without HPS). A previous evaluation consisted of the six-minute walk test (6MWT), an exercise test and manovacuometry. Those who underwent LT were evaluated for the mechanical ventilation time (MV), noninvasive ventilation (NIV) use, and survival two years after the procedure. In the statistical analysis, we used the Kolmogorov-Smirnov test, Student's t-test, the linear association square test, and the Kaplan-Meier survival curve. The data were analyzed with the SPSS 16.00 program and considered significant at P<0.05. RESULTS: The HPS group demonstrated a lower peak of oxygen consumption (VO2peak) (14.2±2.3 vs 17.6±2.6) P<0.001 and a shorter distance walked on the 6MWT (340.8±50.9 vs 416.5±91.4) P<0.001 before LT compared with the non-HPS group. The transplanted patients with HPS remained longer hours in MV (19.5±4.3 vs 12.5±3.3) P=0.02, required more NIV (12 vs 2) P=0.01, and had lower survival two years after the procedure (P=0.01) compared with the transplanted patients without HPS. CONCLUSION: Patients with HPS had worse exercise capacity before LT, more complications and shorter survival after this procedure than patients without HPS.

RESUMO CONTEXTO: A síndrome hepatopulmonar (SHP) é uma complicação associada à cirrose que pode contribuir para piora da capacidade de exercício e menor sobrevida após o transplante hepático (TxH). OBJETIVO: Avaliar a capacidade de exercício, as complicações e a sobrevida após TxH em cirróticos com SHP e comparar com os resultados de cirróticos sem esse diagnóstico. MÉTODOS: Estudo de coorte prospectivo, composto inicialmente por 178 pacientes, dos quais 90 foram submetidos ao TxH (42 com SHP e 48 sem SHP). Foi realizada uma avaliação prévia composta pelo teste de caminhada dos seis minutos (TC6M), teste ergométrico e manovacuometria. Os submetidos ao TxH tiveram avaliados o tempo de ventilação mecânica (VM), uso de ventilação não invasiva (VNI), e a sobrevida dois anos após o procedimento. Na análise estatística utilizamos os testes de Kolmogorov-Smirnov, o teste t de Student, o teste do quadrado de associação linear, a curva de sobrevida de Kaplan Meier. Os dados foram analisados no programa SPSS 16.00 sendo considerado significativo P<0,05. RESULTADOS: O grupo SHP apresentou menor pico de consumo de oxigênio (VO2pico) (14,2±2,3 vs 17,6±2,6) P<0,001, e menor distância percorrida no TC6M (340,8±50,9 vs 416,5±91,4) P<0,001 antes do TxH. Os pacientes com SHP transplantados permaneceram mais horas em VM (19,5±4,3 vs 12,5±3,3) P=0,02, necessitaram mais de VNI (12 vs 2) P=0,01, e tiveram menor sobrevida dois anos após o procedimento (P=0,01). CONCLUSÃO: Pacientes com SHP apresentaram pior capacidade de exercício antes do TxH, mais complicações e menor sobrevida após a realização desse procedimento.

Embolie pulmonaire mimant un syndrome coronarien aigu ST+ en antéro septal chez une Guinéenne

L'objectif de cette étude était de décrire la ressemblance des manifestations cliniques de l'embolie pulmonaire à celles du syndrome coronarien aigu.Il s'agissait d'une patiente de 60 ans hypertendueadmise pour douleur thoracique d'allure angineuse,dyspnée d'effort évoluant depuis deux jours. Par ailleurs la patiente rapportait une notion de voyage routier récent de plus de 6 heures. Tension artérielle à 170/100mmhg, fréquence cardiaque à 120bpm, fréquence respiratoire à 18cycles/mn , température à370C, SaO2 à 98% à l'air ambiant. L'examen physique est sans particularité. L'électrocardiogramme inscrivait une tachycardie sinusale à 121 cycles/mn,un sus décalage du segment ST en V1, V2 et V3 puis un sous décalage en V5, V6 ,DI ,DII et AVF, une hypertrophie ventriculaire gauche. L'angioscanner thoracique objectivait une embolie pulmonaire de l'artère pulmonaire droite. L'embolie pulmonaire étant la grande simulatrice des pathologies thoraciques en général et en particulier le syndrome coronarien aigu, la vigilance du clinicien est mise à rude épreuve pour ne pas confondre ces deux pathologies qui sont toutes des urgences cardiovasculaires avec une prise en charge bien distincte. Nous mettons ici en exergue les similitudes entre l'embolie pulmonaire et le syndrome coronarien aigu

