Male Hypogonadism In Children

Rarely is a decreased response to testosterone, which delayed to puberty, reduced fertility or does both. The diagnosis is made due to the measurements of the levels of testosterone, luteinizing hormone (LH) and follicle stimulating hormone (FSH), and by a stimulation test with human chorionic gonadotropin or gonadotropin-releasing hormone (GRH). Treatment depends on the cause.

Rarely is a decreased response to testosterone, which delayed to puberty, reduced fertility or does both. The diagnosis is made due to the measurements of the levels of testosterone, luteinizing hormone (LH) and follicle stimulating hormone (FSH), and by a stimulation test with human chorionic gonadotropin or gonadotropin-releasing hormone (GRH). Treatment depends on the cause.

(Male hypogonadism.) Rarely is there a decreased response to testosterone, which leads to a delayed puberty, reduced fertility, or both. The diagnosis is made due to the measurements of the levels of testosterone, luteinizing hormone (LH) and follicle stimulating hormone (FSH), and by a stimulation test with human chorionic gonadotropin or gonadotropin-releasing hormone (GRH). Treatment depends on the cause. Classification There are 3 forms of hypogonadism: the primary and secondary hypogonadism and which is caused by a defective androgen receptor a shape. affect primary hypogonadism when primary (hypergonadotropic) hypogonadism damaged Leydig cells and / or tubules semiferini testosterone production, resulting in oligospermia or azoospermia and elevated gonadotropin levels. The most common is Klinefelter’s syndrome, other causes include disorders of sexual development, such as gonadal dysgenesis (rare), cryptorchidism, bilateral anorchia, Leydig Zellaplasie, Noonan syndrome and myotonic dystrophy. Rare reasons may be Masernorchitis, testicular torsion, chemotherapy with alkylating drugs and trauma. Klinefelter’s syndrome is a dysgenesis of the tubules semiferini which is associated with a 47, XXY karyotype. The extra X chromosome is acquired through a maternal, less frequently by a paternal meiotic nondisjunction (Klinefelter syndrome (47, XXY)). The syndrome is observed in adolescence, when a lack of sexual development noticed or later if infertility is clarified. The diagnosis is based on elevated gonadotropin levels and low to very low levels of testosterone. Errors in sexing and the gonad, such as gonadal dysgenesis (46, XX or 46 XY) and testicular and ovotestikuläre disorders of sexual development, provide rare forms of male hypogonadism is you can go to a male or untervirilisierten male phenotype ambiguous genitalia in. birth and result in some degree of testicular and spermatogenem failure. When cryptorchidism, one or both testicles not deszendiert (cryptorchidism). The etiology is unknown usually. The sperm count is slightly decreased when a testicle is not deszendiert but very low when both testicles are not deszendiert. In the bilateral agenesis of the testes (testicles syndrome disappeared) testicles were probably absorbed after or before birth. The outer genitals and the Wolff-structures are normal, while the Müller-structures are absent. Therefore testicular tissue must have been present for normal differentiation of the sex organs take place and the testosterone and Müller-inhibiting factor could be produced during the first 12 weeks. In a Leydig Zellaplasie the congenital absence of Leydig cells partially developed or intersexual sexual organs caused. Although the Wolff-speed has become a part of the existing amount of testosterone for the differentiation of normal external genitalia is not enough. , Structures of Müller-course missing because the Sertoli cells produce enough inhibiting hormone. One finds elevated serum gonadotropin levels and decreased testosterone levels. Noonan syndrome can occasionally occur as autosomal dominant disease or. The phenotype includes a hyper-elasticity of the skin, hypertelorism, ptosis, low-set ears, a small stature, a shortening of the fourth metacarpal, a high palate and primarily right-sided cardiovascular disease (eg. As pulmonary valve stenosis, Vorhofventrikeldefekte). The testicles are often small or kryptorchid. Testosterone levels may decreased gonadotropin levels have increased. A disturbed androgen synthesis is caused by an enzyme defect that impairs androgen; it can affect any pathway from cholesterol to the synthesis of dihydrotestosterone. This inborn error can (Steroidogenic-Acute-Regulatory- [Star] protein deficiency, 17?-hydroxylase deficiency, 3?-hydroxysteroid dehydrogenase deficiency overview of congenital adrenal hyperplasia z. B.) occur at a congenital adrenal hyperplasia, when the same enzyme defect occurs in the adrenal glands and testes, leading to impaired androgen and intersexual external genitalia in various expression for Folge.Sekundärer hypogonadism the causes of a secondary hypogonadism include a panhypopituitarism, hypothalamic or Hypohysentumoren, isolated gonadotropin deficiency, Kallmann syndrome, Laurence-Moon- syndrome, isolated LH deficiency, Prader-Willi syndrome and functional or acquired disorders of the CNS (trauma, infection) a. The causes of the secondary hypogonadism have to be distinguished from the constitutional delay of puberty, which is a functional form of secondary hypogonadism. Some acute or chronic systemic disorders (chronic renal failure, anorexia nervosa) can lead to hypogonadotropic hypogonadism, which subsides once the underlying disorder is eliminated. The relative hypogonadism is increasingly common among long-term survivors after cancer in childhood with kraniospinaler irradiation prior. Panhypopituitarism