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Polycystic liver disease (PLD) is a hereditary disease characterized by the presence of 20 or more liver cysts. It is classified into three types: isolated autosomal dominant PLD, PLD with autosomal dominant polycystic kidney disease, and PLD with autosomal recessive polycystic kidney disease. Genetic alterations, ciliary dysfunction of the biliary epithelial cells, and aberrant cell signaling pathways are the main factors contributing to the pathophysiology of PLD; however, other complicated mechanisms are also involved. The Gigot and Schnelldorfer classifications are widely used in clinical practice. Most patients with PLD are asymptomatic; however, a few patients with advanced-stage disease may develop symptoms and complications that impair their quality of life and require treatment. The known treatment options for PLD are somatostatin analogues, aspiration with sclerotherapy, fenestration, hepatic resection, and liver transplantation. Although liver transplantation remains the only curative treatment for PLD, medical therapies are gradually being developed with the increasing knowledge of the disease’s pathophysiology. This review focuses on the clinical manifestations and diagnosis of PLD, as well as treatment strategies, to support clinicians regarding the clinical management of the disease.
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Background/Aims@#Tenofovir alafenamide (TAF) has shown less favorable effect on lipids compared to tenofovir disoproxil fumarate (TDF) in clinical trials. However, data regarding these outcomes in patients with chronic hepatitis B (CHB) are scarce. Therefore, this study aimed to evaluate the effect of TAF on the lipid in patients with CHB. @*Methods@#A total of 237 TAF-treated CHB patients compared with TDF, inactive CHB, and non-hepatitis B virus (HBV)-infected control groups using propensity score matching (PSM). @*Results@#Following PSM, each analysis was conducted on cohorts via the matching of 70:140 (TAF:TDF), 89:89 (TAF:inactive CHB), 140:560 (TAF:non-HBV infected control), and 368:1,472 (TDF:non-HBV-infected control). A significant decrease in the total cholesterol (TC) level was noted at 48 weeks in the TDF group compared to the TAF group (176.3±32.9 vs. 156.7±27.7, P<0.001) and the non-HBV-infected control group (175.0±29.5 vs. 156.2±28.3, P<0.001). However, no significant change in TC was observed in the TAF group and inactive CHB or non-HBV-infected control groups at 48 weeks. For the subgroup analyses of TAF vs. non-HBV-infected control subjects and inactive CHB patients whose detailed lipid profile information were available, no between-group differences in TC, low-density lipoprotein (LDL)-cholesterol, highdensity lipoprotein (HDL)-cholesterol, TC/HDL ratio, and LDL/HDL ratio were observed at 48 weeks. @*Conclusions@#TDF seems to have a lipid-lowering effect compared to the non-HBV-infected control and TAF-treated groups. However, in real practice, TAF might not worsen the lipid profiles of subjects compared to non-HBV-infected controls and patients with inactive CHB.
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Background/Aims@#Low-level viremia (LLV) after nucleos(t)ide analog treatment was presented as a possible cause of hepatocellular carcinoma (HCC) in patients with chronic hepatitis B (CHB). However, detailed information on patients’ adherence in the real world was lacking. This study aimed to evaluate the effects of LLV on HCC development, mortality, and cirrhotic complications among patients according to their adherence to entecavir (ETV) treatment. @*Methods@#We performed a retrospective observational analysis of data from 894 consecutive adult patients with treatment-naïve CHB undergoing ETV treatment. LLV was defined according to either persistent or intermittent episodes of <2,000 IU/mL detectable hepatitis B virus DNA during the follow-up period. Good adherence to medication was defined as a cumulative adherence ≥90% per study period. @*Results@#Without considering adherence in the entire cohort (n=894), multivariate analysis of the HCC incidence showed that LLV was an independent prognostic factor in addition to other traditional risk factors in the entire cohort (P=0.031). Good adherence group comprised 617 patients (69.0%). No significant difference was found between maintained virologic response and LLV groups in terms of the incidence of liver-related death or transplantation, HCC, and hepatic decompensation in good adherence group, according to multivariate analyses. @*Conclusions@#In patients with treatment-naïve CHB and good adherence to ETV treatment in the real world, LLV during treatment is not a predictive factor for HCC and cirrhotic complications. It may be unnecessary to adjust their antiviral agent for patients with good adherence who experience LLV during ETV treatment.
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Background@# Effective treatment of Graves’ disease during pregnancy is important because uncontrolled hyperthyroidism is associated with increased fetal and maternal morbidity and mortality. While there have been case reports of patients with Graves’ disease who failed to achieve euthyroid state during pregnancy, anesthesiologists rarely encounter patients with uncontrolled hyperthyroidism undergoing urgent Cesarean section.Case: A 31-year-old pregnant patient had uncontrolled hyperthyroidism due to Graves’ disease despite medical treatment. Her signs and symptoms suggested fetal distress and aggravation of the disease, leading to hospitalization. After a failed induction for vaginal delivery, an urgent Cesarean section was performed under spinal anesthesia via an interlaminar approach using 9 mg of 0.5% heavy bupivacaine and 20 μg of fentanyl. It resulted in successful delivery, with no perioperative complications for the mother and neonate. @*Conclusions@# This case demonstrates that spinal anesthesia may provide clinical stability to patients with uncontrolled hyperthyroidism undergoing urgent Cesarean section.
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This correction is being published to correct the 1st author's English name in above article.
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BACKGROUND/AIMS: Minor disorders of peristalsis are esophageal motility disorders categorized by the Chicago Classification (CC), version 3.0, which was announced in 2014. This study evaluated the efficacy of anti-reflux therapy in patients with minor peristaltic disorders. METHODS: Patients with minor peristaltic disorders in accordance with CC v3.0 were included. We reviewed the medical records of patients with esophageal high-resolution manometry findings, and investigated the demographic and clinical information as well as the medical therapy. Thereafter, the response to treatment was assessed after at least 4 weeks of treatment. RESULTS: A total of 24 patients were identified as having minor disorders of peristalsis from January 2010 to December 2015. The mean follow-up period was 497 days, and there were 17 patients (70.8%) patients with ineffective esophageal motility. In terms of anti-reflux therapy, proton pump inhibitors (PPIs) with prokinetic agents and PPIs alone were prescribed in 19 patients (79.2%) and 5 patients (20.8%), respectively. When the rate of response to the treatment was assessed, the responders rate (complete+satisfactory [≥50%] responses) was 54.2% and the non-responders rate (partial [<50%]+refractory responses) was 45.8%. Patients in the responder group were younger than those in the non-responder group (p=0.020). Among them, 13 patients underwent 24-hour multichannel intraluminal impedance-pH, and 10 patients (76.9%) were pathologic gastroesophageal reflux. CONCLUSIONS: The majority of esophageal minor peristaltic disorders were accompanied by gastroesophageal reflux, and therefore, they might respond to acid inhibitor. Further well-designed, prospective studies are necessary to confirm the effect of anti-reflux therapy in these patients.