Renal Complications in Haemophilia and Christmas Disease

Abstract The extent of renal involvement in congenital bleeding disorders has been assessed by a study of renal structure and function in 35 patients. The investigations consisted of estimation of blood-urea and creatinine clearance, intravenous pyelogram, isotope renogram, quantitative proteinuria, and red- and white-cell excretion rates. They were done at a time when the patients were not suffering from frank haematuria. Twenty-seven patients (77 per cent) had an abnormality of renal function as detected by at least one investigation. The majority of lesions in the upper renal tracts were apparently the result of clot formation which gave rise either to filling defects or to obstructive lesions. Thirteen patients had an abnormality of the intravenous pyelogram and seven patients an abnormality of the isotope renogram. The renal lesions did not, in general, give rise to clinical renal impairment and in only four patients was the blood-urea elevated above normal levels. Although there was a tendency for the severe haemophiliacs with frequent haematuria to have most renal abnormalities, it was found that five of the 12 patients who denied any history of frank haematuria had filling defects or obstructive changes in the upper renal tract. It was thought that this was due to microscopic haematuria in these patients associated with clot formation as an increased urinary red-cell excretion was a relatively frequent finding in this study. The patients who had received the fibrinolytic inhibitor, aminocaproic acid, did not show a greater incidence of renal complications than the rest of the patients. But, in view of the large number of renal complications found in this series and the fact that the fibrinolytic enzyme system appears to have a function in maintaining the patency of the urinary tract, the use of aminocaproic acid for haemorrhage from the upper renal tract is not recommended.