menotropins and Kallmann-Syndrome

Topics: Chorionic Gonadotropin; Diagnosis, Differential; Estrogens; Extracellular Matrix Proteins; Female; Gonadotropin-Releasing Hormone; Humans; Kallmann Syndrome; Male; Menotropins; Mutation; Nerve Tissue Proteins; Prognosis; Receptor, Fibroblast Growth Factor, Type 1; Testosterone

We report the case of a pregnancy in a patient with Kallmann's syndrome. The patient responded adequately to gonadotropin releasing hormone (GnRH) stimulation test, and after the third cycle of stimulation the patient became refractory to the treatment. The patient did not respond to exogenous follicle stimulating hormone (FSH) or human menopausal gonadotropins (hMG) for ovulation induction. For these reasons, we used a short course of leuprolide acetate (LUPRON) followed by hMG, which resulted in ovulation and a subsequent pregnancy which was carried to term. We present an alternate approach to ovulation induction in patients with Kallmann's syndrome and a review of the literature.

Topics: Female; Follicle Stimulating Hormone; Gonadotropin-Releasing Hormone; Humans; Infant, Newborn; Kallmann Syndrome; Leuprolide; Menotropins; Ovulation; Ovulation Induction; Pregnancy; Pregnancy Outcome

Topics: Adult; Chorionic Gonadotropin; Drug Therapy, Combination; Female; Follicular Phase; Humans; Infertility, Female; Kallmann Syndrome; Menotropins; Ovulation Induction; Pregnancy; Pregnancy, Twin

The Kallmann syndrome is a very rare congenital association of gonadotropin-releasing hormone deficiency and hyposmia or anosmia. Clinically it is characterized by low serum concentrations of testosterone and inadequate low levels of luteinizing hormone and follicle-stimulating hormone as well as incomplete sexual maturation, lack of secondary sexual features (facial and body hair growth, deepening of the voice), micropenis and sometimes even cryptorchidism. The reduced or absent sense of smell is typical for the Kallmann syndrome and distinguishes this syndrome from other causes of hypogonadotropic hypogonadism. Additional findings may include synkinesia, hearing loss, unilateral renal aplasia, brachy- or syndactyly, agenesis of corpus callosum, cleft palate and dental agenesis. A 19-year-old man presented to our male infertility clinic with delayed sexual maturation, eunuchoid habitus, micropenis, cryptorchidism, erectile dysfunction and absence of ejaculation, anemia and osteoporosis as well as low serum concentrations of luteinizing hormone, follicle-stimulating hormone and testosterone in combination with hyposmia.

Topics: Cholecalciferol; Chorionic Gonadotropin; Diagnosis, Differential; Drug Therapy, Combination; Follicle Stimulating Hormone; Humans; Infertility, Male; Inhibin-beta Subunits; Kallmann Syndrome; Luteinizing Hormone; Male; Menotropins; Testosterone; Vitamin D Deficiency; Young Adult

We report a case of Kallmann syndrome in which the aging male's symptoms rating scale (AMS) was useful for assessment of subjective symptoms. A 30-year-old male was admitted to Tsukuba University Hospital with a complaint of delayed puberty in May 2003. He presented with hypogonadism, gynecomastia and anosmia. The plasma levels of luteinizing hormone (LH), follicle stimulating hormone (FSH) and testosterone were very low. However, the LH-releasing hormone test and human chorionic gonadotropin (hCG) loading test identified normal function of pituitary and Leydig cell. After 12 months of treatment with hCG and human menopausal gonadotropin (hMG), the amount of pubic hair and the volume of testes had increased as well as the level of serum testosterone. The total score of AMS after treatment has been improved from 39 to 20 as compared with that before treatment.

Topics: Adult; Chorionic Gonadotropin; Humans; Kallmann Syndrome; Male; Menotropins; Penile Erection

A 25-year-old woman, diagnosed with Kallmann's syndrome and wanting to become pregnant, visited our hospital. Because her serum gonadotropin levels indicated hypogonadotropic hypogonadism, a main symptom of Kallmann's syndrome, we attempted to induce ovulation using a low-dose human menopausal gonadotropin (hMG) step-up protocol. In this protocol, 75 IU of hMG was used as an initial dose and this was continued for the first 14 days because adequate follicular development was not achieved. The dose of hMG was subsequently increased to 150 IU for the next 7 days. After 22 days from the start of stimulation, two follicles had developed, and were ovulated using an injection of human chorionic gonadotropin. She became pregnant, and her pregnancy was uneventful during the first trimester; however, in the second trimester both uterine contractions and blood pressure could not be controlled, and at 27 weeks' gestation she delivered a male infant weighing 830 g by cesarean section.

