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PubMed1975

The response of pituitary gonadotropes to synthetic LRF in children with glucocorticoid-treated congenital adrenal hyperplasia: lack of effect of intrauterine and neonatal androgen excess.

Previous studies have demonstrated that the secretory reserve of the pituitary gland during childhood and adolescence is characterized by an age-related increase in LH secretion and a sex dichotomy of FSH secretion evoked by LRF. To determine if prenatal and neonatal androgen excess alters hypothalamic-pituitary-gonadal function, as in the neonatally androgenized rodent, we assessed pituitary sensitivity to 100 mug synthetic LRF in 9 girls and 2 boys with glucorticoid-treated congenital virilizing adrenal hyperplasia (CAH). In the 7 prepubertal girls with CAH, plasma LH following LRF rose to 2.0 plus or minus 0.4 (SE) ng/ml (LER 960) and did not differ from normal prepubertal children (1.7 plus or minus 0.1) and was lower (P less than 0.002) than in normal pubertal children (4.9 plus or minus 0.3). The 2 pubertal girls had LH rises in the pubertal range. In the 2 boys with CAH, the one with the more advanced bone age, 11-6/12 yr, developed true precocious puberty following initiation of cortisone treatment and had a pubertal LRF-evoked LH release on 3 occasions; the more immature boy, bone age 10, had a prepubertal LH rise. In the 7 prepubertal girls with CAH, plasma FSH rose to 8.1 plus or minus 1.6 (SE) ng/ml (LER 869), which did not differ from normal prepubertal girls (5.3 plus or minus 1.9), but was significantly (P less than 0.001) greater than in normal prepubertal boys (2.9 plus or minus 0.4). In these girls with CAH, basal plasma T (27.4 plus or minus 4.0 (SE) ng/dl) and 17-OHP (2703 plus or minus 1143 (SE) ng/dl) were greater than in prepubertal children, but plasma E1 and E2 were in the normal range. The pubertal boy had plasma T, E1, and E2 levels appropriate for his degree of sexual maturation. The results suggest: (1) age-related LH secretion evoked by LRF in glucocorticoid-treated CAH is appropriate for skeletal maturation; (2) the sex dichotomy of FSH secretion following LRF is preserved in CAH despite intrauterine and variable postnatal exposure to excess androgen and continuing slightly elevated plasma T during childhood; and (3) precocious puberty may follow initiation of cortisone treatment in a child with CAH whose bone age and secretory reserve of pituitary gonadotropins are in the pubertal range.

Adolescent↗

PubMed1975

Assessment of pituitary gonadotropin reserve using luteinizing hormone-releasing hormone (LRH) in states of altered thyroid function.

Menstrual irregularities commonly accompany hyper- and hypofunction of the thyroid gland. Investigation of the pituitary-ovarian axis was undertaken in 14 hyperthyroid and 8 hypothyroid patients. The pituitary luteinizing hormone (LH) and follicle stimulating hormone (FSH) responses to 100 mug luteinizing hormone-releasing hormone (LRH) was assessed. These responses were found to be similar to matched control groups in hyperthyroid premenopausal women with and without menstrual disturbances and in hyperthyroid men. The postmenopausal hyperthyroid women had an exaggerated LH response, although the FSH response was comparable to that of the control group. These results indicate normal pituitary LH and FSH reserve in hyperthyroidism. In hypothyroidism a gradation of gonadotropin response to LRH was found. In 2 of the patients there was an inadequate LH response in the presence of a normal FSH response. This demonstrates that in some cases of primary hypothyroidism there is limited pituitary LH reserve.

Adult↗

PubMed1976

Hypersecretion of LH and FSH by a pituitary adenoma.

A 51-year-old man who had a pituitary adenoma that appeared to be hypersecreting LH and FSH is described. Not only were serum LH and FSH concentrations above the normal ranges, but the serum concentrations of testosterone, free testosterone, and dihydrotestosterone were also above normal. Serum LH and FSH concentration increased in response to synthetic thyrotropin-releasing hormone as well as to synthetic gonadotropin-releasing hormone. The elevated hormone concentrations decreased following an initial partial hypophysectomy and decreased further following repeat hypophysectomy.

Adenoma↗

PubMed1977

The effect of osmotic stimuli on prolactin secretion and renal water excretion in normal man and in chronic hyperprolactinemia.

