Central nervous system imaging in girls with central precocious puberty: when is necessary?

ABSTRACT Objectives The determinants of an increased risk of an organic pathology underlying central precocious puberty (CPP) in girls remain contentious. The present study aimed to determine the clinical and hormonal findings that can be used to differentiate organic and idiopathic CPP in girls as a screening method so that only those considered likely to have organic CPP undergo cranial magnetic resonance imaging (MRI). Subjects and methods The medical records of 286 girls that received GnRH agonist (GnRHa) therapy for CPP were retrospectively evaluated. Chronological and bone age, height, pubertal stage, and basal/stimulated gonadotropin and estradiol (E2) levels, as well as cranial MRI findings at the time CPP was diagnosed were recorded. Clinical and hormonal parameters that can be used to differentiate between girls with organic and idiopathic CPP were identified using ROC curves. Results Organic CPP was noted in 6.3% of the participants. Puberty started before age 6 years in 88.9% of the girls with organic CPP. Mean E2 and peak luteinizing hormone (LH) levels were higher in the girls with organic CPP than in those with idiopathic CPP that were matched for pubertal stage, as follows: early stage puberty (Tanner 2 and 3): E2: 62.4 ± 19.8 pg/mL vs. 29.1 ± 9.5 pg/mL; peak LH: 16.8 ± 3.2 IU/L vs. 12.2 ± 3.7 IU/L; advanced stage puberty (Tanner 4): mean E2: 87.6 ± 3.4 pg/mL vs. 64.6 ± 21.2 pg/mL; peak LH: 20.8 ± 0.4 IU/L vs. 16.6 ± 5.8 IU/L (P < 0.001 for all). Thresholds for differentiating organic and idiopathic CPP in girls with early-stage puberty were 38.1 pg/mL for E2 (100% sensitivity and 80.4% specificity) and 13.6 IU/L for peak LH (100% sensitivity and 66.4% specificity). Conclusion Pubertal symptoms and signs generally begin before age 6 years and hormone levels are much higher than expected for pubertal stage in girls with organic CPP. Based on the present findings, cranial MRI is recommended for girls aged < 6 years, as the risk of diagnosing an organic pathology is highest in this age group. Hormone levels higher than expected for pubertal stage might be another indication for cranial MRI, regardless of patient age. Cranial MRI should be performed in girls with early-stage puberty, and an E2 level > 38 pg/mL and/or a peak LH level > 13.6 IU/L.

Ultrassonografia no auxílio do diagnóstico de vesiculite seminal em touros de corte / Ultrasonography in the diagnosis of seminal vesiculites in beef bulls

A vesiculite é um processo inflamatório das glândulas vesiculares, podendo ser unilateral ou bilateral, que acomete reprodutores. O objetivo do presente trabalho foi avaliar a utilização da ultrassonografia como meio de diagnóstico precoce das alterações das vesículas seminais em touros. O presente trabalho foi realizado no município de Videira, Santa Catarina. Analisou-se um total 42 reprodutores, com média de idade de 15 meses, das raças Aberdeen Angus e Polled Hereford em semi-confinamento. Foi realizado exame clinico do estado geral dos animais e exame andrológico dos reprodutores. As amostras de sêmen para a realização do exame das características físicas do ejaculado foram obtidas por eletroejaculação. Através da palpação retal, realizou-se a avaliação das glândulas vesiculares por ultrassonografia. A análise estatística dos dados foi realizada por meio de análise de variância (ANOVA) para comparação entre médias com nível de significância de 5%. A presença de vesiculite foi observada em 31 animais (73,8%) dos 42 analisados. Dos 31 animais portadores 11 animais (35,5%) apresentaram vesiculite bilateral e 20 apresentaram vesiculite unilateral (64,5%; P<0,05). Animais com perímetro escrotal maior tendem a desenvolver vesiculite unilateral, tal fato pode ser explicado pela precocidade sexual agravado pela sodomia entre os animais. O uso do ultrassom auxilia de forma preventiva a detecção de animais portadores de vesiculite em reprodutores.(AU)

