Negative pressure dressing for promoting wound healing of purpura fulminans in a newborn with homozygous protein C deficiency.

A negative pressure dressing to promote wound healing of purpura fulminans in a girl aged 35 days with homozygous protein C deficiency is reported. Two wounds of 11 x 11 cm2 at the abdominal wall and 14 x 14 cm2 at the left trunk were covered with sterile sponges embedded with a multiple-hole drain tube and transparent plastic film. The exposed end of the drain was then connected to the wall suction apparatus to create negative pressure at -120 mmHg. The dressing was changed every 2 days. Within 4-6 weeks, the wounds were completely healed and skin grafting was not required.     

National survey of patients with hemophilia and other congenital bleeding disorders in Thailand

A national survey of patients with hemophilia and other congenital bleeding disorders in Thailand was conducted in the years 2000 to 2002. Questionnaires were sent to physicians working at hospitals throughout the country. Although the overall response rate to the questionnaires was 19%, the two highest rates of 80% and 73.7% were found at university and regional hospitals, respectively, where most of the patients received their diagnosis and treatment. A total of 1,450 patients comprised of hemophilia 1,325 cases, von Willebrand disease, 69 cases, congenital factor VII deficiency, 15 cases, hereditary platelet dysfunction, 22 cases, and undefined causes of congenital bleeding disorders, 19 cases. Most were pediatric patients <15 years of age. Treatment was mainly given on demand for a bleeding episode, while only 8.6% received additional home treatment for early bleeding episodes. Replacement therapy primarily relied on fresh frozen plasma, cryoprecipitate and cryo-removed plasma. Factor concentrate was seldom used because of the high price. As a result, hemophilia care services in Thailand should be strengthened by providing comprehensive education for medical personnel, making available simple laboratory kits to determine hemophilia A and B, ensuring an adequate supply of blood components and affordable factor concentrate, and establishing home care treatment.     

Cost-effectiveness in establishing hemophilia carrier detection and prenatal diagnosis services in a developing country with limited health resources

The cost-effectiveness of carrier detection and prenatal diagnosis for hemophilia at the International Hemophilia Training Center, Bangkok, Thailand was studied. From 1991 to 2002, 209 females from 124 families with hemophilia A and B were included. There were 180 hemophilia A carriers and 29 hemophilia B carriers which could be classified into 78 obligate and 131 possible carriers. The phenotypic analysis for possible carriers involved the determination of levels of factor VIII or IX clotting activity (FVIII:C, FIX:C) and the ratio of FVIII:C and von Willebrand factor antigen. The result revealed that 49 females (37.4%) were diagnosed as carriers, 65 females (49.6%) were normal and 17 females (13%) were undetermined. Additional genotypic analysis was provided to 46 families with 74 females with obligate, proven or undetermined carriers within the reproductive life. The polymorphisms associated with factor VIII and IX genes were used including Bcl I for the factor VIII gene and combined use of Mse I, Sal I, Nru I, Hha I and Dde I for the factor IX gene. The informative rate was 59.4% (44/74). Consequently, 12 prenatal diagnoses for fetus at risk were performed. Sex determination was initially determined and followed by the diagnosis of hemophilia through informative gene tracking and/or the measurement of fetal levels of FVIII:C or FIX:C. The result revealed that 3 male fetuses were affected. The total cost of carrier detection and prenatal diagnosis that the families had to pay in the government hospital was 238,600 Baht (US dollars 5,965). It was compared to the estimated cost of minimal replacement therapy using lyophilized cryoprecipitate for the survival time of 30 years in one patient with hemophilia of 1,012,500 Baht (US dollars 25,312.5). The cost of prevention was much less than the replacement therapy. In conclusion, it is cost-effective to establish the service for carrier detection and prenatal diagnosis for hemophilia especially in developing countries with limited health resources.     

Acquired aplastic anemia in children: a review of 100 patients

A retrospective study of acquired aplastic anemia in 100 Thai children treated with testosterone and prednisolone during 1969 to 1987 is reported. The age ranged from 3-14.5 years (mean 10.3 years). The male to female ratio was 2.3:1. The duration of follow up ranged from 1-17 year (mean 5 years). Prior exposure to possible etiologic agents was found in 36% : antipyretics, Ya-chood, insecticides, benzene, chloramphenicol and paint. The presenting symptoms were bleeding and anemia 72%, fever with either bleeding or anemia 28%. The common sites of bleeding were purpura, epistaxis, gum and teeth, and the gastrointestinal tract. All patients received appropriate supportive treatment and testosterone combined with prednisolone. The results of treatment were evaluated in 80 cases who were followed up until death or at least one year after the diagnosis. Two-thirds of the patients presented with laboratory findings similar to severe aplastic anemia; the fatality rate was 50% (40/80). Most of them succumbed to infection or bleeding in the first 6 months after diagnosis. The recovery rate was 50% with complete, partial and initial responses in 28%, 17% and 5% respectively. The late malignancy rate was 2%. For severe aplastic anemia or for patients who have poor prognostic factors, early approaches of bone marrow transplantation or antilymphocyte globulin administration should be considered.     

