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2.
Arch. argent. pediatr ; 120(1): S1-S8, feb 2022. tab, ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1353845

ABSTRACT

Desde hace varias décadas, los análogos de la hormona liberadora de gonadotrofinas (aGnRH) son el tratamiento de elección en la pubertad precoz central (PPC) en niñas y en niños. Causan una inhibición del eje hipotálamo-hipófiso-gonadal, disminuyen la secreción de gonadotrofinas, estradiol y testosterona; como consecuencia, producen una regresión de los caracteres sexuales secundarios durante el tratamiento. En los últimos años, estos análogos también se utilizan en adolescentes transgénero, en adolescentes y adultas jóvenes con enfermedades oncológicas, en algunas situaciones muy particulares en niños y niñas con talla baja, y en pacientes con trastornos del neurodesarrollo. En Argentina, los más utilizados son el acetato de triptorelina y el acetato de leuprolide en sus formas de depósito. Estos medicamentos han demostrado eficacia y seguridad. El objetivo de esta publicación es realizar una revisión y actualización del uso de los aGnRH en niños, niñas y adolescentes.


For several decades, gonadotropin releasing hormone analogs (GnRHa) are the medical treatment selected for central precocious puberty (CPP) in girls and boys. They generate an inhibition of the hypothalamus-pituitarygonadal axis decreasing LH, FSH, estradiol and testosterone secretion and, in this way, they produce a regression of secondary sexual characters under treatment. In the last years, these analogs are also used in trans adolescents, in adolescents and young adults with oncological diseases, in some very particular situations in children with short stature and in patients with neurodevelopmental disorders. In Argentina the most commonly used formulations are triptorelin and leuprolide acetate depot forms. These analogs have proven both their efficacy and their safety. The aim of this paper is to review and update about the use of GnRHa in children and adolescents.


Subject(s)
Humans , Male , Female , Child , Adolescent , Puberty, Precocious/drug therapy , Gonadotropin-Releasing Hormone/analogs & derivatives , Luteinizing Hormone , Gonadotropin-Releasing Hormone/therapeutic use , Leuprolide/therapeutic use , Triptorelin Pamoate/therapeutic use
3.
An. bras. dermatol ; 97(1): 58-62, Jan.-Feb. 2022. graf
Article in English | LILACS | ID: biblio-1360094

ABSTRACT

Abstract McCune - Albright syndrome is a genetic disease with cutaneous mosaicism caused by post-zygotic activating mutations in GNAS locus, it has a triad of fibrous bone dysplasia, café-au-lait macules and precocious puberty. We examined a 22-year-old female patient with café au lait spot in right side of the abdomen, with a chessboard - like distribution, extending to right thigh with geographical contours, she has also an ovarian cyst, scoliosis and truncal obesity. Biopsies were taken from the hyperpigmented area and processed for light microscopy and for transmission electron microscopy. Light microscopy showed increased melanin pigment with HE staining. Immunohistochemistry with melanocytic markers (HMB-45 and Melan-A) revealed a normal number of melanocytes. Transmission electron microscopy demonstrated normal epidermal structures, such as desmosomes, cytokeratin filaments and hemidesmosomes. With high magnifications an irregular melanossomal contour was seen, with some indentations in their outline.


Subject(s)
Humans , Female , Adult , Young Adult , Puberty, Precocious , Fibrous Dysplasia of Bone , Fibrous Dysplasia, Polyostotic/diagnosis , Cafe-au-Lait Spots , Microscopy, Electron, Transmission
4.
Arch. endocrinol. metab. (Online) ; 65(6): 758-767, Nov.-Dec. 2021. tab, graf
Article in English | LILACS | ID: biblio-1349985

ABSTRACT

ABSTRACT Objective: Contrast-enhanced brain magnetic resonance imaging (MRI) is routinely performed in children with central precocious puberty (CPP). We evaluated the value of a dedicated sellar MRI protocol without contrast enhancement in girls with CPP. Subjects and methods: This study included 261 girls diagnosed with CPP. We performed sellar MRI scanning without gadolinium enhancement of the hypothalamic-pituitary area (HPA) at the pituitary level, including additional T2-weighted imaging of whole-brain scans to check for other lesions. We evaluated the prevalence of intracranial lesions via this MR protocol. In addition,the correlation between the clinical parameters and morphology of the pituitary gland on the images was assessed. Results: Intracranial lesions were detected in 17 (6.5%) of the 261 girls. Of the 17 girls with abnormalities, 16 (94.1%) had findings in brain areas other than the HPA. The weight, height, Tanner stage of patients were significantly (p < 0.05) higher in the group with greater pituitary height. Patient weight and height, Tanner stage of breast development, and luteinizing hormone (LH) levels were significantly (p < 0.05) greater in those with a higher pituitary grade as determined on sellar MRI. Conclusion: A dedicated unenhanced sellar MRI protocol provides valuable information on brain lesions and pituitary morphology. We found a significantly low prevalence of brain lesions among girls with CPP. Analysis of the height or shape of the pituitary gland on sellar MRI revealed significant correlations with the weight, height, Tanner stage, and LH levels of the patients.