An osmoregulatory role for prolactin (PRL) in man has been postulated, and PRL secretion has been reported to be influenced by osmotic stimuli. Clinical observation, however, does not support this notion. The effects of water loading, hypertonic saline infusion and nicotine on serum PRL and on renal water metabolism were investigated in 6 normal subjects and in 8 patients with chronic hyperprolactinemia (four with and four without demonstrable pituitary tumors). None of the patients had thyroid, adrenal or vasopressin deficiency. Renal walter handling in these patients was indistinguishable from normal. Likewise, serum PRL was not affected by the stimuli employed in either the normal subjects or the patients. No correlation between degree or duration of hyperprolactinemia and renal water metabolism was found. It is concluded that PRL is not an important osmoregulatory hormone in man.

Adult↗

PubMed1985

Pituitary-testicular axis dysfunction in burned men.

Plasma total testosterone, free testosterone, LH, and sex hormone-binding globulin (testosterone-estradiol-binding globulin) were measured in adult men hospitalized for treatment of burns. Total testosterone was below the normal range in 37 of 41 patients. Free and total testosterone levels declined very rapidly after the burn, remained low for weeks, and rose toward the end of hospitalization, but usually had not reached normal levels at discharge. The more severe the burns, the lower were the testosterone levels. LH levels were below normal during the first 4 days after the burn and remained in the low or mid normal range thereafter. Sex hormone-binding globulin levels were normal or slightly low. The data suggest the presence of a central defect in the release of LH. Large doses of opiate analgesics or an increase in endogenous opiate peptides related to severe persistent pain might be contributing factors to the pituitary-testicular hypofunction found in burned men.

Adolescent↗

PubMed1985

Bromocriptine as primary therapy for prolactin-secreting macroadenomas: results of a prospective multicenter study.

To assess the effectiveness of bromocriptine in reducing the size of PRL-secreting macroadenomas with extrasellar extension, we conducted a prospective multicenter trial in patients without prior radiotherapy, applying a standard protocol of treatment and tumor size evaluation. Basal serum PRL levels [1441 +/- 417 (+/- SEM) ng/ml for women; 3451 +/- 1111 ng/ml for men] fell in all patients and to 11% or less of basal values in all patients but 1. Normal PRL levels were reached in 18 of the 27 patients. In 13 patients (46%), tumor size was reduced by greater than 50%, in 5 patients (18%) by about 50%, and in 9 patients (36%) by approximately 10-25%. The extent of tumor size reduction did not correlate with basal PRL, nadir PRL, percent fall in PRL, or whether PRL levels reached normal. However, a reduction in PRL levels always preceded any detectable change in tumor size. In 19 patients, reduction in tumor size was evident by 6 weeks, but in the other 8, such reduction was not noted until the 6 month evaluation. In the 4 patients in whom bromocriptine was discontinued at the end of 1 yr, tumor reexpansion occurred in 3. Visual fields improved in 9 of the 10 patients in whom they were abnormal. Because of the excellent results found in most of the patients in this series, we suggest that therapy with bromocriptine should be considered as initial management for patients with PRL-secreting macroadenomas.

Adenoma↗

PubMed1990

Changes in endocrine function after adrenal medullary transplantation to the central nervous system.

Ten patients were studied before and after autologous adrenal medullary transplantation to the central nervous system for Parkinson's disease to determine if the presence of new catecholamine-producing tissue near the hypothalamus would alter hypothalamic or pituitary function, mineralocorticoid levels, or catecholamine production. No clinically apparent ill effects occurred. Changes in endocrine function were largely short-term and transient: at 7-10 days after surgery, urinary catecholamine levels were significantly increased, PRL levels were significantly elevated despite markedly increased serum dopamine levels, and gonadal steroid levels (estradiol and testosterone) were significantly lower despite unchanged basal and stimulated levels of gonadotropins. Dehydroepiandrosterone sulfate was significantly reduced at 7-10 days after surgery and remained low at 3-6 months. Other changes at 3-6 months after surgery included increased stimulated corticotropin levels and reduced serum aldosterone response to upright posture. The changes at 7-10 days were probably due to stress or unilateral adrenalectomy or both; the changes at 3-6 months were likely due to unilateral adrenalectomy. We conclude that unilateral adrenalectomy and autologous adrenal medullary transplantation to the central nervous system does not produce clinically important changes in endocrine function; however, possible adverse consequences of long-term reduction of dehydroepiandrosterone sulfate levels cannot be excluded.

Adrenal Medulla↗

PubMed1994

Empty sella in children and adolescents with possible hypothalamic-pituitary disorders.

