Emery-Dreifuss muscular dystrophy with autosomal dominant transmission.

A woman with early-onset, slowly progressive, humeroperoneal muscle weakness had marked restriction of neck flexion with contracture at the elbows. She developed exertional dyspnea at age 25, atrial fibrillation with slow ventricular rate was discovered, and a cardiac pacemaker was implanted. Her father had a similar disorder. There is at least one other report of autosomal dominant transmission of this clinical picture, which had previously only been reported as Emery-Dreifuss muscular dystrophy with X-linked recessive inheritance. Thus, more than one mode of inheritance is possible for this unusual and distinctive form of muscular dystrophy.

Clinical angiographic regression of atherosclerosis after partial ileal bypass.

Clinical documentation of atherosclerotic plaque regression has been difficult to obtain. This is a report of a patient with severe and early atherosclerotic cardiovascular disease with regression of at least three major atherosclerotic lesions demonstrated by coronary arteriography 10 years after partial ileal bypass operation. The patient's total plasma cholesterol was reduced over these 10 years, ranging from 40% to 23%, from the preoperative level of 757 mg/dl. Sequential arteriograms were assessed independently by several arteriographers and blindly by the Arteriography Review Panel of the Program on Surgical Control of the Hyperlipidemias (POSCH). The readings were analyzed by 4 grading methods. Unanimously, marked regression was read in the proximal left circumflex artery (70% leads to 20%), middle segment of the right coronary artery (45% leads to 20%), and in the distal right coronary artery (80% leads to 50%). Thus, by any and all of the methods used, there was significant regression of arteriographically demonstrated atherosclerotic lesions.

Changes in ventilation and perfusion in anesthetized dogs following lobar lavage with saline solution.

The therapeutic application of endobronchial lavage has been inhibited by concern regarding alterations in the exchange of gases, particularly hypoxemia, and by the technical complexity of the procedure. Using a cuffed fiberoptic bronchoscope with which lavage could be restricted to one lobe, we studied the sequence of alterations in the exchange of gases after lavage and the attendant disturbances in ventilation and perfusion in ten mongrel dogs following lobar lavage with 1,850 ml of physiologic saline solution. Sequential scintiphotographs of ventilation and perfusion and sequential measurements of arterial blood gas levels were obtained. Linkage of the computer to the scintillation camera allowed quantification of data on ventilation and perfusion from the lobar area of interest. Lavage induced a reduction in regional ventilation and perfusion in all dogs. The decline in ventilation preceded that in perfusion, and the computed ratio of ventilation over perfusion reached its lowest point during the first 30 minutes after lavage. The arterial oxygen pressure (PaO2) declined in all dogs from a mean baseline value of 87 mm Hg to a mean value of 76 mm Hg at 30 minutes after lavage and gradually returned to a mean of 85 mm Hg by six hours after lavage. The lowest value for PaO2 occurred after balloon occlusion prior to lavage. Calculations of shunt showed a minimal increment in the intrapulmonary shunt (Qs/Qt). In the normal dog, lobar lavage induces mild hypoxemia due to an imbalance of ventilation and perfusion that resolves within six hours.