Hepatology Forum最新文献

The association of dermatomyositis (DM) and autoimmune hepatitis (AIH) is rare and presents a diagnostic and therapeutic challenge. We describe the case of a 36-year-old man with DM diagnosed in 2012 and treated with corticosteroid and methotrexate. The patient achieved total remission 18 months later. In 2022, an AIH was diagnosed (cytolysis, cholestasis, anti-LC1, and anti-SLA antibodies) while DM was in remission. Liver function normalized after two months of treatment with mycophenolate mofetil and corticosteroids. Liver damage in systemic autoimmune diseases can result from viral, iatrogenic, or autoimmune processes. The association between DM and AIH is exceptional and has only been documented in one previous observation. Autoantibodies are essential for diagnosing and managing patients with inflammatory myopathy and AIH. In conclusion, this exceptional association of AIH and DM raises many questions regarding the presence of etiopathogenic links, such as genetic predisposition, autoimmunity disorders, viral infection triggers, or simply a happenstance.

Hepatocellular cancer (HCC) is the most common primary malignant tumor of the liver. The organs that HCC most commonly metastasizes to are the lungs, intra-abdominal lymph nodes, bones, and adrenal glands. Brain metastases have been reported rarely. Herein, we report a 54-year-old female patient who was diagnosed with cryptogenic cirrhosis by liver biopsy in 2010. Solid lesions were detected on radiological examination during follow-up in 2019. The patient's complaints of severe headache, nausea, and vomiting continued during the follow-up, and imaging was performed. A contrast-enhancing lesion, 2 cm in size, was reported in the left half of the clivus on T1 examination after intravenous contrast administration. A biopsy was performed on the mass extending from the clivus to the nasopharynx. The biopsy concluded that it was an HCC metastasis. Intracranial metastases of HCC have been reported very rarely compared to other extrahepatic sites. HCC cases with intracranial metastases have a poor prognosis. Intracranial metastases should be considered in the differential diagnosis in patients presenting with central nervous system findings.

Background and aim: The impact of fluid status changes on liver stiffness measurements (LSM) using transient elastography (TE) in dialysis patients remains unclear. This study aimed to evaluate LSM variations during hemodialysis (HD) and analyze contributing factors.

Materials and methods: A cross-sectional study was conducted on dialysis patients at a tertiary care hospital. TE and bioelectrical impedance analysis were performed at four time points: before dialysis, immediately after, the first day after, and the second day after dialysis. LSM values were compared across these time points.

Results: Seventy patients were enrolled, with two cases showing consistently extremely elevated LSM values exceeding 20 kPa, considered outliers. The mean LSM values were 7.6±7.0 kPa before dialysis, 6.12±2.94 kPa immediately after, 6.64±5.27 kPa on the first day, and 6.94±5.12 kPa on the second day after dialysis. The mean pre-HD LSM was significantly higher than immediately after and on the first day after dialysis, with mean differences of 1.54 kPa (95% CI 0.22-2.86, p=0.02) and 1.02 kPa (95% CI 0.15-1.9, p=0.02), respectively. The ultrafiltration volume positively correlated with the LSM difference pre- and post-HD (r=0.315, p=0.008). Patients with residual fluid overload had significantly higher post-HD LSM compared to euvolemic patients (p=0.003).

Conclusion: LSM values significantly decreased after dialysis and remained lower for up to 24 hours. Transient elastography should preferably be performed within 24 hours post-dialysis when the patient is in a euvolemic state.

Acute immunoallergic hepatitis presents as acute liver injury, often accompanied by nonspecific findings of fever, rash, and abdominal pain, and is often induced by drug ingestion. Allopurinol has been implicated in multiple cases of acute immunoallergic hepatitis. We present the case of a young East Asian male with gout who experienced acute immunoallergic hepatitis, complicated by DRESS syndrome with a severe cutaneous reaction, as a result of allopurinol intake. The patient was positive for the HLA-B58*01 gene, a significant risk factor for developing allopurinol-induced liver injury. The patient's liver injury and skin reaction improved with the administration of IV methylprednisolone, followed by a course of oral prednisone. Our case prompts clinicians to prescribe allopurinol with caution in certain high-risk populations and emphasizes the importance of administering corticosteroids early in such a presentation to avoid long-term liver damage.

Glycogen Storage Disease Type 1b (GSD Type 1b) is predominantly diagnosed in childhood. Rare cases emerging in adulthood present a unique set of clinical challenges, particularly concerning liver lesions. We report a 22-year-old male diagnosed unusually late with GSD Type 1b, underlining the hepatic complexities involved. He initially presented with hepatomegaly and solid nodular lesions in the liver. An abdominal magnetic resonance imaging (MRI) revealed a sizable hepatocellular adenoma (HCA), subsequently removed through surgical segmentectomy. Histopathology confirmed the lesion as a hepatocyte nuclear factor-1 alpha (HNF-1alpha) mutation-positive HCA. Follow-up MRI revealed the persistence of multiple smaller liver nodules, necessitating continued clinical surveillance. Hepatic adenomas are a common complication in GSD Type 1 patients, posing management challenges due to their size, multiplicity, and risk of malignancy. While liver transplantation is a last resort option, it can worsen metabolic control. Sodium-glucose cotransporter-2 (SGLT-2) inhibitors offer a potential alternative for improving glycemic regulation and possibly affecting the adenoma size.

