Surgical treatment of intersex disorders.

Despite the progress made in understanding the factors regulating sexual differentiation, infants born with ambiguous genitalia face significant problems. The authors reviewed a group of 84 children with ambiguous genitalia managed surgically between 1986 and 1993. The most frequent condition was male pseudohermaphroditism (PM) (58%); 31% had female pseudohermaphroditism. Fifty-seven percent of patients were raised as males and 43% as females. In each group of patients, feminine and masculine reconstructive operations were performed. In only 31% of PM and 60% of PF cases was the diagnosis made within the first 2 months of life. In 41% of PF and 40% of PM patients, treatment was begun before the second year of life, which we consider an acceptable time. The timing and type of vaginoplasty were determined by the point of entry of the vagina into the urogenital sinus. Of the 29 patients reared as females, 22 required perineal vaginoplasty, had pull-through vaginoplasty, and 2 had colovaginoplasty. Since 1986, we have applied Mollard's clitoroplasty, which preserves the neurovascular bundle and is important for experiencing orgasm. Seventeen percent of patients with feminization procedures experienced complications. The optimal time for masculinization procedures is 2 years of age, after obligatory testosterone treatment. If there is utriculus prostaticus (UP) type II or III, it is removed before urethroplasty. This is not done for UP types 0 and 1. In PM cases, the number of feminization and masculinization operations was 2.1 and 4.05 per patient, respectively. It is easier to make a vagina than a phallus, not taking into consideration dimensions, aesthetics, or capability of erection of the phallus.(ABSTRACT TRUNCATED AT 250 WORDS)

[TSH receptor antibodies determined by the Thybia test in children with hyperthyroidism]. / TSH-receptor antitela odredjena Thybia-testom kod dece sa hipertireozom.

Using a radioreceptor assay Thybia-test, we studied 22 girls with Graves' disease, aged 4.0 to 20.4 years (mean = 13.8 years). Sera of 9 girls were obtained before therapy, 20 during treatment with methimazole, 4 in remission and 2 relapse. The total number of tested sera was 113. Control sera were obtained from 19 age-matched controls. The results of Thybia-test were positive in 7 of 9 girls with Graves' disease before treatment (77.8%) and in both girls in relapse. Of 89 samples of sera taken during drug therapy Thybia-test was positive in 51. Only 3 of 13 sera taken in remission gave positive results. The highest mean values of Thybia-test were detected in patients in relapse (46.2%), and in patients before therapy (23.9%). During treatment mean values became lower (14.4%) and were the lowest in remission of the disease (6.9%). The results of Thybia-test in controls ranged from 0% to 7.5% (mean = 1.93 +/- 2.78%). Significant differences were noticed between mean values of Thybia-test of patients before therapy and controls as well as between patients during therapy and controls. Six girls were studied after discontinuation of thyreosuppressive therapy. In 5 results of Thybia-test were negative. Three of them are still in long remission. Two were in short remission and relaps in both of them was followed by convertion of Thybia-test to positive. At the end of treatment the only patient with positive Thybia-test was in remission and Thybia-test was-later converted to negative. Our study shows that Thybia-test is a valuable method in addition to others used to correlate the natural history of the disease with the results of therapy. We are still unable to make a definite statement on the significance of this method in prediction of relapse and remission of Graves' disease.

[Treatment of unstable diabetes mellitus in children and adolescents using insulin administered by a semi-automated syringe, NovoPen]. / Lecenje nestabilonog dijabetes melitusa kod dece i adolescenata insulinom uz pomoc poluautomatske brizgalice-NovoPen.

An insulin device, NovoPen, incorporating human actrapid insulins was used in a group of 6 poor--controlled diabetic adolescents, aged 15 years (mean 8-18.7) with duration of diabetes 8.2 +/- 4.8 years (mean +/- S.D). The study was carried out in insulin-dependent diabetic young-people, to test the efficacy and acceptance of an intensified insulin therapy (an injection of short-acting insulin before each of the three meals using NovoPen plus an evening injection of human ultratard insulin from a conventional syringe) in the hope of achieving the improved control. All patients completed a 6-months NovoPen-therapy and preferred to continue with the multiple injection regimen in the long term. Metabolic control was improved, the values of haemoglobin A1c decreased from 16.8% to 13.4% (statistically significant) during the study. The use of the NovoPen injection device proved to be a safe and acceptable method of therapy; it offered the patients a marked freedom and liberalization of everyday life.

Diagnostic value of hand X-rays in Turner's syndrome.

Previously described radiological signs of Turner's syndrome were evaluated in X-rays of the left hand from 17 patients with Turner's syndrome (age 10.4--15.3 years) and 17 age-matched girls with constitutional short stature. While none of the signs clearly distinguished between the two groups, ballooning of the tips of the terminal phalanges with a high ratio between the tip and mid-shaft diameters seemed to be the most useful of Turner's syndrome. Disproportionately long phalanges in the 4th finger and the presence of a coarse reticular pattern in the carpal bones appeared to be the next most useful signs. The presence of a short 4th metacarpal or a narrow carpal angle, and assessment for Madelung's deformity were of little value in distinguishing between the two groups.