The vesiculite is an inflammatory process of the vesicular glands, unilateral or bilateral, that affects bulls. The purpose of this study was to evaluate the use of ultrasound as a complementary method for early detection of changes in seminal vesicles in bulls. This study was conducted in the municipality of Videira, Santa Catarina. We evaluated 42 bulls, with an average age of 15 months, Aberdeen Angus and Polled Hereford breeds and in semi-confinement. Clinical animal examination and andrological exam were performed in all animals. Semen samples were obtained by electroejaculation and physical characteristics of the ejaculate were performed. Rectal palpation was performed by ultrasonography to evaluate vesicular glands changes. Statistical analysis of the data was performed using analysis of variance (ANOVA) for comparison between means with significance level of 5%. The presence of vesiculitis was observed in 31 (73.8%) from the 42 analyzed bulls. Of the 31 animals, 11 animals (35.5%) presented bilateral vesiculitis and 20 showed unilateral vesiculitis (64.5%; P<0.05). Animals with a larger scrotal perimeter tend to develop unilateral vesiculitis, which can be explained by the sexual precocity observed by sodomy among animals. In this way, the use of ultrasound helps preventively to detect animals with vesiculitis in breeding animals.(AU)

Características reprodutivas e suas associações com outras características de importância econômica na raça Nelore / Reproductive traits and their associations with other economically important traits in Nelore

Objetivou-se com este trabalho estimar as herdabilidades (h²) e as correlações genéticas (r g) entre idade ao primeiro parto (IPP) e primeiro intervalo de partos (PIEP) e outras características como peso (PS) ao ano (A) e ao sobreano (S), altura do posterior (ALT) e perímetro escrotal (PE450) em animais da raça Nelore. Os parâmetros genéticos foram estimados em uma análise multicaracterística por modelo animal, utilizando-se a inferência bayesiana via algoritmo de "Gibbs Sampling". Os parâmetros genéticos estimados sugerem a existência de variabilidade genética para IPP (h² = 0,26), sendo que a seleção para a diminuição da IPP de fêmeas Nelore deve responder à seleção individual, sem causar antagonismo do valor genético dos animais para PS (r g = -0,22 (A) e -0,44 (S)) e PE450 (r g = 0,02). A seleção para a diminuição da IPP, no longo prazo, pode levar a um aumento da ALT dos animais, embora essa associação seja relativamente baixa (-0,35). A estimativa de herdabilidade a posteriori para a característica PIEP foi baixa, 0,11±0,03. As r g entre PIEP e as demais características estudadas indicam que a seleção para essas características de crescimento não afetará o PIEP.

Heritability (h²) and genetic correlations (r g) were estimated between reproductive traits such as age at first calving (AFC), first calving interval (FCI) and other economically relevant traits, i.e., weight (W) at year (Y) and at 18 months of age (S), scrotal circumference (SC), and hip height (HH) in Nelore cattle. The genetic parameters were estimated in a multiple-trait analysis, with animal models using the Bayesian inference by Gibbs Sampling algorithm. The genetic parameters estimated in this work suggest the existence of genetic variability for AFC (h² = 0.26), where the selection for the reduction of Nelore females AFC should respond to mass selection, without causing genetic antagonism in the selection of W (r g = -0,22 (Y) and -0,44 (S)), and SC (r g = 0,02). The selection for the AFC in the long term could lead to an increase in the animal's frame, although this association is relatively low (-0.35). The posteriori heritability estimate for FCI was low, 0.11±0.03. The r g between FCI and the other traits studied indicate that selection for these growth traits will not affect the FCI.

