Organic Central Precocious Puberty Research Articles

Central precocious puberty in boys: secular trend and clinical features.

Recent studies suggest that boys enter puberty at a younger age, and the incidence of male central precocious puberty (CPP) is increasing. In this study, we explore the incidence of male CPP and identify key clinical and auxological indicators for organic CPP (OCPP). A retrospective registry-based study. The medical records of 43 boys treated with CPP at the Helsinki University Hospital between 1985 and 2014 were reviewed. Clinical, auxological, and endocrine data of the CPP patients were included in the analyses. Based on brain MRI, 26% of patients had OCPP. Between 2010 and 2014, the CPP incidence in boys was 0.34 per 10 000 (95% CI 0.20-0.60). Between 1990 and 2014, the male CPP incidence increased (incidence rate ratio [IRR] 1.10, P = .001). This increase was driven by rising idiopathic CPP (ICPP) incidence (IRR 1.11, 95% CI 1.05-1.19, P < .001), while OCPP incidence remained stable (P = .41). Compared with the patients with ICPP, the patients with OCPP were younger (P = .006), were shorter (P = .003), and had higher basal serum testosterone levels (P = .038). Combining 2 to 4 of these readily available clinical cues resulted in good to excellent (all, area under the curve 0.84-0.97, P < .001) overall performance, differentiating organic etiology from idiopathic. The estimated incidence of CPP in boys was 0.34 per 10 000, with 26% of cases associated with intracranial pathology. The increase in CPP incidence was driven by rising ICPP rates. Patients with OCPP were characterized by shorter stature, younger age, and higher basal testosterone levels, providing valuable cues for differentiation in addition to brain MRI. Utilizing multiple cues could guide diagnostic decision-making.

Psychological aspects of precocious puberty child during the COVID-19 pandemic

HIGHLIGHTS 1. Presents a longitudinal case of a child with Organic Central Precocious Puberty (CPP).2. Focused on psychological aspect during the COVID-19 pandemic. ABSTRACT Objective: To present a longitudinal case of a child with organic Central Precocious Puberty (CPP) that focused on medical, growth and development, and parent’s psychological aspect during the COVID-19 pandemic. Case Report: A 14-month old girl attended with major complaints of breasts enlargement and menstruation. The Tanner's stage was at A1M3P1 and the vagina showed reddish-brown spots. The patient’s bone age was advanced (3 years and 6 months). USG examination showed a corpus uterine: cervix ratio of 2:1. GnRH stimulation test showed an elevated of FSH/LH and estradiol. MRI showed an extra-axial dense mass that leads to Hypothalamic Hamartoma (HH). The definitive diagnosis of this patient was organic CPP with HH. The patient was managed with GnRH analog. Precocious puberty (PP) becomes a financial and psychosocial burden for parents. The COVID-19 pandemic adds a double burden for the parents. Conclusion: During the COVID-19 pandemic, parents with PP children had a good psychological aspect if the child was comprehensively handled with adequate motivation and psychoeducation.

Pathological brain lesions in girls with central precocious puberty at initial diagnosis in Southern Vietnam.

PurposeCranial magnetic resonance imaging (MRI) is recommended to identify intracranial lesions in girls with central precocious puberty (CPP). Yet, the use of routine MRI scans in girls with CPP is still debatable, as pathological findings in girls 6 years of age or older with CPP are limited. Therefore, we aimed to identify the prevalence of brain lessons in CPP patients stratified by age group (0–2, 2–6, and 6–8 years).MethodsThis retrospective cross-sectional study recruited 257 girls diagnosed with CPP for 6 years (2010–2016). MRI was used to detect brain abnormalities. Levels of luteinizing hormone, follicle-stimulating hormone, and sex hormones in blood samples were measured.ResultsMost girls had no brain lesions (82.9%, n=213), and of the minor proportion of girls with CPP that exhibited brain lesions (17.1%, n=44), 32 girls had organic CPP. Pathological findings were detected in 33.3% (2 of 6) of girls aged 0–2 years, 15.6% (5 of 32) of girls aged 2–6 years, and 3.6% (8 of 219) of girls aged 6–8 years. Hypothalamic hamartoma and tumors in the pituitary stalk were the most common pathological findings. The likelihood of brain lesions decreased with age. Girls with organic CPP were more likely to be younger (6.1±2.4 vs. 7.3±1.3 years, P<0.01) than girls with idiopathic CPP.ConclusionsOlder girls appeared to have a lower prevalence of organic CPP. Clinicians should cautiously use cranial MRI for girls aged 6–8 years with CPP.