Subject(s)
Humans , Female , Puberty, Precocious/epidemiology , Puberty, Precocious/diagnostic imaging , Luteinizing Hormone , Magnetic Resonance Imaging , Prevalence , Contrast Media , Gadolinium
5.
Rev. cuba. invest. bioméd ; 40(2): e627, 2021.
Article in Spanish | LILACS, CUMED | ID: biblio-1347462

ABSTRACT

Introducción: El inicio de la pubertad está determinado por la interacción entre factores genéticos y reguladores. En las niñas ocurre alrededor de los ocho años. Puede afectarse cuando existe una pubertad adelantada, pubertad precoz o una variante de la pubertad. Para su diferenciación los estudios imagenológicos se han convertido en una herramienta vital. Objetivo: Describir aspectos fisiopatológicos, clínico-epidemiológicos y del diagnóstico por imágenes de la pubertad precoz y de sus variantes puberales en las niñas. Métodos: Se realizó una revisión sistemática a través de términos de búsqueda según descriptores en ciencias de la salud. Se utilizaron artículos de libre acceso en las bases de datos de PubMed, SciELO y Google Académico. De un total de 125 artículos, se utilizaron 45 de mayor interés: 34 en idioma español y nueve en inglés, que incluyeron libros y revistas. Análisis e integración de la información: Constituye un desafío distinguir la pubertad precoz de la telarquia, pubarquia, adrenarquia o menarquia aislada de aparición precoz, ya que son variantes del desarrollo puberal. Para su diagnóstico es importante descartar una causa tratable subyacente. El examen físico, análisis de laboratorio y estudios por imágenes, permiten diferenciar las formas completas de sus variantes y las posibles causas que determinan la estimulación de la producción hormonal. Conclusiones: El conocimiento del desarrollo normal de la pubertad y la aparición de sus variantes en las niñas resultan de gran utilidad. Su detección temprana ayudaría a recibir atención médica y descartar mediante métodos de imágenes sus causas secundarias(AU)


Introduction: The start of puberty is determined by the interaction between genetic and regulatory factors. Among girls this occurs around the age of eight years. However, the process may be affected by the presence of early puberty, precocious puberty or a variant of puberty. Imaging studies have become a vital tool for their differentiation. Objective: Describe the pathophysiological, clinical-epidemiological and imaging diagnostic aspects of precocious puberty and its pubertal variants in girls. Methods: A systematic review was conducted using search terms obtained from health sciences descriptors. The documents reviewed were open access papers from the databases PubMed, SciELO and Google Scholar. Of a total 125 papers retrieved, a selection was made of the 45 of greatest interest: 34 in Spanish and 9 in English, including books and journals. Data analysis and integration: Distinguishing precocious puberty from thelarche, pubarche, adrenarche or isolated premature menarche is a challenge, for these are variants of pubertal development. Ruling out an underlying treatable cause is important for diagnosis. Physical examination, laboratory analysis and imaging studies make it possible to differentiate complete forms from their variants and the possible causes determining the stimulation of hormone production. Conclusions: Knowledge about the normal development of puberty and the emergence of its variants in girls is very useful. Early detection would pave the way for medical care, including imaging studies to rule out its secondary causes(AU)


Subject(s)
Humans , Female , Physical Examination , Puberty, Precocious , Medical Care , Adrenarche
6.
Arch. argent. pediatr ; 119(5): e420-e427, oct. 2021. tab, ilus
Article in English, Spanish | LILACS, BINACIS | ID: biblio-1292096

ABSTRACT

Introducción. El síndrome de McCune-Albright (SMA) es un trastorno genético caracterizado por displasia ósea fibrosa, manchas cutáneas color "café con leche" e hiperfunción autónoma de uno o varios órganos endocrinos. El SMA es producido por mutaciones activadoras del gen GNAS1. La endocrinopatía más frecuente es la gonadal, que se manifiesta como pubertad precoz periférica. Objetivo. Describir las características clínicas y los estudios de laboratorio e imágenes en el momento del diagnóstico y a lo largo de la evolución de la enfermedad, con énfasis en la tríada clásica del síndrome. Población y métodos. Estudio clínico observacional, descriptivo, retrospectivo de las historias clínicas de pacientes con SMA de la División de Endocrinología del Hospital de Niños Ricardo Gutiérrez desde 1974 hasta 2019. Resultados. Se presentan 12 niñas. Todas tuvieron pubertad precoz periférica (PPP) secundaria a quistes ováricos funcionantes. La edad de presentación fue temprana (2,6 ± 1,3 años). Los niveles de gonadotrofinas estuvieron suprimidos o en rango prepuberal con niveles de estradiol generalmente elevados. Diez niñas tuvieron manchas "café con leche" desde el nacimiento. Durante la evolución se detectó displasia fibrosa poliostótica en todas las pacientes. Los tratamientos utilizados para disminuir la recurrencia de los quistes ováricos y los efectos del hiperestrogenismo mostraron diferente eficacia. Conclusiones. En esta serie, la aparición de PPP contribuyó al diagnóstico temprano del SMA y fue de difícil tratamiento. En la evolución persistió la hiperfunción gonadal y empeoraron las lesiones óseas.


