Parathyroid hyperplasia and carcinoma within one gland

A 47-year-old Scottish woman vacationing in the United States presented with a serum calcium level greater than 20 mg/dl and a parathyroid hormone level 16 times greater than normal after a one-week history of severe vomiting and unrelenting abdominal pain. Surgical exploration of the thymus revealed the very rare association of a large (7 by 4 by 0.8 cm) parathyroid carcinoma adjacent to apparently normal parathyroid tissue, separated by a thin fibrous band. Two other hyperplastic and one normal parathyroid glands were also identified. Postoperatively, the patient became hypocalcemic and, for the past nine months, has received maintenance 1-alpha-hydroxycholecalciferol therapy (1 microgram per day) with normal calcium and barely detectable parathyroid hormone levels.     

The University of Rochester Associated Hospitals program in internal medicine: Seven years experience with an innovative city-wide residency for preparation of general internists

The Associated Hospitals Program (AHP), with a total of 61 residents, engages the staff, patients and facilities of six hospitals and selected community health centers and private practices. The AHP is interlocked with separately accredited residencies in internal medicine in the participating hospitals and provides experiences, especially with ambulant patients, beyond those of more traditional programs in internal medicine. Strategies used by the AHP are minimally dependent upon grants from governmental and private sources for support of the basic three-year program. About one-third of the residents enter practice after completing the basic program; the larger share have a wide range of engagements in the fourth and fifth post-doctoral years for enhancement of clinical and teaching abilities and for critical study of health care. Eighty-seven, or 94 percent, of the 93 alumni are engaged in practice, and many also in teaching, of general internal medicine. Included in the 87 alumni are 12 of 18 residents who had two-year fellowships in medical subspecialties after completion of the AHP residency. The alumni have wide geographic distribution; 33 are practicing in communities with populations of less than 100,000 and 17 of these 33 are in communities of less than 25,000 persons.     

Thrombolytic therapy for acute transmural myocardial infarction. Intracoronary versus intravenous

The application of coronary angiography in coordination with streptokinase administration directly into the occluded coronary artery has served to focus attention on the clinical potential of such therapy. About 75 percent of patients with acute transmural myocardial infarction have been shown to have reperfusion after intracoronary administration of streptokinase. However, the data do not prove that the beneficial effect required regional perfusion. Analysis of biochemical data suggests that the active agent was not confined to the locale of the thrombus, but in fact circulated in significant concentration; furthermore, systemic (intravenous) treatment resulted in reperfusion of a significant proportion (50 percent) of coronary arteries as well. Comparative studies are needed to critically compare angiographic results after regional or systemic therapy and also to assess the impact of reperfusion on possible reduction in long-term morbidity and mortality. Although intracoronary therapy appears to be more effective for inducing reperfusion, intravenous therapy has the potential for greater clinical impact, since it can be instituted more quickly after the onset of symptoms and does not require specialized cardiac catheterization facilities.     

Noninvasive diagnosis of mitral prosthesis malfunction

We describe the utilization of echocardiography and phonocardiography in the diagnosis of malfunction of the Smeloff-Cutter mitral prosthesis in a patient in whom corrective surgery was subsequently performed without the necessity of cardiac catheterization studies. The noninvasive studies also delineated prosthesis malfunction as the etiology of pulsus alternans, noted clinically in this patient.     

Aortic regurgitation first appearing 12 years after successful septal myectomy for hypertrophic obstructive cardiomyopathy

Substantial aortic regurgitation developed in a patient with hypertrophic (obstructive) cardiomyopathy (HCM) who underwent septal myectomy. It was first noted 12 years after surgery. There was no evidence for surgical damage to the valve, subacute bacterial endocarditis, coexisting discrete subaortic stenosis or any other known etiology of aortic incompetence. This experience suggests that aortic regurgitation occasionally may be a late mode of deterioration in surgically treated patients with hypertrophic cardiomyopathy. Possible mechanisms for the development of aortic incompetence in such patients are discussed.     

Successful thrombolytic therapy in two patients with renal vein thrombosis

Thrombolytic therapy was administered to two patients with angiographically documented renal vein thrombosis. One patient with bilateral thrombotic disease complicating membranous glomerulonephritis was treated with systemic streptoklnase; the second patient had unilateral renal vein thrombosis associated with chronic pyelonephritis and was treated with streptokinase and urokinase administered directly into the involved vessel. Lysis of all thrombus material was demonstrated by follow-up renal venography and was associated with a gratifying improvement of clinical well-being and laboratory function. These cases demonstrate the feasibility of dissolving renal vein thrombi of presumed recent onset and suggest that rapid lysis of the thrombus accelerates functional and clinical recovery.     