Splenic tyrosine kinase promotes pulmonary angiogenesis in rats with hepatopulmonary syndrome / 生理学报

The present paper was aimed to study the role of spleen tyrosine kinase (Syk) in angiogenesis in hepatopulmonary syndrome (HPS) and the underlying mechanism. Sprague Dawley (SD) rats were randomly divided into three groups: sham operation group (sham group), common bile duct ligation (CBDL) 5-week group (5W group) and R788 intervention group (R788 group). HPS model was established by CBDL. Rats in R788 group were intraperitoneally injected with R788 (20 mg/kg) once daily to week 5 after CBDL operation. The protein expression levels and distribution of Syk, p-Erk1/2, and p-Akt in lung tissue were detected by Western blot and immunohistochemistry. Immunofluorescence staining was used to observe the location of Syk expression and the number of angiogenesis in lung tissue. The results showed that, compared with sham group, 5W group exhibited up-regulated protein expression level of Syk, increased phosphorylation levels of Erk1/2 and Akt, and increased number of pulmonary microvessels. Compared with 5W group, R788 group exhibited down-regulated protein expression level of Syk, decreased phosphorylation levels of Erk1/2 and Akt, and decreased number of pulmonary microvessels. These results suggest that Syk may promote pulmonary angiogenesis in HPS model rats by activating downstream Erk1/2 and Akt signaling pathways, which provides a theoretical basis and potential drug therapeutic targets for the clinical treatment of HPS.

Value of Contrast Transesophageal Echocardiography in the Detection of Intrapulmonary Vascular Dilatations in Hepatosplenic Schistosomiasis / Valor do Ecocardiograma Transesofágico com Contraste no Diagnóstico da Dilatação Vascular Intrapulmonar na Esquistossomose Hepatoesplênica

Abstract Background: Hepatopulmonary syndrome (HPS), found in cirrhotic patients, has been little studied in hepatosplenic schistosomiasis (HSS) and includes the occurrence of intrapulmonary vascular dilatations (IPVD). Contrast transesophageal echocardiography (cTEE) with microbubbles is more sensitive than contrast transthoracic echocardiography (cTTE) with microbubbles in the detection of IPVD in cirrhosis. Objective: To assess the performance of the cTEE, compared with that of cTTE, in detecting IPVD for the diagnosis of HPS in patients with HSS. Methods: cTEE and cTTE for investigation of IPVD and laboratory tests were performed in 22 patients with HSS. Agitated saline solution was injected in peripheral vein during the cTEE and cTTE procedures. Late appearance of the microbubbles in the left chambers indicated the presence of IPVD. Results of the two methods were compared by the Student's t-test and the chi-square test (p < 0.05). Results: cTEE was performed in all patients without complications. Three patients were excluded due to the presence of patent foramen ovale (PFO). The presence of IPVD was confirmed in 13 (68%) of 19 patients according to the cTEE and in only six (32%, p < 0.01) according to the cTTE. No significant differences in clinical or laboratory data were found between the groups with and without IPVD, including the alveolar-arterial gradient. The diagnosis of HPS (presence of IPVD with changes in the arterial blood gas analysis) was made in five patients by the cTEE and in only one by the cTTE (p = 0.09). Conclusion: In HSS patients, cTEE was safe and superior to cTTE in detecting IPVD and allowed the exclusion of PFO.