Características zootécnicas de touros da raça Nelore submetidos a um programa de seleção para precocidade sexual / Zootecnic characteristics of Nelore bulls subjected to a selection program for sexual precocity

Foram utilizados 24 tourinhos da raça Nelore (Bos taurus indicus) divididos em três grupos de oito animais cada, de acordo com o perímetro escrotal (PE): Grupo 1 (G1) - PE acima de 22,8cm; Grupo 2 (G2) - PE entre 21,6 e 22,6cm; e Grupo 3 (G3) - PE entre 20,1 e 21,2cm. Para a análise da área de olho do lombo (AOL) e espessura de gordura (EG), os 24 animais foram divididos em dois grupos : G1 (mais precoces) e G2 (menos precoces), classificados de acordo com a idade em que cada animal atingiu a puberdade. O G1 apresentou maior PE à puberdade e menor peso à desmama em comparação ao G2 e ao G3. As medidas de AOL não apresentaram diferenças entre os grupos G1 e G2. Os animais do G1 atingiram a precocidade sexual em idade menor, enquanto a deposição de gordura maior e mais cedo ocorreu nos animais do G2, considerados mais tardios. A utilização de características zootécnicas em um programa de seleção para precocidade sexual pode constituir um avanço para a produtividade pecuária, aumentando os ganhos genéticos e diminuindo os custos de produção.

Twenty-four Nelore bulls (Bos taurus indicus) were divided in three groups of eight animals each according to EP: Group 1 - EP above average (EP>22.8cm); Group 2 - EP 5 percent below average (EP between 21.6 and 22.6cm); and Group 3 - EP from 10 to 5 percent below average (EP between 20.1 and 21.2cm). For the AOL and EG analysis the 24 animals were divided in two groups: G1 (more precocious) and G2 (less precocious), classified according to the age in which each animal reached puberty. G1 presented greater EP at puberty and less weight in comparison to G2 and G3. The AOL measures did not present difference between G1 and G2 groups. The animals in G1 reached puberty at a younger age, while the fat deposition occurred in G2. The use of productive characteristics in a program to select animals for sexual precocity can constitute a great advance for cattle productivity, increasing the genetic gain and decreasing production costs.

Gonadotropin-releasing Hormone Stimulation Test for Precocious Puberty / 대한진단검사의학회지

BACKGROUND: Gonadotropin-releasing hormone (GnRH) stimulation test is the gold standard to identify central precocious puberty (CPP). This test requires multiple blood samples at different time points to measure gonadotropin levels, and is therefore expensive, time-consuming, and uncomfortable for patients. We aimed to simplify the GnRH stimulation test to require fewer blood samples. METHODS: A study of 166 girls with precocious puberty was undertaken. Blood samples were obtained at 0, 15, 30, 45, 60, 90, and 120 min after GnRH administration, and the levels of luteinizing hormone (LH) and follicle-stimulating hormone (FSH) were measured. For each parameter, the sensitivities and specificities were estimated and ROC curves were constructed. RESULTS: One hundred and twenty-eight patients (77.1%) were diagnosed for CPP. Peak LH levels were achieved 30 min after GnRH stimulation in patients with CPP. Further, 98.4% of the 45-min samples were diagnostic for CPP, and the cumulative frequency of LH values of > or =5 IU/L was 100% at 45 min. Using this cut-off value for LH, the ROC curve for LH at 45 min showed the highest sensitivity (98.4%) and specificity (100%) in the diagnosis of CPP. CONCLUSIONS: Values of LH measured from a single blood sample obtained at 45 min in the GnRH stimulation test may be adequate for the diagnosis of CPP. Two samples, taken at 30 and 45 min after stimulation, were able to accurately diagnose CPP in 100% of the patients in this study.

