PURPOSE: With growing number of obese children, the prevalence of nonalcoholic fatty liver disease (NAFLD) in pediatric population is increasing. Nonalcoholic steatohepatitis (NASH) is a severe form of NAFLD, and can cause morbid complications. It is important to identify patients in order to grade pathologic severities and treat those children who possibly have NASH. This study was performed to evaluate whether the pharmacological therapy is also effective as well as the body weight reduction in pediatric NAFLD. METHODS: Among the 52 children presenting with obesity and hepatopathy, NAFLD was diagnosed through liver biopsy in 29 children, who were 7 to 14 years of age, from January 2006 to December 2011. The patients were advised to reduce their body weight through diverse methods. Medication with Ursodeoxycholic acid (UDCA) and vitamin E was performed in children whose liver functions did not improve or their weight reductions were not successful. The therapeutic effects were monitored and assessed via the biochemical profiles and the physical measurements. RESULTS: The therapy of vitamin E and UDCA combined with body mass index (BMI) reduction showed significantly higher rate of improvement in clinical profiles, which could be seen in data of aspartate aminotransferase (AST), alanine aminotransferase (ALT), AST/ALT ratio, alkaline phosphatase, total bilirubin and gamma-glutamyl transpeptidase. Children whose BMI were successfully reduced showed favorable clinical improvements without any medication, but those without BMI reduction did not show any improvement despite medications. CONCLUSION: This study showed that the first line of therapy should be the BMI reduction in NAFLD and drug therapy combined with BMI reduction could have additive therapeutic effect in children with NAFLD.
Alanine Transaminase ; Alkaline Phosphatase ; Aspartate Aminotransferases ; Bilirubin ; Biopsy ; Body Mass Index ; Body Weight ; Child ; Fatty Liver ; gamma-Glutamyltransferase ; Humans ; Liver ; Obesity ; Prevalence ; Ursodeoxycholic Acid ; Vitamin E ; Vitamins ; Weight Loss

BACKGROUND: Gelsolin and matrix metalloproteinase 12 (MMP12) expression has been reported in Langerhans cell histiocytosis (LCH), but the clinical significance of this expression is unknown. We investigated the associations of these proteins with clinical manifestations in patients diagnosed with LCH. METHODS: We performed a retrospective analysis of clinical data from patients diagnosed with LCH and followed up between 1998 and 2008. Available formalin-fixed, paraffin-embedded specimens were used for gelsolin and MMP12 immunohistochemical staining. We analyzed the expression levels of these proteins and their associations with LCH clinical features. RESULTS: Specimens from 36 patients (20 males, 16 females) with a diagnosis of LCH based on CD1a positivity with clinical manifestations were available for immunohistochemical staining. Median patient age was 62 months (range, 5 to 207). The expression of gelsolin varied; it was high in 17 patients (47.2%), low in 11 patients (30.6%), and absent in 8 patients (22.2%). The high gelsolin expression group had a higher tendency for multi-organ and risk organ involvement, although the trend was not statistically significant. MMP12 was detected only in 7 patients (19.4%) who showed multi-system involvement (P=0.018) and lower event-free survival (P=0.002) in comparison to patients with negative MMP12 staining. CONCLUSION: Gelsolin and MMP12 expression may be associated with the clinical course of LCH, and MMP12 expression may be particularly associated with severe LCH. Further studies of larger populations are needed to define the precise role and significance of gelsolin and MMP12 in the pathogenesis of LCH.
Disease-Free Survival ; Gelsolin ; Histiocytosis ; Histiocytosis, Langerhans-Cell ; Humans ; Immunohistochemistry ; Langerhans Cells ; Male ; Matrix Metalloproteinase 12 ; Proteins ; Retrospective Studies

BACKGROUND AND OBJECTIVES: Cigarette smoking is a risk significant factor in coronary artery disease (CAD) and vasospastic angina (VSA). However, it is largely unknown whether smoking adds to any long-term clinical risk in VSA patients. SUBJECTS AND METHODS: A total of 2797 patients without significant CAD underwent acetylcholine (Ach) provocation test between November 2004 and October 2010. Patients were divided into three groups, based on the presence of coronary artery spasm (CAS) and smoking habits (non-CAS group: n=1188, non-smoking CAS group: n=1214, smoking CAS group: n=395). All CAS patients were prescribed with anti-anginal medications for at least 6 months. The incidence of major clinical outcomes and recurrent angina of these groups were compared up to 3 years. RESULTS: There were considerable differences in the baseline clinical and angiographic characteristics among the three groups, but there was no difference in the endpoints among the three groups (including individual and composite hard endpoints) such as death, myocardial infarction, de novo percutaneous coronary intervention, cerebrovascular accident, and major adverse cardiac events. However, there was a higher incidence of recurrent angina in both the non-smoking CAS group and smoking CAS group, as compared to the non-CAS group. In multivariable adjusted Cox-proportional hazards regression analysis, smoking CAS group exhibited a higher incidence of recurrent angina compared with the non-CAS group (hazard ratio [HR]; 2.46, 95% confidence interval [CI]; 1.46-4.14, p=0.001) and non-smoking CAS group (HR; 1.76, 95% CI; 1.08-2.87, p=0.021). CONCLUSION: Cigarette smoking CAS group exhibited higher incidence of recurrent angina during the 3-year clinical follow-up compared with both the non-CAS group and non-smoking CAS group. Quitting of smoking, paired with intensive medical therapy and close clinical follow-up, can help to prevent recurrent angina.
Acetylcholine ; Coronary Artery Disease ; Coronary Vessels ; Follow-Up Studies ; Humans ; Incidence ; Myocardial Infarction ; Percutaneous Coronary Intervention ; Smoke ; Smoking* ; Spasm ; Stroke ; Tobacco Products*