A 45-year-old woman who complained of weight gain and irregular menstruation was diagnosed as having Cushing's syndrome due to a 3 cm sized left adrenal adenoma. She underwent left adrenalectomy, and she also underwent combined anterior pituitary tests before and 9 months after the surgery. The growth hormone and adrenocorticotropic hormone levels failed to respond to hypoglycemia before the surgery, but their responses recovered after the surgery. Cortisol and thyroid stimulating hormone failed to respond to hypoglycemia and thyrotropin releasing hormone (TRH) before the surgery, respectively, but these were improved after the surgery. Luteinizing hormone, follicle stimulating hormone, and prolactin adequately responded to gonadotropin-releasing hormone and TRH, respectively, before and after the surgery. However, the basal levels of these hormones were higher after adrenalectomy, suggesting that hypercortisolemia had a significant influence on all the pituitary hormones.
Adenoma* ; Adrenalectomy ; Adrenocorticotropic Hormone ; Cushing Syndrome* ; Female ; Follicle Stimulating Hormone ; Gonadotropin-Releasing Hormone ; Growth Hormone ; Humans ; Hydrocortisone ; Hypoglycemia ; Hypopituitarism ; Luteinizing Hormone ; Menstruation ; Middle Aged ; Pituitary Hormones ; Prolactin ; Thyrotropin ; Thyrotropin-Releasing Hormone ; Weight Gain

BACKGROUND: ackground: Sheehan's syndrome secondary to severe postpartum hemorrhage is one of the major causes of pituitary insufficiency in Korea. Most of these patients do not manifest symptoms or signs of gross endocrinopathies. Earlier detection of pituitary insufficiency is of clinical importance. The combined pituitary stimulation test that uses the four hypothalamic releasing hormones is a rapid, safe, and effective way to evaluate anterior pituitary function. However, the criteria for a normal response has not been established in Korea. METHODS: Combined anterior pituitary stimulation tests were performed on fourteen healthy women who had no history of endocrine disease. Combined tests of anterior pituitary reserve were done no forty-five patients who suffered from massive postpartum hemorrhage which required transfusing, along with subsequent shock or changing consciousness and in thirty-nine patients who experienced mild postpartum hemorrhage. RESULTS: 1) In the severe hemorrhage group, thirty-three of forty-five women (73.3%) showed blunted responses in more than one of the anterior pituitary hormones in the combined pituitary stimulation tests. However, in the mild hemorrhage group, only eighteen of thirty-nine women (46.2%) demonstrated blunted responses of more than one of the anterior pituitary hormones. 2) In the severe hemorrhage group, the TSH response was blunted in twenty-five patients (55.6%), prolactin in eleven patients (24.4%), ACTH in ten patients (22.2%), LH in ten patients (22.2%), GH in nine patients (20%), and FSH in five patients (11.1%). 3) The results of combined pituitary stimulation tests in the normal control group were different from the results of other studies. CONCLUSION: It is recommended that the women who experienced a severe postpartum hemorrhage should be evaluated by using the combined pituitary stimulation test. Moreover, criteria for a normal response to the combined pituitary stimulation test should be established in Korea.
Adrenocorticotropic Hormone ; Consciousness ; Endocrine System Diseases ; Female ; Hemorrhage ; Humans ; Hypopituitarism ; Korea ; Pituitary Hormone-Releasing Hormones ; Pituitary Hormones, Anterior ; Postpartum Hemorrhage* ; Postpartum Period* ; Prolactin ; Shock

