Hepatobiliary Involvement Research Articles

Advanced cystic fibrosis liver disease: Endovascular, endoscopic, radiologic, and surgical considerations.

Up to 90% of people with CF (pwCF) will have some form of hepatobiliary involvement. This manuscript aims to explore the different endovascular, endoscopic, radiological and surgical procedures available to diagnose and manage the most severe form of CF hepatobiliary involvement (CFHBI) known as advanced cystic fibrosis liver disease (aCFLD),seen in 10% of pwCF. These procedures and interventions include liver biopsy, hepatic venous pressure gradient measurement, gastrostomy tube placement to optimize nutrition, paracentesis, endoscopic variceal control of bleeding and portosystemic shunting before liver transplantation. By utilizing advanced diagnostic or surgical techniques, healthcare professionals of pwCF can more effectively manage patients with CFHBI and aCFLD and potentially improve patient outcomes.

Early detection of hepatobiliary involvement in cystic fibrosis: Biomarkers, radiologic methods, and genetic influences.

Cystic fibrosis-related hepatobiliary involvement (CFHBI) is a term used to describe a spectrum of hepatobiliary involvement ranging from a transient elevation of transaminase levels to advanced cystic fibrosis-associated liver disease (aCFLD). While CFHBI is common among people with cystic fibrosis (PwCF), aCFLD is rare impacting only approximately 5%-10% of the CF population. After respiratory/cardiorespiratory issues and transplant-related complications, aCFLD is now the 4th leading cause of mortality among PwCF. Additionally, aCFLD is an independent predictor of all-cause mortality and is associated with significant morbidity. Despite this recognition, our ability to predict those patients at greatest risk for aCFLD, identify early aCFLD, and monitor the incremental progression of CFHBI is lacking. Here, we review the strengths and weaknesses of the common biomarkers and imaging modalities used in the evaluation and monitoring of CFHBI, as well as the current understanding of genetic modifiers related to aCFLD.

Cystic fibrosis hepatobiliary involvement: an update on imaging in diagnosis and monitoring.

Analysis of the liver using imaging for persons with cystic fibrosis (CF) continues to evolve as new medical therapies are developed improving and extending life. In the 2010s, therapies targeted at modulating protein folding became available to those with CF. Therapeutic options have continued to expand, now providing both correction of protein folding and stabilization for most gene mutations that code for the CF transmembrane receptor protein (CFTR). Today, approximately 80% of persons with CF are eligible for highly effective modulator therapy. With these advancements, the impact of CF on the liver has become more complex, adding metabolism of CFTR modulators to intrinsic CF hepatobiliary involvement (CFHBI) and adding not previously appreciated vascular changes within the liver due to increased longevity in persons with CF. A combination of serum biomarkers and imaging is needed to add clarity to the diagnosis and monitoring of the severity of liver disease. A substantial portion of persons with CF will develop at least CFHBI and a subset will develop advanced cystic fibrosis-associated liver disease (aCFLD); therefore, diagnosis and monitoring need to begin in childhood. In this review, we cover the use of and need for imaging, including elastography, ultrasound, and magnetic resonance imaging (MRI), in diagnosing and monitoring CFHBI and its associated complications.

Whole-exome sequencing for genetic diagnosis of idiopathic liver injury in children.

Genome-wide approaches, such as whole-exome sequencing (WES), are widely used to decipher the genetic mechanisms underlying inter-individual variability in disease susceptibility. We aimed to dissect inborn monogenic determinants of idiopathic liver injury in otherwise healthy children. We thus performed WES for 20 patients presented with paediatric-onset recurrent elevated transaminases (rELT) or acute liver failure (ALF) of unknown aetiology. A stringent variant screening was undertaken on a manually-curated panel of 380 genes predisposing to inherited human diseases with hepatobiliary involvement in the OMIM database. We identified rare nonsynonymous variants in nine genes in six patients (five rELT and one ALF). We next performed a case-level evaluation to assess the causal concordance between the gene mutated and clinical symptoms of the affected patient. A genetic diagnosis was confirmed in four rELT patients (40%), among whom two carried novel mutations in ACOX2 or PYGL, and two had previously-reported morbid variants in ABCB4 or PHKA2. We also detected rare variants with uncertain clinical significance in CDAN1, JAG1, PCK2, SLC27A5 or VPS33B in rELT or ALF patients. In conclusion, implementation of WES improves diagnostic yield and enables precision management in paediatric cases of liver injury with unknown aetiology, in particular recurrent hypertransaminasemia.

