trelstar and Syndrome

Duplication of the pituitary gland (DPG)-plus syndrome is a very rare developmental disorder with few cases described in the literature and characterized by multiple midline and central nervous system malformations. The hypothalamus and hypophysis involvement may be clinically associated with endocrine abnormalities. A 5.9-year-old female child was admitted to our Clinic for premature thelarche and acceleration of growth. DPG-plus syndrome with paired infundibula and pituitary glands was diagnosed after birth, when she appeared small for gestational age and she presented with lingual hypoplasia, cleft palate, right choanal stenosis, nasopharyngeal teratoma, and facial dysmorphisms. Neuroimaging revealed a duplication of the infundibula, the pituitary gland, and the dens of the epistropheus despite surgical removal of a rhino-pharyngeal mass performed at the age of two months. An array-CGH revealed a 2p12 deletion. At our evaluation, bone age assessment resulted advanced and initial pubertal activation was confirmed by Gonadotropin-Releasing Hormone stimulation test. Hormonal suppression treatment was started with satisfactory results. This case shows that DPG-plus syndrome must be considered in presence of midline and craniofacial malformations and endocrinological evaluations should be performed for the prompt and appropriate management of pubertal anomalies.

Topics: Abnormalities, Multiple; Child; Craniofacial Abnormalities; Female; Humans; Magnetic Resonance Imaging; Pituitary Diseases; Pituitary Gland; Puberty, Precocious; Syndrome; Tomography, X-Ray Computed; Triptorelin Pamoate

The value of luteal phase supplementation with human chorionic gonadotropin (hCG) was assessed after a combined protocol of ovarian stimulation, using a long acting gonadotropin releasing hormone analog (GnRH-a) and human menopausal gonadotropins (hMG), in a randomized prospective study of 36 consecutive cycles in an in vitro fertilization (IVF) program. The patients were allocated on the transfer day to either luteal phase supplementation with hCG (Group A, n = 18) or none (Group B, n = 18). Nine patients of Group A conceived as compared with 3 in Group B. Five patients, all in Group A, developed ovarian hyperstimulation syndrome (OHSS) (3 moderate and 2 severe forms). Analysis of the hormonal profiles disclosed similar progesterone (P), estradiol (E2), and E2/P ratio up to the 6th post ovum pick-up day. Then, E2 and mainly P levels decreased only in Group B resulting in a rising E2/P ratio. These findings stress the importance of luteal support in IVF cycles treated with GnRH-a. In light of the increased risk of OHSS among hCG treated patients, further studies are needed to assess the optimal preparation needed.

Topics: Chorionic Gonadotropin; Embryo Transfer; Estradiol; Female; Fertilization in Vitro; Gonadotropin-Releasing Hormone; Humans; Menotropins; Menstruation; Ovarian Diseases; Ovary; Pregnancy; Progesterone; Prospective Studies; Syndrome; Triptorelin Pamoate

In a crossover study conducted over a six-month period in eight patients with well-characterized premenstrual syndrome, physical and behavioral symptoms were relieved by daily administration of an agonist of gonadotropin-releasing hormone. The reversible "medical ovariectomy" attained with this agonist suggests that it may be an effective and rational treatment for this distressing syndrome in the short term. Whether prolonged therapy would be safe and effective, or even necessary, remains to be determined.

Topics: Adult; Clinical Trials as Topic; Female; Gonadotropin-Releasing Hormone; Hormones; Humans; Menstruation; Premenstrual Syndrome; Syndrome; Triptorelin Pamoate

Topics: Adult; Chorionic Gonadotropin; Female; Follicle Stimulating Hormone; Gonadotropin-Releasing Hormone; Humans; Menopause; Ovarian Diseases; Ovarian Follicle; Ovulation; Syndrome; Triptorelin Pamoate

Moderate ovarian hyperstimulation syndrome occurred after LA was administered to control menorrhagia in an anephric woman who required hemodialysis. We postulate that women who require dialysis may be at special risk for the development of this syndrome.

Topics: Adult; Delayed-Action Preparations; Estradiol; Female; Follicle Stimulating Hormone; Gonadotropin-Releasing Hormone; Hormones; Humans; Kidney Failure, Chronic; Leuprolide; Menorrhagia; Ovarian Cysts; Ovary; Renal Dialysis; Syndrome; Triptorelin Pamoate

Topics: Chorionic Gonadotropin; Delayed-Action Preparations; Drug Therapy, Combination; Female; Fertilization in Vitro; Gonadotropin-Releasing Hormone; Humans; Ovarian Diseases; Ovary; Ovulation Induction; Syndrome; Triptorelin Pamoate

Severe ovarian hyperstimulation syndrome (OHSS) was recorded in 8 of 413 patients after the use of gonadotropin-releasing hormone agonists (GnRH-a) associated with gonadotropins for in vitro fertilization. Seven of the 8 patients were pregnant. Common factors associated with the development of OHSS were high serum estradiol values on the day of ovulation induction and many follicles greater than or equal to 12 mm. Based on this experience, a new therapeutic schedule was used in a group of 10 patients who, after GnRH-a and gonadotropin stimulation, were judged to be at high risk of OHSS on the day of human chorionic gonadotropin (hCG). No hCG was administered and gonadotropins were stopped. The administration of GnRH-a was continued and, after a further period of pituitary desensitization, follicular stimulation was recommended with a lower dose of gonadotropins. No cases of OHSS occurred and 3 patients became pregnant.

