DE   EN   ES   FR   IT   PT Hematology Herzhmansky-Pudlaka's syndrome

Herzhmansky-Pudlaka's syndrome


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.

  • Сайт детского здоровья