Coagulation and fibrinolysis in children, adolescents, and young adults with inflammatory bowel disease.

BACKGROUND: Patients with Crohn's disease and ulcerative colitis have an increased risk of thromboembolic events. METHODS: Data were collected from 24 patients aged 4.5 to 23 years who had inflammatory bowel disease. Platelet count, antithrombin, fibrinogen, prothrombin fragment F1+2, soluble thrombomodulin, tissue plasminogen activator, D-dimer, and plasminogen activator inhibitor-1 antigen were investigated. In addition the response to activated protein C, the factor V R506Q mutation, protein C, free protein S antigen, and lipoprotein (a) were analyzed. These data were compared with medical treatment, duration, and disease activity, estimated with the Pediatric Crohn's Disease Activity Index or the Clinical Colitis Activity Index. RESULTS: Forty-five percent of our patients showed an increase in fibrinogen, 29% in prothrombin fragment F1+2, and 20% in platelet count, plasminogen activator inhibitor- antigen, and soluble thrombomodulin. Thrombomodulin was higher in active disease than in inactive disease and in Crohn's disease than in ulcerative colitis. Fibrinogen was also higher with Crohn's disease and tended to be higher in active disease than in ulcerative colitis and inactive disease. Plasminogen activator inhibitor-1 antigen was significantly higher in patients with Crohn's disease than in those with ulcerative colitis and was higher in the patient group treated with steroids. CONCLUSION: As has been shown in adults, young patients with active and inactive inflammatory bowel disease were found to have abnormal coagulation and fibrinolysis. The relevance as a thromboembolic risk factor is discussed.

False-positive gliadin and endomysium antibodies and exocrine pancreatic insufficiency as pitfalls in the differential diagnosis of duodenal Crohn's versus celiac disease.

We report a 13-yr-old boy with Crohn's disease in the upper gastrointestinal tract presenting with abdominal pain, failure to thrive, recurrent fever, iron-deficient anemia, and exocrine pancreatic insufficiency. Initially, latent celiac disease was suggested because of normal endoscopic findings, the finding of non-specific inflammation on histological evaluation of duodenal biopsies, positive IgA and IgG gliadin, as well as endomysium antibodies and exocrine pancreatic insufficiency. There was no response to a gluten-free diet. A reevaluation revealed Crohn's disease.

Inefficacy of fresh frozen plasma in the treatment of L-asparaginase-induced coagulation factor deficiencies during ALL induction therapy.

A prospective longitudinal study was conducted to determine whether single-donor fresh frozen plasma (FFP) substitution was able to influence L-asparaginase-associated hypoproteinemia. Within a 36-month period, 20 of 42 children with ALL received a total of 42 prophylactic FFP doses at a median of 10 (5-20) mliter/kg when fibrinogen levels decreased to < 60 mg/dL and thrombin time was lengthened. Laboratory monitoring before, during and after FFP substitution showed no short-term improvements and demonstrated only a minimal increase in fibrinogen and alpha 2-antiplasmin. Plasma levels of antithrombin and plasminogen remained unchanged. Furthermore, administration of FFP had no influence on thrombin generation, the plasmin/alpha 2-antiplasmin complex or enhanced D-dimer formation.