Compliance and satisfaction with deferasirox (Exjade®) compared with deferoxamine in patients with transfusion-dependent beta-thalassemia

Objective

The current standard option for iron chelation therapy (ICT) in transfusion-dependent patients with beta-thalassemia is deferoxamine (DFO). We aimed to compare the compliance with DFO vs. deferasirox (Exjade^®), a novel oral iron chelator, in patients with transfusion-dependent beta-thalassemia.

Methods

In this cross-sectional study, 220 patients from Southern Iran who were receiving DFO or Exjade^® for the last 2 years were investigated in 2012. Satisfaction, compliance, convenience, and life disturbances of the patients with ICT were evaluated. Assessments were performed at four different occasions during 1 year.

Results

According to the results, 114 patients received treatment with DFO and 106 patients were treated with Exjade^®. In comparison with the patients who were treated with DFO, those receiving Exjade^® reported a significantly higher rate of compliance and convenience (P < 0.05). However, no significant difference was observed between the two groups regarding their satisfaction (P > 0.05). In the DFO group, 44.9% of the patients reported irritation and pain at the injection site.

Conclusions

Considering higher rate of compliance and convenience with Exjade^® and the comparable efficacy of these two modalities of ICT documented in previous studies, Exjade^® can be used as a preferable choice of ICT in iron-overload patients with beta-thalassemia.     

Diabetic ketoacidosis associated with Guillain-Barré syndrome with autonomic dysfunction

A 37-year-old woman was admitted in a comatose state, after exhibiting fever and diarrhea. Diabetic ketoacidosis was diagnosed due to an increased blood glucose level (672 mg/dl), metabolic acidosis, and positive urinary ketone bodies. On the fifth hospital day, despite recovery from the critical state of ketoacidosis, the patient suffered from dysphagia, hypesthesia and motor weakness, followed by respiratory failure. Cerebrospinal fluid analysis was suggestive of Guillain-Barre syndrome (GBS). Autonomic dysfunction was manifested as tachycardia and mild hypertension in the acute stage. Marked orthostatic hypotension persisted long after paresis was improved, indicating an atypical clinical course of GBS.     

Remission with Cabergoline in Adolescent Boys with Cushing’s Disease

Cabergoline is a long-acting dopamine receptor agonist used for treatment of patients with uncured Cushing’s disease (CD) and, as a first-line treatment, was used in only limited numbers of patients. This report presents two adolescent boys with CD who were treated with cabergoline. Two adolescent boys with clinical and laboratory findings of CD are presented. No pituitary adenoma was detected by radiological investigation in either patient. Adrenocorticotropic hormone (ACTH) hypersecretion and lateralization was found by inferior petrosal sinus sampling in both patients. The initial cabergoline dose was 1mg/week and was adjusted up to 1.5 mg/week in the second patient, based on his urinary free cortisol (UFC) level. The patients responded to cabergoline treatment with normal UFC levels on the 4th and 6th months of treatment. The boys reached complete remission at the end of the 17th and 24th months, respectively. Cabergoline is effective in the control of cortisol secretion and can be considered as a first-line treatment in cases of CD. Conflict of interest:None declared.     

Lupus patients with fatigue-is there a link with fibromyalgia syndrome?

OBJECTIVE: To determine whether fibromyalgia syndrome (FMS) was more common in patients with lupus who were complaining of fatigue. METHODS: We interviewed 216 patients attending two lupus clinics, all of whom fulfilled the revised American College of Rheumatology (ACR) criteria for lupus. The patients completed a questionnaire and were examined to determine the presence of fatigue and whether they fulfilled the ACR criteria for FMS. Disease activity was measured using the British Isles Lupus Assessment Group (BILAG) index and the Systemic Lupus International Collaborating Clinics (SLICC)/ACR damage score. Measurements of erythrocyte sedimentation rate, complement C3, lymphocyte count and DNA titre were also performed. RESULTS: Fifty per cent of our patients complained of fatigue, but only 10% of these patients fulfilled criteria for FMS. FMS did not correlate with any measure of disease activity although patients with FMS had lower mean DNA antibody titres and mean SLICC/ACR damage scores. CONCLUSION: A minority of lupus patients with fatigue fulfil the ACR criteria for FMS. Other possible factors leading to fatigue should be considered.     

