Testicular microlithiasis: an unreported feature of McCune-Albright syndrome in males

OBJECTIVE: To ascertain the incidence of testicular microlithiasis (TM) in boys with McCune-Albright syndrome (MAS). STUDY DESIGN: Study population consisted of 8 boys with MAS whose medical records were reviewed with emphasis on their past genitourinary histories. All of the boys underwent a clinical and ultrasonographic (US) scanning of the scrotal and inguinal regions. US results in boys with MAS were compared with those obtained in two control populations consisting of 20 healthy subjects and 12 boys with idiopathic and untreated central precocious puberty (CPP). RESULTS: Clinical examination revealed urological abnormalities in no patients, whereas US showed a typical picture of TM in 5 of 8 boys. TM was observed in none of the subjects belonging to control populations (v=15.2 and 11.3, respectively; P <.001). CONCLUSIONS: In a series of 8 boys with MAS we demonstrated a high prevalence (62%) of TM that was associated with neither malignant nor nonmalignant conditions. This finding is unlikely to be only occasional, considering the very low prevalence of TM reported until now in healthy children and young adults and in our results in control populations. TM may be another marker for MAS.     

Galactorrhea-associated granulosa cell tumor in a child

Granulosa cell tumor of the ovary is a rare form of ovarian cancer in children. An 11-year-old girl was admitted with complaints of galactorrhea and abdominal mass. Abdomino-pelvic ultrasound and computed tomography revealed an ovarian tumor. Her prolactine and estradiol levels were increased but luteinizing hormone and follicle-stimulating hormone were decreased. An exploratory laparotomy revealed a giant solid mass, which was completely removed and determined as juvenile granulosa cell tumor. The clinical, hormonal, and radiological findings and the therapy of galactorrhea associated with granulosa cell tumor in a child are discussed. To our knowledge, this is first time it has been described in childhood.     

Treatment with a gonadotropin-releasing-hormone analog and attainment of full height potential in a male monozygotic twin with gonadotropin-releasing hormone-dependent precocious puberty

We report on a pair of male monozygotic twins, one unaffected and the other affected with gonadotropin-releasing hormone (GnRH)-dependent precocious puberty, and discuss the role of treatment with a GnRH analog in the attainment of full height potential in GnRH-dependent precocious puberty. At 1.6 years of age, the affected twin was studied for tall stature (+3.8 SD), and was diagnosed as having GnRH-dependent precocious puberty due to a hypothalamic hamartoma of the tuber cinereum. He was treated with oral cyproterone acetate (110–170 mg/m² daily) from 1.8 through 5.0 years of age, with oral cyproterone acetate and intranasal buserelin acetate (700–900 μg/m² daily) from 5.0 through 7.5 years, and with intranasal buserelin acetate alone (1100– 1400 μg/m² daily) from 7.5 through 12.6 years. He attained a final height of 171.0 cm at 14.9 years of age (+0.10 SD) and his twin 170.0 cm at 15.3 years of age (−0.10 SD), with their target height being 174.5 ± 9.0 cm. Conclusion This study indicates that GnRH analog treatment may preserve near full height potential in some patients with GnRH-dependent precocious puberty. Received: 30 March 1999 / Accepted: 31 May 1999     

Type 1 diabetic children have abnormal lipid profiles during pubertal years

Aim/Hypothesis: To study the prevalence of hypercholesterolemia, hypertriglyceridemia and the relationship between metabolic control, pubertal status and plasma lipoprotein levels in children with diabetes mellitus.
Subjects and methods: A cross‐sectional study was conducted on 126 subjects with type I diabetes followed at our institution. There were 57 boys and 69 girls (mean age: 13.4±3.4 yr; mean duration of diabetes: 7.3±2.1 yr), on whom fasting lipoprotein levels and pubertal status were determined. Mean glycated hemoglobin (HbA1c) of the preceeding year was used in the analysis. Cholesterol (CT) and triglyceride (TG) levels were transformed into standard deviations (SD) using age dependent normal values.
Results: 1) CT levels of DM children (mean level: +0.9±1.2 SD) are higher for both sexes and at each age. Sixteen percent of the cases had CT level ≥2 SD. Within the range of the HbA1c observed (9.1±1.2%), CT levels are not correlated with the degree of metabolic control. In contrast to non‐diabetic children, CT levels of the diabetic children did not vary throughout pubertal stages. CT levels correlated highly with apolipoprotein B (r=0.79; p<0.00001 and r²=82%, in univariate and multivariate analysis, respectively. 2) Plasma TG levels are comparable in the diabetic children (mean level: ?0.11±0.9 SD) and non‐diabetic children. Only 5% of the diabetic children have a TG level ≥2 SD. The TG levels are significantly, but weakly, positively correlated with duration of diabetes and the degree of metabolic control (r²=12% and 16%, respectively, p<0.0001 for both).
Conclusions: Plasma CT levels of type I diabetic children are increased in comparison to non‐diabetic children and do not follow the usual decreasing pattern during puberty.     

