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Premature thelarche: identification of clinical and laboratory data for the diagnosis of precocious puberty.

PURPOSE: Two groups of girls with premature breast development were studied retrospectively. We tried to identify clinical, radiological, and hormonal parameters that could distinguish between a benign, nonprogressive premature thelarche and a true precocious puberty. METHODS: The clinical outcome of 88 girls with breast enlargement before 6.1 years of age was analyzed. Taking into account the progression of their sexual maturation, we allocated the children into 2 groups: "Isolated Premature Thelarche" (n = 63) and "Precocious Puberty" (n = 25) groups. Chronological and bone ages, height and growth velocity centiles, computerized tomography of hypothalamus-pituitary area, pelvic ultrasonography, gonadotropin response to luteinizing hormone-releasing hormone stimulation as well as basal levels of luteinizing hormone, follicle-stimulating hormone, estradiol, and prolactin were studied in both groups. Statistical analysis were performed using the Student t test to compare the sample means. Fisher's exact test and chi2 test were used to analyze the nonparametric variables. RESULTS: Isolated premature thelarche most frequently affected girls younger than 2 years who presented exaggerated follicle-stimulating hormone response to luteinizing hormone-releasing hormone stimulation test. The precocious puberty group had higher initial stature, accelerated growth rate and bone age, increased uterine and ovarian volumes, high spontaneous luteinizing hormone levels by immunofluorimetric assay, as well as a high luteinizing hormone response and peak luteinizing hormone/follicle-stimulating hormone ratio after luteinizing hormone-releasing hormone stimulation. CONCLUSION: At initial presentation, girls who undergo true precocious puberty present advanced bone age, increased uterine and ovarian volumes in addition to breast enlargement, as well as an luteinizing hormone-predominant response after a luteinizing hormone-releasing hormone stimulation test.

Age Determination by Skeleton↗

Use of aromatase inhibitors in precocious puberty.

During puberty, estrogen causes breast maturation and growth of the uterine lining in girls, and accelerates linear growth and bone maturation in both boys and girls. Decreasing the biosynthesis of estrogen can attenuate these processes. In 12 girls with the McCune-Albright syndrome (MAS), in which precocious puberty is due to production of estrogen from ovarian cysts, testolactone (40 mg/kg per day) decreased the volume of ovarian cysts, the frequency of menses, and the rates of growth and bone maturation, for periods of 1-4 years. In a 6-month pilot study of 12 children (eight boys; four girls) with congenital adrenal hyperplasia, testolactone, in combination with an antiandrogen (flutamide), a mineralocorticoid (fludrocortisone acetate, Florinef), and a reduced glucocorticoid dose, improved the control of growth and bone maturation compared with conventional therapy. In a 6-year study of 10 boys with familial male precocious puberty, testolactone, in combination with an antiandrogen (spironolactone), decreased rates of growth and bone maturation, and increased predicted adult height. All patients who developed evidence for gonadotropin-dependent puberty were also treated with a GnRH analog. Testolactone had no important adverse effects in any group of patients, although the need for a four-times-daily dosing schedule made compliance difficult for many families. We conclude that suppressing of estrogen with testolactone was effective therapy, and that more potent and specific inhibitors of aromatase could further improve the treatment of these disorders.

Adrenal Hyperplasia, Congenital↗

Puberty gingivitis in insulin-dependent diabetic children. I. Cross-sectional observations.

This cross-sectional study examined the gingivitis occurring at puberty in a population of insulin-dependent juvenile diabetics. Seventy-seven children between the ages of 6 and 15 years were examined for gingivitis levels, stages of pubertal maturation and blood levels of glucose and glycosylated hemoglobin. Bacterial plaque was sampled from one or more approximal tooth surfaces of every subject and cultured under anaerobic and aerobic conditions on nonselective and selective media. The total cultivable flora and percentage of certain presumptive periodontopathic bacteria were determined. Before puberty, children with "high" levels of glycosylated hemoglobin also had higher gingivitis levels than children with "normal" metabolic control of diabetes. During puberty, the level of gingivitis increased independently from both fasting blood glucose levels and per cent glycosylated hemoglobin. The microbiota of marginal plaque was predominantly composed of facultatively anaerobic bacteria. The percentages of Capnocytophaga sp and Actinomyces naeslundii were statistically higher at the onset of puberty, suggesting that a specific bacterial shift in the microbial composition of marginal plaque occurs in response to host changes in juvenile diabetic children at this age period.