Introduction. McCune-Albright syndrome (MAS) is a genetic disorder defined by fibrous dysplasia of bone, café-au-lait skin spots, and autonomous hyperfunction of one or more endocrine organs. MAS is caused by activating mutations of the GNAS1 gene. The most frequent type of endocrinopathy is gonadal endocrinopathy in the form of peripheral precocious puberty. Objective. To describe the clinical characteristics, laboratory and imaging tests at the time of diagnosis and over the course of the disease, focusing on the classical triad of MAS. Population and methods. Observational, descriptive, retrospective clinical study of patients with MAS seen at the Department of Endocrinology of Hospital de Niños Ricardo Gutiérrez between 1974 and 2019. Results. Twelve girls are described, all of whom developed peripheral precocious puberty (PPP) secondary to functional ovarian cysts. Their age at presentation was early (2.6 ± 1.3 years). Gonadotropin levels were suppressed or in the prepubertal range with generally high estradiol levels. Ten girls had café-au-lait skin spots since birth. During the course of disease, polyostotic fibrous dysplasia was detected in all patients. The treatments used to reduce ovarian cyst recurrence and hyperestrogenism effects showed varied effectiveness. Conclusions. In this series, the onset of PPP helped to make an early diagnosis of MAS and was difficult to treat. The course of disease showed persistent gonadal hyperfunction and worsening of bone injuries


Subject(s)
Humans , Female , Infant , Child, Preschool , Child , Adolescent , Puberty, Precocious/diagnosis , Puberty, Precocious/etiology , Puberty, Precocious/therapy , Fibrous Dysplasia, Polyostotic/complications , Fibrous Dysplasia, Polyostotic/diagnosis , Fibrous Dysplasia, Polyostotic/therapy , Retrospective Studies , Follow-Up Studies , Cafe-au-Lait Spots/diagnosis , Cafe-au-Lait Spots/therapy , Neoplasm Recurrence, Local
7.
Rev. méd. Urug ; 37(1): e701, mar. 2021. tab, graf
Article in Spanish | LILACS, BNUY | ID: biblio-1180965

ABSTRACT

Resumen: Introducción: la displasia fibrosa poliostótica es una patología de muy baja prevalencia, por lo que su diagnóstico pasa desapercibido en la mayoría de los casos. Cuando se asocia a endocrinopatías o a lesiones cutáneas hiperpigmentadas corresponde al síndrome de McCune-Albright. Caso clínico: escolar de 8 años, sexo femenino, que presentó una fractura patológica de fémur izquierdo traumática, en la cual se diagnosticó una displasia fibrosa poliostótica. Por presentarse acompañada de pubertad precoz periférica configura el denominado síndrome de McCune-Albright. El control y tratamiento fue multidisciplinario. El equipo de traumatología realizó osteosíntesis con placa y tornillos de la lesión ósea con evolución a la consolidación en plazos habituales (tres meses). A los seis meses de seguimiento la niña se encuentra sin dolor y sin repercusiones funcionales para la vida diaria. Del punto de vista endocrinológico se realizó tratamiento de su pubertad precoz con inhibidores de la aromatasa con el fin de mejorar su talla final y evitar repercusiones psicológicas y emocionales. En este estudio se analizan características de esta patología y su pronóstico vital y funcional.


Summary: Introduction: polyostotic fibrous dysplasia is a low prevalence disease, and for this reason, it often goes undetected. When associated to endocrinopathies and/or hyperpigmented skin lesions we speak about McCune Albright syndrome. Clinical case: eight-year old school girl who presented pathological fracture of the left femur, which was diagnosed as polyostotic fibrous dysplasia. As it was accompanied by peripheral precocious puberty it constituted an indicative clinical picture of the so-called McCune Albright. Control and treatment were multidisciplinary. The traumatology team performed osteosynthesis with plaques and nails to fix the bone lesion, and evolution consolidated in a regular time frame (3 months). Upon six months follow-up, the girl has no pain and presents no functional repercussion in daily life. From the endocrinological perspective, the girl received precocious puberty treatment with aromatase inhibitors with the purpose of improving her final size and avoid psychological and emotional implications. The study presents the characteristics of this condition, as well as its vital and functional prognosis.


