Thoracic outlet syndrome with tetany of the hands.

A patient presenting with tetany was found to have thoracic outlet obstruction. This was treated by transaxillary first rib resection, and good relief of the carpal spasm was obtained. The mechanism whereby neurovascular compression may produce tetany is discussed.     

Cushing's disease with cure by resection of a pituitary adenoma. Evidence against a primary hypothalamic defect

A patient with Cushing's disease was treated by transphenoidal resection of a pituitary adenoma. Pituitary-adrenal suppressibility, responsiveness to metyrapone and circadian rhythm were observed postoperatively. Reversion to physiologic regulation after resection of an adenoma implies that the lesion secreted ACTH autonomously (primary hyperpituitarism). Current evidence favors the pituitary adenoma, when present, as the primary etiologic factor in pituitary-dependent Cushing's syndrome. Since adenomas occur in a majority of cases, initial therapy is best directed at the pituitary.     

Variables associated with changes in spirometry in patients with obstructive lung diseases.

One hundred fifty subjects were enrolled in a long-term study of obstructive lung diseases; 84 of these were subjected to five or more spirometric studies over a period of two or more years. Stepdown regression analysis was performed to determine the association between many different variables and the annual rates of change in the forced vital capacity (FVC) and forced expiratory volume in 1 second (FEV1). The following associations were noted to be significant (p less than 0.03); more favorable rates of change of the FVC and FEV1 were associated with a higher alpha1-antitrypsin level and older age. Less favorable changes were associated with more years of cigarette smoking, more airway reactivity and more frequent lower respiratory tract illnesses.     

Risk factors for postoperative pneumonia

Prospectively studied were 520 patients undergoing elective thoracic, upper abdominal and lower abdominal surgeries to analyze risk factors for postoperative pneumonias.Over-all, pneumonias developed in 91 of the 520 patients studied (17.5 percent). The acquisition of pneumonia was highly associated with preoperative markers of the severity of underlying diseases such as low serum albumin concentrations on admission (P < 0.005) and high American Society of Anesthesiologists pre-anesthesia physical status classification (P < 0.0001). History of smoking (P < 0.001), longer preoperative stays (P < 0.0001), longer operative procedures (P < 0.0001) and thoracic or upper abdominal sites of surgery (P < 0.0001) were also significant risk factors for postoperative pneumonias. Although massive obesity, old age and male sex were also associated with increased incidences of pneumonia, statistical significance was lost when these variables were controlled for site or duration of surgery.We were able to identify risk factors for pneumonia and to define a subpopulation of patients in which the risk of pneumonia was negligible. The acquisition of pneumonia by a low-risk patient should alert the physician to the possibility of a potentially preventable nosocomial infection.     

Predictors of survival in subjects with chronic airflow limitation

In a study of chronic airflow limitation, we followed 140 subjects living in Utah at altitudes of 1,300 to 1,500 meters for seven to 13 years. Twelve-year survival probabilities were determined and compared with an age- and sex-matched Utah population. The lowest 12-year survival probability was 0.40 for those patients with a forced expiratory volume in one second/forced vital capacity ($\text{FEV}{}_1\text{FVC}$) of less than or equal to 0.40, indicating that there is much variability in survival. Other indicators of a lower survival probability (and increased death risk ratio) were an FEV₁ percent predicted less than or equal to 50, an FEV₁ less than or equal to 1.5 liters, male gender, partial pressure of oxygen (P_O2) [exercise] less than or equal to 50 mm Hg, partial pressure of carbon dioxide (P_CO2) [rest] greater than 39 mm Hg, P_CO2 (exercise) greater than 39 mm Hg, FVC percent predicted less than or equal to 80, P_O2 (rest) less than or equal to 55 mm Hg, and a carbon monoxide diffusing capacity (DL_CO) percent predicted less than or equal to 80. Current smokers had a poorer survival probability than the reference population and an increased death risk when compared with the nonsmokers in the study. Pack/years of smoking also affected survival. Other variables associated with reduced survival were a diagnosis of chronic bronchitis combined with emphysema, more rapid annual declines in the FEV₁ and/or FVC, low alpha₁-antitrypsin levels, a 20 percent improvement in FEV₁ following the use of a bronchodilator aerosol, and a lower socioeconomic class. Differences between these findings and those noted in other studies are in the main due to differences in the characteristics (such as age, diagnosis, and extent of disease) of the patients in the study populations. The findings have relevance in estimating a patient's prognosis and for developing guidelines for disability determination purposes.     

