Hæmophilia in Northern Ireland

AMONG the bleeding disorders, hxmophilia has always attracted the greatest interest. The familial nature of the disease, its early onset in life, its predilection for males, and the inefficiency of the blood clotting have all been recognized. Recent work, however, has cast doubt on the long-established view that homophilia is a single entity. It has been shown that all cases, which present clinically as hamophilia and which have a characteristic hereditary pattern, do not necessarily have the same basic defect in the blood. Indeed the impaired blood coagulation may be due to the deficiency of one of three clotting factors. As it is impossible to differentiate these types of himophilia on clinical grounds the final diagnosis can only be made in the laboratory. In 1955, in co-operation with the Medical Research Council, we carried out a survey of all possible hxmophilics in Northern Ireland. We began with a nucleus of patients already known to us. With the help of the Northern Ireland Hospitals Authority and Northern Ireland General Health Services Board, we were able to approach the great majority of family doctors and consultants in Northern Ireland, from whom we received every co-operation. Although no survey of this kind can claim to be complete, we believe it to represent the majority of the "bleeders" in Northern Ireland. We established a register of all hemophilics in this area and offered facilities for the treatment of bleeding episodes. In cooperation with the Dental Department of the Royal Victoria Hospital, we introduced a service for regular dental inspection and conservative treatment. We feel that the information obtained during this survey and our subsequent experience in management are of sufficient general interest to warrant publication.