CONGENITAL AND FAMILIAL HEMOLYTIC DISEASE IN CHILDREN

Abstract

Congenital and familial hemolytic icterus, although familiar to the pediatrician, has still many points of clinical interest which have hitherto been insufficiently investigated. For this reason, we have considered it worth while to publish a few personal observations on this disease made since 1926 in a great number of cases divided among fourteen different families. This article will be limited to a commentary on the clinical features and the transmission of the disease, avoiding all unnecessary references and leaving aside known facts and historical reviews. Our personal experience is too incomplete to allow us to draw definite conclusions on the analysis of results, on the pathogenesis, on the pathologic physiology and, above all, on the therapy of the disease. The condition was first described by Hayem, 1 as splenomegalic icterus; it was investigated by Gilbert and his pupils 2 and was classed as a distinct clinical entity in 1900 by