Herzhmansky-Pudlaka's syndrome
Contents:
Description:
Herzhmansky — Pudlaka a disease (F. Heřmansky, the Czech therapist, 1916 — 1980; P. Pudlak, the modern Czech therapist) — the hereditary disease which is characterized by disturbance of structure and function of thrombocytes
Symptoms of the Syndrome of Herzhmansky-Pudlaka:
It is shown by full albinism, bleeding caused by disturbance of aggregation of thrombocytes, and adjournment of a tseroid in cells of reticuloendothelial system. Changes in lungs are very similar to those at an idiopathic fibroziruyushchy alveolitis, only macrophages contain granules of a tseroid.
Reasons of the Syndrome of Herzhmansky-Pudlaka:
Herzhmansky-Pudlaka's syndrome is inherited autosomal рецессивно.
Treatment of the Syndrome of Herzhmansky-Pudlaka:
Treatment only symptomatic.
