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The hypothalamus, in addition to regulating the anterior and posterior pituitary, controls water balance through thirst, regulates food ingestion and body temperature, influences consciousness, sleep, emotion and other behaviors. Much has been learned of these effects in human disease through the clinical manifestations that occur with hypothalamic lesions. This study reviews the clinical pathologic correlations that have been made in recent years showing that regions of the hypothalamus exert functions in humans that are similar to those identified in experimental animals. Clinical pathologic correlations have not always provided precise analysis of hypothalamic function. The hypothalamus is small and often lesions that come to clinical attention achieve considerable size before their recognition, making local anatomic dissections of the effects of the lesions difficult. Nevertheless, the use of modern non-invasive techniques including CT scans and magnetic resonance imaging (MRI) have provided new information not previously available. This paper reviews several cases of hypothalamic disorder recognized recently. (1) A 33-year-old black man with hypothalamic sarcoidosis. Manifestations of hypothalamic dysfunction included panhypopituitarism, aggressive hyperphagia, polydipsia (partially due to hyperglycemia secondary to diabetes mellitus), drowsiness, depression, and irritability. (2) A 37-year-old woman with a large intrahypothalamic tumor (biopsy showed pituitary adenoma), with drowsiness, poikilothermia, lack of satiety, confusion, and memory loss. She becomes depressed when she is transiently more alert (as after hypertonic contrast-dye infusion). (3) A 60-year-old man with hypothalamic compression by a pituitary tumor, associated with syndrome of inappropriate ADH (SIADH), severe anorexia, memory loss, but preserved thirst. After surgical decompression of the tumor his appetite acutely recovered, but he developed severe hypo(poikilo)thermia. (4) A 45-year-old woman with a suprasellar craniopharyngioma presented with severe drowsiness, hyperphagia, depression, and memory loss post-operatively, which responded to antidepressants (except for the memory loss). She had extremely labile blood pressures and serum Na for about 1 week post-operatively.
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PMID:Neurologic manifestations of hypothalamic disease. 148 Jul 55

A 45-year-old hypertensive female with insulin-treated diabetes mellitus presented to our clinic with elevated urinary norepinephrine (NE) concentrations and a negative 131-metaiodobenzylguanidine (MIBG) scintigraphy, errouneously limited to the abdomen, for evaluation of a pheochromocytoma (Pheo). Despite antihypertensive medications blood pressure remained highly variable and frequently elevated. Further biochemical testing, including a glucagon provocation test and a clonidine-suppression test, revealed autonomous NE secretion. In order to avoid repeat MIBG-scintigraphy, other non invasive imaging techniques were performed, including real time sonography (7.5 MHz) of the neck which revealed a tumor. Fine needle aspiration of this tumor tissue demonstrated cells compatible with Pheo. Histology and immunohistochemistry of the excised tumor confirmed the diagnosis of Pheo. After surgical removal of the tumor, urinary and plasma NE levels normalized. Without any medication the blood pressure of the patient was now only slightly hypertensive. Only half of the daily insulin dose was needed to maintain the patient euglycemic.
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PMID:Cervical pheochromocytoma: a rare localization and a difficult diagnosis. 149 Nov 26

Ninety-five patients with thoracic esophageal cancer who had undergone radical esophagectomies through right thoracotomies from 1986 to 1989 were statistically analyzed semi-quantitatively to identify the risk factors predicting "operative (within 45 days of operation) or hospital death." Age, pulmonary function (%VC or %FEV1.0), cardiac function (EKG or Master test), renal function (Ccr), hepatic function (R15'ICG), diabetes mellitus (75 OGTT), extent of tumor invasion to the adventitia, and the type of operative procedure were each scored according to severity; 0 (no risk), 1, 2, or 3 (high risk). Patients with no severe postoperative complications had an accumulated score of less than 8 and comprised Group I, while those suffering an an "operative death" had a total score of 8 or more, and comprised Group II. Group III included those suffering a "hospital death." There was a significant difference between Group I and Group II (p < 0.005), but not between Group I and Group III. "Operative deaths" could be preoperatively predicted from the risk factors. However, "hospital deaths" could not be predicted, since they resulted mainly from recurrences of the cancer. In cases with scores of 8 or more the operative procedure should be changed to a simpler one for an improved prognosis.
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PMID:Preoperative prediction of mortality following surgery for esophageal cancer. 149 50

