Effects of an Early Postnatal Treatment of Hypogonadotropic Hypogonadism with a Continuous Subcutaneous Infusion of Recombinant Follicle-Stimulating Hormone and Luteinizing Hormone

Bougnères, Pierre; François, Marie; Pantalone, Letitia; Rodrigue, Danielle; Bouvattier, Claire; Demesteere, E.; D, Roger; Lahlou, Najiba

doi:10.1210/jc.2008-0121

Cited by 112 publications

(102 citation statements)

References 33 publications

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“…AMH and inhibin B are expected to be normal in infants with abdominal wall or inguinal region defects or with impaired INSL3 signalling. AMH and inhibin B levels are low in patients with central hypogonadism, as discussed below (Young et al, 1999(Young et al, , 2005Andersson, 2000;Bougnères et al, 2008).…”

Section: Foetal-onset Hypogonadism Foetal-onset Primary Hypogonadismmentioning

confidence: 98%

“…Multiple pituitary hormone deficiency: In male infants with congenital multiple pituitary hormone deficiency (Table 1), the presence of micropenis, cryptorchidism and/or microorchidism is usually associated to low levels of gonadotropins, gonadal hormones and hormones of the GH-IGF axis (Jensen et al, 2005;Bougnères et al, 2008). Early treatment with FSH results in testicular growth -owing to Sertoli cell proliferation -and serum AMH and inhibin B elevation (Bougnères et al, 2008).…”

Section: Foetal-onset Central Hypogonadismmentioning

confidence: 99%

“…Early treatment with FSH results in testicular growth -owing to Sertoli cell proliferation -and serum AMH and inhibin B elevation (Bougnères et al, 2008).…”

Section: Foetal-onset Central Hypogonadismmentioning

confidence: 99%

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Male hypogonadism: an extended classification based on a developmental, endocrine physiology‐based approach

et al. 2012

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SUMMARYNormal testicular physiology results from the integrated function of the tubular and interstitial compartments. Serum markers of interstitial tissue function are testosterone and insulin-like factor 3 (INSL3), whereas tubular function can be assessed by sperm count, morphology and motility, and serum anti-Mü llerian hormone (AMH) and inhibin B. The classical definition of male hypogonadism refers to testicular failure associated with androgen deficiency, without considering potential deficiencies in germ and Sertoli cells. Furthermore, the classical definition does not consider the fact that low basal serum testosterone cannot be equated to hypogonadism in childhood, because Leydig cells are normally quiescent. A broader clinical definition of hypogonadism that could be applied to male patients in different periods of life requires a comprehensive consideration of the physiology of the hypothalamic-pituitary-testicular axis and its disturbances along development. Here we propose an extended classification of male hypogonadism based on the pathophysiology of the hypothalamic-pituitary-testicular axis in different periods of life. The clinical and biochemical features of male hypogonadism vary according to the following: (i) the level of the hypothalamic-pituitary-testicular axis primarily affected: central, primary or combined; (ii) the testicular cell population initially impaired: whole testis dysfunction or dissociated testicular dysfunction, and: (iii) the period of life when the gonadal function begins to fail: foetal-onset or postnatal-onset. The evaluation of basal testicular function in infancy and childhood relies mainly on the assessment of Sertoli cell markers (AMH and inhibin B). Hypergonadotropism should not be considered a sine qua non condition for the diagnosis of primary hypogonadism in childhood. Finally, the lack of elevation of gonadotropins in adolescents or adults with primary gonadal failure is indicative of a combined hypogonadism involving the gonads and the hypothalamic-pituitary axis.

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Section: Foetal-onset Hypogonadism Foetal-onset Primary Hypogonadismmentioning

confidence: 98%

Section: Foetal-onset Central Hypogonadismmentioning

confidence: 99%

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Male hypogonadism: an extended classification based on a developmental, endocrine physiology‐based approach

et al. 2012

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“…The onset of AMH expression in fetal life and the maintenance of its basal levels are gonadotropin-independent (13). However, serum AMH is low in patients with congenital hypogonadotropic hypogonadism, and increases in response to exogenous FSH administration (14)(15)(16), indicating that serum AMH is a useful marker of FSH action on the prepubertal testis. Low serum AMH correlates with small testes (6).…”

Section: Amh and Sertoli Cell Physiologymentioning

confidence: 99%

New perspectives in the diagnosis of pediatric male hypogonadism: the importance of AMH as a Sertoli cell marker