Neuroimaging in 205 consecutive Children Diagnosed with Central Precocious Puberty in Denmark.

A brain magnetic resonance image (MRI) is considered part of routine evaluation in children diagnosed with central precocious puberty (CPP) to rule out intracranial pathology. We evaluated the occurrence of pathological findings on neuroimaging among children diagnosed with CPP. A retrospective study based on an evaluation of 1544 children referred with early signs of puberty from 2009-2019. Of these, 205 children (29 boys) with confirmed CPP had a brain MRI performed, and we report MRI results, pubertal stage, bone age, and hormonal analyses. All abnormal MRI results were re-evaluated by a trained neuroradiologist. A new intracranial pathology was found by brain MRI in 6 out of 205 patients aged 1.5 to 6.1 years. The occurrence of intracranial pathology was 3/162 (1.8%) and 3/24 (12.5 %) in girls and boys respectively. Organic causes of precocious puberty are more frequent in boys with CPP than in girls. No cases of organic CPP were seen above age 6.1 years of age. The age cut off value for routine brain MRI could be lowered to seven or perhaps even six years of age for girls, except in rapidly progressing puberty or presence of neurological symptoms. In our study of children with central precocious puberty (CPP), intracranial pathology is a rare cause and occurs only in younger children. It supports the general trend, that younger children are at higher risk of having organic causes to CPP and supports the clinical practice, that only high-risk patients with CPP should undergo routine brain MRI.

Is cranial imaging necessary in girls between 6-8 years diagnosed with central precocious puberty?

There is no clear consensus on whether a cranial MRI should be performed in all cases of central precocious puberty(CPP). In this study, we aimed at evaluating the incidence of intracranial lesions and analyzing cranial imaging results in females with CPP. In the retrospective study medical records of the case, the age at the time of admission, anthropometric measurements, bone age, Tanner stages, serum follicle-stimulating hormone (FSH), serum luteinizing hormone(LH), serum estradiol (E2) levels, the peak LH level during the gonadotropin-releasing hormone (GnRH) stimulation test and the cranial MRI findings at the time of the diagnosis of CPP were collected. The mean age diagnosis of the 154 girls included in the study was 6.9 ±1.08. Nine (5.8%) of 154 patients were diagnosed with organic-caused CPP. Four of the nine cases diagnosed with organic CPP had a previously known CNS pathology. The other five cases did not have any neurological findings at the time of diagnosis. Incidental lesions were detected at cranial MRI of nine of the 145 cases diagnosed with idiopathic CPP. The basal E2, basal LH, basal FSH, peak LH and peak LH/FSH levels of the cases with organic CPP were higher than those with idiopathic CPP. In our study, approximately 90% of organic CPP due to intracranial lesions were between 6-8 years. Therefore, we believe that cranial imaging should be performed in all females with CPP.

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

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). 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. 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). 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.

OR15-04 Central Precocious Puberty without Central Nervous System Lesions: Is It Really Idiopathic?

