Volume 92, Issue 7

Seventy-three members of a 100-member kindred with asymptomatic proteinuria, nephrotic syndrome, and progressive renal failure were studied. Of those studied, 11 members had progressed to end-stage renal disease and seven had significant proteinuria (> 1 g/24 hours) with normal renal function. The genetic mode of inheritance was autosomal dominant with variable penetrance and expressivity. Histopathologic changes were variable but included focal segmental glomerulosclerosis and diffuse glomerulosclerosis. Renal failure usually occurred in the fifth decade of life. The most consistent clinical finding was proteinuria without microscopic hematuria or other significant urinary sediment elements. This disease differed from Alport's hereditary nephritis and congenital nephrotic syndrome in age of onset, urinary findings, and associated conditions, that is, nerve deafness. The hereditary proteinuria and nephrotic syndrome described in this kindred represents another facet in the spectrum of hereditary renal disease.

Adult respiratory distress syndrome is a common respiratory problem with a wide array of precipitating causes and an overall mortality rate of more than 50%. Signs on physical examination tend to be nonspecific as do laboratory findings associated with the syndrome. Because of its diverse etiology, there is no one specific treatment for adult respiratory distress syndrome. Therapy is primarily supportive and centers around the use of mechanical ventilator support. The authors discuss the pathogenesis and management of this syndrome together with some of the newer approaches to mechanical ventilation.

Tobacco smoke can alter both the structural and the immunologic defenses of the lungs against infection. The gases from smoke can also inhibit repair of the damages they create. Smokers have been found to have increased bacterial adherence to the respiratory tract, decreased IgA and IgG, and a decrease in vital capacity two to three times greater than that of nonsmokers. These and other respiratory tract alterations put smokers at an added disadvantage when acute lower respiratory tract infections strike. A history of smoking influences the diagnosis of respiratory tract infections because smokers are more prone than nonsmokers to infection by certain organisms. The major causes and characteristics of lower respiratory tract infections, methods of outpatient diagnosis, and the pros and cons of various modes of therapy are discussed.

Lithium has been used in the treatment of mania since 1949. Although its effectiveness is well recognized, the mechanism by which it stabilizes manic behavior is not well understood. In addition, it has some serious toxic properties combined with a narrow therapeutic range. For this reason, monitoring of serum lithium levels is mandatory. Suggested mechanisms of action and protocols for monitoring of serum levels are presented.

This monthly series was developed from the AOA Task Force on AIDS Writers' Workshop, held August 16 to 18, 1991, in New York. The workshop was sponsored by an education grant from Burroughs Wellcome. It will provide brief clinical updates and perspectives on the human immunodeficiency virus (HIV). Readers may request tear sheets from the AOA editorial offices.

When faced with the task of program evaluation, many directors of medical education may find themselves overwhelmed. This article presents suggestions for conducting an evaluation, including the six basic elements to consider before embarking on such an undertaking. With an emphasis on selecting a model framework as a guide for conducting a program evaluation, this article examines one model-Stufflebeam's CIPP model-in depth.

The retention of a foreign body following surgery is a longstanding and dreaded problem. We report the retention of a fetal scalp monitoring lead that was embedded in the wall of the patient's jejunum and discovered 2 years after cesarean section. In reviewing the American medical literature, we believe this case is, perhaps, unique. The treatment of this entity is discussed as well as ways to help to prevent it.

Extrapancreatic fluid collections are frequently seen in acute pancreatitis. Vascular damage with life-threatening hemorrhage is also a known complication. In the case report presented, we include documentation by computed tomography of an apparent abdominal aortic wall dissection by fluid in a patient with acute pancreatitis. Conservative therapy resulted in complete resolution without hemorrhage or aneurysm formation.

Anomalous origin of the left main coronary artery from the right sinus of Valsalva is an uncommon problem, occurring in four distinct patterns. When the left main coronary artery passes between the aorta and the pulmonary trunk, acute myocardial ischemia or sudden death may occur. Angiography is required in establishing a diagnosis. Surgical correction or bypass surgery are sometimes indicated. The authors report two cases of anomalous origin of the left main coronary artery and discuss the anatomic variants, clinical significance, and technical considerations in both diagnosis and management.

The Mirizzi syndrome is an uncommon cause of obstructive jaundice. In 1948, Mirizzi characterized a syndrome in which common hepatic duct obstruction occurs because of impacted stones in the gallbladder neck or cystic duct. The authors report a case demonstrating this syndrome. They discuss the radiographic features and report the features found on a computed tomographic cholangiogram that have not been previously described. Computed tomography-cholangiography may be helpful in evaluating patients with suspected Mirizzi syndrome.

Synchronous laryngeal and nasal septal lesions in pemphigus vulgaris are rare. The authors report a case of pemphigus vulgaris in an elderly man who was seen with lesions of the supraglottic larynx and nasal septum. The classic skin changes of pemphigus eventually developed, and the patient responded to daily treatments of 80 mg of prednisone. Steroids and immunosuppressive agents remain the treatment of choice, but a favorable prognosis is still dependent on early diagnosis and institution of therapy.