scholarly journals Systemic Mastocytosis: A Rare Case of Increased Liver Stiffness

2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
Stefanie Adolf ◽  
Gunda Millonig ◽  
Helmut Karl Seitz ◽  
Andreas Reiter ◽  
Peter Schirmacher ◽  
...  

Assessment of liver stiffness (LS) by transient elastography (Fibroscan) has significantly improved the noninvasive diagnosis of liver fibrosis. We here report on a 55-year-old patient with drastically increased LS due to previously unknown systemic mastocytosis. The patient initially presented with increased weight loss, nocturnal pruritus, increased transaminases, bilirubinemia, and thrombocytopenia. Abdominal ultrasound showed ascites, hepatomegaly, and splenomegaly. In addition, LS was 75 kPa (IQR 0 kPa) clearly exceeding the cut-off value for F4 cirrhosis of 12.5 kPa. However, histological analysis of the liver specimen indicated liver involvement by systemic mastocytosis and excluded liver cirrhosis. An additional CT scan detected disseminated bone lesions. After three months of treatment with Midostaurin, LS slightly decreased down to 31.9 kPa (IQR 8.3 kPa). This case illustrates that diffused sinusoidal neoplastic infiltrates are a pitfall in the non-invasive diagnosis of liver cirrhosis. In conclusion, refined clinical algorithms for increased LS should also include mastocytosis in addition to inflammation, congestion, and biliary obstruction.

2015 ◽  
Vol 17 (1) ◽  
pp. 5 ◽  
Author(s):  
Adriana Bintintan ◽  
Romeo Ioan Chira ◽  
Vasile Virgil Bintintan ◽  
Georgiana Nagy ◽  
Roberta Maria Manzat-Saplacan ◽  
...  

Aims: Non-invasive methods are required to diagnose presence and grading of esophageal varices in patients with he- patic cirrhosis and in this respect we have evaluated the role of transient elastography and abdominal ultrasound parameters. Material and methods: Cirrhotic patients were prospectively evaluated by transient elastography and Doppler ultrasound for diagnosis of presence and grading of esophageal varices, the results being compared with the findings of the esophagogas- troduodenoscopy. Results: Sixty patients with hepatic cirrhosis were analysed. The parameters that reached statistical signifi- cance for diagnosis of esophageal varices were: liver stiffness (LSM) > 15 kPa, hemodynamic liver index (PVr1) ≥ 0.66, portal vascular resistance (PVR) > 17.66 and splenoportal index (SPI) > 4.77. The only parameter that reached statistical power for the diagnosis of large esophageal varices was LSM at a cut-off value of 28.8 kPa. Conclusions: Assessment of LSM in patients with liver cirrhosis can predict both the presence of esophageal varices and of large esophageal varices. The PVr1, PVR and SPI Doppler indexes can be used to diagnose the presence of esophageal varices but have no role in the prediction of large esophageal varices. Further studies are required to confirm these results and offer a stronger clinical significance.


2020 ◽  
Author(s):  
Muriel Webb ◽  
Oren Shibolet ◽  
Yoav Lurie ◽  
Yakov Maor ◽  
Helena Katchman ◽  
...  

Abstract Background: Budd-Chiari syndrome (BCS) is a rare disease defined as hepatic venous outflow obstruction at any level from the hepatic venules up to the cavo-atrial junction. Transjugular Intrahepatic Portosystemic Shunt (TIPS) is performed as a decompressive treatment in some patients.Aim: To evaluate the potential role of Transient Elastography (TE) in assessing liver stiffness in patients with primary BCS.Methods: Twenty one BCS patients and 10 patients with liver cirrhosis with different underlying etiologies underwent abdominal ultrasound and TE.Results: Ninety-five percent of BCS patients had liver stiffness compatible with F4 with a median of 21 kPa, values which are usually obtained in patients with liver cirrhosis. Ten BCS and 10 cirrhotic patients underwent repeated TE with a median of 320 days between exams for BCS and 4.5 years for cirrhotic patients. The change of liver stiffness in BCS patients was 5.75 kPa (range − 0.4 to 26.6), compared with − 4.85 kPa (range − 15.6 to 15.0) in cirrhotic patients (p-value = 0.0029). Change in liver stiffness from baseline to follow-up in BCS patients who underwent TIPS (n = 4) was 0.2 kPa (range − 0.4 to 15.3), whereas in patients without intervention (n = 6) it was 6.75 kPa (range 1.3 to 26.6). The difference was not statistically significant.Conclusion: Liver stiffness in BCS patients is a dynamic progressive process with parameters of TE resembling liver cirrhosis. Even if TIPS seem to slow down the increment of liver stiffness, because of decreased liver congestion, it kept most patients with high score. The TE in BCS patients may be considered for monitoring for stable or upfront disease deterioration.


