Hemodynamic studies and results of therapy in 50 patients with bacteremic shock

Hemodynamic studies are reported in 50 patients with bacteremic shock. Differences between gram-positive and gram-negative infection and factors relating to survival in shock are examined. Failure of cardiac output to improve following plasma volume expansion suggests impaired cardiac function in some patients. Responses to norepinephrine, dopamine, isoproterenol and corticosteroids are reported, and use of these agents in patients with bacteremic shock is discussed.     

Acute dermatomyositis-polymyositis and malignancy

A partial review of selected published case reports of AD-P associated with malignancy has been enhanced by the presentation of pertinent data on 15 unreported examples of the association. It is noteworthy that the first case in current literature of AD-P associated with a malignancy was described in 1916. The brief clinical report of a patient with proximal muscle weakness and skin lesions, with the obvious association with a malignancy (adenocarcinoma of the stomach), describes an example that has been repeated many times with different types of tumors but with essentially no variations in the clinical findings.In 1959 Williams identified 590 cases of AD with an overall tumor rate of 15%,³¹ and recently Barnes identified 258 cases of AD associated with a malignancy.³³The original designation, dermatomyositis or AD, has now been expanded to include proximal muscle polymyositis with systemic involvement, which syndrome at the current state of the art is indistinguishable clinically and pathologically from AD except for the lack of skin lesions. It may be that at some future time one or more immunologic features may differentiate the clinical entity polymyositis from AD and further subdivide each of these entities from similar clinical syndromes associated with a malignancy. However, the problem in management in either AD or polymyositis is similar.¹A number of patients with a malignancy and muscle weakness or neuropathy have been reported. These associations have been mentioned briefly, but insufficient data are available to determine whether these should be considered as a variant of AD-P or only casually related conditions with certain clinical features in common.Most of the patients described in the literature of AD-P with an associated malignancy have had skin lesions; a minority only have lacked this feature. However, unless a patient is followed carefully, it is possible for a transient or evanescent erythema or insignificant skin lesions to be present and not recorded in the case record.In the patients with malignancy and AD-P, there are no clinical features or laboratory findings unique to the malignancy in contrast to a carcinoma unassociated with AD-P, nor are the clinical features or laboratory findings of AD-P with malignancy different from those without a malignancy, except for one feature. The fatal determination, usually attributed to the malignancy, may be associated with a florid type of dermatomyositis, better described as rapidly progressive dermatomyositis, relentless muscle weakness, and failure of the respiratory and pharyngeal musculature.Approximately 15% of the patients with typical AD-P have an associated malignancy. Sometimes it precedes the symptoms of the collagen-vascular disease by one or more years. In other patients, the reverse is true by a similar period of time. In a few patients with AD-P, two related or chronologically unrelated tumors have appeared. Most reports favor a more than casual relationship, e.g., the recent study by Barnes.³³ Another recent survey casts doubt on this relationship.⁹⁷ We still lack epidemiologic information based upon statistically reliable data of cases with the combined condition, nor are there comprehensive data in this country of malignancy in patients without AD-P, in respect to decades of life, sex, and site and type of tumor.³⁴The cause either of AD-P or most malignancies is unknown. It is easy to speculate positively versus negatively as to whether or not two diseases might have had their initiating pathogenesis at the same time, with clinical symptoms or laboratory findings of one or the other predominating for weeks, months, even years. I tend to favor a concomitant pathogenesis associated with disproportionate development of clinical symptoms. At the moment a virus participating in the pathogenesis seems intriguing, but firm proof is lacking.Females tend to predominate because of the larger number of genital and breast tumors, which is not quite counterbalanced by tumors in lung and gastrointestinal tract, which are more common in the male.³³ In the newly reported cases presented in this review, the males exceeded the females by a ratio of 2:1, probably because a majority of the cases came from army or VA hospitals.The clinical appearance of a malignancy associated with AD-P, in adult life, usually comes after age 40 yr. A few examples of noninvasive tumor with AD-P in the prepuberty years have been reported. One girl age 10 yr had a chromophobe adenoma. The tumor probably was present when symptoms of AD appeared. In the second patient, a boy with hyperplasia of the adrenals, symptoms appeared at age 10 yr. Also, three examples of leukemia have been reported in children. Note should also be made of the combined appearance of AD-P and malignancy in a 17-yr-old male with reticuloendotheliosis. This is borderline between childhood and adulthood. One patient in the previously unreported cases had a probable Wilms tumor removed at age 17 yr and developed systemic symptoms of AD 7 yr later. However, not one of these observations negate the statement that AD-P with malignancy is an adult disease.The treatment of dermatomyositis in patients with an associated malignancy is the same as in those without a malignancy; the same holds for the tumor. Furthermore, one might expect that the combined use of corticosteroids and immunosuppressive drugs, useful in either AD-P or a malignancy, might have an enhanced beneficial effect. There are too few cases reported or observed unreported to make any statements in this category. One patient in my series, E.A., received large doses of corticosteroids and a course of methotrexate, but the course was inevitably regressive.Several of the clinical features frequently observed early in the course of AD-P without an associated malignancy were not reported in the cases in the literature nor seen in the series of new cases observed.¹ This concerns the incidence of arthralgias, sometimes called atypical RA, sclerodermatous changes either in the skin or subcutaneous calcinosis, or Raynaud phenomenon. The incidence of each of these three clinical findings may be assumed to be low in either the reported cases or in the cases described and previously unreported.     

