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Biomedical subjects

D Almagro

Publications and source records attributed to D Almagro.

15 recordsLinked to original sources

Successful surgical treatment of haemophilic pseudotumour, filling the defect with hydroxyapatite.

A preliminary report is made on the use of hydroxyapatite for surgical procedure of pseudotumour, a rare but serious complication of the bleeding diathesis in patients with inherited bleeding disorders. Surgical or percutaneous treatment and refilling with fibrin sealant is shown to be a successful therapy in a 19-year-old male with severe haemophilia B. The pseudotumour, in the upper pad of the left leg, was filled with hydroxyapatite after surgery. We suggest that the use of hydroxyapatite is a new and useful option in the surgical treatment of haemophilic pseudotumour.

Adult↗

Factor concentrates for haemophilia in the developing world.

The success in the management of haemophilia in the last two decades has been predominantly due to the availability of sufficient quantities of safe factor concentrates. Unfortunately, the prohibitive cost of these products has prevented this benefit from being available to the vast majority (approximately 80%) of haemophiliacs living in the developing world. A few developing countries have established facilities for the production of low- to intermediate-purity factor concentrates locally. The infrastructure required to achieve this can be very basic. The experience in South Africa, Thailand, Cuba and Brazil, described herein, shows that this approach provides factor concentrates which are very economical in comparison with more purified commercial products. This has had a major impact on the quality of haemophilia care in these countries. Wide availability of low-cost factor concentrates has made these products accessible to a large number of haemophiliacs and even made home therapy possible. The effort to provide these products results in improvement of the blood transfusion services. This, in turn, contributes to better facilities for patients with other transfusion-dependent diseases and society in general. Installation of small plasma fractionation plants is also a viable option. This not only allows processing of large pools of plasma for greater quantities of factor concentrates but also provides albumin and immunoglobulin. The revenue generated from the sale of the other products has been used to improve and subsidize haemophilia care. It is concluded that local production of intermediate purity factor concentrates in developing countries is absolutely necessary. A well organized transfusion service is required to collect adequate quantities of plasma for fractionation.

Blood Coagulation Factors↗

Prothrombin Habana: a new dysfunctional molecule of human prothrombin associated with a true prothrombin deficiency.

A Cuban family with a new congenital dysprothrombinaemia is described. The propositus was a 5-year-old female who presented with umbilical bleeding after birth followed by easy bruising and bleeding tendency throughout her life. The main laboratory features of the defect included prolongation of prothrombin time and partial thromboplastin time. Prothrombin activity was less than 10% in several one- and two-stage systems. However, the staphylocoagulase-complexed prothrombin level and immunologic methods yielded levels of about 50%. The migration of the abnormal prothrombin was more anodic in single and bidimensional immunoelectrophoresis system and did not change by the addition of calcium. Family studies revealed that the father had approximately 50% prothrombin activity and antigen, whereas the mother had 45% prothrombin activity but about 100% prothrombin antigen. We suggest that the propositus is heterozygous for an abnormal prothrombin and heterozygous for true prothrombin deficiency.

Child, Preschool↗