pituitrin and Dwarfism--Pituitary

Topics: Adolescent; Blood Glucose; Child; Diabetes Insipidus; Dwarfism, Pituitary; Growth Hormone; Humans; Infant, Newborn; Insulin; Kidney Tubules; Osmolar Concentration; Radioimmunoassay; RNA; RNA Nucleotidyltransferases; Thyroid Function Tests; Vasopressins

Topics: Adrenal Cortex Hormones; Adrenal Gland Diseases; Adrenal Hyperplasia, Congenital; Child; Dwarfism, Pituitary; Endocrine System Diseases; Humans; Metabolism, Inborn Errors; Mixed Function Oxygenases; Pedigree; Pituitary Hormones, Anterior; Thyroid Diseases; Thyroid Hormones; Vasopressins

Children initially diagnosed with isolated GH deficiency (IGHD) have a variable rate to progress to combined pituitary hormone deficiency (CPHD) during follow-up.. To evaluate the development of CPHD in a group of childhood-onset IGHD followed at a single tertiary center over a long period of time.. We retrospectively analyzed data from 83 patients initially diagnosed as IGHD with a mean follow-up of 15.2 years. The Kaplan-Meier method and Cox regression analysis was used to estimate the temporal progression and to identify risk factors to development of CPHD over time.. From 83 patients initially with IGHD, 37 (45%) developed CPHD after a median time of follow up of 5.4 years (range from 1.2 to 21 years). LH and FSH deficiencies were the most common pituitary hormone (38%) deficiencies developed followed by TSH (31%), ACTH (12%) and ADH deficiency (5%). ADH deficiency (3.1 ± 1 years from GHD diagnosis) presented earlier and ACTH deficiency (9.3 ± 3.5 years) presented later during follow up compared to LH/FSH (8.3 ± 4 years) and TSH (7.5 ± 5.6 years) deficiencies. In a Cox regression model, pituitary stalk abnormalities was the strongest risk factor for the development of CPHD (hazard ratio of 3.28; p = 0.002).. Our study indicated a high frequency of development of CPHD in patients initially diagnosed as IGHD at childhood. Half of our patients with IGHD developed the second hormone deficiency after 5 years of diagnosis, reinforcing the need for lifelong monitoring of pituitary function in these patients.

Topics: Adolescent; Adrenocorticotropic Hormone; Adult; Child; Child, Preschool; Cohort Studies; Disease Progression; Dwarfism, Pituitary; Female; Follicle Stimulating Hormone; Follow-Up Studies; Human Growth Hormone; Humans; Hypopituitarism; Hypothalamus; Kaplan-Meier Estimate; Longitudinal Studies; Luteinizing Hormone; Magnetic Resonance Imaging; Male; Pituitary Gland; Proportional Hazards Models; Retrospective Studies; Thyrotropin; Time Factors; Vasopressins; Young Adult

Topics: Adult; Amenorrhea; Diabetes Insipidus; Dwarfism, Pituitary; Female; Gonadotropins; Humans; Infertility, Female; Insemination, Artificial; Pregnancy; Pregnancy Outcome; Vasopressins

Magnetic resonance (MR) imaging was performed in ten patients with pituitary stalk transection who had idiopathic pituitary dwarfism. Contiguous sagittal T1-weighted images were obtained in all cases, and, in some, axial or coronal images were taken for further evaluation. On MR images, normal anterior and posterior lobes of the pituitary gland can be clearly differentiated because the posterior lobe has a characteristic high intensity on T1-weighted images. In the ten patients, the high-intensity posterior lobe was not seen, but a similar high signal intensity was observed at the proximal stump in seven patients. This high-intensity area is the newly formed ectopic posterior lobe, which secretes antidiuretic hormone just as the posterior lobe would. When the ectopic lobe completely compensates for the impaired posterior lobe, endocrinologic data indicate normal posterior lobe function. However, MR imaging can reveal the transection of the pituitary stalk and formation of the ectopic lobe.