Goodpasture's syndrome in a patient with the nail-patella syndrome

A patient with the nail-patella syndrome in whom end-stage renal failure developed as the result of Goodpasture's syndrome is described. Lesions characteristic of both rare diseases were seen on renal morphology. It is postulated that the glomerular membrane alteration of the nail-patella syndrome predisposed to the development of antiglomerular basement membrane antibody and hence Goodpasture's syndrome. A review of the incidence of renal failure in the nail-patella syndrome suggests that renal involvement can no longer be regarded as benign and that immune mechanisms may be related to progressive renal disease in some cases.     

Pathogenesis of hypertension in Cushing's syndrome

Steroid production, plasma renin activity (PRA) and plasma renin substrate (PRS) were measured in eight patients with hypertension due to Cushing's syndrome of benign origin. Despite elevation of cortisol secretion in all patients, hypokalemia and suppressed PRA was noted in the one subject with a functioning adrenal adenoma. PRA was normal in six patients on an unrestricted sodium intake but was markedly increased in the two patients on low salt diets. PRS was significantly increased during active disease, but decreased substantially with treatment. The absence of uniform hypokalemia and of suppression of renin indicates that mineralocorticold production could not account for the increase in arterial pressure. It is suggested that glucocorticoid-induced hypertension may be initiated by alterations in vascular responsiveness to pressor agents and that elevated PRS levels may contribute to increased angiotensin formation.     

The malignant potential of the dysgenetic germ cell in Klinefelter's syndrome

We report two cases of extragonadal germ cell tumors in patients with Klinefelter's syndrome and review the other cases of germ cell tumors reported in this syndrome. Especially in choriocarcinoma, the presence of Klinefelter's syndrome may be overlooked because of the similar clinical presentations and testicular histology. We recommend karyotypes be carried out in patients with germ cell tumors since the dysgenetic gonad of Klinefelter's syndrome may be important in the pathogenesis of these malignancies. Also patients with Klinefelter's syndrome should be followed closely because of their increased risk of development of germ cell tumors.     

Thyroid disease in children. A survey of subjects potentially exposed to fallout radiation

We found no significant difference in any type of thyroid disease between children in Utah and Nevada exposed to fallout radiation in the 1950's and control groups living in Utah and Arizona at present. Of the 5,179 children surveyed, thyroid abnormalities were found in 201. Adolescent goiter was the most common disorder with a prevalence of 15 per 1,000 for boys and 20 per 1,000 for girls. There were seven cases of hyperthyroidism, and the rates for lymphocytic thyroiditis were 8 and 16 per 1,000 for boys and girls, respectively. Twenty benign neoplasms and two carcinomas of the thyroid were found.Only 6 of the 201 children with thyroid disease knew of their disease prior to our examination. The inapparent thyroid disease discovered in this group of children through meticulous examination may have its counterpart in other populations as well. This study demonstrates the need, therefore, for careful examination of thyroids even in apparently healthy children.     

A nationwide outbreak of Reye's syndrome: Its epidemiologic relationship to influenza B

Between December 15 and June 30, 1974, 379 cases of confirmed Reye's syndrome were reported to the Center for Disease Control. Of these, 316 occurred during February and March 1974. A simultaneous surveillance system for influenza B indicated that this clustering of cases of Reye's syndrome correlated both temporally and geographically with influenza B outbreaks. The incidence of Reye's syndrome was higher in rural than in urban centers. Epidemiologically, two groups of cases of Reye's syndrome emerge: those which occur in older children (median age 11 years), cluster in time and geographic region, and are associated with antecedent influenza B infection; and those which occur sporadically throughout the year, are isolated in occurrence, occur in younger children (median age 6 years), and are associated with a wide variety of antecedent viral illnesses.     

