This article presents an update of the results achieved by modern surgery in congenital heart defects (CHDs) over the past 40 years regarding survival and the need for reoperations, especially focusing on the results from the past 2 decades.
From 1971 to 2011, all 7038 patients
Comment In: Circulation. 2015 Jan 27;131(4):328-3025538228
To determine predictors for decision-making on a differential approach to choosing glucocorticosteroids (GCS) for children and adolescents with acute lymphoblastic leukemia (ALL).
The analysis covered 1064 primary patients aged to 1 to 18 years with ALL who had been registered at the clinics of Russia and Belorussia in April 2002 to November 2006. Before induction therapy, the patients were randomized into a dexamethasone (DEXA) 6 mg/m2 group (n=539) and a methylprednisolone (MePRED) 60 mg/m2 one (n=525).
The entire group showed no statistically significant differences in survival rates between the patients receiving DEXA or MePRED. However, an analysis of age groups revealed the benefits of DEXA in children younger than 14 years (the event-free survival (EFS) was 76±2 and 71±2%, respectively (p=0.048); the overall survival (OS) was 81±2 and 77±2%, respectively (p=0.046); therapy-induced mortality was 6.4% (DEXA) andl 1.1% (MePRED) (p=0.01 4); the rate of isolated extramedullary relapses was 1.5% (DEXA) and 4.4% (MePRED) (p=0.009). At the same time, EFS and OS in 14-to-18-year-old adolescents were statistically significantly higher than in those who used MePRED (EFS, 65±6 and 52±6%, respectively (p=0.087); OS, 72±6 and 61±6%, respectively; (p=0.l 7).
The findings suggest that it is possible that the choice of a GCS for ALL therapy must be also based on a patient's age. There is a need for further studies of this matter in prospective randomized multicenter trials in children and adolescents.
Death records were used to analyze cancer mortality in the rural areas of the Kamensky District, Sverdlovsk Region, within the East-Urals radioactive track area. A study group showed a significant increase in cancer mortality as compared with a control group (65 of the 691 examinees; 90% confidence interval (CI) 18-144; the mean colonic radiation dose was 80 and 3 mGy in the study and control groups, respectively). The additional relative risk per colonic dose was 1.3 Gy(-1) (90% CI 0.36-2.9 Gy(-1)). The association of the additional relative risk with the age-related and time factors was studied and revealed.
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is characterized by adrenergically induced ventricular tachycardia (VT) associated with syncope and sudden death.
This study sought to characterize arrhythmias associated with CPVT with respect to provocation by exercise and drugs, electrocardiographic characteristics, and association with long-term outcomes; and to explore the relation between age and clinical presentation.
Seventy patients from 16 families were evaluated with exercise and selective adrenaline challenge, and screened for RyR2 mutations. CPVT was diagnosed in probands with symptoms and stress- or adrenaline-provoked VT, or in asymptomatic relatives with provoked VT or RyR2 mutations. Patients were followed up for recurrent syncope, VT, and sudden death.
Twenty-seven patients including 16 probands were identified (median age 35 years, 67% female). Presentation was cardiac arrest in 33% and syncope in 56%, and 11% were asymptomatic. Polymorphic or bidirectional VT was provoked with exercise in 63% and adrenaline in 82%. The initiating beat of VT was late-coupled and wide (coupling interval 418 ± 42 ms; QRSd 131 ± 17 ms), and QRS morphology suggested an outflow tract origin in 59%. During follow-up of 6.2 ± 5.7 years, 2 patients died despite an implantable cardioverter-defibrillator (ICD), 4 patients received ICD therapy for VT, and 5 patients had inappropriate therapy for supraventricular tachycardia. Patients presenting with late-onset CPVT (age > 21; n = 10) were often female (80%) and less likely to have RyR2 (Ryanodine receptor type 2) mutations (33%), and fatal events were not observed during follow-up (4.1 ± 3.6 years).
Ventricular arrhythmia in CPVT is often initiated from the outflow tract region. Despite ß-blocker therapy and selective ICD implantation, breakthrough arrhythmias occur and may be associated with adverse outcomes.
To estimate the incidence, mortality and cancer risk of the congenital malformation esophageal atresia (EA) in a population-based investigation.
A population-based cohort study of EA patients registered in three nationwide registers in Sweden in 1964-2007. The incidence of EA per total number of live births was assessed. Mortality and cancer occurrence were expressed as standardized mortality ratio (SMR) and standardized incidence ratio (SIR) with 95% confidence intervals (CI). Mortality was further analyzed by Cox regression and expressed as hazard ratio with 95% CI.
The EA cohort comprised 1,126 patients. The mean incidence was 3.16 per 10,000 live births, without any temporal changes (p for trend =0.94). Associated anomalies were present in 42% and chromosomal abnormalities in 5%. EA patients had an almost 12 times higher risk of mortality compared to the background population (SMR 11.8, 95% CI 10.3-13.5). The mortality increase was most pronounced during the first 5 years after birth. Survival improved during the study period (p for trend =0.0001). EA did not entail a strongly increased cancer risk (SIR 0.9; 95% CI 0.2-2.6).
EA has a stable incidence, the survival has improved substantially during recent decades, and the cancer risk might not be increased.
Regularly published national reports on population health in Russian Federation, based on the results of statistic analyses and the data from hygienic and epidemiological studies, demonstrate associations between exposure to hazardous environmental factors and population morbidity. Research into correlations between exposure to such factors and population health status using extensive databases, makes it possible to obtain statistically significant results and put forward hypotheses for epidemiological studies. The aim of this research was to study the levels and dynamics of population mortality, and atmospheric air condition, as well as correlations between them in regional centers of Russian Federation. The study covers the period from 1985 through 1998.
Brain tumors are the second most common neoplasm in childhood and adolescence. With the recent advances in technology, changes in tumor incidence have been reported. This study examines this statement. A 19-year retrospective case review of primary brain tumors in persons younger than 18 years of age at time of diagnosis, who had permanent residence in our catchment area, was performed. Data were examined for changes in presenting symptoms and signs and incidence rates for tumors on the basis of anatomic location and histologic tumor type. An incidence rate of 2.76 per 100,000 people younger than 18 years of age was found. During the period of this study a small, but significant, trend toward increasing incidence was evident. No changes in patterns of presentation or duration of symptoms before diagnosis was observed. The incidence rate based on histologic tumor diagnosis remained fairly constant during the study period.