Postmyocardial infarction (Dressler's) syndrome: report of a case with immunological and viral studies.

A case of PMIX with immunologic and viral studies obtained prior, during, and after a typical exacerbation are reported. Immunologic studies indicated the presence of humoral antiheart autoantibodies, and an increase in the number and proportion of lymphocytes containing surface IgG immunoglobulin during acute activity. Viral studies were negative. Previously reported immunologic and viral data, as well as etiology and pathogenesis of PMIS are discussed.     

Endocarditis due to Actinobacillus actinomycetemcomitans

A case of infective endocarditis in a 38-year-old Saudi male caused by Actinobacillus actinomycetemcomitans is reported. The patient was treated successfully with a combination of benzyl penicillin and gentamicin. Previously reported cases are reviewed.     

Chlorambucil therapy in rheumatoid arthritis: clinical experience in 28 patients and literature review

Our clinical experience in 28 patients receiving chlorambucil for rheumatoid arthritis (RA) and the reports on chlorambucil therapy are reviewed. Our study population and other reports generally represent patients with severe RA who had either failed to improve or developed significant toxicity during previous treatment with conventional slow acting anti-rheumatic drugs (SAARDs). Seventy-two percent of patients had a significant clinical improvement during chlorambucil therapy and reports of complete remission are given, although the incidence of remission is unknown. Hematologic complications are often reported, but appeared more frequently in our experience than previously reported. Hematologic toxicity required that chlorambucil be discontinued in the majority of our cases. Two deaths from suspected drug induced malignancies are reported. Although chlorambucil appears to be effective in the control of active RA, the potential for drug induced toxicity and malignancies may outweigh the benefit of continued use of this experimental therapy in RA.     

Nosocomial pseudoepidemics and pseudoinfections: an increasing problem.

The infection control practitioner often relies on microbiologic data in order to conduct nosocomial infection surveillance and control activities. On the basis of a review of the medical literature, false positive culture and stained smear results representing pseudoinfection are being reported with greater frequency. Documented cases and clusters of pseudoinfectons are reviewed, and the epidemiologic characteristics and methods for detection and prevention of this increasingly recognized problem are discussed.     