BACKGROUND: GH3 cells are a well characterized and widely used model used for the in vitro study of growth hormone (GH) secretion. Thyrotropin releasing hormone (TRH) binds to receptors belonging to the family of G protein-coupled receptors, and secrets both GH & prolactin. Phospholipase D (PLD) is an enzyme that hydrolyses phosphatidylcholine to yield phosphatidic acid and choline, and plays important roles in cellular proliferation and hormonal secretion. To elucidate the pathway of the action of TRH in GH3 cells, we investigated the activities of PLC and PLD in GH3 cells treated with TRH or phorbor 12-myristate 13-acetate (PMA). METHODS: GH3 cells were labeled with [3H] myristate, followed by incubation of with 0.3% ethanol, prior to before the addition of the agonists. The total lipids were extracted from the harvested cells following treatment with the agonists. The PLD activity was assessed by measuring [3H] phosphatidylethanol from the [3H] phospholipid using thin layer chromatography. RESULTS: TRH (1 muM) stimulated the PLC activity by 44-fold over that of the control values. TRH (1 microM), mastoparan (5 muM), and PMA (500 muM) for 30 minutes increased PLD activity by 1.9, 1.5 and 2.2 fold, respectively, in comparison to the controls. The PLD activities after 15, 30, 60, 120 and 240 min treatments of TRH (1 microM) were 142%, 170%, 172%, 160% and 115%, respectively. CONCLUSION: These results suggest that TRH stimulates not only the PLC activity, but also the PLD activity in GH3 cells.
Cell Proliferation ; Choline ; Chromatography, Thin Layer ; Ethanol ; Growth Hormone ; Humans ; Myristic Acid ; Phosphatidic Acids ; Phosphatidylcholines ; Phospholipase D* ; Phospholipases* ; Prolactin ; Thyrotropin-Releasing Hormone

Normal and abnormal follicular growth and steroidogenesis depend on gonadotropins as well as intraovarian peptides, which may mediate or potentiate gonadotropin action. Inhibin also affect follicular development and steroidogenesis and may play a role in dominant follicle selection and follicular atresia. Therefore, we studied the differences of serum inhibin, gonadotropin and androgen levels in the women with only the ultrasound findings and no disorder, and polycystic ovary (PCO) with ovulatory disturbance. We prospectively analysed forty-three women with PCO. The diagnosis of PCO was based on typical appearance of the ovaries on TVS. Twelve women with regular menstrual cycle and normal ovarian morphology were selected as control. Basal levels of inhibin, luteinizing hormone(LH), follicle stimulating hormone(FSH), estradiol(E2), testosterone(T), androstenedione(ADD), dehydroepiandrosterone-sulfate(DS), prolactin and TSH in serum were determined. There were significant differences in basal LH levels and LH/FSH ratio between the control and the women with PCO. The basal levels of inhibin and E2 in the oligo-amenorrheic PCO (N=34) were significantly higher than those in the control. There was higher negative correlation between the inhibin and T levels in the oligo-amenorrheic PCO, but, not in the regular cycling PCO. Also, there was higher positive correlation between the LH and T levels in the oligo-amenorrheic PCO, but not in the regular cycling PCO. These data presume that the initial event of PCO is elevated pituitary LH secretion. Elevated levels of LH may down-regulate LH receptors on granulosa cells and also cause hypertrophy of the thecal layer. High level of androgen secreted by the hypertrophied thermal layer may stimulate inhibin secretion from granulosa cells and can be converted to estrogen by extraovarian tissues and could serve to augment pituitary sensitivity to GnRH with a resultant secretion of more LH follicular development and dominant follicle selection resulted in ovulatory disturbance.
Diagnosis ; Estrogens ; Female ; Follicular Atresia ; Gonadotropin-Releasing Hormone ; Gonadotropins ; Granulosa Cells ; Humans ; Hypertrophy ; Inhibins* ; Lutein ; Menstrual Cycle ; Ovary* ; Peptides ; Prolactin ; Prospective Studies ; Receptors, LH ; Ultrasonography

Congenital central hypothyroidism (C-CH) is a rare disease in which thyroid hormone deficiency is caused by insufficient thyrotropin (TSH) stimulation of a normally-located thyroid gland. Most patients with C-CH have low free thyroxine levels and inappropriately low or normal TSH levels, although a few have slightly elevated TSH levels. Autosomal recessive TSH deficiency and thyrotropin-releasing hormone receptor-inactivating mutations are known to be genetic causes of C-CH presenting in the absence of other syndromes. Recently, deficiency of the immunoglobulin superfamily member 1 (IGSF1) has also been demonstrated to cause C-CH. IGSF1 is a plasma membrane glycoprotein highly expressed in the pituitary. Its physiological role in humans remains unknown. IGSF1 deficiency causes TSH deficiency, leading to hypothyroidism. In addition, approximately 60% of patients also suffer a prolactin deficiency. Moreover, macroorchidism and delayed puberty are characteristic features. Thus, although the precise pathophysiology of IGSF1 deficiency is not established, IGSF1 is considered to be a new factor controlling growth and puberty in children.
Adolescent ; Cell Membrane ; Child ; Glycoproteins ; Humans ; Hypothyroidism* ; Immunoglobulins ; Infant, Newborn ; Neonatal Screening* ; Prolactin ; Puberty ; Puberty, Delayed ; Rare Diseases ; Thyroid Gland ; Thyrotropin ; Thyrotropin-Releasing Hormone ; Thyroxine

