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Biomedical subjects

S G Sheps

Publications and source records attributed to S G Sheps.

At least 91 records · Page 5Linked to original sources

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↗

Isolated renal artery dissection, presentation, evaluation, management, and pathology.

Isolated nontraumatic renal artery dissection is rare. In this communication, 35 cases are presented; 24 cases (group 1) (22 male, 2 female) were diagnosed angiographically, and 11 (group 2) (10 male, 1 female) were observed at autopsy. In group 1, 23 of the patients were hypertensive when they were first seen, and in 17 of them the hypertension was of recent onset. Additional presenting signs and symptoms included flank pain (10 patients), gross hematuria (5), and headaches (6). Renal function was satisfactory. Renal vein renin levels could be lateralized in 8 of 16 patients. Isotope renograms performed in 18 patients, showed unilateral abnormalities in 7, bilateral abnormalities in 6, and normal results in 5. Angiograms showed that the dissection was unilateral in 18 cases and bilateral in 6 cases. Fibromuscular dysplasia was observed radiographically in 22 cases and was bilateral in 12. In group 1, 13 patients were treated with antihypertensive medication only, and 11 underwent operation. At follow-up (mean 52.0 months), the mean blood pressure were 128/88 mm Hg and 139/89 mm Hg for the medical and surgical groups, respectively. Eleven medical and nine surgical patients continued to require antihypertensive drugs at follow-up. Among the 11 patients in group 2, only 4 were hypertensive. In only one case the dissection may have contributed significantly to the patient's death. These studies indicate that isolated nontraumatic renal artery dissection most commonly occurs in young men with coexistent fibromuscular dysplasia. Hypertension is commonly present and therapy should be directed toward its control. In this study, blood pressure control was effectively accomplished with medical therapy.

Adult↗

Acromegaly and pituitary adenoma with phaeochromocytoma: a variant of multiple endocrine neoplasia.

Two women ahd acromegaly due to a pituitary adenoma associated with phaeochromocytoma. Eight additional patients with this combination of tumours have been described by others. Our first patient had sustained hypertension, mild hypercalcaemia, and elevated basal levels of parathyroid hormone and calcitonin associated with malignant phaeochromocytoma and parathyroid hyperplasia. The second patient had episodic hypertension and normal basal serum calcium, parathyroid hormone, and calcitonin levels with a benign cystic phaeochromocytoma. Four of the ten patients died from causes related to the phaeochromocytoma. Three patients had parathyroid hyperplasia. A separate group of four patients with phaeochromocytoma and pituitary adenoma without acromegaly has also been reported. These fourteen patients probably represent a non-familial variant of the multiple endocrine neoplasia syndrome. Our findings suggest that acromegalic patients with hypertension should be screened for phaeochromocytoma.

Acromegaly↗

Midodrine. A new agent in the management of idiopathic orthostatic hypotension and Shy-Drager syndrome.

Five patients with idiopathic orthostatic hypotension, two of whom had central nervous system manifestations (Shy-Drager syndrome), were treated with midodrine, an investigational alpha-adrenergic agonist, in a dosage of 2.5 to 5 mg three times daily. Significant improvement was observed in the standing systolic and diastolic blood pressures at the end of 1 week of therapy in all patients and has been maintained for 10 to 15 months in all patients. No symptomatic side effects were noted. Supine systolic hypertension occurred in two patients and was treated with small doses of metoprolol.

Adult↗

Prevalence of clinically unsuspected pheochromocytoma. Review of a 50-year autopsy series.

Pheochromocytoma is surgically curable, and lethal complications often ensue when the diagnosis is not made. We review 54 autopsy-proven cases of pheochromocytoma seen at the Mayo Clinic over the 50-year period 1928-1977. There were 31 females and 23 males, and the patients' ages ranged from 40 hours to 92 years (mean, 53 years). Pheochromocytoma was multiple in 10 patients (19%), extra-adrenal in 5 (9%), and malignant in 6 (11%). In 13 (24%) pheochromocytoma had been correctly diagnosed in life--after the investigation of hypertension in 8 patients, incidentally at laparotomy for unrelated conditions in 4 patients, and in association with the multiple endocrine neoplasia syndrome in 1 patient. In the group of 41 patients (76%) in whom pheochromocytoma had not been suspected clinically, hypertension also was a common finding (22 patients, 54%). Symptoms such as headache, sweating, and palpitations were nonspecific, but all occurred more often among the group of patients in whom the diagnosis had been made. For both groups, hypertensive or hypotensive or hypotensive crisis precipitated by surgery for unrelated conditions was a common cause of death. Prevention of such deaths requires a high degree of clinical alertness and biochemical screening tests for pheochromocytoma in patients with labile or accelerated hypertension.

