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Homocystinuria is an inborn error of metabolism involving a high incidence of thromboembolism. It sometimes improves with large doses of pyridoxine. We investigated the kinetics and distribution of 111Indoxine-labeled platelets in 11 normal volunteers and 12 patients with homocystinuria, none of whom had clinical evidence of acute thrombosis at the time of the study. Six of the patients were resistant to pyridoxine and had homocystinemia. There were no statistical differences in mean platelet-survival times between pyridoxine responders and nonresponders or between normal subjects and pyridoxine responders or nonresponders, regardless of whether a linear, exponential, or multiple-hit model was used to analyze the kinetic data. Plasma homocystine levels had no apparent effect on mean platelet-survival time. There was no abnormal accumulation of platelets in any of the patients, and the distribution of platelets in liver and spleen was similar to that in normal subjects. Our results suggest that the kinetics and distribution of platelets in patients with homocystinuria who have no clinical evidence of thromboembolism are normal. Thus, the data do not provide evidence for disordered platelet function or for an ongoing interaction of platelets with vessel walls in this condition.
R L Hill-Zobel, and
R E Pyeritz, and
U Scheffel, and
O Malpica, and
S Engin, and
E E Camargo, and
M Abbott, and
T R Guilarte, and
J Hill, and
P A McIntyre, and
E A Murphy, and
M F Tsan
February 1980,
British journal of haematology,
R L Hill-Zobel, and
R E Pyeritz, and
U Scheffel, and
O Malpica, and
S Engin, and
E E Camargo, and
M Abbott, and
T R Guilarte, and
J Hill, and
P A McIntyre, and
E A Murphy, and
M F Tsan
May 1983,
American journal of veterinary research,
R L Hill-Zobel, and
R E Pyeritz, and
U Scheffel, and
O Malpica, and
S Engin, and
E E Camargo, and
M Abbott, and
T R Guilarte, and
J Hill, and
P A McIntyre, and
E A Murphy, and
M F Tsan
February 1983,
The New England journal of medicine,
R L Hill-Zobel, and
R E Pyeritz, and
U Scheffel, and
O Malpica, and
S Engin, and
E E Camargo, and
M Abbott, and
T R Guilarte, and
J Hill, and
P A McIntyre, and
E A Murphy, and
M F Tsan
April 1982,
American journal of hematology,
R L Hill-Zobel, and
R E Pyeritz, and
U Scheffel, and
O Malpica, and
S Engin, and
E E Camargo, and
M Abbott, and
T R Guilarte, and
J Hill, and
P A McIntyre, and
E A Murphy, and
M F Tsan
December 1980,
British journal of haematology,
R L Hill-Zobel, and
R E Pyeritz, and
U Scheffel, and
O Malpica, and
S Engin, and
E E Camargo, and
M Abbott, and
T R Guilarte, and
J Hill, and
P A McIntyre, and
E A Murphy, and
M F Tsan
October 1978,
Mayo Clinic proceedings,
R L Hill-Zobel, and
R E Pyeritz, and
U Scheffel, and
O Malpica, and
S Engin, and
E E Camargo, and
M Abbott, and
T R Guilarte, and
J Hill, and
P A McIntyre, and
E A Murphy, and
M F Tsan
January 1984,
Stroke,
R L Hill-Zobel, and
R E Pyeritz, and
U Scheffel, and
O Malpica, and
S Engin, and
E E Camargo, and
M Abbott, and
T R Guilarte, and
J Hill, and
P A McIntyre, and
E A Murphy, and
M F Tsan
December 1980,
British journal of haematology,
R L Hill-Zobel, and
R E Pyeritz, and
U Scheffel, and
O Malpica, and
S Engin, and
E E Camargo, and
M Abbott, and
T R Guilarte, and
J Hill, and
P A McIntyre, and
E A Murphy, and
M F Tsan
July 1991,
Investigative radiology,
R L Hill-Zobel, and
R E Pyeritz, and
U Scheffel, and
O Malpica, and
S Engin, and
E E Camargo, and
M Abbott, and
T R Guilarte, and
J Hill, and
P A McIntyre, and
E A Murphy, and
M F Tsan
December 1985,
British journal of haematology,
