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Many studies have concentrated on investigating the effects of short and medium term hypobaric conditions in people living at low altitudes. In contrast, only little is known about long term or genetic adaptations to chronic hypobaric conditions in people living at high altitudes. A small number of phenotypic characteristics have been defined in these people so far, comprising hormonal functions, oxygen saturation of hemoglobin, skeletal growth, muscular morphology and function, cardiovascular functions, and fluid and electrolyte changes. Several of these characteristics have been attributed to genetic adaptations. However, so far, no specific genes coding for any of the specific phenotypes found in high altitude dwellers have been detected.

Teenage pregnancies have been considered mainly as perinatological and social problem up to now, in contrast biological and evolutionary aspects were neglected. In the present analysis the hypothesis is discussed that teenage pregnancies may represent a fitness maximizing strategy, with other words an efficient use of the relatively short female reproductive span. Results from auxology, primatology, historical demography and life history research, however, are in contradiction to the hypothesis. Extremely early teenage pregnancies (below the 16th birthday) seem to be a recent phenomenon caused by the decline of sexual maturation in course of the well described secular acceleration trend.

Leptin is mainly synthesized by adipocytes and might represent the connecting link between fatty tissue and brain. In reference to reproduction, leptin resistance could play a role particularly in the pathogenesis of the PCO syndrome. However, there exists at present contradictory data on this, so that further clarification is necessary. Leptin interacts with the steroid synthesis to a degree not yet precisely clarified and possesses receptors in numerous tissues, which suggests extensive local and endocrine effects. Its exact significance for the initiation of puberty still remains unknown. The same is true for first data regarding leptin and endometriosis and the interpretation of hyperleptinemia during pregnancy. It is clear that this protein mediates between fatty tissue and the reproductive function. However, the detailed physiologic and pathophysiologic role of leptin in reproduction can only be clarified through further extensive studies. To date there is not yet a practical importance for the measurement of leptin in routine work in reproductive medicine.

Die phoniatrische Betreuung der kindlichen Singstimme während des Stimmwechsels erscheint gerade bei Sängern in Knabenchören mit hoher stimmlicher Belastung wichtig. Die vorliegende Studie sucht nach Methoden, den Eintrittszeitpunkt der Mutation vorherzusagen und das Vorliegen einer Mutation differentialdiagnostisch von hyperfunktionellen Stimmstörungen oder entzündlichen Erkrankungen des Stimmorganes zu trennen. Dazu wurden 36 Knaben des Leipziger Thomanerchores im Sinne einer Longitudinalstudie alle 3 Monate über 3,5 Jahre bis zum hörbaren Einsetzen der Mutation untersucht. Es erfolgte die Bestimmung von zehn stimmlichen und acht stimmunabhängigen Parametern, die erstmals in solch umfassender Form auf ihre prädiktive Aussagekraft geprüft wurden. Die statistische Auswertung wies besonders den Serumtestosteronspiegel und die Wachstumsrate als Grössen aus, die mit dem Prämutationsverlauf korrelieren und eine konkrete Vorhersage ermöglichen. Das Vorliegen einer Mutation kann ausserdem durch den Verlauf der mittleren ungespannten Sprechstimmlage und unter Hinzuziehung des Genitalstatus bestätigt werden. Durch die exakte und prädiktive Bestimmung des Mutationsbeginnes können Erkrankungen des Stimmapparates vermieden werden, die aus dem intensiven Singen in der Mutation resultieren. Weiterhin geben die Ergebnisse dem Chorleiter wertvolle Informationen für die Planung der Besetzung seiner Knabenstimmen.

The knowledge about metabolism and muscular fatigue has been considerably improved during the recent years. Intramuscular pH should not generally be discussed as a factor of cellular fatigue as it has been shown to increase transiently at the beginning and to be very differently affected in ST-(6,9) and FT-fibers (6,2) at the end of exercise. During maximum exercise, we assume changes of muscle membrane potential due to increasing interstitial potassium concentrations as an important performance-limiting factor. The role, lactate is playing during exercise, has to be thought over. Its production by the binding of two protons to the pyruvate molecule is necessary for an intensive anaerobic and aerobic metabolism. The lactate molecules itself represent a source of energy, which is preferentially used by the heart muscle and by ST-fibers working at lower intensity. During long term endurance events, the glycogen stores are assumed to be a limiting factor. However, no direct casual relationship between glycogen and fatigue mechanisms has been found hithertoo. The anaerobic glycolytic metabolism decreases during long lasting exercise as a result of lowered glycogen stores. Therefore, metabolic acidosis cannot be observed after ultra-long endurance events. In contrast, a respiratory alcalosis is the common finding.

