Humans ; Hypertension, Pulmonary ; metabolism ; Iron ; metabolism ; Metabolic Diseases

Animals ; Endothelium, Vascular ; Humans ; Lipid Metabolism ; Metabolic Diseases

The liver lies at the intersection of multiple metabolic pathways and consequently plays a central role in lipid metabolism. Pathological disturbances in hepatic lipid metabolism are characteristic of chronic metabolic diseases, such as obesity-mediated insulin resistance, which can result in nonalcoholic fatty liver disease (NAFLD). Tissue damage induced in NAFLD activates and recruits liver-resident and non-resident immune cells, resulting in nonalcoholic steatohepatitis (NASH). Importantly, NASH is associated with an increased risk of significant clinical sequelae such as cirrhosis, cardiovascular diseases, and malignancies. In this review, we describe the immunopathogenesis of NASH by defining the known functions of immune cells in the progression and resolution of disease.
Cardiovascular Diseases ; Fatty Liver ; Fibrosis ; Insulin Resistance ; Lipid Metabolism ; Liver ; Metabolic Diseases ; Metabolic Networks and Pathways ; Non-alcoholic Fatty Liver Disease*

Exosomes are extracellular vesicles that contain molecules that regulate the metabolic functions of adjacent or remote cells. Recent in vitro, in vivo and clinical studies support the hypothesis that exosomes released from various cell types play roles in the progression of metabolic disorders including type 2 diabetes. Based on this concept and advances in other diseases, the proteins, mRNA, microRNA and lipids in exosomes isolated from biological fluids have been proposed as biomarkers in metabolic disorders. However, several problems with the development of clinically applicable biomarkers have not been resolved. In this review, the biologic functions of exosomes are briefly introduced, and we discuss the technical and practical pros and cons of different methods of exosome isolation for the identification of exosomal biomarkers of metabolic disorders. Standardization of preanalytical variables and isolation of high-purity exosomes from fully characterized biological fluids will be necessary for the identification of useful exosomal biomarkers that can provide insights into the pathogenic mechanisms of complications of metabolic syndrome and of whole-body metabolism.
Biomarkers* ; Diabetes Mellitus ; Exosomes* ; Extracellular Vesicles ; In Vitro Techniques ; Metabolic Diseases* ; Metabolic Syndrome X ; Metabolism ; MicroRNAs ; RNA, Messenger

Exosomes are extracellular vesicles that contain molecules that regulate the metabolic functions of adjacent or remote cells. Recent in vitro, in vivo and clinical studies support the hypothesis that exosomes released from various cell types play roles in the progression of metabolic disorders including type 2 diabetes. Based on this concept and advances in other diseases, the proteins, mRNA, microRNA and lipids in exosomes isolated from biological fluids have been proposed as biomarkers in metabolic disorders. However, several problems with the development of clinically applicable biomarkers have not been resolved. In this review, the biologic functions of exosomes are briefly introduced, and we discuss the technical and practical pros and cons of different methods of exosome isolation for the identification of exosomal biomarkers of metabolic disorders. Standardization of preanalytical variables and isolation of high-purity exosomes from fully characterized biological fluids will be necessary for the identification of useful exosomal biomarkers that can provide insights into the pathogenic mechanisms of complications of metabolic syndrome and of whole-body metabolism.
Biomarkers* ; Diabetes Mellitus ; Exosomes* ; Extracellular Vesicles ; In Vitro Techniques ; Metabolic Diseases* ; Metabolic Syndrome X ; Metabolism ; MicroRNAs ; RNA, Messenger

Lipid metabolism disorder is an important risk factor to obesity, hyperlipidemia and type 2 diabetes as well as other chronic metabolic disease. It is also a key target in preventing metabolic syndrome, chronic disease prevention. Plant polyphenol plays an important role in maintaining or improving lipid profile in a variety of ways. including regulating cholesterol absorption, inhibiting synthesis and secretion of triglyceride, and lowering plasma low density lipoprotein oxidation, etc. The purpose of this article is to review the lipid regulation effects of plant polyphenols and its related mechanisms.
Animals ; Humans ; Lipid Metabolism ; drug effects ; Metabolic Diseases ; drug therapy ; metabolism ; Polyphenols ; pharmacology

