Pulmonary arterial hypertension associated with congenital heart disease: lessons learnt from the large Turkish Nationwide Registry (THALES).

Pulmonary hypertension is a group of diseases, including pulmonary arterial hypertension associated with congenital heart disease (APAH-CHD), characterized by progressive deterioration in pulmonary hemodynamics associated with substantial morbidity and mortality risk. THALES is a national multicenter, prospective observational registry, providing data on patients with APAH-CHD. The study comprised APAH-CHD patients (>3 months of age) with confirmed diagnosis of right heart catheterization or echocardiographic findings. Initial and follow-up data were collected via regular hospital visits. Descriptive statistics are used for definitive purposes. Overall, 1034 patients aged 3 months-79 years (median 11.2 [Q1-Q3: 2.2-24.3] years) with APAH-CHD were enrolled at 61 centers, 50.3% being retrospectively enrolled. Most had either Eisenmenger's syndrome (49.2%) or systemic-to-pulmonary shunts (42.7%). Patients were mostly in functional class I-II at the time of diagnosis (46.6%). Mean 6-min walk distance (6MWD) was 369 ± 120 m. Mean pulmonary arterial pressure was 54.7 ± 22.2 mmHg for the whole group, and was highest in patients with Eisenmenger's syndrome. Targeted therapies were noted in 398 (38.5%) patients (monotherapy in 80.4%). Follow-up data were available in 506 patients. Survival at 140 months was 79% and was associated with baseline 6MWD >440 m (p = 0.009), brain natriuretic peptide level < 300 ng/L (p < 0.001). Follow-up 6MWD >165 m (p < 0.0001), brain natriuretic peptide level <300 ng/L (p = 0.031), and targeted therapies (p = 0.004) were also predictive of survival. THALES is the largest registry dedicated to APAH-CHD to date and provides important contributions on demographics, clinical characteristics, and gaps in disease management.

Psychosexual and body image aspects of quality of life in Turkish breast cancer patients: a comparison of breast conserving treatment and mastectomy.

AIMS AND BACKGROUND: Assessing psychosexual and body image aspects of quality of life in Turkish breast cancer patients treated by either mastectomy or breast conserving treatment (BCT). METHODS: The study group consisted of 112 patients who had undergone all treatment at a single institution under the care of a multidisciplinary breast team. Following surgery, all patients underwent adjuvant radiotherapy with or without chemotherapy and hormone therapy. At the time of this study all patients were disease free with at least 2 years' follow-up. Twenty percent of the patients were premenopausal and 80% postmenopausal. The patients completed a questionnaire consisting of 42 questions related to their sexual relations and body image. RESULTS: Forty-one percent of sexually active patients had experienced a deterioration of sexual functioning after treatment. This was mainly due to loss of libido (80%), loss of interest in partner (54%), and sexual dissatisfaction (59%). Problems tended to develop early in the course of treatment. Decreased sexual desire was significantly more frequent in patients undergoing mastectomy versus BCT (80% vs 61%; P = 0.043) and in premenopausal versus postmenopausal patients (P = 0.024). Although 80% of patients were satisfied with their appearance as a whole, only 54% liked their naked bodies. There was no significant difference in body image scores between patients undergoing mastectomy or BCT apart from a general feeling of physical unattractiveness in mastectomy patients (3.4 vs 2.8; P = 0.03). CONCLUSIONS: Significant, similar psychosexual and body image problems occur in patients treated for breast cancer with either mastectomy or BCT. Problems arise early in the course of the disease and therefore detection and treatment of these problems should be addressed during the patients' initial assessment and at the start of treatment. These findings are similar to those reported on similar groups of treated women in American and European populations.

Assessment of treatment-related thyroid dysfunction in patients with head and neck cancer.