Genetic Variations of GNRH1, GNRHR and GPR54 Genes in Korean Girls with Central Precocious Puberty / 대한소아내분비학회지

PURPOSE: Central precocious puberty (CPP) is defined as any sign of secondary sexual maturation appears at an age lower than two standard deviations of the mean for the average age. This process is driven by activation of hypothalamic gonadotropin releasing hormone (GnRH) secretion. Many genes expressed in the hypothalamus have been identified to play an important role in the onset and the progression of puberty. In this study, the GNRH1, its receptor (GNRHR), and kisspeptin receptor (GPR54) genes were scanned to investigate sequence alterations and their distribution in Korean girls with CPP. METHODS: One hundred and one Korean girls with CPP were recruited as the case group and 51 normal Korean women as the control group. The DNAs were extracted and amplified by polymerase chain reaction (PCR), and the products were sequenced directly. Statistical analyses were performed, and P values of C in GNRH1, and c.1091T > A in GPR54. However, the other two (c.196C > T in GNRH1 and c.546T > C in GNRHR) were novel. There was no polymorphism that was significantly associated with early onset or rapid progression of puberty. CONCLUSION: Although the size of our study population was relatively small, simple genetic variations in GNRH1, GNRHR, and GPR54 genes are not likely to be a substantial factor directly associated with the onset and progression of puberty.

Genetic Variations of GNRH1, GNRHR and GPR54 Genes in Korean Girls with Central Precocious Puberty / 대한소아내분비학회지

PURPOSE: Central precocious puberty (CPP) is defined as any sign of secondary sexual maturation appears at an age lower than two standard deviations of the mean for the average age. This process is driven by activation of hypothalamic gonadotropin releasing hormone (GnRH) secretion. Many genes expressed in the hypothalamus have been identified to play an important role in the onset and the progression of puberty. In this study, the GNRH1, its receptor (GNRHR), and kisspeptin receptor (GPR54) genes were scanned to investigate sequence alterations and their distribution in Korean girls with CPP. METHODS: One hundred and one Korean girls with CPP were recruited as the case group and 51 normal Korean women as the control group. The DNAs were extracted and amplified by polymerase chain reaction (PCR), and the products were sequenced directly. Statistical analyses were performed, and P values of C in GNRH1, and c.1091T > A in GPR54. However, the other two (c.196C > T in GNRH1 and c.546T > C in GNRHR) were novel. There was no polymorphism that was significantly associated with early onset or rapid progression of puberty. CONCLUSION: Although the size of our study population was relatively small, simple genetic variations in GNRH1, GNRHR, and GPR54 genes are not likely to be a substantial factor directly associated with the onset and progression of puberty.

Prediction of 1 Year Growth in Girls with Precocious Puberty with GnRH Agonist Treatment by Measurement of Growth Velocity at 3 Months after Treatment / 대한소아내분비학회지

PURPOSE: The aim of this study was to assess the usefulness of growth velocity at 3 months after gonadotropinreleasing hormone (GnRH) agonist treatment as a predictive value of 1 year growth velocity after treatment in girls with precocious puberty. METHODS: We studied 30 Korean girls with precocious puberty whose chronologic and bone age were less than 9 years and 11 years old, respectively at diagnosis. They treated with a 4 week interval GnRH agonist subcutaneous injections for at least more than 1 year. The patients who were treated with growth hormone simultaneously excluded for this study. Data were collected from chart review retrospectively. We measured heights and calculated growth velocities of the subjects at 3 months and 1 year after GnRH agonist treatment. RESULTS: The mean chronologic age and bone age of the subjects were 8.3 +/- 0.8 year and 10.4 +/- 0.6 years, respectively. The growth velocity at 3 months, 6 months, 9 months and 1 year after GnRH agonist treatment were 7.1 +/- 2.6, 6.6 +/- 2.1, 5.7 +/- 1.3 and 5.8 +/- 0.9 cm/yr, respectively. The positive correlation between the growth velocity at 3 months and 6 months, 9 months, 1 year after GnRH agonist treatment were shown(P < 0.001, P < 0.001, P = 0.002, respectively). CONCLUSION: In this study, the growth velocity at 1 year after GnRH agonist treatment may be predicted by the growth velocity at 3 months after treatment. The patient whose growth velocity at 3 months after GnRH agnoist treatment was low would be needed to observe the growth pattern carefully and to consider combined growth hormone treatment, if needed. It may be deserve further studies to improve final height in patients with central precocious puberty in various conditions.

