Treatment outcomes and prognostic factors for non- malignancy associated secondary hemophagocytic lymphohistiocytosis in children.

BACKGROUND: Secondary hemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome that requires prompt diagnosis and appropriate treatment. A risk-stratification model that could be used to identify high-risk pediatric patients with HLH who should be considered for second-line therapies, including salvage regimens and allogeneic hematopoietic cell transplantation (HCT), was developed. METHODS: The medical records of 88 pediatric patients (median age 1.4 years, range 0.2-15 years) with non-malignancy associated secondary HLH were retrospectively reviewed. Treatment strategies included dexamethasone, etoposide, and cyclosporine. RESULTS: Survival analysis showed HLH patients with infections other than Epstein-Barr virus (EBV) and unknown causes experienced better 5-year overall survival (OS) than patients with HLH due to autoimmune disease, EBV or immunodeficiency (76% vs. 65, 33.3, 11%, p < 0.001). On multivariate analysis, among all patients, non-response at 8 weeks was the most powerful predictor of poor OS. When treatment response was excluded, hemoglobin < 60 g/L and albumin < 25 g/L at diagnosis were associated with poor OS. In patients with EBV-HLH, hemoglobin < 60 g/L at diagnosis was associated with poor OS. A prognostic risk score was established and weighted based on hazard ratios calculated for three parameters measured at diagnosis: hemoglobin < 60 g/L (2 points), platelets < 30 × 109/L (1 point), albumin < 25 g/L (2 points). Five-year OS of low-risk (score 0-1), intermediate-risk (score 2), and poor-risk (score ≥ 3) patients were 88, 38, and 22%, respectively (p < 0.001). CONCLUSIONS: These findings indicate that clinicians should be aware of predictive factors at diagnosis and consider 8-week treatment response to identify patients with high-risk of disease progression and the need for second-line therapy and allogeneic HCT.

An association and meta-analysis study of 4 SNPs from beta-2 adrenergic receptor (ADRB2) gene with risk of asthma in children.

BACKGROUND: Although various case-control studies have been conducted to investigate the relationship between ADRB2 gene polymorphisms and asthma risk in different population groups, the results have been conflicting and inconclusive. OBJECTIVE: We performed a case-control study to investigate the association of 4 SNPs in the ADRB2 gene with risk of asthma in children, and then conducted a meta-analysis by combining the previous studies. METHODS: A total of 340 patients and 340 age-matched healthy controls were recruited. All of the subjects were genotyped using the PCR-based invader assay. The case-control study was performed to define the contribution of rs1042713, rs1042714, rs1800888, and rs1042711 to the predisposition of asthma. Additionally, we further conducted a meta-analysis of the study findings together with those of previously reported studies. RESULTS: No significant association was found between the polymorphisms rs1042713, rs1042714, rs1800888, and rs1042711 and asthma in the current case-control study. The meta-analysis confirmed that there was no positive association of these SNPs with asthma in children in Asia, South America, Europe and the overall population. CONCLUSIONS: None of the four polymorphisms in ADRB2 gene were associated with a risk of asthma in a current children population.

Coronary anomaly: anomalous right coronary artery originates from the left sinus of Valsalva and coursing between the pulmonary artery and aorta.

Coronary artery anomalies (CAAs) are present at birth, but are usually asymptomatic and are found during coronary angiography or multi-slice computed tomography (MSCT) detection. The most common coronary anomaly is the separating origin of left anterior descending coronary artery (LAD) and left circumflex artery (LCX) from the left sinus of Valsalva, and this variant is benign. Herein, we present three extremely rare cases of anomalous right coronary artery (RCA) detected incidentally during routine coronary angiography and confirmed by multi-slice computed tomography (MSCT) technique. All the anomalous right coronary artery coursed between the pulmonary artery and aorta. We discuss how to make an accurate diagnosis for appropriate management.

Clinical investigation of transradial access for emergent percutaneous coronary intervention in patients with acute myocardial infarction.

BACKGROUND: Use of intensive anticoagulation and antiplatelet therapy in acute myocardial infarction (AMI) potentially increases the risk of bleeding complications during percutaneous coronary intervention via the transfemoral route. Recently, the transradial access has been intensively employed as an alternative means for diagnostic and interventional procedures. A low incidence of vascular access site bleeding complications suggests that the transradial access is a safe alternative to the transfemoral technique in patients with AMI. The safety and efficacy of transradial access for emergent percutaneous coronary intervention in patients with AMI has not been investigated in the People's Republic of China. METHODS: We analyzed data from our single-center registry on 596 consecutive patients between October 2003 and October 2010. The patients were retrospectively divided into a transradial group (n = 296) and a transfemoral group (n = 300). A dedicated doctor was appointed to collect the following data: puncture time, coronary angiography time, percutaneous coronary intervention time, X-ray exposure time, duration of hospitalization, and complication rates associated with puncture, such as puncture site bleeding, hematoma, pseudoaneurysm, and major adverse cardiac events. RESULTS: There were no significant differences in baseline characteristics and angiographic findings between the two groups. There were also no significant differences in coronary angiography time (8.2 ± 2.4 versus 7.6 ± 2.0 minutes), percutaneous coronary intervention time (30 ± 6.8 versus 29.6 ± 8.1 minutes), or X-ray exposure time (4.6 ± 1.4 versus 4.4 ± 1.3 minutes) between the groups. There were significant differences in puncture time (4.4 ± 1.6 versus 2.4 ± 0.8 minutes) and duration of hospitalization (3.2 ± 1.6 versus 5.4 ± 1.8 days) between the groups (P < 0.001). The complication rate using transradial access was 2.03% (6/296) versus 6.0% (18/300) using transfemoral access (P < 0.0001). CONCLUSION: Transradial access for emergent percutaneous coronary intervention is safe and effective in patients with AMI, and it is suggested that this route could be used more widely in these patients.