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PubMed1994

Asthma, glucocorticoids and growth.

Most asthmatic children grow normally. However, the disease itself and the treatments used, e.g. glucocorticoids (GCs) may affect growth, especially delaying puberty. Presently, the extensive use of early anti-inflammatory therapy is changing asthma to a milder disease and the effects on growth will probably further decline. Also, the use of inhaled instead of oral GCs has minimized the risk of systemic side-effects. High doses of inhaled GCs have systemic effects, and because there are individual differences in sensitivity to GCs, growth should be monitored in every child treated with GCs. The preparations with the lowest systemic bioavailability and the lowest dose to control asthma should be used.

Asthma↗
PubMed1998

Final height and endocrine function in thalassaemia intermedia.

We present data of a detailed study of endocrine function in 50 patients (21 males, 29 females) with thalassaemia intermedia, 15-46 years old (mean age 28.7 yr), with raised serum ferritin levels (mean 1540 micrograms/l). Mean haemoglobin concentration was 8.1 g/dl. Half of them had had more than 50 transfusions in their life and had received irregular intramuscular or subcutaneous chelation therapy. Delayed puberty was one of the most frequent (36%) clinical endocrine abnormalities found in our patients. Primary amenorrhea was observed in two patients and secondary amenorrhea in four patients. Two males, aged 19 and 36 years, had hypogonadism. A poor response to GnRH, found in three females and in both males tested, suggested that pituitary dysfunction was wholly or partially responsible for hypogonadism. Gonadal function was normal in all patients studied. Glucose intolerance and primary hypothyroidism were less frequent (24 and 5.7%, respectively) and milder than in thalassaemia major patients. Two patients had low T3 and T4 and normal basal and stimulated response of TSH to TRH. This condition has been found in euthyroid sick syndrome and it is likely that it represents an adaptive response by the body to minimize catabolism when undergoing major stress. As a consequence, we believe that periodic endocrine evaluation should be carried out in subjects with beta-thalassaemia intermedia, particularly in those over 14 years old, in order to detect and to treat endocrine dysfunction.

Adolescent↗
PubMed1998

Pituitary deficiency and lack of gonads in an XY pseudohermaphrodite with beta 39/lepore haemoglobinopathy.

We describe the occurrence of hypothyroidism and hypogonadotropic hypogonadism in an XY pseudohermaphrodite subject affected by beta-thalassemia. The patient, reared as female, diagnosed at 14 months of age as having a beta 39/Lepore hemoglobinopathy, treated with multiple transfusion therapy, was referred at age of 15 years because of delayed puberty. Complete endocrine evaluation showed low levels, both basal and after combined LHRH-TRH and hCG stimuli, of FSH, LH, TSH, estradiol (E2), testosterone (T), progesterone (P), androstenedione (A), and FT4 levels, and normal PRL, cortisol, 17OHP and ACTH levels. Imaging studies (ultrasound, magnetic resonance, radioisotope scanning and gonadal vessels phlebography) did not show internal genitalia and gonads. Karyotype resulted 46,XY. PCR amplification of the SRY gene confirmed the presence of the Y chromosome. Female genitalia without uterus in a subject with Y chromosome SRY gene, and no detectable testes indicate a condition of male pseudohermaphroditism associated with testicular regression. Low gonadotropin and sex steroid levels are suggestive of combined acquired hypothalamic-pituitary and gonadal impairment, due to iron deposition in both organs. We cannot exclude congenital failure of testosterone synthesis and action in this case, because lack of gonads is an unusual finding in thalassemic hypogonadic subjects.

Adolescent↗
PubMed2002

[Growth rate can be manipulated. Estrogen production in pubertal boys can be blocked by an aromatase inhibitor].

A review of a twelve month clinical trial [1] using a new, effective aromatase inhibitor treatment in boys with delayed puberty shows that the pubertal increase in estrogen levels can be blocked, with concomitant preserved pubertal growth rate. Circulating testosterone levels are greatly enhanced during treatment due to increased gonadotrophin secretion. Despite this, bone age maturation is slow leading to an increased final height prognosis (mean 5.1 cm) for the boys treated with aromatase inhibitor.

Age Determination by Skeleton↗
PubMed2002

The effects of sex steroids on spatial performance: a review and an experimental clinical investigation.