Topics: Adolescent; Birth Injuries; Child; Child, Preschool; Dwarfism, Pituitary; Female; Humans; Magnetic Resonance Imaging; Male; Obstetric Labor Complications; Pituitary Gland, Anterior; Pituitary Gland, Posterior; Pregnancy; Vasopressins

Six children with human growth hormone (hGH) deficiency became hypothyroid during the course of their therapy with hGH. This was accompanied by a decreasing growth rate, clinical symptoms of hypothyroidism and decreased serum T4 concentrations. Three of the 6 patients returned to the euthyroid state, both clinically and biochemically, with cessation of hGH therapy, and reinstitution of hGH precipitated hypothyroidism again in 2 of the three. The patients who remained hypothyroid have evidence of multiple pituitary trophic hormone deficiencies while those who reverted to euthyroidism appear to have isolated hGH deficiency. Evaluation of thyroid function while on hGH showed low T4, free T4 and T3 concentrations. The serum thyrotropin (TSH) response to thyrotropin-releasing hormone (TRH) was absent or markedly blunted in 4 of 6 patients while receiving long-term hGH therapy but was normal or exaggerated in all patients when tested before or after only 5 days of hGH therapy. These data indicate that exogenous hGH results in an inhibition of the TSH response to TRH. The mechanism of this inhibition is unclear, but we postulate that it may be mediated by somatostatin secretion in response to pulse doses of hGH.

Topics: Age Determination by Skeleton; Body Height; Craniopharyngioma; Diabetes Insipidus; Dwarfism, Pituitary; Female; Growth; Growth Hormone; Humans; Hypopituitarism; Hypothyroidism; Male; Thyroid Gland; Thyroxine; Vasopressins

Topics: Acromegaly; Addison Disease; Adrenalectomy; Blood Glucose; Cushing Syndrome; Diabetes Insipidus; Dwarfism; Dwarfism, Pituitary; Female; Glucagon; Growth Hormone; Humans; Hyperthyroidism; Hypogonadism; Hypopituitarism; Insulin; Lysine; Male; Pyrogens; Radioimmunoassay; Vasopressins

A 29-year-old woman with evidence of a craniopharyngioma and documented panhypopituitarism is described. Clinical and laboratory evaluation revealed deficiencies of follicle-stimulating hormone, luteinizing hormone, thyroid-stimulating hormone, growth hormone, prolactin, adrenocorticotropic hormone and antidiuretic hormone. Prompt release of several pituitary hormones was noticed after administration of the hypothalamic releasing hormones FSH/LH-RF and thyrotropin-releasing hormone, whereas insulin-induced hypoglycemia, levodopa, chlorpromazine and clomiphene citrate, all of which act at the level of the hypothalamus, did not alter basal pituitary secretion. The patient's height of 60 inches, despite panhypopituitarism, and the interpretation of the above data are discussed in the light of current concepts regarding the dynamics of the hypothalamic-hypophyseal system.

Topics: Adolescent; Adrenocorticotropic Hormone; Adult; Child; Chlorpromazine; Clomiphene; Craniopharyngioma; Dwarfism, Pituitary; Female; Follicle Stimulating Hormone; Gonadotropin-Releasing Hormone; Growth Hormone; Humans; Hypopituitarism; Hypothalamo-Hypophyseal System; Insulin; Levodopa; Luteinizing Hormone; Pituitary Function Tests; Pituitary Gland; Pituitary Hormones; Prolactin; Thyrotropin; Thyrotropin-Releasing Hormone; Vasopressins

Topics: Agenesis of Corpus Callosum; Cerebral Ventriculography; Child; Child, Preschool; Diabetes Insipidus; Drinking Behavior; Dwarfism, Pituitary; Electroencephalography; Epilepsy; Humans; Hypothermia; Male; Puberty, Precocious; Recurrence; Vasopressins

Topics: Adolescent; Adrenocorticotropic Hormone; Anesthesia, General; Birth Injuries; Central Nervous System; Child; Child, Preschool; Dwarfism, Pituitary; Female; Humans; Hydrocortisone; Hypoglycemia; Insulin; Male; Metyrapone; Pituitary-Adrenal Function Tests; Vasopressins

Topics: Adolescent; Bone Development; Child; Cortisone; Dwarfism; Dwarfism, Pituitary; Growth; Growth Hormone; Humans; Hypogonadism; Ketones; Metyrapone; Mineralocorticoid Receptor Antagonists; Pituitary Diseases; Pituitary-Adrenal Function Tests; Statistics as Topic; Thyroid Function Tests; Thyroid Hormones; Vasopressins