Resumo Fundamento: A síndrome hepatopulmonar (SHP), presente em pacientes cirróticos, é pouco estudada na esquistossomose hepatoesplênica (EHE) e inclui a ocorrência de dilatações vasculares intrapulmonares (DVP). O ecocardiograma transesofágico com contraste (ETEc) de microbolhas é mais sensível que o ecocardiograma transtorácico com contraste (ETTc) de microbolhas na identificação de DVP na cirrose. Objetivo: Avaliar o desempenho do ETEc comparado ao ETTc na identificação de DVP para diagnóstico de SHP em pacientes com EHE. Métodos: Incluímos 22 pacientes com EHE submetidos a ETEc e ETTc para pesquisa de DVP, além de exames laboratoriais. Os ETEc e ETTc foram realizados empregando-se solução salina agitada, injetada em veia periférica. A visualização tardia das microbolhas em câmaras esquerdas indicava presença de DVP. Os resultados foram comparados entre os dois métodos pelos testes t de Stu dent e qui-quadrado (significância p < 0,05). Resultados: Todos os 22 pacientes realizaram ETEc sem intercorrências. Foram excluídos três pela presença de forame oval patente (FOP), e a análise final foi realizada nos outros 19. A DVP esteve presente ao ETEc em 13 pacientes (68%) e em apenas seis ao ETTc (32%, p < 0,01). Não houve diferenças significativas nos dados clínicos e laboratoriais entre os grupos com e sem DVP, incluindo a diferença alveoloarterial de oxigênio. O diagnóstico de SHP (presença de DVP com alterações gasométricas) ocorreu em cinco pacientes pelo ETEc e em apenas um pelo ETTc (p = 0,09). Conclusão: Em pacientes com EHE, o ETEc foi seguro e superior ao ETTc na detecção de DVP não identificada ao ETTc, o que possibilitou adicionalmente excluir FOP.

Melatonin effects on pulmonary tissue in the experimental model of Hepatopulmonary Syndrome / Efeitos da melatonina sobre o tecido pulmonar no modelo experimental de Síndrome Hepatopulmonar

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.

Síndrome hepatopulmonar y trasplante hepático: experiencia en el departamento de trasplantes del Hospital Nacional Guillermo Almenara Irigoyen - EsSalud / Hepatopulmonar syndrome and liver transplantation: experience in the transplantation department of the Guillermo Almenara Irigoyen National Hospital - EsSalud

Introducción: El síndrome hepatopulmonar (SHP) es una complicación grave de la enfermedad hepática, la cual se caracteriza por la presencia de vasodilatación intrapulmonar e hipoxemia progresiva, siendo el trasplante de hígado el único tratamiento efectivo. Objetivo: Mostrar nuestros resultados de los pacientes con síndrome hepatopulmonar sometidos a trasplante hepático. Materiales y métodos: Estudio retrospectivo, descriptivo y trasversal. Desde marzo del 2000 a diciembre del 2016 se realizaron 226 trasplantes de hígado. Del total, se excluyeron a 25 pacientes: 12 retrasplantes, 9 trasplantes dobles higadoriñon, 2 trasplantes con falla hepática aguda, 2 trasplantes en pacientes no cirróticos. De los 201 pacientes con diagnóstico pretrasplante de cirrosis hepática, 19 tuvieron criterios de SHP; quienes fueron distribuidos según edad, sexo, nivel de hipoxemia (pO2), score CHILD, score MELD. La reversibilidad de la hipoxemia post trasplante se midió con una cutt off de p0(2) >75 mmHg. Resultados: La prevalencia del SHP en nuestra serie fue 9,45%. La edad promedio fue 41 años (14-65); la relación M/F de 1,65. El 78,94% (15/19) fueron adultos. 89,5% (17/19) fueron score de CHILD B y C, y el 68,4% tuvieron SHP severo y muy severo. En el 94,11% de los pacientes se demostró reversibilidad del SHP. La tasa de mortalidad temprana en los pacientes con SHP fue 10,4%. Conclusiones: La prevalencia del SHP fue del 9,45%. Los pacientes trasplantados con y sin SHP tuvieron similar sobrevida.

Introduction: Hepatopulmonary syndrome (HPS) is a serious complication of liver disease, which is characterized by the presence of intrapulmonary vasodilation and progressive hypoxemia. Liver transplantation is the only effective treatment. Objective: To show our results of patients with hepatopulmonary syndrome undergoing liver transplantation. Materials and methods: Retrospective, descriptive and cross-sectional study. From March 2000 to December 2016; 226 liver transplants were performed. Of the total, 25 patients were excluded: 12 retransplantation, 9 liver-kidney combined transplants, 2 transplants for acute liver failure, 2 transplants in non-cirrhotic patients. Of the 201 patients with pretransplant diagnosis of liver cirrhosis, 19 filled criteria for SHP; who were distributed according to age, sex, hypoxemia level (pO2), Child-Pugh score and MELD score. The reversibility hypoxemia after liver trasplantation was measured with a cut-off of p0(2) >75 mmHg. Results: The prevalence of SHP in our series was 9.45%. The average age was 41 years (14-65); the M / F ratio of 1.65. The 78.94% (15/19) were adults. 89.5% (17/19) were Score of Child-Pugh B and C, and 68.4% had severe and very severe SHP. In 94.11% of patients, reversibility SHP founded. The early mortality rate (30 days) in patients with SHP was 10.4%. Conclusions: The prevalence of HPS in our series was 9.45%. Transplanted patients with and without SHP had similar survival.