Adolescent↗

Association between lipoprotein(a) and insulin-like growth factor I during puberty and the relationship to microalbuminuria in children and adolescents with IDDM.

OBJECTIVE: To study pubertal changes in serum lipoprotein(a) [Lp(a)] and insulin-like growth factor I (IGF-I) in insulin-dependent diabetes mellitus (IDDM) and the relationship to microalbuminuria. RESEARCH DESIGN AND METHODS: Seventy-nine children and adolescents (59 with normoalbuminuria, 20 with microalbuminuria) with > or = 5 years of IDDM were investigated together with 54 healthy control subjects in a cross-sectional study. Fasting serum Lp(a); apolipoprotein (apo) A-1 and B; total, low-density lipoprotein (LDL), and high-density lipoprotein cholesterol; triglycerides; and IGF-I were analyzed as were HbA1c and overnight albumin excretion rate (AER). Pubertal development was assessed by Tanner staging. RESULTS: Lp(a), apoB, triglycerides, and total and LDL cholesterol were higher (P < 0.001) and apoA-1 was lower (P = 0.03) in normoalbuminuric IDDM patients than in healthy control subjects. Lp(a) was increased during puberty (stages 2-4) in IDDM patients but not in healthy subjects, whereas IGF-I was significantly increased during puberty in healthy control subjects only. In IDDM patients Lp(a) correlated to insulin dose, total cholesterol, and LDL cholesterol, but not to IGF-I, HbA1c, systolic and diastolic blood pressure, diabetes duration, age, or sex. In multiple regression analysis with Lp(a) as the dependent variable, puberty was the only significant contributor to the regression (r2 = 0.33, P = 0.008). Microalbuminuria was seen only in the pubertal stage 4-5. Lp(a) tended to be higher (P = 0.06) as did apoB, whereas IGF-I was lower (P < 0.001) in this group than in normoalbuminuric patients of the same pubertal stages. In multivariate analysis, with log AER as the dependent variable, apoB/apoA-1, systolic blood pressure, age, and IGF-I but not Lp(a) added to the regression (r2 = 0.47, P < 0.0001). CONCLUSIONS: Lp(a) is elevated during puberty in normoalbuminuric subjects with IDDM, independent of metabolic control and IGF-I. Lp(a) tends to be further increased in microalbuminuria but does not seem to be a contributing determinant of log AER whereas low IGF-I does. Prospective studies are required to establish the temporal relationship between increased Lp(a) and microalbuminuria in children and adolescents with IDDM.

Adolescent↗

Enhanced progression of urinary albumin excretion in IDDM during puberty.

OBJECTIVE: To determine whether the progression of urinary albumin excretion rate (AER) is higher during puberty than before or after this period. RESEARCH DESIGN AND METHODS: A prospective study was conducted in which normoalbuminuric prepubertal (n = 20), pubertal (n = 28), and postpubertal (n = 26) IDDM groups matched for diabetes duration and long-term metabolic control were followed for 3 years. At 6-month intervals, 24-h urine collection was used to determine AER. RESULTS: AER increased significantly over a period of 3 years in the pubertal (P = 0.001) and postpubertal (P = 0.003) subjects but not in prepubertal subjects. The annual progression of AER was significantly higher in the pubertal group than in the prepubertal (P = 0.001) or postpubertal (P = 0.001) groups. Six pubertal, two postpubertal, and none of the prepubertal subjects developed microalbuminuria (AER > or = 20 micrograms/min on two consecutive occasions) over a 3-year period (P = 0.047). Multiple logistic regression analysis showed that the risk of development of microalbuminuria was increased in pubertal subjects compared with the prepubertal and postpubertal subjects (adjusted relative risk [95% CI]: 4.3 [1.5-9.3], P = 0.012, and 2.1 [1.1-5.0], P = 0.023, respectively). CONCLUSIONS: Puberty represents an independent risk of the development of microalbuminuria in diabetes. This findings suggests that the endocrine changes of puberty lead to an accelerated process of early kidney damage in diabetes. In pediatric diabetes care, screening for microalbuminuria is needed soon after the onset of puberty.