Several computed tomographic scan studies have described empty sellae in children with hypothalamic-pituitary disorders. Magnetic resonance imaging, however, is a more precise technique for visualizing the intrasellar content, such as the stalk and pituitary lobes. Using magnetic resonance imaging, we studied 339 children and adolescents (mean age +/- SD, 12.7 +/- 4.5 yr) with possible hypothalamic-pituitary disorders to ascertain the frequency of primary empty sella and examine its relationships with other intrasellar abnormalities, pituitary function, and adverse perinatal events. One hundred and ninety-three patients had isolated GH deficiency, 43 had multiple pituitary hormone deficiency, 10 had diabetes insipidus, 17 had hypogonadotropic hypogonadism, 5 had idiopathic delayed puberty, 47 had precocious puberty, and 24 had other hypothalamic pituitary disorders of hyperfunction. One tenth (10.9%) of the patients (37 cases) had empty sella, with a marked variation of incidences among the disorders listed above. A statistically higher frequency of subjects with empty sellae was found only in patients with multiple pituitary hormone deficiency. Patients with and without empty sellae were not different in regard to age or sex. The incidence of empty sella in the various groups of patients was as follows: isolated GH deficiency, 8.8% (17 cases); multiple pituitary hormone deficiency, 34.9% (15 cases); hypogonadotropic hypogonadism, 5.9% (1 case); idiopathic delayed puberty, 40% (2 cases); and precocious puberty, 4.2% (2 cases). No patients with isolated diabetes insipidus or other hypothalamic-pituitary disorders had empty sellae. In the patients with empty sellae, abnormalities of the stalk or posterior lobe were found in 1 patient with isolated GH deficiency (5.9%), 13 patients with multiple pituitary hormone deficiency (86.7%), and no patients with puberty disorders. Likewise, adverse perinatal events were found only in 1 patient with isolated GH deficiency and 9 patients with multiple pituitary hormone deficiency. These findings suggest that empty sella is not rare in children and adolescents evaluated for hypothalamic-pituitary disorders, particularly if there is multiple pituitary hormone deficiency. Empty sella can be found regardless of abnormalities of the stalk and posterior lobe, and adverse perinatal events do not seem to be the primary etiological factor. Empty sella is usually associated with pituitary hypofunction, but it can be found in patients with hyperfunction of the hypothalamic-pituitary-gonadal axis.

Adolescent↗

PubMedDate not supplied

Anesthesia for transsphenoidal surgery of the hypophyseal region.

For pituitary surgery--by either the transcranial or the transsphenoidal approach--anesthetic management conforms to the general principles of neuroanesthesia as discussed in texts and review articles. In addition, the endocrine functions and the anatomic situation of the pituitary gland must be considered. Preoperative and perioperative evaluation of the patient's endocrine status and appropriate therapeutic measures are important. Preservation of neurohypophyseal function and, frequently, of adequate adenohypophyseal function is an advantage of the transsphenoidal approach to the sella. Potential problems with the transsphenoidal technic include damage to suprasellar and parasellar structures and harzards associated with intraoperative or postoperative bleeding. Anesthetic aspects peculiar to the transsphenoidal approach include positioning of the patient, surgical use of solutions containing epinephrine and topical cocaine, intraoperative air studies, and management of emergence. Anesthesia for the transsphenoidal approach to the sella is discussed on the basis of our experience with 148 surgical procedures in 142 patients.

Adolescent↗

PubMedDate not supplied

Anterior pituitary function before and after trans-sphenoidal microsurgical resection of pituitary tumors.

Anterior pituitary lobe function was measured pre- and postoperatively in 40 patients with pituitary tumors who were managed surgically by the trans-sphenoidal approach. Of 23 patients with normal anterior lobe function preoperatively, 21 (91%) were normal postoperatively. Of 17 preoperatively impaired patients, 7 (40%) regained normal function, 3 (18%) improved, 4 (24%) remained the same, and 3 (18%) were further impaired by the surgery. In this small series, the likelihood of recovery of pituitary function varied inversely with the degree of preoperative impairment and the size of the tumor, suggesting that more aggressive surgical management of small, minimally symptomatic pituitary tumors is justified.

Adenoma↗

PubMed1986

Subarachnoid hemorrhage with normal cerebral angiography: a prospective study on sellar abnormalities and pituitary function.

