Incidence and late prognosis of Cushing's syndrome: A population-based study
ABSTRACT The main purpose was to assess the incidence and late outcome of Cushing's syndrome, particularly in Cushing's disease. Information for all patients diagnosed with Cushing's syndrome during an 11-yr period in Denmark was retrieved. The incidence was 1.2-1.7/million.yr (Cushing's disease), 0.6/million.yr (adrenal adenoma) and 0.2/million.yr (adrenal carcinoma). Other types of Cushing's syndrome were rare. In 139 patients with nonmalignant disease, 11.1% had died during follow-up (median, 8.1 yr; range, 3.1-14.0), yielding a standard mortality ratio (SMR) of 3.68 [95% confidence interval (CI), 2.34-5.33]. The SMR was partly attributable to an increased mortality within the first year after diagnosis. Eight patients died before treatment could be undertaken. The prognosis in patients with malignant disease was very poor. Patients in whom more than 5 yr had elapsed since initial surgery were studied separately, including a questionnaire on their perceived quality of health. In 45 patients with Cushing's disease who had been cured through transsphenoidal neurosurgery, only 1 had died (SMR, 0.31; CI, 0.01-1.72) compared with 6 of 20 patients with persistent hypercortisolism after initial neurosurgery (SMR, 5.06; CI, 1.86-11.0). In patients with adrenal adenoma, SMR was 3.95 (CI, 0.81-11.5). The perceived quality of health was significantly impaired only in patients with Cushing's disease and appeared independent of disease control or presence of hypopituitarism. It is concluded that 1) Cushing's syndrome is rare and is associated with increased mortality, in patients with no concurrent malignancy also; 2) the excess mortality was mainly observed during the first year of disease; and 3) the impaired quality of health in long-term survivors of Cushing's disease is not fully explained.
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ABSTRACT: Pituitary adenomas give rise to physical and psychological symptoms, which may persist after biochemical cure. Growing attention has been paid to quality of life (QoL) in these patients. We aimed to systematically analyze QoL assessment methods and QoL outcome in these patients. We conducted a systematic literature search up to January 2014 in PubMed, Web of Knowledge, PsycInfo and EMBASE. 102 papers assessing QoL in patients with a pituitary adenoma were included. In clinical (original) studies in which QoL was the primary outcome parameter (n = 54), 19 studies combined a generic questionnaire with a disease-specific questionnaire. QoL was found to be impaired in patients with active disease relative to controls, and generally improved during biochemical cure. However, no normalization occurred, with patients with remitted Cushing's disease demonstrating the smallest improvement. Somatic factors (e.g., hypopituitarism, sleep characteristics), psychological factors (illness perceptions) and health care environment (rural vs. urban) were identified as influencing factors. Intervention studies (predominantly evaluating medical interventions) have been found to improve QoL. The growing number of studies assessing QoL generally described the negative impact of pituitary adenomas. QoL research in this patient group could be further elaborated by the development of disease-specific questionnaires for prolactinoma and non-functioning adenoma, consequent use of generic and disease-specific questionnaires and using a long-term (longitudinal) follow-up. Surgical and pharmacological interventions improve but not normalize QoL. We postulate that there might be margin for further improvement of QoL, for instance by using psychosocial interventions, in addition to optimal medical treatment.Pituitary 01/2015; DOI:10.1007/s11102-015-0636-7 · 2.22 Impact Factor
Article: Comorbidities in Cushing's disease.[Show abstract] [Hide abstract]
ABSTRACT: Cushing's disease is a rare disorder characterized by overproduction of ACTH from a pituitary adenoma leading to hypercortisolemia that in turn leads to increased morbidity and mortality. Here we review the comorbidities associated with Cushing's disease and their impact on quality of life and mortality. Recent evidence suggests that correction of hypercortisolemia may not lead to complete resolution of comorbidities associated with this condition. In particular, increased cardiovascular risk may persist despite long-term remission of hypercortisolemia. This may be related to persistence of visceral adiposity, adverse adipokine profile, glucose intolerance, hypertension, dyslipidemia, atherosclerosis and a procoagulant phenotype. Prior prolonged exposure to glucocorticoids also may have irreversible effects on the central nervous system, leading to persistent cognitive and mood alterations. Osteoporosis and fractures, especially vertebral fractures, can further add to morbidity and a poor quality of life. Normalization of cortisol levels leads to significant improvement in comorbidities but long-term data regarding complete resolution are lacking and need further study. Early diagnosis and treatment of hypercortisolemia, aggressive management of comorbidities along with long-term follow-up is crucial for the optimal recovery of these patients.Pituitary 02/2015; DOI:10.1007/s11102-015-0645-6 · 2.22 Impact Factor
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ABSTRACT: Maintenance of homeostasis during anaesthesia in the patient with two major metabolic disorders whose systemic effects either compliment or contradict each other is a challenge to the anaesthesiologist. A 25-year-old male patient with Cushing's syndrome and known hyperhomocysteinemia was scheduled for open adrenalectomy. Both these disorders compound the hypercoagulable state and differ in glucose metabolism. In addition, obesity, difficult airway, electrolyte and metabolic derangements that accompany Cushing's syndrome warrant special attention. He was on anticoagulant therapy and inferior vena cava filter following an episode of pulmonary thromboembolism with deep vein thrombosis. Perioperative hydrocortisone was administered. Thoracic epidural catheter was placed at T10-T11 interspace, standard general anaesthesia was administered without nitrous oxide. Patient was extubated following an uneventful procedure and discharged home on 10(th) post-operative day. Understanding the anaesthetic implications and the pathophysiological interactions of multiple metabolic disorders with a potential for multisystem involvement is key to the successful management of these patients.Indian journal of anaesthesia 03/2015; 59(3):182-5. DOI:10.4103/0019-5049.153041