PURPOSE: In this study, efforts have been made to evaluate the sella MRI in 14 patients with growth hormone deficiency to define more clearly the structural abnormalities of the hypothalamo- hypophyseal axis, examine frequency of these abnormalities, and determine whether a relationship exists between the sella MRI findings and types of endocrine dysfunction. METHODS: Sella MRI was performed in 14 patients who were diagnosed as growth hormone deficiency (GHD) based on a strict diagnostic criteria. If abnormal sella MR findings were associated, then combined anterior pituitary function test was carried out. RESULTS: Among the 14 patients with GHD, complete deficiency was found in 10 patients and partial deficiency in 4 patients. Three with complete GHD showed normal MRI findings and isolated GHD only. Others revealed 3 pituitary stalk interruption syndrome (PSIS), 2 hypoplastic pituitary gland, 1 prolactinoma, and 1 Langerhans histiocytosis. These patients have multiple endocrine dysfunctions in addition to GHD. Three out of 4 patients with partial GHD, showed normal sella MR findings and isolated GHD. Only one patient with partial GHD had a delayed puberty and hypoplasia of pituitary gland. CONCLUSION: Sella MRI enables us to identify mophologic pictures in GHD previously considered 'idiopathic'. Abnormal sella MR findings strongly suggest that multiple hormone deficiencies are associated. This study justifies that routine sella MRI should be recommended in an attempt to identify the etiology of GHD in case that the diagnosis of GHD is strictly made.
Axis, Cervical Vertebra ; Diagnosis ; Growth Hormone* ; Histiocytosis ; Humans ; Magnetic Resonance Imaging* ; Pituitary Function Tests ; Pituitary Gland ; Prolactinoma ; Puberty, Delayed

BACKGROUND: Anesthetic requirements are affected by the preoperative levels of some hormones. This study investigated to identify the hormonal status such as plasma level and maximal secretary capacity correlating with propofol and remifentanil requirements in pituitary adenoma patients who show various hormonal secretory states perioperatively. METHODS: From 51 adult female pituitary adenoma patients, preoperative basal values and maximal stimulated levels of various hormones related to the axis of hypothalamus-pituitary-target organs on combined pituitary function test were recorded. Total intravenous anesthesia using target controlled infusion with propofol and remifentanil was administered. The effect-site concentration (Ce) of propofol reaching anesthetic induction and the consumed dosages of propofol and remifentanil during operation were measured. Anesthetic maintenance was controlled within 30% of preanesthetic hemodynamic variables by remifentanil and within ranges of BIS 45 +/- 10 by propofol. Spearman correlations between hormonal status and anesthetic requirements such as propofol Ce for induction, total consumed doses of propofol and remifentanil were performed with a statistical significance at P of 0.05. RESULTS: The preoperative basal level of ACTH was correlated positively with propofol induction Ce and maintenance dose, and the maximal secretory capacity of prolactin also correlated positively with propofol induction Ce. Remifentanil consumption dose was not related with any hormones measured regardless of either preoperative basal levels or maximal secretory levels. CONCLUSIONS: Propofol requirements may be related with preoperative plasma level of ACTH or maximal secretory capacity of prolactin.
Adrenocorticotropic Hormone ; Adult ; Anesthesia, Intravenous ; Axis, Cervical Vertebra ; Female ; Hemodynamics ; Humans ; Piperidines ; Pituitary Function Tests ; Pituitary Neoplasms ; Plasma ; Prolactin ; Propofol

Functional defects of the pituitary gland are a rare cause of pubertal delay. The pituitary stalk is an important structure that connects the hypothalamus and pituitary gland. A defect in fusion of the pituitary stalk and anterior pituitary gland will block the function of the anterior pituitary gland. A 28-year-old man was referred to our clinic with poorly developed secondary sexual characteristics. He had undeveloped facial, axillary, and pubic hair and was Tanner stage I. Laboratory tests gave random serum testosterone < 0.025 ng/mL, luteinizing hormone (LH) < 0.1 mIU/mL, follicle-stimulating hormone (FSH) 0.626 mIU/mL, thyroid-stimulating hormone (TSH) 6.85 microIU/mL, and fT4 6.96 pmol/L. Sella magnetic resonance imaging (MRI) showed no pituitary stalk enhancement. The response in the combined pituitary function test revealed multiple hormonal defects, while the TSH response to thyrotropin-releasing hormone (TRH) was exaggerated and delayed. Therefore, we concluded that pituitary stalk dysgenesis had led to hypothalamic-type panhypopituitarism.
Adult ; Follicle Stimulating Hormone ; Hair ; Humans ; Hypopituitarism ; Hypothalamus ; Luteinizing Hormone ; Magnetic Resonance Imaging ; Pituitary Function Tests ; Pituitary Gland ; Pituitary Gland, Anterior ; Puberty, Delayed ; Testosterone ; Thyrotropin ; Thyrotropin-Releasing Hormone