2014 ◽  
Vol 155 (7) ◽  
pp. 270-276 ◽  
Author(s):  
Gabriella Pár ◽  
Andrea Trosits ◽  
Ferenc Pakodi ◽  
Imre Szabó ◽  
József Czimmer ◽  
...  

Introduction: One of the most serious complications of liver cirrhosis is variceal bleeding. Early recognition of the oesophageal varices is of primary importance in the prevention of variceal bleeding. Endoscopy is the only means to directly visualize varices and measure their size, as one of the most important predictor of the risk of bleeding. During the course of cirrhosis repeated oesophago-gastro-bulboscopic examinations are recommended. As these interventions are expensive and often poorly accepted by patients who may refuse further follow-up, there is a need for non-invasive methods to predict the progression of portal hypertension as well as the presence and the size of oesophageal varices. After several combinations of biological and ultrasonographical parameters proposed for the detection of advanced fibrosis, it was suggested that liver stiffness measured by transient elastography, a novel non-invasive technology may reflect not only fibrosis and portal pressure but it may even predict the presence or absence of large oesophageal varices in patients with cirrhosis. Aim: The aim of the authors was to study the diagnostic accuracy of transient elastography using FibroScan for selecting patients who are at risk of bearing large (Paquet-grade ≥ II) oesophageal varices and high risk of bleeding. Method: The authors performed upper tract endoscopy and transient elastography in 74 patients with chronic liver disease (27 patients with chronic hepatitis and 47 patients with liver cirrhosis). The relationships between the presence of oesophageal varices (Paquet-grade 0–IV) and liver stiffness (kPa), as well as the hematological and biochemical laboratory parameters (prothrombine international normalized ratio, platelet count, aspartate aminotransferase, alanine aminotransferase, albumin, and aspartate aminotransferase/platelet ratio index) were investigated. The predictive role of liver stiffness for screening patients with varices and those who are at high risk of variceal bleeding was also analysed. Results: Liver stiffness values significantly correlated with the grade of oesophageal varices (Paquet-grade) (r = 0.67, p<0.0001). The liver stiffness value of 19.2 kPa was highly predictive for the presence of oesophageal varices (AUROC: 0.885, 95% CI: 0.81–0.96) and for the presence of high grade varices (P≥II) (AUROC: 0.850, 95% CI: 0.754–0.94). Using the cut-off value of 19.2 kPa, the sensitivity of transient elastography was 85%, specificity was 87%, positive predictive value was 85%, negative predictive value was 87% and validity was 86% for the detection of varices. Liver stiffness values less than 19.2 kPa were highly predicitive for the absence of large (P≥II) varices (sensitivity, 95%; specificity, 70%; positive predictive value, 54%; negative predictive value, 97%). Conclusions: Transient elastography may help to screen patients who are at high risk of bearing large (P≥II) oesophageal varices which predict variceal bleeding and, therefore, need endoscopic screening. Lives stiffness values higher than 19.2 kPa indicate the need for oesophageal-gastro-bulboscopy, while liver stiffness values lower than 19.2 kPa make the presence of large oesophageal varices unlikely. Orv. Hetil., 2014, 155(7), 270–276.