Background: The etiological diagnosis of central precocious puberty (CPP) has been classically divided into causes with or without central nervous system (CNS) lesions. Among the cases without CNS lesions, most of them are classified as idiopathic. In clinical practice, about 90% of girls and 40% of boys with CPP are considered having the idiopathic form. In the last two decades, pioneering studies have revealed underlying genetic causes in patients with apparently idiopathic CPP.Objective: To describe the frequency of genetic causes identified in a large cohort of patients with CPP followed in a single research center and to evaluate its role in the distribution of the etiology of CPP.Patients and methods: A retrospective evaluation was performed analyzing the etiological diagnosis of 276 patients (246 girls, 30 boys) with CPP followed in a single university hospital outpatient clinic from 2006 to 2019. The great majority (230 patients) presented without CNS lesions, being classified as idiopathic CPP group. Among the idiopathic CPP group, 170 of them had DNA samples available and were included for genetic analysis. Patients included for genetic analysis were systematically investigated for genetic causes of CPP using standard methodologies of genetic-molecular analysis. Briefly, they were studied as follows: 120 by Sanger sequencing; 18 by target panel sequencing; 27 by whole-exome sequencing; 5 by whole-genome sequencing; 113 by specific DNA methylation analysis; and 38 by genomic microarray.Results: Among the 276 patients with CPP, 46 (16.7%) had pathological CNS lesions: 19 boys and 27 girls, indicating the prevalence of CPP with CNS lesions (organic) of 63.3% in boys and 11% in girls. The most common cause of organic CPP was hypothalamic hamartoma (20 cases). Meanwhile 230 patients (83.3%) encompassed the apparently idiopathic CPP group. Main characteristics of this idiopathic CPP group were: 219 girls and 11 boys; 158 sporadic (69%), 68 familial (29.5%) and 4 adopted (1.5%). In the subset of patients with DNA available (162 girls, 8 boys), the frequency of genetic causes was 11.8% (20 cases: 18 girls and 2 boys). Analyzing by sex, the frequency of genetic causes was higher in boys (25%) than in girls (11.1%). The identified genetic defects were the following: 9 cases with inactivating MKRN3 mutations (8 families), 6 cases with inactivating DLK1 mutations (2 families), 1 case with activating KISS1R mutation, 1 case with activating KISS1 mutation, 2 sporadic cases with maternal uniparental disomy of chromosome 14, and 1 sporadic case with epimutation at DLK1 locus.Conclusion: Pathogenic genetic defects were identified in 11.8% of patients with apparently idiopathic CPP involving four distinct genes. Altogether, these genetic findings indicate a context of changing in the distribution of the etiological diagnosis of CPP in both sexes, highlighting the genetic causes.

Gender-related differences in etiology of organic central precocious puberty

Central precocious puberty (CPP) is idiopathic in 90% of girls and 60% of boys, while some cases are caused by lesions of central nervous system (CNS), a condition often referred to as organic CPP. We aimed to analyze the etiology of organic CPP in a large cohort of girls and boys and determine gender-related differences. Medical files of 256 girls and 120 boys diagnosed and treated for CPP in a single center in the last two decades were reviewed. Patients were classified into four groups with respect to previous history and MRI findings: (1) previously established CNS pathology at the time of diagnosis, (2) novel CNS pathology previously asymptomatic, (3) incidentalomas considered to be unrelated to CPP, and (4) completely normal MRI. Group 1 and 2 were considered as organic CPP whereas group 3 and 4 were considered as idiopathic CPP. Prevalence of CNS pathology was significantly higher in boys than girls (21.7% vs 6.2%). Previous CNS pathologies such as developmental anomaly of CNS, parenchymal injury, necrotic lesions and hydrocephalus were present in 3.5% of girls and 8.3% of boys. Prevalence of novel CNS pathology as determined by imaging among neurologically asymptomatic patients was 2.8% in girls and 14.5% in boys. The most common novel CNS pathologies in boys were hamartomas (5%) and suprasellar arachnoid cysts (3.3%); which were significantly lower in girls (0.8 and 0.8% respectively). Onset of organic CPP was before six years in girls, and seven years in boys. Organic CPP was 3.5 times more common in boys compared to girls. It is possible to detect an underlying CNS pathology in one out of every five boys with CPP. Frequency and distribution of organic etiology also differ between girls and boys, hypothalamic hamartomas and suprasellar arachnoid cysts being more common in boys than girls. The likelihood of novel intracranial pathology associated with CPP is quite low in girls with an onset after six years of age and in boys with an onset after seven years of age.