Males typically surpass females in spatial performance, an outcome that may be linked to testosterone and estrogen. The authors (a) review physiological mechanisms, developmental periods, and past empirical work relevant to sex steroids' effects on human spatial performance and (b) report an experimental study of the role of actively circulating sex steroids in adolescents being treated for delayed puberty (N = 55; mean age = 13.70 years). Sex steroids (simulating early, middle, and late puberty) and placebos were given alternately over 21 months and spatial tests were given every 3 months. Spatial performance showed traditional sex differences but did not vary with levels of actively circulating sex steroids.

Adolescent↗
PubMedDate not supplied

[Immunogenetic characteristics of nuclear families of adolescents with delayed sexual development].

The paper submits data on the antigenic composition of HLA system in members of nuclear families of adolescent boys presenting with the delay in sexual maturation. Positive and negative associations of particular antigens with sexual underdevelopment are stated. Estimated in the families was the highest incidence rate of gaplotypes A 28-B 40, A 28-B 8, A 1-B 40, and A 3-B 40. The authors come out with a suggestion that it might be possible to use the identified antigens in prognostication of delayed puberty.

Adolescent↗
PubMed2006

Impact of long-term iron chelation therapy on growth and endocrine functions in thalassaemia.

A large number of children treated from the time of diagnosis with modern transfusion and chelation therapy are now entering early adolescence or early adulthood, and only now can we evaluate how many complications, secondary to iron overload, can be prevented by daily s.c. desferrioxamine (DFX) therapy. In 1989, we planned a multi-centre study on growth and endocrine complications in patients who started chelation therapy with DFX early in life. Height, weight, endocrine complications, haematological variables and compliance with DFX were evaluated in a study group of 238 patients aged 2-17 years with beta-thalassaemia major regularly followed in 13 paediatric and haematological Italian centres. The LMS method by Cole and Green and the Mann-Whitney test were applied for statistical analysis. Twenty-six patients with thalassaemia (12.4%) had growth hormone insufficiency, five patients (2.1%) had primary hypothyroidism and four patients (1.7%) had hypoparathyroidism. Delayed puberty was present in 18.4% of boys and 17.7% of girls. At the beginning of chelation, standing height was in the normal range when compared to Swiss standards, while in the following years a progressive decline of growth was observed in both sexes. In conclusion, our study noted a positive effect of DFX therapy on sexual maturation and endocrine complications. Nevertheless, short stature has persisted despite major advances in treatment.

Adolescent↗
PubMedDate not supplied

[XYY syndrome. Report of a case].

A sixteen year old boy was admitted to our department for delayed puberty. The personal history was not significant and physical examination revealed only a genital hypogonadism. The endocrinology study of the patient showed a hypogonadotropic hypogonadism. The IQ was low. The personality showed infantilism and aggressive impulses. A karyotype revealed 47,XYY. We discuss here the clinical implications of the case.

Adolescent↗
PubMed1991

[General and local growth disorders in chronic arthritis in childhood].

In juvenile chronic arthritis both general and localized growth disorders are found. The general growth rate is especially impaired in the severe forms of the disease. In addition, delayed puberty with retarded pubertal growth spurt can temporarily result in growth retardation. Long term treatment with high dosage corticosteroids may intensify the growth failure, leading to severe stunting. Local growth disturbances are caused primarily in the area of the affected joints and secondarily as a result of functional disorders. The individual joints react in a specific, age-dependent manner. Growth acceleration and retardation, as well as various deformities of the bone structures, can be observed. Hands, knees and feet present typical examples of different growth disorders. In the toddler the inflammatory growth stimulus predominates first. This leads to accelerated ossification of the wrist and ankle as well as growth increase in knees, fingers and toes. In the further course and in the older child, shortening and growth reduction predominate. Here the premature fusion of the bones observed for example in fingers and toes, but also in the area of the distal part of the ulna, plays an important role. Arthritis can lead to deformities in all the joints. These deformities are more severe in children with early onset of the disease. Secondary growth disorders are caused by minor use of the joints. When wrists or elbows are involved, the entire hand may remain smaller. In the feet the growth impairment is often even more prominent, due to arthritis of the hips, knees or ankles. Both the general and the local growth disorders can be compensated to a large extent if treatment is begun at an early stage and the disease can be brought to remission. It is of great importance to preserve mobility of the joints in view of the interaction between function and growth.