Goodpasture's syndrome: Failure of nephrectomy to cure pulmonary hemorrhage

Two patients with Goodpasture's syndrome are described. The diagnosis was typical both on clinical and pathologic evidence. Both patients presented with pulmonary symptoms with hemoptysis and suffered from rapidly progressive and irreversible glomerulonephritis. Histologic study of the lungs in one patient demonstrated extensive intra-alveolar hemorrhage. Histologic examination of the kidneys in both patients disclosed changes of rapidly progressive glomerulonephritis with epithelial crescents. Immunofluorescent studies showed linear staining of the glomerular capillary basement membrane with (IgG) and (C′₃). Bilateral nephrectomy was carried out in both patients in an attempt to control the pulmonary manifestations. There was no improvement in the first patient and he died 2 days after the operation. The nephrectomy has not prevented recurrence of the pulmonary hemorrhage in the second patient in whom the lungs were less severely diseased; he is maintained on longterm hemodialysis, and the pulmonary disease is best controlled by corticosteroids. Bilateral nephrectomy is not always as successful as has been claimed in previous reports.     

Extralymphatic Hodgkin's disease: Prognosis and response to therapy

Three hundred eighteen patients with pathologic stage IA and IB, IIA and IIB, and IIIA Hodgkin's disease who entered into Stanford University Medical Center randomized trials comparing radiation therapy alone to radiation therapy plus six cycles of adjuvant chemotherapy were evaluated. Of these, 54 patients had extralymphatic (E) lesions. There were five relapses among these patients (9 percent), not different from the 37 relapses among the remaining 264 patients (14 percent) with Hodgkin's disease confined to the lymphatic system. Actuarial survival and freedom from relapse were not significantly different for patients with or without extralymphatic disease. The survival of patients with extralymphatic disease was similar whether they received radiation therapy alone or radiation therapy plus chemotherapy.     

Primary empty sella syndrome with visual field defects

A patient with an enlarged, asymmetric sella turcica and visual field defects suggestive of a pituitary or parasellar tumor underwent extensive roentgenographic and pituitary function studies. No abnormalities in pituitary luteinizing hormone, follicle-stimulating hormone, thyroid-stimulating hormone, ACTH, prolactin or vasopressin secretion were detected. Growth hormone secretion was provoked by arginine infusion but not by hypoglycemia. Pneumoencephalography revealed air in the sella turcica, and no evidence of tumor. Thus, an enlarged sella turcica in a patient with visual field defects but normal pituitary function may suggest the presence of an “empty sella syndrome.”     

Empty sella of normal size in Sheehan's syndrome

This report describes the findings of computed tomography of the sella turcica region in 13 women in whom postpartum hypopltuitarism developed in the absence of a pituitary tumor. Seven patients had the typical history of severe postpartum hemorrhage (Sheehan's syndrome). Computed tomographic scanning in these patients revealed absence (in six patients) or marked reduction (in one patient) of the amount of demonstrable pituitary tissue. The pituitary fossa was occupied by material with the density of cerebrospinal fluid, resulting in the appearance of an “empty sella.” Sella size was within the normal range in six of the seven patients and enlarged in one. The six remaining patients lacked a characteristic history of vascular collapse, although several did have postpartum complications. The radiologic findings in four of these patients were similar to those seen in the patients with typical Sheehan's syndrome: one patient had a partially empty sella and another had normal results of computed tomographic scanning. Thus, 11 of 13 women with postpartum hypopituitarism were noted to have an empty or partially empty sella of normal size. This report represents the first radiologic documentation of Sheehan's syndrome as a cause of the secondary empty sella, with the characteristic finding being an empty sella of normal size.     

Multiple myeloma, cryoglobulinemia and xanthomatosis. Distinct clinical and biochemical syndromes in two patients.

Studies were carried out in two patients with multiple myeloma (immunoglobulin G, [IgG], K light chain), cryoglobulinemia and xanthomatosis with clinical features and lipid transport abnormalities which were quite different. One patient had nodular xanthomatosis and lipemia with delayed triglyceride and apolipoprotein removal. In vivo heparin resistance was present and heparin-paraprotein interaction was shown in vitro. The lipoprotein removal defect may have been due to impaired uptake of the "remnants" of glyceride-rich lipoproteins. Abnormalities were found both in primary platelet aggregation and in the platelet release reaction. The second patient had diffuse plane xanthomatosis with normal lipids. An orange cryoprecipitate contained IgG, beta- and prebeta lipoproteins, albumin, carotenoids and about half of the serumcholesterol. Triglyceride turnover was normal. These observations show that M-proteins may interfere with lipid transport by at least two mechanisms and illustrate the clinical diversity of xanthomatous myeloma.     