Spectrum of complications in blood culture positive enteric  fever in children aged 1-15 years: A 10-year-experience from a tertiary care centre in Eastern India

Background: Enteric fever is caused by Salmonella enterica serovar typhi and Paratyphi A, B and C. Blood culture is the gold standard for diagnosis, but is not always feasible due to logistical issues. This may delay definite diagnosis and treatment resulting in increased complications.Objectives: To document the spectrum of complications involving different organ systems in blood-culture positive enteric cases Method: This was a single centre retrospective observational study conducted in the Institute of Child Health (ICH), Kolkata, India. The study period was from January 2013 to December 2022. Children aged 6 months to 15 years, admitted in ICH, with a history of fever of any duration, whose blood culture revealed Salmonella species were included. Clinical symptoms, complications and the outcome of treatment were noted. Data were statistically analysed using SPSS software.Results: Around one third of the total study population presented with complications. Age group of 5-10 years was the most vulnerable for the infection and its complications. Central nervous system (CNS) involvement, hepato-biliary involvement and gastrointestinal (GI) involvement were the common complications.Conclusions: Enteric fever involved a broad spectrum of complications involving the CNS, hepato-biliary system and the GI system.

Liver Metastasis in Advanced Ovarian Cancer after Chemotherapy: A Case Report

Ovarian cancer is the most lethal disease among gynecologic malignancies; about 1/4 of ovarian cancer patients are in stage IV when diagnosed. Based on the data of Globocan, there were 295,414 newly diagnosed ovarian cancer patients and 184,799 deaths registered in 2018. For ovarian epithelial cancer patients with stage IV disease, the mean overall survival (OS) is around 20 months, and the 5 year-OS is about 20%. About 1 in 5 patients with advanced-stage ovarian cancer have hepatobiliary involvement. The study on HGSOC aims to understand the molecular and genetic features, diagnosis, and prognosis of HGSOC, as well as its response to therapy. The method involves analyzing the genetic traits, chromosomal instability, and dysregulation of signaling pathways associated with HGSOC, as well as the detection of stemness and EMT levels in response to chemotherapy and radiation therapy. The results highlight the unique characteristics of HGSOC, such as nearly universal mutation in and dysfunction of p53, genomic instability, and the role of cancer stem cells and EMT in chemo- and radiation resistance. The implication of the study is the need for a better understanding of the molecular basis of HGSOC for improved diagnosis, prognosis, and treatment strategies.

Towards a Standardized Classification of the Hepatobiliary Manifestations in Cystic Fibrosis (CFHBI): A Joint ESPGHAN/NASPGHAN Position Paper.

The broad spectrum of hepatobiliary involvement in cystic fibrosis (CF) has been commonly referred to as cystic fibrosis liver disease (CFLD). However, differences in the definitions of CFLD have led to variations in reported prevalence, incidence rates, and standardized recommendations for diagnosis and therapies. Harmonizing the description of the spectrum of hepatobiliary involvement in all people with CF (pwCF) is deemed essential for providing a reliable account of the natural history, which in turn supports the development of meaningful clinical outcomes in patient care and research. Recognizing this necessity, The European Society for Paediatric Gastroenterology Hepatology and Nutrition (ESPGHAN) and the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) commissioned and tasked a committee to develop and propose a systematic classification of the CF hepatobiliary manifestations to increase uniformity, accuracy, and comparability for clinical, registry, and research purposes. This report describes the committee's combined expert position statement on hepatobiliary involvement in CF, which has been endorsed by NASPGHAN and ESPGHAN. We recommend using CFHBI (Cystic Fibrosis Hepato-Biliary Involvement) as the updated term to describe and classify all hepatobiliary manifestations in all pwCF. CFHBI encompasses the current extensive spectrum of phenotypical, clinical, or diagnostic expressions of liver involvement observed in pwCF. We present a schematic categorization of CFHBI, which may also be used to track and classify the changes and development of CFHBI in pwCF over time. The proposed classification for CFHBI is based on expert consensus and has not been validated for clinical practice and research purposes. Achieving validation should be an important aim for future research.

