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Biomedical subjects

S G Sheps

Publications and source records attributed to S G Sheps.

At least 73 records · Page 4Linked to original sources

Chronic idiopathic anhidrosis.

We describe the cases of eight patients with chronic idiopathic anhidrosis. These patients were heat intolerant and became hot, flushed, dizzy, dyspneic, and weak but did not sweat when the ambient temperature was high or when they exercised. Four patients had preganglionic sudomotor lesions and in the remaining 4 the lesion appeared to be postganglionic. The patients did not have orthostatic hypotension, other evidence of generalized autonomic failure, or symptomatic somatic neuropathy. One patient regained thermoregulatory sweat function and no patient's condition progressed to generalized autonomic failure. Chronic idiopathic anhidrosis appears to be distinctly different from other autonomic neuropathies that tend to carry much poorer prognoses.

Adult↗

Gastrointestinal motility disturbances in patients with orthostatic hypotension.

Orthostatic hypotension syndromes may be associated with visceral denervation, resulting in disturbances of the gastrointestinal and urinary systems. This report analyzes the findings in 9 patients with neurogenic orthostatic hypotension and gastrointestinal symptoms. Antral and proximal small intestinal motor dysfunction was demonstrated in all patients: a significant reduction (p = 0.007) in the number of interdigestive motor complexes during 3 h of fasting (mean 0.4 vs. 1.3 controls); absence of gastric component in interdigestive motor complexes observed; incoordination of fasting and fed antral activity; and development of nonpropagating bursts of phase III-like activity in the intestine that were of at least 2-min duration, high amplitude and frequency, and associated with tonic elevations in baseline pressure. These motor abnormalities may provide a potential means for the diagnosis of impaired autonomic supply to the upper gut.

Adult↗

Use of 131I-MIBG scintigraphy in the evaluation of suspected pheochromocytoma.

Studies at the University of Michigan have shown that 131I-metaiodobenzylguanidine (131I-MIBG) is an effective agent for the diagnosis and localization of pheochromocytomas and paragangliomas. We conducted a study that confirmed and expanded that finding. From January 1983 to March 1984, 48 patients at our institution had 51 131I-MIBG scans during the workup of suspected sporadic or metastatic pheochromocytoma. Scintigrams were obtained after 500 microCI of 131I-MIBG had been administered intravenously. The final diagnosis (true-positive, false-negative, or false-positive result) was made at operation and pathologic examination. A true-negative diagnosis was confirmed by normal plasma and fractionated urinary levels of catecholamines and metabolites and, in most patients, computed tomography (CT). There were 20 true-positive studies (6 pheochromocytomas, 4 paragangliomas, and 10 metastatic or recurrent pheochromocytomas) and 24 true-negative studies. One patient with a suspected recurrent paraganglioma near the bladder had a false-positive 131I-MIBG scan (and also a false-positive (CT). Among six patients with false-negative scintigrams (three pheochromocytomas, one paraganglioma, and two metastatic lesions), one also had a false-negative CT. The overall sensitivity of 131I-MIBG scanning was 77%, specificity was 96%, and accuracy was 86%. This test is fairly sensitive in the workup of patients with known or suspected recurrent or metastatic pheochromocytoma. It may also be helpful in the evaluation of suspected sporadic pheochromocytoma when CT findings are normal.

3-Iodobenzylguanidine↗

Two-dimensional echocardiographic detection of mediastinal pheochromocytoma.

A patient had a functioning pheochromocytoma in the aorticopulmonary region of the middle mediastinum. Two-dimensional echocardiography, utilizing the suprasternal approach, was useful in detecting the tumor and in defining its anatomic relationships to other vascular structures within the mediastinum.

Adult↗

Bilateral subtotal adrenal resection for bilateral pheochromocytomas in multiple endocrine neoplasia, type IIa: a case report.

Our treatment philosophy for the management of the adrenal glands in patients with multiple endocrine neoplasia, type IIa has been bilateral total adrenalectomy. In the patient described, exceptional and pressing reasons necessitated preservation of adrenocortical function. Bilateral pheochromocytomas were resected with preservation of the adrenal cortices. Adrenocortical function was normal 36 months after resection. Although the patient remained symptom free, elevated basal immunoreactive plasma calcitonin levels suggested the presence of residual or metastatic medullary thyroid carcinoma. There was no evidence of recurrent pheochromocytoma.

Adrenal Gland Neoplasms↗

Evaluation of pindolol dosage in hypertension by automatic indirect BP monitoring.

Pindolol, a nonselective beta-adrenergic blocking drug, lowered systolic and diastolic BP equally well during once-daily and twice-daily dosage. Side effects were few. Absence of supine bradycardia distinguished this drug from other beta-adrenergic blockers and likely was attributable to the agent's intrinsic sympathomimetic activity. Blood pressure variability was less during therapy. Automatic indirect BP monitoring reliably confirmed office and home BP recordings and indicated good control throughout waking and sleeping periods.

Adult↗

Catecholamine-secreting paraganglioma of the interatrial septum.