We experienced one case of severe pituitary dwarfism in a 16 years old male boy that magnetic resonance image(MRI) revealed transection of the pituitary stalk with the ectopic location of posterior pituitary gland and hypoplastic anterior pituitary gland. The serum growth horrnone(GH) response to clonidine and L-dopa revealed severe GH deficiency. The patient revealed normal response in TSH, prolactin and LH, FSH to TRH and LHRH respectively. So far, the primary cause of idiopathic pituitary dwarfism in many patients was due to hypoxic injury to hypothalmus by perinatal insults. In this patients, there was no history of perinatal insults and postnatal head trauma. We report a case of severe dwarfism due to hypogenesis of anterior pituitary gland.
Clonidine ; Craniocerebral Trauma ; Dwarfism ; Dwarfism, Pituitary ; Gonadotropin-Releasing Hormone ; Humans ; Levodopa ; Male ; Pituitary Gland ; Pituitary Gland, Anterior ; Pituitary Gland, Posterior ; Prolactin

OBJECTIVETo investigate the incidence of abnormal karyotypes and Y chromosome microdeletion in Chinese men with azoospermia, and the relationship with reproductive hormones.

METHODSFour hundred and eighty nine cases of azoospermic patients and 20 fertile men were studied. Karyotypes and Y chromosome microdeletion were analyzed by G-banding and mutiplex polymerase chain reaction, respectively. Chemiluminescene immunoassay technique was applied to measure the serum levels of follicle-stimulating hormone (FSH), luteinizing hormone (LH), testosterone (T), and prolactine (PRL).

RESULTSChromosome abnormalities were found in 102 out of 489 azoospermic patients (20.86%), among them 86 (84.31%) cases had sex chromosome abnormalities, with 73 cases being Klinefelter syndrome. Y chromosome microdeletions were detected in 58 (11.86%) cases out of the 489 patients, and deletion of the AZFc region was the leading group (63.8% of all deletions), followed by AZFbc (19.0%), AZFabc (10.3%), AZFb or AZFa (3.4%). FSH, LH levels were significantly increased and T level was decreased in azoospermic patients compared with the fertile men group (P<0.01). Furthermore, in the azoospermic patients with Klinefelter syndrome or AZFabc microdeletions, FSH and LH levels were increased more significantly, and were statistically different from azoospermic patients with normal karotype or without Y chromosome microdeletion (P<0.05).

CONCLUSIONIn the Chinese men with azoospermia, the incidence of abnormal karyotype and Y chromosome microdeletion were similar to those described previously in other populations. In azoospermia with Klinefelter syndrome or AZFabc microdeletions, FSH and LH levels increased markedly indicating the protracted stimulation of gonadotrophs due to lack of androgen feedback.

Adult ; Azoospermia ; blood ; genetics ; Case-Control Studies ; Chromosomes, Human, Y ; genetics ; Follicle Stimulating Hormone ; blood ; Genetic Association Studies ; Genetic Loci ; Humans ; Karyotyping ; Luteinizing Hormone ; blood ; Male ; Prolactin-Releasing Hormone ; blood ; Seminal Plasma Proteins ; genetics ; Sequence Deletion ; Testosterone ; blood