High impact training may influence the pubertal development of athletes. The effects of high intensity training on pubertal development of female and male elite gymnasts are presented. 22 female and 18 male elite gymnasts were enlisted in this study. Prepubertal and pubertal stages were determined and hormonal levels regarding the hypophyseal, gonadal and adrenal axes were measured. The LH/FSH ratio necessary for the advancement of full pubertal maturation was assessed at 13.9 amd 13.0 years in female and male gymnasts, respectively. Female gymnasts demonstrated, on average, a delayed bone age of 1.7 years compared to their chronological age, whereas both bone and chronological age were identical in male gymnasts. There were no significant pubertal increase of estrogen levels in female gymnasts during their peripubertal development indicating a low amount of fat mass in female elite gymnasts. Intensive physical training of elite female gymnasts combined with inadequate nutritional intake markedly affect pubertal development. These peripubertal effects are not observable in male gymnasts due to different training regimes in male and female elite gymnast. Regular monitoring of female gymnast during their vulnerable growth phase is necessary to minimize life-long physiological and psychological side effects of high impact training.

Normal puberty is characterized through an extensive amount of biological manifestations and clinical phenotypes, which develop over a period of two to three years. The clinical maturation process is determined by the beginning of the function of the hypothalamus-pituitary-gonad-axis. Comparable to the situation at the beginning of the climacterium, during the adolescent period often a disturbed balance between estrogen and progesterone can be found in the sense of an ovarian luteal insufficiency with the consequence of a relative progesterone lack. The most evident symptoms in many cases are irregular vaginal bleedings. A careful differential diagnosis is the basis for the differentiation between physiological changes during the pubertal development and pathologies. Some disturbances resulting from luteal deficiency may lead to serious consequences like impairment of fertility or diseases of the mammary gland, especially if they exist untreated over a longer period. Therefore early and adequate treatment with a suitable progestin is of great importance for the further destiny of the patient. Furthermore, the multitude of differential partial effects amongst the available progestins require a differentiated choice of the right one.

Using stable isotope dilution/gas chromatography-mass spectrometry, we determined the plasma concentrations of 5 alpha-androstane-3 alpha,17 beta-diol (AD) and 5 alpha-androstane-3 alpha,17 beta-diol-glucuronide (ADG) in 20 patients with premature pubarche (16 patients with idiopathic premature pubarche, 4 patients with late onset 21-hydroxylase deficiency) and in 55 healthy children with Tanner stages P1 to P4. No differences between sexes were found in healthy children with Tanner stages P1 and P2. Patients with idiopathic premature pubarche (median, range, nmol/1: 0.22; 0.12-0.31) or late onset 21-hydroxylase deficiency (0.27; 0.23-0.29) had higher plasma AD concentrations than healthy prepubertal children (0.09; 0.00-0.17). Regarding ADG, patients with idiopathic precocious puberty (1.35; 0.25-4.74) or late onset 21-hydroxylase deficiency (4.01; 3.50-4.58) had also higher plasma concentrations than healthy prepubertal children (0.35; 0.00-0.75). Thus, AD and ADG, which both represent end metabolites of peripheral androgen metabolism, can be regarded as markers of androgenicity. Steroid analysis by mass spectrometry is recommended, whenever uncertainties of immunological determinations are to be avoided.

This report deals with the pathology, diagnosis and therapy of cryptorchidism from the andrological point of view. Hypothalamic-pituitary gonadal failure and a reduced transformation of gonocytes are the key factors in fertility disorders. The aim of all well-timed therapy must be to improve the fertility potential of the sexually mature male. At present, the determination through biopsy of the fertility index during orchiopexy offers the only possibility of prospective diagnosis in this respect. In the future, it will be necessary to have a standardized procedure in order to guarantee the long-term effect of correctly timed orchiopexy on fertility.

Modern surgical treatment of hypospadias is reviewed. Early microsurgical correction of hypospadias is gaining ground in view of early presentation of the patient; this means minute tissue relations that require very precise reconstruction. The operative correction is described as an example of a complex treatment: psychosexual adjustment, endocrinological development and sexual function must all be taken into increasingly frequent account.

Benign prostatic hyperplasia (BPH) is primarily a anatomical diagnosis. The prepubertal weight of the gland is about 1.6 g and at the end of the third decade around 20 g. With histological evidence for BPH there is an increase of weight and volume with aging. The human prostate is divided into zones which form, however, a fairly regular gland. The prostate is organized similar to a grape, these parts are surrounded with the epithelial cells which express different compounds and proteins and finally disappear by apoptosis. The stromal compartment is kind of a skeleton, both compartments (epithelial and stromal part) present manifold interactions based on androgens, androgen receptors and growth factors.

