A Giant Hepatic Hemangioma Presenting With Bilateral Pulmonary Embolism: A Case Report.

Hepatic hemangiomas are the most common benign tumors found in the liver. Herein, we present a case of a giant hepatic hemangioma (>4 cm) complicated by bilateral pulmonary embolism originating from the inferior vena cava thrombus with clinical and radiological features of portal hypertension. A 52-year-old woman presented to the emergency department of our institution with a history of blackouts. She underwent an extensive workup for potential causes of syncope, and a massive bilateral pulmonary embolism was detected. On examination, the patient was conscious, and her vital signs were within normal ranges. Abdominal examination revealed massive ascites and irregular nodular hepatomegaly without splenomegaly. Laboratory test results revealed normal liver function. The patient had elevated D-dimer levels. The serum-ascites albumin gradient was not elevated. Tumor marker levels were all within the normal range, and autoimmune profile results and test results for thrombophilia markers were negative. Abdominopelvic CT demonstrated hepatomegaly and a giant fungating chronic hepatic hemangioma occupying the right lobe along with an infrarenal inferior vena cava thrombus. The patient was discharged and prescribed a therapeutic dose of enoxaparin and diuretics. As the patient was not a candidate for resection due to the large hemangioma size and invasion of the liver tissue, she was referred to another center for a liver transplant. Hepatic hemangiomas are benign lesions and are usually managed conservatively since surgical intervention is controversial and is reserved for symptomatic or complicated cases. With an anatomically challenging lesion, enucleation/resection could not be achieved, and liver transplantation was the best achievable option.

Position statement on the diagnosis and management of non-alcoholic fatty liver disease.

Non-alcoholic fatty liver disease (NAFLD) is a major national and international health burden. It is one of the most common liver diseases worldwide and the most common cause of abnormal liver enzymes in many developed countries. Non-alcoholic fatty liver disease is also known as an important cause of cryptogenic cirrhosis and second leading cause for liver transplantation. It is commonly associated with metabolic syndrome. Non-alcoholic steatohepatitis (NASH) is the progressive phenotype of NAFLD. In spite of promising performance of non-invasive tools, liver biopsy remains the gold standard test for NASH diagnosis. Over decades, many drugs have been investigated in phase 2 and 3; however, no approved therapy to date. Despite the alarming global rates of NAFLD, there are no local community-based studies on the prevalence of NAFLD or local practice guidelines on its management; this expert review aims to fill this gap.

Fibroscan Compared to FIB-4, APRI, and AST/ALT Ratio for Assessment of Liver Fibrosis in Saudi Patients With Nonalcoholic Fatty Liver Disease.

BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) is being increasingly recognized as a cause of chronic liver disease. It has also been associated with devastating outcomes such as decompensated liver cirrhosis and hepatocellular carcinoma, as well as diabetes and metabolic syndrome. OBJECTIVES: This study was conducted in order to assess liver fibrosis using Fibroscan, and to compare these results to the use of Fibrosis-4 (FIB-4) scores, AST platelet ratio index (APRI scores), and the AST/ALT ratios on NAFLD patients. PATIENTS AND METHODS: A cross sectional study was conducted on NAFLD patients who underwent Fibroscan examinations between September 1, 2011 and June 30, 2014. Demographic data was collected, including sex, age, and nationality; serum alanine aminotransferase levels (ALT, 30 - 65 U/L), serum aspartate aminotransferase levels (AST, 15 - 37 U/L), and platelet counts (150 - 400 k/µL) were also determined. The stages of fibrosis (F0 1 - 6, F1 6.1 - 7, F2 7 - 9, F3 9.1 - 10.3, and F4 ≥ 10.4) were defined in kPa. For each patient, the AST/ALT ratio was also measured. The results of APRI and FIB-4 were compared with the Fibroscan fibrosis scores. RESULTS: The results of 122 patients were analyzed, including 65 (53.3%) males with a mean age of 50.2 years (SD: 13.7; range: 18 - 86). The males were significantly younger than the females (48.7 years (SD: 16.03) versus 51.8 years (SD: 10.3 P = 0.05), respectively). The mean stiffness score was 12.02 (SD: 12.7) kPa. Forty-four patients (36%) had advanced fibrosis. The mean platelet and serum ALT levels were normal. There was a significant positive correlation between the Fibroscan results and the AST/ALT ratios, the APRI scores, and the FIB-4 results. Similarly, there was a significant positive correlation between age and fibrosis score, and a significant negative correlation between platelet count and stiffness score. CONCLUSIONS: The data showed that more than one-third of the cohort exhibited advanced fibrosis, demonstrating the need for the early diagnosis and treatment of NAFLD. The use of Fibroscan with other serum markers has been shown to be helpful for the diagnosis of severe fibrosis.

