Zur Genese des Hypogonadismus beim kongenitalen adrenogenitalen Syndrom

Kießling, Werner; Schwarz, Gerhard

doi:10.1007/bf00502919

Cited by 2 publications

(4 citation statements)

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“…5,68,69,[92][93][94] However, complications of small testes and aspermia have been reported in some patients with inadequately controlled disease. 5,68,80,95 In contrast to this observation, some investigators have reported normal testicular maturation and normal spermatogenesis and fertility in patients who had never received glucocorticoid treatment 5,78,93,96,97 or in those whose glucocorticoid therapy was discontinued for several years. 5,9 Thus, male patients with CAH and excessive adrenal androgens may have either normal gonadal function or hypogonadism.…”

Section: Male Reproductive Functionmentioning

confidence: 90%

“…In most untreated or poorly treated adolescent girls and in some adolescent boys, spontaneous true pubertal development does not occur until proper treatment is instituted (Table 4). 67,68,78–80 Studies suggest that excess adrenal androgens (aromatized to estrogens) inhibit the pubertal pattern of gonadotropin secretion by the hypothalamic‐pituitary axis 67. The inhibition probably occurs via a negative feedback effect; whether it is primarily at the hypothalamus or pituitary is not known.…”

Section: Pubertal Maturation In Classical Congenital Adrenal Hypermentioning

confidence: 99%

“…Several long‐term studies indicate that in a majority of successfully treated male patients with CAH, pubertal development, normal testicular function, and normal spermatogenesis and fertility occur 5,68,69,92–94. However, complications of small testes and aspermia have been reported in some patients with inadequately controlled disease 5,68,80,95. In contrast to this observation, some investigators have reported normal testicular maturation and normal spermatogenesis and fertility in patients who had never received glucocorticoid treatment5,78,93,96,97 or in those whose glucocorticoid therapy was discontinued for several years 5,9.…”

Section: Pubertal Maturation In Classical Congenital Adrenal Hypermentioning

confidence: 99%

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An Update of Congenital Adrenal Hyperplasia

New

2004

Annals of the New York Academy of Sciences

149

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Congenital adrenal hyperplasia (CAH) is a family of autosomal recessive disorders caused by mutations that encode for enzymes involved in one of the various steps of adrenal steroid synthesis. These defects result in the absence or the decreased synthesis of cortisol from its cholesterol precursor. The anterior pituitary secretes excess adrenocorticotrophic hormone (ACTH) via feedback regulation by cortisol, which results in overstimulation of the adrenals and causes hyperplasia. Symptoms due to CAH can vary from mild to severe depending on the degree of ensymatic defect. In the classical form of CAH, there is a severe enzymatic defect owing to mutations in the CYP21 gene. Classically affected female fetuses undergo virilization of the genitalia prenatally and present with genital ambiguity at birth; however, prenatal treatment of CAH with dexamethasone to prevent ambiguity has been successfully utilized for over a decade. In the less severe, late-onset form of CAH, prenatal virilization does not occur. The milder enzyme deficiency was termed nonclassical 21-hydroxylase deficiency (NC21OHD) in 1979 and was later found to be the most common autosomal recessive disorder in humans. Disease frequency of NC21OHD varies between ethnic groups with the highest ethnic-specific disease frequency in Ashkenazi Jews at 1/27. NC21OHD is diagnosed by serum elevations of 17-OHP that plot on a nomogram between the range for unaffected individuals and levels observed for classical CAH and is typically confirmed with molecular genetic analysis. Similar to classical CAH, nonclassical 21-hydroxylase deficiency may cause premature development of pubic hair, advanced bone age, accelerated linear growth velocity and diminished final height in both males and females. Severe cystic acne has also been attributed to nonclassical CAH. Women may present with symptoms of androgen excess, including hirsutism, temporal baldness, and infertility. Menarche in females may be normal or delayed and secondary amenorrhea is a frequent occurrence. Polycystic ovary syndrome may also be seen in these patients. In males, early beard growth, acne, and growth spurt may prompt the diagnosis of NC21OHD. Although many males appear to be asymptomatic, they may present with oligozoospermia or diminished fertility. Individuals presenting to dermatology and infertility clinics with symptoms of hyperandrogenemia are rarely screened for NC21OHD. However, with hormonal and molecular genetic screening, previously undiagnosed patients may be identified and can therefore receive glucocorticoid treatment, which has been shown to reverse symptoms within 3 months.

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Section: Male Reproductive Functionmentioning

confidence: 90%

Section: Pubertal Maturation In Classical Congenital Adrenal Hypermentioning

confidence: 99%

Section: Pubertal Maturation In Classical Congenital Adrenal Hypermentioning

confidence: 99%

See 1 more Smart Citation

An Update of Congenital Adrenal Hyperplasia

New

2004

Annals of the New York Academy of Sciences

149

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“…In most untreated or poorly treated adolescent girls and in some adolescent boys, spontaneous true pubertal development does not occur until proper treatment is instituted (Wilkins et al 1952;Kiessling and Schwarz 1966;Klingensmith et al 1976Klingensmith et al , 1977Pang et al 1977 a). Studies suggest that excess adrenal sex steroids inhibit the pubertal pattern of gonadotropin secretion by the hypothalamic-pituitary axis (Klingensmith et al 1976).…”

Section: A 21-hydroxylase Deficiencymentioning

confidence: 99%

Congenital Adrenal Hyperplasia

New

Levine

1984

Monographs on Endocrinology

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This work is subject to copyright. All rights are reserved, whether the whole or part of the material is concerned, specifically those of translation, reprinting, fe-use of illustrations, broadcasting, reproduction by photocopying machine or similar means, and storage in data banks. Under §54 of the German Copyright Law where copies are made for other than private use. a fee is payable to 'Verwertungsgesellschaft Wort'. Munich. 1[. Springer-Verlag Berlin. Heidelberg 1984 Softcover reprint of the hardcover 1 st edition 1984The use of registered names, trademarks, etc. in this publication does not imply. even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use.Product Liability: The publisher can give no guarantee for information about drug dosage and application thereof contained in this book. In every individual case the respective user must check its accuracy by consulting other pharmaceutical literature.

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Zur Genese des Hypogonadismus beim kongenitalen adrenogenitalen Syndrom

Cited by 2 publications

References 3 publications

An Update of Congenital Adrenal Hyperplasia

An Update of Congenital Adrenal Hyperplasia

Congenital Adrenal Hyperplasia

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