About a third labium (labium tertium pudendi feminae). In a half years investigation the question was studied, how often you may find variations from the well known pattern of the vulva: In 5.3% of 1180 Patients we found between the labium majus and minus a third labium, located ventral in the rima pudendi: Most frequently we found this third labium in the females between 21 and 40 years old: 6.3%. In the women older than 60 years the labium tertium was rarer: 3.1%. The labium tertium was 4.9 cm long in the average. Besides these 5.3% of females with a clear third labium we found in 15.8% of all our investigated females a smaller, shorter labium tertium, in the average 2.6 cm long. In the patients with a clearly developed labium tertium we saw it in about half of all our patients bilateral, in the other half only on one side, on the right side more often than on the left side. In the less clearly developed labia tertia we found these in about a third of all the patients (29.9%) bilateral, in the other patients it was developed about as often on the right as on the left side. Macroscopically the labium tertium as to its appearance and structure is the same as the labium minus. It evolves from the labium minus in most of all cases laterally. The possible reasons for the fact are discussed that this third labium which is easy to see, was not described before!

The decision, whether or not an abnormality of breast development needs consideration, fundamentally depends on the knowledge of the time sequence of thelarche, menarche and pubarche. Their occurrence is normally one to two years apart. Neonatal hypertrophy or asymmetries are frequent and do not need treatment. Painful enlargement of the breast might appear during puberty and can be treated symptomatically or with progestogen gel. Persistent micromastia or macromastia (virginal hypertrophy) is a more serious event. After thoroughful consultation the patient might be referred to the plastic surgeon. If the breast development is deficient after the age of 14, further diagnostic steps should be undertaken in order to exclude abnormalities of sexual differentiation. The most significant symptom for the latter is primary amenorrhea.

A group of 609 Czech 16 to 18 years old girls was divided according to the age of the first menses into three subgroups: first with menarche between 10 to 12 years, second in 13 and 14 years, third in 15 and 16 years. It was found that the age of menarche did not distinctly influence the course of their sexual development during the puberal period. The mean age of first rendezvous and coitarche did not significantly differ between the followed three subgroups. Out of the whole group of 609 probands 304 girls already had sexual intercourse. The percentage of coitally orgastic girls was highest in the subgroup with an early first menstrual bleeding between 10 and 12 years of age and the difference to the other two subgroups with a later menarche was statistically highly significant (p < 0.005). The opinion is expressed that changes in brain which enable girls to fall in love for the first time are from the point of view of biological prerequisites more important for the beginning of full sexual life in pubertal girls than hormonal development manifested by first menstrual bleeding.

Normal puberty has a characteristic harmony or consonance between the individual modalities of sexual maturation. Thus, the objective description and detailed knowledge of physical pubertal development is the basis of the clinical analysis of pubertal disorders. An aim of the review is to provide details of the visible changes the clinician must note in order to document the normal progression of puberty. Disorders of puberty can be classified by the timing of onset of sexual characteristics into either precocious or delayed puberty. A more useful concept, however, than the abnormalities of timing of puberty is whether the pubertal development is true (consonant) or if there is a pseudo-puberty (absence of consonance). This is of greater significance in relation to the pathophysiology and, therefore, the investigations and therapies which will be required. The use of either well known or new agents that may be used for treatment of pubertal disorders are presented.

Six prepubertal girls presented with precocious pseudopuberty associated with ovarian follicular cysts. Five patients normalized spontaneously after several months, in one patient the cyst was removed by laparotomy. Elevated estrogen serum levels and failure of gonadotropin responses after gonadotropin releasing hormone were the classical findings in these patients during the acute period of the disease. In a later period, however, estrogen and gonadotropin levels may be normalized although breast development is still present. Surgical treatment was performed in one patient although spontaneous remission would have been probable. Three patients showed several episodes of precocious pseudopuberty. In three patients ACTH testing was performed after dexamethason suppression. All three patients showed elevated 17-hydroxyprogesterone responses which were similar to those in heterocygotes for C21-hydroxylase deficiency.

In a longitudinal study the british pediatrician J.M. Tanner evaluated the development of breasts (thelarche), of pubic hairs (pubarche) and age of menarche. In Hungary we did a similar evaluation in 680 girls in the years 1980 to 1986. Comparing these data in a cross-sectional study with similar data from Münster we named the results central-european study. The results are similar the Tanner ones exceptionally the duration of breast development, which was shorter than in the hitherto published longitudinal studies. Mean age of menarche in our study was 12.5 years compared with 13.5 years in the former ones. This is not a confirmation to acceleration of puberty, because in other countries age of menarche was stabile. Additionally, to thelarche, pubarche and menarche we collected data about axillarche.