Topics: Adult; Cesarean Section; Female; Gestational Age; Humans; Infant, Newborn; Infant, Premature; Infertility, Female; Kallmann Syndrome; Male; Menotropins; Obstetric Labor, Premature; Ovulation Induction; Pregnancy

Hypogonadotropic hypogonadism (HH) is an uncommon cause of male infertility. Even specialized centers see small numbers of patients and have little experience in restoring fertility in these patients. To explore the phenotypic heterogeneity and treatment outcome of HH, we summarized our 30-year experience.. Medical charts of 36 male patients (age range 11 to 42 years) with HH (81% primary, 19% secondary) treated at university affiliated male infertility centers were reviewed. Pubic hair stage, genital stage, testicular volume (TV), sexual function and sperm production were determined before and after human chorionic gonadotropin/human menopausal gonadotropin (hCG/hMG) treatment. Differences were analyzed statistically.. The population was stratified according to initial TV into a small testis subset (TV less than 4 ml in 23) and a large testis subset (TV 4 ml or greater in 13). Comparison of these 2 subsets revealed significant differences in the incidence of cryptorchidism (39% vs 8%) and Kallmann's syndrome (22% vs 0%), pubic hair stage (1.3 +/- 0.5 vs 3.5 +/- 1.4), genital stage (1.2 +/- 0.4 vs 3.9 +/- 0.9) and TV (1.6 +/- 0.9 vs 7.5 +/- 3.5 ml). Long-term administration of hCG/hMG for 12 to 240 months (average 56 +/- 11) resulted in sperm production in only 36% of the small testis subjects but in 71% of the large testis subjects.. Initial TV values provide insight into phenotypic variables such as time of onset and severity in patients with primary or secondary HH, and may predict sperm output in response to hCG/hMG therapy.

Topics: Adolescent; Adult; Child; Chorionic Gonadotropin; Genetic Heterogeneity; Humans; Infertility, Male; Kallmann Syndrome; Long-Term Care; Male; Menotropins; Organ Size; Phenotype; Retrospective Studies; Sexual Maturation; Spermatogenesis; Testis; Treatment Outcome

Kallmann syndrome is a very rare hereditary disease. It is characterized by hypogonadotropic hypogonadism in association with anosmia ot hyposmia, both of which occur as a result of the failure of neuronal migration of the luteinizing hormone releasing hormone (LHRH)--secreting neurons and the neurons of the vemeronasal nerve. It can be autosomal dominant, autosomal recessive, or X-linked mode of inheritance. We report a case of Kallmann syndrome that presented with delay puberty, color blindness, gynecomastia, and absence of smell. Plasma levels of LH, FSH and testosterone were very low. The patient's adrenal and thyroid hormone levels were normal. Chromosome analysis showed 46, XY karyotype without deletion in KAL gene (Xp22.3) from FISH. After 9 months of treatment by HCG and HMG, the amount of pubic hair and the volume of bilateral testes, as well as the level of testosterone had increased. Most importantly, motile sperm count be found in semen.

Topics: Adult; Chorionic Gonadotropin; Humans; Kallmann Syndrome; Male; Menotropins; Sexual Maturation

Kallmann syndrome is characterized by hypogonadotropic hypogonadism and anosmia. We observed the improvement of gonadal failure upon exposure to gonadotropin, but not gonadotropin-releasing hormone, in the female patients.

Topics: Adolescent; Female; Fertility Agents, Female; Gonadotropin-Releasing Hormone; Gonadotropins; Humans; Kallmann Syndrome; Menotropins

Topics: Adult; Chorionic Gonadotropin; Fertility; Follicle Stimulating Hormone; Gonadotropin-Releasing Hormone; Humans; Kallmann Syndrome; Luteinizing Hormone; Male; Menotropins; Psychotherapy

Topics: Adult; Estrogens; Female; Follicle Stimulating Hormone; Humans; Kallmann Syndrome; Luteinizing Hormone; Menotropins; Ovulation Induction; Recombinant Proteins

This case report describes the use of GIFT to achieve pregnancy for a man with Kallmann's syndrome who obtained only marginal sperm counts with both the pulsatile GnRH infusion pump and gonadotropin injections. Failure of this man to achieve a pregnancy with hormonal therapy alone and in combination with IUI suggests that assisted reproductive technologies should be considered in male patients with Kallmann's syndrome when suboptimal sperm concentrations are achieved despite exogenous hormonal stimulation.

Topics: Adult; Chorionic Gonadotropin; Female; Gamete Intrafallopian Transfer; Humans; Infertility, Male; Insemination, Artificial, Homologous; Kallmann Syndrome; Male; Menotropins; Pregnancy; Superovulation

A successful zygote intra-Fallopian transfer has been performed using spermatozoa from a patient with Kallmann's syndrome. Spermatogenesis was induced by hormonal treatment but even so, semen quality remained slightly impaired and no pregnancy occurred over 4 years, despite ovulation induction in the patient's spouse. Finally she conceived after assisted procreation by in-vitro fertilization and delivered a healthy baby.

Topics: Adult; Chorionic Gonadotropin; Female; Gonadotropin-Releasing Hormone; Humans; Infertility, Male; Kallmann Syndrome; Male; Menotropins; Pregnancy; Sperm Count; Sperm Motility; Spermatogenesis; Zygote Intrafallopian Transfer