Hereditary complement (C2) deficiency with dermatomyositis

A 60 year old white man in previous good health presented with a 6 month history of progressive muscle weakness. Clinical and laboratory findings were typical of dermatomyositis. Muscle biopsy confirmed the presence of inflammatory myopathy; deposits of immunoglobulin G (IgG), immunoglobulin M (IgM) or third component of complement (C3) were not detected by immunofluorescence. No evidence was found for an associated neoplasm.An unexpected finding was the total absence of serum hemolytic complement activity. Further investigation revealed that the complement defect was attributable to a selective and total absence of the second component of complement (C2), as determined by both functional and immunoprecipitin assays. Family studies indicated that the defect was inherited in an autosomal recessive manner, as has been observed in the previously reported C2-deficient kindreds.This case demonstrates that typical muscle lesions of dermatomyositis can occur in the presence of a complement defect which would preclude activation of the classic (C1-C4-C2) complement pathway. The case is of further interest as one of a series of recently reported associations of rheumatic diseases with hereditary complement deficiencies.Study of the functional properties of the propositus' C2-deficient serum demonstrated normal generation of chemotactic activity in the presence of endotoxin or aggregated IgG, and normal or near normal bactericidal activity against Salmonella typhi 0 901 and Hemophilus influenzae, type b. These findings emphasize the importance of the alternate (properdin) pathway of complement activation in these functions.     

Acidosis of synovial fluid correlates with synovial fluid leukocytosis.

The antibacterial activity of aminoglycoside antibiotics is significantly reduced by lowering the pH of the incubation medium. Since gram-negative septic arthritis responds poorly to aminoglycoside antibiotic therapy, we sought to determine whether synovial fluid acidosis contributes to this poor outcome. Synovial fluid samples from 22 patients with various forms of acute and chronic arthritis were examined for white blood cell count and pH. A close correlation (r = -0.92, p is less than 0.001) between an increasing white blood cell count and a decreasing pH was demonstrated. Since septic arthritis is associated with high white blood cell counts, in synovial fluid, the resultant low pH may contribute to the poor response to gram-negative septic arthritis treated with aminoglycoside antibiotics.     

Hypoglycemia as a provocative test of prolactin release

Insulin-induced hypoglycemia has been traditionally used to test growth hormone (GH) and cortisol reserve. In order to determine its usefulness as a provocative test for prolactin (PRL) release, 31 healthy men and women, 38 patients with definite pituitary abnormalities (pituitary tumors, 17; hypopituitarism [other causes]—complete, 4, or partial, 17), and 17 patients with suspected pituitary dysfunction (delayed puberty, 5; short stature, 4; secondary amenorrhea, 6; empty sella, 2, received regular i.v. insulin (0.05–0.15 U/kg), and the plasma was assayed serially for PRL, GH, cortisol, and glucose. In the 31 healthy subjects, PRL increased from 16.3 ± 1.8 ng/ml (mean ± SEM) to 45.5 ± 7.9 (p < 0.001) at 60 min and was still elevated at 120 min (25.8 ± 3.1 ng/ml). The maximal rise to 52.2 ± 8.0 ng/ml occurred between 40 and 90 min. There was no significant sex difference in the maximal PRL increase, maximal increment, or concentration at any time. In 21 of the 31 subjects, PRL increased at least 10 ng/ml with a doubling of baseline levels—criteria for a positive response. In addition, 12 of the healthy subjects received thyrotropin-releasing hormone (TRH) (500 μg i.v.) while 5 received chlorpromazine (50 mg i.m.). There was no significant difference among the maximal prolactin increments following insulin (36.7 ± 7.9 ng/ml), TRH (46.4 ± 6.3 ng/ml), or chlorpromazine (63.4 ± 21.9 ng/ml). In patients with definite pituitary abnormalities, 28 of 38 had diminished PRL release after insulin. Of these 28, 23 also had inadequate GH and 13 impaired cortisol release. In the 10 partially hypopituitary subjects with normal PRL responses, GH increased normally in 7 and cortisol in all. Thirteen of the 17 patients with suspected pituitary dysfunction had adequate PRL increases, while the GH and cortisol responses were intact in 16 and 17 subjects, respectively. Overall, the PRL response was concordant with changes in GH in 44 of 55 patients and in cortisol in 32 of 55 patients. It is concluded that insulin-induced hypoglycemia (1) releases PRL in most normal subjects and (2) is useful in determining the integrity of the hypothalamic pituitary axis for PRL release in patients with suspected abnormalities of pituitary function. Moreover, in combination with TRH, it may aid in localizing the site of abnormality in patients with these disorders.     