Adolescent↗

Low IGF-I and elevated testosterone during puberty in subjects with type 1 diabetes developing microalbuminuria in comparison to normoalbuminuric control subjects: the Oxford Regional Prospective Study.

OBJECTIVE: To describe longitudinal variations in pubertal hormonal variables in subjects with and without microalbuminuria (MA). RESEARCH DESIGN AND METHODS: Blood samples collected annually from subjects recruited at diagnosis of type 1 diabetes and followed prospectively through puberty (median follow-up 9.3 years, range 4.7-12.8) were analyzed for total and free IGF-I, IGF binding protein-1, testosterone, sex hormone-binding globulin, and HbA(1c). A total of 55 subjects who developed MA (MA(+) group) were compared with 55 age-, sex-, and duration-matched control subjects who did not develop MA (MA(-) group). RESULTS: For female subjects, total IGF-I (MA(+) 1.2 mU/l vs. MA(-) 1.4 mU/l, P = 0.03) and free IGF-I levels (MA(+) 1,767 ng/l vs. MA(-) 2010 ng/l, P = 0.002) were lower, whereas the free androgen index (MA(+) 2.4 vs. MA(-) 2.0, P = 0.03) was higher in those with MA. These changes were less pronounced in male subjects. For both sexes, in a Cox model after adjusting for puberty, the presence of MA was associated with lower free IGF-I levels, higher testosterone standard deviation score (SDS), and poor glycemic control. We found that 22 of 55 case subjects (40%) developed persistent MA, whereas 33 (60%) had transient MA. In the persistent MA group compared with the transient and control groups, total IGF-I levels were lower (1.1 vs. 1.3 vs. 1.4 mU/l, P = 0.002) as were free IGF-I levels (1,370.9 vs. 1,907.3 vs. 1,886.7 ng/l, P < 0.001), whereas HbA(1c) levels were higher (11.8 vs. 10.3 vs. 9.9%, P < 0.001). CONCLUSIONS: Poor glycemic control and differences in IGF-I levels and androgens, particularly in female subjects, accompany development of MA at puberty. These differences may in part account for the sexual dimorphism in MA risk during puberty and could relate to disease progression.

Adolescent↗

Role of surgical resection in the treatment of hypothalamic hamartomas causing precocious puberty. Report of six cases.

Hypothalamic hamartomas (HHs) are benign tumors that are often associated with central precocious puberty. Resection of HHs has been recommended as a treatment option for selected cases of pedunculated lesions, especially in young children. The role of surgery has to be evaluated in the light of the availability of effective medical treatment with gonadotropin-releasing hormone analogs (GnRHas). The authors report the long-term results of total resection of HHs in two children with central precocious puberty and compare it with medical management in four children. The two surgically treated patients underwent total resection of pedunculated HHs at 1.75 (Case 1) and 3.25 years (Case 2) and have received follow-up care for 9 and 8 years, respectively. There were no postoperative complications and computerized tomography scanning confirmed complete tumor removal in both patients. Both patients subsequently experienced some regression of secondary sexual characteristics. The response of luteinizing hormone to GnRH became prepubertal in one patient and was diminished in the other. However, the growth velocity remained elevated (> 7 cm/year), bone age remained advanced (> +2 standard deviations) 5 years after surgery, decreasing adult height prediction. In one child, GnRHa therapy was initiated 7 years postsurgery. Four children were treated solely with GnRH agonists and have received follow-up care for 2.3, 6, 9, and 9 years, respectively. These patients have had a complete regression of endocrinological abnormalities, including a normalization of growth velocity and reduction in the rate of skeletal maturation. No side effects were noted with decapeptyl treatment, and one child developed sterile abscesses while receiving Lupron-Depot. The proven efficacy of GnRHa in suppressing puberty and reducing bone age advancement leads the authors to advise against surgery as the initial management of central precocious puberty caused by HHs.

