Reappraisal of Left Ventricular to Right Atrial (LV-RA) Shunt in Pediatric Patients With Isolated Perimembranous Ventricular Septal Defect

Background The purpose of this study was to investigate the follow-up results of perimembranous ventricular septal defect (VSD) with left ventricular to right atrial (LV-RA) shunt since infancy and to analyze the morphologic variations of this shunt. Methods and Results The study group comprised 232 consecutive pediatric patients with isolated perimembranous VSD and aneurysm, of whom 134 (58%) had LV-RA shunts. Follow-up echocardiography was performed to assess for the size of both the VSD and LV-RA shunt. There were no significant differences between groups in terms of sex, age at the initial echocardiography, follow-up period, number of patients with tricuspid regurgitation, and initial VSD size. There was a significant difference between groups in spontaneous closure (p=0.039). The event-free probability (no surgical repair of the defect) was not significantly different between the groups (p=0.129). Conclusions Perimembranous VSD with LV-RA shunt in infancy is common and associated with less chance of spontaneous closure. Color Doppler echocardiography can greatly improves the diagnostic efficacy and assist in understanding the mechanisms leading to this particular anomaly. (Circ J 2008; 72: 1487 - 1491).     

Focus-on-Teens, sexual risk-reduction intervention for high-school adolescents: impact on knowledge, change of risk-behaviours, and prevalence of sexually transmitted diseases

A community-based intervention, Focus-on-Kids (FOK) has demonstrated risk-behaviour reduction of urban youth. We modified FOK to Focus-on-Teens (FOT) for high schools. High school adolescents (n=1190) were enrolled over successive school semesters. The small-group sessions were presented during the school-lunch hours. Confidential surveys were conducted at baseline, immediate, six-, and 12-month postintervention for demographics, parental communication/monitoring, sexual risk behaviours and sexually transmitted diseases (STDs)/HIV/condom-usage knowledge. Sexually active participants were encouraged to volunteer for urine-based STDs testing at the School-Based Health Centres. Many (47.4%) students reported having had sexual intercourse at baseline. Overall behaviours changed towards 'safer' sex behaviours (intent-to-use and using condoms, communicating with partner/parents about sex/condoms/STDs) with time (P<0.05). Proportion of students with complete correct knowledge of STDs/HIV increased to 88% at time 4 from 80% at baseline after adjusting for age, gender and sexual activity (P<0.05). High prevalence of STDs was detected in 875 participants who reported for urine testing at time 1: trichomonas, 11.8%; chlamydia, 10.1% and gonorrhoea, 4.1%. Prevalence decreased significantly for 310 participants who re-tested; chlamydia: 27.4% to 6.1% and gonorrhoea: 11.3% to 3.2%. FOT was successfully implemented as an STDs/HIV risk-reduction intervention. Sustained improvements of knowledge about STDs/HIV/condom usage, decreases in sexual risk behaviours supported the effectiveness of this intervention.     

Aberrant co-expression of CD19 and CD56 as surrogate markers of acute myeloid leukemias with t(8;21) in Taiwan

Aberrant antigen expression in acute myeloid leukemia (AML) has been extensively studied in the West with limited reports from Taiwan. We carried out this retrospective study to characterize the frequency and significance of aberrant antigen expression of AML in Taiwan. Among 111 cases, 58 (52%) showed aberrant antigen expression, most frequently CD7 (27%) and CD56 (23%). Aberrant CD7 expression was observed in all non-AML-M3 subtypes, most frequently in AML-M7 (4/6, 67%); while CD19 expression was only observed in AML-M2 (5/36, 14%). CD56 expression was most common in AML-M5 (4/8, 50%). The relative frequency of CD19 and CD56 expression in AML with t(8;21) was higher than those with other chromosomal abnormalities or normal karyotype (P = 0.011 and 0.005, respectively). In non-M3 AML, aberrant antigen expression was identified in 56/96 (58%) cases, in contrast to 2/15 (13%) AML-M3 cases (P = 0.001). CD7, CD19 and CD56 expression was not correlated with remission rate. We concluded that aberrant immunophenotype was more frequent in non-M3 leukemias in Taiwan. The relative frequency of CD19 and/or CD56 expression in AML with t(8;21) was significantly higher than those without this translocation and co-expression of these two antigens may serve as the surrogate markers for AML with t(8;21).     