Systemic lupus erythematosus in patients with chronic renal failure

The clinical courses of 36 patients with systemic lupus erythematosus (SLE) in whom chronic renal failure developed and who required dialysis for more than three months were studied. At the time dialysis was initiated, 14 of 36 patients (38.9 percent) had clinically active SLE, but only three of 24 (12.5 percent) had activity in subsequent years while receiving dialysis therapy. In the majority of patients, however, renal disease progressed to end-stage despite clinical quiescence of SLE. During the follow-up period (mean +/- SD, 36 +/- 39.8 months), eight patients died--six from infections and two from cardiac disease. Actuarial survival rates at one, two, and five years after dialysis treatment were 91.1, 78.8, and 68.9 percent, respectively. This study suggests that the progression of renal disease to end-stage in patients with SLE may be mediated by nonimmunologic mechanisms as well as SLE-related immunologic insults. In most of these patients undergoing long-term dialysis, SLE remains clinically inactive despite persistent serologic abnormalities. Survival of the patients undergoing dialysis is comparable with that of the general dialysis population.     

Hypophyseal metastatic renal cell carcinoma and pituitary adenoma: Case report and review of the literature

A patient with a remote history of nephrectomy for renal cell carcinoma presented with a visual field defect. At surgery, metastasis from the hypernephroma and an adenoma were found coexisting in the anterior pituitary gland. Although hypernephromas are known to act as “recipient” tumors in cases in which two primary neoplasms coexist, it is unusual for a renal cell carcinoma to metastasize into another tumor.     

Renal response to mechanical ventilation in patients with chronic hypercapnia

Alkalosis in hypercapneic patients caused by abrupt respirator-induced reduction of the partial pressure of carbon dioxide leads to bicarbonate diuresis. Potassium is the predominant cation accompanying bicarbonate loss in the urine.The determining variable for induction of proportional changes in renal bicarbonate and potassium excretion is the partial pressure of carbon dioxide. The reduction of the partial pressure of carbon dioxide modifies bicarbonate regeneration directly by limiting the hydration reaction and facilitates potassium loss indirectly by reducing extracellular, and by inference intracellular, hydrogen ion activity.     

Age and prior caffeine use alter the cardiovascular and adrenomedullary responses to oral caffeine

The effects of age and chronic caffeine use (approximately 300 mg/day) on the cardiovascular and humoral responses to 250 mg of oral caffeine (the equivalent of 2 to 3 cups of coffee) were examined. Older subjects had greater increases in blood pressure than younger subjects (p less than 0.03), and caffeine nonusers had greater blood pressure increases than caffeine users, regardless of age (p less than 0.05). Caffeine increased the product of systolic blood pressure and heart rate (an estimate of myocardial oxygen demand) in older caffeine nonusers, but this effect was absent in older caffeine users (p less than 0.01). Cardiovascular effects of caffeine could not be related temporally to changes in plasma epinephrine, which were greater in caffeine nonusers and younger subjects, or to plasma norepinephrine, renin activity or vasopressin, which did not change. Thus, age accentuates and moderate prior caffeine use attenuates the cardiovascular effects of oral caffeine; these effects are not mediated solely through the sympathoadrenal system.     

Pituitary adrenal recovery following short-term suppression with corticosteroids.

To provide clinical guidelines for the use of high-dose short-term glucocorticoid therapy, we studied recovery of the hypothalamic-pituitary-adrenal axis in 10 normal men following the administration of suppressive doses of prednisone (25 mg twice daily for five days). Cortisol responses to insulin-induced hypoglycemia and synthetic ACTH before treatment were compared with responses two and five days after concluding the prednisone course when adrenal function was not influenced by the presence of exogenous steroid. Two days after prednisone therapy, peak cortisol responses to both hypoglycemia (11.0 +/- 0.9 microgram/dl mean +/- SEM) and synthetic ACTH (13.3 +/- 1.4 microgram/dl) were significantly reduced compared to pretreatment (20.6 +/- 1.6 and 27.3 +/- 2.5 microgram/dl, respectively, p less than 0.001). Five days after concluding the prednisone therapy, peak cortisol response to hypoglycemia had returned to near pretreatment levels although peak cortisol response in the adrenal gland to synthetic ACTH (22.3 +/- 1.1 microgram/dl) remained reduced (p less than 0.05). These data suggest that brief courses of high-dose prednisone therapy may limit the adrenal component of the hypothalamic-pituitary-adrenal response to stress for up to five days.