Cardiac complications of wegener granulomatosis: A case report of complete heart block and review of the literature

Wegener granulomatosis is a necrotizing vasculitis whose target organs are classically the upper and lower respiratory tracts and the kidneys. There has been other end-organ involvement documentation, emphasizing the disseminated nature of this disease, but the literature concerning cardiac involvement is limited. The few case reports and general reviews show that the two most common histologic cardiac manifestations are pericarditis and coronary arteritis, each occurring in 50% of the reported cases. The most frequent clinical manifestation is cardiac arrhythmias that are manifested as supraventricular tachyarrhythmias. We report an unusual cardiac manifestation, a case of complete heart block, occurring during the active stage of Wegener granulomatosis. The problem this case presented and the management are reported. The literature dealing with the cardiac involvement in Wegener granulomatosis is reviewed, and the specific histopathologic findings and the pathophysiologic mechanisms of this involvement are discussed.     

Mitral valve prolapse: Recent concepts and observations

The conditions associated with prolapse of the posterior leaflet of the mitral valve are multiple. The mechanisms of mitral valve prolapse as well as the pathogenesis of pain and ectopic impulse formation are reviewed. Propranolol appears to be the drug of choice for the symptomatic treatment of patients with this syndrome since it decreases myocardial oxygen demand and wall tension thus reducing or abolishing the discrepancy between myocardial oxygen demand and supply within the mitral apparatus. It has also been reported to modify the auscultatory findings associated with this condition.

The frequency of this mitral valve abnormality in patients with obstructive coronary artery disease is reviewed. It appears that prolapse of the posterior leaflet scallops in patients with significant obstructive coronary artery disease represents an intermediate stage before mitral insufficiency occurs. This group of patients with papillary muscle dysfunction includes those with prolapsed leaflets without mitral insufficiency, those with systolic murmurs and compensated heart failure and others with progressive cardiac decompensation and severe mitral regurgitation.     

Studies of acute myocardial infarction with intermittent bundle branch and hemiblock

Two cases of transient hemiblock occurring during the course of acute myocardial infarction are reported. The transient manifestation permits the accurate evaluation of the diagnostic features of the hemiblocks as modified by acute infarction. One case reflects the development of left bundle branch block due to bilateral post-divisional block which inter alia permits the study of left bundle branch block in the presence of acute myocardial infarction.     

Heparin resistance and decreased hepatic triglyceride hydrolase release during long-term estrogen-progestin treatment

These studies were undertaken to define the mechanism for the depression of post-heparin triglyceride hydrolase activity in women treated with estrogen-progestin oral contraceptives. Six treated and six control women were studied. Total, protamine-inhibited, and protamine-resistant triglyceride hydrolase activities were measured after six different intravenous doses of heparin in each subject in order to determine the dose-response relationships for lipase release. As has been reported during short-term treatment with estrogens, long-term treatment with oral contraceptive agents is accompanied by selective depression of protamine-resistant (hepatic) lipase activity. This depression can be partly reversed by the administration of large heparin doses, but maximally releasing heparin does fail to restore postheparin protamine-resistant activity to control values. These data are compatible with the idea that the releasable pool of hepatic triglyceride hydrolase activity is diminished in women who receive oral contraceptive agents and that the pharmacokinetics of its release are altered in such a way that only relatively high concentrations of heparin displace the enzyme from this pool.     

Osteomalacia and osteitis fibrosa in a man ingesting aluminum hydroxide antacid

A 53-year-old man with a history of long-term aluminum hydroxide antacid ingestion reported diffuse bone pain and multiple stress fractures over a two-year period. An undecalcified transiliac bone biopsy specimen revealed osteomalacia with osteitis fibrosa; plasma parathyroid hormone and cyclic AMP levels were normal. Following withdrawal of antacids and treatment with calcium and phosphorus, an initially elevated plasma, 1,25-dihydroxyvitamin D level fell to within the normal range, accompanied by decreased bone pain, healed stress fractures, and increased axial bone mineral content as determined by computed tomography of lumbar trabecular bone. Phosphate deprivation and 1,25-dihydroxyvitamin D excess may contribute to the poor mineralization and exaggerated resorption of bone observed in this syndrome. The clinical, biochemical, radiologic, and histologic features of previously reported cases are reviewed. Early recognition of this syndrome is important, since appropriate therapy promotes skeletal remineralization and prevents morbidity.     