Child↗

[Changes of airway responsiveness, and relative factors in asthmatics with remission at puberty].

OBJECTIVE: To study the airway responsiveness and relative factors in asthmatics with natural remission at puberty. METHOD: The airway responsiveness was evaluated by challenge of methacholine (PC20), blood sIL-2R, eosinophil count, basophil count and basophil releasability were detected in 18 asthmatics with remission at puberty (group I), in 20 asthmatics without remission at puberty (group II), and in 30 healthy controls (group III). RESULT: The mean value of PC20 in the group I was significantly lower than that of the group III (P < 0.01), but still higher than that of the group II (P < 0.01). The blood sIL-2R and eosinophil values were droped almost to those of the group III, significantly lower than those of the group II. The basophil value and positive rate of human basophil degranulation test stimulated by mannitol with hyperosmolarity were still abnormal, significantly higher than those of the group III, and the basophil level was significantly related to the airway responsiveness. CONCLUSION: The asthmatics with remission at puberty have a certain degree of airway hyperresponsivenes that is related to the abnormal of basophil.

Adult↗

Precocious puberty associated with growth hormone deficiency in a patient with craniopharyngioma: report of one case.

Isosexual precocious puberty in girls is not uncommon, but its association with craniopharyngioma and growth hormone deficiency is rarely reported. We present a patient with this combination. An 8-year-old girl developed breasts and then had menarche at 7 9/12 years old. Growth hormone deficiency was suspected due to inappropriate height and growth velocity in association with idiopathic precocity and a poor predicted adult height of 138.8 cm. Growth hormone deficiency was confirmed by clonidine and insulin stimulation tests. Intracranial lesion was suspected due to precocity associated with GH deficiency. MRI of the sella's region revealed a 1 cm mass in the hypothalamus. After surgical resection, pathology of the tumor disclosed a craniopharyngioma which has rarely been reported to cause precocious puberty. The precocious puberty regressed after surgery. Growth hormone deficiency persisted and GH therapy was given to improve growth. The growth rate of patients with both growth hormone deficiency and precocious puberty may be maintained within the normal prepubertal range by the effect of sex steroid. We suggest that in patients with central type precocity in association with an inappropriate growth status, physicians should investigate the underlying intracranial lesion, and the possibility of growth hormone deficiency.

Biopsy, Needle↗

[Evaluation of bone mineral density and selected metabolic markers of bone in boys with constitutional delay of growth and puberty].