Ten consecutive patients with subarachnoid hemorrhage (SAH) of unknown cause were examined for sellar abnormalities and pituitary dysfunction. The diagnosis of SAH rested on a typical history and the demonstration of blood in the cerebrospinal fluid (computed tomography (CT), lumbar puncture). Initial CT and four-vessel angiography were performed in all patients, and five had repeat angiography. Endocrinological assessment was done after the initial angiography, and abnormal findings from this first study were reassessed 3 months later. The follow-up study included a high resolution CT scan of the sellar region. One patient had panhypopituitarism. Insufficient growth hormone secretion alone or combined with a low plasma concentration of estradiol or testosterone was found in another seven patients. The sella was ballooned or enlarged in three patients, and four patients had at follow-up a partly empty sella. A spontaneous development of a partly empty sella from an adenoma with suprasellar extension was observed in the patient with panhypopituitarism. This patient had experienced pituitary apoplexy with resulting SAH. Based on sellar enlargement, an empty sella, and pituitary dysfunction, this pathogenesis was suggested in another three patients. Our findings suggest that hemorrhagic necrosis in a pituitary adenoma may be an important cause of SAH with normal angiography.

Adenoma↗

PubMed1989

Endocrine function in patients with large pituitary tumors treated with operative decompression and radiation therapy.

Large pituitary tumors are still a common problem. Thirty consecutive patients underwent operative decompression and radiation therapy for large sellar and suprasellar tumors. They were studied prospectively in terms of their endocrine outcome. Ten of the patients had panhypopituitarism both before and after treatment. The other 20 patients had partial hormonal deficits prior to treatment. Ten (50%) of the 20 patients who had partial preoperative deficits went on to develop delayed onset of worsening in their endocrine function; 9 of the 10 developed panhypopituitarism, and 1 patient developed decreased thyroid function. The mean time from surgery until the onset of delayed worsening in endocrine function was 26.1 months. The mean age of those patients who developed delayed onset of worsening in pituitary function was significantly higher than that of those who did not develop further hormonal loss (40.5 +/- 3.1 years versus 52.0 +/- 4.1 years, P less than 0.05). None of the 10 patients with delayed onset of worsening in pituitary function had anatomical evidence by computed tomographic scan of tumor recurrence. Delayed onset radiation effect is the most likely cause of the late onset of worsening in endocrine function.

Combined Modality Therapy↗

PubMed1989

Pituitary hyposecretion and hypersecretion produced by a Rathke's cleft cyst presenting as a noncystic hypothalamic mass.

A 53-year-old man presented with polydipsia, polyuria, lightheadedness on standing, and syncope. Visual field deficits suggesting left optic tract compression and pituitary dysfunction consisting of diabetes insipidus, hyperprolactinemia, and anterior pituitary insufficiency were diagnosed. On the computed tomography scan, an isodense, ring-enhancing, parasellar mass was localized primarily in the suprasellar region and also extended into the sella. It was believed that the pituitary hormone hypersecretion and hyposecretion were due to hypothalamic dysfunction from the mass which was initially believed to be a craniopharyngioma. At surgery a Rathke's cleft cyst was resected. The unusual presentation of this Rathke's cleft cyst presenting as a hypothalamic lesion is discussed.

Craniopharyngioma↗

PubMed1993

Fibrosing pseudotumor of the sella and parasellar area producing hypopituitarism and multiple cranial nerve palsies.

We present an unusual patient with a medical history of a fibrosing pseudotumor of the left orbit that had been stable for 8 years who presented with acute anterior hypophyseal failure. During the next 10-month period, sequential magnetic resonance scans showed a rapid growth of a plaque-like sellar and parasellar mass extending into the right cavernous sinus, right Meckel's cave, along the dural surfaces of the clivus, dens, and body of the second cervical vertebra. A transsphenoidal biopsy revealed sphenoid and intrasellar pseudotumor that invaded the adenohypophysis and had microscopic features identical to those of the previously excised orbital pseudotumor. Rapid growth of the pseudotumor continued despite a course of radiotherapy. Palsies of cranial nerves V and VI and of the sensory root of the cranial nerve VII developed on the right side. Steroid therapy was associated with improvement of the cranial nerve palsies. This is the first report of the sellar fibrosing pseudotumor producing not only anterior hypophyseal failure, but also cranial nerve dysfunction secondary to plaque-like extension into the cavernous sinus, Meckel's cave, and cranial base dura. This intracranial plaque-like extension of a fibrous pseudotumor corresponds to a hypertrophic intracranial pachymeningitis, which is a rare, previously described phenomenon associated to the syndrome of multifocal fibrosclerosis.

Biopsy↗

PubMed1993

Prepubescent pituitary null cell macroadenoma with silent macroscopic apoplexy: case report.