This report describes a case of epinephrine predominant pheochromocytoma successfully managed intraoperatively with an infusion of diltiazem. A 50-yr-old woman with a 10-yr history of diabetes mellitus was admitted to the hospital because of thirst and general fatigue. A cystic left adrenal tumor was found on computed tomographic scan. Although resting plasma catecholamine levels were normal, plasma norepinephrine and epinephrine levels obtained from the left adrenal vein were 1.6 ng.ml-1 (normal, 0.04-0.35) and 6.2 ng.ml-1 (normal, less than 0.12), respectively. Diltiazem was administered i. v. at a rate of 3 micrograms.kg-1.min-1 before induction of anesthesia. Anesthesia was induced with enflurane 2-3% and nitrous oxide in oxygen, followed by tracheal intubation facilitated with vecuronium. Anesthesia was maintained with enflurane 1-3% and nitrous oxide in oxygen. Paralysis was maintained with vecuronium. Hypertension during the manipulation of the tumor was controlled by increasing the inspired concentration of enflurane or by increasing the infusion rate of diltiazem to 5 micrograms.kg-1.min-1. There was no tachyarrhythmia. The infusion of diltiazem was continued until the draining vein from the tumor had been ligated. Hypotension, after removal of the tumor, was treated by the rapid infusion of fluid. Plasma norepinephrine and epinephrine levels during tumor manipulation were 1.18 ng.ml-1 and 6.57 ng.ml-1, respectively.
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PMID:[Use of diltiazem in the anesthetic management of epinephrine predominant pheochromocytoma]. 149 89

In 1919, glucose intolerance became the earliest recognized metabolic abnormality in cancer patients. Prior to the development of severe malnutrition, colon, gastric, sarcoma, endometrial, prostate, localized head, neck, and lung cancer patients had many of the metabolic abnormalities of type II (noninsulin dependent) diabetes mellitus. These metabolic abnormalities include glucose intolerance, an increase in both hepatic glucose production (HGP) and glucose recycling, and insulin resistance. In a study of over 600 cancer patients, a diabetic pattern of glucose tolerance test was noted in over one-third of the patients. An increased rate of HGP, commonly seen in diabetics, has been noted in almost all types of cancer patients studied to date. Etiology of the increased glucose production in the cancer patient is not known, but abnormalities in the counter regulatory hormones, especially growth hormone, may contribute to the development of abnormal glucose metabolism. A second possible stimulus for the increase in HGP could be the glucose needs of the tumor. Abnormally high glucose utilization rates in small amounts of tumor tissue have recently been described. This suggests that small tumors may have large needs for glucose calories. An increase in anaerobic glycolysis in the tumor tissue can increase lactate production in the tumor-bearing human, thus supplying substrate to the liver to increase glucose production rates. In this paper, the nature of abnormal glucose metabolism in cancer patients is described.
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PMID:A review of cancer cachexia and abnormal glucose metabolism in humans with cancer. 150 7

Adrenal tumors are usually diagnosed by clinical symptoms of hormone excess. The increasing use of ultrasound and computed tomography results in the detection of a substantial number of incidentally discovered adrenal tumors. Most of these tumors are nonfunctional adrenocortical adenomas, but a few cases of subclinical cortisol production in "incidentalomas" have been reported. We investigated prospectively the prevalence of autonomous cortisol production in 68 patients (44 females and 24 males, aged 25-90 yr) with adrenal incidentalomas at our institution. As a screening procedure all patients with incidentalomas underwent an overnight dexamethasone suppression test (1 mg). Patients who failed to suppress serum cortisol below 140 nmol/L (5 micrograms/dL) underwent more comprehensive studies (prolonged dexamethasone suppression test, determination of the diurnal rhythm of cortisol secretion in saliva, and CRH stimulation test). Eight patients (12% of all patients with incidentalomas; 5 females and 3 males, aged 25-71 yr) were finally identified as having cortisol-producing tumors, and the findings in these patients were compared with those of overt Cushing's syndrome in 8 patients (8 females, aged 26-50 yr) suffering from cortisol-producing adrenal adenomas. The tumor size of patients with cortisol-producing incidentalomas ranged from 2-5 cm. No specific signs and symptoms of hypercortisolism were present, but arterial hypertension (seven of eight subjects), diffuse obesity (four of eight subjects), and noninsulin-dependent diabetes mellitus (NIDDM; two of eight subjects) were frequently observed. Baseline cortisol levels were in the normal to upper normal range, whereas baseline ACTH levels were suppressed in five of the eight patients. In none of the patients was serum cortisol suppressible by low dose or high dose dexamethasone. The ACTH and cortisol responses to CRH were normal in two, blunted in one, and suppressed in four patients. Unilateral adrenalectomy was performed in seven patients and resulted in temporary adrenal insufficiency in four of them. After surgery, improvement of arterial hypertension, a permanent weight loss in obese subjects, and a better metabolic control of NIDDM were noted in the majority of patients. The following conclusions were reached. Incidentally diagnosed adrenal tumors with pathological cortisol secretion in otherwise clinically asymptomatic patients are more frequently observed than previously assumed. Adrenocortical insufficiency is a major risk in these patients after adrenalectomy. After surgery, hypertension, obesity, and NIDDM may improve. Patients with asymptomatic adrenal incidentalomas, therefore, should be screened for cortisol production by means of an overnight dexamethasone suppression test.
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PMID:Preclinical Cushing's syndrome in adrenal "incidentalomas": comparison with adrenal Cushing's syndrome. 151 73

