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Sudden death and Wegener's granulomatosis of the pituitary.

Involvement of brain parenchyma or meninges in ANCA-associated small-vessel vasculitis such as Wegener's granulomatosis (WG) is not uncommon. In contrast, involvement of the pituitary is exceedingly rare with only a few cases reported so far. The diagnosis is usually made on the basis of imaging techniques and abnormal pituitary function tests in the setting of active systemic vasculitis. However, histology-proven involvement of the pituitary by WG has not been reported so far. We report a case of WG with histology-proven granulomatous necrotizing inflammation of the pituitary and hypothalamo-pituitary stalk, disclosed at autopsy after the patient had died suddenly and unexpectedly in his sleep. In a setting of histology-proven WG, these findings were regarded as a pituitary manifestation of the disorder. A distinct cause of death could not be found, hence we speculate that hypothalamo-pituitary inflammation due to WG may have caused the sudden death in this patient.

Adult↗

The effect of multiparity on intrasellar prolactinomas.

A complete reassessment of ovulation, pituitary reserve and function, and sella turcica anatomy was carried out in nine multiparous patients with intrasellar prolactinomas to determine whether long-term bromocriptine therapy was required and to document the natural history of the disease after two or more pregnancies. After the last pregnancy, bromocriptine was discontinued and pituitary function and anatomy and prolactinoma activity were reassessed with documentation of ovulation (basal body temperature graphs and menstrual history), search for fat droplet-positive galactorrhea, pituitary fossa tomography, computerized tomographic scan, triple-bolus testing, and visual fields. These data were compared with a similar workup carried out prior to the first pregnancy. Three groups of eventual outcomes were identified radiologically. Anterior pituitary gland function and reserve remained normal in all, and no neurological sequelae were noted. Four patients did not require long-term treatment. A hypothesis of autoinfarction of the adenoma is raised, since three patients were shown to have empty sellae.

Adult↗

Pituitary apoplexy diagnosed by magnetic resonance imaging: a case report.

Pituitary apoplexy is a neuroendocrine emergency produced by hemorrhage or infarction of pituitary tumors, and its accurate diagnosis has relied mainly on clinical manifestations and computed tomography (CT) scan of sella in the past. A case is reported of pituitary apoplexy which was demonstrated on magnetic resonance imaging (MRI). The 72-year-old male patient was admitted to the Nephrology Section of VGH-Taipei with symptoms of general malaise, poor appetite for four months and apparent hyponatremia (Na 102 mEq/L) on laboratory findings. Under the impression of syndrome of inappropriate secretion of antidiuretic hormone (SIADH), fluid restriction and infusion of isotonic saline were prescribed, but in vain. The patient was transferred to the Endocrinology Section because of lowered serum cortisol, T3, T4, and hs-TSH levels. Dynamic pituitary function tests confirmed the diagnosis of panhypopituitarism. Contrast enhanced CT scan of sella revealed displacement of the pituitary stalk to the right side. T1-weighted MRI showed persistent high intensity in left sella and T2-weighted MRI showed persisting isointense to white matter. The hyponatremia and clinical symptoms resolved within a few days after replacement therapy with glucocorticoid and thyroid hormone. It was concluded that MRI is more sensitive than CT scan for detecting subacute or chronic pituitary apoplexy.

Aged↗

[Arginine test].

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Arginine↗

Magnetic resonance imaging of the hypothalamic-pituitary axis in the diagnosis of growth hormone deficiency.

In order to investigate the relationship between pituitary appearance and the diagnosis of growth hormone deficiency (GHD), we have assessed magnetic resonance imaging (MRI) scans and GH status during provocation tests in 110 patients (78 males; median age 9.8, range 0.1-20 yr), evaluated for possible GH disorders. On the basis of pituitary function tests, patients were divided into GH deficient (GH peak < 15 mIU/l [5.8 ng/ml]) (n = 82) or GH sufficient (GH peak > 15 mIU/l) (n = 28). The former were further divided into those with multiple hormone deficits (MPHD) (n = 19) or isolated GHD - severe IGHD (peak GH < 8 mIU/l [3.1 ng/ml]) or partial IGHD (8-15 mIU/l). The appearance of the hypothalamic-pituitary (H-P) axis was classified as: (1) normal, (2) isolated hypoplastic stalk (HPS), (3) isolated hypoplastic anterior lobe (HPAL) (PHT SDS < -2.0), (4) HPS + HPAL or (5) ectopic posterior lobe (EPL). The last two were considered severe abnormalities. PHT SDS (mean +/- SD -2.0 +/- 2.2) was correlated to log peak GH levels in the whole group (r = 0.45; p < 0.0001) and in the GHD group (r = 0.39; p < 0.0001). Sixty-five out of 82 in the GHD group had a H-P axis abnormality (45 severe abnormalities), while 13 out of the 28 patients in the GH sufficient group also had an abnormality (3 severe, but none with an EPL). All patients with MPHD had a MRI abnormality, most commonly an EPL (79%). Thus the presence of any MRI abnormality as a marker for GHD would generate a sensitivity of 79%, but a specificity of only 54%, indicating that this could not be used to confirm GHD. However, the presence of either an EPL or HPS + HPAL on MRI is highly specific (100% and 89% respectively) and predictive of GHD (positive predictive value 100% and 79% respectively), indicating that these abnormalities provide confirmation of the diagnosis. We recommend that if clinical, auxological and biochemical data indicate a diagnosis of GHD, then a MRI scan should be undertaken to define the pituitary anatomy and to help confirm the diagnosis.

Adolescent↗

Enhanced neuroendocrine response to insulin tolerance test performed under increased ambient temperature.

The hypothesis that an increase in ambient temperature modulates neuroendocrine response in clinically used provocative pituitary function tests was verified. Healthy male volunteers were subjected to insulin tolerance tests in two randomized trials. In the first trial hypoglycemia was induced by a bolus injection of insulin (0.1 U per kg of BW, i.v.) at room temperature. In the second trial, the subjects were exposed to increased ambient temperature for 45 min before insulin injection and for 45 min thereafter. The environmental temperature was selected to increase body temperature less than 1C. Under conditions of increased temperature basal hormone levels as measured in antecubital venous blood samples failed to be modified and the hypoglycemia was less severe. Nevertheless, the responses of most (beta-endorphin, ACTH, prolactin, catecholamines), but not all (growth hormone, cortisol), hormones to hypoglycemia were exaggerated. The remarkable increase in ACTH and beta-endorphin release was not accompanied by concomitant increase of plasma cortisol response. The sympathetic-adrenomedullary system was significantly activated, which was manifested particularly by enhanced norepinephrine release. Growth hormone response to hypoglycemia was not modified, while that of prolactin was enhanced. Thus during evaluation of neuroendocrine function under clinical conditions, changes in ambient and body temperature should not be underestimated.

Adrenocorticotropic Hormone↗