BACKGROUNDS: Pituitary hyperplasia can mimic pituitary adenoma. In MRI, enlarged pituitary gland is enhanced homogenously with upward convexity of the superior margin of the gland .The best definition of hyperplasia in the pituitary hyperplasia seems to be a multiplication of one or more cell types. But definition, etiology and clinical courses of this disease are not clear, METHOD: We reviewed clinical symptoms, MRI, and pathologic findindings in 6 patients with pituitary hyperplasia. RESULT: 1. Major clinical symptoms were headache (100%), visual field defect (84%), polyuria/polydipsia (64%), and irregular mensturation (32%). Other symptoms were amenorrhea (16%) and galactorrhea (16%). 2. Three of five cases showed abnormal responses to combined pituitary function test, 3. MRI findings were pituitary hyperplasia (4), macroadenoma (l), and microadenoma (1). 4. In two operated cases, there was no adenoma. One case showed hyperplasia of lactotroph cells, the other was hyperplasia of gonadotroph cells confirmed by the examination of immunocytochemistry. CONCLUSION: Pituitary hyperplasia should be considered in patients with enlarged pituitary gland without focal mass lesion.
Adenoma ; Amenorrhea ; Female ; Galactorrhea ; Gonadotrophs ; Headache ; Humans ; Hyperplasia* ; Immunohistochemistry ; Lactotrophs ; Magnetic Resonance Imaging ; Pituitary Function Tests ; Pituitary Gland ; Pituitary Neoplasms ; Pregnancy ; Visual Fields

BACKGROUND: Primary empty sella syndrome (PES) is thought to arise from an incompetent diaphragma allowing progressive herniation of arachnoid membrane with secondary compression and atrophy of the pituitary gland. As a consequence of the improvement and widespread use of neuroradiological techniques, such as computerized tomography (CT) and magnetic resonance imaging (MRI), empty sella is more frequently disclosed. The aim of this study is to assess the associated clinical characteristics and endocrinologic disturbance in empty sella syndrome. METHODS: From January 1986 to June 1996, 171 patients with empty sella syndrome have undergone analysis for clinical characteristics and associated disease. RESULT: In our study, PES was diagnosed in 131 of the 171 patients (77%). Primary empty sella syndrome was frequent in middle aged women (female:male 115:16, mean age: 50.6+12.6 years). The common clinical features were headache (80.2%), obesity (72.5%), and hypertension (27.5%). Most of patients with PES have normal pituitary function (75%). The frequent pituitary dysfunction was hyperprolactinemia in PES (21%). Partial and total emptiness of sella on sella CT or MRI were in 111 (84.7%) patients, and in 20 (15.4%) patients, respectively. The most common associated disease with empty sella syndrome was pituitary adenoma. CONCLUSION: PES should be considered as a possible cause in obese middle aged women with unexplained headache. The combined pituitary function test should be considered for evaluation of pituitary dysfunction when clinically suspected.
Arachnoid ; Atrophy ; Empty Sella Syndrome* ; Female ; Headache ; Humans ; Hyperprolactinemia ; Hypertension ; Magnetic Resonance Imaging ; Membranes ; Middle Aged ; Obesity ; Pituitary Function Tests ; Pituitary Gland ; Pituitary Neoplasms

PURPOSE: Short stature is a very common reason for visits to pediatric endocrine clinics. It could be the first sign of an underlying disease. The purpose of this study is to investigate the etiologies and general characteristics of subjects who visited an outpatient clinic due to short stature. METHODS: We retrospectively reviewed the medical records of 3,371 patients who visited Severance Children's Hospital with the chief complaint of short stature from 2010 to 2012. Medical history, auxological data, and laboratory tests including bone age were collected and analyzed. Chromosome studies or combined pituitary function tests were performed if needed. RESULTS: Approximately 89.4% of the subjects with the chief complaint of short stature who visited the outpatient clinic were of normal height, and only 10.6% of subjects were identified as having short stature. Of the subject of short stature, 44.7% were classified as having normal variant short stature; that is, familial short stature (23.0%), constitutional delay in growth (17.7%), and mixed form (3.9%). Pathological short stature was found in 193 subjects (54.2%). Among pathological short stature, most common etiology was growth hormone deficiency (GHD) (38.9%). CONCLUSION: A majority of children had a normal height. Among children with short stature, pathological short stature and normal variants occupied a similar percentage. GHD was the most common cause of pathological short stature and found in about 20% of the children with short stature. In pathological short stature, the height, height velocity, and IGF-1 level were lower than in normal variants.
Ambulatory Care Facilities ; Child* ; Growth Hormone ; Humans ; Insulin-Like Growth Factor I ; Medical Records ; Pituitary Function Tests ; Retrospective Studies