The normal course of precocious puberty is progredient resulting eventually in full sexual maturation. In few cases a transient form of the disease has been recorded, characterized by a temporary appearance of signs of sexual maturation. We report on a female patient with an undulating course of the disorder: after precocious initiation of puberty, a temporary interruption and subsequent resumption of sexual maturation occurred. Presently, the patient is successfully treated with an LHRH-analogon. Within the framework of this case problems in diagnosis and treatment of precocious puberty are being discussed.

About 50% of our patients with concern about their actual or final height are adolescents (girls greater than 11 years, boys greater than 13 years). Growth data of 103 adolescent patients (70 boys, 33 girls) seen in 1988/89 are analysed. 85% of the patients (90% of the boys) complained of short stature (length less than 3rd centile) whereas tall stature (height greater than 97th centile) was more frequently concerning girls (9 out of 16 patients). Genetic short stature and constitutional delay of growth and adolescence (CDGA) were most often diagnosed in short stature patients. When retardation of physical maturation is a major concern in patients with CDGA, treatment with a low dose of sex steroids leads to an acceleration of growth and pubertal development. This advancement of maturation is important for reducing the psychosocial difficulties of the concerned adolescents. Constitutional tall stature was the most frequent diagnosis in our tall adolescent patients. If predicted final height is above 185 cm for girls or 200 cm for boys the administration of height dose sex steroids (ethinylo-estraidol or conjugated oestrogen for girls, testosteron for boys) for height reduction can be tried, since a reduction in height between 3.5 cm and 7.9 cm has been reported for girls and similar data exists for boys. Nevertheless a cautious approach must be advocated for the administration of oestrogens because possible long term hazards can not yet be excluded.

Endocrinological function was evaluated in 31 children after successful treatment of acute lymphoblastic leukaemia. All patients had received combination chemotherapy and 12-24Gy of cranial irradiation according to the German therapy protocols BFM-81, BFM-83 and BFM-86. Height, weight, bone age and pubertal development, as well as hypothalamic-pituitary function were measured. Long-term linear growth was unaffected in all patients. However, 9 patients showed subnormal serum growth hormone levels in response to pharmacological stimulation of the pituitary. All patients had normal levels of T3 and T4, but one patient showed an increased response of thyrotropin to thyrotropin releasing hormone. All prepubertal and postpubertal children demonstrated appropriate secretion of follicle-stimulation hormone (FSH) and luteinizing hormone (LH) after stimulation with LH-releasing hormone (LH-RH). 3 pubertal girls showed adequate oestradiol levels, but abnormally high levels of gonadotropins in response to LH-RH. Sexual development was normal in two of them, but the third had a late menarche and irregular menses. The significance of these findings is discussed in the context of recommendations possibly to further reduce or completely delete prophylactic cranial irradiation.

True precocious puberty represents a serious developmental disorder necessitating immediate therapeutic measures, whereas premature thelarche is a harmless variation from the norm. For the differential diagnosis the ultrasonographic evaluation of the internal genitalia is of value. Girls with precocious puberty reveal a statistically significantly greater uterus length and volume as well as ovarian size. The stimulated LH/FSH quotient allows verification of the diagnosis. This value is found always to be greater than 1 in precocious puberty and always less than 1 in premature thelarche.

On 596 healthy boys and adolescents from 4 to 17 years of age measurements of the length and the width of the left testis as well as of the thickness of scrotal skin were carried out by means of a caliper, and the testis volume was secondarily calculated. Age specific mean values (means) and their +/- 2 s-limits are listed. Nonlinear s-shaped regression lines of the three main parameters clearly underline that the greatest increase is to be found in the length of testis. Consequently, as an indicator for gonadal developmental disturbances instead of testis volume measuring of testis length is recommended for routine in the physical examination of inpatients and outpatients as well as for the screening of schoolboys and male teenagers.

No precise distinction is usually made now between puberty and adolescence. Therefore, we describe the physiology and pathology of both phases with their characteristic clinical features, hormonal changes, and therapeutic consequences. In addition, we refer to the typical problems of this age group, the psychic and somatic variants of the norm; special regard is paid to puberal gynecomastia and obesity.

Mycoses affect people of all ages world wide. Furthermore, different fungi can produce the same clinical signs and symptoms, equally similar fungi can produce many different clinical pictures. Despite of this, particular fungal infections are found more common in people of certain age groups than in others. For example, candidosis is seen frequently in babies whereas fungal infections of the feet rarely occur in children. However, there is little difference in the fungi affecting adolescents and adults. Then again, elderly people are more vulnerable to candidal infections. Besides this, there is a current increase in fungal infections in immunosuppressed patients of any age.

Reconstruction of the adolescent female breast after full-thickness burns differs from that of the mature female breast. Whereas a final correction is aspired to in the adult, the operative procedure should be adjusted to the evolution of the breast in the adolescent. Our therapeutic guidelines as well as the advantages and disadvantages of the different procedures are discussed.