Resumo: Introdução: a displasia fibrosa poliostótica é uma doença de prevalência muito baixa, por isso seu diagnóstico passa despercebido na maioria dos casos. Quando associada a endocrinopatias e / ou lesões cutâneas hiperpigmentadas, corresponde à síndrome de McCune Albright. Caso clínico: estudante do sexo feminino, 8 anos, com quadro de fratura patológica traumática do fêmur esquerdo, com diagnóstico de displasia fibrosa poliostótica. Por estar acompanhada de puberdade precoce periférica, configura a chamada síndrome de McCune Albright. O controle e o tratamento foram multidisciplinares. A equipe de trauma realizou osteossíntese de placa e parafuso da lesão óssea com progressão à consolidação nos prazos usuais (3 meses). Aos 6 meses de seguimento, a paciente não apresenta dor e tampouco repercussões funcionais no dia a dia. Do ponto de vista endocrinológico, sua puberdade precoce foi tratada com inibidores da aromatase para melhorar sua altura final e evitar repercussões psicológicas e emocionais. Este estudo analisa as características desta patologia, seu prognóstico vital e funcional.


Subject(s)
Puberty, Precocious , Fibrous Dysplasia, Polyostotic
8.
Evid. actual. práct. ambul ; 24(3): e002160, 2021. ilus
Article in Spanish | LILACS, BINACIS, UNISALUD | ID: biblio-1348604

ABSTRACT

En este artículo, los autores revisan el concepto de pubertad precoz y los factores que pueden influenciar su desarrollo, en especial en las niñas y en el contexto de las medidas de aislamiento social preventivo y obligatorio durante periodos prolongados a causa de la actual pandemia por el SARS-CoV-2. (AU)


In this article, the authors review the concept of precocious puberty and the factors that may influence its development, especially in girls and in the context of preventive and mandatory social isolation measures for prolonged periods of time due to the current SARS-CoV-2 pandemic. (AU)


Subject(s)
Humans , Female , Child , Puberty, Precocious/psychology , Social Isolation/psychology , COVID-19/complications , Pandemics , SARS-CoV-2
9.
Article in English | WPRIM | ID: wpr-922405

ABSTRACT

OBJECTIVES@#To systematically evaluate the effect of gonadotropin-releasing hormone analogue (GnRHa) treatment on the final adult height of children over 6 years of age with central precocious puberty (CPP) or early and fast puberty (EFP).@*METHODS@#PubMed, MEDLINE, Embase, Cochrane Library, CNKI, and Wanfang Data were searched for related articles on GnRHa treatment for children with CPP or EFP. Stata 12.0 software was used to perform a Meta analysis of related data.@*RESULTS@#A total of 10 studies were included, and the total sample size was 720 children, with 475 children in the GnRHa treatment group and 245 children in the control group. The Meta analysis showed that compared with the control group, the GnRHa treatment group had significantly better final adult height (@*CONCLUSIONS@#GnRHa treatment is safe and effective in improving the final adult height of children over 6 years of age with CPP or EFP.


Subject(s)
Adult , Body Height , Child , Gonadotropin-Releasing Hormone , Humans , Puberty , Puberty, Precocious/drug therapy
10.
Rev. cuba. endocrinol ; 31(3): e177, sept.-dic. 2020. graf
Article in Spanish | LILACS, CUMED | ID: biblio-1156396

ABSTRACT

Introducción: El síndrome de Mc Cune-Albright (SMA) es una rara entidad asociada con la displasia fibrosa poliostótica, con la presencia de manchas de color café con leche y también con la hiperfunción endocrina. La alteración hormonal más frecuente es la pubertad precoz. El SMA se debe a mutaciones activadoras del gen GNAS1. Objetivo: Describir las características clínicas de una paciente con síndrome de Mc Cune-Albright con una pubertad precoz. Métodos: Se realizó una revisión de la historia clínica como fuente primaria y fueron incorporados todos los elementos clínicos, bioquímicos, imagenológicos y genéticos que conformaron la valoración integral de la paciente. Presentación de caso: Se presenta un caso poco frecuente de síndrome de Mc Cune-Albright en una niña de siete años de edad con mamas Tanner II-III, sangrado vaginal, vello axilar y pubiano escaso, manchas café con leche y lesiones óseas. Lleva tratamiento con tamoxifeno, lo que ha logrado mantener frenada la progresión del desarrollo puberal. Conclusiones: Aunque esta entidad es de carácter benigno y la prevalencia es extremadamente baja, el inicio puberal precoz y el compromiso de la talla final pueden producir impacto psicológico en la calidad de vida y en el desarrollo normal del individuo(AU)