Premature Thelarche in Later Childhood Demonstrates a Pubertal Response to GnRH Stimulation Test at One Year after Breast Development

Premature thelarche in later childhood (such as at 5–7 yr of age) is not always easy to distinguish from GnRH-dependent precocious puberty. In this study, a GnRH stimulation test was performed on 21 girls from 5 to 7.5 yr of age with early breast development. In 8 of 11 girls within 1 yr after thelarche, i.e., breast development, the GnRH stimulation test showed a prepubertal response, and in all 10 girls at more than 1 yr after breast development, the GnRH stimulation test showed a pubertal response. In observations of 4 girls with a prepubertal response, the GnRH stimulation test showed to a pubertal response by 1 yr or more after breast development in 3 of 4 the girls. These results indicate the possibility that almost all cases of breast development in later childhood consist of premature thelarche and that premature thelarche in later childhood may easily lead to early puberty at 1 yr or more after breast development. Careful observations are therefore recommended for at least 1 yr, even if early breast development is considered to be associated with premature thelarche in later childhood.     

Regional differences of the sleep electroencephalogram in adolescents

The sleep electroencephalogram (EEG) was recorded from anterior (Fz/Cz) and posterior (Pz/Oz) bipolar derivations in two developmental groups: 20 pre- or early pubertal (Tanner 1/2, mean age 11.4 +/- 1.1 years, 11 boys) and 20 late pubertal or mature adolescents (Tanner 4/5, 14.1 +/- 1.3 years, 8 boys). A sleep-state independent reduction of EEG power over almost the entire frequency range was present in Tanner 4/5 compared with Tanner 1/2 adolescents. Spectral characteristics of the sleep EEG yielded state- and frequency-dependent regional differences that were similar in both developmental groups. Anterior predominance of power in delta and sigma ranges occurred in non-rapid eye movement sleep. Rapid eye movement sleep EEG power was greater in low delta, alpha, and sigma ranges for the posterior derivation and in theta and beta ranges for the anterior derivation. The decay rate of the sleep homeostatic process--reflected by the exponential decline of the 2-Hz EEG power band across the sleep episode--did not differ for derivations or groups. These results indicate that the nocturnal dynamics of sleep homeostasis are independent of derivation and remain stable across puberty.     

性早熟儿童及其父母心理卫生状况初步研究

目的：探讨性早熟对儿童心理行为及其父母心理健康状况有无影响。方法：采用Achenbach儿童行为量表（CBCL）（父母用表）对27例中枢性性早熟女孩及正常儿童进行测试．同时采用症状自评量表（symptom checklist 90，SCL-90）对其父母进行心理卫生状况调查。结果：①Achenbach儿童行为测试显示性早熟女孩行为问题总初分大于对照组，社交退缩、抑郁、躯体主诉3个因子分较对照组明显升高，社会能力方面，性早熟儿童除社交能力低于对照组外，其他无差别，②SCL—90测试显示性早熟儿童父母躯体化，人际敏感、抑郁、焦虑症状因于分明显高于对照组。结论：性早熟女孩存在内向性行为问题。患儿父母存在焦虑、抑郁、躯体化、人际敏感方面的心理健康问题。     

Sex differences in FSH-regulatory peptides in pubertal age boys and girls and effects of sex steroid treatment

BACKGROUND: FSH concentrations are higher in girls than in boys before puberty. We hypothesized that steroid-mediated changes in FSH-regulatory proteins underlie the sex differences in FSH secretion and pubertal timing. METHODS: FSH-regulatory proteins, LH, FSH and sex steroids were measured in five boys, 10 girls, and five girls with Turner syndrome before and during sex steroid treatment (girls, 0.05 mg/day estradiol; boys, 5 mg/day testosterone) for up to 4 weeks. Blood was obtained every 15 min from 20.00 to 08.00 h before and during sex steroid treatment. RESULTS: The mean FSH concentration was higher in girls than in boys (P = 0.0044). Activin-A concentrations were greater (P < 0.0001) and inhibin-B concentrations lower (P < 0.0001) in girls compared with boys. Steroid treatment (i) suppressed LH/FSH concentrations in all subjects; (ii) increased the mean activin-A concentration in all but the Turner girls (P = 0.001); and (iii) decreased inhibin-B concentrations in boys (P = 0.005) but not in girls. Total follistatin and follistatin 288 concentrations did not differ by sex. CONCLUSIONS: Sex steroids regulate circulating activin-A and inhibin-B concentrations in children. The lower inhibin-B and higher activin-A concentrations may explain the higher FSH and earlier onset of puberty in girls.     

Reproduction in male swamp wallabies (Wallabia bicolor): puberty and the effects of season

This study describes pubertal changes in testes and epididymides and seasonal changes in the adult male reproductive organs and plasma androgen concentrations of the swamp wallaby (Wallabia bicolor). Pre-pubescent males had testes with solid seminiferous cords and spermatogenesis only to the stage of gonocytes. Their epididymides had empty lumina along their entire length. The testes of three males undergoing puberty had some lumen formation and mitotic activity. Their epididymides were similar in appearance to those of adult males but were entirely devoid of any cells within the lumen of the duct. Three other pubescent males showed full lumen formation in the testes and spermatogenesis up to the elongating spermatid stage. Their epididymides were similar in appearance to those of adult males but with no spermatozoa in the duct. However, cells of testicular origin were found in the lumen of the duct in all regions suggesting that testicular fluids and immature germ cells shed into the rete testes flow through the seminiferous tubules into the epididymis before the release of mature testicular spermatozoa. The weights of testes and epididymides of adult males showed no change throughout the year but prostate weight and plasma androgen concentrations varied significantly with season, with maximums in spring and summer and minimums in winter. The volume fraction of Leydig cells and seminiferous tubules was significantly lower in winter than in summer; but, despite this, maturing spermatozoa were found in the testes throughout the year. Females in the area conceived year-round, suggesting that seasonal changes in the male reproductive tract did not prevent at least some males from breeding throughout the year.