Adolescent↗
PubMed1985

[A case of an XX man with presence of H-Y antigen in his mother].

An adolescent of 17 years of age presented with delayed puberty and cryptorchidism underwent hormonal and cytogenetic investigation. The FSH and LH levels were raised and there was a significant response to LHRH. The karyotype was 46 XX. Cytogenetic studies showed no mosaic or translocation of the Y-chromosome. The HY antigen was present on the surface of the normal lymphocytes stimulated by phytohaemagglutinin or the pokeweed mitogen or the transformed lymphocytes. The mother also had the HY antigen at concentrations intermediary between those of normal men and women. These results suggest an autosomic location of the HY gene and of the determination of the gonadal orientation by the levels of HY antigen.

Adolescent↗
PubMed1986

Gynecological concerns in young and adolescent physically active girls.

Young girls should be advised to develop the regular exercise habit and to maintain it throughout life. Prepubertal athletes are more likely than their sedentary friends to experience menarcheal delay. Postmenarcheal athletes have increased susceptibility to oligomenorrhea and amenorrhea. Athletes with delayed puberty deserve examination and possibly further evaluation. Adolescent athletes with oligomenorrhea or amenorrhea deserve examination and hormonal evaluation. Hormonal replacement therapy is unnecessary prior to age 16, is optional between ages 16 and 18, and is recommended after age 18.

Adolescent↗
PubMed1983

Severe chronic mucocutaneous candidiasis. Favourable response to oral therapy with ketoconazole.

Severe, early-onset, chronic mucocutaneous candidiasis was associated with bronchiectasis, oesophageal stricture, short stature and delayed puberty in a male aged 18 years. Topical treatment with antifungal agents, and several courses of intravenously administered transfer factor, amphotericin, and miconazole achieved only minor or transient improvement in the patient's condition. Correction of iron deficiency anaemia did not lead to alleviation of candidiasis. Skin reactivity to Candida antigen was absent and T-lymphocytes, which responded normally to phytohaemagglutinin (PHA), poke-weed mitogen and concanavalin A, had negative macrophage-inhibiting factor (MIF) and blastogenic responses to Candida antigen. Treatment with the orally effective imidazole derivative, ketoconazole, produced improvement within three days and clearing of mucosal lesions within five weeks. The patient then entered puberty spontaneously at the age of 20 years. After 18 months of treatment with ketoconazole, without side effects, the clinical manifestations of mucocutaneous candidiasis have not recurred. Because of the possibility of continuous, long-term administration, ketoconazole represents the best currently available agent for the treatment of chronic mucocutaneous candidiasis.

Adolescent↗
PubMed1983

Kallmann's syndrome. A case report.

A patient with hypogonadotrophic hypogonadism and anosmia (Kallmann's syndrome) presenting as delayed puberty is described. The clinical, hormonal and testicular histological features are noted. The basic principles of treatment are discussed.

Adolescent↗
PubMed1981

Effects of neonatal pyridostigmine treatment on sexual maturation and adult behaviour in female rats subjected to maternal deprivation.

The present study deals with the effects on adult behaviour of early postnatal maternal, i.e. psychosocial and/or nutritional deprivation and of altering the brain levels and/or turnover rates of acetylcholine in preweaning deprived rats. The following data were obtained: 1. Female rats separated from their mothers and littermates for 16 hours each day during the first two weeks of life showed a significant delay of puberty onset in comparison with non-separated controls. Furthermore, these animals exhibited significantly reduced emotionality as well as learning capability and memory capacity in adulthood. 2. Administration of the acetylcholinesterase inhibitor pyridostigmine during the first two weeks of life to the deprived females did not compensate the puberty-delaying effect of separation. However, the neonatally pyridostigminized female rats exhibited an increased emotional reactivity in adult life. 3. Female rats deprived from their mothers and treated with pyridostigmine during the neonatal period displayed increased avoidance learning and memory capacity in adulthood as compared to neonatally deprived but untreated animals. It might be concluded from our results that defects of brain differentiation produced by early postnatal deprivation could be prevented, at least in part, by simultaneous administration of pyridostigmine.

Acoustic Stimulation↗
PubMed1980

[Adolescents in pediatric practice (author's transl)].