Practical approach to abnormal liver enzymes in patients with inflammatory bowel disease

Inflammatory Bowel Diseases (IBD) are chronic inflammatory conditions that can impact organ systems beyond the gastrointestinal tract. Extraintestinal manifestations (EIMs) of IBDs are common and can occur at any stage of the disease. While EIMS most commonly involve the musculoskeletal system, up to 35% of individuals with IBD exhibit hepatobiliary involvement at some point during the course of their disease, often independently of disease activity. Chronic hepatobiliary diseases are noted in 5% of patients with IBD. These diseases manifest with indicative symptoms, abnormal liver biochemistry tests, or radiological abnormalities. This review provides a comprehensive outline and approach to abnormal liver enzymes in individuals with IBD.

Study of spectrum of hepatobiliary involvement in children with sickle cell disease

Study of spectrum of hepatobiliary involvement in children with sickle cell disease

Update on Cystic Fibrosis in Pediatric Patients.

Cystic fibrosis is an inherited, multisystem disease that affects the gastrointestinal system in numerous ways. This article reviews the nutritional, gastrointestinal, and hepatobiliary manifestations of cystic fibrosis with an emphasis on the effects of CFTR modulator therapy. The life expectancy of individuals with cystic fibrosis has increased substantially in recent years. CFTR modulator therapy improves pulmonary function and results in weight gain. An individualized approach to nutrition is encouraged. Pancreatic exocrine function may improve with intervention early in life. The use of non-invasive methods to screen for hepatobiliary involvement is recommended. Highly effective CFTR modulators lead to increased survival and improved quality of life for many individuals. Their effects on gastrointestinal symptoms and hepatobiliary disease are not fully understood. Patient-reported outcome measures and biomarkers are important clinical endpoints for studying the effects of modulators.

Afectarea hepatică în mucoviscidoză

Among the systemic impairment in cystic fibrosis there is also hepatobiliary tract involvement, with some genetic determinants of the disease manifesting as cystic fibrosis-associated liver disease (CFLD). The pathogenesis, natural history and cyrrhogenic evolution of CFLD are discussed in relation to the multiple phenotypes of cystic fibrosis. The CFLD therapy is nonspecific, the disease progression to cirrhosis is unpredictable, and the CFTR modulators offer a still underinvestigated therapeutic alternative.

Clinical, biochemical and pulmonary CT imaging features for hepatobiliary involvement in COVID-19.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection is a viral disease with primary pulmonary involvement and systemic impact. This article aims to assess the importance of clinical, biological, demographic and radioimaging parameters in COVID-19 patients in characterizing the incidence and severity of the hepatobiliary involvement. We performed an observational cohort study on 132 consecutive patients, evaluating their demographics, hospitalization period, peripheral oxygen saturation (SpO2) in the ambient air, as well as biochemical markers of hepatobiliary involvement: aspartate aminotransferase (AST), alanine aminotransferase (ALT), total bilirubin (TB), direct bilirubin (DB), gamma-glutamyl transferase (GGT), serum albumin, total serum proteins, D-dimers; coagulation tests such as prothrombin time (PT), activated partial thromboplastin time (aPTT), and international normalized ratio (INR); inflammatory markers: fibrinogen, serum ferritin, C-reactive protein (CRP), interleukin-6 (IL-6), and tumor necrosis alpha (TNF-α). Hepatobiliary involvement was further stratified by type of affection pattern: hepatocytolysis, cholestasis or mixed type. All patients received a computerized tomography (CT) chest scan in the first or second day of hospital admission. We observed lower SaO2 and longer hospitalization days in patients with hepatobiliary involvement, as well as longer coagulation times (PT and INR), lower serum albumin and higher serum ferritin (p<0.05). No significant correlations have been found between the degree or type of pattern of lung involvement as seen on CT scans performed and biochemical liver changes. Hepatobiliary involvement occurred in 72% of patients in the study group, associated with longer hospitalization period, prolonged coagulation parameters, lower serum albumin levels, raised serum ferritin and CRP levels. Cholestatic and mixed types of injury were associated with higher ferritin levels, while mixed type alone presented higher D-dimers levels compared with the cholestatic or hepatocytolysis groups. No significant correlation was found between lung involvement by CT evaluation and hepatobiliary involvement.

Disseminated langerhans cell histiocytosis presenting as decompensated childhood cirrhosis

Childhood cirrhosis is a rare disease of multifactorial etiopathogenesis. One of the rare underlying causes is Langerhans cell histiocytosis (LCH). The multifocal multisystemic variant of LCH can rarely involve the hepatobiliary system. Indirect and direct forms of hepatobiliary involvement are known. Early diagnosis of underlying liver involvement in cases of LCH is crucial to prevent decompensation and ameliorate prognosis post-liver transplantation. This is a case of an 18-month-old male child who developed cutaneous lesions in the early infancy. He was brought to clinical attention due to progressively increasing abdominal girth. His laboratory, clinical and radiological examination suggested multisystem pathology, subsequently confirmed on biopsy as LCH with predominant involvement of hepatobiliary, pulmonary, and integumentary systems. Unfortunately, he succumbed within a week of diagnosis.

