Gonadal dysgenesisQ55.8

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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DefinitionThis section has been translated automatically.

Lack of functional germ cells. Chromosomal aberration (XO) with female phenotype, intersexual genotype and male nuclear sex (chromatin negative).

Clinical featuresThis section has been translated automatically.

A distinction is made: Klinefelter's syndrome.

TherapyThis section has been translated automatically.

In the absence of pubertal development hormone substitution, see Tables 1 and 2. After completion of this initial phase of rapid androgenisation, gonadal function is stimulated with the aid of GnRH or gonadotropins until mature germ cells are formed (see Table 3). This procedure applies both to patients with current and later desire for children, since the spermatogenesis initiated once can be restimulated more quickly by renewed GnRH or gonadotropin therapy. The therapy is carried out until sperm appears in the ejaculate or until pregnancy occurs. The fertility prognosis is favourable. Often, sperm formation can be successfully induced. GnRH or gonadotropin therapy of two and more years. Once spermiogenesis has fully matured or pregnancy has been achieved, testosterone therapy (required for life to maintain secondary sexual characteristics and androgen-dependent functions, including the prevention of osteoporosis) is switched back to, as it is considerably cheaper and is sufficient to compensate for the androgen deficit. If the desire to have children again, a new GnRH or gonadotropin therapy is carried out.

TablesThis section has been translated automatically.

Testosterone substitution in gonadal dysgenesis

Application

Example preparations

Dosage

Intramuscular

Testoviron Depot 250 mg

every 2-3 weeks

Testosterone Depot 250 mg

every 2-3 weeks

orally

Andriol

2-4 cps/day

transdermal

Testogel

25-25 mg/day

Androderm

2 Membranes/day


Criteria for monitoring testosterone replacement therapy

Psychological and sexual parameters

General well-being, mental and physical activity, mood, libido, erections, sexual activity

Somatic parameters

body proportions, body weight, muscle mass and strength, fat distribution, hair (beard, pubes, forehead hairline), sebum, voice change

Laboratory parameters

Testosterone (SHBG, free testosterone, testosterone in saliva), gonadotropins (DHT, estradiol), erythropoiesis (Hk, Erys, Hb), liver enzymes

Prostate/seed blisters

ejaculate volume, prostate size/inner echoes, PSA in serum, uroflow

Bones

Bone Density


Therapy options for stimulating spermatogenesis

Preparation

Trade name

Application form

Dosage

GnRH pulsatile

Lutrelef

s.c., external micro pump (Zyklomat Pulse)

520 μg/Pulse every 120 min.

Alternatively

HCG

(Human Chorionic Gonadotropin)

Choragon

i.m. or s.c.

10002500 IE 2 times/week

(Monday and Friday)

Predalon

in combination with

HMG (Menotropin; Human Menopausal Gonadotropin)

Menogon

i.m. or s.c.

150 IE 3 times/week

(Monday, Wednesday, Friday)

Authors

Last updated on: 29.10.2020