Introduction: Mc Cune-Albright syndrome (SMA, by its acronym in Spanish) is a rare entity associated with polyostotic fibrous dysplasia, with the presence of brown spots with milk and also with endocrine hyperfunction. The most common hormonal alteration is precocious puberty. SMA is caused by GNAS1 gene´s activator mutations. Objective: Describe the clinical characteristics of a patient with Mc Cune-Albright syndrome with precocious puberty. Methods: A review of the medical history was carried out as a primary source and all the clinical, biochemical, imaging and genetic elements that made up the comprehensive assessment of the patient were incorporated. Case presentation: A rare case of Mc Cune-Albright syndrome occurs in a seven-year-old girl with Tanner II-III breasts, vaginal bleeding, axillary and pubic hair, brown spots with milk and bone lesions. She is treated with tamoxifen, which has managed to keep the progression of pubertal development slow. Conclusions: Although this entity is benign in nature and the prevalence is extremely low, early pubertal onset and the compromise of the final size can have a psychological impact on the quality of life and normal development of the individual(AU)


Subject(s)
Humans , Female , Child , Puberty, Precocious/diagnostic imaging , Quality of Life , Tamoxifen/therapeutic use , Fibrous Dysplasia, Polyostotic/diagnostic imaging , Medical Records
11.
Arch. endocrinol. metab. (Online) ; 64(5): 591-596, Sept.-Oct. 2020. tab
Article in English | LILACS | ID: biblio-1131134

ABSTRACT

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.


Subject(s)
Humans , Female , Child , Puberty, Precocious/diagnostic imaging , Luteinizing Hormone , Central Nervous System , Retrospective Studies , Gonadotropin-Releasing Hormone , Follicle Stimulating Hormone
12.
Arch. endocrinol. metab. (Online) ; 64(2): 121-127, Mar.-Apr. 2020. tab, graf
Article in English | LILACS | ID: biblio-1131067

ABSTRACT

ABSTRACT Objective To determine whether first-voided urinary LH (FV-ULH) - level measurement can adequately assess pubertal suppression as much as standard tests can. Subjects and methods The study group included patients with central precocious puberty and rapidly progressing early puberty who received up to 3 - 4 doses of GnRHa therapy monthly and did not have adequate hormonal suppression after GnRH stimulation (90-minute LH level > 4 IU/L). Design: All of the participants underwent an LHRH test just after admission to the study. According to the stimulated peak LH levels, the patients were divided into 2 groups and followed until the end of the first year of treatment. The concordance between FV-ULH and stimulated LH levels was assessed. Results The FV-ULH levels in patients with inadequate hormonal suppression were significantly high compared to patients with adequate hormonal suppression. FV-ULH levels were very strongly correlated with stimulated LH levels (r = 0.91). Its correlation with basal LH levels was significant (r = 0.65). However, this positive correlation was modestly weakened after the first year of treatment. The cutoff value for FV-ULH of 1.01 mIU/mL had the highest sensitivity (92.3%) and specificity (100%). Conclusion FV-ULH levels, using more reliable and sensitive assay methods, can be used to monitor the adequacy of GnRHa therapy.


Subject(s)
Humans , Male , Female , Child , Puberty, Precocious/diagnosis , Luteinizing Hormone/urine , Gonadotropin-Releasing Hormone/administration & dosage , Leuprolide/administration & dosage , Triptorelin Pamoate/administration & dosage , Puberty, Precocious/urine , Puberty, Precocious/drug therapy , Prospective Studies , ROC Curve , Sensitivity and Specificity , Treatment Outcome
14.
Article in Chinese | WPRIM | ID: wpr-828670

ABSTRACT

OBJECTIVE@#To study the association of body fat ratio with precocious puberty in girls. Previous studies have shown that body mass index (BMI) is associated with the girls' age of puberty but have not revealed the association of body fat ratio with age of puberty.@*METHODS@#Based on the consensus on the diagnosis and treatment of central precocious puberty (CPP), 128 children with precocious puberty who were admitted to the hospital from July to August, 2017, were divided into a CPP group with 87 children and a peripheral precocious puberty (PPP) group with 41 children. A total of 51 girls without any puberty development signs were enrolled as the control group. Dual-energy X-ray absorptiometry was used to measure the body fat ratios of upper limbs, legs, trunk, android area, gynoid area, and the whole body. The association between body fat ratios and precocious puberty was analyzed with reference to age, BMI, BMI-Z score, bone age, ovarian volume, and hormone levels.@*RESULTS@#Compared with the control group, the CPP and PPP groups had significantly higher body fat ratios of upper limbs, legs, trunk, android area, gynoid area, and the whole body, legs/whole body fat ratio, and (upper limbs+legs)/trunk fat ratio (P0.05). For the girls with precocious puberty, the high body fat ratio group had significantly higher luteinizing hormone (LH) base value, luteinizing hormone releasing hormone (LHRH)-stimulated LH peak value, and LH/follicle-stimulating hormone peak value than the low body fat ratio group (P<0.05). Compared with the control group, both the high body fat ratio and low body fat ratio groups had a significantly higher LH base value (P<0.05).@*CONCLUSIONS@#The increase in body fat may be a factor inducing precocious puberty in girls, but further studies are needed to determine the mechanism.


