ABSTRACT
El autor de este artículo repasa las características clínicas de la pubertad precoz y la pubertad temprana, las pruebas diagnósticas indicadas en la evaluación de los pacientes que la presentan y las recomendaciones actuales de tratamiento. (AU)
The author of this article reviews the clinical features of early puberty, the diagnostic tests for the patients Ì evaluation andthe current treatment recommendations. (AU)
Subject(s)
Humans , Male , Female , Child , Puberty, Precocious/therapy , Gonadotropin-Releasing Hormone/agonists , Puberty, Precocious/classification , Puberty, Precocious/pathology , Puberty, Precocious/blood , Puberty, Precocious/diagnostic imaging , Gonadotropin-Releasing Hormone/therapeutic use , Sex CharacteristicsABSTRACT
In the last two decades, the diagnosis and treatment of precocious puberty has undergone important changes. The use of supersensitive assays to determine gonadotropins and gonadal hormones has increased the sensitivity and decreased the number of blood samples required to assess the diagnosis. The introduction of gonadotropin-releasing hormone (GnRH) agonists produced a revolution in the diagnosis and treatment of this disorder. Recently, the use of long acting GnRH agonists improved the adherence of patients to medical treatment and decreased the need for uncomfortable repeated doses. The medications in the treatment of the GnRH independent causes of precocious puberty, and the important revelations in the pathophysiology of these disorders, have advanced our knowledge and management of the affected children.
Subject(s)
Adolescent , Child , Female , Gonadotropin-Releasing Hormone/diagnosis , Humans , Male , Puberty, Precocious/classificationABSTRACT
Se revisa la fisiología de la maduración sexual, desde la vida intrauterina hasta la pubertad, con énfasis en los eventos que ocurren durante la pubertad tanto en la mujer como en el varón. Luego se describen las alteraciones que ocurren en el desarrollo de la pubertad: pubertad precoz y pubertad tardía , sus causas, metodología diagnóstica y posibilidades tarapéuticas. Y, en la tercera parte del artículo se comentan las variaciones normales de la pubertad: adrenarquia prematura, telarquia prematura, menarquia prematura y ginecomastia puberal.
Subject(s)
Humans , Male , Female , Puberty/physiology , Puberty, Precocious/classification , Puberty, Precocious/diagnosis , Puberty, Precocious/therapy , Gynecomastia/diagnosis , Gynecomastia/physiopathology , Gynecomastia/therapy , Sexual Maturation/physiology , Puberty, Delayed/classification , Puberty, Delayed/diagnosis , Puberty, Delayed/therapyABSTRACT
A puberdade precoce (PP) na menina e definida com o surgimento de caracteristicas sexuais secundarias antes dos 8 anos de idade. As causas podem ser dependentes de gonadotrofinas (PP verdadeira), independentes de gonadotrofinas (Pseudo PP) e as variantes da normalidade. O cisto folicular autonomo pode determinar sinais de PP pela producao local de estradiol. O caso relatado revela uma menina de 4 anos que apresentou sangramento vaginal, broto mamario e leucorreia fisiologica associados a presenca de um cisto folicular autonomo. O diagnostico fundamentou-se no exame fisico, grau do conteudo vaginal, determinacao radiologica da idade ossea, dosagens hormonais, ultra-sonografias pelvica e mamaria. O tratamento destes cistos pode ser conservador ou cirurgico. Neste caso, optou-se pelo uso de acetato de medroxiprogesterona que mostrou-se satisfatorio. Alem disso, foi feita uma revisao dos achados ultra-sonograficos relevantes para o diagnostico da PP
Subject(s)
Humans , Female , Follicular Cyst , Follicular Cyst/diagnosis , Follicular Cyst/therapy , Puberty, Precocious/classification , Puberty, Precocious/complications , Puberty, Precocious/diagnosis , Puberty, Precocious/etiology , Puberty, Precocious/therapySubject(s)