Fatores de correção para perímetro escrotal ao sobreano para tourinhos mestiços Aberdeen Angus x Nelore / Adjustment factors for scrotal circumference at yearling for crossbred Aberdeen Angus x Nelore young bulls

Obtiveram-se fatores de correção (FC) para o perímetro escrotal ao sobreano (PES) para os efeitos de grupo genético (GG), heterozigose individual (HI), peso ao sobreano (PS) e idade do animal à pesagem de sobreano (IDS), utilizando-se registros de peso corporal e medidas de perímetro escrotal obtidos de 11.662 tourinhos das raças Aberdeen Angus, Nelore e de produtos do cruzamento entre elas, criados nas regiões Sul, Sudeste e Centro-Oeste do Brasil, nascidos entre 1987 e 2001. Os coeficientes de regressão que geraram os FC foram estimados pelo método dos quadrados mínimos, adotando um modelo que incluiu os efeitos de grupo de contemporâneos ao sobreano (GC), GG, heterozigose materna (HM), HI, PS e IDS. Todos os efeitos incluídos no modelo foram significativos (P<0,01) sobre PES. A média observada e respectivo desvio-padrão para PES foi 29,90±3,55cm. Foi observado efeito polinomial de segunda ordem de PS sobre PES. Foram verificados ganhos decrescentes no PES à medida que o POS aumentou, ou seja, os maiores acréscimos foram observados em animais recém desmamados. O efeito linear de PS sobre PES foi 0,06695804±0,00345000cm/kg, e o efeito quadrático, 0,00005252±0,00000508cm/kg². Para IDS o efeito linear foi de 0,02176450±0,00038568cm/dia. Aumentos de HI e HM promoveram incremento no PES e de GG, decréscimos. Os fatores incluídos no modelo foram importantes fontes de variação que devem ser considerados no ajuste de PES para seleção de tourinhos para melhora da precocidade sexual.

Adjustment factors (AF) for scrotal circumference at yearling (SCY) were figured out for effects of genetic group (GG), individual heterozygosis (IH), yearling weight (YW), and age of the animal at yearling weight (AYW) using body weight and scrotal circumference records from 11,662 Aberdeen Angus, Nelore, and their crosses. The animals were born from 1987 to 2001 and were raised in the South East and Central West Regions of Brazil. The regression coefficients to obtain AF were estimated by least squares means method. The model included the fixed effects of contemporaneous group at yearling (CG), maternal heterozygosis (MH), IH, and the covariates YW (linear and quadratic effects) and AYW (linear effect). All the factors included in the model showed significant effects (P<0.01) on SCY. The mean and standard deviation for SCY were 29.90±3.55cm. Quadratic effect of YW on SCY was also observed. Decreases in SCY with the increase in YW was found. High SCY was observed immediately after post-weaning. The YW effects on SCY were 0.06695804±0.00345000cm/kg (linear effect) and -0.00005252±0.00000508cm/kg² (quadratic effect). The AYW linear effect on SCY was 0.02176450±0.00038568cm/day. The factors included in the model are important sources of variation to adjust SCY for the selection of young bulls in order to improve sexual precocity.

Parâmetros genéticos para idade ao primeiro parto estimados por diferentes modelos para rebanhos da raça Nelore / Genetic parameters for age at first calving estimated by different models for Nellore breed herds

