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Results for “Puberty, Delayed”

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PubMed1995

Testosterone deficiency and extreme retardation of puberty in homozygous sickle-cell disease.

Homozygous sickle-cell (SS) disease is associated with retardation of physical and sexual development but most Jamaican SS children commence their adolescent growth spurt before 16 years of age. Analysis of growth data from children in the Jamaican Cohort Study noted extreme growth retardation, defined as absence of the adolescent growth spurt and pre-pubertal sexual development (Tanner stage 1 or 2) at age 16 years, in 8/52 (15%) SS boys. These and two boys from the general sickle-cell clinic with a similar growth pattern provided a study group of 10 boys who were investigated for a possible endocrine explanation for their extreme retardation of physical maturation. A sub-optimal testosterone response (< 10 nmol/l) to human chorionic gonadotrophin and an exaggerated gonadotrophin response to gonadotrophin hormone releasing hormone was consistent with poor testicular function in 5 boys. Retardation of adolescent growth and development is common in boys with SS disease but, when extreme, requires early investigation to identify potentially correctable mechanisms.

Adolescent↗
PubMed1995

[Studies on sexual development, sexual behavior and ability to experience sex of young women with Ullrich-Turner syndrome].

In this investigation 25 female patients were included with the Turner syndrome (TS). From the 25 patients 6 had spontaneous menstruations, 19 had menstruations only after a hormonal stimulation. In a semi-structured interview the patients were questioned about their sexual activities, behavior and orgastic capacity. The findings were related to a matched normal female population. Moreover an analysis of personality traits by means of the Freiburger Persönlichkeitsinventar [FPI], the Giessen Test [GT], and a questionnaire on education styles of parents [EMBU] has been undertaken. The endocrine profile of the patients with induced menarche showed an elevated value of LH and FSH in serum, and a low level of estradiol. The sexual development of all patients was normal during the prepubertal phase. However the biological puberty was delayed by three years in the study patients, in relation to a normal population. Each of these women had a normal female identity and was heterosexual oriented. In concordance with the literature, patients who were substituted too late, showed defined deficits in their psychic well being, as well as in their social and psychosexual functioning. These deficits only were significant in relation to delayed menarche, but not to chronological age, gynecological age, karyotype, body height or parental education style. Therefore, an early and adequately dosed hormonal intervention and a consequently and regularly performed substitution therapy with sexual steroids has to be undertaken beside the well known intervention with growth hormone.

Adolescent↗
PubMed1994

Congenital hypopituitarism as part of suprasellar dysplasia. A case report.

Congenital hypopituitarism may be due to hypothalamic failure. The case presented below belonged to this category. In addition, the demonstration of absent septum pellucidum placed this case in the category of suprasellar dysplasia. The patient was 21 years old, with primary amenorrhea and lack of development of secondary sex characteristics. The laboratory findings confirmed the diagnosis of hypothyroidism, hypocortisolism, hypogonadotropism, hyperprolactinemia and normal growth hormone. Stimulation studies revealed a subnormal response of cortisol to adrenocorticotrophic hormone stimulation, subnormal response of follicle stimulating hormone and luteinizing hormone to gonadotropin releasing hormone stimulation, normal response of prolactin to thyrotropin releasing hormone stimulation and exaggerated response of thyroid stimulating hormone to thyrotropin releasing hormone stimulation. The patient was treated with thyroid supplementation. Magnetic resonance imaging showed a hypoplastic infundibulum, ectopic neurohypophysis, small anterior pituitary gland and absent septum pellucidum. Congenital hypopituitarism may be part of a large spectrum of midline brain abnormalities.

Abnormalities, Multiple↗
PubMed1976

Lifelong alterations in endocrine function resulting from brief perinatal hypothyroidism in the rat.

The late consequences of a brief period of perinatal hypothyroidism were studied in the rat by giving propylthiouracil (PTU) prenatally to the mothers and/or neonatally for 5 days to the pups. Perinatal hypothyroidism produced a delay in eye opening, a diminution in weaning weight, a delay in puberty and first estrus, and a prolongation of estrus cycles. The neo-PTU rats usually had a persistently enlarged thyroid gland associated with an elevated pituitary, hypothalamic, and serum thyroid-stimulating hormone (TSH) concentration. The metabolic clearance rate of TSH and response to luteinizing hormone-releasing hormone (LH-RH) were normal. The response to thyrotropin-releasing hormone (TRH) stimulation was significantly blunted in adult neo-PUT males, suggesting secondary or tertiary hypothyroidism. As a result of these studies, serious thought should be given to the possible consequences of antithyroid drug therapy of pregnant women, and certainly all pregnant hypothyroid women should receive full replacement therapy.