BACKGROUND: Triphasic waves are one of the electroencephalographic patterns that can be usually seen in metabolic encephalopathy. The aim of this study is to compare the clinical and electrophysiologic profiles between patients with and without triphasic waves in metabolic encephalopathy, and reassess the significance of triphasic waves in metabolic encephalopathy. METHODS: We recruited 127 patients with metabolic encephalopathy, who were admitted to our hospital. We divided these admitted patients into two groups; those with and without triphasic waves. We analyzed the difference of duration of hospitalization, mortality rate during admission, Glasgow Coma Scale, severity of electroencephalographic alteration, and presence of acute symptomatic seizures between these two groups. RESULTS: Of the 127 patients with metabolic encephalopathy, we excluded 67 patients who did not have EEG, and 60 patients finally met the inclusion criteria for this study. Patients with triphasic waves had more severe electroencephalographic alterations, lower Glasgow Coma Scale, and more acute symptomatic seizures than those without triphasic waves. After adjusting the clinical variables, Glasgow Coma Scale and acute symptomatic seizures were only significantly different between patients with and without triphasic waves. CONCLUSIONS: We demonstrated that patients with triphasic waves in metabolic encephalopathy had more significant impairment of the brain function.
Brain ; Brain Diseases, Metabolic* ; Electroencephalography ; Glasgow Coma Scale ; Hospitalization ; Humans ; Metabolism ; Mortality ; Seizures

Isolated 3-methylcrotonyl-CoA carboxylase deficiency is an autosomal recessive disorder affecting leucine metabolism; it is one of the most common inborn metabolic diseases detected in newborn screening. Mutations in the genes MCCC1 or MCCC2 cause a defect in the enzyme 3-methylcrotonyl-CoA carboxylase, with MCCC2 mutations being the form predominantly reported in Korea. The majority of infants identified by neonatal screening usually appear to be asymptomatic and remain healthy; however, some patients have been reported to exhibit mild to severe metabolic decompensation and neurologic manifestations. Here we report the clinical features of a patient with asymptomatic 3-methylcrotonyl-CoA carboxylase deficiency and novel heterozygous MCCC1 mutations.
Humans ; Infant ; Infant, Newborn ; Korea ; Leucine ; Mass Screening ; Metabolic Diseases ; Metabolism ; Neonatal Screening ; Neurologic Manifestations

Global developmental delay (GDD) is a relatively common early-onset chronic neurological condition, which may have prenatal, perinatal, postnatal, or undetermined causes. Family history, physical and neurological examinations, and detailed history of environmental risk factors might suggest a specific disease. However, diagnostic laboratory tests, brain imaging, and other evidence-based evaluations are necessary in most cases to elucidate the causes. Diagnosis of GDD has recently improved because of remarkable advances in genetic technology, but this is an exhaustive and expensive evaluation that may not lead to therapeutic benefits in the majority of GDD patients. Inborn metabolic errors are one of the main targets for the treatment of GDD, although only a small proportion of GDD patients have this type of error. Nevertheless, diagnosis is often challenging because the phenotypes of many genetic or metabolic diseases often overlap, and their clinical spectra are much broader than currently known. Appropriate and cost-effective strategies including up-to-date information for the early identification of the "treatable" causes of GDD are needed for the development of well-timed therapeutic applications with the potential to improve neurodevelopmental outcomes.
Child* ; Developmental Disabilities ; Diagnosis ; Humans ; Metabolic Diseases ; Metabolism ; Neuroimaging ; Neurologic Examination ; Phenotype ; Risk Factors

Cholesterol ; metabolism ; Drugs, Chinese Herbal ; therapeutic use ; Humans ; Metabolic Diseases ; prevention & control ; Phytotherapy