Grinspon

Rey

2011

Arq Bras Endocrinol Metab

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SUMMARYSertoli cells are the most active cell population in the testis during infancy and childhood. In these periods of life, hypogonadism can only be evidenced without stimulation tests, if Sertoli cell function is assessed. AMH is a useful marker of prepubertal Sertoli cell activity and number. Serum AMH is high from fetal life until mid-puberty. Testicular AMH production increases in response to FSH and is potently inhibited by androgens. Serum AMH is undetectable in anorchidic patients. In primary or central hypogonadism affecting the whole gonad and established in fetal life or childhood, serum AMH is low. Conversely, when hypogonadism affects only Leydig cells (e.g. LHb mutations, LH/CG receptor or steroidogenic enzyme defects), serum AMH is normal or high. In pubertal males with central hypogonadism, AMH is low for Tanner stage (reflecting lack of FSH stimulus), but high for the age (indicating lack of testosterone inhibitory effect). Treatment with FSH provokes an increase in serum AMH, whereas hCG administration increases testosterone levels, which downregulate AMH. In conclusion, assessment of serum AMH is helpful to evaluate gonadal function, without the need for stimulation tests, and guides etiological diagnosis of pediatric male hypogonadism. Furthermore, serum AMH is an excellent marker of FSH and androgen action on the testis. Arq Bras Endocrinol Metab. 2011;55(8):512-9 Keywords Hypogonadotrophic hypogonadism; hypergonadotrophic hypogonadism; testis; anorchia; cryptorchidism; disorders of sex development SUMáRioAs células de Sertoli são a população de células mais ativa nos testículos durante a primeira e segunda infância. Neste período, o hipogonadismo só pode ser evidenciado sem o uso de testes estimulatórios se a função das células de Sertoli for avaliada. O AMH é um marcador útil do nú-mero e da atividade das células de Sertoli no período pré-puberal. A concentração sérica de AMH é alta da metade da vida fetal até a metade da puberdade. A produção de AMH pelos testículos aumenta em resposta ao FSH e é potencialmente inibida por androgênios. O AMH sérico não é detectável em pacientes anorquídicos. No hipogonadismo central ou primário afetando a gônada inteira, ou estabelecido na vida fetal ou infância, a concentração de AMH sérica é baixa. Por outro lado, quando o hipogonadismo afeta apenas as células de Leydig (por exemplo, nas mutações, LHb, defeitos do receptor de LH/CG ou das enzimas esteroidogênicas), a concentração de AMH sé-rico é normal ou alta. Em meninos púberes com hipogonadismo central, a concentração de AMH é baixa para o estágio na escala de Tanner (refletindo a falta de estímulo pelo FSH), mas alta para a idade (indicando a falta do efeito inibidor da testosterona). O tratamento com FSH provoca um aumento do AMH sérico, enquanto a administração de hCG aumenta os níveis de testosterona, que fazem a downregulation do AMH. Em conclusão, a concentração sérica de AMH é útil na avaliação da função gonadal, excluindo a necessidade de testes estimulatórios, e direciona o diagnóstico et...

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“…Sin embargo, se debe destacar que las gonadotrofinas pueden no estar elevadas, aún en casos de anorquia, entre los seis y los 11 años 31 (Ver Figura 6). La detección de AMH e inhibina B bajas con gonadotrofinas no elevadas es altamente sugestivo de hipogonadismo secundario 32 . En el síndrome de Klinefelter, las hormonas del eje hipófiso-gonadal están normales en esta etapa de la vida 33,34 , si bien algunos autores han encontrado niveles ligeramente disminuidos de testosterona 35,36 .…”

Section: Evaluación Clínicaunclassified

Criptorquidia: desde la embriología al tratamiento

Blanco¹,

Gottlieb

Grinspon

et al. 2015

Rev. Medicas UIS

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RESUMENLa criptorquidia es la anomalía genital más común en el recién nacido varón y a pesar de que su evaluación y tratamiento han progresado con las décadas, siguen existiendo muchas controversias al respecto. En todo niño, el examen físico genital debe buscar la presencia de las gónadas en el escroto, en su ausencia, debe tratar de distinguirse si la anomalía es unilateral o bilateral, definiéndose con la mayor precisión posible la posición de estas y distinguiéndose entre testículo criptórquido, ectópico y retráctil junto con la valoración de la existencia de tejido testicular funcional a través de estudios hormonales. El tratamiento puede ser hormonal o quirúrgico, este último no se recomienda antes del año de edad además corresponde a la terapia más exitosa para reubicar el testículo en el escroto en aquellos pacientes con gónadas en posición inguinal alta, abdominal o en posición ectópica, o en aquellos en los que la terapia hormonal ha fallado. Por otro lado, la terapia hormonal se recomienda en mayor medida cuando las gónadas están en posición inguinal media, baja o escrotal alta. El tratamiento apunta a reducir, aunque no siempre logra evitar los posibles problemas a largo plazo de infertilidad y cáncer de testículo. MÉD UIS. 2015;28(3):371-80.Palabras clave: Fertilidad. Neoplasia Testicular. Orquidopexia. Cryptorchidism: from embryology to treatment ABSTRACTCryptorchidism is the most frequent genital anomaly in the newborn male. Although significant progress has been made in the assessment and treatment of cryptorchidism, several controversies still exist. In every boy, the physical examination should seek the presence of the gonads in the scrotum. Otherwise, it should be made clear whether cryptorchidism is unilateral or bilateral, the position of the gonad should be defined as precisely as possible, distinguishing between cryptorchidism, ectopia and retractile testes. Hormonal laboratory studies help assessing the existence of functional testicular tissue. Treatment may be hormonal or surgical. The latter is not recommended before the age of 1 year. Surgery has the highest rates of success in patients with gonads in high inguinal, abdominal or ectopic position, or when hormonal treatment has failed. Hormonal treatment may be successful in patients with gonads in low inguinal or high scrotal position. Treatment aims to reduce, although it does not always avoids, the risks of infertility and of testicular cancer in the long term. MÉD UIS. 2015;28(3):371-80.

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Effects of an Early Postnatal Treatment of Hypogonadotropic Hypogonadism with a Continuous Subcutaneous Infusion of Recombinant Follicle-Stimulating Hormone and Luteinizing Hormone

Abstract: The present regimen induced physiological postnatal testes growth and high-normal activation of Leydig and Sertoli cells.

Cited by 112 publications

References 33 publications

Male hypogonadism: an extended classification based on a developmental, endocrine physiology‐based approach

Male hypogonadism: an extended classification based on a developmental, endocrine physiology‐based approach

New perspectives in the diagnosis of pediatric male hypogonadism: the importance of AMH as a Sertoli cell marker

Criptorquidia: desde la embriología al tratamiento

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