Biomedical subjects

I M Risseeuw-Appel

Publications and source records attributed to I M Risseeuw-Appel.

5 recordsLinked to original sources

PubMed1996

Acquired Von Willebrand disease in children with a Wilms' tumor.

The association between acquired von Willebrand disease and Wilms' tumor has been reported in eight cases: four cases reports and one prospective study of Coppes et al. (J. Clin Oncol 10:422-427, 1992) who found this in four out of 50 patients. We retrospectively studied 73 children who were diagnosed with a Wilms' tumor between 1970 and 1993. All patients were treated according to the running international SIOP protocol. According to our local diagnostic workup protocol, blood samples for screening coagulation tests were obtained at diagnosis and during preoperative chemotherapy. Since 1984, factor VIII analysis was added. In four patients, no coagulation screen was done. Bleeding time and screening tests apart from APTT were normal in all 69 children tested before or within 2 days after starting therapy. In 47 out of 73 patients, an APTT was performed before starting therapy. In 19 patients (40%), it was prolonged (>33 sec). In 8 of them (17%), the prolongation was severe (> or = 40 sec). In 11 out of the 19 patients, factor VIIIc, factor VIIIag, and factor VIII RcoF determinations were done. In two children, all three factors were decreased suggestive for von Willebrand disease. One of the 19 patients with a prolonged APTT had hematuria. The others had no increased bleeding tendency or signs of bleeding in the tumor. In all patients, the prolonged APTT normalised during preoperative chemotherapy within 6 weeks. Frequent blood samples were obtained of the two children with acquired von Willebrand disease and showed normalisation of the coagulation disorder after 1 and 2 weeks, respectively. No specific therapy to correct the coagulation abnormalities was given to any patient.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1996

The relationship between plasminogen activator inhibitor-1 and proinflammatory and counterinflammatory mediators in children with meningococcal septic shock.

Proinflammatory cytokines (tumor necrosis factor [TNF]-alpha and interleukin [IL]-6 and -8), counterinflammatory compounds (IL-10 and soluble TNF receptors p55 and p75 [sTNFR-55 and -75]), and hemostatic parameters were determined in 38 patients with meningococcal septic shock. Eleven patients (29%) died. Serum levels of pro- and counterinflammatory compounds and plasma levels of plasminogen activator inhibitor (PAI)-1 were significantly higher in nonsurvivors. The interval between appearance of petechiae and blood sampling was shorter in nonsurvivors than in survivors (3.6 +/- 2.4 vs. 6.1 +/- 3.3 h; P = 0.4). This interval correlated strongly with the levels of TNF-alpha, IL-6, -8, and -10, sTNFR-55 and -75, and PAI-1. However, with the exception of PAI-1, differences between concentrations of these mediators disappeared after adjustment for the interval. PAI-1 levels correlated with TNF-alpha concentrations (r = .75; P < .001) and were 1.9 (P = .01) times higher in nonsurvivors at a similar TNF-alpha concentration. Thus, an increased PAI-1 response to TNF-alpha may be associated with fatality, probably because of polymorphism of the PAI-1 gene.

Adolescent↗

PubMed1996

Age-related differences in outcome and severity of DIC in children with septic shock and purpura.

We studied the influence of age on mortality and severity of clotting abnormalities in 79 children (median age: 3.1 years) with meningococcal sepsis. Parameters of coagulation and fibrinolysis and plasma levels of cytokines were prospectively measured on admission. The mortality rate was 27%. The age of survivors was significantly different from that of non-survivors (p = 0.013). With the exception of FVII, vWF and t-PA, parameters of coagulation and fibrinolysis, as well as plasma cytokine levels were related to outcome. Patients were divided in two groups: younger and older than median age. The mortality in children < or = 3.1 years was 40% versus 13% in children > 3.1 years (p = 0.006). In contrast to cytokine levels, which were not different between the two age groups, fibrinogen, prothrombin, factors V, VII, VIII, vWF, protein C, antithrombin, FDP, and the ratio PA1-1/t-PA were related to age, indicating a more severe coagulopathy in children < or = 3.1 years despite a similar degree of inflammatory response. A relative deficiency of coagulation factors due to an immature state of the clotting system, as well as an inadequate fibrinolytic response, both related to age may have caused this more severe coagulative response in younger children, and may have contributed to the higher mortality rate.

Adolescent↗

PubMed1994

Minimal effects of E. coli and Erwinia asparaginase on the coagulation system in childhood acute lymphoblastic leukemia: a randomized study.

A randomized study was done in twenty newly diagnosed children with acute lymphoblastic leukemia. Ten children were treated with Escherichia coli L-asparaginase, and ten with Erwinia chrysanthemi L-asparaginase. L-asparaginase (ASP) treatment started halfway during ALL-induction treatment with vincristine, prednisone, daunorubicin and intrathecal methotrexate. The mean activated partial thromboplastin time (APTT) level in all children demonstrated a significant fall (P < 0.001) from 28.25 sec at diagnosis to 23.0 sec at the start of ASP treatment. In this same time interval, the mean fibrinogen level declined markedly from 3 g/l to 1.2 g/l (P < 0.001), probably due to prednisone therapy. The APTT stayed shortened during ASP therapy, whereas the hypofibrinogenemia recovered significantly faster in the Erwinia group (P < or = 0.01). Factors (F) II, V, VII and X stayed within the normal range, while F VIII and F IX were elevated. During the entire period of induction therapy, the ATIII activity remained within the normal range in both treatment groups. The protein C values, however, demonstrated a steady decline from 140% at start of ASP treatment to a mean of 81% and 93%, respectively, at the end of the ASP therapy in the E. coli and Erwinia group. Five of the ten children treated with E. coli ASP demonstrated protein C levels below 70% at the end of ASP therapy, opposed to none of the Erwinia treated patients (P = 0.03). We suggest that the effect of ASP resulting in decreased coagulation factor synthesis is in part counterbalanced by the effect of prednisone on the coagulation system, when ASP is administered at the end of ALL induction treatment. The overall effect of ASP either of E. coli or of Erwinia on the hemorrhagic system reveals a slight imbalance towards thrombosis, mainly because of a gradual decrease in protein C activity. This imbalance is less pronounced in the Erwinia group.

Asparaginase↗

PubMedDate not supplied

Transfusion syphilis: a case report.

An infant girl acquired syphilis after exchange transfusion with fresh whole blood in 1977 in Rotterdam. She showed typical features of early infectious syphilis three and a half months after the transfusion. The parents had no histories or clinical or serologic signs indicative of syphilis. Although the blood donor had negative serologic tests for syphilis shortly before giving the blood, later examination indicated that he must have become infected with Treponema pallidum shortly before donation of the blood used in the transfusion of the infant.

Exchange Transfusion, Whole Blood↗