ABSTRACT
Background: Although differentiated thyroid carcinoma (DTC) is the most frequent endocrine pediatric cancer, it is rare in childhood and adolescence. While tumor persistence and recurrence are not uncommon, mortality remains extremely low. Complications of treatment are however reported in up to 48% of the survivors. Due to the rarity of the disease, current treatment guidelines are predominantly based on the results of small observational retrospective studies and extrapolations from results in adult patients. In order to develop more personalized treatment and follow-up strategies (aiming to reduce complication rates), there is an unmet need for uniform international prospective data collection and clinical trials. Methods and analysis: The European pediatric thyroid carcinoma registry aims to collect clinical data for all patients ≤18 years of age with a confirmed diagnosis of DTC who have been diagnosed, assessed, or treated at a participating site. This registry will be a component of the wider European Registries for Rare Endocrine Conditions project which has close links to Endo-ERN, the European Reference Network for Rare Endocrine Conditions. A multidisciplinary expert working group was formed to develop a minimal dataset comprising information regarding demographic data, diagnosis, treatment, and outcome. We constructed an umbrella-type registry, with a detailed basic dataset. In the future, this may provide the opportunity for research teams to integrate clinical research questions. Ethics and dissemination: Written informed consent will be obtained from all participants and/or their parents/guardians. Summaries and descriptive analyses of the registry will be disseminated via conference presentations and peer-reviewed publications.
ABSTRACT
AIM: The aim was to assess perceived psychological stress, emotional state, and quality of life (QoL) in men and women with congenital adrenal hyperplasia (CAH) in comparison to age- and sex-matched control individuals. METHODS: Participants: 26 patients with CAH (11 men, 15 women, age 14-48 years) and 26 age- and sex-matched control individuals without CAH. Psychological assessment: Perceived Stress Scale, Profile of Mood States2, and WHO Brief Quality of Life Questionnaire. RESULTS: There were no significant differences in perceived stress, emotional state and QoL scores between men with CAH and control men. Perceived distress (median 14.0 score vs. 10.0 score, p = 0.020), tension-anxiety (median 9.0 score vs. 3.5 score, p = 0.026), depression-dejection (median 12.0 score vs. 5.0 score, p = 0.040), fatigue-inertia (median 13.0 score vs. 7.5 score, p = 0.007) were higher and environmental domain of QoL (median 16.0 score vs. 17.0 score, p = 0.001) was lower in women with CAH than in control women. CONCLUSION: Women with congenital adrenal hyperplasia have higher perceived distress, tension-anxiety, depression dejection, fatigue-inertia, and worse environmental aspect of quality of life than age-matched control women, while perceived psychological stress, emotional state and quality of life in men with congenital adrenal hyperplasia do not differ from that of age-matched control men.