OBJECTIVE: To assess thyroid dysfunction in head and neck cancer patients who have received external beam radiotherapy according to radiotherapy fields and dose, tumor site and other local or systemic treatments retrospectively and prospectively and propose a follow-up schedule. MATERIAL AND METHODS: A total of 378 patients was classified into two groups. Group I (n = 345) consisted of surgically treated 153 laryngeal, 80 nasopharyngeal and 112 oral cavity/oropharyngeal carcinoma patients; these patients were evaluated retrospectively for treatment-related thyroid dysfunction using their data files. Group II included 33 patients with head and neck cancer who were evaluated prospectively. Thyroid function tests were performed at the beginning of the radiotherapy and every three months after the radiotherapy course, and thyroid dysfunction regarding surgery, radiotherapy and chemotherapy was evaluated. RESULTS: In Group I, the median follow-up for 153 operated laryngeal carcinoma patients was 44 months. Four (2.6%) of them were found to have clinically apparent hypothyroidism. After a median follow-up of 36 months, none of the 80 nasopharyngeal carcinoma patients showed signs of hypothyroidism. Clinically apparent hypothyroidism was detected in only 1 (0.8%) of the oral cavity/oropharyngeal carcinoma patients after a median follow-up of 25 months. In Group II, 1 (3%) patient was found to have thyroid dysfunction postoperatively prior to radiotherapy. At the time of analysis, 29 (87.8%) patients were euthyroidic, 2 (6.1%) patients had subclinical and 2 (6.1%) patients had clinical hypothyroidism. All patients with thyroid dysfunction have had combined surgery and radiotherapy, and none of the patients treated with radical radiotherapy has experienced hypothyroidism. CONCLUSIONS: Even after a short follow-up, the incidence of thyroid dysfunction was 12.2% in head and neck cancer patients treated with combined surgery and radiotherapy. We recommend thyroid function tests in these patients prior to and once every 3-6 months after the radiotherapy course.

Turkish people's knowledge of cancer and attitudes toward prevention and treatment.

BACKGROUND: The basic level of cancer knowledge of the population is as important in controlling cancer as diagnostic tools, screening, and new approaches to prevention, early diagnosis, and treatment. METHODS: A questionnaire consisting of 24 questions was given to 630 healthy Turkish people to determine their basic knowledge and attitudes regarding cancer. RESULTS: The majority of respondents (78%) were found to have little knowledge of cancer despite the fact that the average level of education of the sample was superior to that of the general population. Educational level was the major predictive factor influencing the basic knowledge and attitudes of the respondents. CONCLUSION: The overall education of the public as well as the basic level of knowledge of cancer must be improved in cooperation with oncology societies and media in order to overcome the cancer burden in Turkey.

Validation of the Medical Research Council and a newly developed prognostic index in patients with malignant glioma: how useful are prognostic indices in routine clinical practice?

Although different prognostic indices for malignant gliomas have been developed, their validity outside of clinical trials has not been widely tested. The aim of this study was to determine whether the Medical Research Council (MRC) brain tumour prognostic index was able to stratify patients for survival managed in routine practice, and secondly to compare the results with our newly developed prognostic score which included tumour grade and only 3 prognostic groups. The MRC and the new prognostic index were calculated for a group of 119 adult patients with malignant glioma managed by surgical resection/biopsy and post-operative radiotherapy. For the MRC and new score, 6 and 3 prognostic groups were defined, respectively. For all patients median survival was 11 (2-66) months. The overall survival rate at 12 and 24 months were 43% and 18%, respectively. The MRC median and two-year survival rates were 14 months and 26% for a score of 1-10, 14 months and 27% for a score of 11-15, 13 months and 22% for a score of 16-20, 8 months and 10% for a score of 21-25, 8 months and 0% for those scoring 26-33. There was only one patient in the 34-38 group. For the new prognostic index, median and two-year survival rates were respectively 16 and 26%; 12 and 23%; 8 and 7% for the good, intermediate and poor prognostic groups. Both indices were significant factors for survival in univariate analysis (MRC index, p = 0.0089, new index p = 0.0002), but not in multivariate analysis. Both the MRC and our newly devised prognostic score were able to separate patients into good and poor prognostic groups, which may aid in treatment decisions, although there was less differentiation between the MRC groups especially over the first year. Both scores use routinely available factors. However, inclusion of tumour grade in the new score may be an advantage over the MRC index.