Subject(s)
Adipose Tissue , Child , Female , Follicle Stimulating Hormone , Gonadotropin-Releasing Hormone , Humans , Luteinizing Hormone , Puberty, Precocious , Sexual Maturation
15.
Article in Chinese | WPRIM | ID: wpr-828498

ABSTRACT

Aromatase is the rate-limiting enzyme in estrogen biosynthesis. The third generation aromatase inhibitors (AIs), represented by letrozoleand and anastrozole, can combine with aromatase, effectively reducing the estrogen level in the body. Because of its high efficiency, selectivity and reversibility, it has been used in the treatment of McCune-Albright syndrome, familial male-limited precocious puberty, gynecomastia, and adolescent boy with short stature. The good efficacy and safety of AIs have been observed. However, so far the drug instructions of AIs usually do not show indications for children; there are risks of adverse reactions involving liver and kidney function, lipid metabolism, hyperandrogenemia and bone metabolism; especially the long-term effects on reproductive system and bone metabolism are still not clear. Therefore, it is necessary to prescribe it carefully and follow up closely. It was not recommended that AIs be routinely used to improve adult height of adolescent boy with short stature. And more clinical evidences are needed for the safety and effectiveness of AIs prescribed in pediatrics.


Subject(s)
Adolescent , Aromatase Inhibitors , Therapeutic Uses , Child , Estrogens , Humans , Male , Puberty, Precocious
16.
Article in Chinese | WPRIM | ID: wpr-828494

ABSTRACT

OBJECTIVE@#To evaluate the efficacy and safety of the third-generation aromatase inhibitor letrozole in the treatment of McCune-Albright syndrome (MAS) girls with peripheral precocious puberty.@*METHODS@#Twenty-one MAS girls with peripheral precocious puberty treated in Pediatrics Department of Ruijin Hospital, Shanghai Jiao Tong University School of Medicine from March 2012 to June 2017 were enrolled in the study. Patients presented with repeated vaginal bleeding, premature breast enlargement, café-au-lait spots or dysplasia of bone fibers, and low levels of luteinizing hormone (LH) and follicle-stimulating hormone (FSH); and the congenital adrenal hyperplasia, estrogen-producing tumors, and exogenous estrogen intake were excluded. Letrozole were administrated at a dose of 0.5-2 mg·m ·d for 6 to 12 months. The patients were observed for changes in breast staging, vaginal bleeding, sex hormone levels, liver function and bone age changes, and changes in uterine and ovarian volume.@*RESULTS@#After treatment, bone age/chronological age (BA/CA)was decreased from 1.23±0.30 to 1.11±0.18 ( < 0.01); the predicted adult height (PAH) increased from (156.2±5.9)cm to (158.4±2.1)cm after treatment ( < 0.05); the vaginal bleeding was reduced and the estradiol level decreased, while the teststosterone level and the uterus showed no significant increase, and no adverse reactions such as ovarian torsion and abnormal liver function were observed.@*CONCLUSIONS@#Precocious puberty is one of the most common endocrine manifestations in MAS. Our findings suggest that letrozole may be an effective and safe therapy to precocious puberty in girls with McCune-Albright Syndrome.


Subject(s)
Aromatase Inhibitors , Child , China , Female , Fibrous Dysplasia, Polyostotic , Humans , Letrozole , Puberty, Precocious
17.
Article in Chinese | WPRIM | ID: wpr-828491

ABSTRACT

OBJECTIVE@#To assess the efficacy of letrozole in treatment of children with congenital adrenal hyperplasia (CAH) due to steroid 21-hydroxylase deficiency (21-OHD).@*METHODS@#Twenty eight children, including 19 boys and 9 girls aged 4-10y, with CAH due to 21-OHD were enrolled in the study. At the first six months of study, all children received conventional treatment with hydrocortisone or fludrocortisone, then letrozole was added to original regimen. The height velocity (HV), difference between bone age and chronological age (BA-CA), height standard diviation score based on bone age (HtSDS ), predicted adult height (PAH), Tanner phase, sex hormone, and possible adverse reaction were evaluated and compared between those before and after letrozole treatment.@*RESULTS@#After 6 months of letrozole treatment, there was significant deceleration of HV, but it would recover soon. There was significant increase of HtSDS after 12 months of letrozole treatment ( < 0.05 or < 0.01), and significant changes in BA-CA after 18 months of letrozole treatment ( < 0.05). PAH of female children was significantly increased during letrozole treatment ( < 0.05), whereas PAH of male children was significantly increased 18 months after letrozole treatment ( < 0.05). Follicle-stimulating hormone (FSH) and luteinizing hormone (LH) levels were significantly increased, but did not meet the diagnostic criteria of central precocious puberty. Estradiol was significantly decreased ( < 0.01), but no changes in testosterone level was observed. During 24 months letrozole treatment, no hirsutism, severe acne, headache, bone pain, obesity, hypertension, rash and other adverse reactions were observed.@*CONCLUSIONS@#Letrozole can delay bone maturation and improve PAH, which can be used with conventional treatment for children with CAH due to 21-OHD, especially for those with high BA and low PAH.


