Advances in Diagnostic Imaging of Hepatopulmonary Syndrome

Hepatopulmonary syndrome (HPS) is a serious pulmonary complication of progressive liver disease that leads to a poor clinical prognosis. Patients with HPS may develop acute respiratory failure, which requires intensive care and therapy. At present, the only effective treatment is liver transplantation; therefore, early diagnosis and timely treatment are of considerable significance. The three main features of HPS are liver disease, oxygenation disorder, and intrapulmonary vascular dilatation (IPVD). Diagnosing HPS is challenging due to the difficulty in detecting the presence or absence of IPVD. As such, imaging examination is very important for detecting IPVD. This paper reviews the imaging methods for diagnosing HPS such as ultrasound, dynamic pulmonary perfusion imaging, pulmonary angiography, and computed tomography.

Download Full-text

Hepatopulmonary syndrome as the first and only manifestation of cirrhosis in a patient with hypopituitarism

BMJ Case Reports ◽

10.1136/bcr-2021-244805 ◽

2021 ◽

Vol 14 (9) ◽

pp. e244805

Author(s):

Eman Alabsawy ◽

Yassen Serry ◽

Sreelakshmi Kotha ◽

Philip Berry ◽

Giovanni Tritto

Keyword(s):

Liver Disease ◽

Fatty Liver Disease ◽

Hepatopulmonary Syndrome ◽

Progressive Dyspnoea ◽

Alcoholic Fatty Liver ◽

End Stage Liver Disease ◽

Pituitary Dysfunction ◽

Vascular Dilatation ◽

End Stage ◽

Alcoholic Fatty Liver Disease

Hepatopulmonary syndrome (HPS) is characterised by the development of intrapulmonary arteriovenous blood shunts and vascular dilatation with consequent hypoxaemia, usually in the context of end-stage liver disease (ESLD). The estimated incidence of HPS in ESLD has been reported to be 13%–47%. Chronic liver disease has been described in patients with hypothalamic–pituitary dysfunction, mainly in the form of non-alcoholic fatty liver disease due to metabolic syndrome, with occasional progression to cirrhosis. We report a challenging case of a 27-year-old man with a background of hypopituitarism with no known liver disease who presented with progressive dyspnoea and hypoxaemia and was eventually diagnosed with severe HPS.

Download Full-text

Hepatopulmonary Syndrome: A Brief Review

Romanian Journal of Internal Medicine ◽

10.1515/rjim-2016-0015 ◽

2016 ◽

Vol 54 (2) ◽

pp. 93-97 ◽

Cited By ~ 2

Author(s):

Zulkifli Amin ◽

Hilman Zulkifli Amin ◽

Nadim Marchian Tedyanto

Keyword(s):

Quality Of Life ◽

Portal Hypertension ◽

Liver Disease ◽

Survival Rate ◽

Lung Transplantation ◽

Poor Prognosis ◽

Pulmonary Complication ◽

Hepatopulmonary Syndrome ◽

Arteriovenous Shunts

AbstractHepatopulmonary syndrome (HPS) is a pulmonary complication of liver disease characterized by arterial hypoxemia. Mechanisms related to this event are diffusion-perfusion flaw, ventilation-perfusion (V/Q) mismatch, and direct arteriovenous shunts. Diagnosis of HPS is based on the presence of liver disease or portal hypertension, increased alveolar-arterial (A-a) PO2, and intrapulmonary vascular dilatations (IPVD). Lung transplantation (LT) remains the most effective therapy for HPS. In spite of its poor prognosis, we could improve the quality of life and survival rate of patients.

Download Full-text

A RARE CASE OF CYANOSIS AND CLUBBING –LIVER INDUCED VASCULAR DISORDER OF LUNG

Journal of Health and Allied Sciences NU ◽

10.1055/s-0040-1703788 ◽

2014 ◽

Vol 04 (02) ◽

pp. 149-151

Author(s):

Pothukuchi Venkata Krishna ◽

Manasa Manne ◽

Venkata Ravikumar Chepuri

Keyword(s):

Liver Disease ◽

Poor Prognosis ◽

Rare Case ◽

Rare Complication ◽

Hepatopulmonary Syndrome ◽

Liver Disorder ◽

Cyanotic Congenital Heart Disease ◽

Chronic Liver ◽

Vascular Disorder ◽

Vascular Dilatation

Abstract:The hepatopulmonary syndrome (HPS) is under recognized complication of chronic liver disease. Hepatopulmonary syndrome has three components: liver disease, pulmonary vascular dilatation, and a defect in oxygenation. If hypoxemia and dyspnea develop in these patients in the absence of known intrinsic cardiopulmonary disorder, the hepatopulmonary syndrome must be considered. Clinical features include digital clubbing, cyanosis, spider neavi. It is a rare complication of liver disease of varied etiology and indicates a poor prognosis. We are reporting a case of severe clubbing of fingers associated with severe cyanosis and chronic liver disorder in a very young girl to highlight that other causes also should be thought of in addition to cyanotic congenital heart disease in such a young age.