Relationship of the percentage of circulating endothelial progenitor cell to the severity of coronary artery disease

Previous study demonstrated that the percentage of circulating endothelial progenitor cells was reduced in patients with coronary artery disease. However, the relationship of the percentage of circulating endothelial progenitor cells to the severity of coronary artery disease has not been investigated. The percentages of circulating endothelial progenitor cells were measured in 78 consecutive patients with unstable angina, as well as in 32 healthy volunteers. Dual-stained cells expressing CD34 and vascular endothelial growth factor receptor-2 were judged to be endothelial progenitor cells and were analyzed using flow cytometry. On stepwise multiple linear regression analysis, the percentages of circulating endothelial progenitor cells were independently decreased in patients with unstable coronary artery disease compared with those in the healthy volunteers (P < 0.05). Among patients with unstable coronary artery disease, the percentage of patients with at least one occluded vessel was significantly higher in patients with multi-vessel disease than in patients with single-vessel disease (P < 0.04). On stepwise multiple linear regression analysis, the percentages of circulating endothelial progenitor cells were independently decreased in patients with multi-vessel coronary artery disease compared with those in patients with single-vessel coronary artery disease (P < 0.03). Among patients with unstable coronary artery disease, the percentage of circulating endothelial progenitor cells was significantly related to the severity of coronary artery disease.     

Successful salvage of peritoneal catheter in unresolved methicillin-resistant staphylococcus aureus peritonitis by combination treatment with daptomycin and rifampin

Peritoneal dialysis patients are at an increased risk of Gram-positive organism infections because of disrupted skin barrier function, presence of a peritoneal catheter, and a deficient immunological system. In particular, the treatment of methicillin-resistant Staphylococcus aureus (MRSA) infections is clinically challenging. Herein, we present a case of MRSA peritonitis that showed no response to a 14-day treatment with intraperitoneal vancomycin. To overcome unresponsiveness to vancomycin, we shifted the regimen to intraperitoneal daptomycin (given every 6 h through manual peritoneal dialysate exchanges) and oral rifampin (300 mg twice daily). The peritonitis resolved without sequelae or relapse. We suggest daptomycin and rifampin as an alternative combination therapy for MRSA infections that may otherwise remain unresolved.     

Regenerative phenotype in mice with a point mutation in transforming growth factor beta type I receptor (TGFBR1)

Regeneration of peripheral differentiated tissue in mammals is rare, and regulators of this process are largely unknown. We carried out a forward genetic screen in mice using N-ethyl-N-nitrosourea mutagenesis to identify genetic mutations that affect regenerative healing in vivo. More than 400 pedigrees were screened for closure of a through-and-through punch wound in the mouse ear. This led to the identification of a single pedigree with a heritable, fast, and regenerative wound-healing phenotype. Within 5 wk after ear-punch, a threefold decrease in the diameter of the wound was observed in the mutant mice compared with the wild-type mice. At 22 wk, new cartilage, hair follicles, and sebaceous glands were observed in the newly generated tissue. This trait was mapped to a point mutation in a receptor for TGF-β, TGFBR1. Mouse embryonic fibroblasts from the affected mice had increased expression of a subset of TGF-β target genes, suggesting that the mutation caused partial activation of the receptor. Further, bone marrow stromal cells from the mutant mice more readily differentiated to chondrogenic precursors, providing a plausible explanation for the enhanced development of cartilage islands in the regenerated ears. This mutant mouse strain provides a unique model to further explore regeneration in mammals and, in particular, the role of TGFBR1 in chondrogenesis and regenerative wound healing.     

