Sheldon G. Sheps

Carotid body tumor (chemodectoma): Clinicopathologic analysis of ninety cases☆

Abstract The clinicopathologic findings in ninety cases of carotid body tumors seen at the Mayo Clinic from 1931 through 1966 are compared with those in 500 reported cases in the literature. In the ninety cases reviewed here, there were sixty-two men and twenty-eight women. In twenty cases surgery had been performed elsewhere; seventy patients were operated on here, with total excision in forty-nine, including one patient with bilateral tumors. The over-all surgical mortality rate was 5.7 per cent, usually related to carotid arterial damage or ligation. There were no tumor recurrences, although cervical node metastasis was noted in one patient and spinal metastasis in another. A classification was suggested for future surgical cases. Group 1 tumors are relatively small and minimally attached to the carotid vessels. Surgical excision usually can be carried out without difficulty. Group 2 tumors are usually larger and show moderate arterial attachment. These tumors are amenable to careful surgical removal. Group 3 tumors are usually large and incarcerate the carotids. Here the tumor must be approached with great care and vessel replacement should be considered.

Long-term follow-up study of periarteritis nodosa

Abstract The records of 130 patients with histologically proved periarteritis nodosa seen at the Mayo Clinic from 1946 through 1962 were reviewed. Intensive corticosteroid or ACTH therapy had been given to 110 of these patients. The expected survivorship, calculated by the life-table method, revealed a five year survival of 48 per cent for the treated patients and 13 per cent for the untreated ones. The presence of hypertension or renal disease at the initial examination seemed to worsen the prognosis. Both disorders were less frequent in patients who had received steroid therapy early in the course of their disease. Subdivision, on clinical grounds, into those with and without pulmonary involvement appeared to have no value in regard to the prognosis. Early and vigorous steroid therapy was of significant value in improving the results in patients with periarteritis nodosa. The majority of patients had to continue this treatment on a long-term basis to suppress symptoms or to prevent exacerbations. Side effects were infrequent.

Renal artery stenosis: A clinical-pathologic study in normotensive and hypertensive patients

Abstract Moderate or severe renal artery stenosis was found in 53 per cent of 295 unselected patients examined at necropsy; it was present in 49 per cent of 256 normotensive patients and in 77 per cent of thirty-nine hypertensive patients. Severe stenosis of the renal arteries was uncommon in normotensive patients less than fifty years of age; thereafter, the frequency increased with increasing age. Afferent arteriolar sclerosis of a moderate or severe degree was uncommon in normotensive patients less than sixty years old. The presence of atheromatous narrowing of the main renal arteries does not necessarily indicate a causal relationship to the presence of systemic hypertension.

Renal arteriovenous fistula: A reversible cause of hypertension and heart failure

Abstract A case of renal arteriovenous fistula is presented in which radioisotope renography and differential studies of renal function strongly suggested the presence of ipsilateral renal ischemia, subsequently confirmed. Another case, due to hypernephroma, is described in which aorticorenal arteriography revealed vascular lakes within the tumor, together with marked extrarenal venous dilation. Fifty-three previously reported cases of renal arteriovenous fistula are reviewed in detail, with special attention to the relationship of diastolic hypertension to the renal and vascular pathology.

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.

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.

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.

Long-Term Follow-up Study of Periarteritis Nodosa.

Excerpt During the 16 years ending December 31, 1962, 130 patients with a histologic diagnosis of periarteritis nodosa were treated at the Mayo Clinic. The greatest incidence was in the fourth and ...