Experiences of living with asthma – a focus group study with adolescents and parents of children with asthma

Objective: The goal for asthma treatment is that every individual, so far as possible, shall live without symptoms and exacerbations. Patients and health care professionals sometimes have different perceptions of what is important for achieving good quality of life. This work aims to describe the experiences among adolescents as well as those of parents with young children living with asthma. Methods: Four focus group interviews were performed, two with parents of young children and two with adolescents. The data were qualitatively analyzed, using Systematic Text Condensation. Result: Three themes relevant to the participants’ experiences of living with asthma were presented; strategies, frustrations and expectations. The adolescents wanted to be like their peers and developed their own strategies for self-management of asthma, which included not always taking medication as prescribed. The parents emphasized frustration regarding not being believed, lack of understanding feelings of loneliness, or anxiety. One identified expectation was that the participants wanted to be met with competence and understanding in asthma care from health care professionals. Another expectation expressed among parents was that teachers in nursery and primary schools should have more knowledge and understanding on how to care for children with asthma. Conclusion: Living with asthma leads to developing personal strategies in self-management of asthma. Moreover both parents and adolescents had expectations of being met by competent and understanding health care professionals. Developing a partnership between patients and health care professionals could be a successful way to improve the care of patients with asthma.     

Should all patients with metabolic syndrome be treated with statins?

The metabolic syndrome is a constellation of cardiovascular risk factors that include atherogenic dyslipidemia, elevated blood pressure, abdominal/truncal obesity, and glucose intolerance. Current National Cholesterol Education Program (NCEP) recommendations for specific treatment of the metabolic syndrome are centered on therapeutic lifestyle changes. A recent publication from the NCEP Adult Treatment Panel III suggests pharmacologic intervention for parameters of metabolic syndrome, including dyslipidemia, hypertension, and hyperglycemia, to prevent cardiovascular disease, based on risk category. This review looks at the clinical trial evidence to support the use of statins in metabolic syndrome.     

Do patients with obstructive sleep apnea wake up with headaches?

BACKGROUND: There is a controversy regarding the association of obstructive sleep apnea (OSA) and morning headaches. This study investigates whether this relationship exists. METHODS: This is a retrospective study of 80 consecutive patients with OSA who underwent sleep polysomnography from December 1996 to March 1997. Patients were interviewed about their headache history. Headaches were classified according to International Headache Society criteria and the severity graded by the Chronic Pain Index. Headache characteristics were compared with those of 22 control patients with periodic limb movement disorder. Headache response to continuous positive airway pressure or uvulopalatopharyngoplasty in the patients with OSA was also assessed. RESULTS: Forty-eight (60%) patients had headaches in the year prior to study. Twenty-five of the 48 patients had headaches that did not fit any category. Of these 25 patients, 23 (48% of total group) reported awakening headaches. These awakening headaches were significantly more common in the OSA group compared with the periodic limb movement disorder group, 9 (41%) of whom had headaches, none of which occurred on awakening. The proportion of common types of headaches in both groups was similar. The awakening headaches were brief (shorter than 30 minutes), and their occurrence and severity correlated with OSA severity. Of the 29 patients with OSA who were treated with continuous positive airway pressure or uvulopalatopharyngoplasty, awakening headaches improved by a mean of 80% compared with minimal improvement of migraine, tension, and cervicogenic headaches. CONCLUSIONS: Awakening headaches are associated with OSA. These headaches are of brief duration, and their occurrence and severity increase with increasing OSA severity. Treatment of OSA with continuous positive airway pressure or uvulopalatopharyngoplasty can reduce these headaches.     

Is 'knowing people with HIV/AIDS' associated with safer sex in men who have sex with men?

OBJECTIVE: To examine the sexually protective role of knowing person(s) with HIV/AIDS (PWHA) by conducting a multidimensional analysis distinguishing the number of PWHA known (by disease status and relationship category) and aspects of the relationship with the closest PWHA (emotional closeness, length of time known, disease status, type of relationship). DESIGN: Cross-sectional study of white, Latino, and African-American men who have sex with men recruited at street locations in West Hollywood, California, in 1997. METHODS: The analyses conducted with linear regression models focused on men (n = 334) who reported that they were seronegative or of unknown serostatus and thus at risk for HIV infection. Unprotected sex was defined as percentage of anal intercourse partners in the past 12 months with whom unprotected anal intercourse (UAI) occurred at least once. RESULTS: The number of PWHA known was not associated with the percentage of UAI partners in multivariate or univariate analyses. Greater emotional closeness to a person who was HIV-positive without AIDS was associated with reduced UAI in multivariate models even after excluding participants whose close PWHA was a lover or sex partner. Younger men (18-25 years) knew fewer PWHA, reported less emotional closeness to a PWHA, and had a higher level of UAI than did older men. CONCLUSIONS: Emotional closeness to a seropositive person without AIDS may be a sexually protective factor. The results suggest the possibility that lower levels of emotional closeness to a PWHA may partially underlie the elevated sexual risk behavior of younger men who have sex with men.