Incidence and clinical outcome of icteric type hepatocellular carcinoma

BACKGROUND: To elucidate the incidence of the rare cases of icteric type hepatocellular carcinoma (IHCC), we conducted a retrospective study. The clinical outcomes of IHCC cases were also analyzed. METHODS: From January 1986 to December 1997, newly diagnosed HCC cases were evaluated and reviewed from an admission registration database in a medical center. Patients who had HCC tumors which were located within biliary tracts, and caused obstructive jaundice as the initial clinical manifestation of HCC, were defined as cases of IHCC. Besides clinical manifestations, the outcomes and survival times of various treatment strategies among the IHCC cases were compared retrospectively. RESULTS: The incidence of IHCC was 0.53% (10/1897). The median age of the 10 IHCC patients (seven men, three women) was 56.1 years (range = 41-72 years). Most primary HCC lesions (80%) were of expansive type: either single nodular type or multinodular type. All the patients eventually died of cholangitis and sepsis. Besides biliary drainage, seven patients of moderate to good liver reserve function received transarterial chemoembolization (TACE) for palliative treatment, and three of these received additional radiotherapy (R/T) because of the limited effect of TACE (total dosage ranged from 1600 to 5000 cGy). One patient received only radiotherapy (total dosage 1600 cGy) because of having a single tumor and poor liver reserve function. The median survival time among those eight patients who received palliative treatment was 13.4 months (range = 8-26 months), which was significantly longer than for the other two patients without treatment (2 and 4 months). CONCLUSIONS: Icteric type hepatocellular carcinoma (IHCC) is a rare presentation of HCC. Palliative treatment strategies, including TACE and/or R/T showed a beneficial effect in improving the survival time.     

Association of heme oxygenase-1 GT-repeat polymorphism with blood pressure phenotypes and its relevance to future cardiovascular mortality risk: an observation based on arsenic-exposed individuals

Objective

Heme oxygenase (HO)-1 is up-regulated as a cellular defense responding to stressful stimuli in experimental studies. A GT-repeat length polymorphism in the HO-1 gene promoter was inversely correlated to HO-1 induction. Here, we reported the association of GT-repeat polymorphism with blood pressure (BP) phenotypes, and their interaction on cardiovascular (CV) mortality risk in arsenic-exposed cohorts.

Methods

Associations of GT-repeat polymorphism with BP phenotypes were investigated at baseline in a cross-sectional design. Effect of GT-repeat polymorphism on CV mortality was investigated in a longitudinal design stratified by hypertension. GT-repeat variants were grouped by S (<27 repeats) or L (≥27 repeats) alleles. Multivariate analyses were used to estimate the effect size after accounting for CV covariates.

Results

Totally, 894 participants were recruited and analyzed. At baseline, carriers with HO-1 S alleles had lower diastolic BP (L/S genotypes, P = 0.014) and a lower possibility of being hypertensive (L/S genotypes, P = 0.048). After follow-up, HO-1 S allele was significantly associated with a reduced CV risk in hypertensive participants [relative mortality ratio (RMR) 0.27 (CI 0.11, 0.69), P = 0.007] but not in normotensive. Hypertensive participants without carrying the S allele had a 5.23-fold increased risk [RMR 5.23 (CI 1.99, 13.69), P = 0.0008] of CV mortality compared with normotensive carrying the S alleles.

Conclusions

HO-1 short GT-repeat polymorphism may play a protective role in BP regulation and CV mortality risk in hypertensive individuals against environmental stressors.     

Impact of liver diseases on the development of type 2 diabetes mellitus

The prevalence of type 2 diabetes mellitus (T2DM) is higher in patients who have liver diseases such as nonalcoholic fatty liver disease, chronic viral hepatitis, hemochromatosis, alcoholic liver disease and cirrhosis. It is suggested that there is a pathogenic link between the presence of T2DM and the severity of liver injury. However, evidence related to the impact of hepatic inflammation on the development of T2DM has not yet emerged. This article provides an overview of the evidence for an increased pre...