Hepatopulmonary syndrome caused by hypothalamic obesity and nonalcoholic fatty liver disease after surgery for craniopharyngioma: a case report

Hypothalamic obesity is often complicated in patients with craniopharyngioma due to hypothalamic damage by the tumor itself, treatment modalities, and associated multiple pituitary hormone deficiency. Hypothalamic obesity causes secondary diseases such as nonalcoholic fatty liver disease (NAFLD) and diabetes mellitus (DM). We report a 19-year-old female who was diagnosed with craniopharyngioma, developed hypothalamic obesity after tumor resection, and progressed to hepatopulmonary syndrome. She manifested NAFLD 1 year after tumor resection. Two years later, the craniopharyngioma recurred, and she underwent a second resection. Three years after her second operation, she was diagnosed with type 2 DM, after which she did not visit the outpatient clinic for 2 years and then suddenly reappeared with a weight loss of 25.8 kg that had occurred over 21 months. One month later, she presented to the Emergency Department with dyspnea. Laboratory findings revealed liver dysfunction and hypoxia with increased alveolar artery oxygen gradient. Liver biopsy showed portal hypertension and micronodular cirrhosis. Echocardiography and a lung perfusion scan demonstrated a right to left shunt. She was finally diagnosed with hepatopulmonary syndrome and is currently awaiting a donor for liver transplantation. Patients surviving craniopharyngioma need to be followed up carefully to detect signs of hypothalamic obesity and monitored for the development of other comorbidities such as DM, NAFLD, and hepatopulmonary syndrome.

Prevalencia y severidad del síndrome hepatopulmonar en pacientes con cirrosis hepática del Hospital Nacional Cayetano Heredia, entre enero y diciembre del 2015 / Prevalence and severity of the hepatopulmonar syndrome in patients with liver cirrhosis attended at Cayetano Heredia Hospital, between January and December 2015

Introducción: El sindrome hepatopulmonar (SHP) es una complicación poco frecuente de la cirrosis hepática (CH) que disminuye considerablemente la calidad de vida de las personas que la padecen. Objetivos: Determinar la prevalencia y severidad del SHP en los pacientes con CH atendidos en el Hospital Nacional Cayetano Heredia (HCH) en el periodo comprendido entre enero a diciembre del 2015. Material y métodos: Estudio transversal, con tamaño de muestra necesario para determinar la prevalencia puntual calculado en 297 pacientes. Resultados: La prevalencia del SHP fue de 0,7% y los casos identificados se clasificaron como SHP leve y severo. Conclusión: La prevalencia del SHP es muy baja en la población de pacientes con cirrosis hepática atendidos en el Hospital Nacional Cayetano Heredia

Introduction: The hepatopulmonary syndrome (HPS) is a rare complication of liver cirrhosis (LC) which significantly diminishes the quality of life for people who suffer. Objectives: To determine the prevalence and severity of HPS in patients with CH treated at the Cayetano Heredia (HCH) Hospital in the period from January to December 2015. Materials and methods: Cross-sectional study with sample size needed to determine the point prevalence calculated in 297 patients. Results: The prevalence of HPS in 0.7% and the identified cases were classified as mild and severe SHP. Conclusion: The prevalence of HPS is very low in the population of patients with liver cirrhosis treated at the Cayetano Heredia Hospital

Ventilatory support and hospital stay after liver transplant in cirrhotic patients with hepatopulmonary syndrome / Suporte ventilatório e tempo de hospitalização após transplante hepático em cirróticos com síndrome hepatopulmonar