Download Full-text

Semi-Quantitative Ultrasonographic Evaluation of NAFLD

Current Pharmaceutical Design ◽

10.2174/1381612826666200417142444 ◽

2020 ◽

Vol 26 (32) ◽

pp. 3915-3927 ◽

Cited By ~ 2

Author(s):

Stefano Ballestri ◽

Claudio Tana ◽

Maria Di Girolamo ◽

Maria Cristina Fontana ◽

Mariano Capitelli ◽

...

Keyword(s):

Liver Disease ◽

Fatty Liver ◽

Imaging Techniques ◽

Metabolic Complications ◽

Nonalcoholic Fatty Liver ◽

Non Invasive ◽

Increased Risk ◽

Risk Of Progression ◽

Progressive Liver Disease

: Nonalcoholic fatty liver disease (NAFLD) embraces histopathological entities ranging from the relatively benign simple steatosis to the progressive form nonalcoholic steatohepatitis (NASH), which is associated with fibrosis and an increased risk of progression to cirrhosis and hepatocellular carcinoma. NAFLD is the most common liver disease and is associated with extrahepatic comorbidities including a major cardiovascular disease burden. : The non-invasive diagnosis of NAFLD and the identification of subjects at risk of progressive liver disease and cardio-metabolic complications are key in implementing personalized treatment schedules and follow-up strategies. : In this review, we highlight the potential role of ultrasound semiquantitative scores for detecting and assessing steatosis severity, progression of NAFLD, and cardio-metabolic risk. : Ultrasonographic scores of fatty liver severity act as sensors of cardio-metabolic health and may assist in selecting patients to submit to second-line non-invasive imaging techniques and/or liver biopsy.

Download Full-text

TYPE III PRO-COLLAGEN PEPTIDE IN LIVER DISEASE IN HAEMOPHILIA

10.1055/s-0038-1644022 ◽

1987 ◽

Author(s):

R S Evely ◽

F E Preston ◽

D R Triger ◽

C R M Hay ◽

M C Greves ◽

...

Keyword(s):

Liver Disease ◽

Liver Biopsy ◽

Statistical Significance ◽

Type Iii ◽

Amino Terminal ◽

Collagen Peptide ◽

Collagen Iii ◽

Liver Biopsies ◽

Progressive Liver Disease ◽

Valuable Predictor

During the past 10 years we have carried out liver biopsies on haemophiliacs with biochemical evidence of chronic liver disease (CLD). To date 44 biopsies have been obtained from 35 patients. Histological diagnoses are Chronic Persistent Hepatitis (CPH) 24, Chronic Aggressive Hepatitis (CAH) 11 and Cirrhosis 9. Serial biopsies indicate that progressive liver disease is now a serious problem in haemophilia. Liver biopsy is not without risk and therefore it is important to identify factors which may be of value in predicting the nature of the liver disease or its progression. Since intra-hepatic fibrosis is a feature of CLD we measured Type III amino terminal propeptide of pro-collagen (PC III) by radio-immunoassay on samples taken within a mean of 4.8 months of the liver biopsy. A normal range was established as 4.3 - 15.7ng/ml on healthy subjects (median 7.0). Median values and ranges for patients with CPH (N=13), CAH (N=5) and cirrhosis (N=5) were 8 (5.4 - 23.4), 14.2 (7.2 - 19.8) and 14.2 (11.2 - 23.0)ng/ml respectively. Although pro-collagen III values tended to be higher in progressive liver disease (CAH and cirrhosis) this did not reach statistical significance. It would, therefore, appear that unlike serum IgG, pro-collagen III will not be a valuable predictor of progressive liver disease in haemophilia. A larger study is necessary to clarify this.

Download Full-text

SENP1-mediated deSUMOylation of JAK2 regulates its kinase activity and platinum drug resistance

Cell Death and Disease ◽

10.1038/s41419-021-03635-6 ◽

2021 ◽

Vol 12 (4) ◽

Author(s):

Jing Li ◽

Ruiqin Wu ◽

Mingo M. H. Yung ◽

Jing Sun ◽

Zhuqing Li ◽

...