Humans , Male , Female , Child , Androgens/physiology , Estrogens/physiology , Gonadotropins, Pituitary/physiology , Puberty, Precocious/physiopathology , Adrenal Hyperplasia, Congenital/physiopathology , Sex Characteristics , Puberty, Precocious/classification , Puberty, Precocious/diagnosis , Sexual MaturationABSTRACT
Eighty children (58 girls and 22 boys) with isosexual precocity seen in the past eight years were evaluated clinically and investigated to identify the underlying cause. Of these, 50% (29 girls and 11 boys) had centrally mediated true precocious puberty (TPP). The girls could be classified into five major groups (I) Central precocious puberty 29-subclassified into idiopathic (ITPP, 15) and organic or neurogenic (NTTP, 14), (II) Premature thelarche (PT, 20), (III) Premature menarche (PM, 2), (IV) Premature adrenarche (PA, 5), and, (V) Others: hypothyroid (n = 1), and McCune Albright Syndrome (n = 1). ITPP as a cause of precocity in girls was seen less often (52%) and NTPP more often (48%) compared to most Western series, with tubercular meningitis as the cause in 31% and hypothalamic hamartomas in 10%. Though the LH and estradiol levels were significantly higher (p < 0.05) in TPP, compared to PT, these were not helpful in differentiating because of considerable overlap. LH-predominant-response (LH/FSH ratio > 1) to LHRH testing was seen in TPP. Amongst the 22 boys, 11 (50%) had TPP, ITPP in 27% and NTPP in 73%. Hamartomas (n = 4) and TBM (n = 3) contributed equally to NTPP; pineal tumor was seen in one. The adrenal (n = 7) and testicular (n = 2) causes together involved 41% of the boys with precocity, congenital adrenal hyperplasia (CAH) CAH, 11-beta hydroxylase being the commonest cause. Of the 6 boys witdeficiency was found in four and nonsalt losing form of 21-hydroxylase deficiency in 2. Testicular and adrenal tumors and testotoxicosis were noted in one case each. The etiologic factors were more varied in boys.
Subject(s)
Adrenal Gland Diseases/complications , Age Determination by Skeleton , Breast/growth & development , Central Nervous System Diseases/complications , Child , Child, Preschool , Female , Humans , Infant , Male , Menarche/physiology , Penis/growth & development , Puberty, Precocious/classification , Retrospective Studies , Testicular Diseases/complicationsABSTRACT
La hiperplasia hipofisiaria originada por falla primaria de un órgano endocrino no es rara. Se han informado casos de hipotiroidismo primario en niños y adultos como etiología de hipertrofia de la hipófisis (1-8). En ocasiones se han observado casos de adenomas y microadenomas hipofisiarios causados por esta alteración, tanto en humanos como en animales de experimentación (2, 6, 9, 10); otras entidades en las cuales se ha documentado aumento del tamaño de la hipófisis incluyen falla gonadal primaria y en pacientes con adrenalectomía total (1, 5). Los primeros casos informados se remontan a 1851 (5); en 1960 Van Wyck y Grumbach describieron un síndrome en niños caracterizado por pubertad precoz, galactorrea, hipotiroidismo primario y aumento del tamaño de la silla turca (!!); a partir de este momento los informes en la literatura demuestran la asociación entre estos dos hallazgos, que pueden llevar a notables confusiones diagnósticas y terapéuticas, debido a las alteraciones endocrinológicas y neurológicas asociadas; un diagnóstico adecuado permitirá un tratamiento oportuno de acuerdo a la patología de base. Las principales alteraciones neurológicas asociadas se producen por compresión de la vía visual por la hiperplasia de la hipófisis, ocasionando desde cambios leves en los campos visuales, papiledema, hasta ceguera (2, 6). Nosotros describimos el caso de una niña con pubertad precoz, talla baja y aumento del tamaño de la hipófisis, demostrado por estudios neurorradiológicos, en quien disminuyó el tamaño hipofisiario luego del tratamiento de sustitución hormonal.