Com objetivo de estimar parâmetros genéticos e estudar a utilização de diferentes efeitos em avaliações genéticas para idade ao primeiro parto (IPP) por diferentes modelos, foram utilizados registros de IPP de animais da raça Nelore, nascidos entre os anos de 1990 e 2005. Foram considerados os seguintes modelos (M): M1, incluindo o efeito fixo de GC1 (constituído pelos animais nascidos na mesma fazenda e ano), além da covariável, peso aos 365 dias de idade (efeito linear e quadrático), totalizando 24.263 registros de IPP; M2, considerando os efeitos fixos de GC1, ano e estação de parição, totalizando 59.792 registros de IPP e M3, incluindo os efeitos fixos de GC2 (agrupando os animais nascidos na mesma fazenda, ano e que conceberam no mesmo manejo reprodutivo), ano e estação de parição, totalizando 59.792 registros de IPP. As estimativas dos componentes de variância e herdabilidade e os valores genéticos (VG) foram obtidos pelo método da máxima verossimilhança restrita, com a inclusão da matriz de parentesco disponível. As diferenças esperadas na progênie (DEPs) foram obtidas dividindo os VG por dois. Após a obtenção desses resultados, foram realizadas correlações entre os VG e o ranqueamento das DEPs dos reprodutores para IPP, utilizando-se o procedimento PROC CORR (SAS, 2003). Ao se considerar o ano e a estação de parto nos modelos de análise (M2 e M3), esses produziram um maior R², indicando que tais modelos conseguiram explicar, em maior grau, as diferenças existentes entre os animais para IPP. As herdabilidades estimadas foram de baixa magnitude (0,14 e 0,15). As correlações entre os VG obtidas por diferentes modelos foram 0,73 (M1 x M2); 0,91 (M2 x M3) e 0,66 (M1 x M3).

With the objective of estimate genetic parameters and study the utilization of different effects on the genetic evolution for the age of the first calving (AFC) by different models, they were used AFC records from Nellore breed animals, born from 1990 to 2005. They were considered the following models (M): M1, including the fixed effect of the contemporary group (CG), CG1 (grouping the animals that were born at the same farm and year), besides the co variable, weight at 365 days of age (linear and quadratic effects), totalizing 24,263 records of AFC; M2, considering the effects of CG1, year and season of the calving, totalizing 59,792 records of AFC and M3, including effects CG2 (grouping the animals born at the same farm, year and submitted at the same reproductive management), year and season of calving totalizing 59,792 records of AFC. The components of variance and Genetic Value (GV) were obtained by Restricted Maximun Likelihood Method, with the inclusion for the relationship matrix. The Differences Expected on the Progeny (DEPs) were obtained by dividing the GV by two, after this they were estimated the correlations between GV and ranks of the reproductor's DEPs for AFC, utilizing the procedure CORR (SAS, 2003). While considering year and season of calving in the analysis models (M2 and M3), it was produced a bigger R², indicating that those models could explain, in a larger scale, the existing differences between the animals for AFC. The heritabilities estimated were of low magnitude (0,14 and 0,15). The correlations between the GV obtained by the different models were .73 (M1 x M2); .91 (M2 x M3) and .66 (M1 x M3).

Effects of polymorphisms of LHR and FSHR genes on sexual precocity in a Bos taurus x Bos indicus beef composite population

The purpose of the present research was to investigate the effects of polymorphisms of luteinizing hormone receptor (LHR) and follicle-stimulating hormone receptor (FSHR) genes, evaluated by polymerase chain reaction-restriction fragment length polymorphism in European-Zebu composite beef heifers from six different breed compositions. The polymorphism site analysis from digestion with HhaI and AluI restriction endonucleases allowed the genotype identification for LHR (TT, CT and CC) and FSHR (GG, CG and CC) genes. A high frequency of heterozygous animals was recorded in all breed compositions for both genes, except in two compositions for LHR. The probability of pregnancy (PP) at first breeding was used to evaluate the polymorphism effect on sexual precocity. The PP was analyzed as a binary trait, with a value of 1 (success) assigned to heifers that were diagnosed pregnant by rectal palpation and a value of 0 (failure) assigned to those that were not pregnant at that time. Heterozygous heifers showed a higher pregnancy rate (67 and 66% for LHR and FSHR genes, respectively), but no significant effects were observed for the genes studied (P = 0.9188 and 0.8831 for LHR and FSHR, respectively) on the PP. These results do not justify the inclusion of LHR and FSHR restriction fragment length polymorphism markers in selection programs for sexual precocity in beef heifers. Nevertheless, these markers make possible the genotype characterization and may be used in additional studies to evaluate the genetic structure in other bovine populations.

