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Pseudo-precocious puberty in a male patient and the melatonin-testosterone relationship.

We describe a 14 year-old boy with a pineal germ cell tumor which secreted beta HCG. Serum testosterone levels were markedly elevated with concomitant decreased LH secretion. 24-h serum melatonin levels were suppressed and lacked the normal nocturnal rise. Pineal radiation therapy was followed by tumor regression and the diminution of beta HCG stimulated testosterone, which in turn inhibited melatonin and LH. When beta HCG and testosterone were normalized after tumor radiation, a recovery of normal melatonin and LH secretory pattern occurred. These results indicate that circulating testosterone down-regulates pineal melatonin.

Adolescent↗

Gonadotropin independent precocious puberty.

Clinicians should now be fully aware of this intriguing condition of GIPP. The condition is characterized by pubertal sex steroid concentrations and gametogenesis in the presence of prepubertal or suppressed gonadotrophins. In patients with MPP especially without a family history, one should exclude the possibility of pseudoprecocious puberty due to premature production of sex steroids without pituitary gonadotrophins resulting from a primary disorder of the gonad or adrenal gland or to autonomous secretion of gonadotrophin by a tumor. Similarly in patients with recurrent ovarian cyst formation, persistence of the cysts especially with a significant solid component beyond three months should alert a clinician to the possibility of juvenile granulosa cell tumor of the ovary /75/. After confirmation of the diagnosis appropriate treatment should be instituted.

Amino Acid Substitution↗

Diagnosis of central precocious puberty: endocrine assessment.

The new methods which are currently available have provided substantial help in performing the differential diagnosis of CPP, especially in detecting very early modifications of gonadotropin secretion. Nocturnal sampling is not a practical tool and generates discomfort for patients, and daytime samples do not yet have sufficient sensitivity; thus, determination of LH levels after GnRH stimulation is the most important test. We emphasize that the use of the GnRH agonist test improves the discrimination of the endocrine investigation, making it easier to differentiate CPP from other conditions, such as thelarche. Determination of sex steroids is necessary, but its use in isolation is not recommended.

Brain Diseases↗

Treatment of central precocious puberty: lessons from a 15 years prospective trial. German Decapeptyl Study Group.

There is still controversy about the auxological outcome of GnRH agonist treatment in patients with CPP and about the favorable age and auxological characteristics at start of treatment for achieving a normal final height (FH) or for preserving height potential. We analyzed the FH data of 52 young women from a prospective multicentric trial which was started in 1985. The aim of this analysis was to determine factors that may predict a favorable FH or a good height gain. Chronological age (CA) was 5.2 +/- 2.1 yr (+/- SD) at start of puberty, 6.2 +/- 2.0 yr at start of triptorelin depot treatment, 11.1 +/- 1.1 yr at end of treatment, and 16.7 +/- 2.6 yr at FH evaluation. After 4.8 +/- 2.2 yr (1.1-9.9 yr) of treatment duration, FH was 160.6 +/- 8.0 cm (vs 154.9 +/- 9.6 cm of initial height prediction [PAH], p<0.05). A FH within TH range or in excess of mean TH was achieved by 78% or 41% of patients. FH was above the 3rd percentile of the normal German population in 29% of patients (63% had an initial PAH < 156 cm). The group of patients with start of puberty at age < or = 6 yr (Group 1) showed a significantly higher height gain (FH - initial PAH) and lower height deficit compared to TH than older patients (Group 2). Furthermore, the percentage of patients from Group 1 reaching TH range or mean TH showed a significant increase with GnRH agonist treatment whereas this was not the case in Group 2. Stepwise regression analysis showed that height SDS at end of treatment, age at menarche, bone age (BA) at start of treatment, and BA advancement at end of treatment were determinants of FH (r2=0.923). Initial BA advancement and treatment duration were the factors that explained 68% of the variability of height gain. Although BA advancement at initiation of treatment was negatively associated with FH it was a positive predictor of height gain. In addition, height gain correlated significantly with CA and BA at start of treatment (r= -0.430, p=0.004 and r=0.359, p=0.018). Growth after interruption of treatment had no significant predictive effect on FH. It is concluded that a higher percentage of patients below 6 yr of age at start of puberty do profit from GnRH agonist treatment with respect to achieving a normal FH. BA, BA advancement, and height SDS at treatment start are important factors for determining outcome.

Aging↗

Precocious puberty secondary to topical testosterone exposure.

We report a case of pronounced virilization, including marked penile and pubic hair growth, accelerated height velocity and skeletal maturation, and increased muscle mass in a 2.67 year-old boy resulting from presumed inadvertent, long-term exposure to a topical testosterone cream being used by his father.

Child, Preschool↗