Keyword(s):

Ovarian Cancer ◽

Drug Resistance ◽

Kinase Activity ◽

Regulatory Mechanism ◽

Platinum Resistance ◽

Platinum Drug ◽

Clinical Prognosis ◽

Platinum Resistant ◽

Poor Clinical Prognosis ◽

Stat Pathway

AbstractThe JAK2/STAT pathway is hyperactivated in many cancers, and such hyperactivation is associated with a poor clinical prognosis and drug resistance. The mechanism regulating JAK2 activity is complex. Although translocation of JAK2 between nucleus and cytoplasm is an important regulatory mechanism, how JAK2 translocation is regulated and what is the physiological function of this translocation remain largely unknown. Here, we found that protease SENP1 directly interacts with and deSUMOylates JAK2, and the deSUMOylation of JAK2 leads to its accumulation at cytoplasm, where JAK2 is activated. Significantly, this novel SENP1/JAK2 axis is activated in platinum-resistant ovarian cancer in a manner dependent on a transcription factor RUNX2 and activated RUNX2/SENP1/JAK2 is critical for platinum-resistance in ovarian cancer. To explore the application of anti-SENP1/JAK2 for treatment of platinum-resistant ovarian cancer, we found SENP1 deficiency or treatment by SENP1 inhibitor Momordin Ic significantly overcomes platinum-resistance of ovarian cancer. Thus, this study not only identifies a novel mechanism regulating JAK2 activity, but also provides with a potential approach to treat platinum-resistant ovarian cancer by targeting SENP1/JAK2 pathway.

Download Full-text

CD276 is an important player in macrophage recruitment into the tumor and an upstream regulator for PAI-1

Scientific Reports ◽

10.1038/s41598-021-94360-9 ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Sibel Durlanik ◽

Katrin Fundel-Clemens ◽

Coralie Viollet ◽

Heinrich J. Huber ◽

Martin Lenter ◽

...

Keyword(s):

Clinical Prognosis ◽

Rigorous Evaluation ◽

Macrophage Recruitment ◽

Upstream Regulator ◽

Lack Of Information ◽

Important Player ◽

Inhibitory Signaling ◽

Support Tumor Growth ◽

Poor Clinical Prognosis ◽

Pai 1

AbstractMore than 70% of colorectal, prostate, ovarian, pancreatic and breast cancer specimens show expression of CD276 (B7–H3), a potential immune checkpoint family member. Several studies have shown that high CD276 expression in cancer cells correlates with a poor clinical prognosis. This has been associated with the presence of lower tumor infiltrating leukocytes. Among those, tumor-associated macrophages can comprise up to 50% of the tumor mass and are thought to support tumor growth through various mechanisms. However, a lack of information on CD276 function and interaction partner(s) impedes rigorous evaluation of CD276 as a therapeutic target in oncology. Therefore, we aimed to understand the relevance of CD276 in tumor-macrophage interaction by employing a 3D spheroid coculture system with human cells. Our data show a role for tumor-expressed CD276 on the macrophage recruitment into the tumor spheroid, and also in regulation of the extracellular matrix modulator PAI-1. Furthermore, our experiments focusing on macrophage-expressed CD276 suggest that the antibody-dependent CD276 engagement triggers predominantly inhibitory signaling networks in human macrophages.

Download Full-text

Suppression of tumor-associated neutrophils by lorlatinib attenuates pancreatic cancer growth and improves treatment with immune checkpoint blockade

Nature Communications ◽

10.1038/s41467-021-23731-7 ◽

2021 ◽

Vol 12 (1) ◽

Author(s):

Sebastian R. Nielsen ◽

Jan E. Strøbech ◽

Edward R. Horton ◽

Rene Jackstadt ◽

Anu Laitala ◽

...

Keyword(s):

Cd8 T Cells ◽

Immune Cell ◽

Immune Checkpoint Blockade ◽

Ductal Adenocarcinoma ◽

Cancer Growth ◽

Cancer Therapies ◽

Cell Type ◽

Clinical Prognosis ◽

Tumor Associated Neutrophils ◽

Poor Clinical Prognosis

AbstractPancreatic ductal adenocarcinoma (PDAC) patients have a 5-year survival rate of only 8% largely due to late diagnosis and insufficient therapeutic options. Neutrophils are among the most abundant immune cell type within the PDAC tumor microenvironment (TME), and are associated with a poor clinical prognosis. However, despite recent advances in understanding neutrophil biology in cancer, therapies targeting tumor-associated neutrophils are lacking. Here, we demonstrate, using pre-clinical mouse models of PDAC, that lorlatinib attenuates PDAC progression by suppressing neutrophil development and mobilization, and by modulating tumor-promoting neutrophil functions within the TME. When combined, lorlatinib also improves the response to anti-PD-1 blockade resulting in more activated CD8 + T cells in PDAC tumors. In summary, this study identifies an effect of lorlatinib in modulating tumor-associated neutrophils, and demonstrates the potential of lorlatinib to treat PDAC.

Download Full-text