Genetic correlation between heifer pregnancy and scrotal circumference measured at 15 and 18 months of age in Nellore cattle

Data of pregnancy diagnosis from 24,945 Nellore heifers, raised under tropical conditions in Brazil and exposed to breeding at about 14 months of age, were analyzed simultaneously with 13,742 (analysis 1), 36,091 (analysis 2), 8,405 (analysis 3), and 8,405 (analysis 4) scrotal circumference (SC) records of contemporary young bulls in order to estimate heritability (h(2)) for yearling heifer pregnancy (HP) and for SC measured at around 15 (SC15) and 18 (SC18) months of age and to estimate genetic correlation between HP and SC15 (SC18). Heifer pregnancy was considered as a categorical trait, with the value 1 (success) assigned to heifers that were detected as pregnant by rectal palpation approximately 60 days after the end of a 90-day breeding season and the value 0 (failure) otherwise. In analyses 1 and 3, SC was measured at around 15 months of age and in analysis 2 and 4 it was measured at around 18 months of age. Only 8,848 animals from datasets 1 and 2 were common in both files, which means the same animals measured at different ages. Datasets used in analyses 3 and 4 included the same animals, measured at 15 and at 18 months of age, respectively. Heritability estimates for HP were similar in all analyses, with values ranging from 0.66 +/- 0.08 to 0.67 +/- 0.008. For SC15, the estimates were 0.57 +/- 0.05 in analysis 1 and 0.60 +/- 0.07 in analysis 3. For SC18, the estimates were 0.53 +/- 0.03 in analysis 2 and 0.64 +/- 0.06 in analysis 4. The estimates of genetic correlation between HP and SC15 were 0.15 +/- 0.10 in analysis 1 and 0.11 +/- 0.11 in analysis 3. For the correlation between HP and SC18, the values were 0.27 +/- 0.10 in analysis 2 and 0.16 +/- 0.11 in analysis 4. Based on standard errors and confidence intervals, the best heritability and genetic correlation estimates were obtained from analysis 2, which included more data and a better pedigree structure. Pearson correlation between HP and SC breeding values was similar to the gene...

Update in the etiology and treatment of sexual precocity / 소아과

The pubertal activation of gonadotrophin releasing hormone(GnRH) requires coordinated changes in excitatory or inhibitory amino acids, growth factors, and a group of transcriptional regulators. The age of onset of puberty is progressing to younger age. Factors affecting early puberty include genetic traits, nutrition(body fat) and exposure to endocrine disrupting chemicals. In rapidly progressing central precocious puberty, gonadotrophin releasing hormone(GnRH) agonists(GnRHa) appear to increase final height if treated early stage. Further large scaled long-term follow-up study of the effects of GnRHa on final height is needed.

Analysis of 57 Children less than 4-Year-Old with Sexual Precocity / 实用儿科临床杂志

Objective To analyze the characteristics and diagnosis of children aged

Etiology and Age Incidence of Precocious Puberty / 대한소아내분비학회지

PURPOSE: Differential diagnosis of sexual precocity is important. Sexual precocity, defined as the appearance of secondary sexual characteristics before the age of 8 years in girls and 9 years in boys, was studied to evaluate the sex incidence and etiology of sexual precocity. METHODS: We reviewed the etiology and age incidence of precocious puberty in 14 boys and 89 girls examined between 1994. 11. 1-2002. 7. 31 at Samsung Seoul Hospital. All underwent standard anthropometric measures. They were assigned to diagnostic categories on the basis of clinical assessment, endocrine evaluation, radiologic imaging, and pelvic ultrasonography. RESULTS: In 89 girls, 27 had idiopathic precocious puberty, 3 had precocious puberty with organic brain lesion, 12 had gonadotropin-independent precocious puberty, 44 had premature thelarche, 2 had premature menarche, 1 had premature adrenarche. The majority of idiopathic precocious puberty girls(78%) were aged between 7-7.9 years. In 14 boys, 2 had idiopathic precocious puberty, 3 had precocious puberty with organic brain lesion, 6 had gonadotropin-independent precocious puberty, 3 had gynecomastia. CONCLUSION: Sexual precocity occurs more frequently in girls than boys. Premature thelarche is the most common form of sexual precosity. In boys, majority of central precocious puberty had organic brain lesion, so brain imaging study should be performed. In girls, majority of idiopathic precocious puberty were aged between 7-7.9 years. Reexamination of the age limit for defining when puberty should be considered precocious in Korean girls is necessary.