Adolescent↗

Orthostatic hypotension.

This article reviews the physiology and pathology of orthostatic blood pressure regulation. It describes the diverse clinical disorders of postural blood pressure adjustment, focusing attention on idiopathic orthostatic hypotension and the Shy-Drager syndrome, with emphasis on clinical aspects, pharmacology, and pathologic anatomy. Proposals are made for the diagnostic evaluation of patients with orthostatic hypotension and for steps to be taken in therapeutic management.

Adult↗

Antihypertensive agents: role of therapeutic drug monitoring.

Hypertension is a disease more common to advanced societies, possibly due to diet and life style. It is a disease that can be readily controlled, and active efforts to decrease blood pressure correlate with decreased incidence of cardiovascular disease. Important in the armamentarium of the physician dealing with hypertension are a variety of drugs. The common drugs include diuretics (thiazides, loop of Henle diuretics, potassium-sparing diuretics), beta-blockers, alpha-agonists, neuron-blocking drugs, and vasodilators. The role of monitoring the blood concentration of each of these drugs in the interest of enhancing clinical usefulness is reviewed in light of the pharmacology of each drug group. We conclude that clonidine, guanethidine, hydralazine, and prazosin have properties that lend themselves to monitoring. Propranolol, metoprolol, nadolol, and alpha-methyldopa are drugs that have pharmacological properties that complicate monitoring. The drugs reserpine and minoxidil have properties that preclude monitoring, and the diuretics should only be monitored under unusual circumstances.

Antihypertensive Agents↗

Evaluation of the Del Mar Avionics automatic ambulatory blood pressure-recording device.

We have the opportunity to evaluate a portable ambulatory device for the recording of sequential indirect arterial blood pressure and continuous electrocardiogram (Del Mar Avionics automatic ambulatory blood pressure recording device). With careful attention to technique, only 11% of systolic and 5% of diastolic blood pressure readings differed by more than 10 mm Hg as compared with a trained technician's observations simultaneously in the same arm. The device has been useful in the evaluation of borderline (labile) hypertension, hypertensive drug therapy programs, and various episodic cardiovascular phenomena--for example, vasodepressor syncope and pheochromocytoma crises.

Adult↗

Prospective study of immune response to hydralazine and development of antideoxyribonucleoprotein in patients receiving hydralazine.

To examine the relationship between the immune responses to hydralazine, a drug known to induce systemic lupus erythematosus, and to deoxyribonucleoprotein (DNP) we followed prospectively 21 hypertensive patients treated with hydralazine for the first time. Within one year, antibodies to hydralazine developed in 16 of these patients and anti-DNP in seven of these. In one patient whose serum had a positive antinuclear antibody test prior to treatment, a mild hydralazine systemic lupus erythematosus syndrome developed preceded by rises in the levels of both anti-hydralazine and anti-DNP. Studies by radioimmunoassay on serums of three additional patients, not followed in this study but known to have hydralazine-induced systemic lupus erythematosus, revealed no evidence for either (1) cross-reactivity between anti-DNP and anti-hydralazine or (2) antibodies specific for a hydralazine-DNP complex. In some way, perhaps related to the mechanism by which carrier molecules enhance the immunogenuity of haptens, hydralazine increases the antigenicity of DNP. This effect depends on the development of immunity to hydralazine as well.

Antibodies↗

Familial pheochromocytoma and islet cell tumor of the pancreas.

The occurrence of pheochromocytoma(s) or pancreatic islet cell tumor(s), or both, in two or more members of three unrelated families in a manner consistent with autosomal dominant inheritance suggests that this tumor association is a genetically determined syndrome. Among 11 affected patients (aged five to 53 years), 10 had pheochromocytoma (bilateral in six), four had islet cell tumor (multicentric in one), and three had both tumors. Clinical presentation was due to pheochromocytoma in 10 cases (symptoms or signs commencing before age 10 years in three patients) and islet cell carcinoma in one case. Four patients are dead as a result of the tumors--two from pheochromocytoma and two from islet cell carcinoma.

Adenoma, Islet Cell↗

Differences between sporadic pheochromocytoma and pheochromocytoma in multiple endocrime neoplasia, type 2.