Brucellosis-associated hepatitis.

Brucellosis is a zoonotic infection that can affect almost every organ. A mild elevation of aminotransferase levels is usually observed in liver involvement. However, the development of clinical hepatitis is rare. In this study, we aimed to present the hospitalized cases with brucellosis hepatitis in our clinic in a 13-year period. A hundred and three patients with significant hepatobiliary involvement, diagnosed by microbiological analysis, were included in the study. For the presence of hepatitis, it was required that the aminotransferases must be ≥ 5 times more than the upper limit and/or the total bilirubin level must be ≥ 2mg/dl and/or the local hepatic lesion must be demonstrated. Of the cases, 35.9%, 17.5%, and 46.6% had clinical hepatitis, cholestatic hepatitis, and both clinical and cholestatic hepatitis, respectively. The most frequent symptom was fever (85.4%) while the most preferred treatment options were combinations containing aminoglycosides. It was observed that the mean time-interval to decrease to normal values of ALT, AST, and bilirubin values was 15.2 ± 7.8days while the patients having their treatment regimens. In our study, which focused on liver involvement, it was found that a chronic liver disease did not develop in any of the cases. Our study showed that, even in the presence of hepatitis, clinical response and laboratory improvement were high with appropriate treatment. It was observed that the improvement in aminotransferases and total bilirubin values delayed in the cases with blood culture positivity, secondary organ involvement, and alanine aminotransferase/aspartate aminotransferase > 1.

Clinical Profiles of Children With Sickle Cell Anaemia Presenting With Acute Clinical Events: A Single-Center Study.

Sickle cell disease is a common genetic disorder characterised by chronic haemolytic anaemia and vaso-occlusive crisis. Sickle cell anaemia (SCA) has both short-term effects in the form of acute clinical events and long-term repercussions seen with chronic multiorgan involvement. It is associated with significant morbidity and mortality. In India, the disease is largely undocumented. Thus, there is an urgent need to highlight the features of the disease so that locally appropriate models of care may be implemented. This study aims to evaluate acute clinical events in SCA and to provide data that may help to reduce the rate of morbidity and mortality associated with this disease by early interventions. A cross-sectional observational study was conducted between November 2020 and May 2022 at Indira Gandhi Government Medical College and Hospital, Nagpur, Central India. The inclusion criteria included previously diagnosed patients of SCA (homozygous sickle cell disease) on high-performance liquid chromatography (HPLC) between the age groups of six months and 12 years, presenting with acute clinical events. The exclusion criteria included patients younger than six months and older than 12 years of age, and all patients with other haemoglobinopathies and sickle cell trait. The study was approved by the Institutional Ethical Committee. All the data was entered into a well-designed Microsoft Office Excel spreadsheet (v 2019, Microsoft, Washington, USA). All the clinical, biochemical, and haematological data were tabulated and analysed. A total of 100 children with sickle cell disease diagnosed by HPLC were enrolled during the study period. About 215 acute clinical events among the 100 cases were recorded, for which they were admitted to the paediatric ward or PICU. The majority (35%, n=35) were seen in the age group of six to nine years (school-going age). About 52% were male and 48% were female (male-to-female ratio= 1.08:1). Pain was the most common symptom. The highest incidence of 36.75% (n=79) was seen with acute painful crises and was the most common indication of hospitalisation, followed by acute febrile illness (AFI) (34.42%, n=74), aplastic crisis (10.23%, n=22), splenic sequestration crisis (9.77%, n=21), hepatobiliary involvement (3.72%, n=8), acute chest syndrome and haemolytic crisis (each 1.86%, n=4), and stroke (1.40%, n=3). In cases of having foetal haemoglobin (HbF) ≥20%, the incidence of acute painful crisis (p=0.0001), hand-foot syndrome (p=0.047), aplastic crisis (p=0.033), splenic sequestration crisis (p=0.039), and AFI (p=0.035) was low as compared to cases having HbF ≤20% which was statistically significant. The incidence of acute painful crisis, hand-foot syndrome, and an aplastic crisis was significantly low in patients receiving hydroxyurea therapy as compared to patients who were not on hydroxyurea. Out of 100 cases, four died during the study period, three died because of splenic sequestration crisis with septic shock, and one died due to hepatic encephalopathy due to haemolytic crisis with septic shock. Acute clinical events in sickle cell disease can have significant morbidity and mortality in the paediatric age group. The nutritional status of sickle cell disease children must be given due importance. Early initiation of hydroxyurea must be encouraged to maintain higher HbF levels, which plays a significant role in reducing morbidity.