Congenital hypopituitarism is a rare disorder with absence or reduction of hormones produced by the pituitary gland. The clinical manifestations are hypoglycemia, prolonged jaundice, hyponatremia, micropenis, underdeveloped clitoris, lethargy, convulsion, pallor, cyanosis, apnea, hypotension, temperature instability etc. in neonates and growth failure, delayed or absent puberty in older infants and children. We experienced a case of congenital hypopituitarism who was a 20-year-old male patient with short stature and delayed puberty. Combined pituitary function test revealed panhypopituitarism and magnetic resonance imaging of brain showed anterior pituitary aplasia and ectopic posterior pituitary gland.
Adolescent ; Apnea ; Brain ; Child ; Clitoris ; Cyanosis ; Female ; Humans ; Hypoglycemia ; Hyponatremia ; Hypopituitarism* ; Hypotension ; Infant ; Infant, Newborn ; Jaundice ; Lethargy ; Magnetic Resonance Imaging ; Male ; Pallor ; Pituitary Function Tests ; Pituitary Gland ; Pituitary Gland, Posterior* ; Puberty ; Puberty, Delayed ; Seizures ; Young Adult

Congenital hypopituitarism is a rare disorder with absence or reduction of hormones produced by the pituitary gland. The clinical manifestations are hypoglycemia, prolonged jaundice, hyponatremia, micropenis, underdeveloped clitoris, lethargy, convulsion, pallor, cyanosis, apnea, hypotension, temperature instability etc. in neonates and growth failure, delayed or absent puberty in older infants and children. We experienced a case of congenital hypopituitarism who was a 20-year-old male patient with short stature and delayed puberty. Combined pituitary function test revealed panhypopituitarism and magnetic resonance imaging of brain showed anterior pituitary aplasia and ectopic posterior pituitary gland.
Adolescent ; Apnea ; Brain ; Child ; Clitoris ; Cyanosis ; Female ; Humans ; Hypoglycemia ; Hyponatremia ; Hypopituitarism* ; Hypotension ; Infant ; Infant, Newborn ; Jaundice ; Lethargy ; Magnetic Resonance Imaging ; Male ; Pallor ; Pituitary Function Tests ; Pituitary Gland ; Pituitary Gland, Posterior* ; Puberty ; Puberty, Delayed ; Seizures ; Young Adult

PURPOSE: Occasionally, in nonglaucomatous optic atrophy, the optic nerve head may appear to be cupped to such a degree that normal tension glaucoma is suspected. We report such a case of visual field defects from schizencephaly in a patient initially suspected to have normal tension glaucoma, using ophthalmoscopic examination only. METHODS: An 18-year-old male patient visited our clinic because of bilateral visual disturbance which had developed 5 years previously. Ophthalmoscopic examination revealed an increased cup to disc ratio, peripapillary atrophy, and loss of temporal neuroretinal rim tissue. The authors suspected normal tension glaucoma in both eyes. However, because the patient was too young and the visual field test revealed atypical field defects of the left superior quadrants in both eyes, we performed MRI. The results showed right schizencephaly with an absence of septum pellucidum. In order to rule out hypothalamic- pituitary dysfunction which can coexist with the absence of septum pellucidum, we conducted combined pituitary function test (cocktail test) which revealed normal results. RESULTS: The patient was initially suspected to have normal tension glaucoma on ophthalmoscopic examination alone. However, because the patient was too young and showed nonglaucomatous visual field defects, MRI was performed and revealed right schizencephaly.
Adolescent ; Atrophy ; Humans ; Low Tension Glaucoma* ; Magnetic Resonance Imaging ; Male ; Malformations of Cortical Development* ; Optic Atrophy ; Optic Disk ; Pituitary Function Tests ; Septum Pellucidum ; Visual Field Tests ; Visual Fields

A 39-year-old woman presented with a 20 day history of recurrent hypoosmolar hyponatremia. Because her volume status seemed to be normal, the most suspected causes of her hyponatremia were adrenal insufficiency and hypothyroidism. Endocrinologic examination, including a combined pituitary function test, showed TSH and ACTH deficiency without GH deficiency, and hyperprolactinemia was also present. Sella MRI showed a pituitary mass, stalk thickening and loss of the normal neurohypophysial hyperintense signal on the T1 weighted image. Pathologic exam demonstrated granulomatous lesions and Langhans' multinucleated giant cells with inflammatory cell infiltration. After high dose methylprednisolone pulse therapy (1 g/day for 3 days) with subsequent prednisolone and levothyoxine replacement, there was no more recurrence of the hyponatremia. The sella MRI on the 6th month showed decreased mass size, narrowed stalk thickening and the reappearance of the normal neurohyphophysial hyperintense signal. She is currently in a good general condition and is receiving hormone replacement therapy.
Adrenal Insufficiency ; Adrenocorticotropic Hormone ; Adult ; Female ; Giant Cells ; Hormone Replacement Therapy ; Humans ; Hyperprolactinemia ; Hyponatremia ; Hypothyroidism ; Methylprednisolone ; Pituitary Function Tests ; Prednisolone ; Recurrence