Subject(s)
Adrenal Hyperplasia, Congenital , Drug Therapy , Body Height , Child , Child, Preschool , Female , Humans , Letrozole , Therapeutic Uses , Male , Puberty, Precocious
18.
Rev. chil. pediatr ; 90(6): 598-605, dic. 2019. tab
Article in Spanish | LILACS | ID: biblio-1058190

ABSTRACT

INTRODUCCIÓN: La radioterapia, quimioterapia y la cirugía empleada en el tratamiento de los tumores cerebrales tienen efectos en el eje hipotálamo-hipofisario y pueden resultar en disfunción endocrina hasta en el 96% de los casos. PACIENTES Y MÉTODO: Estudio retrospectivo y descriptivo en pacientes diagnos ticados de meduloblastoma sometidos a tratamiento con quimio y radioterapia en los últimos 20 años en un hospital terciario. Se analizan variables edad, sexo, peso, talla, índice de masa corporal (IMC) al final del seguimiento, estadio de maduración sexual, niveles séricos de TSH y T4 libre, ACTH/cortisol e IGF-1, FSH, LH, estradiol, testosterona, perfil lipídico (colesterol total) y prueba de función dinámica de hormona de crecimiento. RESULTADOS: Muestra total de 23 pacientes. El déficit de hormona de crecimiento es la secuela más frecuente (82 %) seguido de disfunción ti roidea (44,8%) y disfunción puberal (24,1%). Solo se diagnosticó un caso de diabetes insípida y 2 casos de déficit de corticotrofina. CONCLUSIONES: El seguimiento a largo plazo de los supervivientes de meduloblastoma tratados con quimio y radioterapia revela una prevalencia muy alta de disfun ción endocrina, particularmente de deficiencia de hormona del crecimiento y de hipotiroidismo. Creemos oportuna la monitorización y el seguimiento a largo plazo de estos pacientes con el fin de garantizar un manejo terapéutico adecuado de aquellas disfunciones tratables.


INTRODUCTION: Radiation therapy, chemotherapy, and surgery used to treat brain tumors have effects on the hy pothalamic-pituitary-adrenal axis and can result in endocrine dysfunction in up to 96% of cases. PATIENTS Y METHOD: Retrospective and descriptive study in patients diagnosed with medulloblasto ma who underwent treatment with chemo and radiotherapy in the last 20 years in a tertiary hospital. The variables analyzed were age, sex, weight, height, body mass index (BMI) at the end of follow-up, sexual maturity stage, serum levels of TSH and free T4, ACTH/cortisol and IGF-1, FSH, LH, estradiol, testosterone, lipid profile (total cholesterol), and growth hormone dynamic function test. RESULTS: Total sample of 23 patients. Growth hormone deficiency is the most frequent sequelae (82%) fo llowed by thyroid dysfunction (44.8%), and disorders of puberty (24.1%). Only one case of diabetes insipidus and two cases of corticotropin deficiency were diagnosed. CONCLUSIONS: Long-term follow- up of medulloblastoma survivors treated with chemo and radiotherapy reveals a very high prevalence of endocrine dysfunction, especially growth hormone deficiency and hypothyroidism. We believe that monitoring and long-term follow-up of these patients is necessary in order to ensure adequate therapeutic management of those treatable dysfunctions.


Subject(s)
Humans , Male , Female , Child, Preschool , Child , Cerebellar Neoplasms/therapy , Chemoradiotherapy/adverse effects , Medulloblastoma/therapy , Puberty, Precocious/etiology , Thyroid Diseases/etiology , Cerebellar Neoplasms/blood , Retrospective Studies , Adrenocorticotropic Hormone/deficiency , Human Growth Hormone/deficiency , Diabetes Insipidus/etiology , Endocrine System Diseases/etiology , Overweight/etiology , Cancer Survivors , Hypogonadism/etiology , Medulloblastoma/blood
19.
Arch. argent. pediatr ; 117(6): 388-391, dic. 2019. tab
Article in English, Spanish | LILACS, BINACIS | ID: biblio-1046280