Variceal hemorrhage: Saudi tertiary center experience of clinical presentations, complications and mortality.

AIM: To determine the clinical presentation, underlying etiology and short- and long-term outcomes of acute variceal bleeding (AVB). METHODS: A retrospective descriptive cohort study of cirrhotic patients with AVB who were admitted to King Abdul Aziz University Hospital between January 2005 and December 2009. We obtained demographic data for all patients. For each patient we also obtained the clinical data at presentation; cause of liver cirrhosis, bleeding presentation (hematemesis and/or melena), presence of ascites, hepatic encephalopathy and renal impairment (RI) or hepatorenal syndrome. We carried out complete blood count, prothrombin time evaluation, and liver function tests. We also report all episodes of re-bleeding after the first episode of AVB, both during the initial admission and after discharge. We recorded the length of stay for each patient and thereby calculated the mean duration of stay for all patients. The length of follow-up after the first AVB and the outcome for each patient at the end of the study period were recorded. Causes of mortality either related to liver disease or non-liver disease cause were determined. RESULTS: A 125 patients were enrolled in the study. The number of episodes of AVB for each patients varied between 1 and 10. Survival from the first attack of AVB to death was 20.38 mo (SD 30.86), while the length of follow-up for the living patients was 53.58 mo (SD 24.94). Total number of AVB admissions was 241. Chronic hepatitis C, the commonest underlying etiology for liver disease, was present in 46 (36.8%) patients. Only 35 (28%) patients had received a primary prophylactic ß-blocker before the first bleeding episode. The mean hemoglobin level at the time of admission was 8.59 g/dL (SD 2.53). Most patients had Child-Pugh Class C 41 (32.8%) or Class B 72 (57.6%) disease. Hematemesis was the predominant symptom and was found in 119 (95.2%) patients, followed by melena in 75 (60.0%) patients. Ascites of variable extent was documented in 93 (74.4%) patients. We identified hepatic encephalopathy in 31 (28.8%) patients and spontaneous bacterial peritonitis in 17 (13.6%). Bleeding gastric varices was the cause of AVB in 2 patients. AVB was associated with shock in 22 patients, 13 of whom (59.1%) had Child-Pugh class C disease. RI was noted in 19 (46.3%) of 41 patients in Child-Pugh class C and 14 (19.4%) of 72 patients in Child-Pugh class B. None of the patients with Child-Pugh class A disease had RI. Emergency endoscopy was effective in controlling the bleeding, although the re-bleeding rate was still high, 12 (9.6%) during the same admission and 55 (44%) after discharge. The re-bleeding rate was higher in patients with ascites, occurring in 40/55 (72.2%). The length of hospital stay was 1-54 d with a mean of 8.7 d. Three patients had emergency surgery due to failure of endoscopic treatment and balloon tamponade. The overall long term mortality was 65%. Survival from the first attack of AVB to death was 20.38 ± 30.86 mo, while the length of follow-up for the living patients was 53.58 ± 24.94 mo. Patients with Child-Pugh score C had a higher risk of liver disease-related mortality (67.6%). RI (developed during admission) was the main factor that was associated with mortality (P = 0.045). CONCLUSION: The majority of patients with liver disease who present at the emergency unit for AVB are at an advanced stage of the disease. The outcome is poorer for patients who develop RI during hospitalization.

Chronic hepatitis C in saudi arabia: three years local experience in a university hospital.