2021 ◽  
Vol 75 (2) ◽  
pp. 125-133
Author(s):  
Soňa Franková ◽  
Jan Šperl

Portal hypertension represents a wide spectrum of complications of chronic liver diseases and may present by ascites, oesophageal varices, splenomegaly, hypersplenism, hepatorenal and hepatopulmonary syndrome or portopulmonary hypertension. Portal hypertension and its severity predicts the patient‘s prognosis: as an invasive technique, the portosystemic gradient (HPVG – hepatic venous pressure gradient) measurement by hepatic veins catheterisation has remained the gold standard of its assessment. A reliable, non-invasive method to assess the severity of portal hypertension is of paramount importance; the patients with clinically significant portal hypertension have a high risk of variceal bleeding and higher mortality. Recently, non-invasive methods enabling the assessment of liver stiffness have been introduced into clinical practice in hepatology. Not only may these methods substitute for liver biopsy, but they may also be used to assess the degree of liver fibrosis and predict the severity of portal hypertension. Nowadays, we can use the quantitative elastography (transient elastography, point shear-wave elastrography, 2D-shear-wave elastography) or magnetic resonance imaging. We may also assess the severity of portal hypertension based on the non-invasive markers of liver fibrosis (i.e. ELF test) or estimate clinically signifi cant portal hypertension using composite scores (LSPS – liver spleen stiff ness score), based on liver stiffness value, spleen diameter and platelet count. Spleen stiffness measurement is a new method that needs further prospective studies. The review describes current possibilities of the non-invasive assessment of portal hypertension and its severity.


QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Nadia Abdelaaty Abdelkader ◽  
Amira Mahmoud AlBalakosy ◽  
Ahmed Fouad Helmy Sherief ◽  
Mohamed Soliman Gado

Abstract Background Hepatitis C virus (HCV) infection affects approximately 170 million people worldwide, causing liver cirrhosis and hepatocellular carcinoma (HCC) and leading to liver transplantation and ultimately death. Accurate evaluation of liver fibrosis in patients with chronic liver diseases is crucial, as liver fibrosis is important in order to make therapeutic decisions, determine prognosis of liver disease and to follow-up disease progression. Multiple non-invasive methods have been used successfully in the prediction of fibrosis; however, early changes in noninvasive biomarkers of hepatic fibrosis under effective antiviral therapy are widely unknown. The aim of this study is to evaluate changes of transient elastography values as well as FIB-4 and AST to platelet ratio index (APRI) in patients treated with DAAs. Objectives The aim beyond this study is to evaluate the changes in liver stiffness in hepatitis C Egyptian patients before and at least one year after treatment with DAAs using transient elastography and non-invasive liver fibrosis indices as FIB-4 and APRI scores. Patients and methods The present study was conducted on 100 patients with chronic hepatitis C patients attended to Ain Shams University Hospitals, Viral hepatitis treatment unit between October 2017 and December 2018, who were followed-up during treatment and after treatment for at least one year (retrospective and prospective study). Total number of cases during the study period was 117 patients. 17 patients were excluded from the study due to missed follow-up. Eventually, 100 patients were enrolled in the study fulfilling the inclusion criteria. Results The mean age of our patients is 47.9 years with Male predominance (52 males and 48 females). There was a significant improvement of, platelets counts, ALT and AST levels, which in turn cause significant improvement in FIB-4 and APRI scores. There was a significant improvement of liver stiffness after end of treatment, regardless of the DAA regimen used, as evidenced by Fibroscan. Conclusion Fibrosis regression –assessed by non-invasive markers of fibrosis is achievable upon removal of the causative agent.


2021 ◽  
pp. 43-47
Author(s):  
Veenit Kumar Prasad ◽  
Bapilal Bala ◽  
Biswadev Basumazumder ◽  
Achintya Narayan Ray

INTRODUCTION: Alcoholic liver disease is one of the major causes of premature deaths worldwide. Alcohol induced liver injury is the most prevalent cause of liver disease and effects 10% to 20% of population worldwide. Alcoholic liver disease comprises a wide spectrum of pathological changes ranging from steatosis, alcoholicsteato-hepatitis, Cirrhosis and nally hepatocellular carcinoma. Our aims in this study are to detect this change by non invasive method by liver broscan and its clinical implications. MATERIALS AND METHODS: Total 200 patients were taken for observational study, conducted at Coochbehar Government Medical college and hospital both outpatient department and indoor patients from May 2019 to January 2020. Liver stiffness was assessed by ultrasound based method of transient elastography using Fibroscan machine. Gradation of liver stiffness was expressed in kilopascals (KPa). RESULTS: Maximum number of patients of alcoholic liver disease were between 40 - 49 years of age (42.5%). Male patients is 87.5% and female patients 12.5%. distribution of Rural population is 36 % and Urban population is 64%. Majority of population85 patients (42.5%) had fatty liver and 40 patients (20%) have hepatomegaly, 41 patients (20.5%) had Coarse echotexture of liver parenchyma and 54 patients (27%) had Splenomegaly, 62 patients (31%) had Nodular liver and 62 patients. It is observed that 11 patients (5.5%) had Fibroscan score ≤7.5 and 47 patients (23.5%) had broscan score 7.6 -9.9 and 40 patients (20%) had broscan score 10-12.4, 36 patients (18 %) had broscan score 12.5 – 14.6 and 66 patients (33%) have broscan score ≥ 14.7. CONCLUSIONS: Transient Elastography (TE) is a newer non invasive assessment technique to detect the progression of brosis or brosis in alcoholic liver disease patient. Major advantage is it is noninvasive (costeffective) so that we can early detect progression of this cirrhosis and can give efforts to halt the disease progression.


