摘要:

Liver disease and thromboembolism are the most frequent and severe side effects associated with the use of clotting‐factor concentrates in haemophiliacs. Knowledge and careful evaluation of the risk factors related to their development appear to be the most reasonable policy for prevention. Hemolysis due to significant isoantibody infusion and bleeding associated to abnormal platelet function occur more rarely during intensive replacement therapy with clotting factor concentrates. Finally, abnormalities of renal function have been observed in a number of patients, though they seem to be of minor clinical importance. These complications do not justify withdrawal or limitation of the very effective and life‐changing use of concentrates. However, awareness of their occurrence and of their danger requires that specialized hemophilia centres carry out, at frequent intervals, clinical and laboratory testing of the target organs to allow an early detect

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1977.tb01510.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

摘要:

The incidence of post‐transfusion hepatitis and liver dysfunction is presented in fifty‐four patients with classical hemophilia who received episodic and/or prophylactic Factor VIII concentrate. 42.5 % had persistent biochemical evidence of liver dysfunction with elevated SGOT and SGPT; 3.8 % have persistent (HB8) antigenicity and 90 % have (HBsAb) antibodies. The results are the same for those who were treated episodically and received an average of 753 units Factor VIII per week as those treated prophylactically who received an average of 686 Factor VIII units per week. The incidence of clinical and/or subclinical disease is unaffected by the transfusion regimen or the amount of concentrate used. The necessity for close follow is emphasized for determination of chronic liver disease and its further ther

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1977.tb01511.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

摘要:

The incidence of hepatitis B surface antigen (HBsAg) and antibody (HBsAb) in 101 Greek multitransfused haemophiliacs with or without a previous history of jaundice was studied. Using the haemagglutination inhibition assay, HBsAg was detected in 9.9 %, HBsAb in 61.3 % and HBsAg plus Ab in 11.8 % of the cases. The incidence of jaundice was 14.8 %. Liver function was tested by means of Normotest, SGOT, SGPT and serum protein electrophoresis. Abnormal values of liver function tests were found in 47 % of the patients. The abnormal findings were related to the presence of HBsAg or Ab and to the history of jaundice. There was no significant difference between the various groups of patients.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1977.tb01512.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

摘要:

Modifications of Factor VIII related antigen (F. VIII R. A.) were investigated in three hemophilic patients during the course of post transfusion hepatitis. F. VIII R. A. was found to be elevated and correlated with the increase of the S.G.P.T. During the acute phase of the disease the Factor VIII related protein was tested for its ristocetin cofactor activity, its electrophoretic mobility on crossed antigen‐antibody electrophoresis and its elution pattern on Sepharose 4B columns; all these properties proved to be abnormal.Liver function tests and F. VIII R. A. were measured in 36 multitransfused hemophiliacs. There was a significant increase of the F. VIII R. A. in the plasma of patients with abnormal liver function tests. Only a few of the multitransfused hemophiliacs had clinical symptoms of hepatitis, although 72 % had elevated transaminase level

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1977.tb01513.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

摘要:

The relationship of chronic hepatitis B and/or liver dysfunction to treatment in 113 hemophiliacs was evaluated by the enzyme tests, SGOT and SGPT, and by the presence of circulating hepatis B surface antigen (HbsAg) or antibody (anti‐Hbs). The hemophiliacs were divided into three groups according to treatment pattern. Individuals who had received mutiple doses of plasma fractions, derived from four or more commercial lots were placed in Group I «large Exposure». Group II «Small Exposure» had been treated with fractions from three or fewer lots and Group III «Cryo» had never received commercial fractions, but had been treated with cryoprecipitate. Abnormal liver function tests (LFT's) were found in 87 % of Group I and 76 % of Group II, but in only 16 % of the «Cryo» group. Differences in LFT's were not great between treated VIII and IX deficient patients. All patients treated with 100,000 units or more showed either persistent or intermittent abnormalities. In the high exposure group, the history of past, overt hepatitis had no influence on observed LFT's. The sera of all patients in the high exposure and all, except one, in the low exposure groups were positive for HbsAg or anti‐Hbsby RIA. Splenomegaly was found in 13 % of fraction‐treated patients.We conclude that there is biochemical evidence of liver disease following large exposure to commercial VIII or IX fractions, which should temper the physician's decision to start treatment with these fractions. On the other hand, evidence that their continued use produces mounting liver dysfunction is insufficient to withdraw this very effective and life‐changing treatment from th

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1977.tb01514.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

摘要:

Seven patients with classic hemophilia A had alteration of primary hemostasis after treatment with lyophilized antihemophilic globulin (LAHG). The following test results, which were normal before treatment, became abnormal after treatment: bleeding time, bleeding intensity, and platelet adhesiveness. In two patients, the fibrin‐fibrinogen‐degradation products increased to more than 40 μg/ml. In three patients, the bleeding symptoms became worse with LAHG therapy although no inhibitor against Factor VIII was demonstrated. In one of these patients, the bleedings symptoms disappeared when the use of LAHG was discontinued and prednisone was given; at the same time, the altered primary hemostasis returned to normal. In the remaining two patients, prednisone did not have any effect. In these two patients, however, the bleeding stopped, and the bleeding time became normal immediately after freshly prepared blood‐group compatible cryoprecipitate was

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1977.tb01515.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

摘要:

Fractionation technology has made Factor IX concentrates available. In addition to a very high incidence of hepatitis, thrombosis is being recognized as a consequence of their use. Contradictory reports exist in the literature as to the incidence of thrombotic events. A recent study of thrombohemorrhagic phenomena after infusion with Factor IX concentrates in patients with congenital Factor IX deficiency (hemophilia B) or liver disease, revealed an incidence of twenty episodes in 188 cases (11 %). It is imperative to delineate the thrombo‐genic factors and find methods for their removal to insure the safety of Factor IX product

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1977.tb01516.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

摘要:

Although haematuria is a relatively common symptom of hemophilia A and B, renal disease seemed to be a rarity and it has usually been held that this symptom was benign in nature. However detailed studies of renal function in a series of such patients using radiological and biochemical tests of renal function show significant differences compared to normal. These abnormalities seem to be associated with recurrent haematuria but do not appear to be related directly to replacement therapy with plasma concentrate and do not occur more frequently in patients who have received long term fibrinolytic inhibitors. Other rare renal disorders associated with haemophilia include nephrotic syndrome, trauma, acute tubular necrosis, analgesic nephropathy and chronic pyelonephritis.

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1977.tb01517.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

摘要:

Serum samples from 134 Greek haemophiliacs multitransfused with fresh frozen plasma and factor ‐ VIII or factor ‐ IX concentrates were investigated for the presence of blood group antibodies. All samples were tested against a panel of selected cells of known but differing genotype. The incidence of blood group antibodies was 12.7 96. Anti‐D was found in 6 out of the 17 immunized patients, i.e., 35.3 % of D‐negative haemophiliacs were sensitized. It is concluded that the high incidence of immunization is a serious complication of repeated plasma trans

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1977.tb01518.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

ISSN:0036-553X    

DOI:10.1111/j.1600-0609.1977.tb01519.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY