Marfan syndrome with coronary artery lesions in a North American Indian.

Marfan syndrome has not been well documented in North American Indians. A 19-year old Ojibwa man had evidence of this syndrome -- specifically, tall stature, long, thin extremities (particularly, fingers and toes), increased urinary excretion of hydroxyproline, aortic aneurysm, aortic regurgitation and pathologic evidence of aortic rupture and alastic tissue fragmentation. Intimal hyperplasia was present in the extramural coronary arteries, while the intramural arteries, usually thought to be involved, were normal.

Intracardiac electrography in endocardial cushion defects.

Conduction of the sino-atrial impulse from the high right atrium to the ventricles was studied by intracardiac electrography in 21 unoperated patients, age 3 months to 11 years, with endocardial cushion defects (ECD). The high right atrium-to-low right atrium conduction time was prolonged in 15 of 18 subjects (mean 57 +/- 20 msec). The low right atrium-to-His bundle conduction time (LRA-H) was normal in 16 of 17 subjects (mean 82 +/- 30 msec), prolonged in one.

The mechanism for nonspecific lipase from rat pancreas.

Purified nonspecific lipase from rat pancreas appears to mediate the followin reaction sequence: (2) HE +nB in equilibrium HEBn; (2) HEBn + S inequilibrium HEBnS; (3) HEBnS IN EQUILIBRIUM EBnS' + ROH; (4) EBnS' + h2o vector HEBn + R'COO- + H+, where E equils enzyme, B equils bile salt, and S equils R'COOR. Evidence is presented for the occurrence, sequence and reversibility or irreversibility of the above four reactions. Also discussed are the activation energies for both a "good" and a "poor" substrate, the correlation between V and subsitituent function (o-) for a series of substituted benzoic acid esters, and the effects of steric hindrance.

Purification and partial characterization of nonspecific lipase from rat pancreas.

Nonspecific lipase (also referred to as micelle lipase and secondary ester hydrolase) has been purified to electrophoretic homogeneity starting from acetone powder of rat pancreas. The purified enzyme is found to have a molecular weight (gel filtration) of 64 000 +/- 2000, and an equivalent weight (titration with E-600) of 65 000. Nonspecific lipase is seen to be very sensitive to inhibition by organophosphates but resistant to quinine.