Etiological trends in male central precocious puberty.

Purpose In the present study, the etiological trends in male central precocious puberty (CPP) were examined, and annual distribution was evaluated.Methods Seventy-one male CPP subjects who started puberty before 9 years of age were included in this study. All individuals were diagnosed as having CPP at Samsung Medical Center between 2001 and 2016. Chronological age at puberty onset, diagnosis of CPP, bone age, weight (kg), height (cm), puberty stage, brain magnetic resonance imaging findings, testosterone level, basal gonadotropin level, and gonadotropin level after gonadotropin releasing hormone stimulation were analyzed.Results The 71 patients were divided into 2 groups: idiopathic (group I) and organic (group II) when the lesion was identified as associated with the central nervous system (CNS) or when the patient received chemotherapy for non-CNS tumors before CPP diagnosis, respectively. Forty-four cases (62%) were idiopathic, and 27 (38%) were organic. The proportion of idiopathic CPP was higher than that of organic CPP during the study period. In 51.9% of organic cases, puberty started before 8 years of age, whereas it started after that age in 93.2% of the idiopathic cases.Conclusions In the present study, among all male CPP cases, 62% were idiopathic. The probability of idiopathic CPP prevalence was higher in males when the puberty onset was after 8 years of age with no history of cranial radiotherapy or chemotherapy.

Long-Term Outcomes of Patients with Central Precocious Puberty due to Hypothalamic Hamartoma after GnRHa Treatment: Anthropometric, Metabolic, and Reproductive Aspects

Background: Hypothalamic hamartoma (HH) represents the commonest cause of organic central precocious puberty (CPP). Follow-up of these patients in adulthood is scarce. Objective: To describe the anthropometric, metabolic, and reproductive parameters of patients with CPP due to HH before and after treatment with gonadotropin-releasing hormone analog (GnRHa). Methods: We performed a retrospective and cross-sectional study in a single tertiary center including 14 patients (7 females) with CPP due to HH. Results: The mean duration of GnRHa treatment was 7.7 ± 2.4 years in boys and 7.9 ± 2.1 years in girls. GnRHa treatment was interrupted at the mean chronological age (CA) of 12.1 ± 1.1 years in boys and 10.7 ± 0.5 years in girls. At the last visit, the mean CA of the male and female patients was 21.5 ± 3.2 and 24 ± 3.9 years, respectively. Eleven of the 14 patients reached normal final height (FH) (standard deviation score -0.6 ± 0.9 for males and -0.6 ± 0.5 for females), all of them within the target height (TH) range. The remaining 3 patients had predicted height within the TH range. The mean body mass index and the percentage of body fat mass was significantly higher in females, with a higher prevalence of metabolic disorders. All patients presented normal gonadal function in adulthood, and 3 males fathered a child. Conclusion: All patients with CPP due to HH reached normal FH or near-FH. A higher prevalence of overweight/obesity and hypercholesterolemia was observed in the female patients. Finally, no reproductive disorder was identified in both sexes, indicating that HH per se has no deleterious effect on the gonadotropic axis in adulthood.

Central precocious puberty following the diagnosis and treatment of paediatric cancer and central nervous system tumours: presentation and long-term outcomes.