We studied the gross and microscopic pathology of the adrenal gland in 69 cases of sporadic phenochromocytoma in order to develop a profile of the neoplasm to compare with that of pheochromocytoma observed in the syndrome of multiple endocrine neoplasia, type 2 (MEN 2). The results showed that sporadic pheochromocytoma was a unicentric (93%), unilateral (100%) neoplasm, which was associated with normal extratumoral adrenal medulla (100%). The findings contrast with those encountered in the adrenal gland in MEN 2, in which the tumor involvement is frequently multicentric, usually bilateral, and associated with extratumoral medullary hyperplasia in cases of early involvement. Therefore, the interpretation of the results of pathologic examination of a pheochromocytoma should be immediately communicated to the surgeon.

Adolescent↗

Saralasin infusion in renovascular hypertension: increased response rate in seated patients.

Most series report a significant percentage of nondepressor (negative) responses to saralasin infusions in patients with surgically curable renovascular hypertension. Usually the infusions have been performed with the supine position. In the present study saralasin infusions were done in 33 acutely sodium-depleted hypertensive patients--17 with renovascular and 16 with the essential variety. Most patients were infused in the seated and supine positions to test the hypothesis that there might be a higher incidence of positive (vasodepressor) responses to the saralasin infusion when patients are in a sitting position. The results did, in fact, show a larger number of positive responses to saralasin in the seated patients with renovascular hypertension than in those in the supine position (88% versus 71%). There was a modest increase in the rate of false-positive responses in the patients with essential hypertension who were infused in the seated position (31% versus 23%). However, inasmuch as the saralasin infusion test is proposed as a screening procedure for renovascular hypertension, some false-positive responses are acceptable.

Angiotensin II↗

Extra-adrenal intrathoracic functioning paraganglioma (pheochromocytoma) in childhood.

Extra-adrenal pheochromocytomas are more common in children (30%) than in adults (10%). Of the extra-adrenal sites, the intrathoracic site is the most rare. A 15-year-old boy had a pheochromocytoma successfully removed from the left paraspinal region of his chest. Four years before removal, he had radiation therapy of 3,500 rads to the tumor because of its apparent nonresectability. After therapy, there was transient clinical and biochemical improvement. Preoperative angiography and computed tomography helped define the anatomy of the tumor vessels and the relationship to the thoracic aorta of the tumor. These studies also aided in discounting any other site for the pheochromocytoma, either adrenal or extramedullary. A slow-growing pulmonary metastatic lesion was subsequently identified and successfully excised.

Adrenal Gland Neoplasms↗

The Mayo Three-Community Hypertension Control Program. III. Outcome in a community-based hypertension clinic.

As part of a broader community program to evaluate approaches to hypertension control, a Community Hypertension Clinic, staffed by two nurse practitioners, was set up in a rural community. Hypertensive persons were identified either by an initial central blood pressure screening or by a subsequent home screening. Slightly more than half of the hypertensive patients at initial screening, or 256 persons, elected to go to the Community Hypertension Clinic for second-stage screening, whereas the remainder elected to see their physicians or to do neither. After secondary screening at the Clinic, 120 patients eventually came under care and were managed by the nurse practitioners. After 2 years of follow-up, 57% of the Clinic patients had office-recorded diastolic blood pressures of less than 90 mm Hg. The Community Hypertension Clinic dropout rate was only 5% after 30 months of operation, for participants whose duration of follow-up ranged from 12 to 27 months (median 16 months), when a repeat home blood pressure screening examination was performed. Comparison of outcomes was thus possible between persons who attended the Community Hypertension Clinic and those who were referred to their physicians' offices. Persons with more severe hypertension most often elected to go to the Clinic, whereas patients with milder degrees of hypertension tended to go to their private physicians for follow-up or failed to make the recommended second-stage screening contact altogether. Greater declines in blood pressure were observed in the Clinic group.

Adult↗

Analysis for urinary catecholamines by liquid chromatography with amperometric detection: methodology and clinical interpretation of results.

A method is presented for the quantitative analysis of urinary unconjugated norepinephrine, epinephrine, and dopamine as discrete entities. The procedure requires initial purification of the specimen on aluminum oxide and a boric acid-gel. We used "high-performance" reversed-phase paired-ion chromatography, with a flow-through amperometric cell as the detector. The CV was 6% for determination of norepinephrine, 11% for epinephrine, and 6% for dopamine monitored at physiologic concentrations of these compounds in urine. In a population study, urine specimens from 117 normal pediatric and adult subjects, 85 hypertensive patients, and 22 patients with surgically proved pheochromocytoma were analyzed. The specificity of the method for detection of pheochromocytoma was 100%, with a sensitivity of 97%.

Adolescent↗