Schirger, A

Primary lymphedema, a disorder causing persistent swelling in an extremity, is rare in children and adolescents; it affects 1.15/100,000 persons less than age 20 years. It primarily affects girls near menarche. The records of 125 children and adolescents, aged 0 to 20 years, who were examined at the Mayo Clinic were analyzed; 99 of these patients were contacted to obtain follow-up data. The influences of estrogen and inflammation are thought to be important etiologic factors in primary lymphedema. The diagnosis can be made on the basis of a thorough history and physical examination. Lymphangiograms, venograms, and biopsies add nothing to the diagnosis because of the low incidence of tumor in children and adolescents. Conservative treatment is recommended: a Jobst-type stocking, elevation, and proper foot care. Diuretics are not recommended. Careful psychologic counseling, especially in adolescents, is highly recommended.

Data on 11 patients with orthostatic hypotension and primary systemic amyloidosis have been presented. Dizziness and light-headedness or syncope significantly hampered the involved patients. Three were incapacitated by orthostatic hypotension. Diarrhea, nausea, vomiting, and loss of weight were common, and adrenal insufficiency was seriously considered in the differential diagnosis of six of the 11 patients. Abnormal sweating was found in all six in whom this test was done. The drug 9-α-fluorohydrocortisone showed promise in symptomatic therapy of the condition. The possibility of primary systemic amyloidosis should be considered in the presence of orthostatic hypotension. Rectal biopsy and bone-marrow aspiration are recommended as initial biopsy procedures unless another organ suitable for biopsy is obviously involved.

The arterial blood pressure was measured during exercise in six patients with idiopathic orthostatic hypotension. In five there was a pronounced fall of arterial pressure while the subjects exercised in the supine position on a horizontal table. The systolic and diastolic pressures fell by an average of 50 and 32 mm. of mercury, respectively. During comparable exercise with the table tilted 15 degrees head downward, the pressures fell to a similar degree. Thus, an abnormal response of blood pressure occurred under conditions in which venous pooling was unlikely to be present. It is suggested that the fall in blood pressure during exercise in the supine position was the result of failure of compensatory constriction of other vascular beds and not of failure of the cardiac output to increase. Thus, the net peripheral resistance in such patients is less than that in normal persons performing comparable exercise.