The aim of this study was to evaluate bone mineral density and some markers of bone metabolism (alkaline phosphatase activity, osteocalcin concentration, deoxypirydinoline excretion), as well as the influence of physical activity and dietary calcium supplementation on bone density and bone metabolism in boys with a constitutional delay of growth and puberty (CDGP), in relation to calendar age, bone age and pubertal stage. The study was done in 41 boys aged 8-18 years with CDGP diagnosed on the basis of family history and typical pattern of growth, with prior exclusion of hormonal and non-hormonal causes of height deficiency. According to Tanner's criteria, boys were assigned to 4 groups corresponding to 4 stages of puberty (Tab. 1). Total body (total BMD) and lumbar spine (L2L4 BMD) bone mineral density were obtained using DEXA densitometry. The activity of alkaline phosphatase (AP) and serum osteocalcin (OC) concentration served as markers of bone formation. 24-h urinary deoxypirydinoline (DPD) excretion was a marker of bone resorption. Using a questionnaire, daily dietary calcium intake and physical activity were established. A positive correlation between calendar age, total BMD and L2L4 BMD was found (R = 0.70 and R2 = 0.49; R = 0.72 and R2 = 0.51; p < 0.001, respectively). Bone age was positively correlated with total BMD and L2L4 BMD (for both correlations R = 0.68, R2 = 0.47; p < 0.001). Total BMD adjusted for calendar age revealed osteoporosis in 4 cases (9.7%), osteopenia in 19 cases (46.3%) and normal bone density in 18 cases (43.9%). After adjustment of BMD to bone age, 2 boys (4.9%) were diagnosed with osteoporosis, 9 (21.9%) with osteopenia, and 30 (73.2%) with normal bone density. L2L4 BMD adjusted for calendar age showed osteoporosis in 6 cases (14.6%), osteopenia in 21 cases (51.2%) and normal density in 14 cases (34.2%). After adjustment of BMD to bone age, 3 boys (7.3%) were diagnosed with osteoporosis, 10 (24.4%) with osteopenia, and 28 (68.3%) with normal bone density. A significant correlation between stage of puberty, total BMD (p < 0.045) and L2L4 BMD (p < 0.001) was found. Values for total BMD and L2L4 BMD in boys with CDGP in relation to pubertal stage are presented in Table 2. Mean AP activity, serum OC concentration, DPD excretion, dietary calcium intake and physical activity in boys at consecutive stages of puberty are presented in Table 3. No significant correlation between pubertal stage, markers of bone metabolism, daily dietary calcium intake or physical activity was found. The boys were next grouped according to level of physical activity: low (< 10 h/week) or high (> 10 h/week). Table 4 summarizes the results for both groups. No significant differences between the groups were found concerning the parameters studied. Two groups with a low (< 1.2 g) and high (> 1.2 g) dietary calcium intake were also formed. The results for both groups are presented in Table 5. A significant correlation between dietary calcium intake and total BMD (R = 0.34 and R2 = 0.11; p < 0.03) was revealed. On the basis of these results it can be concluded that BMD values in boys with CDGP should be adjusted for bone age, thus significantly limiting the diagnosis of osteoporosis and osteopenia. BMD values for these boys increase with consecutive pubertal stage, while markers of bone metabolism do not reveal significant abnormalities (markers of bone formation increase until stage IV, markers of bone resorption show fluctuating values).

Absorptiometry, Photon↗

Changes in airway responsiveness and relative factors in asthma with remission at puberty.

OBJECTIVE: To elucidate the airway responsiveness and its relative factors in asthma with natural remission at puberty. METHOD: The airway responsiveness was evaluated by provocation of methacholine (PC20). Blood sIL-2R, IL-4 and IFN-gamma values in the cultural supernatant of mononuclear cells, eosinophil count, basophil count and basophil releasability were detected in 18 asthmatic cases with remission at puberty (group I), in 20 asthmatics without remission at puberty (group II), and in 30 healthy controls (group III). RESULT: The mean value of PC20 in group I was significantly lower than that in group III (P < 0.01), but still higher than that in group II (P < 0.01). The blood sIL-2R, and eosinophil counts dropped almost to those of group III, significantly lower than those of group II. IL-4 in the culture supernatant of mononuclear cells was similar to group III, but significantly lower than that in group II. However IFN-gamma was also similar to group III, significantly higher than that of group II. The basophil count and positive rate of human basophil degranulation test stimulated by mannitol with hyper-osmolarity were still abnormal, significantly higher than those of group III, and the basophil level was significantly related to the airway responsiveness. CONCLUSION: The asthmatics with remission at puberty have a certain degree of airway hyper-responsiveness that is related to the abnormality of basophil.

Adolescent↗

[A preliminary study on relationship between concentrations of human growth hormone in urine (HGH-U) and puberty].

OBJECTIVE: The aim of this study is to investigate relationship between the concentration of human growth hormone in urine (HGH-U) and puberty, and to find a simple, accurate, and acceptable way of judging puberty of patients. METHODS: The concentration of HGH-U was measured by using radioimmunoassay in 277 students of Chengdu, and students were divided into three different groups, according to their ages (9, 13, 17 years old). Half of them are male. In order to evaluate the relationship between the concentration of HGH-U and puberty, the information of age, height, weight, growth velocity and sex character of all individuals were obtained, and analyzed their relation with the concentration of individuals' HGH-U. RESULTS: 1. There was no significant difference in the concentration of HGH-U between the 9-year-old group and the 13-year-old group, but there is significant difference between the concentration of HGH-U in the 9-year-old, 13-year-old groups and that of the 17-year-old group, and the concentration in the latter group is lower than those of the former two groups. 2. The concentration of HGH-U was negatively correlated with height, body weight, age, sex character and growth velocity (P < 0.05), but the correlation coefficient is low. 3. The coefficients of variation of HGH-U in all group were higher than 30%, which indicated a low dispersion degree. CONCLUSION: The concentration of HGH-U cannot be used as an independent indicator to evaluate puberty of children.

