Development of inhibitors in haemophilia. Ongoing epidemiological study.

A number of 513 consecutive patients (494-haemophilia A and 19-haemophilia B) from eight haemophilia treatment centers have been investigated with Bethesda assay for the presence of factor VIII or IX inhibitors. The overall prevalence of inhibitors was 15.20%, 18.48% in severe, 5.60% in moderate and 12.24% in mild forms. The prevalence was higher than reported in most of the western countries. The age at start of substitution (p = 0.9775), the frequent switching of factor concentrates (p = 0.8931) were not relevant factors for the development of inhibitors. It is worth to be mentioned the unexpectedly occurrence of inhibitors in prior inhibitor negative (6/72) patients (during surgical interventions) probably due to their previous scarce substitution, occurrence which seems not being connected with the continuous infusion modality of factor VIII administration (p = 0.8341). In controversial situations, in the field of low titer (≤ 1 BU/ml) inhibitors for a reliable interpretation of the results the performance of recovery index and half-life time assessment of FVIII/IX was undertaken.

Preliminary clinical and methodologic observations on the determination of serum gamma-glutamyltranspeptidase and of the alpha-amylase clearance in nephropathies.

Serum gamma-glutamyltranspeptidase (GGTP) and alpha-amylase clearance were determined in a total group of 90 patients of whom 60 with renal diseases and 30 with extrarenal diseases. The renal patients were distributed, according to diagnosis in the following groups: acute glomerulonephritis, chronic glomerulonephritis, acute pyelonephritis, chronic pyelonephritis, nephrotic syndrome and manifest chronic renal failure. The 30 controls were hospitalized for different extrarenal diseases such as: pneumonia, gastroduodenal ulcer, arterial hypertension stage I and angina pectoris. Serum GGTP assay was performed in 60 patients (40 renal patients and 20 controls) using Boehringer monotest kits and in 30 patients (20 renal patients and 10 controls) using Romanian kits (I.C.C.F.). No changes suggesting a particular type of nephropathy were observed. The results obtained by using the two types of kits for the serum GGTP assay have proved to be very close. Alpha-amylase clearance was determined in all the patients with Spofa (R.S.C.) tablets concomitantly with the urea and creatinine clearance. Important decreases of alpha-amylase clearance in concordance with decreases of urea and creatinine clearances were observed in all the patients with severe renal failure. More moderate decreases of alpha-amylase clearance were observed in the patients with acute and chronic glomerulonephritis. The utility of this clearance as a test of glomerular filtration and sometimes as a prognostic test, is discussed.