Impact of screening on prognosis in the multiple endocrine neoplasia type 2 syndromes: natural history and treatment results in 105 patients.
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Adult↗
Biomedical subjects
B Hamberger
Publications and source records attributed to B Hamberger.
Parathyroid carcinoma--problems in diagnosis and treatment.
Four patients with parathyroid carcinoma operated on at the Karolinska Hospital were reviewed. In three patients the parathyroid carcinoma was suspected at the primary operation and successfully treated with excision of the tumour and ipsilateral hemithyroidectomy. In one patient the diagnosis was made only after local recurrence of the tumour and the appearance of lung metastasis. Although removal of local recurrence and distant metastasis was not curative, the patient improved for a long period of time. When surgical resection did not successfully control the hypercalcemia, Mithramycin, 12.5 micrograms/kg intravenous daily was given for five days. With two days interruption the treatment was repeated. The hypercalcemia could in this manner be controlled for almost one and a half years. It is concluded that parathyroid carcinoma is a relatively rare endocrine tumour which may be cured by adequate initial operation. When surgery is not feasible to control hypercalcemia, Mithramycin seems to be the drug of choice even for long-term therapy.
Adult↗
Anesthetic agents and sympatho-adrenal response to hemorrhage in the rat.
The sympatho-adrenal activity as reflected in plasma catecholamine levels was studied in rats subjected to a standardized hemorrhage. A comparison was performed between awake rats and rats anesthetized with diethylether, enflurane, barbiturate or ketamine. As expected diethylether increased sympathoadrenal activity. Enflurane induction increased adrenaline and decreased noradrenaline levels. Upon hemorrhage the sympatho-adrenal activity was depressed compared to the awake state. Barbiturate anesthesia depressed sympatho-adrenal activity. Ketamine increased noradrenaline levels and a relative hypertension was noticed during bleeding. The results focus attention on important differences in sympatho-adrenal activity between awake rats and rats anesthetized with different agents.
Anesthetics↗
Surgery for primary hyperparathyroidism. Experience with 400 patients during 10 years (1972-1981).
The records of 400 patients operated on for primary hyperparathyroidism during 10 years were reviewed. Seventy-three per cent of the patients were women. The mean age at operation was 58 years. Renal stones was the main indication for surgery in 52 per cent of the men, but only in 17 per cent of the women. Forty-four per cent of the women and 22 per cent of the men had neuromuscular symptoms. Fifteen per cent of the patients were asymptomatic. Hypercalcemia was moderate in most cases with mean S-Ca less than 3.0 mmol/l in 80 per cent. A conservative surgical approach with microscopical identification of one or two glands only was used in 2/3 of the cases. Subtotal parathyroidectomy was performed in less than 10 per cent. Single adenoma was found in 70 per cent, double adenomas in 4 per cent, hyperplasia in 17 per cent and cancer in 1 per cent. The outcome of the operation was normocalcemia in 86 per cent. Seven per cent had persistent hypercalcemia. Seven per cent were hypoparathyroid and had to take calcium or vitamin D to be normocalcemic. The frequency of hypoparathyroidism was highest in the group of patients where all four parathyroid glands had been biopsied during the operation. The results support a conservative surgical strategy with careful bilateral exploration without biopsy of all four glands.
Adenoma↗
Elevated plasma levels of noradrenaline in duodenal ulcer.
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Adult↗
Plasma catecholamines during surgical stress: differences between neurolept and enflurane anaesthesia.
To evaluate the influence of enflurane and neurolept anaesthesia on the sympatho-adrenal response to surgery, arterial plasma concentrations of adrenaline and noradrenaline were measured at 11 carefully defined events before, during and after cholecystectomy in two groups of five patients. During steady-state anaesthesia prior to operation and after cholangiography, when the operative procedure had been interrupted for 10 min, adrenaline concentrations were similar in the two groups. During periods of stress such as intubation, skin incision and abdominal exploration, adrenaline levels were 4-6 times higher in the neurolept patients compared to the enflurane patients (P less than 0.01), in whom adrenaline levels were very stable. Noradrenaline levels also varied with stress but without difference between the two groups. Systolic blood pressure was approximately 20 mmHg higher during operation in the neurolept group than in the enflurane group (P less than 0.05). It is concluded that enflurane blocks the sympatho-adrenal response to surgical stress more effectively than conventional neurolept anaesthesia.
Adult↗
Is there a relationship between gastric acid secretion and plasma catecholamines in duodenal ulcer disease?
