Biomedical subjects
Publications and source records attributed to R Escribano Albarrán.
A patient with precoucious puberty, caused by the secretion of ecthopic gonadotrophin, due to a teratoma of mediastinal localization accompanied by cellular immunodeficiency is described. A review of literature several cases have been from different hospitals, showing a low frequency of the intrathoratic localization of these teratomas and it is exceptional functional characteristics is made.
Two patients affected with Alport's syndrome belonging respectively to two families with a high incidence of urinary, auditive and ocular symtoms, are described. The most relevant features are commented pointing out that Alport's syndrome could be a structural malformation of the whole or only one part of a type of colagen probably specific of the basal membrane of the glomerulus which could be more easily affected forming toxic metabolites. Finally a reference to the histologic study is made.
In a boy initially diagnosed as X-linked hypogammaglobulinemia, the later clinical and analytical course and the application of new immunological techniques led to the new diagnosis of common variable immunodeficiency. The patient shows panhypogammaglobulinemia, a scarce number of plasma cells with a normal number of precursors and circulating B lymphocytes with membrane bound immunoglobulins and C3 receptors. Delayed hypersensitivity is absent despite normal amount of circulating T lymphocytes which were able to transform when stimulated by PHA. The anergy seems primary and not related to the clinical malnutrition. The authors make a differential diagnosis between both illnesses as a tentative pathogenetic interpretation of B cell development arrest.