Arteriosclerosis, neoplasms and diabetes are the three main killers in the industrialized world, complemented by infectious and parasitic diseases in the less developed countries. Neoplasms, the second most frequent killer in the industrialized world, exhibit the highest diversity of pathologic structures involved. Arteriosclerosis, the first killer in the industrialized world, is more complex, dealing with aberrations of the arterial wall. In contrast to neoplasms, cases are restricted to a closed circulatory system as noted in the various phyla. Neoplasms are also known from the kingdom of plants and fungi, whereas arteriosclerosis is restricted to specific animal phyla, at least theoretically. Even more restricted is the distribution of diabetes which is known only in certain vertebrates. All three disease groups have a substantial impact on man's health.
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PMID:Comparison of arteriosclerosis, neoplasms and diabetes as the three main killers of man in the industrialized world. 152 Aug 47

Acromegaly is an uncommon disorder and may present in a variety of ways, leading to considerable delay in diagnosis. Unlike other pituitary tumors, tumors associated with acromegaly tend to be fairly large in most patients. Thus, symptoms may be commonly due to the tumor mass as well as to hormone oversecretion. Mortality is two- to threefold increased due to cardiovascular, respiratory, and neoplastic causes. An increase in diabetes mellitus and hypertension may contribute to the first of these. Early treatment may reverse the diabetes, soft tissue changes, sleep apnea, cardiovascular disease, and neuromuscular disease. The effect of early treatment on neoplasia is unclear, and patients probably should continue to be screened, especially for colon neoplasia, even after appropriate therapy for the acromegaly. Hypopituitarism may be present initially as a result of tumor mass but may also develop as a result of ablative therapy.
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PMID:Clinical manifestations of acromegaly. 152 14

Autoimmunity is likely the cause of a variety of diseases including systemic lupus erythematosus, rheumatoid arthritis and diabetes. Normally, the body's immune system serves as a defense against a variety of conditions, including, injury, infection and neoplasm. However, for reasons that are currently unclear, the normal regulation of the immune system can breakdown resulting in autoaggressive responses. T cells, especially CD4+ cells, appear to play a predominant role in most autoimmune diseases. We summarize our workshop which focussed on the role of the T cells in autoimmune diseases. We summarize our workshop which focussed on the role of the T cells in autoimmune diseases; the T cell receptor in autoantigen recognition (emphasizing the role of selective T cell receptor V regions in the autoimmune response); and a discussion of possible therapeutic interventions.
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PMID:The T cell receptor in autoimmune diseases. 153 79

A testicular granulosa cell tumor of the adult type was studied in a 61-year-old man with a history of diabetes and urinary bladder carcinoma. The tumor was composed of three areas, each with a distinctive histologic pattern: solid, cystic, and cordlike. Most of the tumor cells in the three patterns had a round to ovoid euchromatic nucleus with one or two large nucleoli and scanty cytoplasmic organelles, mainly mitochondria and rough endoplasmic reticulum. These cells showed slight immunopositivity for vimentin and no immunostaining for cytokeratins. Occasionally, elongated nuclei displaying membrane infoldings were observed. Pleomorphic nuclei were rare. The number of mitotic figures was low. Differential aspects of testicular and ovarian granulosa cell tumors are discussed.
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PMID:Testicular granulosa cell tumor of the adult type. 153 15


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