HIV Transmission by Seronegative Blood Components: Report of 2 Probable Cases

HIV seroconversion was reported in 2 haemophiliacs after having corrective orthopaedic surgery. They received solvent-detergent/heat-treated factor VIII concentrate, HIV-seronegative cryoprecipitate and fresh frozen plasma during the course of surgery. HIV seroconversion was found on days 31 and 71 after surgery. It is highly probable that the infections were acquired by transfusions of seronegative blood components. In countries with a relatively low prevalence of HIV infection, transmission of HIV by transfusion of derivatives of seronegative blood is occasionally reported as a rare complication of blood transfusion [1–3]. In Thailand the prevalence of HIV infection and the incidence of new infections in the general population and in blood donors has recently increased dramatically (fig. 1) [4–5]. As a result of these components prepared from HIV-seronegative blood donations pose a significant hazard to recipients because of the risk of viraemia during the ‘window period’ of HIV infection. Here we report HIV infection in 2 haemophilia patients treated with HIV-seronegative (using Fujirebio agglutination or second-generation Abbott ELISA) cryoprecipitate and fresh frozen plasma in 1991, prepared locally from single-unit donations. All donors were voluntary. Anti-HIV was tested in every unit of donor blood before processing to blood components. Although not proven, it is highly probable that the infections were acquired by transfusions of seronegative blood components.     

Transfusion requirements in patients with dengue hemorrhagic fever

Dengue viruses are endemic in Thailand and Southeast Asian countries. A retrospective study of 175 patients with dengue virus infection admitted at the Department of Pediatrics, Ramathibodi Hospital in 1997 was carried out. Fifteen and 160 patients were clinically diagnosed with dengue fever and dengue hemorrhagic fever (DHF), respectively. DHF was commonly found in patients whose ages ranged from 10 to 14 years. The mean body weight was at the 54th percentile for age. In the management, 10.6% of patients with DHF required blood component therapy which included platelet concentrate (64.7%) in patients who exhibited active bleeding, packed red cells (47%) in patients who exhibited a rapid drop in the hematocrit and fresh frozen plasma (29.4%) in patients with circulatory failure who did not respond to intravenous fluid. The transfusion requirement was significantly correlated with the occurrence of bleeding (p < 0.008) and bleeding in the gastrointestinal tract (p < 0.0001) but not correlated with the number of platelet counts (p = 0.207). As a result, physicians in charge should be aware of the transfusion requirement and communicate this to the blood bank in advance for the preparation of appropriate blood components.     

Detection of known haemophilia B mutations and carrier testing by microarray

The molecular basis of haemophilia B is heterogeneous and many mutations of the Factor IX (FIX) gene have been characterised. Using the allele-specific arrayed primer extension (AS-APEX) technology, we have designed a FIX array to simultaneously analyse 69 mutations found in British, Thai and Chinese patients. This technology overcomes the problem of multiple reverse dot-blot analysis and has a 100% accuracy in the detection of both affected subjects and carriers in families with known mutations. In seven unknown mutations from Thailand, the array could detect the specific mutation in five and in the remainders the normal primer at specific spots failed to extend due to a mutation a few nucleotides upstream, thus allowing their identification. Hence this FIX array can detect 53% of the 2891 mutation entries in the FIX database. Each of the microarray slide can be used for three different test samples and would be useful for carrier testing for common mutations and prenatal diagnosis. It is simpler and more cost effective than genome sequencing and would be particularly useful in laboratories with limited technical capabilities.     

Allogeneic peripheral blood stem cell transplantation in children with homozygous beta-thalassemia and severe beta-thalassemia/hemoglobin E disease

To determine the outcome of children with homozygous beta-thalassemia (beta/beta) and severe beta-thalassemia/hemoglobin E disease (beta/E) who underwent allogeneic peripheral blood stem cell transplantation (PBSCT). The authors conducted a cohort study of allogeneic PBSCT in beta/beta and beta/E patients who had 6/6 or 5/6 HLA-matched sibling donors. All patients received a conditioning regimen including busulfan and cyclophosphamide, except one who received busul-fan and cyclophosphamide plus antithymocyte globulin. Graft-versus-host disease (GVHD) prophylaxis consisted of cyclosporine A and methotrexate for eight patients and cyclosporine and mycophenolate mofetil for one patient. Donors received G-CSF for 4 days before leukapheresis collections. There were five beta/beta and four beta/E patients in this study. The median age was 9 years (range 1.5-10 years). The median CD34+ cell count was 7.4 x 10(6) cells/kg recipient body weight. All patients achieved neutrophil and platelet engraftment with a median time of 15 days and 21 days respectively. Acute GVHD grade 2 to 4 appeared in four patients (grade 2, n = 3; grade 4, n = 1). Three patients developed chronic GVHD (limited, n = 2; extensive, n = 1). All patients were alive with a median follow-up time of 23 months (range 7-52 months). Neither graft failure nor graft rejection was observed. Allogeneic PBSCT is feasible for children with beta/beta and beta/E, although the incidence of GVHD was apparently high compared with bone marrow transplant study in Thais.     

Nursing roles in orthopaedic joint correction in haemophiliac patients.

The roles of nurses in 16 orthopaedic joint corrections of 14 haemophilia A patients (eight severe, six moderate) are described. The patients' ages ranged from 10 to 37 years with a mean age of 17 years and 4 months. The nursing tasks could be divided into three stages. The first is preoperation, the nurse acting as care provider on a team of experts involved in planning the corrective surgery for the affected joints; and as a counsellor for preparing the patients and family members to cope with the challenging operation. The second is intraoperation, the nurse having the role of care provider, giving factor concentrate either by bolus injection (seven episodes) or continuous infusion (nine episodes). The third is postoperation, as a comprehensive care provider, giving cryoprecipitate and/or factor concentrate and monitoring bleeding doses, and as a trainer, teaching the patients how to dissolve blood components and self-venepuncture with aseptic technique. Home treatment for early bleeding was given for 11 episodes, while 6-month prophylaxis was given for five. The accomplishment of these different roles required good communication and nurturing skills, a well-adjusted personality and a warm and positive attitude. The successful performance of nursing roles allows the haemophiliac patients to have a near-normal quality of life.