COVID-19 related biliary injury: A review of recent literature

Since its emergence in 2019, it has become apparent that coronavirus 2019 (COVID-19) infection can result in multi systemic involvement. In addition to pulmonary symptoms, hepatobiliary involvement has been widely reported. Extent of hepatic involvement ranges from minor elevation in liver function tests (LFTs) to significant hepatocellular or cholestatic injury. In majority of cases, resolution of hepatic injury or improvement in LFTs is noted as patients recover from COVID-19 infection. However, severe biliary tract injury progressing to liver failure has been reported in patients requiring prolonged intensive care unit stay or mechanical ventilation. Due to the timing of its presentation, this form of progressive cholestatic injury has been referred to as COVID-19 cholangiopathy or post-COVID-19 cholangiopathy, and can result in devastating consequences for patients. COVID-19 cholangiopathy is recognized by dramatic elevation in serum alkaline phosphatase and bilirubin and radiologic evidence of bile duct injury. Cholangiopathy in COVID-19 occurs weeks to months after the initial infection and during the recovery phase. Imaging findings and pathology often resemble bile duct injury associated with primary or secondary sclerosing cholangitis. Etiology of COVID-19 cholangiopathy is unclear. Several mechanisms have been proposed, including direct cholangiocyte injury, vascular compromise, and cytokine release syndromes. This review summarizes existing data on COVID-19 cholangiopathy, including reported cases in the literature, proposed pathophysiology, diagnostic testing, and long-term implications.

Systemic sclerosis and primary biliary cholangitis: Longitudinal data to determine the outcomes.

Several studies described the cross-sectional characteristics of systemic sclerosis patients and coexisting primary biliary cholangitis, but longitudinal prognostic data are lacking. To describe the systemic sclerosis-primary biliary cholangitis phenotype, including baseline characteristics and outcomes. We performed a multicentre the European Scleroderma Trials and Research Group study of systemic sclerosis patients with primary biliary cholangitis or with primary biliary cholangitis-specific antibodies, matched with systemic sclerosis controls free from hepatobiliary involvement matched for disease duration and cutaneous subset. Data were recorded at baseline and at the last available visit. A total of 261 patients were enrolled (115 primary biliary cholangitis-systemic sclerosis, 161 systemic sclerosis). At baseline, systemic sclerosis-primary biliary cholangitis patients had a higher prevalence of anti-centromere antibodies (p = 0.0023) and a lower prevalence of complete absence of digital ulcers. The milder vascular involvement was confirmed at follow-up when crucial differences emerged in the percentage of patients experiencing digital ulcers; a significantly higher number of patients who never experienced digital ulcers were observed among primary biliary cholangitis-systemic sclerosis patients (p = 0.0015). Moreover, a greater incidence of pulmonary arterial hypertension (p < 0.001) and of conduction blocks (p = 0.0256) was observed in systemic sclerosis patients without primary biliary cholangitis. Patients with primary biliary cholangitis had higher levels of liver enzymes at baseline than systemic sclerosis patients; a significant decrease in liver enzymes was observed at follow-up. Out of 18 patients with cholangitis, one received a liver transplant at follow-up. Our data show that systemic sclerosis-primary biliary cholangitis exhibit a mild systemic sclerosis and primary biliary cholangitis phenotype with outcomes being in general favourable.

Hepatobiliary Involvement in Neonates With COVID-19 Infection: A Previous Case Series Studies

Background: The most common symptoms in neonates with severe COVID-19 infection have been respiratory problems. The virus may adversely affect organs such as the liver. Methods: Studies focusing solely on liver involvement in newborns with COVID-19 infection were searched in Pubmed, Scopus, and Google Scholar databases. Eight studies were finally reviewed. Results: Direct viral invasion (COVID-19-induced hepatitis or systemic inflammatory response) and drug-induced may contribute to liver damage in neonates. SARS-CoV-2 may be the latest spark in toxoplasmosis, other agents, rubella, cytomegalovirus, herpes infections, and fetal liver involvement may be induced by transplacental transmission. Conclusions: Hepatic dysfunction is infrequent but important in neonates with COVID-19 infection and the mechanism of liver damage associated with COVID-19 may differ from that in adults.