Secreting pituitary adenomas have marked prevalence in pediatric adenomas. We described a case of an 11-year-old boy with pituitary null cell macroadenoma with silent macroscopic pituitary apoplexy. The patient was admitted with bitemporal hemianopsia and a gradual deterioration of visual acuity. An endocrinological study revealed a low concentration of testosterone and in the plasma a decreased response of luteinizing hormone and follicle-stimulating hormone to luteinizing hormone-releasing hormone. This patient may be the first case of prepubescent null cell macroadenoma confirmed with immunohistochemical study in addition to the findings of classical histopathology.

Adenoma, Chromophobe↗

PubMed1993

Cushing's disease in a child caused by a corticotropin-releasing hormone-secreting intrasellar gangliocytoma associated with an adrenocorticotropic hormone-secreting pituitary adenoma.

Cushing's disease resulting from intrasellar corticotropin-releasing hormone (CRH)-secreting gangliocytomas is very rare, and only two such cases have been reported in the literature to date. The authors present a third case in which an adrenocorticotropic hormone-secreting pituitary adenoma was found in addition to a gangliocytoma in a 10-year-old girl with clinical and endocrinological symptoms of Cushing's disease. Computed tomographic and magnetic resonance imaging scans showed a suprasellar and parasellar tumor. A green-colored, heterogeneous tumor and a small adenoma were removed transsphenoidally. Histological examination revealed a large gangliocytoma immunoreactive for CRH and a small, mucoid cell pituitary adenoma immunoreactive for ACTH. This is the first case of such a tumor causing Cushing's disease in a child. It might exemplify induction of an ACTH-secreting pituitary adenoma by means of chronic overstimulation of CRH.

Adenoma↗

PubMed1994

Novel features of tumors that secrete both growth hormone and prolactin in acromegaly.

The most prominent previously reported clinical features of growth hormone (GH) and prolactin (PRL)-secreting pituitary adenomas associated with acromegaly have included the high incidence of galactorrhea in women and a generally more favorable response to dopamine agonist therapy. The authors analyzed a consecutive series of 62 acromegalic patients treated with transsphenoidal microsurgery. GH-PRL tumors were found in 30% of the patients. There was a significant difference in sex distribution between acromegalics with the GH-PRL tumor subtype and all other acromegalics. Women represented 73% of the GH-PRL immunostain subtype, as compared with the overall sex distribution of 33 women (53%) and 29 men (47%) for the entire series of acromegalic patients. Individuals with the GH-PRL subtype had significantly higher postoperative GH levels than those with the GH subtype, and significantly higher postoperative GH levels when compared with all other acromegalics with a variety of immunostain subtypes. Linear regression analysis of the pre- and postoperative GH data revealed that the increased postoperative GH levels in the GH-PRL immunostain subtype were independent of the invasiveness of the tumor and of sex of the subject. When the same linear regression technique was used, lower preoperative levels of thyroxine and thyroid-stimulating hormone were observed in the GH-PRL subtype. These data suggest inherent differences characteristic of tumors that secrete both growth hormone and prolactin.

Acromegaly↗

PubMed1994

Pseudotumoral lymphocytic hypophysitis successfully treated by corticosteroid alone: first case report.

We report the first case of pseudotumoral lymphocytic hypophysitis successfully treated by corticosteroids without surgery. A 27-year-old woman had been monitored for chronic headache 13 months after giving birth, associated with amenorrhea and galactorrhea. Cranial magnetic resonance imaging revealed a markedly enlarged pituitary gland with a suprasellar extension; the only biochemical abnormality was a mild hyperprolactinemia. Because of a putative diagnosis of prolactinoma, bromocriptine was prescribed at a dose of 5 mg daily, soon followed by the transitory appearance of menstruation. Two years later, panhypopituitarism was present and was revealed by acute adrenal insufficiency. Magnetic resonance imaging revealed that the pituitary mass was the same as previously described, but hormonal investigation showed evidence of complete hypopituitarism and no hyperprolactinemia. Nuclear antibodies were negative as well as other autoantibodies. Human leukocyte antigen serological Class II typing was DR3/DR4. Lymphocytic hypophysitis was then suspected; in the absence of visual complication and because this patient refused surgery, corticosteroids were attempted at a daily dose of 60 mg of prednisone for 3 months, progressively decreased for the next 6 months. Under this treatment, a gradual recovery of all pituitary hormones was observed and magnetic resonance imaging showed a reduction of two-thirds in pituitary mass. Five months after the end of corticoid treatment, our patient relapsed with panhypopituitarism and an increase of pituitary volume. She underwent steroid treatment, and a biopsy was performed and confirmed the diagnosis of autoimmune hypophysitis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