[Health status and quality of life in ß-thalassemia adults in Marseille, France]. / État de santé et qualité de vie des patients ß-thalassémiques adultes à Marseille, France.

INTRODUCTION: The life expectancy of ß-thalassemia patients has increased over the last 20 years. In this study, we evaluated the current health status and quality of life of these patients managed in a reference center in Marseille. METHODS: This is a single-center, descriptive study conducted between June and August 2019 in patients over 18 years of age with ß-thalassemia major or intermedia. Clinical and paraclinical data were collected retrospectively and the SF-36 health survey questionnaire was proposed to each patient. RESULTS: 43 of 64 selected patients were included and divided into 2 groups: 35 patients with transfusion-dependent ß-thalassemia and 8 patients with non-transfusion-dependent ß-thalassemia. Liver iron overload is the most frequent complication, present in 80% of transfusion-dependent and 62.5% of non-transfusion-dependent patients. Cardiac iron overload is present only in the transfusion dependent ß-thalassemia group (20%). Hypogonadotropic hypogonadism remains the most common endocrine disorder (41.9%) followed by osteoporosis (37.2%). Among the 31 patients who completed the SF-36 questionnaire, physical and mental quality of life scores were lowered in transfusion dependent (respectively 42.7 and 46.8) as in non-transfusion-dependent patients (respectively 43.8 and 28.9). CONCLUSION: Despite an improvement in medical care, our patients with ß-thalassemia show an alteration in their quality of life that will need to be characterized in the entire French cohort.

[Bilateral adrenal hemorrhage under apixaban in primary antiphospholipid syndrome]. / Nécrose hémorragique bilatérale des surrénales sous apixaban : penser au syndrome des antiphospholipides ?

INTRODUCTION: Adrenal hemorrhage is a classical but rare complication of antiphospholipid syndrome, revealing diagnosis in one third of the cases. Anti-vitamin K therapy is the standard treatment but direct oral anticoagulants are discussed as an alternative. In the latest recommendations, it is advised not to use direct oral anticoagulants in the setting of antiphospholipid syndrome. CASE REPORT: We present a case of bilateral adrenal hemorrhage revealing primary antiphospholipid syndrome with triple positive antibody profile, in a 47-year-old man treated by apixaban for previous venous thromboembolism. CONCLUSION: To our knowledge, it is the first case of adrenal hemorrhage occurring during apixaban treatment in a patient with antiphospholipid syndrome. This case illustrates the inefficacy of direct oral anticoagulants to prevent thrombotic events in antiphospholipid syndrome, in accordance with the latest recommendations.

[Therapeutic failure in scabies: An observational study]. / Gales en échec de traitement : étude observationnelle.

BACKGROUND: Several sources suggest an escalation of scabies in France. AIM: To describe a population of patients continuing to present with scabies despite multiple treatments in order to identify factors associated with persistence of infection. PATIENTS AND METHODS: A descriptive cross-sectional study in adults and children consulting for persistent scabies despite at least one previous treatment. A standardized questionnaire explored potential sources of treatment failure. RESULTS: Thirty-one patients were analyzed. Initial symptoms were noted to have started between two and 52 weeks earlier (mean: 19 weeks). The mean number of prior consultations with a general practitioner was 3.1 (0-10) and 1.7 with a dermatologist (0-7). The mean number of patients per household was 3.5 (1-9). At least one dose of oral ivermectin (maximum of 6 doses per household) was prescribed for 84 % of patients (29 % of whom were not fasted at the time). Further, 74 % of patients received at least one local application of esdepallethrin and piperonyl butoxide (maximum: 5 courses), four received benzyl benzoate and two received permethrin; however, 58 % did not reapply the substance after hand washing. All households bought the prescribed treatments despite the costs. Close contacts of patients were treated in 58 % of households. Decontamination of bedding and clothing was carried out properly in 90 % of households. DISCUSSION: Persistence of infection appears to be linked to: (1) insufficient treatment of close contacts; (2) absence of a second treatment between days 7 and 14; (3) insufficient efficacy of the available treatments, doubtless due to multiple factors (intrinsic resistance of Sarcoptes, failure to repeat treatment, poor explanation of methods for dosing and application, and oral intake of treatments). Access to non-reimbursed treatments was not identified as a problem and decontamination of bedding and clothing was correctly performed in most cases. CONCLUSION: Though certain fundamental aspects of scabies treatment must be better known, longer consultations and provision of efficacious treatments are also a priority.

Leptospirosis presenting as honeymoon fever.

An increasing number of travelers from western countries visit tropical regions, questioning western physicians on the prophylaxis, the diagnosis and the therapeutic management of patients with travel-associated infection. In July 2014, a French couple stayed for an adventure-travel in Columbia without malaria prophylaxis. A week after their return the woman presented with fever, myalgia, and retro-orbital pain. Three days later, her husband presented similar symptoms. In both patients, testing for malaria, arboviruses and blood cultures remained negative. An empirical treatment with doxycycline and ceftriaxone was initiated for both patients. Serum collected from the female patient yielded positive IgM for leptospirosis but was negative for her husband. Positive Real-Time PCR were observed in blood and urine from both patients, confirming leptospirosis. Three lessons are noteworthy from this case report. First, after exclusion of malaria, as enteric fever, leptospirosis and rickettsial infection are the most prevalent travel-associated infections, empirical treatment with doxycycline and third generation cephalosporin should be considered. In addition, the diagnosis of leptospirosis requires both serology and PCR performed in both urine and blood samples. Finally, prophylaxis using doxycycline, also effective against leptospirosis, rickettsial infections or travellers' diarrhea should be recommended for adventure travelers in malaria endemic areas.