No abstract available.
Aortic Valve Stenosis ; Natural History

Multiple endocrine neoplasia type 1 (MEN 1) is an autosomal dominant hereditary disorder caused by germline mutation of the MEN1 gene. It is characterized by tumors of the anterior pituitary gland, parathyroid glands, and endocrine pancreas. Thymic carcinoid tumor is uncommon and associated with a high mortality, but its natural history has not been investigated yet. We report a case of asymptomatic MEN 1 with a thymic carcinoid tumor. A 37-year-old man underwent a routine medical checkup and upper gastrointestinal endoscopy revealed a duodenal neuroendocrine tumor (NET). Further studies showed the coexistence of pancreatic tumor, parathyroid hyperplasia, pituitary adenoma, and thymoma. The patient underwent duodenal endoscopic mucosal resection, distal pancreatectomy, subtotal parathyroidectomy, and thymectomy. The pathological test revealed a duodenal NET, pancreatic NET, parathyroid hyperplasia, and thymic carcinoid tumor. He was treated for MEN 1. We report this asymptomatic case of MEN 1 with a literature review.
Adult ; Carcinoid Tumor ; Endoscopy, Gastrointestinal ; Germ-Line Mutation ; Humans ; Hyperparathyroidism ; Hyperplasia ; Islets of Langerhans ; Mortality ; Multiple Endocrine Neoplasia Type 1 ; Multiple Endocrine Neoplasia ; Natural History ; Neuroendocrine Tumors ; Pancreatectomy ; Parathyroid Glands ; Parathyroidectomy ; Pituitary Gland, Anterior ; Pituitary Neoplasms ; Thymectomy ; Thymoma

OBJECTIVE: To analyze the angiographic features and clinical course, including treatment outcomes and the natural course, of fusiform middle cerebral artery aneurysms (FMCAAs) according to their location, size, and configuration.METHODS: We reviewed the literature on adult cases of FMCAAs published from 1980 to 2018; from 25 papers, 112 FMCAA cases, for which the location, size, and configuration could be identified, were included in this study. Additionally, 33 FMCAA cases in our hospital were included, from which 16 were assigned to the observation group. Thus, a total of 145 adult FMCAA cases were included. We classified the FMCAAs according to their location (l-type 1, beginning from prebifurcation; l-type 2, beginning from bifurcation; l-type 3, beginning from postbifurcation), size (small, <10 mm; large, ≥10 mm; giant, ≥25 mm), and configuration (c-type 1, classic dissecting aneurysm; c-type 2, segmental ectasia; c-type 3, dolichoectatic dissecting aneurysm).RESULTS: The c-type 3 was more commonly diagnosed with ischemic symptoms (31.8%) than hemorrhage (13.6%), while 40.9% were found accidentally. In contrast, c-type 2 was more commonly diagnosed with hemorrhagic symptoms (14.9%) than ischemic symptoms (10.6%), and 72.3% were accidentally discovered. According to location, ischemic symptoms and hemorrhage were the most frequent symptoms in l-type 1 (28.6%) and l-type 3 (34.6%), respectively. Most of l-type 2 FMCAAs were found incidentally (68.4%). Based on the size of FMCAAs, only 11.1% of small aneurysms were found to be hemorrhagic, while 18.9% and 26.0% of large and giant aneurysms were hemorrhagic, respectively. Although four aneurysms of the 16 FMCAAs in the observation group increased in size and one aneurysm decreased in size during the observation period, no rupture was seen in any case and there were no significant predictors of aneurysm enlargement. Of 104 FMCAAs treated, 14 cases (13.5%) were aggravated than before surgery and all the aggravated cases were l-type 1.CONCLUSION: While ischemic symptoms occurred more frequently in l-type 1 and c-type 3, hemorrhagic rather than ischemic symptoms occurred more frequently in l-type 3 and c-type 2. In case of l-type 1 FMCAAs, more caution is required in determining the treatment due to the relatively high complication rate.
Adult ; Aneurysm ; Aneurysm, Dissecting ; Dilatation, Pathologic ; Hemorrhage ; Humans ; Intracranial Aneurysm ; Middle Cerebral Artery ; Natural History ; Rupture