Primary Biliary Cholangitis (PBC) is a chronic cholestatic liver disease that can lead to cirrhosis and the development of hepatocellular carcinoma. PBC is not known to be associated with hepatic angiosarcoma. Second-line treatments for PBC, including obeticholic acid, have had less than a decade of clinical use. We present a case of a patient with PBC treated with obeticholic acid who subsequently developed hepatic angiosarcoma. The patient is now on active surveillance following resection of the angiosarcoma. The development of this rare and aggressive cancer should prompt closer post-marketing surveillance for obeticholic acid.

Background and aim: Hydatid cysts are caused by Echinococcus larvae and are prevalent in endemic areas worldwide. We analyzed post-procedure complications and outcomes of patients with liver hydatid cysts.

Materials and methods: We included patients who were managed either by surgery or percutaneous drainage (PAIR) for hydatid liver cysts at Harran University Faculty of Medicine Hospital between January 2017 and February 2021. We recorded age, sex, segmental location, size, number, Gharbi classification, treatment modality, length of hospital stays, and complications.

Results: We included a total of 209 patients who were managed by hydatid liver cysts. Among them, 74 post-procedural complications were developed in a total of 69 (33%) patients. Biliary fistula was the most prevalent complication (n=38,18.2%). Hospitalization duration was a median of 5 days (2-36) and was 5 days (2-36) in patients who underwent surgery and 3 days (range:2-7) in patients managed by PAIR. Patients with cyst diameter ≥9.5 cm were predicted to have an increased risk of complications with 70% sensitivity and 60% specificity. More patients experienced any difficulties in patients who were managed by PAIR than those who underwent surgery (65.4% vs 28.4%, p<0.001).

Conclusion: Our study showed that both surgery and PAIR are safe and effective management strategies for patients with liver cyst hydatid. PAIR was associated with more complications but a shorter duration of hospitalization.

Background and aim: The primary aim of this study was to investigate the concordance of Transient Elastography FibroScan^® (FS) measurements, Fibrosis-4 (FIB-4), and the Aspartate Aminotransferase to Platelet Ratio Index (APRI) scores with each other and with liver biopsies in predicting histological fibrosis.

Materials and methods: In this single-center, cross-sectional, retrospective collected data cohort study spanning seven consecutive years, simultaneous FS measurements, FIB-4, and APRI scores of 778 patients with different diagnoses who had undergone liver biopsy were evaluated.

Results: A total of 417 (53.6%) of the patients were female. The median age was 51 years. The diagnoses were HBV (n=228), metabolic dysfunction-associated steatotic liver disease (MASLD) (n=185), HCV (n=58), cryptogenic (n=53), primary biliary cholangitis (n=40), autoimmune hepatitis (AIH) (n=28), overlap syndrome (OS) (n=23), multiple diagnoses (n=42), and other diagnoses (n=83). All three methods showed a strong correlation with histological fibrosis, and FS demonstrated a statistically significantly superior relationship compared to FIB-4 and APRI. In AIH and OS, FIB-4 and APRI scores do not show a consistent increase with histological stage; however, FS does. In MASLD, all three methods correlate with histologic stage, but FS measurements appear significantly superior.

Conclusion: Although FIB-4, APRI, and FS correlate well with histological fibrosis, especially in MASLD, evaluation with FS, if available, should be preferred. In the evaluation of fibrosis in AIH and OS, laboratory-based indicators should be avoided.

Background and aim: Liver transplantat (LT) is still associated with a significant need for blood product transfusion. This study aimed to identify preoperative factors that can predict the need for platelet transfusion in adults undergoing LT.

Materials and methods: A retrospective analysis of the database from liver transplant recipients was performed to evaluate the use of platelet transfusion during and after LT. Two groups of recipients were assigned, with or without perioperative platelet transfusion (groups A and B, respectively). Preoperative LT recipient variables such as age, gender, body mass index, pre-transplant laboratory tests, cause of liver transplant, the Model for End-Stage Liver Disease score, and other selected perioperative variables, including surgical data, were compared between the two groups.

Results: Of 150 patients, 70 who received platelet transfusions were included in group A. Regarding the preoperative recipient variables, the two groups showed significant differences in the Model for End-Stage Liver Disease score (p=0.013), pre-transplant platelet count (p<0.001), and international normalized ratio (p<0.001). The results of logistic regression analysis showed that pre-transplant platelet count <50×10⁹/L (odds ratio, 0.979; 95% confidence interval [0.969-0.989]; p<0.001), serum creatinine ≥123.76 µmol/L (1.4 mg/dL) (OR, 4.35; 95% CI [1.566-12.097]; p=0.005), international normalized ratio ≥1.5 (OR, 2.771; 95% CI [1.198-6.412]; p=0.017) were identified as predictors for the use of platelet transfusion in LT.

Conclusion: Pre-liver transplant recipients' platelet count, serum creatinine, and international standardized ratio are crucial in predicting platelet utilization during and after LT.