Some problems concerning adolescents arising in pediatric practice are reviewed: morphological problems (stature, nutrition, vertebral statics), puberty problems (delayed puberty, acne vulgaris, menstrual disorders, contraception, mammary development), health problems proper (especially epilepsy) and, lastly, problems related to environment: school, family, media. The pediatrician seems to be the doctor most qualified to approach adolescent just as he is for children.

Adolescent↗
PubMedDate not supplied

[Methods for correcting retardation of sexual development in the irregular puberty syndrome in boys].

A total of 46 boys aged 11 to 13 with the abnormal puberty syndrome were examined. The authors assessed various methods of correction of delayed puberty by the time course of anthropometric and genitometric indices and blood testosterone, LH and FSH levels 1 year after the initiation of treatment, at the age of 14 to 16 and 17 to 19. The ejaculate composition of 12 adolescents over 17 was examined. In 3/4 of the adolescents the indices of physical development and pubescence, blood testosterone and gonadotropic hormone levels achieved physiological values by the age of 14-16, but in 1/4 of the patients delayed puberty with lowered FSH and testosterone levels and raised LH in the blood was noted. The normalization of puberty was noted at the age of 17 to 19 with normal spermatogenesis. The necessity and methods of therapeutic correction of delayed puberty in boys and adolescents with the abnormal puberty syndrome were discussed.

Adolescent↗
PubMed1998

Androgen replacement in children with constitutional delay of puberty: the case for aggressive therapy.

Puberty describes the complex physiological transition between childhood and adulthood. Dramatic physical changes occur, most notably the development of secondary sexual characteristics and the pubertal growth spurt. During the adolescent growth spurt, growth velocity increases from pre-pubertal rates of 4-6 cm per year to as much as 10-15 cm per year. Accompanying the increase in gonadal steroids is an increase in amplitude of growth hormone secretory bursts. Evidence suggests that adequate growth hormone and gonadal steroids are both necessary for the attainment of a normal pubertal growth velocity, and the complex interplay between these two hormonal axes is under intense investigation. Delayed onset of puberty, or constitutional delay of growth and adolescence, is a common phenomenon presenting particularly in boys. Physiologically, it represents an extension of the normal pre-pubertal hypogonadotropic hypogonadal state. Without intervention, these children will spontaneously undergo puberty and often reach their genetic height potential, but their delay compared with that of their peers is often of concern to the children and their families. Recent evidence suggest long-term physiological benefits of early androgen replacement therapy in these boys, including maximizing attained bone mineral density. Androgen replacement therapy in male adolescents with constitutional delay of growth and adolescence is beneficial psychologically as well as physiologically and should be initiated promptly.

Adolescent↗
PubMed2004

Inhibin B in pubertal development and pubertal disorders.

The inhibin B pubertal surge is a prominent signal of gonadal maturation in females as well as in males. In boys, it denotes the final functional maturation of Sertoli cells, which is accompanied by a progressive suppression of antimüllerian hormone production. In girls it reflects the initial recruitment of preantral follicles and their evolution to the antral stage. In both the prepubertal quiescent phase and the active peripubertal phase there is a striking sexual dimorphism, inhibin B levels being significantly higher in boys than in girls, in contrast to follicle-stimulating hormone (FSH) levels. Determining inhibin B levels together with FSH levels is of considerable help for diagnosing disorders of pubertal development. In girls with central precocious precocity, inhibin B levels are in accordance with the clinical stage of maturation, by contrast to normal or low levels in the McCune-Albright syndrome. In boys with delayed puberty, inhibin B levels are very low in congenital defects of the gonadotropin-releasing hormone-FSH-testis axis, but they are normal or intermediate in constitutional delayed puberty. Together with antimüllerian hormone, inhibin B is a useful marker of the presence of Sertoli cells in bilateral cryptorchidism and in the androgen insensitivity syndrome. In addition, inhibin B measurement, together with that of inhibin A, is helpful for the diagnosis and follow-up of inhibin-secreting tumors: granulosa cell tumors in girls and Sertoli cell tumors of the Peutz-Jeghers syndrome in boys. In conclusion, inhibin determination is an essential tool in the assessment of physiological development as well as in the management of pubertal disorders.

Humans↗