ABSTRACT Objective To compare mechanical ventilation time, need for non-invasive ventilation, length of intensive care unit stay, and hospital stay after liver transplant in cirrhotic patients with and with no diagnosis of hepatopulmonary syndrome. Methods This was a prospective cohort study with a convenience sample of 178 patients (92 with hepatopulmonary syndrome) who were diagnosed as alcoholic or hepatitis C virus cirrhosis. The statistical analysis included Kolmogorov-Smirnov test and Students t test. Data were analyzed using SPSS version 16.0, and p values <0.05 were considered significant. Results Out of 178 patients, 90 underwent transplant (48 with no hepatopulmonary syndrome). The Group diagnosed with Hepatopulmonary Syndrome had longer mechanical ventilation time (19.5±4.3 hours versus 12.5±3.3 hours; p=0.02), an increased need for non-invasive ventilation (12 versus 2; p=0.01), longer intensive care unit stay (6.7±2.1 days versus 4.6±1.5 days; p=0.02) and longer hospital stay (24.1±4.3 days versus 20.2±3.9 days; p=0.01). Conclusion Cirrhotic patients Group diagnosed with Hepatopulmonary Syndrome had higher mechanical ventilation time, more need of non-invasive ventilation, as well as longer intensive care unit and hospital stay.

RESUMO Objetivo Comparar tempo de ventilação mecânica, necessidade de uso de ventilação não invasiva, tempo de permanência na unidade de terapia intensiva e tempo de hospitalização após transplante hepático em cirróticos com e sem diagnóstico de síndrome hepatopulmonar. Métodos Estudo de coorte prospectiva com amostra de conveniência composta por 178 pacientes (92 com síndrome hepatopulmonar) com diagnóstico de cirrose por álcool ou pelo vírus da hepatite C. A análise estatística foi realizada por meio do teste Kolmogorov-Smirnov e do teste t de Student. Os dados foram analisados pelo programa SPSS versão 16.0, e valores de p<0,05 foram considerados significantes. Resultados Dos 178 pacientes, 90 foram transplantados (48 sem síndrome hepatopulmonar). O Grupo com Síndrome Hepatopulmonar apresentou maior tempo de ventilação mecânica (19,5±4,3 horas versus 12,5±3,3 horas; p=0,02), maior necessidade de uso de ventilação não invasiva (12 versus 2; p=0,01), maior permanência na unidade de terapia intensiva (6,7±2,1 dias versus 4,6±1,5 dias; p=0,02) e maior tempo de hospitalização (24,1±4,3 dias versus 20,2±3,9 dias; p=0,01). Conclusão O Grupo com Síndrome Hepatopulmonar apresentou maiores tempo de ventilação mecânica, necessidade de uso de ventilação não invasiva, permanência na unidade de terapia intensiva e tempo de hospitalização.

Schistosomiasis and hepatopulmonary syndrome: the role of concomitant liver cirrhosis

BACKGROUND Hepatopulmonary syndrome (HPS) is defined as an oxygenation defect induced by intrapulmonary vasodilation in patients with liver disease or portal hypertension. It is investigated in patients with liver cirrhosis and less frequently in those with portal hypertension without liver cirrhosis, as may occur in hepatosplenic schistosomiasis (HSS). OBJECTIVES To investigate the prevalence of HPS in patients with HSS, and to determine whether the occurrence of HPS is influenced by concomitant cirrhosis. METHODS We evaluated patients with HSS with or without concomitant liver cirrhosis. All patients underwent laboratory testing, ultrasound, endoscopy, contrast echocardiography, and arterial blood gas analysis. FINDINGS Of the 121 patients with HSS, 64 were also diagnosed with liver cirrhosis. HPS was diagnosed in 42 patients (35%) and was more frequent among patients with concomitant liver cirrhosis than in those without cirrhosis (42% vs. 26%), but the difference was not significant (p = 0.069). HPS was more common in those with spider naevi, Child-Pugh classes B or C and high model for end stage liver disease (MELD) scores (p < 0.05 each). MAIN CONCLUSIONS The prevalence of HPS was 35% in this study. The occurrence of liver cirrhosis concomitantly with HSS may have influenced the frequency of patients presenting with HPS.

