GERMINATIVE APLASIA (Latin germinativus germinal; an aplasia) — the clinical syndrome caused by inborn lack of formative cells — gonocytes.
A syndrome under the name G. and. del Castillo (E. V. of del Castillo) with soavt, in 1947 is for the first time described; in literature meets also under the names «syndrome of one cells of Sertoli» [Heller (Page G. Heller) et al., 1951; J. Teter, 1968], «del Castillo's syndrome», «dysgeneses of seed tubules» [M.M.Grumbach et al., 1957].
Del Castillo suggested that the reason of formation of a syndrome of G. and. the delay of migration of gonocytes in an embryonal rudiment of a gonad is. However this representation of del Castillo should be considered wrong since it is proved that the gonad is not differentiated if gonocytes do not reach sexual bookmarks. Apparently, G. and. arises in later stages of development of gonads as a result of death of germinative cells in normally created gonad. Similar states are described in literature at radiation exposure and at other diseases.
The main signs of a syndrome in del Castillo's description are: small sizes of testicles; normal sizes of a penis, scrotum and sufficient development of men's secondary sexual characteristics; normal physical status; azoospermism (see); absence in the anamnesis of instructions on any disturbing factors which could be a cause of death of sex cells; histologically — the small sizes of seed tubules, total absence of a spermatogenic epithelium in the presence of Sertoli's cells, a normal basal membrane of cells of Leydiga; datum level follicle-stimulating hormone (see).
Unlike Klaynfelter's syndrome (see. Klaynfeltera syndrome ) at G. and. the normal men's genotype 46XY is observed, there is no gynecomastia (see).
Treatment — replacement therapy sex hormones (see).
The forecast concerning ability of the patient with G.'s syndrome and. to fertilization adverse.
Bibliography: Starkova H. T. Fundamentals of clinical andrology, page 109, M., 1*973; Teter E. Hormonal disturbances at men and women, the lane with polsk., page 555, Warsaw, 1968; del Castillo E. Century, Trabucco A. and. de la Balze F. And, Syndrome produced by absence of the germinal epithelium without impairment of the Sertoli or Leydig cells, J. clin. Endocr., V. 7, p. 493, 1947, bibliogr.
I. V. Golubeva.