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PubMed1987

Urticaria associated with antigonadotropin-releasing hormone antibody in a female Kallman's syndrome patient being treated with long-term pulsatile gonadotropin-releasing hormone.

The administration of pulsatile gonadotropin-releasing hormone (GnRH) has received increasing attention as a method of inducing ovulation or initiating puberty. Few side effects have been reported, although urticarial allergic reactions have been reported in the male. An 18-year-old female with hypogonadotropic hypogonadism and anosmia due to lack of endogenous GnRH was treated for 230 days using subcutaneous GnRH in an attempt to induce physiologic puberty. Just before anticipated menarche, therapy was discontinued because of the appearance of an urticarial reaction at the injection site as well as at previous injection sites. The presence of immunoglobulin G (IgG) antibodies against GnRH were subsequently identified in the patient's serum. These results further confirm the potential for antibody production to this small natural peptide in the female not previously exposed to GnRH. Some practical considerations for this form of therapy are highlighted.

Adolescent↗
PubMed1986

Low-dose testosterone effect on somatic growth.

Low-dose testosterone has been found to preserve the growth potential of hypogonadal children requiring anabolic or androgenic therapy. Five girls with Turner syndrome were treated when their chronologic ages were 13 to 14 years and their bone ages were 10.6 to 12.75 years; six hypogonadal boys were treated when their chronologic ages were 11 to 15 years and their bone ages were 10.9 to 14.2 years. Depot testosterone was given as an anabolic agent in an average dose of 28 mg/m2/mo for 6 months to the patients with Turner syndrome and was given to initiate puberty in an average dose of 44 mg/m2/mo for 6 months to the hypogonadal boys. Growth rate doubled on these doses of testosterone, and bone age did not advance disproportionately. Consequently, height potential was preserved. Pubic hair advanced one Tanner stage during the 6-month treatment. Clitoral hypertrophy was observed in only one of the five girls with Turner syndrome and regressed when testosterone therapy was discontinued. Four hypogonadal boys were continued on low-dose testosterone until their bone ages passed 14 years of age and their growth rate waned. Then, the testosterone dosage was increased in increments to 100 to 200 mg/m2/mo. This group reached a height of 100.3 +/- 0.8% of the height initially predicted. In addition, all attained an adult height at least 15 percentiles greater than that before therapy. These studies indicate that testosterone in very low doses resembles "anabolic steroids" in that growth is stimulated without an inordinate androgenic effect. Furthermore, these studies show that institution of low-dose therapy in the early teenage years stimulates pubertal growth normally without loss of height potential.

Adolescent↗
PubMed1985

Male hypogonadism--current concepts and trends.

Important advances have been made recently in our knowledge of hypothalamic-pituitary-testicular functions. Current understanding of the hypothalamic control of pituitary gonadotrophin secretion and the mechanisms of testicular feedback is beginning to yield benefits in the clinical management of certain reproductive disorders. But much remains to be learned about the neuroendocrine control of hypothalamic GnRH secretion--an area which encompasses the mechanisms regulating the onset of puberty. Spermatogenesis is a highly complex process involving subtle interactions between endocrine, paracrine and autocrine regulators acting on different cell populations within and without the testis. Until basic research can advance the very limited knowledge in this area, we cannot expect to improve the currently unsatisfactory management of infertile men. It is not surprising that only limited information can accrue from conventional semen analysis and measurement of systemic hormones. Nor is it unexpected that empirical or seemingly rational treatments for male infertility are of dubious benefit. In future, assessment of the infertile man should be improved by studying sperm functional capacities and testicular synthesis and metabolism of gonadal steroids and by the definition of representative markers of Sertoli cell and epididymal functions. Reversible male contraception is another objective that remains beyond our reach at present. Hormonal disruption of spermatogenesis is often incomplete and invariably suppresses Leydig cell function, so that androgen replacement for sustaining sexual function is mandatory. Alternative approaches to male contraception aimed at the epididymis and sperm-egg interaction seem more promising avenues for future exploration.

Aging↗
PubMed1979

Combined test of hypothalamic-pituitary function in growth retarded children treated with growth hormone. I. Secretion of growth hormone and somatomedin before and after treatment.

In 23 growth retarded children two consecutive insulin tolerance tests (ITT) were performed to establish a diagnosis of growth hormone (GH) deficiency. Nine children did not respond (GH peak value less than 8 mU/1), whereas 14 were classified as having partial GH deficiency (GH peak value less than 20 mU/1). All were treated for an average period of 40 months with human growth hormone (HGH). In a combined stimulation test at the end of the treatment period 9 children demonstrated a persistent GH deficiency, whereas a normal response was found in 14 of the previous partial GH deficient children. During treatment the monthly growth rate rose from 0.21 cm 0.58 cm in the GH deficient children and from 0.31 cm to 0.70 cm in the partial deficient children, in most of whom spontaneous pubertal development occurred during treatment. Somatomedin (SM) values were decreased in the GH deficient children before and after treatment but increased to normal levels during treatment. Growth velocity in these children during treatment was correlated to SM values before treatment. In the partial GH deficient children SM values were subnormal before but normal after treatment. This supports the assumption that in some children with constitutional delay in puberty a reversible functional hypopituitarism exists, which is normalized after the onset of puberty, due to androgens sensitizing growth hormone releasing mechanisms. Treatment with HGH may induce increased growth velocity in some of these patients.

Body Height↗
PubMedDate not supplied

Reproductive defects in patients of both sexes with cystic fibrosis: a review.

Current knowledge of reproductive duct function in both male and female cystic fibrosis patients is reviewed. Almost all male patients are sterile because of azoospermia. The mechanistic basis of the obstructive defect leading to pre- and post-natal destruction of selected portions of the sperm-conducting network remains unknown. Female cystic fibrosis patients are fertile. Pulmonary status appears to be the most important factor in determining pregnancy outcome. In contrast to earlier concerns about the normality of breast milk, breast milk composition of cystic fibrosis mothers is normal, and they can successfully breast feed their infants.

Adolescent↗
PubMed1979

[Applications of the urocytogram in primary-secondary amenorrhea in adolescents].

In the urinary sediment, the cellular material mainly originates from cells of the bladdertrigone. These cells are submitted to hormonal stimulation and their study constitutes the base of a cytological method called "urocytogram". The urinary sediment was examined in thirty-five young girls with backward puberty. The repeated examinations contribute to differential diagnosis and help in etiologic diagnosis. Whenever it is necessary to investigate adolescent's sex hormones, an urocytogram, a simple and painless method, is indicated.

Adolescent↗
PubMed1983

[Andrology].

Explore the source record for details and available documents.

Adenosine Triphosphate↗
PubMed1984

[Cohen's syndrome in 2 sisters].

A new familial observation of Cohen syndrome in two sisters is reported. Both children exhibited the typical features of Cohen syndrome, i.e. obesity, hypotonia, mental deficiency and a dysmorphic syndrome mainly involving the facies and extremities. More accurate delineation of the nosologic limits of this syndrome is ensured by a critical review of the 17 observations published in the medical literature, which include 9 familial cases: the contribution of subsequent observations to the initial description is pointed out. Attention is drawn to the unusual course of the obesity which typically occurs after five years of age, in contrast to common obesities. Furthermore, Cohen syndrome is remarkable by the morphologic abnormalities, particularly those of the facies, which should suggest the diagnosis. As in our observation, data from the literature is in support of autosomic recessive inheritance which implies restrictive genetic counseling.

Abnormalities, Multiple↗