A Case of Ruptured Juvenile Granulosa Cell Tumor / 대한산부인과학회잡지

Sex cord-stromal tumors of the ovary are the third most common types of neoplasms that develop in the ovary and account for about 5-8% of all ovarian malignancies. Juvenile granulosa cell tumor (JGCT) is one of the sex cord-stromal tumors of the ovary has distinct differences from adult granulosa cell tumor (AJCT) with regard to clinical and pathological features as well as biological behavior most frequently occuring in the first two decades of life. Usually Call-exner bodies are rare, and luteinization is frequent in JGCT. The tumor may be solid, cystic, or both. In premenarcheal girls, juvenile granulosa cell tumor usually (82%) elicits the signs of sexual precocity. The tumor should removed as soon as the diagnosis is established. Surgery is the best treatment choice for low stage juvenile granulosa cell tumor in children, but for those with high stage juvenile granulosa cell tumor or recurrent tumor, the best treatment and sensitivity of tumor to radiation therapy and chemotherapy have not yet been determined clearly. About 90% are diagnosed in early stage so, prognosis of juvenile granulosa cell tumor in children is good in most cases, but tumor with more advanced stage has worse clinical outcome correlated with its stage, presence of ruptures, grade of nuclear atypia, degree of mitotic activity. And the clinical stage at the time of diagnosis is considered most important prognostic factor. We experienced a case of ruptured juvenile granulosa cell tumor so, we present a case with brief review of literature.

Sexual Precocity:Sex Incidence and Etiology / 대한소아내분비학회지

PURPOSE:Differential diagnosis of sexual precocity has been aided by new imaging techniques and endocrine evaluation. Sexual precocity, defined as the appearance of secondary sexual characteristics before the age of 8 years in girls and 9 years in boys, was studied to evaluate the sex incidence and etiology of sexual precocity. METHODS:A total 95 children(87 girls and 8 boys) who were diagnosed with sexual precocity at St. Mary's Hospital, Catholic University Medical College from 1984 to 1996, were studied. All underwent standard anthropometric measures. They were assinged to diagnostic categories on the basis of clinical assessment, endocrine evaluation, radiologic imaging, and pelvic ultrasonography. RESULTS: 1) The most common cause of sexual precocity was premature thelarche, 50 cases(52.6%) of all children. 2) Central precocious puberty was noted in 7 girls and 3 boys(female to male ratio 2.3:1). The causes were not identified in 6 cases(85.7%) in girls, but the causes were not found in 1 cases(33.3%) in boys. The idiopathic central precocious puberty occurs at least two fold more frequently in girls than in boys. 3) Incomplete precocious puberty was noted in 29 girls and 5 boys(female to male ratio 5.8:1). In the causes of 29 girls with incomplete precocious puberty, congenital adrenal hyperplasia were found in 23 cases, autonomous functional ovarian cyst in 5 cases and McCune-Albright syndrome in 1 case. In the causes of 5 boys with incomplete precocious puberty, virilizing adrenal cortical tumor were found in 3 cases, congenital adrenal hyperplasia in 1 case, and hepatoblastoma in 1 case. CONCLUSION: Sexual precocity occurs at least ten fold more frequently in girls than in boys. Premature thelarche is the most common form of sexual precocity.