Características clínicas y laboratoriales de pacientes hospitalizados con hipertensión portal y complicaciones vasculares pulmonares en el Instituto Nacional de Salud del Niño / Clinical and laboratory characteristics of patients with pulmonary hypertension and pulmonary vascular complications hospitalized at the Instituto Nacional de Salud del Niño

El síndrome hepatopulmonar (SHP) y la hipertensión portopulmonar (HTPP) son distintas complicaciones vasculares pulmonares de la hipertensión portal (HTP) y se asocian con una mayor morbilidad y mortalidad. Objetivos: Describir las Características Clínicas y Laboratoriales de los pacientes con HTP y complicaciones vasculares pulmonares hospitalizados en el Instituto Nacional de Salud del Niño. Materiales y métodos: se incluyeron los pacientes con HTP hospitalizados desde enero del 2012 hasta junio del 2013 y que durante su evolución cursaron con SHP o HTPP. Para el análisis se les dividió en un primer grupo de pacientes con cirrosis hepática y un segundo grupo con obstrucción extra hepática de vena porta. Resultados: De 22 pacientes con HTP el 45,5% fueron varones y el rango de edad fue entre 1 mes y 17 años. La etiología en el grupo de cirrosis (n=14) fue: hepatitis autoinmune (35,7%), cirrosis criptogénica (35,7%), error innato del metabolismo (14,3%), hepatitis viral crónica por virus C (7,15%) y atresia de vías biliares extra hepática (7,15%). Las complicaciones vasculares pulmonares, se presentaron más frecuentemente en los pacientes con cirrosis hepática (1 caso de síndrome hepatopulmonar y un caso de hipertensión portopulmonar). En ellos se encontró más frecuentemente disnea, astenia, edema, desnutrición, ascitis, hiperesplenismo y hemorragia digestiva por várices esofágicas, además de valores elevados de ALT, fosfatasa alcalina y menores niveles de albúmina sérica. Conclusiones: En niños con HTP, las complicaciones vasculares pulmonares son muy infrecuentes. En la evaluación de estos pacientes debería incluirse la oximetría de pulso para detectar hipoxemia y posteriormente, de ser necesario una ecocardiografía Doppler y de contraste. Ante el hallazgo de hipertensión sistólica pulmonar es necesario realizar un cateterismo cardiaco derecho.

The hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPHN) are distinct pulmonary vascular complications of portal hypertension (PHT) and are associated with increased morbidity and mortality. Objectives: To describe the clinical and laboratory characteristics of patients with pulmonary hypertension and pulmonary vascular complications hospitalized at the Instituto Nacional de Salud del Niño. Materials and methods: We included patients with HTP hospitalized from January 2012 to June 2013 and that during its evolution progressed with SHP or HTPP. For analysis, they were divided into a first group of patients with liver cirrhosis and a second group with extrahepatic portal vein obstruction. Results: Of 22 patients with HPT 45.5% were male and the age range was between 1 month and 17 years. The etiology in the group of cirrhosis (n=14) was: autoimmune hepatitis (35.7%), cryptogenic cirrhosis (35.7%), inborn error of metabolism (14.3%), chronic viral hepatitis C (7.15%) virus and atresia extra-hepatic bile ducts (7.15%). Pulmonary vascular complications more frequently occurred in patients with liver cirrhosis (1 case of HPS and a case of PPHTN). They most often dyspnea, asthenia, edema, malnutrition, ascites, hypersplenism and gastrointestinal bleeding from esophageal varices was found. Also, they had elevated ALT values, alkaline phosphatase and serum albumin values decreased. Conclusions: In children with pulmonary hypertension, pulmonary vascular complications are rare. In the evaluation of these patients pulse oximetry should be included to detect hypoxemia and subsequently a Doppler echocardiography and contrast echocardiography necessary. Dueto the finding of systolic pulmonary hypertension it is necessary to perform right heart catheterization.

Compromiso pulmonar en la enfermedad hepática: una actualización / Pulmonary commitment in hepatic disease: uma atualização / Compromisso pulmonar na doença hepática

La enfermedad Hepática es una causa importante de morbilidad y Mortalidad en el mundo; asociada a compromiso orgánico múltiple. En el pulmón, tres condiciones clínicas han sido descritas; estos cuadros Incluyen, el Síndrome Hepatopulmonar (SHP), que obedece a un trastorno de la oxigenación por dilatación de la vasculatura pulmonar. La hipertensión portopulmonar (HPP), mediada por desequilibrio entre agentes vasodilatadores y vasoconstrictores que conducen a un aumento de la presión media de la arteria pulmonar y por último el Hidrotórax Hepático (HH), que es la condición menos prevalente, se fundamenta principalmente en anomalías anatómicas del diafragma, con o sin relación a la presencia de ascitis. La presencia SHP o HPP es predictor independiente de mortalidad, resaltando su importancia en la elegibilidad de pacientes para trasplante hepático ortotópico como medida curativa.