Familial effects on plasma sex-steroid content in man: Testosterone,estradiol and sex-hormone-binding globulin

We investigated whether familial factors influence the plasma content of sex-steroids and sex-hormone-binding globulin (SHBG) in 98 adult males of 66 families. They had no apparent endocrine dysfunction. The 0800–1100 hr plasma levels of testosterone, 5α-dihydrotestosterone (DHT), estradiol-17β (E₂) and estrone (E₁) were measured by radioimmunoassay. The free fractions of E₂ and testosterone were determined by equilibrium dialysis, and the binding capacity of SHBG w as also calculated. The data were analyzed by analysis of variance. We observed that the differences in the plasma content of testosterone (p = .02), SHBG binding capacity (p = .01), and E₂ (p = .03), free E₂ index (p = .05) were all substantially less variable within groups of brothers than among non-brothers. The variability of the plasma concentration of DHT, free testosterone and E₁ was not significantly less within brothers than among non-brothers. The correlation between either plasma testosterone content (r = .14) or SHBG binding capacity (r = .12) and percent of ideal body weight was not significant statistically. Age had no effect on the results. Our data suggest that genetic and/or environmental factors may affect the plasma content of testosterone, E₂ and SHBG binding capacity.     

A-V conduction disturbances in Reiter's syndrome

High grade atrioventricular (A-V) block is a rarely described complication of Reiter's syndrome. This 65 year old man had recurrent episodes of arthritis, conjunctivitis and urethritis beginning at age 16. A prolonged P-R interval was first noted at age 32. The conduction disturbance progressed to intermittent episodes of high grade and complete heart block by age 65. His bundle electrograms located the site of block above the level of the bundle of His. Atrial pacing to rates of 150/min produced 5:1 A-V block, whereas exercise and atropine administration resulted in 1:1 A-V conduction. In view of these results, artificial pacemaker therapy is not indicated. The association of conduction disorders and Reiter's syndrome is reviewed.     

Pathologic patterns of chronic obstructive pulmonary diseases in patients with normal and deficient levels of alpha 1 antitrypsin

Twenty-nine patients with chronic obstructive pulmonary disease (COPD), selected on the basis of their alpha₁ antitrypsin (AAT) phenotype or trypsin inhibitory capacity (TIC), were studied by clinical and physiologic methods. Six were homozygous, 10 were heterozygous and 13 were normal with respect to their AAT levels. Spirometry, lung volume and diffusing capacity measurements indicated that the extent of pulmonary disease was greater in the patients with homozygous AAT deficiency as compared to the other groups. Quantitative measurements of regional ventilation and perfusion were made using xenon 133 and the results were evaluated by statistical technics. Two different methods of discriminant analysis of the regional ventilation and perfusion data clearly showed the three groups of patients to be significantly different from each other. Since the heterozygous AAT deficient patients had a pattern of disease which distinguished them from patients with both severely deficient and normal AAT levels, it is concluded that heterozygosity for AAT is an important factor in the development of COPD.     

Left ventricular function at high altitude examined by systolic time intervals and M-mode echocardiography

To better understand the effects of high-altitude hypoxia on cardiac performance, healthy lowlandresiding volunteers were studied in 2 groups: 10 subjects after acute ascent to 12,500 ft (3,810 m) (acute group) and 9 subjects after chronic exposure for 6 weeks to 17,600 ft (5,365 m) and 11,000 ft (3,353 m) (chronic group). Systolic time intervals and M-mode echocardiograms were recorded at low and high altitudes. Heart rate was 21% greater at high altitude for all subjects. Preejection period/left ventricular ejection time (PEP/LVET) increased by 16% in the acute group and by 22% in the chronic group. Heart size was smaller at high altitude in both groups, with left atrial and left ventricular (LV) diameters decreasing by 10 to 12%. These changes were statistically significant (p ≤ 0.01). Despite the increase in PEP/LVET, echocardiographic measurements of LV function (percent fractional shortening and mean normalized velocity of circumferential fiber shortening) remained normal. LV isovolumic contraction time was shorter at high altitude, suggesting heightened, rather than depressed, contractility. LV function does not appear to deteriorate at high altitude. Alterations in systolic time intervals probably result from decreased preload, as reflected by smaller heart size, rather than from heart failure or depressed LV contractility.