Insulin and ACTH production by a streptozotocin responsive islet cell carcinoma

We describe a patient with insulin and ACTH production by an islet cell carcinoma with hepatic metastases and cite three previously reported cases of this bihormonal production. The patient was treated with multiple instillations of streptozotocin, a drug with specific beta cell toxicity. After therapy, insulin and ACTH levels declined. These responses after the administration of a drug with beta cell specificity are evidence that both insulin and ACTH were produced by beta cells rather than by multiple cell types.     

Haemophilus parainfluenzae endocarditis. Two cases and review of the literature in the past decade.

Twenty-five cases of Haemophilus parainfluenzae endocarditis have been reported in the past 10 years, providing a better current perspective of this disease. We have recently diagnosed and treated two patients with H. parainfluenzae endocarditis, and both underwent surgical intervention for complications of their disease. H. parainfluenzae and the other Haemophilus species causing endocarditis often present with a subacute course, often escape early cultural detection and mimic fungal endocarditis in the propensity for large vessel embolization. Multiple emboli and occlusion of major arterial vessels are especially notable features of H. parainfluenzae endocarditis and have occurred in approximately 30 per cent of the cases reported in the past 10 years. In contradistinction to other types of bacterial endocarditis, the most common cause of death in this series has been neurologic complications following embolization.Development of large vegetations appears to be common and may be an intrinsic property of the Haemophilus species, but it is likely that it also reflects the duration of the disease. Delay in recovery of the organism from blood cultures is characteristic of H. parainfluenzae endocarditis and may be due to the strict requirement for V factor exhibited by some strains. Echocardiography has proved useful in suggesting the diagnosis of endocarditis when blood cultures are negative.Optimal antibiotic therapy of H. parainfluenzae endocarditis has not been determined, but the reported clinical experience suggests that combination therapy with ampicillin and an aminoglycoside is the current treatment of choice. Failure to eradicate the organism after a prolonged trial of appropriate antibiotic therapy is not unusual. Indications for surgery in H. parainfluenzae endocarditis may have to be amended to include potential embolization, especially if large vegetations are demonstrated on echocardiography.     

Treatment of Goodpasture's syndrome with plasmapheresis. A case report and review of the literature.

A case is reported of antiglomerular basement membrane antibody-induced Goodpasture's syndrome in which the patient required hemodialysis and was treated with immunosuppressive agents and plasmapheresis. A severe (80 per cent) cresentic lesion was reversed, and creatinine was stabilized at 2.5 mg/dl at one year follow-up. Earlier reports of therapy without plasmapheresis showed that 88 per cent of the patients would either die or require long-term hemodialysis. Fifteen other reported cases of Goodpasture's syndrome in which the patients were treated with plasmapheresis are reviewed. When reported, short-term follow-up showed that nine of these patients were alive without need of dialysis, five wee receiving dialysis, and only two had died. This suggests that plasmaheresis and immunosuppressive therapy may reverse the renal lesion in some patients with Goodpasture's syndrome.     

Congenital absence of the pericardium associated with atrial septal defect and sick sinus syndrome

We describe the case of a 42-year-old woman with a rare combination of congenital pericardial absence, atrial septal defect within the fossa ovalis and sick sinus syndrome. The features of the different examinations performed (chest X-ray, ECG, ambulatory ECG-monitoring, echocardiography, and cardiac catheterization) are reported and discussed.     

Thyroid-stimulating hormone and cyclic adenosine 3',5'-monophosphate in the regulation of thyroid gland function.