Liver disease is a major cause of morbidity and mortality in the world; it is associated with multiple organ involvement. In the lung, three clinical conditions are described; these conditions include, Hepatopulmonary Syndrome (HPS), which is due to a oxygenation defect by the development of pulmonary vascular dilatation. Portopulmonary (HPP) hypertension, it's mediated by an imbalance between vasodilator and vasoconstrictor agents leading to an increase in mean pulmonary artery pressure and finally the Hydrothorax Liver (HH), that is the least prevalent condition, it's based on anatomical diaphragm abnormalities, with or without ascites. The presence of SHP or HPP is an independent predictor of mortality, highlighting its importance in the eligibility of patients for orthotopic liver transplantation as a curative measure.

A doença hepatica é uma das principais causas de morbidade e mortalidade no mundo; está associado ao compromisso de vários órgãos. No pulmão, três condições clínicas são descritas; estas condições incluem, Síndrome Hepatopulmonar (HPS), que é devido a um defeito de oxigenação do dilatação vascular pulmonar. A hipertensão portopulmonar (HPP), é mediada por um desequilíbrio entre vasodilatadores e agentes vasoconstritores, levando a um aumento da pressão arterial média da artéria pulmonar e, finalmente, ao hidrotorax hepatico (HH), essa é a condição menos prevalente, é baseada em anormalidades anatômicas do diafragma, com ou sem ascite. A presença de SHP ou HPP é um preditor independente de mortalidade, destacando sua importância na elegibilidade de pacientes para transplante de hepatico como medida curativa.

Development of a Pulmonary Arteriovenous Fistula after a Modified Glenn Shunt in Tetralogy of Fallot and Its Resolution after Shunt Takedown in a 57-Year-Old Patient

Pulmonary arteriovenous fistula (PAVF) is a complication of the Glenn shunt. A 57-year-old tetralogy of Fallot (TOF) patient, who had undergone a Glenn shunt and TOF total correction, complained of dyspnea and cyanosis. PAVFs were present in the right lung, and right lung perfusion was nearly absent. After coil embolization, takedown of the Glenn shunt, and reconstruction of the right pulmonary artery, the patient's symptoms were relieved. Extrapulmonary radioisotope uptake caused by the PAVFs shown in lung perfusion scans decreased, and right lung perfusion increased gradually. Although the development and resolution of PAVFs after a Glenn shunt have been reported in the pediatric population, this may be the first report on this change in old age.

Quercetin alleviates pulmonary angiogenesis in a rat model of hepatopulmonary syndrome

Quercetin shows protective effects against hepatopulmonary syndrome (HPS), as demonstrated in a rat model. However, whether these effects involve pulmonary vascular angiogenesis in HPS remains unclear. Therefore, this study aimed to assess the effect of quercetin on pulmonary vascular angiogenesis and explore the underlying mechanisms. Male Sprague-Dawley rats weighing 200-250 g underwent sham operation or common bile duct ligation (CBDL). Two weeks after surgery, HIF-1α and NFκB levels were assessed in rat lung tissue by immunohistochemistry and western blot. Then, CBDL and sham-operated rats were further divided into 2 subgroups each to receive intraperitoneal administration of quercetin (50 mg/kg daily) or 0.2% Tween for two weeks: Sham (Sham+Tween; n=8), CBDL (CBDL+Tween; n=8), Q (Sham+quercetin; n=8), and CBDL+Q (CBDL+quercetin; n=8). After treatment, lung tissue specimens were assessed for protein (immunohistochemistry and western blot) and/or gene expression (quantitative real-time PCR) levels of relevant disease markers, including VEGFA, VEGFR2, Akt/p-Akt, HIF-1α, vWf, and IκB/p-IκB. Finally, arterial blood was analyzed for alveolar arterial oxygen pressure gradient (AaPO2). Two weeks after CBDL, HIF-1α expression in the lung decreased, but was gradually restored at four weeks. Treatment with quercetin did not significantly alter HIF-1α levels, but did reduce AaPO2 as well as lung tissue NF-κB activity, VEGFA gene and protein levels, Akt activity, and angiogenesis. Although hypoxia is an important feature in HPS, our findings suggest that HIF-1α was not the main cause for the VEGFA increase. Interestingly, quercetin inhibited pulmonary vascular angiogenesis in rats with HPS, with involvement of Akt/NF-κB and VEGFA/VEGFR-2 pathways.