2020 ◽  
pp. 1-2
Author(s):  
Revathy Marimuthu Shanmugam ◽  
Vinay C ◽  
Sathya Gopalasamy ◽  
Chitra Shanmugam

BACKGROUND: Many noninvasive surrogate marker for Portal hypertension or for the presence or grade of esophageal varices were studied..Splenomegaly along with splenic congestion secondary to splenic hyperdynamic circulation is seen secondary to Portal hypertension in cirrhotic patients that can be quantified by elastography. AIM:The aim of this study was to investigate whether spleen stiffness, assessed by TE, useful tool for grading chronic liver diseases and to compare its performance in predicting the presence and size of esophageal varices in liver cirrhosis patients. METHODOLOGY:86 patients with cirrhosis and 80 controls underwent transient elastography of liver and spleen for the assessment of liver stiffness (LSM) and spleen stiffness (SSM) . Upper GI endoscopy done in all Cirrhotic patients. RESULTS: Spleen stiffness showed higher values in liver cirrhosis patients as compared with controls: 58.2 kpa vs14.8 kpa (P < 0.0001) and also found to be significantly higher in cirrhotic patients compared with varices and those without varices (69.01 vs 42.05 kpa, P < 0.0001). Liver stiffness was also found to be higher in cirrhotic patients with varices when compared to patients without varices (38.5vs 21.2 kpa). Using both liver and spleen stiffness measurement we can predicted the presence of esophageal varices correctly. CONCLUSION: Spleen stiffness can be assessed using transient elastography, higher value correlated well with liver cirrhosis and presence of esophageal varices although it couldn’t correlate with grade of Esophageal Varix. Combined assessment of spleen and liver stiffness had better prediction of presence of Esophageal Varix.


Author(s):  
Naglaa El-Toukhy Ramadan El-Toukhy ◽  
Sharaf Elsayed Ali Hassanien ◽  
Ramy A. Metwaly ◽  
Medhat A. Khalil ◽  
Badawy A. Abdulaziz

Background and Study Aims: Portal hypertension is one of the most important complications of liver cirrhosis. The prevalence of varices among cirrhotic patients is variable. Therefore, endoscopic screening of all patients with liver cirrhosis would result in a large number of unnecessary additional burdens to endoscopic units. Our aim was to assess the diagnostic accuracy of spleen stiffness measured by transient elastography (Fibroscan) for prediction of the presence of varices in patients with hepatitis C related cirrhosis. Patients and Methods: The study was carried out on 100 patients with HCV-induced cirrhosis and were divided into 2 groups according to presence or absence of varices by Esophago-gastro-duodenoscopy: Group I: patients with HCV-induced cirrhosis with varices; Group II: patients with HCV-induced cirrhosis without varices. Clinical and laboratory parameters, andominal ultrasonography, Upper gastrointestinal endoscopy and transient elastography to assess the liver and spleen stiffness were carried out to all studied persons. Results: Spleen stiffness had significant diagnostic value to differentiate between cirrhotic patients with varices and cirrhotic patients without varices , it had significant diagnostic value in presence of esophageal varices at cut-off (≥46.4 K Pascal) the sensitivity for detection of esophageal varices was 93%, specificity 100%, positive predictive value (PPV) was 80%, negative predictive value (NPV) was 100%; accuracy was 95% and area under the curve was 0.98 denoting that spleen stiffness is a good predictor of esophageal varices. Conclusion: Spleen stiffness was considered as an excellent predictor of esophageal varices and better than liver stiffness in prediction of esophageal varices presence and had significant diagnostic value to differentiate between the patients with varices and patients without varices at cut off (≥46.4 K Pascal) and it may have a role in variceal grading.


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