To estimate the prevalence of central precocious puberty (CPP) after treatment for tumours and malignancies involving the central nervous system (CNS) and examine repercussions on growth and pubertal outcomes. Retrospective study of patients with tumours near and/or exposed to radiotherapy to the hypothalamus/pituitary axis (HPA). Patients with CPP were evaluated at puberty onset, completion of GnRH agonist treatment (GnRHa) and last follow-up. Multivariable analysis was used to test associations between tumour location, sex, age at CPP, GnRHa duration and a diagnosis of CPP with final height <-2SD score (SDS), gonadotropin deficiency (LH/FSHD) and obesity, respectively. Eighty patients (47 females) had CPP and were followed for 11·4 ± 5·0 years (mean ± SD). The prevalence of CPP was 15·2% overall, 29·2% following HPA tumours and 6·6% after radiotherapy for non-HPA tumours. Height <-2SDS was more common at the last follow-up than at the puberty onset (21·4% vs 2·4%, P = 0·005). Obesity was more prevalent at the last follow-up than at the completion of GnRHa or the puberty onset (37·7%, 22·6% and 20·8%, respectively, P = 0·03). Longer duration of GnRHa was associated with increased odds of final height <-2SDS (OR = 2·1, 95% CI 1·0-4·3) and longer follow-up with obesity (OR = 1·3, 95% CI 1·1-1·6). LH/FSHD was diagnosed in 32·6%. There was no independent association between CPP and final height <-2SDS, and LH/FSHD and obesity in the subset of patients with HPA low-grade gliomas. Patients with organic CPP experience an incomplete recovery of growth and a high prevalence of LH/FSHD and obesity. Early diagnosis and treatment of CPP may limit further deterioration of final height prospects.

Premature pubarche before one year of age: distinguishing between mini-puberty variants and precocious puberty.

BackgroundThe aim of this study was to facilitate the distinction between the benign “mini-puberty of early infancy” and precocious puberty (PP).Material/MethodsWe compared 59 patients (21 boys and 38 girls) seen for pubic hair development before one year of age diagnosed as mini-puberty to 13 patients (2 boys) in whom pubertal development before one year revealed a PP.ResultsThe boys with mini-puberty presented with pubic hair development and prepubertal testicular volume, with low plasma testosterone concentrations. Their gonadotropin responses to gonadotropin releasing hormone (GnRH) test showed predominant luteinising hormone increase in 9/13. The girls presented with pubic hair development that was accompanied by breast development in 47% of cases, with low plasma estradiol concentrations. Their gonadotropin responses showed predominant follicle-stimulating hormone increase in the 17 evaluated.The patients with PP had organic central PP (5 hypothalamic hamartoma) or idiopathic central PP (n=6), or peripheral PP (one ovarian tumor and one congenital adrenal hyperplasia). The diagnosis was challenging only in 3 girls with idiopathic central PP presenting with prepubertal plasma estradiol concentrations and responses to GnRH test.ConclusionsThe diagnosis of PP was easily determined based on the clinical presentation and the pubertal concentrations of testosterone in boys or of estradiol in girls, as was the diagnosis of central or peripheral origin of PP based on gonadotropin response to the GnRH test. Once PP is excluded, these patients need careful follow–up and physician consultation is needed if clinical pubertal signs progress.

Diagnostic Work-Up of 449 Consecutive Girls Who Were Referred to be Evaluated for Precocious Puberty

A decrease in age at pubertal onset has been observed internationally. The aim of this study was to describe a large cohort of Caucasian girls referred with signs of early puberty according to etiology and compare biochemical characteristics. In this single-center study, we included 449 consecutive Caucasian girls who were referred with signs of early puberty during the years 1993-2009. We evaluated pubertal stage, height, weight, and bone age. FSH, LH, estradiol, and inhibin B were determined, and a standard GnRH test was performed. Brain magnetic resonance imaging was performed to rule out pathologies. During the period from 1993-2008, we found an increase in the number of girls in most diagnostic groups. Of 449 girls, 88 had central precocious puberty (CPP), and 12 of these had an organic origin. A total of 129 had early-normal variant (8-9 yr), 69 had premature thelarche, and 49 premature adrenarche. Receiver operating characteristic analyses revealed that basal LH was superior in predicting the maximal LH level during GnRH testing in comparison with FSH, estradiol, and inhibin B levels. Basal LH levels were above the age-related 2 sd in 26.2, 19.6, 65.1, and 75.0% of girls with, respectively, early-normal variant, premature thelarche, idiopathic CPP, and organic CPP, but LH levels below the detection limit were also seen among girls with a pubertal GnRH test. We observed an increasing number of girls referred because of early pubertal signs. An elevated basal LH was highly predictive of a pubertal GnRH test result, whereas a low LH did not exclude central pubertal activation.

