Linear growth in thalassemic children treated with intensive chelation therapy. A longitudinal study.

The aim of our study was to investigate the effect of a definite schedule of treatment on the growth of children with beta-thalassemia major. Ten children (6 girls, 4 boys) participated in this longitudinal study. They were treated with transfusions to maintain the levels of hemoglobin above 10.5 g/dl from the time of diagnosis and desferrioxamine mesylate 40 mg/kg/day by subcutaneous pump, 5 days a week, during 7 +/- 1.4 years. The patients were followed up from the age at diagnosis (18.9 +/- 9.8 months) until they reached their adult bone age (19.1 +/- 1.6 and 20.8 +/- 0.8 years in girls and boys, respectively). We observed growth retardation before the age of 10 in 3 out of 4 boys and in 1 out of 6 girls. Between the ages of 10 and 15, the growth failure was evident in other children (1 boy and 2 girls), and became more marked in the above mentioned children. After the age of 15, amelioration of the growth failure was observed in relation to sexual maturity. Eight out of 10 patients attain normal stature in accordance with their target height. We had evidence of poor compliance with the chelation therapy in the 2 children of short adult stature. These patients had higher serum ferritin levels than the children with normal height within the study period: 4,461 +/- 1,469 and 1,429 +/- 479 micrograms/l respectively (p = 0.049). Our study reveals a positive effect of intensive chelation therapy on the linear growth in these patients.

Testicular adrenal-like tissue in a patient with 17 alpha-hydroxylase deficiency.

Testicular adrenal-like tissue (TALT) have been observed in patients with congenital adrenal hyperplasia, and is usually associated with 21-hydroxylase deficiency; in 3 cases with 11 beta-hydroxylase deficiency. We report a case of male pseudohermaphroditism with 17 alpha-hydroxylase deficiency (17OHD) who also had TALT. To our knowledge, this is the first report about the association of 17OHD and TALT. Also, the patient had high levels of serum aldosterone--an unusual finding in patients with 17OHD. A possible pathogenic mechanism is discussed.

[Activity of total cholesterol and its HDL fraction in vasectomized patients. A 3-month follow-up]. / Estudio del comportamiento del colesterol total y su fraccíon HDL en pacientes vasectomizados. Tres meses de seguimiento.

Vasectomy is an ever-increasingly used contraceptive method owing to its effectiveness and easy execution, although various studies have questioned its innocuousness. We have studied various parameters in 70 patients three months before and after the vasectomy was performed. The analytical factors controlled were as follows: glycemia, triglycerides, total cholesterol, HDL-cholesterol, LDL cholesterol, FSH, LH, testosterone and estradiol. Other data handled were arterial tension and body weight index. We questioned them about physical activity, distributing them into three groups, and about family history of arteriosclerosis, distributing them into two groups. Three months after the operation we found an increase in body weight index of 24.11 +/- 2.49 to 24.77 +/- 2.51, with a p less than 0.01. Total cholesterol also rose from 201 Cae 39 mgr% to 208 +/- 38 mgr% with a p less than 0.05. We also observed a drop in arterial tension. Patients with no family history of arteriosclerosis had an increase of total cholesterol of 199 +/- 39 mgr% to 210 +/- 35 mgr% with a p less than 0.001. In the heavy smoker group, total cholesterol also rose from 203 %/- 48 mgr% to 214 +/- 40 mgr% with a p less than 0.05. We discuss our results and our surgical complications and our conclusion in that, based on our study, we cannot assert the vasectomy plays a role as a risk factor of arteriosclerosis.