Optimization glucocorticoid therapy in female infants with 21 hydroxylase deficiency

Females with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency usually present to medical care early in life for the ambiguous genitalia and/or salt wasting symptoms with dehydration. Several studies have demonstrated loss of height potency during the first year of life that has considerable consequences on final adult height. Whether this decreased height potential is caused by inadequate suppression of adrenal androgens, excess steroid treatment or the salt wasting state itself is a matter of debate. Besides the challenge of achieving optimum growth velocity, there is the concern about behavioral masculinization and atypical gender identity caused by exposure to high androgens early in life. The objective of the present study is to evaluate management of 21 females with congenital adrenal hyperplasia due to 21-hydroxylase deficiency during the period of infancy. The present study included 21 females presenting to the Diabetic Endocrine and Metabolic Pediatric Unit [DEMPU] for genital ambiguity and/or salt wasting symptoms and diagnosed as 21-hydroxylase deficiency congenital adrenal hyperplasia [21-OHD CAH]. Diagnosis based on clinical, electrolytes, hormonal assay and karyotype was 21-OHD [10 of the salt wasting [SW] type in 12 cases and the non-salt wasting [NSW] type in 9 cases. Initial dose of hydrocortisone acetate was 15 mg/sqm/d in the NSW type, and 30 mg/sqm/d in the SW type. Fludrocortisone acetate [0.05-0.1 mg/d] was given to the 12 cases with SW type along with salt in diet. Follow up was done 3-monthly for 1 year after diagnosis and initiation of treatment with clinical assessment for salt wasting symptoms and signs of dehydration, pigmentation, serum Na and K, 17-OHP, A, and PRA. Length was measured every 3 months and growth velocity was calculated for the first year of treatment. Adjustment of hydrocortisone dose was done so that adrenal androgens [17 OHP and A] were kept < 10 ng/dl; also PRA was kept within normal [

Silent myocardial ischemia in patients with diabetes mellitus during exercise tolerance testing

50 patients with diabetes were compared with 20 nondiabetic subjects, with respect to the prevalence of silent myocardial ischemia, by means of treadmill exercise testing. Results of treadmill exercise testing showed ischemia in 14 of the 50 diabetic patients [mean age 49.2 +/- 1.3] and in 2 of 20 nondiabetic control subjects [mean age 49 +/- 6] [28% VS 10%, p <0.05]. Diabetics with "positive treadmill" had a higher serum total cholesterol and lower serum high density cholesterol than "negative treadmill" diabetics. Diabetic patients with retinopathy had a higher prevalence of silent myocardial ischemia [40%] than those who did not [25%, p <0.05]. Diabetics above the age of 40 years should be screened with treadmill exercise testing

Plasma lipoproteins in patients with acute myocardial infarction

Thirty patients with acute myocardial ifarction [AM] were the subject for this study. All patients were young [mean age 35.3 +/- 3.7 Y.]. and had no diabetes, hypertension or secondary cause for hyperlipidaemia, history of cigarette smoking was present in 28 patients. A family history of coronary artery disease [CAD] was obtained in 8 patients. Non of the patients had xanthomas. 20 Healthy subjects were studied as a control. The mean plasma total cholesterol [TC], low density lipoprotein cholesterol [LDLc] were higher in patients group as compared to normal controls [211.8 +/- 57.7 VS 178.3 +/- 23.7 mg/dL and 140 +/- VS 109.3 +/- 20.4 mg/dL repectively], while the mean high density cholesterol [HDLC] was lower in patient group as compared to normal controls [33.4 +/- 12.4 VS 49.9 +/- 6.7 mg/dL]. The ratio of TCIHDL was higher in patients than controls [6.3 +/- 4.6 VS 3.6 +/- 0.53]. Eight patients with AMI had normal plasma Tc and LDLc, but had a very low levels of HDLc, which indicates that HCLc is a predictive risk factor for CAD independent of Tc or LDLc