Tehran University Medical Journal

---

Background: This study was performed in order to detect the frequency of different types of precocious puberty, predisposing factors and to show which group of patients need to treatment.

Materials and Methods: In this study, 74 patients who referred to pediatric endocrine clinic of Imam Khomeini Hospital and private office from 1993-2000 were assessed. A complete history was taken and physical examination was done in all patients by pediatrics endocrinologist. Bone age was done in all patients and specific hormonal tests were measured in some of them.

Results: Among 74 patients, 50 cases (67.6 percent) were female and 24 cases (32.4 percent) were male. The age of the patients was between 0.8-10 years old with mean 5.37 year. 75 percent of male patients had central (complete) precocious puberty and 25 percent had peripheral (incomplete) precocious puberty. 40 percent of female patients had central precocious puberty, 46 percent had premature telarche, 8 percent had premature pubarche, 2 percent had premature menarche and 4 percent had other kinds of precocious puberty (one case with hypothyroidism and one case with Mccune-Albright syndrome). 10 percent of female patients with central precocious puberty had predisposing factor and 90 percent of them were idiopathic. 38.8 percent of male patients with central precocious puberty had predisposing factor and 61.2 percent of them were idiopathic. 100 percent of male patients with peripheral precocious puberty had congenital Adrenal Hyperplasia. 38 percent of female patients and 100 percent of male patients needed to treatment.

Conclusion: According to this study and references, precocious puberty in female usually benign but in male patients it should be evaluated thoroughly because they usually have a serious disorder which needs treatment.

---

The development of testicular masses in male patients with congenital adrenal hyperplasia due to 21 hydroxylase deficiency has been recognized for many years. We present here the eighth and ninth reported patient with bilateral testicular tumors associated with 11 hydroxylase deficiency. They were two brothers aged 7.5 and 5 yr. who had bilateral testicular tumors and diagnosed because of signs and symptoms of true precocious puberty and high blood pressure. The patients testicular enlargement is suggestive of bilateral testicular adrenal rest.

---

Ambiguous genitalia means difference between gonads morphology and external genitalia. Gender assignment and surgical correction must be done as early as possible to assure unambiguous bonding between the parents and the child. The purpose of this study was to evaluate Ambiguous genitalia in a referral children surgical center.
Materials and Methods: We retrospectively reviewed records of 200 patients admitted to children medical center hospital in a period of 16 years for Ambiguous genitalia surgery. Data about clinical and laboratory examination collected and analyzed.
Results: The prevalence of female pseudohermaphrodism, male pseudohermaphrodism, ture hermaphrodism and Mixed gonadal digenesis was 70.5%, 20.5%, 5% and 4%, respectively. 76.5% and 23.5% of the patients was converted to female and male, respectively. 29% had congenital Adrenal hyperplasia of whom 86.5%, 10.5% and 3% had 21-hydroxylase deficiency, 11-/? hydroxylase deficiency and 3/?HSD deficiency, respectively. 70.5% had 46xx karyotype and 21.5% had 46xy karyotype and the remainder were musaic. 52.5% had relative parents. The malformation were corrected by vaginoplasty (105 patient) and clitoral resection (51 patients). A variety of anomalies was seen.
Conclusion: Age of presentation ranged from 1 day to 13 years. 151 patients were diagnosed in infancy, but only 43 patient was brought for treatment in infancy to this center by parents.