Prevalence of dyslipidemia in children and adolescents with diabetes mellitus type I

Dyslipidemia is frequently observed in patients with diabetes mellitus and has led to development of screening programs and intervention studies. Dyslipidemia has been identified as an important risk factor for coronary heart disease. 128 patients with type I diabetes attending a single pediatric endocrine clinic underwent anthropometric and biochemical assessment. Anthropometric measurements followed WHO criteria. Blood samples were analyzed for glycated hemoglobin [HbA1C], cholesterol [chol], triglycerides [TG], low density lipoprotein [LDL] and high density lipoprotein [HDL], and blood pressure was recorded. Patients' mean age was 12.6 +/- 4.1 years. Patients' mean age at the onset of diabetes was 7.1 +/- 2.8 years. Mean duration of diabetes was 6.9 +/- 3.2 years. 48.5% of patients had some form of dyslipidemia. 21.4% had isolated hypertriglyceridemia, 11.6% isolated hypercholesterolemia and 15.5% mixed hyperlipidemia. Factors associated with dyslipidemia included longer duration of diabetes, higher mean age, higher mean HbA1C [p 0.001]. Hypertriglyceridemia was more frequent in female patients and subjects with higher BMI [p<0.05]. The mean value of TG 199.9 +/- 74.1 mg/dl, TC 178.5 +/- 29 mg/dl and LDL 141.2 +/- 37 were significantly higher in patients with poor metabolic control [mean value of HbA1C 9.3 +/- 1.8] than the diabetic patients with better control [mean value of HbA1C 7.1 +/- 0.77], TG 156.8 +/- 55.9 mg/dl; TC 143.5 +/- 37.6 mg/dl and LDL 108 +/- 21.2. Our findings indicated that type I diabetic patients with poor metabolic control are at higher risk of developing dyslipidemia. However, given the well documented problems of lifestyle regulation and compliance in optimizing control especially in this age group, we need to develop alternative and simple interventional strategies to improve outcome. Monitoring of lipids should be extended and yearly screening of patients for dyslipidemia recommended

[Congenital adrenal hyperplasia, diagnosis and treatment]

Congenital adrenal hyperplasia [CAH] is an of autosomal recessive disorder of adrenal steroidogenesis leading to a deficiency of cortisol. The deficiency of cortisol results in increased secretion of corticotropin which, in turn leads to adrenocortical hyperplasia and overproduction of intermediary metabolites. In 90% of the cases, with this disease, there is a problem with the enzyme 21 hydroxylase [21 OHD]. Severe and mild forms of these disorders, caused by variations in the severity of the genetic mutations have been reported. Depending on the enzymatic step that is deficient, there may be signs, symptoms and laboratory findings of mineralocorticoid deficiency or excess; incomplete virilization or premature androgenization of the affected male and virilization or sexual infantalism in the affected female. It appears that this disease is more prevalent in Iran because of the custom of family marriages, however its exact prevalence needs correct diagnosis and documentation of cases. In this article the process of the disorders, diagnosis and treatment will be discussed

Report of 285 patients with congenital adrenal hyperplasia and evaluation of approximate prevalence of the disease in Iran

In this study, 285 cases of congenital adrenal hyperplasia who were followed in the Tehran University Hospitals and Institute of Endocrinology and Metabolism are reported. Among these cases, 165 [57.9%] were female and 120 [42.1%], male. The most common type of congenital adrenal hyperplasia in these patients was the salt-losing type of 21-hydroxylase deficiency [57.9%]; 11-hydroxylase deficiency was present in 13.68% of patients. There were only 3 cases with 3-beta hydroxysteroid dehydrogenase deficiency, 2 cases with 17-alphahydroxylase deficiency and one with 20, 22 desmolase deficiency. Presenting complaints were in decreasing order of frequency: ambiguous genitalia, vomiting and dehydration, precocious puberty, hypertension, failure to thrive, hirsutism and primary amenorrhea. The age of patients at the time of diagnosis was between 2 days to 17 years and the most common age was in the first two years of life especially in the neonatal period. A positive family history of the same disease was present in 17 siblings of our patients. [21-OHD = 14 H-OHD = 3]. There were 27 cases of death among these patients [23 male and 4 female that 24 cases had 21-OHD and 2 cases had 3 beta HSD deficiency and one case had 20,22-desmolase deficiency]