This tutorial defines the concepts of disease progression in the context of clinical pharmacology. Disease progression describes the natural history of disease, such as pain, or biomarker of drug response, such as blood pressure. The action of a drug, such as inhibiting an enzyme or activating a receptor, leads to a change in disease status over time. Two main types of drug response can be defined based on the pattern of the time course of disease status. The most common is a symptomatic effect equivalent to a shift up or down of the natural history curve. Less common but quite clinically important is a disease-modifying effect equivalent to a change in the rate of disease progression.
Blood Pressure ; Disease Progression ; Natural History ; Pharmacology, Clinical

Multiple studies have shown that oral antiviral therapies reduced the incidence of hepatocellular carcinoma (HCC) and improved the survival of patients with chronic hepatitis B when compared with that of untreated patients. In particular, entecavir and tenofovir share the qualities of high efficacy in reducing the HBV DNA levels, and they have excellent tolerability and safety. These drugs modified the natural history of liver fibrosis, improve liver function, decrease the incidence of HCC, decrease the need for liver transplantation, and improve survival. Many studies have suggested that long-term antiviral therapy reduces the risk of HCC and liver cirrhosis in patients with chronic hepatitis. The mechanism of these drugs in reducing the risk of HCC is not clear. This article reviews the mechanisms of carcinogenic HBV by conducting a review of the literature on the efficacy of therapy for reducing the risk of HCC. A few recent articles have suggested that tenofovir offers advantages over entecavir in terms of HCC prevention, but these articles have the inherent limitations of observational data. No other head-to-head randomized trials exist. Further randomized studies would help provide stronger evidence of the association between the type of antiviral agent and the HCC outcomes. Only achieving complete viral eradication from the liver will truly decrease the mortality and incidence of HCC.
Antiviral Agents ; Carcinoma, Hepatocellular ; DNA ; Hepatitis B, Chronic ; Hepatitis, Chronic ; Humans ; Incidence ; Liver ; Liver Cirrhosis ; Liver Transplantation ; Mortality ; Natural History ; Tenofovir

HBV is the most common etiology of both liver cirrhosis and hepatocellular carcinoma in Korea. Despite much progress made, the currently available antiviral therapies cannot eradicate or eliminate this virus. Hence, the benefits and risks of antiviral therapy should be carefully evaluated on an individual basis and within the context of the clinical situation. The ultimate goals of treatment are to decrease the mortality from liver disease. The benefits of antiviral therapy come from prevention of progression of liver disease. Understanding the natural history of chronic HBV infection is a key step in the decision making process to treat patients with chronic HBV infection. Generally, chronic hepatitis B patients in the immune tolerant phase and immune inactive phase are not recommended to undergo antiviral treatment, except for those patients in special conditions (e.g., immunosuppression or anticancer chemotherapy). Chronic hepatitis B patients in the immune active phase are recommended for antiviral therapy. For patients with liver cirrhosis, treatment should be considered when serum HBV DNA is detectable regardless of the serum level of ALT.
Carcinoma, Hepatocellular ; Decision Making ; DNA ; Hepatitis B virus ; Hepatitis B, Chronic ; Hepatitis, Chronic ; Humans ; Immunosuppression ; Korea ; Liver Cirrhosis ; Liver Diseases ; Mortality ; Natural History ; Risk Assessment

Nonalcoholic fatty liver disease (NAFLD) is the most common cause of chronic liver disease in children. The global prevalence of pediatric NAFLD from general populations is 7.6%. In obese children, the prevalence is higher in Asia. NAFLD has a strong heritable component based on ethnic difference in the prevalence and clustering within families. Genetic polymorphisms of patatin-like phospholipase domain–containing protein 3 (PNPLA3), transmembrane 6 superfamily member 2, and glucokinase regulatory protein (GCKR) are associated with the risk of NAFLD in children. Variants of PNPLA3 and GCKR are more common in Asians. Alterations of the gut microbiome might contribute to the pathogenesis of NAFLD. High fructose intake increases the risk of NAFLD. Liver fibrosis is a poor prognostic factor for disease progression to cirrhosis. Magnetic resonance spectroscopy and magnetic resonance proton density fat fraction are more accurate for steatosis quantification than ultrasound. Noninvasive imaging methods to assess liver fibrosis, such as transient elastography, shear-wave elastography, and magnetic resonance elastography are useful in predicting advanced fibrosis, but they need further validation. Longitudinal follow-up studies into adulthood are needed to better understand the natural history of pediatric NAFLD.
Asia ; Asian Continental Ancestry Group ; Child ; Diagnosis ; Disease Progression ; Elasticity Imaging Techniques ; Epidemiology ; Fibrosis ; Follow-Up Studies ; Fructose ; Gastrointestinal Microbiome ; Genetics ; Glucokinase ; Humans ; Liver Cirrhosis ; Liver Diseases ; Magnetic Resonance Spectroscopy ; Microbiota ; Natural History ; Non-alcoholic Fatty Liver Disease ; Phospholipases ; Polymorphism, Genetic ; Prevalence ; Protons ; Ultrasonography

