Evidence for specific recognition sites mediating clearance of lysosomal enzymes in vivo.

A study of the clearance of liver lysosomal enzymes was carried out in the rat. Purified rat liver lysosomal beta-D-glucuronidase (EC 3.2.

The mass spectrometric identification of dipeptides in the urine of a patient suffering from chronic skin ulceration and oedema.

The identification of a complex ninhydrin positive mixture present in the urine of a child suffering from chronic skin ulceration and oedema by direct chemical ionisation mass spectrometric analysis is described. The compounds were shown to be dipeptides, of which glycylproline was the major constituent. At least 15 dipeptides were identified in the urine, most of which contained proline or hydroxyproline in the carboxy terminal position.

Late spontaneous closure of a large ventricular septal defect.

The authors report a case of a large ventricular septal defect with pulmonary hypertension, cardiomegaly and heart failure in early infancy, exhibiting marked improvement at five years of age. Subsequent followup, revealed spontaneous closure of the defect demonstrated by hemodynamic studies between 7 and 8 years of age, at which time the patient became completely asymptomatic with disappearance of all abnormal physical findings.

Persistent atrial standstill.

The authors present a case of permanent atrial standstill with syncopal attacks, in a patient with chronic Chagas' Heart Disease. The recognition of this dysrhythmia was based upon the conventional and intracavity electrocardiographic tracings in addition to phonomecanographic and hemodynamic data. The recording of the His Bundle electrogram demonstrated the absence of atrial activity, with the His potential preceding all ventricular complexes and an advanced conduction defect distal to the bundle of His.