Clinical and laboratory characteristics of female precocious puberty

OBJECTIVE: To investigate the etiologic characteristics of female precocious puberty and to compare the clinical and laboratory characteristics of patients with idiopathic and organic central precocious puberty.

Pubertad precoz completa isosexual: hallazgos clínicos, de laboratorio y ecografía pélvica

To determine whether initial presentation varies according to aetiology, whether such differences allow differential diagnosis between idiopathic and organic forms, and whether CNS imaging can be avoided in some patients with central precocious puberty (CPP). Children referred for evaluation of precocious puberty were evaluated, and the subpopulation of children with CPP was enrolled in this prospective observational study. Clinical, laboratory and ultrasound features of 62 consecutive patients with CPP (5 boys and 57 girls) were recorded. We compared the characteristics of idiopathic (3 boys, 49 girls) and organic (2 boys, 8 girls) CPP. There were no differences in pubertal staging, age at puberty onset (7.0 [5.8-7.5] vs. 7.3 [5.1-8.3] years), bone age/chronological age ratio (1.26 [1.2-1.3] vs. 1.23 [1.1-1.3]), maternal menarche (11.7+/-0.2 vs. 11.7+/-0.6 years) between idiopathic and organic CPP, respectively. Organic CPP patients had a poorer height SD (0.35+/-0.4 vs. 1.6+/-0.1; p<0.01), predicted adult height, growth rate and growth rate SD (0.8+/-0.9 vs. 3.7+/-0.7). Girls with organic CPP had significantly higher oestradiol levels (47.5 [25-68] vs. 27 [14-43] pg/ml) than girls with idiopathic CPP. Pelvic ultrasound at the time of diagnosis revealed the presence of pubertal changes in internal genitalia in 43.9% of girls (37.2% idiopathic versus 62.5% organic CPP subpopulation; p=0.18). There is a clinical-ultrasound overlap between idiopathic and organic CPP. Imaging remains necessary in all cases of central precocious puberty, and ultrasound data should not be replaced by other diagnostic investigations.

Predictive factors for organic central precocious puberty and utility of simplified gonadotropin‐releasing hormone tests

The aim of the present study was to determine whether the clinical presentation of patients with central precocious puberty (CPP) permits differentiation between idiopathic and organic forms, and to examine whether luteinizing hormone (LH) determination in single blood sample after gonadotropin-releasing hormone (GnRH) administration is sufficient to diagnose CPP. Potential clinical and laboratory predictors for the presence of central nervous system (CNS) abnormalities were assessed. Sensitivities and specificities of LH and follicle-stimulating hormone (FSH) levels at 0, 15, 30, 60, 90 and 120 min were compared after GnRH stimulation. In 45 girls with signs of breast development, 26 were diagnosed as having CPP. The age of onset in patients with organic CPP was 4.75 +/- 2.01 years (range 1.2-7.1 years, median 5.0 years), whereas the age in patients with idiopathic CPP was 7.09 +/- 0.87 years (range 5.0-7.9 years, median 7.0 years). This parameter is the only one showing statistical significance. In addition, the specimen at 30 min after GnRH stimulation yielded highest sensitivity for the diagnosis of CPP. The earlier the onset of disease, the higher the possibility of presence of CNS lesion. According to the mean GnRH-stimulated LH levels and sensitivity at each time, a single blood sample obtained for LH determined after GnRH administration at 30 min can be used to diagnose CPP.

El tratamiento con triptorelina en las niñas con pubertad precoz central provoca incremento del índice de masa corporal