A 29-year-old woman was found to have clinical and biochemical evidence of a functioning paraganglioma (pheochromocytoma) five years after the onset of symptoms. Despite extensive investigation of known potential sites of paragangliomas (including laparatomy), the suspected tumor eluded localization. Adrenergic blocking agents controlled her blood pressure but did not affect the other manifestations of catecholamine excess. Ischemic perforation of the colon and refractory gram-negative sepsis resulted in death. At autopsy, a large paraganglioma was found within the heart; the unique tumor location was an important factor in the fatal clinical course.

Adult↗

Pheochromocytoma in the pediatric age group: current status.

Pheochromocytoma is an unusual tumor in the pediatric age group. Several aspects serve to differentiate children with pheochromocytoma from their adult counterparts. Children have fewer malignant tumors, more extraadrenal tumors, and greater bilaterality and multiplicity of tumor. Similarly, they have an increased incidence of multiple endocrine neoplasia (MEN) and familial disease. The records of 16 children (age 17 years and younger) with pheochromocytoma were evaluated. One newborn died at 36 hours of age, and a 3-year-old died four days after exploratory laparotomy. Of the 14 survivors (with an average follow-up of 8 years), three had manifestations of multiple endocrine neoplasia type 2 and two had familial pheochromocytoma only. One patient had malignant paragangliomas. Three patients had bilateral tumors; one of these had MEN. Four patients underwent operation before the use of alpha- and beta-adrenergic blockade was routine at our institution. An additional patient received blockade in preparation for the removal of the pheochromocytoma but was not prepared for preoperative angiography. Hypertensive complications were encountered in four of these five children; in one, this complication was fatal. There were no intraoperative complications in the 12 patients who had received appropriate blocking agents. Eleven of the 14 survivors are currently normotensive without the need for antihypertensive medication. Two patients have medically controlled hypertension, and one continues to have severe hypertension. An overview of this series underscores the characteristic features of pheochromocytoma in the pediatric age group. Angiography has been replaced by high-resolution computed tomography for localization.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Pheochromocytoma: value of computed tomography.

A review of 60 histologically proved pheochromocytomas confirmed the value of computed tomography (CT) in the evaluation of this tumor. CT was found to be an accurate means of locating the tumor in 52 patients presenting for the first time and 8 patients with evidence of recurrence.

Adolescent↗

Occurrence of pheochromocytoma in Rochester, Minnesota, 1950 through 1979.

For the 30 years from 1950 through 1979, the population of Rochester, Minnesota, averaged 45,800, resulting in almost 1.4 million person-years of observation. During that time, 11 cases of pheochromocytoma were diagnosed in this population; thus, the average annual incidence rate was 0.8 per 100,000 person-years. With the inclusion of two additional diagnosed familial cases, the incidence rate would be 0.95 per 100,000 person-years. Five of the 11 cases were diagnosed initially at autopsy.

Adrenal Gland Neoplasms↗

Digital ischemia associated with small bowel malignancy.

A 59-yr-old woman presented with digital ischemia and was found to have a small-bowel malignancy. After resection of the neoplasm, her digital ischemic symptoms resolved. Five years after the operation, she has no evidence either of recurrence of the intestinal malignancy or of the digital ischemia. This report contrasts with earlier studies, which have suggested that patients with digital ischemia associated with malignancy have a poor prognosis.

Adenocarcinoma↗

Pindolol--a new beta-adrenergic blocking agent with intrinsic sympathomimetic activity in the management of mild and moderate hypertension.

Pindolol, a well-tolerated noncardioselective beta-adrenergic blocking drug, effectively reduced blood pressure in patients with mild to moderate hypertension without causing orthostatic hypotension. In common with other beta-adrenergic blocking agents, pindolol blocked the normal increase in the standing pulse rate. The drug, however, did not decrease the supine pulse rate, a feature that can be interpreted as evidence of the postulated intrinsic sympathomimetic activity of pindolol.

Adolescent↗

Paragangliomatosis with numerous catecholamine-producing tumors.

Catecholamine-producing tumors may be found wherever paraganglion tissue exists, and they may be associated with a variety of conditions (multiple endocrine neoplasia types 1, 2a and 2b). The patient described in this report had 21 paragangliomas removed between the ages of 13 and 17 years, and she has evidence of additional tumors. This is the greatest number of paragangliomas reported, and the tumors involved numerous sites. In addition, the patient has multiple congenital anomalies that have not been reported previously in association with paraganglioma. Unifying theories of pathogenesis and several features in the management of this patient are discussed.

Abnormalities, Multiple↗

Malignant nonfunctioning paraganglioma of the retroperitoneum producing renovascular hypertension.

Malignant nonchromaffin paraganglioma (chemodectoma) is an unusual tumor arising from chemoreceptor cells derived from the neural crest. Although these tumors generally occur in the head and neck, where the term "carotid body tumor" applies, more than 20 cases of retroperitoneal origin have been described. This case report presents the clinical, radiographic, and pathologic features of a chemodectoma arising in the retroperitoneal area that produced severe hypertension by unilateral real vascular compression. The patient underwent exploratory laparotomy, and a left nephrectomy was done. Follow-up examination 1 year after surgery disclosed no clinical evidence of disease, and he had normal blood pressures without medication.

Adult↗