With increasing sensitivity, availability, and use of magnetic resonance imaging (MRI) in the past three decades, there is also an increase in incidental abnormal findings. The most common findings of these incidental abnormalities are white matter lesions that are interpreted as demyelinating based on radiological criteria. MRI findings suggestive of multiple sclerosis (MS) in patients without typical MS symptoms and with normal neurologic findings are now defined as a radiologically isolated syndrome (RIS) which reported relatively low incidence, especially hard to find case report in Korea. The natural history and pathophysiologic processes of RIS remain largely unknown. The nature, prevalence, and long-term prognosis of RIS, as well as the role of disease-modifying therapy should be established in the future.
Demyelinating Diseases ; Humans ; Incidence ; Korea ; Magnetic Resonance Imaging ; Multiple Sclerosis ; Natural History ; Neurologic Manifestations ; Prevalence ; Prognosis ; White Matter

Multiple studies have shown that oral antiviral therapies reduced the incidence of hepatocellular carcinoma (HCC) and improved the survival of patients with chronic hepatitis B when compared with that of untreated patients. In particular, entecavir and tenofovir share the qualities of high efficacy in reducing the HBV DNA levels, and they have excellent tolerability and safety. These drugs modified the natural history of liver fibrosis, improve liver function, decrease the incidence of HCC, decrease the need for liver transplantation, and improve survival. Many studies have suggested that long-term antiviral therapy reduces the risk of HCC and liver cirrhosis in patients with chronic hepatitis. The mechanism of these drugs in reducing the risk of HCC is not clear. This article reviews the mechanisms of carcinogenic HBV by conducting a review of the literature on the efficacy of therapy for reducing the risk of HCC. A few recent articles have suggested that tenofovir offers advantages over entecavir in terms of HCC prevention, but these articles have the inherent limitations of observational data. No other head-to-head randomized trials exist. Further randomized studies would help provide stronger evidence of the association between the type of antiviral agent and the HCC outcomes. Only achieving complete viral eradication from the liver will truly decrease the mortality and incidence of HCC.
Antiviral Agents ; Carcinoma, Hepatocellular ; DNA ; Hepatitis B, Chronic ; Hepatitis, Chronic ; Humans ; Incidence ; Liver ; Liver Cirrhosis ; Liver Transplantation ; Mortality ; Natural History ; Tenofovir

HBV is the most common etiology of both liver cirrhosis and hepatocellular carcinoma in Korea. Despite much progress made, the currently available antiviral therapies cannot eradicate or eliminate this virus. Hence, the benefits and risks of antiviral therapy should be carefully evaluated on an individual basis and within the context of the clinical situation. The ultimate goals of treatment are to decrease the mortality from liver disease. The benefits of antiviral therapy come from prevention of progression of liver disease. Understanding the natural history of chronic HBV infection is a key step in the decision making process to treat patients with chronic HBV infection. Generally, chronic hepatitis B patients in the immune tolerant phase and immune inactive phase are not recommended to undergo antiviral treatment, except for those patients in special conditions (e.g., immunosuppression or anticancer chemotherapy). Chronic hepatitis B patients in the immune active phase are recommended for antiviral therapy. For patients with liver cirrhosis, treatment should be considered when serum HBV DNA is detectable regardless of the serum level of ALT.
Carcinoma, Hepatocellular ; Decision Making ; DNA ; Hepatitis B virus ; Hepatitis B, Chronic ; Hepatitis, Chronic ; Humans ; Immunosuppression ; Korea ; Liver Cirrhosis ; Liver Diseases ; Mortality ; Natural History ; Risk Assessment