Access to fludrocortisone and to hydrocortisone in children with congenital adrenal hyperplasia in the WHO Eastern Mediterranean Region: it takes a village

Congenital adrenal hyperplasia (CAH), if untreated, carries high morbidity and mortality. A higher incidence of CAH is expected in countries where consanguinity is common, such as in the countries of the WHO Eastern Mediterranean Region (EMRO). CAH is managed through lifelong treatment with fludrocortisone and hydrocortisone. In this analysis, performed in the 22 EMRO countries and territories plus Algeria, we review which countries offer a neonatal screening programme for CAH and describe the barriers and opportunities to access oral fludrocortisone and oral and injectable hydrocortisone. Neonatal CAH screening was only available nationally in Qatar, Kuwait and partially in Lebanon and Saudi Arabia. We reviewed the national lists of essential medicines (NEMLs) and found that 13/23 (57%) countries included fludrocortisone and 18/23 (78%) included oral hydrocortisone. Fludrocortisone was not included by any of the low-income countries and oral hydrocortisone was only included by one low-income country. We then contacted paediatric endocrinologists in each country to assess perceived availability of these medicines. Overall, there was a relatively good consistency between inclusion of fludrocortisone and hydrocortisone in the NEML and their actual availability in a country. We propose several mechanisms to improve access, including prequalification by the WHO, a common registration process for groups of countries, pooled procurement, working with local pharmaceutical companies, special access status for medicines not yet registered in a country and compounding. We suggest that access to medicines requires a collaboration between health professionals, families of patients, health authorities, pharmaceutical companies and the WHO

Latitude gradient influences the age of onset of rheumatoid arthritis : a worldwide survey

The age of onset of rheumatoid arthritis (RA) is an important outcome predictor. Northern countries report an age of RA onset of around 50 years, but apparently, variability exists across different geographical regions. The objective of the present study is to assess whether the age of onset of RA varies across latitudes worldwide. In a proof-of-concept cross-sectional worldwide survey, rheumatologists from preselected cities interviewed 20 consecutive RA patients regarding the date of RA onset (RAO, when the patient first noted a swollen joint). Other studied variables included location of each city, rheumatologist settings, latitudes (10A degrees increments, south to north), longitudes (three regions), intracountry consistency, and countries' Inequality-adjusted Human Development Index (IHDI). Data from 2481 patients (82% females) were obtained from 126 rheumatologists in 77 cities of 41 countries. Worldwide mean age of RAO was 44 +/- 14 years (95% CI 44-45). In 28% of patients, RA began before age 36 years and before age 46 years in 50% of patients. RAO was 8 years earlier around the Tropic of Cancer when compared with northern latitudes (p <0.001, 95% CI 3.5-13). Multivariate analysis showed that females, western cities, and latitudes around the Tropic of Cancer are associated with younger age of RAO (R (2) 0.045, p <0.001). A positive correlation was found between the age of RAO and IHDI (r = 0.7, p <0.01, R (2) 0.5). RA often begins at an early age and onset varies across latitudes worldwide. We postulate that countries' developmental status and their geographical and geomagnetic location influence the age of RAO.Peer reviewe

Osteoid osteoma of the patella simulating knee arthritis: Case report

Osteoid Osteoma (OO) is an uncommon benign tumour and causes severe pain, being worse at night, and it responds dramatically to nonsteroidal antiinflammatory medications. An osteoid osteoma of the patella is very rare and if it arises, close to chondral surface differential diagnosis may be challenging. Patients with OO of the patella often present with knee pain that is also a typical symptom of trauma or of other diseases such as arthritis, which are much more common than OO. We present the case of a 19-year-old woman, basket-ballplayer, with a three year history of intense Anterior Knee Pain (AKP) that was first attributed to arthritis. A CT scan was performed that revealed the localization of an osteoid osteoma of the patella. The patient was successfully treated with open surgical technique, and the diagnosis was confirmed after histopathologic analysis. After one year of treatment, there was no relapse of the pain and no residual recurrent tumour. This unusual locati on was at the origin of unexplained pain and delayed diagnosis made so later. Although a rare entity, OO of the patella with its atypical clinical features could be included in the differential diagnosis of persistent anterior knee pain in the young adult. High clinical suspicion is necessary to avoid delay in diagnosis and irrelevant procedures for the patient. Key words: Osteoid osteoma, Knee pain, Intra-articular, Patella, Tumour resectio

A misleading appearance of a common disease: A unique presentation of extra pulmonary and multifocal tuberculosis: case report and literature review

Tuberculosis (TB) continues to be a common cause of infectious disease, and it’s a common illness for vulnerable populations in resource-limited settings. Extra Pulmonary Tuberculosis (EPTB) accounts for about 20% of TB cases&nbsp; worldwide. Until now, the diagnostic of ETB is not initially considered especially in the setting of a vague clinical presentation, particularly when it is a multifocal localization defined as the presence of lesions, affecting at least two extrapulmonary sites, with or without pulmonary involvement. Elsewhere multifocal forms are exceptional even in endemic countries and affect mainly immunocompromised patients. Here, we report an uncommon case of extra pulmonary and multifocal tuberculosis, with vertebral, digestive and lymph node involvement in a young immunocompetent patient. Diagnosis was confirmed by pathology after the surgery. Key words: Tuberculosis, Extra pulmonary, Spine, Infection, Bon

Juvenile angio-Behçet’s disease: report and brain MRI findings of 3 cases

Background: Behçet’s Disease (BD) is a vasculitis of unknown origin; it is characterized by recurrent mouth and genital ulcerations, uveitis and diverse systemic manifestations. It is very rare in children. Vascular tropism is mainly characterized by phlebothrombosis; arterial involvement is less frequent. Case presentations: We report here three cases of juvenile angio-Behçet in two boys aged 11 and 16 years-old and a 14 year-old girl. All three children were admitted for a newly-diagnosed BD characterized by multiple, migrating and recurring phlebothromboses, treated with anticoagulants and corticosteroids and requiring cyclophosphamide pulses, along with a severe uveitis in one patient, having required the addition of azathioprine, with favorable outcome. Complications such as pulmonary embolism and Budd-Chiari syndrome were present in case 3, which improved under immunosuppressants. In order to prevent future thrombosis, anticoagulants were maintained for long periods as well as imunosuppressants. Magnetic Resonance Imaging (MRI) of the brain revealed subclinical findings in the 3 cases. Conclusions: Development of venous thrombosis in juvenile BD cases should not be overlooked and special attention is required for these cases in order to improve their disease outcome. Performing advanced radiologic investigations is useful to detect subclinical cases and delineate the extent of affection. Prognosis remains variable but often bad, depending on the presence of vascular, ocular and neurological complications. Keywords: Juvenile angio-Behçet, Phlebothrombosis, MRI brain, Rare disease, Immunosuppressant