can be congenital or anatomical causes (eg. As septo-optic dysplasia or Dandy-Walker syndrome) and leads either to a lack of hypothalamic releasing factors or pituitary hormones. The acquired hypopituitarism can of tumors, neoplasms, or their treatment, vascular disorders, infiltrative disorders (eg. B. sarcoidosis, Langerhans cell histiocytosis), resulting infections (z. B. encephalitis, meningitis), or trauma. Hypopituitarism in childhood may cause growth retardation, hypothyroidism, diabetes insipidus, hypoadrenalism and lack of sexual development at puberty. The hormone deficiency can – depending on its origin in the anterior or posterior pituitary – have a different number of manifestations. Kallmann syndrome is characterized by a anosmia due to agenesis of the olfactory bulbs and a hypogonadism due to lack of hypothalamic GnRH. The primary defect is a lack of GnRH production; the neurosecretory neurons do not migrate from the olfactory placode to the hypothalamus. The genetic defect is known; inheritance is classic X-linked, but can also be autosomal dominant or autosomal recessive trait. Other manifestations include a micro phallus, cryptorchidism, midline defects, and unilateral renal agenesis. The presentation is clinically heterogeneous and some patients have Normosmie. Mark of Laurence-Moon syndrome are obesity, mental retardation, retinitis pigmentosa and polydactyly. Insulated LH deficiency (fertile Eunuchoidimus) is a rare form of hypogonadism because of an isolated loss of LH secretion in boys, while the FSH secretion is normal. During puberty, there is a testicular growth, since the major portion of the testicular volume from seminiferous tubules consists whose maturation is stimulated by FSH. Spermatogenesis may occur as tubular progressive development. However, the absence of LH results in Leydig cell atrophy and testosterone deficiency. Therefore, in these patients the development of normal secondary sexual characteristics remain off the longitudinal growth continues unhindered. It comes to eunuchoidal proportions, as the testosterone-induced epiphyseal closure fails. Prader-Willi syndrome is characterized by decreased fetal movements, muscular hypotonia, failure to thrive, which is later followed by obesity mental retardation and hypogonadotropic hypogonadism. The syndrome is caused by a destruction or disruption of the gene or genes at the paternal chromosome 15 or if the maternal disomy of chromosome 15 °. Failure to thrive due to the hypotension and feeding difficulty in infancy disappear at the age of 6-12 months. From 12 -18. Month introduces insatiable hunger for weight gain and psychological problems. The barrel-shaped obesity is the most outstanding feature. The rapid weight gain continues in adulthood. The small stature may be caused by a growth hormone deficiency. Behavior features include an emotional instability, delayed gross motor skills, facial abnormalities (e.g., as close temporal distance, almond-shaped eyes, a mouth with a thin upper lip and after solid bottom corners of the mouth), skeletal abnormalities (eg. As scoliosis, kyphosis, osteopenia). The hands and feet are small. The other features are cryptorchidism and hypoplasia of the penis and scrotum. The Constitutional Pubertätssverzögerung is the lack of pubertal development before the age of 14 years, which is more common in boys. By definition, show children with constitutional delay signs of sexual maturation until the age of 18 years, but the pubertal delay and short stature can lead to anxiety in adolescents and their families. Many children have parents or siblings with delayed sexual development in the family history. The boys have a short stature during childhood and / or adolescence, but ultimately reach their genetic target. The growth rate is normal, and the growth curves are in the lower percentiles of growth charts. The pubertal growth spurt is delayed and the Größenperzentil begins at the expected time in the Pubtertät decrease, which may contribute to psychosocial problems in some children. The skeletal age is delayed and is most consistent with the size of the child’s age (age at which the body size of a Kndes in the 50th percentile) instead of the chronological age match. The diagnosis is a diagnosis of exclusion of GH deficiency, hypothyroidism, systemic conditions that can slow down puberty (z. B. chronic inflammatory bowel disease, eating disorders) and hypogonadism (primary or as a result of Gonadotropinmangels). Symptoms and signs The clinical presentation depends on whether, when or how the testosterone and sperm production are affected (appearance in adults Male hypogonadism. Symptoms and complaints) If an androgen deficiency or a disturbance of androgen activity during the first trimester (<12 weeks of gestation) occurs, the differentiation of the inner mesonephric duct and of the external genitalia is impaired. The clinical appearance ranging from an intersexual appearance of the external genitals to the fully trained female genitalia. An androgen deficiency in the 2nd and 3rd trimester causes a micro phallus and an incomplete or missing Hodendeszensus. An androgen deficiency that develops in early childhood, of little consequence; but if it occurs at puberty, the development of secondary sexual characteristics may be impaired. These patients have poor muscle development, a falsetto voice, lack of age-typical growth of the penis and testicles, a small scrotum, barely pubic or axillary hair and lack of body hair. You can develop and gynecomastia a eunuchoidal (the arm span exceeds body size by 5 cm, the distance pubis ground is> 5 cm longer than the distance pubis-peak) due