Thrombocytafunkció-zavar albínó gyermekekben.

Translated title of the contribution: Thrombocyte dysfunction in children with albinism

Z. Pap, E. Ajzner, C. Kiss, J. Emódy, I. Závodszky, A. Horváth, L. Muszbek

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

Seven children with albinism were examined for haemorrhagic diathesis. In 6 patients coagulation screening tests (prothrombin time, activated partial thromboplastin time, thrombin time) were normal, in 1 patient prolongation of activated partial thromboplastin time due to mild factor XI deficiency was found. Platelet counts were in the reference range, however, the template bleeding time of 6 out of 7 patients was prolonged. Evaluation of platelet aggregation and secretion in a lumi-aggregometer demonstrated that only the child with normal bleeding time had normal platelet function, and 6 children with prolonged bleeding time had impaired aggregation and release reaction. Five out of these 6 patients had severe decrease of ATP secretion even when high dose of thrombin was used as agonist. The number of dens granules in the platelets of these patients, as measured by the uptake of mepacrine, was significantly less than in normal controls. These findings clearly suggested storage pool deficiency. In one of these patients storage pool deficiency was associated with mild factor XI deficiency. The high frequency of haemorrhagic diathesis in albino children emphasizes, the importance of the screening of patients with albinism for bleeding diathesis.

Original languageHungarian
Pages (from-to)467-471
Number of pages5
JournalOrvosi Hetilap
Volume138
Issue number8
Publication statusPublished - Feb 23 1997

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Albinism
Blood Platelets
Bleeding Time
Platelet Storage Pool Deficiency
Factor XI Deficiency
Hemorrhagic Disorders
Partial Thromboplastin Time
Thrombin Time
Quinacrine
Disease Susceptibility
Platelet Count
Platelet Aggregation
Thrombin
Reference Values
Adenosine Triphosphate
Hemorrhage

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Pap, Z., Ajzner, E., Kiss, C., Emódy, J., Závodszky, I., Horváth, A., & Muszbek, L. (1997). Thrombocytafunkció-zavar albínó gyermekekben. Orvosi Hetilap, 138(8), 467-471.

Thrombocytafunkció-zavar albínó gyermekekben. / Pap, Z.; Ajzner, E.; Kiss, C.; Emódy, J.; Závodszky, I.; Horváth, A.; Muszbek, L.

In: Orvosi Hetilap, Vol. 138, No. 8, 23.02.1997, p. 467-471.

Research output: Contribution to journalArticle

Pap, Z, Ajzner, E, Kiss, C, Emódy, J, Závodszky, I, Horváth, A & Muszbek, L 1997, 'Thrombocytafunkció-zavar albínó gyermekekben.', Orvosi Hetilap, vol. 138, no. 8, pp. 467-471.
Pap Z, Ajzner E, Kiss C, Emódy J, Závodszky I, Horváth A et al. Thrombocytafunkció-zavar albínó gyermekekben. Orvosi Hetilap. 1997 Feb 23;138(8):467-471.
Pap, Z. ; Ajzner, E. ; Kiss, C. ; Emódy, J. ; Závodszky, I. ; Horváth, A. ; Muszbek, L. / Thrombocytafunkció-zavar albínó gyermekekben. In: Orvosi Hetilap. 1997 ; Vol. 138, No. 8. pp. 467-471.
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