Plasma adrenaline, noradrenaline and dopamine concentrations have been analyzed in three different groups of duodenal ulcer (DU) patients under various regimens of acid reduction in an attempt to find out if acidity per se has any influence on plasma catecholamine concentrations. Treatment with cimetidine in patients with asymptomatic chronic DU disease, reduced gastric acidity to the same level as after highly selective vagotomy (HSV), but increased plasma noradrenaline concentrations insignificantly. In a group of DU patients subjected to HSV, plasma noradrenaline levels were significantly higher 1 year after surgery than 6 weeks thereafter despite the fact that the basal acid output was the same. In a third group of DU patients, plasma noradrenaline and dopamine levels were found to be 20% higher during the active, untreated ulceration than after 4 weeks of ranitidine treatment when the ulcer had healed. It is concluded that the elevated plasma noradrenaline levels found in DU patients are not caused by the hypersecretion of acid per se, nor does there seem to be any general correlation between acidity and the catecholamine levels in peripheral plasma.
Catecholamines↗
The role of catecholamines in the control of gastric acid secretion during insulin hypoglycaemia and modified sham feeding.
The modified sham feeding procedure has been advocated to replace the insulin test as the method of choice for the assessment of denervation after vagotomy. One of the reasons for this changing attitude is the assumption that insulin hypoglycaemia causes non-cholinergic stimulation of acid secretion, possibly mediated via the sympatho-adrenal system. This study has therefore compared the effects of these two different tests on acidity and release of catecholamines into the circulation in 7 duodenal ulcer patients 6 weeks after highly selective vagotomy. It was found that insulin, but not modified sham feeding, caused a marked increase in plasma adrenaline and noradrenaline concentrations whereas gastric acid secretion was of a similar magnitude in the two tests. The results suggest that the increased plasma catecholamine levels during insulin hypoglycaemia do not influence gastric acid secretion in duodenal ulcer patients.
Adult↗
Diagnosis and treatment of phaeochromocytoma.
Hypertension due to a phaeochromocytoma is a rare but curable disease. The yearly incidence of phaeochromocytoma is about 5 per million inhabitants. Diagnosis can be based on urinary catecholamines in most patients but plasma catecholamines are sometimes helpful. Localization of the tumour is usually obtained by computerised tomography. Invasive investigations and operation are not undertaken before alpha-receptor blockade has been instituted. An extraperitoneal approach to the adrenals is preferred. More than 90% of patients are cured.
Adrenal Gland Neoplasms↗
Surgical treatment of primary aldosteronism.
An aldosterone-producing adenoma is the cause of primary aldosteronism in about 85%. Unilateral adrenalectomy cures the potassium wasting and normalizes the blood-pressure in more than 70%, independently of the severity or duration of the hypertension. The location of an aldosteronoma is best obtained by computerized tomography and selective venous catheterization with determination of the aldosterone/cortisol ratio. When the biochemical studies suggest hyperplasia, medical treatment is recommended. It is, however, our policy to remove an adrenal gland with hyperplasia, when catheterization demonstrates unilateral hyperfunction. At operation a posterior or a flank approach is preferably used.
Adenoma↗
Primary hyperparathyroidism. Changing trends over fifty years.
Two groups of patients with PHPT, one (early group) operated on between 1930 and 1960, and the other (late group) from 1972 to 1981 were compared, in order to find out the changing trends in this disease during these years. The number of operated PHPT increased when serum calcium and phosphate were added to routine blood screening. The mean age of patients was lower in the early than in the late group. There was a decrease in the occurrence and severity of bone and renal disease and an increase in the number of asymptomatic patients in the late group. In the early group the serum calcium levels were higher, serum phosphate levels lower and parathyroid adenomas larger. The ratio between uni- and multiglandular parathyroid disease was similar in both groups of patients.
Adenoma↗
The management of patients with primary aldosteronism.
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Adenoma↗
Adrenalectomy: anterior or posterior approach?
The relative merits of the anterior and posterior approaches to the adrenal gland were assessed in a series of 103 patients with bilateral cortical hyperplasia or unilateral cortical adenoma (less than 25 g). In none of the patients was additional concomitant surgery planned. Sixty-four patients were operated on through the anterior and 39 through the posterior approach. Iatrogenic injury of the spleen necessitated splenectomy in 9 (18 percent) of 49 patients undergoing left adrenalectomy anteriorly. In 10 (26 percent) of 39 patients operated on posteriorly, the pleural cavity was entered. Perioperative blood requirement and postoperative morbidity were lower when the posterior approach was used. The hospital stay was significantly shorter after both unilateral and bilateral adrenalectomy when operation was done through the posterior approach. The results of this study lend strong support to a posterior lumbar approach for adrenalectomy for small benign adrenal cortical lesions.
Adenoma↗
Adrenal surgery: trends during the seventies.