Adolescent↗

[Consensus on the diagnosis and treatment of central early puberty].

The beginning of puberty is marked by breast growth in girls and testicular enlargement in boys. These occur at the age of 10.5 +/- 2.0 years in females and 11.5 +/- 2.0 years in males. Recent but controversial publications suggest that these events are being observed at younger ages, at least in the USA. There are no studies demonstrating that this is true in Chile. For this reason we still consider that puberty is precocious when it occurs before 8.0 years in girls and before 9.0 years in boys. True or central precocious puberty (CPP) must be distinguished from peripheral or pseudoprecocious puberty (PPP), from premature telarche and from premature adrenarche. We suggest that the workup of a patient with premature development should include an LHRH test to demonstrate if the hypothalamic-pituitary axis is activated, plasma levels of sex steroids, bone age and pelvic ultrasound in girls. All children with CPP should have a CAT scan or MNR of the brain, since a lesion of the central nervous system is observed in 15% of the girls and 50% of the boys with CPP. Additional studies are needed in cases of PPP. The aim of treating CPP is to avoid adult short stature that results from premature fusion of the epiphysis and to avoid eventual emotional and psychological stress. Treatment consists of monthly intramuscular injections of a depot preparation of LHRH analogs. Suppression of pituitary and gonadal activity produces regression of secondary sex characteristics and slowing down of growth velocity and bone maturation. The opportunity, duration of treatment and their effect on final stature are discussed.

Adolescent↗

[Roles of leptin in the development during girls'puberty].

OBJECTIVES: To study the roles of leptin in the development during puberty in girls and the its relationship with insulin (INS), growth hormone (GH), estradiol (E(2)) and testosterone (T). METHODS: One hundred and fifty girls with simple obese aged 7 to 17 years, and 150 normal healthy girls and 150 girls with malnutrition matched for age (+/- 3 months) and height (+/- 2 cm) were selected. Serum levels of leptin, INS, GH, E(2) and T were measured for them. RESULTS: Their serum level of leptin positively correlated with body mass index (BMI) and age. Serum level of leptin in girls increased steadily from Tanner stage B(1) to stage B(5). At Tanner stage B(2), serum level of leptin in the normal groups (7.72 microg/L) was not significantly different from that in those with malnutrition (7.36 microg/L), but significantly lower than that in the obese groups (12.85 microg/L). At other Tanner stages, there was significant difference in serum level of leptin among obese, normal and malnutrition groups. Serum level of leptin correlated negatively with serum GH and positively with serum INS, but not correlated with E(2) and T. CONCLUSIONS: Leptin may play a role in triggering development during puberty in girls. Serum level of leptin at Tanner stage B(2) may be the threshold dose to trigger the onset of puberty in girls. Quickly increasing level of leptin at Tanner stage B(5) may inhibit the increase of GH, which ushered the end of puberty in girls.

Adolescent↗

Therapeutic strategies in a male with delayed puberty.

Therapeutical strategies in male with delayed puberty. Delayed puberty (absence of pubertal modifications after 14-15 years of age) due to transitory deficit of LHRH secretion often constitutes a difficult differential diagnostic problem for conditions of permanent LHRH deficit which can be identified only after 18 years of age in the idiopathic hypogonadotropic hypogonadism. After a period of clinical observation short-term therapy with pulsatile LHRH administration may take place. Therapy can be necessary: to document the functional integrity of the pituitary-gonadal axis; to promote pubertal modification such as to improve and physiologically sustain the patient; to tray a neuroendocrine activation of endogenous LHRH-LH secretion. Also delayed puberty linked to uremia seems to respond to short-term pulsatile LHRH administration. Pulsatile LHRH administration is the most physiological therapeutical approach to subject with delayed puberty. It seems to constitute a valid alternative to the therapy with testosterone or gonadotropins.