BACKGROUND: Chronic hepatitis C (CHC) is a global infection. In Saudi Arabia, the prevalence of CHC is declining due to the implementation of a blood screening program. However, CHC still remains a leading cause of liver cirrhosis and hepatocellular carcinoma. OBJECTIVES: This is a retrospective study of CHC patients at the King Abdul Aziz University Hospital, Jeddah, Saudi Arabia. PATIENTS AND METHODS: Out of a total of 291 CHC patients from the hepatology clinic at King Abdul Aziz University hospital, Jeddah, 279 patients were included in the present study. They were primarily male (152, 54.5%), with a mean age of 50.41 ± 1.72 years. The majority of patients were either Saudi (108, 38.7%) or Egyptian (60, 21.5%). A total of 61 patients received combination treatment with pegylated interferon and ribavirin, and one patient with sickle-cell anemia received pegylated INF monotherapy. Demographic, clinical and laboratory features of the CHC patients, and their responses to treatment were studied. RESULTS: Decompensated cirrhosis was documented in 60 patients (21.5%), and hepatocellular carcinoma in 14 (5%). The mean level of serum alanine aminotransferase was 83.6 ± 231 u/L. The predominant genotype among the 70 patients tested, was genotype 4, followed by genotype 1 (39 and 18 patients, respectively). The sustained viral response (SVR) rate was 82.99%. The main predictive factors for SVR were baseline HCV viral load and rapid virologic response (RVR). The mean duration of follow-up was 4.2 ± .85 years. There were 24 patients who had liver disease-related mortality. CONCLUSIONS: our data showed that 22% of CHC patients progress to cirrhosis and another 22% had treatment. Liver related mortality was more common in patients with advanced cirrhosis.

Autoimmune liver disease - are there spectra that we do not know?

Autoimmune liver diseases (AILDs) are common leading causes for liver cirrhosis and terminal stage of liver disease. They have variable prevalence among patients with liver disease and have two major clinical and biochemical presentations. Autoimmune hepatitis (AIH) is the typical example of hepatocellular AILD, but it can also be presented under a cholestatic pattern. AIH has a scoring diagnostic system and respond in most cases to the treatment with prednisolone and azathioprine. Primary biliary cirrhosis (PBC) is the second most common AILD, with a cholestatic presentation and characterized by positive antimitochondrial antibody (AMA). It has an excellent response and long term outcome with the administration of ursodeoxycholic acid (UDCA). Another AILD that is thought to be a variant of PBC is the autoimmune cholangitis, being a disease that has biochemical and histological features similar to PBC; but the AMA is negative. Primary sclerosing cholangitis (PSC) is a rare entity of AILD that has a cholestatic presentation and respond poorly to the treatment, with the ultimate progression to advance liver cirrhosis in most patients. Other forms of AILD include the overlap syndromes (OS), which are diseases with mixed immunological and histological patterns of two AILD; the most commonly recognized one is AIH-PBC overlap (AIH-PSC overlap is less common). The treatment of OS involves the trial of UDCA and different immunosuppressants. Here we present three case reports of unusual forms of chronic liver diseases that most likely represent AILD. The first two patients had a cholestatic picture, whereas the third one had a hepatocellular picture at presentation. We discussed their biochemical, immunological and histological features as well as their response to treatment and their outcomes. Then, we compared them with other forms of AILD.

Autoimmune hepatitis: Single-center experience of clinical presentation, response to treatment and prognosis in Saudi Arabia.

BACKGROUND/AIM: Autoimmune hepatitis (AIH) is a common cause of end-stage liver disease worldwide. It is a disease prevalent in children and adults, with female predominance and variable clinical presentations. AIH has favorable responses to steroids and immunomodulators. Diagnosis of AIH is based on clinical and laboratory criteria, as suggested by the International Autoimmune Hepatitis Group. Data on the disease pattern of AIH from the Middle East countries is scarce. MATERIALS AND METHODS: In this retrospective analysis, we studied clinical and laboratory features, immunological data, radiological findings, liver biopsy findings and response to therapy in patients with AIH from the hepatology clinics of King Abdul Aziz, University Hospital, Jeddah, from 1994 to 2008. RESULTS: We diagnosed 41 patients with AIH, and 33 were included in the analysis. The mean age was 32.3 years, with female predominance of 75.7%. De-compensated cirrhosis at presentation was found in 45.5% of the patients. Acute hepatitis was associated with significantly higher levels of the serum ALT and bilirubin (P=0.001 and P=0.03, respectively). All our patients had type 1 AIH. Treatment with prednisolone and azathioprine resulted in complete or partial remission in majority of the patients (54.8%). However, patients with advanced disease showed a poorer response to treatment (P=0.016). Six patients with poor compliance had relapse of AIH. Two patients had a flare of the disease during pregnancy, and they responded well with prednisolone. The longest follow-up was 14 years and the shortest was 2 months. Four patients died from liver disease. CONCLUSION: AIH patients in Saudi Arabia are likely to present with advanced disease at a young age and would have a poorer response to therapy as compared with patients in other countries worldwide.