The most important complications of central precocious puberty (CPP) in girls are loss of height and multiple psychosocial problems. To study the effect of triptorelin therapy in a cohort of girls with CPP. Thirty-four girls diagnosed with organic or idiopathic CPP and treated with monthly triptorelin were studied. Age, height in standard deviation (SD), bone age (Greulich and Pyle), height prediction (Bayle-Pinneau), body mass index (BMI) in SD, uterine size (pelvic ultrasound), target height, cranial magnetic resonance imaging, triptorelin dose, and treatment duration were studied. Triptorelin produced a statistically significant reduction in growth velocity and an increase in BMI after 1 year of therapy and these changes were maintained after discontinuation of therapy. Adult height in these patients was in accordance with their target genetic height, as well as with their predicted height according to the method of Bayley-Pinneau. No significant differences were found between age of menarche in our patients and in controls. Adult height in patients with organic CPP was significantly lower than that in patients with idiopathic CPP. 1. Triptorelin can increase BMI in girls with CPP. 2. The presence of an organic cause in patients with CPP worsens the prognosis for adult height. 3. The Bayley-Pinneau prediction method for "average" bone age is useful for establishing a prognosis of adult height in girls with CPP treated with triptorelin.

Presentation and evolution of organic central precocious puberty according to the type of CNS lesion

To evaluate the influence of the type and treatment of CNS lesion causing central precocious puberty (CPP) on the presentation, hypothalamic-pituitary function and final height. One hundred patients with CPP caused by central nervous system (CNS) lesion. The CPP was the presenting symptom of the lesion in 25 (10 boys) and occurred in 75 patients (23 boys) previously treated for lesions. These were optic glioma or astrocytoma (n = 45), hydrocephalus (n = 22), hypothalamic hamartoma (n = 15), suprasellar arachnoid cyst (n = 10) and others (n = 8). The percentages of patients with increased height, bone age advance, testicular volume, LH/FSH peaks ratio after gonadotrophin-releasing hormone (GnRH) test and plasma testosterone concentration in boys and oestradiol in girls varied from one aetiology to another. The boys with hamartoma were significantly taller and had greater bone age advance, LH peak and testosterone than boys with optic glioma. The girls with hamartoma and suprasellar arachnoid cyst were significantly younger and had greater LH peak than girls in the other groups. All patients treated for optic glioma had hypothalamic-pituitary deficiencies, including GH (100%), thyrotrophin (71.4%), corticotrophin (12.5%) and pubertal (34.3%) deficiencies. Sixty percent of those with suprasellar cysts lacked GH. Final height was below -2 SD in 15/59 (25%) patients, including 5/11 not treated with GnRH analogue, 3/5 not treated with GH despite GH deficiency, and 2 with hydrocephalus as a result of meningomyelocele. The type of CNS lesion influences the presentation of CPP. This is probably caused by differences in the mechanisms inducing puberty and to the hypothalamic-pituitary deficiencies associated with the CPP as a result of a lesion and/or its treatment.

Management and outcome of central precocious puberty

Management and outcome of central precocious puberty

Effect of Gonadotropin-Releasing Hormone Agonist Treatment in Boys with Central Precocious Puberty: Final Height Results

Objective: The small number of boys present in most studies on final height (FH) after gonadotropin-releasing hormone agonist (GnRHa) treatment for central precocious puberty (CPP) offers difficulties in the evaluation of the effects of treatment on FH in males. Method: We therefore combined FH data from The Netherlands, Italy and France to study the effect of GnRHa treatment in a large group of 26 boys with CPP. Results: The mean chronological age at the start of treatment was 7.6 ± 2.0 (SD) years, bone age (BA) was 11.0 ± 2.1 years. All boys were treated with depot formulations of the GnRHa triptorelin with established gonadal suppression for a mean treatment period of 4.7 ± 2.1 years. FH was 172.9 ± 6.6 cm. FH standard deviation score (SDS) was –0.66 ± 1.22, not significantly different from the target height SDS of –0.23 ± 0.75. FH-SDS was significantly lower in the subgroup of 12 patients with organic CPP compared to patients with idiopathic CPP (–1.34 ± 1.06 vs. –0.08 ± 1.06, respectively; p = 0.01), but no difference in height gain was observed. The mean estimated height gain, defined as the difference between predicted and actual adult height was 6.2 ± 8.7 cm using the average tables of Bayley and Pinneau, and 0.3 ± 8.6 cm using the BA advance adjusted tables. Regional differences in height gain were observed between the different countries, reflecting different local practices. Conclusion: We conclude that GnRHa treatment in boys results in a FH close to target height.