16 research outputs found
Influence of foliar fertilization with manganese on germination, vigor and storage time of RR soybean seeds
Treatment Patterns and Use of Resources in Patients With Tuberous Sclerosis Complex: Insights From the TOSCA Registry
Tuberous Sclerosis Complex (TSC) is a rare autosomal-dominant disorder caused by mutations in the TSC1 or TSC2 genes. Patients with TSC may suffer from a wide range of clinical manifestations; however, the burden of TSC and its impact on healthcare resources needed for its management remain unknown. Besides, the use of resources might vary across countries depending on the country-specific clinical practice. The aim of this paper is to describe the use of TSC-related resources and treatment patterns within the TOSCA registry. A total of 2,214 patients with TSC from 31 countries were enrolled and had a follow-up of up to 5 years. A search was conducted to identify the variables containing both medical and non-medical resource use information within TOSCA. This search was performed both at the level of the core project as well as at the level of the research projects on epilepsy, subependymal giant cell astrocytoma (SEGA), lymphangioleiomyomatosis (LAM), and renal angiomyolipoma (rAML) taking into account the timepoints of the study, age groups, and countries. Data from the quality of life (QoL) research project were analyzed by type of visit and age at enrollment. Treatments varied greatly depending on the clinical manifestation, timepoint in the study, and age groups. GAB Aergics were the most prescribed drugs for epilepsy, and mTOR inhibitors are dramatically replacing surgery in patients with SEGA, despite current recommendations proposing both treatment options. mTOR inhibitors are also becoming common treatments in rAML and LAM patients. Forty-two out of the 143 patients (29.4%) who participated in the QoL research project reported inpatient stays over the last year. Data from non-medical resource use showed the critical impact of TSC on job status and capacity. Disability allowances were more common in children than adults (51.1% vs 38.2%). Psychological counseling, social services and social worker services were needed by <15% of the patients, regardless of age. The long-term nature, together with the variability in its clinical manifestations, makes TSC a complex and resource-demanding disease. The present study shows a comprehensive picture of the resource use implications of TSC
Clinical Characteristics of Subependymal Giant Cell Astrocytoma in Tuberous Sclerosis Complex
BACKGROUND: This study evaluated the characteristics of subependymal giant cell astrocytoma (SEGA) in patients with tuberous sclerosis complex (TSC) entered into the TuberOus SClerosis registry to increase disease Awareness (TOSCA). METHODS: The study was conducted at 170 sites across 31 countries. Data from patients of any age with a documented clinical visit for TSC in the 12 months preceding enrollment or those newly diagnosed with TSC were entered. RESULTS: SEGA were reported in 554 of 2,216 patients (25%). Median age at diagnosis of SEGA was 8 years (range, 18 years. SEGA were symptomatic in 42.1% of patients. Symptoms included increased seizure frequency (15.8%), behavioural disturbance (11.9%), and regression/loss of cognitive skills (9.9%), in addition to those typically associated with increased intracranial pressure. SEGA were significantly more frequent in patients with TSC2 compared to TSC1 variants (33.7 vs. 13.2 %, p < 0.0001). Main treatment modalities included surgery (59.6%) and mammalian target of rapamycin (mTOR) inhibitors (49%). CONCLUSIONS: Although SEGA diagnosis and growth typically occurs during childhood, SEGA can occur and grow in both infants and adults
Newly Diagnosed and Growing Subependymal Giant Cell Astrocytoma in Adults With Tuberous Sclerosis Complex: Results From the International TOSCA Study
The onset and growth of subependymal giant cell astrocytoma (SEGA) in tuberous sclerosis complex (TSC) typically occurs in childhood. There is minimal information on SEGA evolution in adults with TSC. Of 2,211 patients enrolled in TOSCA, 220 of the 803 adults (27.4%) ever had a SEGA. Of 186 patients with SEGA still ongoing in adulthood, 153 (82.3%) remained asymptomatic, and 33 (17.7%) were reported to ever have developed symptoms related to SEGA growth. SEGA growth since the previous scan was reported in 39 of the 186 adults (21%) with ongoing SEGA. All but one patient with growing SEGA had mutations in TSC2. Fourteen adults (2.4%) were newly diagnosed with SEGA during follow-up, and majority had mutations in TSC2. Our findings suggest that surveillance for new or growing SEGA is warranted also in adulthood, particularly in patients with mutations in TSC2
The Epidemiological Assessment of Lifestyle Impact on Childhood and Adolescence Psychiatric Disorders in Kohgiloyeh and Boyer-Ahmad Province, Iran
Background: Children and adolescents are potentially at risk of different psychiatric disorders, and it is important to consider their health, especially their mental health. Objectives: We aimed to design a cross-sectional study to investigate the association between lifestyle-related components with the psychiatric disorders in children and adolescents in Kohgiloyeh and Boyer-Ahmad province (Iran) and to determine its association with different lifestyle-related variables. Methods: In this study, about 1000 children and adolescents aged 6 to 18 years in Kohgiloyeh and Boyer-Ahmad province were selected using stratified cluster random sampling. The reliable Persian version of Kiddie-Sads-Present and Lifetime Version (K-SADS-PL) and the Life Style Questionnaire (LSQ) were instructed using interviewing with participants by our clinical psychologists or, if they were under 14 years, with their parents. Besides, demographic data (i.e., gender, age, location, race, nation, and religion) of participants were also collected. Results: The results showed that, except for the physical activity and well-being, other remaining factors have a significant correlation with psychiatric disorders (P < 0.05). The exact p-value for each factor was as follow: physical health (P = 0.007), sports and well-being (P = 0.057), weight control and nutrition (P = 0.001), prevention of diseases (P = 0.04), mental health-cognitive (P = 0.05), spiritual health (P = 0.008), social health (P = 0.05), drug avoidance (P = 0.001), accident prevention (P = 0.02), and environmental health (P = 0.001). Also, we found that the overall effect of lifestyle was significantly associated with children and adolescent mental disorders (P < 0.05). Conclusions: Our findings show a significant role for lifestyle in the children and adolescents psychiatric disorders. So it should be considered in more detail in future studies. © 2020, Author(s)