The initial step in TSH action reflects binding of the hormone to specific receptor sites on the plasma membrane. Such binding has been studied using plasma membranes, homogenates, isolated thyroid cells grown in culture, and thyroid slices. 3-H- and iodinated TSH preparations have been used; the latter have been prepared using both chloramine-T and lactoperoxidase. Some of the discrepancies reported in the literature might reflect the different thyroid and hormone preparations and the variable incubation conditions which have been used. In general, good correlation exists between binding of TSH and activation of adenylate cyclase in thyroid plasma membranes. Data is reviewed related to activation of protein kinase in intact thyroid cells by TSH. Although there is impressive evidence for cyclic AMP mediation of effects of TSH on the thyroid, some data that are inconsistent with this concept are considered, especially in relationship to 32-P incorporation into phospholipid. The role of cyclic GMP in thyroid function is discussed.     

Congenital tricuspid stenosis. Case treated by heterograft replacement of the tricuspid valve

This is the first reported case of successful porcine valve replacement in a patient with congenital tricuspid stenosis. The importance of careful diagnostic studies is stressed, particularly with regard to differentiation from tricuspid atresia and identification of patients with tricuspid stenosis who are anatomically suited for corrective surgery.     

Relapsing polychondritis: review of current status and case report,.

This communication has attempted to review the present state of published knowledge on the syndrome of relapsing polychondritis. Basic anatomic, physiologic, and biochemical changes in this disorder are summarized and the role of metabolic and immunologic alterations in the pathogenesis discussed. An additional case of relapsing polychondritis is reported, and the clinical features of this case, plus those of 131 previously reported, are reviewed with discussion of present day therapeutic experience and prognosis.     

Electrophysiology and pharmacology of cardiac arrhythmias. IX. Cardiac electrophysiologic effects of beta adrenergic receptor stimulation and blockade. Part B.

It is clear from clinical and experimental data that have been reported thus far that verapamil is highly effective in the therapy of cardiac arrhythmias, and that it acts by a different mechanism than most of the commonly used antiarrhythmic drugs. The available clinical data indicate that on intravenous administration verapamil is as good as and perhaps superior to quinidine, procainamide and propranolol for the therapy of many atrial arrhythmias. Unfortunately the extent to which it is useful as longterm prophylaxis has not yet been reported, nor has its toxicity during protracted oral administration.The effects of verapamil on cardiac action potentials clearly indicate that it modifies the slow response to a much greater extent than the fast response. Studies of cardiac tissues from diseased human atria have indicated that slow response action potentials occur frequently. ³¹ It is possible that such action potentials are responsible for the reentrant and automatic arrhythmias which occur in association with clinical cardiac disease. Whether the efficacy of verapamil in the therapy of atrial arrhythmias is primarily due to abolishing slow response activity in diseased atrial tissues or to suppression of propagation through the atrioventricular node is uncertain. However, it is likely that the therapeutic action of the drug may result from altered propagation of an arrhythmia through the atrioventricular junction as well as from the effects of the drug on diseased atrial and ventricular tissues.     

Interruption of the aortic arch and coarctation of the aorta: pathogenetic relations

The pathologic anatomic features and associated cardiac anomalies of 63 cases of interruption of the aortic arch (IAA) type A (54 reported and 9 observed) and 145 cases of type B (124 reported and 21 observed) were analyzed and compared with those seen in 57 autopsy cases of infant coarctation of the aorta (CA). There was no significant sex predominance within the 3 groups and the prognosis without surgery was uniformly poor. Ventricular septal defect was much more common in IAA type B than in type A or CA. Anomalous origin of the subclavian artery and DiGeorge syndrome were commonly associated with IAA type B but were rare in type A and CA. Transposition of the great arteries and double-inlet left ventricle are less common in IAA type B than in types A and CA. On the basis of these findings and the observation by Le Lièvre and Le Douarin that neural crest cells contribute significantly to the formation of the visceral arch system and associated organs, we postulate that IAA type B may be a manifestation of a developmental error involving the neural crest, as is the DiGeorge syndrome. We believe IAA type A and CA to be closely related anomalies that may be prenatally acquired and pathogenetically distinct from IAA type B.