Adolescent↗

Hypothalamic hamartoma and precocious puberty: report of a case.

Hypothalamic hamartoma is reported to be associated with precocious puberty. Here, the authors present a seven-year-old girl whose onset of puberty occurred at the age of two. Under the impression of idiopathic precocious puberty, cyproterone acetate was initially tried. Since the effect of her medication was not satisfactory, it was discontinued at the age of five years and 11 months. However, rapid advance of bone age and vaginal spotting recurred after the withdrawal of treatment. She was re-evaluated at the age of six, and a magnetic resonance image (MRI) study of the head revealed a hypothalamic hamartoma. At that time, a long-acting analog of luteinizing hormone-releasing hormone (LHRHa), leuprolide acetate, was prescribed. Her secondary sex characteristics regressed and her hypothalamic-pituitary-gonad axis was suppressed after treatment. The clinical presentation, mechanism and treatment of precocious puberty caused by hypothalamic hamartomas are fully discussed in this report.

Child↗

[Suprasellar arachnoid cyst associated with precocious puberty: report of an operated case and review of the literature].

The pathogenesis remains unknown in the majority of patients with precocious puberty, and yet infrequently such causative cerebral lesions as hypothalamic hamartomas are associated with sexual precocity. We reported a rare case of suprasellar arachnoid cyst in an infant presenting with precocious puberty, which eventually disappeared after a cyst-peritoneal shunt. It was believed that the mass effect of the arachnoid cyst upon the hypothalamus was, at least in part, responsible for development of precocious puberty. The role of surgical decompression of the cyst was also discussed. A one-year-old girl was admitted to the hospital for evaluation of genital bleeding which had persisted on and off for two months. The height, 80cm, and the weight, 12.4kg, exceeded by far the two standard deviations from the mean level of the normal population. In addition she had the development of breast tissue as classified Tanner's Stage II, and both pubic and axillary hair. The bone age by skeletal survey of the hand was rated as 3 years. Endocrinological examination showed that serum levels of luteinizing hormone (LH), follicle-stimulating hormone (FSH) and estradiol had increased for her age, to levels equivalent to those for females at puberty. An LH-RH test revealed an excessive LH reaction. There were no definite neurological deficits. CT and MRI demonstrated the presence of a large arachnoid cyst involving the suprasellar region as well as the right middle and posterior fossa. After the patient underwent a cyst-peritoneal shunt, the cyst decreased in size and such symptoms as genital bleeding and breast growth disappeared. Serum levels of her LH and FSH also significantly decreased.(ABSTRACT TRUNCATED AT 250 WORDS)

Arachnoid Cysts↗

The NOTOM hypothesis for idiopathic scoliosis: is it nullified by the delayed puberty of female rhythmic gymnasts and ballet dancers with scoliosis?

Nachemson suggested that there are more girls than boys with progressive adolescent idiopathic because of a different timing between skeletal maturation and postural maturation in the sexes during adolescence. We termed Nachemson's concept the neuro-osseous timing of maturation (NOTOM) hypothesis and used it to propose a possible medical treatment for idiopathic scoliosis by delaying puberty through the pituitary using gonadorelin analogues as in idiopathic precocious puberty. The prevalence of scoliosis is reported to be increased in rhythmic gymnasts (RGs) in Bulgaria and in ballet dancers (BDs) in the USA. Both groups exhibit delayed puberty, which, at first sight, nullifies the NOTOM hypothesis for idiopathic scoliosis. While constitutional and environmental factors may determine these scolioses, the different curve types in RGs and BDs suggest that the exercise pattern over many years determines which type of scoliosis develops, although not the curve severity. We support the view that scoliotic RGs should be included in a group of sports-associated scoliosis separate from idiopathic scoliosis. Hence the delayed puberty of RGs and BDs with scoliosis does not nullify the NOTOM hypothesis as their scolioses are not idiopathic. There is a need to focus research on such subjects who have defined constitutional and environmental factors related to their scolioses.

Adolescent↗