ADRENOCORTICAL FUNCTION IN CHILDREN WITH PRECOCIOUS SEXUAL DEVELOPMENT DURING TREATMENT WITH CYPROTERONE ACETATE

Adrenal function was studied in thirty-two children with precocious sexual development who were being treated with cyproterone acetate (CPA) at doses ranging from 68 to 175 mg. m2. day for periods lasting from 2 to 79 months. In eighteen children the adrenocortical function evaluation was made before and during CPA treatment. In these eighteen patients, the mean basal plasma cortisol level during the morning hours was 11.2 +/- 4.6 micrograms/dl (m +/- SD) before treatment and fell significantly to 7.2 +/- 4.1 micrograms/dl (P less than 0.02) during therapy. In fifteen patients tested during insulin hypoglycaemia the cortisol peak fell from 21.6 +/- 5.5 micrograms/dl before treatment to 16.7 +/- 6.8 micrograms/dl (P less than 0.05) during CPA therapy. There was a significant inverse correlation between this peak and the dose of CPA but no correlation was found between the cortisol response and duration of treatment. In eight of twenty patients tested, urinary free cortisol levels were undetectable during treatment. No change in basal plasma ACTH levels were demonstrated using standard radioimmunoassay techniques. In the patient receiving the highest dose of CPA and showing complete suppression of the adrenal axis, prolonged stimulation with ACTH-Depot demonstrated a responsive adrenal gland. Addition of a replacement dose of cortisol to the CPA treatment led to the rapid development of the typical signs of Cushing's syndrome. It was concluded that despite the evidence of adrenal suppression by CPA, cortisol supplementation is not necessary and may not even be contraindicated.     

ADDISON'S DISEASE ASSOCIATED WITH PRECOCIOUS SEXUAL DEVELOPMENT IN A BOY

ABSTRACT. Marilus, R., Dickerman, Z., Kaufman, H., Varsano, I. and Laron, Z. (Institute of Pediatric and Adolescent Endocrinology, Steroid Laboratory and Pediatric Department B, Beilinson Medical Center, Petah Tikva, Sackler School of Medicine, Tel Aviv University, Israel). Addison's disease associated with precocious sexual development in a boy. Acta Paediatr Scand, 70:587,.–An eleven-year-old boy was diagnosed to have Addison's disease with an ACTH level of over 4 500 pg/ml after a short episode of fever and dehydration. From the age of 6 years his skin and buccal mucosa had been hyperpigmented but there were no other subjective complaints. The clinical examination also revealed signs of advanced puberty for his age (3rd-4th stage according to Tanner's criteria), with advanced bone age (13 years). The elevated levels of plasma testosterone and gonadotropins and their response to LH-RH stimulation were appropriate for his clinical stage of puberty and supported the diagnosis of true precocious puberty. It is proposed that the concomitant appearance of Addison's disease and precocious puberty is due to a "drift" phenomenon of LH-RH and/or gonadotropins following the prolonged elevation of ACTH secretion.     

UNILATERAL ADRENALECTOMY AND PITUITARY IRRADIATION IN THE TREATMENT OF ACTH-DEPENDENT CUSHING''S DISEASE IN CHILDREN AND ADOLESCENTS

In four juvenile patients with Cushing's disease, the therapeutic approach used was unilateral adrenalectomy followed by irradiation of the pituitary. The follow-up time of these patients has ranged from 1.5 to 10 years. All four are clinically well. Concentrations of adrenal steroids are within normal limits and they require no additional medication. It is felt that this method gives young patients the chance for normal growth and pubertal development as well as a normal social life, so important in the stressful adolescent years, thus avoiding the handicap incurred by bilateral adrenalectomy and the consequent requirement for continuous substitution therapy.     

Teaching clinical skills to pre-clinical medical students: integration with basic science learning

Clinical skills are usually learned by pre-clinical students in a manner divorced from their basic science foundations. The value of previously learned basic sciences thus fails to be re-enforced. A clinical skills course was developed for an experimental curriculum of medical students in their first year. It was organized and taught by a team of basic and clinical scientists and emphasized the basic pathophysiological principles underlying clinical skills. Sessions were supported by related basic science audiovisual resources and a series of clinical problems with questions obliging the student to reason through basic-science mechanisms. Over the span of the course, Students' interest shifted dramatically from a focus on proficiency in motor skills to an understanding of basic pathophysiological mechanisms underlying observed phenomena. Compared to conventional curriculum students, those in the experimental curriculum failed to show a diminution in perceived value of basic sciences in their future career and, on cumulative, cognitive examinations, scored equally in basic science, but significantly higher in clinical science subjects. A clinical skills course integrating both teachers and concepts from basic, as well as clinical sciences can improve student attitudes toward the basic sciences.     

Prevention of asthma

Infants born of allergic mothers but normal fathers, who had eczema and who were fed cows' milk, had a significantly greater incidence of asthma (P < 0.001) than infants with a similar history but who were breast-fed. An analysis of all breast-fed infants in the study showed that they were less likely to develop asthma than those who were bottle-fed (P < 0.06). There was a lower incidence of allergy in infants born of families with allergic mothers and normal fathers, than in families in which both parents were allergic (P < 0.02). In skin tests of both breast or bottle-fed babies, the two most common allergens eliciting reactions were egg and cat dander.     

AUTONOMOUS OVARIAN HYPERFUNCTION FOLLOWED BY GONADOTROPHIN-DEPENDENT PUBERTY IN McCUNE-ALBRIGHT SYNDROME

A 5-year-old girl with the McCune-Albright syndrome presented with precocious puberty secondary to autonomously functioning ovarian cysts, followed by true central puberty. Progression from gonadotrophin-independent to gonadotrophin-dependent precocious puberty may occur from elevated sex steroid levels leading to the early maturation of the hypothalamic-pituitary axis.