Topics: Buserelin; Estradiol; Europe; Female; Fertilization in Vitro; Gonadotropin-Releasing Hormone; Humans; Ovarian Diseases; Ovary; Pregnancy; Pregnancy Outcome; Risk Factors; Syndrome; Triptorelin Pamoate

In the present paper we examined, whether the combined GnRH-agonist/hMG therapy implies an increased risk of the ovarian hyperstimulation syndrome (OHS). In a retrospective analysis, 525 GnRH-a/hMG cycles were compared with 643 cycles of hMG stimulation, which were simultaneously performed at the Department of Gynecology and Obstetrics of the University of Hamburg. Two different GnRH-agonists were used: Buserelin (Hoechst) given intranasally (410 cycles) and Triptorelin (Ferring) intramuscularly (115 cycles). The clinical results of hMG "only"-therapy revealed an OHS incidence of 7% for grade II and 0.2% for grade III. In contrast, significantly higher incidences were observed after GnRH-a/hMG treatment. In Buserelin/hMG cycles in 23% OHS grade II and in 1.0% OHS grade III occurred, in Triptorelin/hMG cycles in 40% OHS II and in 5.2% OHS III, respectively. The increased incidence of OHS correlated with higher ovarian estrogen production as well as a higher number of follicles following the GnRH-a/hMG stimulation. Furthermore, in GnRH-a/hMG cycles a prolonged duration of follicular maturation occurred due to an increase of the active phase; in addition the amount of hMG-ampoules needed for ovarian stimulation was higher. After GnRH-a/hMG treatment, an endogenous LH-surge was not detected, whereas in 34% of hMG stimulated cycles irregular LH-fluctuations were observed. There was a higher pregnancy rate in GnRH-a/hMG cycles (15%/525 cycles), as compared to hMG stimulation (8%/643 cycles), but the abortion rate was similar (23%, GnRH-a/hMG, versus 13%, hMG). The demonstration of an increased ovarian response leading to better pregnancy rates but also higher risks of OHS is well known from earlier data of hMG stimulation in patients with hypogonadotropic amenorrhoea (WHO group I). This implies that GnRH-agonist pre-treatment shows similar endocrine conditions in normogonadotropic patients.

Topics: Buserelin; Dose-Response Relationship, Drug; Drug Therapy, Combination; Female; Fertilization in Vitro; Gonadotropin-Releasing Hormone; Humans; Infertility, Female; Menotropins; Ovarian Cysts; Ovary; Ovulation Induction; Pituitary Hormone-Releasing Hormones; Pregnancy; Risk Factors; Syndrome; Triptorelin Pamoate

In 143 cycles of in vitro fertilization the ovarian hyperstimulation syndrome (OHSS) occurred in 12 (8.4%) cycles. Six were in the moderate form and 6 severe. Ovarian stimulation by menotropins was preceded by induction of hypopituitary hypogonadism using D-Trp6-LH-RH microcapsules. The OHSS cycles are characterized by improved ovarian response expressed by the increased serum levels of estradiol, number of follicles, oocytes, embryos and pregnancy rate as compared to cycles with no OHSS. All patients recovered uneventfully. The follicular puncture did not have the suggested protective effect against OHSS. It is suggested that the substantial incidence of OHSS is probably related to the excessive ovarian stimulation not interrupted by early luteinization which is practically abolished by this protocol. The role of the given luteal hCG doses in the genesis of OHSS is questioned.

Topics: Adult; Capsules; Chorionic Gonadotropin; Estradiol; Female; Fertilization in Vitro; Gonadotropin-Releasing Hormone; Humans; Menotropins; Ovary; Pregnancy; Syndrome; Triptorelin Pamoate

Current medical and surgical therapies of precocious puberty in McCune-Albright syndrome are often unsatisfactory. We used an aromatase inhibitor, testolactone, to treat precocious puberty in a girl with McCune-Albright syndrome. This child was unresponsive to 28 weeks of treatment with the long-acting agonist of LRH, D-trp6-pro9-NEt-LRH. During testolactone therapy, menses ceased, bone age advancement and height velocity diminished, and plasma oestradiol levels were suppressed. Serum gonadotrophin levels remained in the prepubertal range. Testolactone may be an effective therapy of precocious puberty in girls with McCune-Albright syndrome.

Topics: Androstenedione; Child, Preschool; Endocrine System Diseases; Estradiol; Estrone; Female; Fibrous Dysplasia of Bone; Follicle Stimulating Hormone; Gonadotropin-Releasing Hormone; Humans; Luteinizing Hormone; Puberty, Precocious; Syndrome; Testolactone; Testosterone; Triptorelin Pamoate

Topics: Female; Gonadotropin-Releasing Hormone; Hormones; Humans; Premenstrual Syndrome; Syndrome; Triptorelin Pamoate