ABSTRACT

El hipotiroidismo por tiroiditis de Hashimoto es la causa más frecuente de disfunción tiroidea en niños.Nuestro objetivo fue analizar el impacto en la talla final según la talla y el estadio puberal al momento del diagnóstico en menores de 18 años con hipotiroidismo grave de origen autoinmune. De los 79 pacientes, el 78,5 % fueron mujeres. Los que presentaron bocio (el 56 %) mostraron mejor talla en el diagnóstico que los que no lo tenían (puntaje de desvío estándar de media de talla: 0,2 vs. -2,42; p < 0,0001). Cinco niñas (el 6,3 %) presentaron pubertad precoz. De los pacientes con talla final (n: 33), dentro de los que presentaron talla baja al momento del diagnóstico, los púberes tuvieron una talla final significativamente menor que los prepúberes (puntaje de desvío estándar media: -2,82 vs. -1,52; p = 0,0311).El diagnóstico tardío de hipotiroidismo grave en pediatría tiene un impacto negativo en la talla final, especialmente, en los pacientes puberales al momento del diagnóstico.


Hypothyroidism caused by Hashimoto's thyroiditis is the most common reason for thyroid dysfunction in children. Our objective was to analyze its impact on final stature in relation to height and pubertal stage at the time of diagnosis in children younger than 18 years with severe autoimmune hypothyroidism. Out of 79 patients, 78.5 % were girls. Those with goiter (56 %) had a better height at diagnosis than those without goiter (mean standard deviation score for height: 0.2 versus −2.42; p < 0.0001). Five girls (6.3 %) had precocious puberty. When considering the final stature of patients (n: 33), among those with short stature at the time of diagnosis, pubertal children had a significantly shorter final stature than prepubertal children (mean standard deviation score for height: −2.82 versus −1.52; p = 0.0311). The late diagnosis of severe hypothyroidism in pediatrics has a negative impact on final stature, especially in those who were pubertal patients at the time of diagnosis


Subject(s)
Humans , Male , Female , Child , Adolescent , Puberty, Precocious , Body Height , Hashimoto Disease , Hypothyroidism/diagnosis , Epidemiology, Descriptive , Retrospective Studies
20.
Rev. cuba. endocrinol ; 30(2): e197, mayo.-ago. 2019. graf
Article in Spanish | LILACS, CUMED | ID: biblio-1126436

ABSTRACT

RESUMEN El síndrome de Van Wyk-Grumbach se caracteriza por hipotiroidismo primario de larga duración asociado a pubertad precoz. Se presenta una paciente de 7 años, mestiza, que acude por sangrado vaginal, acompañado de hiperpigmentación de las areolas sin galactorrea, abdomen globuloso, mixedema, hirsutismo, baja talla, astenia y retraso escolar. La química sanguínea mostró anemia, hipercolesterolemia y hipertransaminasemia; los estudios de imágenes constataron derrame pericárdico, retraso marcado de la edad ósea, incremento de las dimensiones de la silla turca y gran quiste del ovario con aparente criterio quirúrgico. Los estudios hormonales confirmaron la sospecha de hipotiroidismo primario asociado a hiperprolactinemia. El tratamiento sustitutivo con levotiroxina sódica revirtió los signos y síntomas de pubertad precoz, lo que evitó la cirugía del quiste de ovario; la recuperación en el ambiente escolar y social fue indiscutible. Reconocer esta entidad evita tratamientos absolutamente contraindicados, como la extirpación de los quistes o el tratamiento quirúrgico de la hiperplasia hipofisaria(AU)


ABSTRACT Van Wyk-Grumbach syndrome is characterized by long-lasting primary hypothyroidism associated with precocious puberty. A case of a 7-year-old female mestizo patient is reported. She came to consultation for vaginal bleeding, accompanied by hyperpigmentation of the areolas without galactorrhea, globular abdomen, myxedema, hirsutism, short stature, asthenia and school delay. Blood chemistry showed anemia, hypercholesterolemia and hypertransaminasemia. Imaging studies showed pericardial effusion, marked delay in bone age, increased dimensions of Turkish chair and large ovarian cyst with apparent surgical criteria. Hormonal studies confirmed the suspicion of primary hypothyroidism associated with hyperprolactinemia. Substitute treatment with levothyroxine sodium reversed the signs and symptoms of precocious puberty, which prevented ovarian cyst surgery; the recovery in the school and social environment was certain. Recognizing this entity avoids absolutely contraindicated treatments, such as the removal of cysts or the surgical treatment of pituitary hyperplasia(AU)


Subject(s)
Humans , Female , Child , Puberty, Precocious , Thyroxine/therapeutic use , Hypercholesterolemia/etiology , Hypothyroidism/diagnosis , Anemia/etiology
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