Impact of intrapulmonary vascular dilatations and hepatopulmonary syndrome on the clinical course of patients after transjugular intrahepatic portosystemic shunt insertion
Jim Benjamin MauzHannah SchneiderDominik BerlinerAnja TiedeLena StockhoffJan B. HinrichsHeiner WedemeyerBernhard MeyerKaren M. OlssonBenjamin MaasoumyTammo Lambert Tergast
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Hepatopulmonary syndrome
Portosystemic shunt
Hepatopulmonary syndrome is a well described complication of chronic liver disease. Though uncommon, it carries a high morbidity and mortality. The pathogenesis of the syndrome has not been clearly defined. Portal hypertension seems to play a crucial role in the pathogenesis of the syndrome, probably by enhancing nitric oxide production. As yet, no pharmacological therapy has been proven effective. Many reports of successful reversal of the syndrome after liver transplantation have been published. We report a patient with hepatopulmonary syndrome who showed a significant and durable (4 months’) improvement in his symptoms, arterial oxygenation, and intrapulmonary shunts, as calculated by radionuclide studies after transjugular intrahepatic portosystemic shunt placement. Transjugular intrahepatic portosystemic shunt may represent a durable treatment option for patients with hepatopulmonary syndrome.
Hepatopulmonary syndrome
Portosystemic shunt
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AIM:To assess the impact of transjugular intrahepatic portosystemic shunt (TIPS) on pulmonary gas exchange and to evaluate the use of TIPS for the treatment of hepatopulmonary syndrome ( HPS ).METHODS: S e ve n p a t i e n t s , t h re e o f t h e m w i t h advanced HPS, in whom detailed pulmonary function tests were performed before and after TIPS placement at the University of Alabama Hospital and at the Hospital Clinic, Barcelona, were considered.RESulTS: TIPS patency was confirmed by hemodynamic evaluation.No changes in arterial blood gases were observed in the overall subset of patients.Transient arterial oxygenation improvement was observed in only one HPS patient, early after TIPS, but this was not sustained 4 mo later.COnCluSIOn: TIPS neither improved nor worsened pulmonary gas exchange in patients with portal hypertension.This data does not support the use of TIPS as a specific treatment for HPS.However, it does reinforce the view that TIPS can be safely performed for the treatment of other complications of portal hypertension in patients with HPS.
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Background
Patients with active systemic lupus erythematosus (SLE) are considered to have raised erythrocyte sedimentation rate (ESR) rather than raised C-reactive protein (CRP). Yet published evidence is low for this statement. DsDNA-antibodies, C3 complement, ferritin and proteinuria are also commonly applied to assess SLE.Objectives
Firstly, to assess how ESR correlates with above mentioned laboratory parameters: in general, in the presence of clinical activity and/or infection or in the absence of both. Secondly, to determine if these parameters are associated with disease flare or infections.Methods
A retrospective analysis of patients of a tertiary referral centre with SLE who underwent inpatient treatment between 2006 and 2015. Data on laboratory parameters, infection and disease flare, judged by the treating physician, were extracted. Patients were divided in four SLE groups: flare only (n=147), infection only (n=48), both (n=23), and neither infection nor flare (n=153). ESR was correlated to CRP, ferritin, proteinuria, C3-reduction and raised dsDNA-antibodies for the whole cohort and within each SLE group. Further, the association between all laboratory parameters and a) disease activity with and without infection, b) the presence of infection with and without disease activity, was tested.Results
We identified 203 SLE patients, 26 males, with a total of 371 visits. Mean age was 45.6 years (SD± 16.5 years). Table 1 (top part) shows the correlation coefficients of ESR with the other laboratory parameters. ESR correlated moderately with CRP amongst all groups (r=0.47–0.58); weakly with ferritin in the general and the flare group (r=0.26); and very weakly with C3-reduction and raised dsDNA-antibodies (r<0.2) in each group. Concerning proteinuria, the correlation was weak for all, for flaring and for silent patients (r=0.22 – 0.35), moderate for patients with both infection and activity (r=0.56). There was no correlation in infected patients (r <0.06). Table 1 (bottom part) displays the p-values for the association of parameters with disease activity or infection, respectively. ESR, reduction of C3, proteinuria and raised dsDNA-antibodies were all associated with disease activity in the whole cohort and in the non-infected group; CRP only in non-infected patients. In the infected group, raised dsDNA-antibodies and proteinuria were the only parameters showing significant relation to disease activity. ESR and CRP were significantly associated with infections when looking at all or at inactive patients, but not in active patients.Conclusions
Both, ESR and CRP are elevated in patients with SLE flare and are weakly correlated with other laboratory activity parameters. Thus, while normal CRP argues against infection elevation of ESR and CRP is not sufficient to distinguish between SLE flare and infection.Disclosure of Interest
None declaredErythrocyte sedimentation rate
Liver disease
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Hepatopulmonary syndrome
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Hepatorenal syndrome (HRS) and hepatopulmonary syndrome (HPS) are two serious complications of liver disease, causing damage not only to the liver but also to the kidneys, lungs, and heart. HRS and HPS affect the patient's circulatory and respiratory systems, with poor prognosis and high mortality in clinical practice. There is a lack of effective treatment other than liver transplantation. Transjugular intrahepatic portosystemic shunt (TIPS) is an effective tool to prolong the survival of patients with advanced liver disease and is mainly used to treat portal hypertension and ascites because it can effectively reduce portal pressure. Studies on the treatment of both of these complications with TIPS are limited and deserve further study because the therapeutic effects of TIPS have the potential to improve the prognosis of severe liver disease. This article reviews the clinical features of HRS and HPS, the consequences of these syndromes, and the potential mechanistic effects after TIPS intervention.
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Liver disease
Portosystemic shunt
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Hepatopulmonary syndrome is a well described complication of chronic liver disease. Though uncommon, it carries a high morbidity and mortality. The pathogenesis of the syndrome has not been clearly defined. Portal hypertension seems to play a crucial role in the pathogenesis of the syndrome, probably by enhancing nitric oxide production. As yet, no pharmacological therapy has been proven effective. Many reports of successful reversal of the syndrome after liver transplantation have been published. We report a patient with hepatopulmonary syndrome who showed a significant and durable (4 months') improvement in his symptoms, arterial oxygenation, and intrapulmonary shunts, as calculated by radionuclide studies after transjugular intrahepatic portosystemic shunt placement. Transjugular intrahepatic portosystemic shunt may represent a durable treatment option for patients with hepatopulmonary syndrome.
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Hepatopulmonary syndrome (HPS) is defined by the presence of the triad of liver disease, arterial hypoxemia, and intrapulmonary vascular dilatation. The clinical implication of this disorder is impairment of gas exchange. Numerous reports in the literature show that this condition is reversible with orthotopic liver transplantation (OLT). However, patients with HPS often present with PaO2 levels that are quite low. OLT with a preoperative PaO2 less than 50 mm Hg is associated with unacceptably high mortality and morbidity. We report a case of severe HPS in which a transjugular intrahepatic portosystemic shunt was successfully used to improve oxygenation, thus allowing a successful elective OLT.
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