We experienced one case of severe pituitary dwarfism in a 10 years old female girl. Magnetic resonance image (MRI) revealed transection of the pituitary stalk stalk with the formation of high intensity ectopic posterior lobe located at the median eminence and agenesis of an anterior lobe of pituitary gland. The serum growth Hormone (GH) response to clonidine and L-dopa revealed severe GH deficiency. The patient had responses to TRH, normal TSH and partial prolactin response, respectively. There was not response LH and FSH to GnRH. The morning cortisol concentration and serum T4 concentration were decreased below the normal range. These findings and no hyperprolactinemia suggested the presence of a vascular connection between the pituitary gland and hypothalamus, which is not visible on MRI. Sofar, the primary cause of idiopathic pituitary dwarfism in many patients is injury to hypothalamus by perinatal insults. In this patient, there was no history of perinatal insults and postnatal head trauma but transection of the pituitary stalk. We report a case of severe pituitary dwarfism due to agenesis with brief review of related litereature.
Child ; Clonidine ; Craniocerebral Trauma ; Dwarfism, Pituitary* ; Female ; Gonadotropin-Releasing Hormone ; Growth Hormone ; Humans ; Hydrocortisone ; Hyperprolactinemia ; Hypothalamus ; Levodopa ; Magnetic Resonance Imaging ; Median Eminence ; Pituitary Gland* ; Pituitary Gland, Anterior* ; Prolactin ; Reference Values

Adolescence is a transition period from childhood to adulthood, and many physical, cognitive, and psychosocial changes are taken place in adolescence. Endocrinologic changes are usually associated with puberty, and play a central role in accomplishing the developmental task of adolescence. During puberty, secretion of gonadotropins, gonadal steroids, growth hormone, insulin like growth factor-1 and inhibin are increased. Insulin resistance is transiently increased in puberty. Gender differences of some hormones, such as testosterone-binding globulin, prolactin, prostate specific antigen, leptin and adiponectin, appear during puberty. The activation of hypothalamic gonadotropin-releasing hormone pulse generator initiates and regulates the reactivation of hypothalamic-pituitary-gonadal axis at puberty. The adolescent growth spurt in normal girls and boys depends on both estradiol and growth hormone. In the male as well as the female, estrogen (not androgen) is the critical sex hormone in the pubertal growth spurt, skeletal maturation, and the accrual of peak bone mass.
Adiponectin ; Adolescent ; Axis, Cervical Vertebra ; Estradiol ; Estrogens ; Female ; Gonadotropin-Releasing Hormone ; Gonadotropins ; Gonads ; Growth Hormone ; Humans ; Hypogonadism ; Inhibins ; Insulin ; Insulin Resistance ; Leptin ; Male ; Mitochondrial Diseases ; Ophthalmoplegia ; Prolactin ; Prostate-Specific Antigen ; Puberty ; Steroids

Methylphenidate (MPH) is the most preferred drug for treatment of the attention deficit hyperactivity disorder (ADHD). Here, we aimed to discuss the possible effects and mechanisms of MPH on precocious puberty (PP) via a case series with seven children who had normal body mass index. In this case series we evaluated seven children with ADHD, who had received MPH for at least 6 months (0.5 mg/kg/dose three times a day, maximum 60 mg) and admitted to Department of Pediatric Endocrinology with PP symptoms. The mean age was 8.16 years. Basal hormonal levels (luteinizing hormone [LH], follicle stimulating hormone, and estrogen/testosterone) were within normal range. Results of LH-releasing hormone stimulation tests demonstrated central pubertal responses. Glutamine, dopamine and noradrenaline are most important excitatory neurotransmitters that have a role at the beginning of puberty. The effect of MPH, cumulating dopamine and noradrenaline in the synaptic gap could be associated with the acceleration of puberty with the excitatory effect of dopamine’s gonadotropin-releasing hormone (GnRH) release, excitatory effect of noradrenaline’s GnRH release and the disappearance of GnRH receptor expression suppressor effect on prolactin disinhibitory effect.
Acceleration ; Adolescent ; Attention Deficit Disorder with Hyperactivity ; Body Mass Index ; Child ; Dopamine ; Endocrinology ; Follicle Stimulating Hormone ; Glutamine ; Gonadotropin-Releasing Hormone ; Humans ; Methylphenidate ; Neurotransmitter Agents ; Norepinephrine ; Prolactin ; Puberty ; Puberty, Precocious ; Receptors, LHRH ; Reference Values