Síndrome hepatopulmonar: poco común, pero mortal / Hepatopulmonary syndrome: uncommon, but deadly

Patients with cirrhosis may present with portal hypertension (PHT), which can lead to various complications. The most common areas cites, variceal bleeding, and hepatic encephalopathy. However, there is another entity o flow prevalence but high morbidity and mortality: the hepato pulmonary syndrome (HPS). We report the case of a 25 year-old woman with cirrhosis secondary to autoimmunehepatitis. She was admitted with respiratory symptoms suggestive of viral etiology, but evolved unfavorably. Various diagnoses of cardiovascular and respiratory conditions were successively ruled out, persisting with severe hypoxemia. Concomitantly, she developed progressive pain in the left hypochondrium area, and imaging studies show edsplenic and perisplenic form of manifestation of PHT. In context fHTP and hypoxemia, the diagnosis of HPS vs portopulmonary syndrome was considered, with the bubble test echocardiogramas a key study for such dilemma.

Los pacientes con daño hepático crónico (DHC) pueden cursar con hipertensión portal (HTP), que puede generar diversas complicaciones. Las más frecuentes son la ascitis, hemorragia variceal y encefalopatía hepática. Sin embargo, existe otra entidad de baja prevalencia, pero elevada morbimortalidad: el síndrome hepatopulmonar (SHP). Reportamos el caso de una mujer de 25 años con DHC secundario a hepatitis autoinmune. Ella ingresó con un cuadro sugerente de infección respiratoria alta de etiología viral, pero evolucionó tórpidamente. Se descartaron sucesivamente diversos diagnósticos de la esfera cardiovascular y respiratoria, persistiendo con hipoxemia grave. En forma concomitante desarrolló dolor en hipocondrio izquierdo de carácter progresivo, y las imágenes evidenciaron manifestaciones de HTP de predominio esplénico y periesplénico. En contexto de HTP e hipoxemia se consideró el diagnóstico de síndrome hepatopulmonar vs síndrome porto pulmonar, siendo el ecocardiograma con test de burbujas un estudio clave para el diagnóstico definitivo.

Molecular mechanism of tumor necrosis factor-alpha monoclonal antibody in hepatopulmonary syndrome in rats / 中华肝脏病杂志

<p><b>OBJECTIVE</b>To investigate the molecular mechanism of tumor necrosis factor-alpha (TNF-a) monoclonal antibody (McAb) in hepatopulmonary syndrome using a rat model.</p><p><b>METHODS</b>Sixty adult male Sprague-Dawley rats, weighing 250 ± 25 g, were randomized to a sham operation group, a common bile duct ligation (CBDL) group, or a CBDL+TNF-alphat McAb treatment group. The CBDL operation group was further divided into five subgroups, and the CBDL+TNF-alpha McAb treatment group was further divided into four subgroups. After the experimental period, all rats were sacrificed for excision of lung and liver tissues. Hematoxylin-eosin (HE) and Masson staining were performed to observe the extent of liver fibrosis,and HE staining was used to histopathologically assess changes in the lung tissue. Immunohistochemistry and western blotting were used to investigate the changes in expression levels of FAK, p-FAK and PTEN in lung.</p><p><b>RESULTS</b>The extent of inflammatory responses and fibrosis in the liver was significantly lower in the CBDL+TNF-alpha McAb treatment group as compared to those in the CBDL group. The inflammatory responses in the lung were also significantly lower in the CBDL+TNF-alpha McAb treatment group as compared to that in the CBDL group. The CBDL+TNF-alpha McAb treatment group also showed less extensive distribution of FAK and p-FAK protein in lung tissues,but more extensive distribution of PTEN protein.</p><p><b>CONCLUSION</b>FAK and PTEN are associated with hepatopulmonary syndrome in rats. The therapeutic effect of TNF-alpha McAb may involve modulation of the expression of FAK and PTEN.</p>