Surgical experience with adrenal disease from 1970 to 1979 was reviewed in 315 patients. The pathologic conditions that were encountered were hypercortisolism (74 patients), hyperaldosteronism (46 patients), adrenocortical carcinoma (35 patients), pheochromocytoma (77 patients), and nonfunctioning adenoma (47 patients). In addition, 5 patients with metastatic lesions, 14 with cysts, and 4 with myelolipoma were surgically treated. The accuracy of localizing adrenal lesions increased from about 50 percent to almost 100 percent during the decade studied. The increase was due mainly to the introduction of computerized tomography, the most important advance in the management of adrenal disease. The present study shows that adrenal surgery can be performed with low morbidity and mortality. Operative deaths were confined to patients with malignant disease or increased secretion of cortisol or catecholamines. Only patients with adrenocortical carcinoma (2 year survival probability, 34 percent) or hypercortisolism due to cortical hyperplasia (5 year survival probability, 76 percent) had significantly decreased survival.
Adrenal Gland Diseases↗
Localization of peptide YY (PYY) in gastrointestinal endocrine cells and effects on intestinal blood flow and motility.
In immunohistochemical studies using antisera to peptide YY (PYY), a 36 amino acid polypeptide isolated from porcine duodenum, it was found that PYY-like immunoreactivity occurred mainly in endocrine cells of the gastrointestinal mucosa. PYY-immunoreactive cells were particularly abundant in the distal intestine and have been observed in five species, including man. By radioimmunoassay it was found that, in the rat, the amount of PYY immunoreactivity was about 100-fold higher in the colon than in the duodenum. The chromatographic profiles of PYY immunoreactivity from the rat colon and porcine PYY on a SP-Sephadex ion exchanger were similar. Furthermore, serial dilutions of extracts from the rat colon and porcine PYY had parallel displacement curves in radioimmunoassay. Close intraarterial administration of PYY in cats caused an intestinal vasoconstriction and an inhibition of jejunal and colonic motility. Simultaneously there was a rise in systemic arterial blood pressure. These effects of PYY were also observed after pretreatment with adrenergic blocking agents. It is concluded that PYY is a gastrointestinal peptide that is present mainly in endocrine cells of distal intestine and that has effect on both intestinal motility and the cardiovascular system.
Animals↗
Metabolic and hormonal adjustments during hemorrhage in cats after interference with the sympatho-adrenal system.
The relative contribution of the splanchnic sympathetic innervation and the adrenal medulla for metabolism and hormone secretion during two different levels of hemorrhagic hypotension was investigated in 3 groups of anesthetized cats, viz, intact, adrenalectomized and splanchnicotomized (adrenalectomy + cutting of splanchnic nerves). In intact cats, hemorrhage caused very marked elevations of arterial plasma glucose, adrenaline, noradrenaline, dopamine, lactate, cAMP, glycerol and glucagon concentrations whereas plasma insulin fell to only 20% of control values. Adrenalectomy attenuated the glucose, adrenaline, noradrenaline and cAMP responses whereas the normal insulin inhibition was abolished. Splanchnicotomy further reduced the hemorrhagic glucose and glycerol responses and, possibly, also that of glucagon. It is concluded that the adrenergic system as a whole is important for the adjustments of the release of glucose, cAMP, glycerol, insulin and glucagon that occur during hemorrhage in cats. The adrenal medulla seems to be of particular importance for the regulation of cAMP release.
Adrenalectomy↗
Effects of food on plasma catecholamine and gastrin levels in patients with duodenal ulcer and normal volunteers.
Changes in plasma concentrations of adrenaline, noradrenaline, dopamine and gastrin in response to a standard meal were studied in 6 normal volunteers and 8 patients with chronic duodenal ulcer (DU) disease. Before the meal plasma gastrin and noradrenaline, but not adrenaline or dopamine, were higher in DU patients than in the controls. Food induced significant increments in plasma gastrin and noradrenaline concentration in both groups, whereas plasma adrenaline and dopamine levels remained unchanged. Plasma gastrin and noradrenaline concentrations were higher in DU patients than in the normal controls both during and after the meal. The results do not support the hypothesis that adrenaline is involved in the pathogenesis of duodenal ulcer disease, whereas the role of the increased plasma noradrenaline concentrations in this disease remains unclear.
Adult↗
Pheochromocytoma: current status and changing trends.
From 1971 through 1980, 106 patients underwent operations for pheochromocytoma. Twelve patients had a pheochromocytoma as a manifestation of the multiple endocrine neoplasia type 2 syndrome. Twenty patients were found to have extra-adrenal paragangliomas. Fifteen patients (14%) had malignant tumors; eight of these tumors were encountered in the group with extra-adrenal lesions. The cumulative survival probability at 5 years was 53%. Determination of urinary metanephrines and vanillylmandelic acid were the most sensitive diagnostic aids, with accuracy rates of 95% and 89%, respectively. In recent years, the measurement of fractionated urinary and plasma catecholamine levels has greatly increased the diagnostic accuracy. Preoperative localization of pheochromocytoma has improved during the last decade. Nephrotomography, selective angiography, and venous sampling have essentially been abandoned in favor of computed tomography. With an accuracy of more than 90%, computed tomography represents the major step forward in the overall management of pheochromocytoma during the past decade.
Adolescent↗