Assessment of the standard pediatric unfractionated heparin dosing protocol.

Current dosing guidelines for unfractionated heparin therapy in pediatric patients are based on recommendations of only one study that evaluated a weight-based dosing nomogram. To test the hypothesis that adhering to a strict weight-based nomogram yields better therapeutic results in pediatric patients, we prospectively monitored 25 consecutive pediatric patients who received unfractionated heparin based on the nomogram, and compared them to control patients whose treatment did not follow the standard nomogram. The mean time needed to achieve the target activated partial thromboplastin time was significantly shorter in the study group than the control group (18.32 ± 9.98 vs. 43.8 ± 30 h). A higher proportion of the study group reached the target activated partial thromboplastin time at 12, 24, and 36 h, compared to controls: 44% vs. 6%, 72% vs. 28%, 100% vs. 58%, respectively. Within the study group, patients under 1 year of age needed more time to achieve the target activated partial thromboplastin time than those over 1-year old. The performance of the standard dosing nomogram was excellent with regard to early anticoagulation target achievement, without increasing the risk of bleeding. Further studies are warranted to refine this nomogram for pediatric patients who are less than 1-year old.

Hemoglobin D/beta-thalassemia and beta-thalassemia major in a Saudi family.

The present report describes the clinical, hematological and molecular characteristics in a family with unique interaction between 3 different mutations discovered during routine workup for bone marrow transplantation. In this report, complete hematological and molecular studies were performed for a large Saudi family. The family consisted of parents and 9 children, which revealed that the father is compound heterozygous for hemoglobin Hb D Punjab/beta-thalassemia, the mother is a carrier for beta-thalassemia and 3 of their children are transfusion dependent beta-thalassemia. Two of the children are compound heterozygous for Hb D Punjab/beta-thalassemia like the father but with different genotype. The other 2 children have Hb D Punjab traits while 2 other children have beta-thalassemia traits. Although, compound heterozygous for Hb D/beta-thalassemia has been well described in the literature, our report emphasizes the importance of careful analysis of the electrophoresis results and the usefulness of molecular studies in premarital screening and other screening hemoglobinopathy programs.