Animals↗
PubMed1993

[Induction of puberty and maintenance of secondary sexual characteristics in males with thalassemia major].

18 males with beta-thalassaemia major were studied; mean age was 17.6 flAF 4.3, 8 of 18 males evaluated were prepubertal (Tanner 1), 5 were early and mid pubertal (Tanner 2-3) and 5 were full pubertal (Tanner 5). All patients (pts) underwent GnRH test and 61% showed positive response. 8 pts. were treated with hCG; 2 were azoospermic, 6 were aspermic. The starting dose was 1000 IU twice weekly; dose adjustments were made basing on testosterone levels and clinical features. Clinical examination, testosterone assays, semen analysis were performed every 3 months during the treatment period. Therapy was started 18-48 months ago and it is still in progress. Within 12 months of treatment 4 pts progressed to Tanner stage 4, 3 pts to Tanner stage 5, 1 showed no response. Semen analysis showed an improvement in 6 pts: after 12 months of therapy 1 pts was aspermic, 5 were azoospermic, 1 was oligozoospermic. Our data showed that hCG administration is effective in inducing and maintaining secondary sexual character.

Adolescent↗
PubMedDate not supplied

Cushing's disease presenting as pubertal arrest.

The clinical features of Cushing's disease in childhood are usually abnormal fat distribution, moon face, growth failure and hirsutism. Laboratory studies include evidence of cortisol and ACTH hypersecretion. This report concerns a boy who presented only with arrested puberty and growth failure as physical evidence of Cushing's syndrome and showed rapid progression of puberty once pituitary adenomas were removed.

Adenoma↗
PubMed1993

[The role of prolactin on sexual maturation of female rat].

The role of prolactin (PRL) in sexual maturation of immature female rats was examined in drug induced hyper-or hypoprolactinemic models. 1. In order to examine the role of PRL before the third week after birth, at which the serum PRL level of female rats is known to be low under physiological conditions, hyperprolactinemia was induced by daily subcutaneous injections of sulpiride from day 10 to day 20. Ovarian and uterine weights, serum hormone levels and in vitro estradiol (E2) release from removed ovary were evaluated on day 15 and day 20. The inhibition of uterine growth on both days a low serum E2 concentration on day 20 and inhibition of in vitro ovarian E2 release were demonstrated as the result of sulpiride treatment. 2. In order to examine the role of PRL after the fourth week, when the serum PRL level is known to become high, hyper- or hypoprolactinemia was induced from day 22 by per os administration of sulpiride or bromocriptine. Timing of the onset of puberty weights of the ovary, uterus and adrenal gland and serum hormone levels at the onset of puberty were evaluated. In the sulpiride treated group the timing of the onset of puberty was not affected and lower adrenal gland weight and a low serum FSH concentration were observed. In the bromocriptine treated group the timing of the onset of puberty was delayed and greater adrenal gland weight was observed. Serum hormone levels were not affected. These results suggest that (i) prolactin could have an inhibitory effect on sexual maturation before the 3rd week.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMed1993

[Endocrine disorders in adolescents and young adults].

Evaluation of 158 patients younger than 25 years-of-age who had been hospitalized in a specialized adult endocrine department during an 11 year-period, brought to light specific endocrine problems connected both to pediatrics and internal medicine. There is need for close collaboration between pediatricians, as experts on disturbances in growth and pubertal development, and internists, with experience of diagnosing rare endocrinological disorders. Such collaboration might also improve the follow-up of young patients into adulthood.

Adolescent↗
PubMed1996

Successful pregnancy after bone marrow transplantation for thalassaemia.

Bone marrow transplantation from an HLA-identical sibling can cure thalassaemia. The risk of chemotherapy-induced sterility, however, represents a deterrent for many patients already at risk of gonadal insufficiency and reduced fertility because of the effects of transfusional iron overload. We report here the first patient transplanted for thalassaemia, after ablative therapy with busulfan and cyclophosphamide, who, despite late pubertal maturation, became pregnant and delivered a full-term, normal infant.

Adult↗