to delayed epiphyseal closure and continuous growth of the long bones. Diagnostic measurement of testosterone, LH and FSH karyotyping (on primary hypogonadism) The diagnosis is often suspected due to developmental abnormalities or delayed puberty, but require confirmation by studies, including the determination of testosterone, LH and FSH. The latter are – particularly for clarification of a primary hypogonadism – sensitive than in testosterone levels. LH and FSH levels can also help determine whether it is primary or secondary hypogonadism: In high concentrations, even with low-normal testosterone levels, speak for primary hypogonadism. Levels that are low or lower than expected for testosterone, indicate secondary hypogonadism. Boy with a short stature, a delayed pubertal development, low testosterone and LH levels typically have impaired spermatogenesis, Elevated FSH levels in serum with normal serum testosterone and LH levels, which are typical of an impaired spermatogenesis, but not for impaired testosterone production. In a primary hypogonadism the karyotype must be determined in order to clarify a Klinefelter syndrome. The determination of testosterone, FSH and LH for the diagnosis of hypogonadism requires the knowledge of the variability of the mirror. Before puberty, testosterone levels are <20 ng / dL (<0.7 nmol / l), in adulthood they are> 300-1200 ng / dl (12-42 nmol / l). The testosterone circadian runs. In the second half of puberty, the levels are higher than the day in the second half of the night. A single blood sample in the morning may be sufficient to determine whether the levels of circulating testosterone are normal. Since 98% of testosterone is bound to a carrier protein in serum (testosterone-binding globulin) varies with changes in these serum proteins and the testosterone levels. Measurement of serum total testosterone (protein-bound and free) is usually the most accurate indicator of testosterone secretion. Although the LH and FSH levels are pulsed in serum tests can be valuable. Puberty begins when the GnRH secretion, and the increases in serum LH increases more than proportionally to FSH. Early in puberty mirrors are preferred in the early morning. LH levels in serum are puberty below 0.3 mU / ml and fluctuate during later stages and adulthood between 2 to 12 mU / ml. LH levels in serum are puberty <3 mU / ml and vary during the 2nd half of puberty and adulthood 5-10 mU / ml. To test the presence and secretion of testicular tissue, an hCG stimulation test is performed. Several protocols are available. In a protocol, a single dose of hCG 100 units / kg i.m. administered. hCG stimulates besides the Leydig cells and LH, with which it shares a structural subunit, and stimulates testicular production of testosterone. Testosterone levels should double within 3-4 days. Treatment operation as required hormone replacement therapy cryptorchidism is corrected early to dispel concerns about the development of cancer in later life and to prevent testicular torsion (cryptorchidism). In a secondary hypogonadism any underlying disorder of the pituitary or hypothalamus is treated. In general, an androgen is the goal, with a low initial dose which is progressively increased over the next 18-24 months. The dose is increased over a period of 18-24 months at 200 mg. Adolescents with androgen deficiency should be treated with long-acting injectable testosterone preparations (enanthate or cypionate 50 mg every 2 or 4 weeks). Alternatively, a transdermal gel or patch can be applied. Treatment of Kallmann syndrome with hCG may treat cryptorchidism and restore fertility. Puberty is usually with testosterone, injectable or gel initiated. It has been previously shown by the GnRH therapy is that it can help with an endogenous secretion of sexual hormones, increasing virilization, and even fertility. The isolated LH deficiency the administration of testosterone induced - on the one caused by aromatase to estrogen conversion - a normal epiphyseal closure. A constitutional delay of puberty may be treated with a testosterone course over 4 to 6 months. After the course is completed, the treatment is stopped and testosterone levels are several weeks or months later measured to distinguish a temporary of permanent shortage. If the testosterone level is not higher than the initial value and / or the pubertal development does not proceed after completion of this treatment, a second course can be given a low dose treatment. If the endogenous puberty has not started after two treatment cycles, the probability of a permanent defect increases and the patient must be re-evaluated for other causes of hypogonadism. Important Points In primary hypogonadism affects a congenital (or acquired rare) testicular disorder testosterone production and / or damage the seminiferous tubules. In secondary hypogonadism congenital or acquired disorders of the hypothalamus or pituitary gland cause gonadotropin deficiency and failure to stimulate normal testicles. The manifestations and its occurrence timing of which vary greatly depending on when the testosterone production is affected. Prenatal androgen deficiency can lead to manifestations, ranging to normal appearing of partial undescended testicles, micro phallus and intersex external genitalia female external genitalia. An androgen deficiency that occurs when puberty is expected to affect the secondary sexual development. The diagnosis is made on the measurement of testosterone, LH and FSH levels. The treatment is performed as required with hormone replacement and surgical procedures.

Health Life Media Team

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