Isolated Hepatobiliary Involvement in an Infant with Langerhans Cell Histiocytosis

Background: Langerhans cell histiocytosis (LCH) is a rare condition in which there is the proliferation of cells of the monocyte-dendritic cell lineage, including Langerhan's cells, with variable clinical manifestations and outcomes. Hepatic involvement is often seen as a part of multi-system LCH and is associated with a fulminant course. Isolated hepatobiliary involvement in LCH is rare. Clinical Description: We report an 11-month-old female infant presenting with prolonged fever associated with progressive jaundice, clay-colored stools, and a massive hepatomegaly. Liver biopsy showed CD1a and Langerin positivity. There was no other system involvement, and hence, the infant was diagnosed with a case of single-system LCH with isolated hepato-biliary LCH. Management and Outcome: A customized chemotherapeutic regimen was started with cytarabine and prednisolone. The patient has responded and there has been gradual improvement in the general clinical condition as well as the laboratory parameters. Conclusions: This case report creates the awareness of possible presentation of pediatric LCH with isolated liver involvement. A high index of suspicion, carefully customized chemotherapeutic regimen may help in the timely diagnosis and successful management.

P729 Evaluation of hepatobiliary pathologies using transient elastography in patients with inflammatory bowel disease: What about the effect of drugs?

Abstract Background In this study, we evaluated hepatobiliary involvement in inflammatory bowel disease (IBD) with FibroScan® (transient elastography) and examined drug effects on liver fibrosis and steatosis. We particularly focused on the effect of azathioprine (AZT) on spleen stiffness (SS). Methods A total of 352 subjects were included in this study, including 298 IBD patients and 54 healthy controls. LSM (liver stiffness measurement), SS, and CAP™ (controlled attenuation parameter) scores were measured by FibroScan®. In addition to disease and patient characteristics; laboratory tests, and effect of the drugs were evaluated and compared within the groups. Results The patient and control groups were similar in terms of age and gender. The characteristics of the groups are shown in Table 1. Mean LSM (5.2±3.1kPa vs 4.5±1,1kPa), mean SS (22.69±13,10kPa vs 17.96±7,44kPa), CAP™ score (hepatosteatosis) (233±56dB/m vs 224±47dB/m) were significantly higher in the patients with IBD than in the control group (p&amp;lt;0.05). Liver fibrosis and steatosis grades are shown in Figure 1. A positive correlation was found between CAP™ score and male gender, hypertension, diabetes, BMI, smoking, penetrating disease, GGT, triglyceride, LSM, and SS (p&amp;lt;0.05). LSM (5.7±3,1kPa vs 5.0±3,1kPa) was significantly higher in patients with extraintestinal involvement (p&amp;lt;0.05). In patients with significant liver fibrosis (F2-3-4), the frequency of anti-TNF treatment was lower (44,4% vs 69,5%; p=0,008) (Figure 2). In patients who use alcohol regularly compared to nondrinkers (25.54±14,83kPa vs 21.05±11,24kPa) SS was significantly higher (p&amp;lt;0.05). Additionally, in patients using AZT≥100 mg/day compared to those using AZT&amp;lt;100 mg/day (23.48±14,61kPa vs 19.99±11,84kPa; p&amp;lt;0,05) SS was significantly higher (Figure 3). AZT dose was positively correlated with SS (Spearman analysis; r=0.182 and p=0.013); however, no correlation was found with the duration or cumulative dose of AZT. The mean level of platelets was significantly higher in patients with IBD than in controls (300±104x10³/µL vs 251±52x10³/µL, p=0,001); however, there was no correlation between SS and trombosit level (p&amp;gt;0,05). 83% of patients were in clinical remission (Mayo score≤2/CDAI&amp;lt;150). No positive correlation was found between disease activity and SS in IBD patients. AZT-associated non-cirrhotic portal hypertension was detected in only one patient. Conclusion In this study, fatty liver, liver fibrosis, and SS were found to be significantly higher in IBD patients than in healthy controls. Although there is no relationship with the duration of AZT therapy, AZT dose was positively correlated with SS. We recommend that spleen stiffness should be evaluated in patients who use high doses of AZT (≥100 mg/day).