Hereditary Mesodermal Dystrophy

Abstract

In the course of investigations in Sunningwell parish, Berkshire, certain members of family " H " were found to transmit the dystrophy of mesodermal tissue which is recognized at an early age by' the occurrence of blue sclerotics, laxity of ligaments, and brittle bones, and during adolescence by the onset of deafness (otosclerosis). The mode of heredity is shown in the accompanying diagram, the informants being the widow of No. II 8-since deceased-and No. III 3. When the latter recalled her examination at the Oxford Eye Hospital twentyfour years earlier, contact became possible with the author of a previous paper (Stobie, 1924), describing the syndrome of blue sclerotics, brittle bones, and progressive deafness amongst villagers who have all proved to belong to the " H " kindred. The present communication deals with a recent clinical and social enquiry amongst the " H " family in so far as this affects the prognosis of their familial disorder. The kindred and affinals of family " H " living in-Sunningwell parish number 78. The population of the parish at the last (1937) census was 637.