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Biologically ineffective gonadotropin secretion: two cases.
Symptoms of gonadal insufficiency, in the presence of high serum levels of gonadotropins, generally indicate primary gonadal failure. An exception to this generalization is the secretion of ineffective gonadotropic hormone secretion by the pituitary. One such case was reported in 1979. However rare it may be, this disorder should still be taken into consideration when evaluating hypogonadal patients. In fact, it may not be so rare as it is supposed. In this report, two such cases are presented. They both show signs of gonadal insufficiency in the presence of high serum gonadotropin concentrations, but responding normally to exogenously administered chorionic gonadotropin.
Endocrine status and growth after malignancy treated in childhood or adolescence.
Six girls and three boys, asymptomatic after treatment for acute lymphoblastic leukemia (ALL) or endodermal sinus tumor (EST), were investigated for endocrine status and growth. Serum follicle stimulating hormone (FSH), luteinizing hormone (LH), estradiol (E2), prolactin (PRL), testosterone (T), androstenedione (A), dehydroepiandrosterone sulfate (DHEAS), testosterone binding capacity (TeBC), 17 alpha-hydroxyprogesterone (17-OHP), progesterone (P), thyroxine (T4), thyroid stimulating hormone (TSH), and cortisol were measured, and pubertal stage and bone age were determined. Growth was evaluated according to accepted curves for height and weight. Four of the girls had normal pubertal development, with serum FSH, LH, and E2 levels correlating to the phase of the menstrual cycle. Only one of the girls had ovulatory cycles (increase in P level). The girl treated for EST by abdominal irradiation had gonadal failure, with postmenopausal serum levels of FSH, LH, and E2. Her karyotype was normal. One of the girls was still prepubertal. None of them was hyperandrogenemic. One boy who was treated with bone marrow transplantation and total body irradiation had gonadal failure. One boy was still prepubertal, and the third boy showed normal pubertal maturation and normal serum FSH, LH, and T levels. All the patients except the boy treated with bone marrow transplantation and irradiation were normoprolactinemic; in addition, all had normal thyroid and adrenal function. Height and weight curves were normal in seven of the patients after the cancer therapy. The girl with EST had finished her growth before the irradiation therapy began. The boy treated with bone marrow transplantation and irradiation failed to exhibit further growth after beginning leukemia therapy at the age of 9.3 years.(ABSTRACT TRUNCATED AT 250 WORDS)
[Clinico-hormonal characteristics of retarded development of the female sexual system of central origin].
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[Induction of puberty by pulsatile luteinizing hormone releasing hormone (LH-RH)--therapy in a boy with Kallmann syndrome].
To induce the lacking development of puberty, a male patient with hypothalamic hypogonadism and anosmia (Kallmann's syndrome) was treated with pulsatile application of gonadotropin-releasing hormone (GnRH) to imitate the endogenous secretion of GnRH. The low-dose pulsatile GnRH treatment which was reported to be successful by various authors proved to be ineffective when administered to our patient subcutaneously as well as intravenously. Serum testosterone levels comparable to the lower normal values in adults and continuous progress of pubertal development were only achieved after increasing the dosage from 2 to 8 micrograms per pulse by subcutaneous application. The course of therapy is reported in detail.
[Hypoprolactinemia in subjects with various pathologies of the reproductive system].
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[Pulsatile long-term gonadotropin-releasing hormone (GnRH) therapy in the male: natural induction of puberty in a 20-year-old boy].
Successful induction of puberty by means of a pulsatile long-term therapy with GnRH--the variant closest to nature--can be performed on an outpatient basis without changing habits and lifestyle, as was demonstrated clinically and biochemically in a 20-year-old previously untreated boy with hypothalamic hypogonadism of unknown etiology. Unusual in this patient's course was the slow--and modest--increase in plasma FSH levels. Evidence that puberty did not occur spontaneously but was in fact due to treatment was gained from gonadotropin profiling by night and day before and after therapy withdrawal.
Gonadal failure in a short teenage girl.
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[Induction of puberty in Kallmann syndrome by pulsatile gonadotropin-releasing hormone stimulation].
A 23-year-old male, diagnosed as having hypogonadotropic hypogonadism associated with hyposmia (Kallmann syndrome), was given a trial of pulsatile Gn-RH therapy for 100 days. The therapy led to rhythmic secretion of LH and FSH by the anterior pituitary gland. Both the mean and the peak gonadotropin levels rose steadily. Following the 9th day of treatment the previously subnormal gonadotropin levels were found to be within the normal range. After 23 days a pulsatile response of the Leydig cells parallel to the rhythmic LH secretion was noticed. The testosterone level increased significantly and was 2.6 ng/ml 3 months after the initiation of the therapy. At the same time an increasing activity of androgen-dependent glands became apparent and secondary male sex characteristics were noted. The seminal volume rose from 0.1 ml to 2.0 ml, but after 3 months of treatment spermatozoa could still not be detected. In summary, pulsatile Gn-RH stimulation represents a good option in the treatment of hypothalamic hypogonadotropic hypogonadism as the therapy resembles physiological stimulation and is well tolerated by the patient.
[24-hour integrated concentration of LH, FSH, TSH and PRL in subjects with hypogonadotropic hypogonadism treated with pulsatile LHRH].
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Hormone responses to clomiphene citrate in young chimpanzees.
The responses of gonadotropin and gonadal steroids to the administration of clomiphene citrate were studied in male and female chimpanzees, aged 3.6 to 9.9 years. Follicle-stimulating hormone (FSH) was significantly reduced after treatment in the prepubertal females (n = 4) and in early pubertal males (n = 2) but not in prepubertal males (n = 5). FSH was unchanged or increased in early pubertal females (n = 2) and late pubertal males (n = 2). There was no consistent response to treatment with clomiphene citrate by luteinizing hormone (LH) in either males or females, nor by 17 beta-estradiol in the females. Testosterone levels were reduced in the early pubertal males only. These results support the hypothesis that negative feedback by gonadal steroids is operative in prepubertal chimpanzees and that puberty is accompanied by a reduction in the sensitivity to such feedback.
Anabolic steroid use in adolescence.
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Growth hormone: diagnostic and therapeutic dilemmas.
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Maturation factors in slipped capital femoral epiphysis.
Several maturation factors relative to growth and epiphyseal development were reviewed retrospectively in 191 patients with slipped capital femoral epiphysis, including bone age, height and weight, thyroid functions, sex hormone levels, and growth hormone levels. Seventy-one percent of 138 patients had weights above the 80th percentile. Active thyroid (T3) was significantly low in 25% of 80 patients studied. Testosterone levels were markedly depressed in 76% of 64 patients tested. In this same group, 87% had low growth hormone levels. The consistently low testosterone and growth hormone levels, along with a tendency toward hypothyroidism, lend support to the biochemical theory of a delicate hormonal imbalance in slipped capital femoral epiphysis.
[Health status of girl gymnasts].
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[Hypogonadotrophic hypogonadism and peripheral adrenal insufficiency in 2 brothers].
Two siblings with chronic adrenal insufficiency, diagnosed at 12 years of age, failed to show a spontaneous onset of puberty in spite of adequate adrenocortical therapy. Testosterone plasma levels were low, without concomitant increase in gonadotropin levels. This association strongly suggest a sex-linked cytomegalic adrenocortical hypoplasia.
[A contribution to the discussion on the subject of growth deficit. The case of a boy with suprasellar tumor].
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[Juvenile diabetes mellitus associated with hypothalamic hypothyroidism].
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