Objectives: The International Diabetes Mellitus Practice Study (IDMPS) is a 5-year survey documenting changes in diabetes treatment practices in developing countries. The primary objective of this survey was to assess the therapeutic management of type 2 diabetes mellitus (T2DM) in real-life medical practice. The secondary objectives were to evaluate the clinical management of type 1 diabetes mellitus (T1DM) and to assess the proportion of all diabetic patients failing to reach the glycated haemoglobin (HbA1c) <7% target. Methods: Data were analysed for 738 patients (240 with T1DM and 498 with T2DM) included in wave 5 of the IDMPS in Morocco in 2011. Results: Nearly two-thirds (61%) of T2DM patients were treated with oral glucose-lowering drugs (OGLDs) alone, 13.1% were treated with insulin alone and 23.3% were treated with OGLDs plus insulin. Insulin use was less frequent, was initiated later and involved a greater use of premixes versus basal/prandial schedules compared to other populations evaluated in the IDMPS. The majority (92.5%) of T1DM patients were treated with insulin alone and the remainder received insulin plus an OGLD. Insulin protocols included basal + prandial dosing (37.5%) and premix preparations (41.3%). The recommended target of HbA1c <7% was achieved by only 22.2% of T1DM patients and 26.8% of T2DM patients. More macrovascular but fewer microvascular complications were reported in T2DM compared to T1DM patients. Late complications increased with disease duration so that 20 years after diagnosis, 75.7% of T2DM patients were found to have at least one late complication. Conclusions:The clinical burden of diabetes is high in Morocco and the majority of patients do not achieve the recommended glycaemia target, suggesting that there is a huge gap between evidence-based diabetic management and real-life practice. Better education of patients and improved compliance with international recommendations are necessary to deliver a better quality of diabetic care.

Numerous studies have shown that diabetic nephropathy (DNP) is associated with an elevated risk of progression toward end-stage renal disease (ESRD) as well as increased cardiovascular mortality. The majority of these studies are from the developed countries. The factors leading to the progression of DNP may not be quite the same in the developing countries. The aim of this study was to evaluate the risk factors of progression toward ESRD and mortality among type-1 diabetes (T1D) patients with DNP in a developing country. This prospective study was conducted enrolling 72 patients with T1D in September 2006, including T1D patients with DNP defined as microalbuminuria, proteinuria, and/or renal failure, and following them up for five years. The mean age was 29.5±7.5 years with a mean duration of diabetes of 17 (11-20) years. At the time of enrollment, 43.1% had arterial hypertension, 69.4% had proliferative retinopathy, 44.4 had clinical neuropathy, 25% lived in rural areas, and 51.4% had macroalbuminuria. Progression toward ESRD was observed in 34.7% of cases. In multivariate analysis, diastolic blood pressure (P = 0.006) and blood hemoglobin (P = 0.003) were identified as the risk factors associated with ESRD. Death occurred in 18.3% of cases, including 92.3% on hemodialysis with a median hemodialysis duration of six (1-60) months. In multivariate analysis, the ESRD was identified as risk factor for death (P <0.001). DNP due to T1D remains a disease involving a heavy burden of morbi-mortality and is difficult to manage in a developing country because of the low socioeconomic level of patients and the lack of reliable epidemiological data.

Objective: The prevalence of severe primary IGF1 deficiency (IGFD) is unclear. IGFD must be identified promptly as treatment with recombinant human IGF1 (rhIGF1) is now available. Our objective was to characterize and assess the prevalence of severe primary IGFD in a large cohort of patients evaluated for short stature at a pediatric endocrinology unit in France. Design: Observational study in a prospective cohort. Methods: Consecutive patients referred to our unit between 2004 and 2009 for suspected slow statural growth were included. Patients were classified into eight etiological categories. IGFD was defined by height %K3 SDS, serum IGF1 levels !2.5th percentile, GH sufficiency, and absence of causes of secondary IGFD. Results: Out of 2546 patients included, 337 (13.5%) were born small for gestational age and 424 (16.9%) had idiopathic short stature. In these two categories, we identified 30 patients who met our criterion for IGFD (30/2546, 1.2%). In these 30 patients, we assessed the response to IGF1 generation test, time course of IGF1 levels, and efficiency of GH replacement therapy. The results indicated that only four of the 30 children were definite or possible candidates for rhIGF1 replacement therapy. Conclusion: The prevalence of severe primary IGFD defined using the standard criterion for rhIGF1 treatment was 1.2%, and only 0.2% of patients were eligible for rhIGF1 therapy.

Background. 47XYY syndrome is a rare sex chromosome variation characterized by an additional Y chromosome. Most patients with 47XYY karyotype have normal phenotype. This disorder seems associated with a higher risk of developing behavioral and cognitive problems, tall stature, and infertility in adulthood. Sexual development disorder is a rare finding. We report a first case with an abnormal left coronary artery originating from the pulmonary artery in a 47XYY patient. Case. A one-month-old child was referred for ectopic testis and micropenis. Physical examination revealed facial dysmorphia, micropenis, and curvature of the penis with nonpalpable testis. Laboratory tests showed decreased total testosterone and anti-Mullerian hormone (AMH) levels. Blood karyotyping revealed a 47XYY chromosomal formula. At the age of 3 months, the patient developed dyspnea and tachycardia. Echocardiography revealed an anomalous left coronary artery from pulmonary artery with left ventricular dysfunction requiring surgical revascularization by direct reimplantation of the left coronary artery system. Our second case was a 3-year-old child referred for hypospadias with nonpalpable left testicle. Physical examination showed hypertelorism. Blood karyotyping revealed a 47XYY chromosomal formula. Conclusion. To our knowledge, this is the first case of 47XYY syndrome associated with this congenital heart malformation and a sexual development disorder.

Our results show that the decline of eGFR in the DN of T2D is not directly influenced by BMI and that the major risk factors contributing to this decline remain low-baseline eGFR and increased baseline albuminuria.