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A Chuansumrit

Publications and source records attributed to A Chuansumrit.

At least 73 records · Page 4Linked to original sources
PubMed1994

A comparison of the allelic frequencies of ten DNA polymorphisms associated with factor VIII and factor IX genes in Thai and Western European populations.

The frequency of five factor VIII gene intragenic and linked DNA polymorphisms and five factor IX gene intragenic polymorphisms was studied in Thai females. The polymorphisms in the FVIII gene were detected by restriction enzymes BclI, XbaI, BglI and at linked loci DX13 (DXS15) and St14 (DXS52) by BglII and TaqI, respectively, and in the FIX gene by MseI, DdeI, XmnI, TaqI and HhaI. With the exception of the BglI restriction fragment length polymorphism (RFLP), which is absent in Thais, factor VIII polymorphism frequencies were similar in Thais and Caucasians. Combined use of XbaI and TaqI/St14 resulted in a heterozygosity rate of greater than 90% in Thai females. For FIX, the recently described MseI RFLP in the 5' flanking region was the most informative polymorphism in Thais, 43% of females being heterozygous. The other four polymorphisms added little to the overall heterozygosity rate. The appropriate polymorphisms were used to track defective factor VIII and IX genes through 22 Thai pedigrees with haemophilia to enable carrier status to be assigned to female family members. The information obtained during this study will form the basis for carrier detection and prenatal diagnosis of haemophilia A and B by DNA polymorphism analysis in Thailand.

Alleles↗
PubMed1994

A single base pair deletion in the promoter region of the factor IX gene is associated with haemophilia B.

Patients with the haemophilia B Leyden phenotype show a distinct pattern of factor IX expression characterized by a post-pubertal increase in FIX levels and the remission of clinical symptoms in adult life. This phenotype has previously been linked to single base mutations within transcription factor binding sites in a region of approximately 40 bp around the major start point of transcription of the FIX gene. Here we report a novel mutation in this region within the transcription factor C/EBP binding site at +1 to +18. The mutation is a single base pair deletion from a triplet of thymine residues at +6 to +8. We show that the extent to which this mutation disrupts the binding of C/EBP to its binding site is less marked than the disruption caused by the +13 A-->G mutation of FIX Norwich (1). This correlates with age-matched phenotypic data we have available for the patient reported here and that of FIX Norwich.

Base Composition↗
PubMed1993

An MseI RFLP in the 5' flanking region of the factor IX gene: its use for haemophilia B carrier detection in Caucasian and Thai populations.

We have identified an MseI restriction fragment length polymorphism (RFLP) in the 5' flanking region of the factor IX gene. RFLPs in the factor IX locus are routinely used as linkage markers to track defective factor IX genes through affected pedigrees, allowing diagnosis of the haemophilia B carrier state in female members of the kindred. Currently, seven intragenic polymorphic loci have been characterized allowing carrier diagnosis in 89% of cases in the Caucasian population. Additional screening with the MseI RFLP reported in this publication increases this figure to 94% of cases. In Asian populations only one of these RFLPs (HhaI) is present at any significant frequency. Hence, carrier detection rates are much reduced in comparison to the corresponding figure of 89% in Caucasians. We report that this MseI RFLP is also present in the Thai population. Indeed, when used in combination with the HhaI polymorphism, the MseI RFLP should significantly improve the carrier detection rate in the Thai population from 11% to 40% of cases.

Alleles↗
PubMed1993

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.

Adolescent↗
PubMed1993

Disseminated Burkitt's lymphoma in a child with AIDS.

Rarity of HIV-associated disseminated lymphomas in children initiated this report. The patient was an 18-month-old girl who had a history of chronic cervical lymphadenopathy since 6 months of age. She was first seen because of rapid enlargement of an inguinal lymphnode. The bone marrow aspirate was compatible with Burkitt's lymphoma, L3 cell-type. She was treated with chemotherapy without satisfactory success. She developed neurological involvement of lymphoma. Accidental sharp injury which contaminated her blood, in a medical personnel leaded to having her blood tested for anti-HIV and it was found positive. Her mother had positive anti-HIV presumably acquired from blood transfusion after an abortion in early 1988. It occurred before the donated blood was compulsively tested for anti-HIV. She died at the age of 24 months.

Burkitt Lymphoma↗
PubMed1993

Hepatopathy-thrombocytopenia vs infection-induced hemophagocytic syndrome in Wilms' tumor: a case report.

A 3-year-old boy with Wilms' tumor, post operative left nephrectomy stage, had HTS on day 99 of the combined chemotherapy which lasted for more than 20 days. He had severe respiratory distress due to a moderate amount of ascites and marked pleural effusion. Because of high fever, thrombocytopenia and marked hemphagocytosis in the bone marrow, he received IVIG for 2 days. Normal platelet count and markedly decreased pleural fluid were attained within 3 days. He subsequently tolerated full doses of combined chemotherapeutic agents with an additional one (doxorubicin). In cases of HTS, IAHS should be suspected. The bone marrow should be done and treatment accordingly so that there is no need to decrease, the dosage of chemotherapeutic agents afterwards.

Child, Preschool↗
PubMed1993

Histiocytoses in children: analysis of 120 cases and the bone marrow findings in infection-induced hemophagocytic syndrome vs malignant histiocytosis.

Retrospective analysis of childhood histiocytoses treated at the Department of Pediatrics, Ramathibodi Hospital from May 1970 till June 30, 1992 to verify the prevalence according to the recent classification, course and prognosis was conducted. Among 120 cases, 54 were Class I or Langerhan Cell Histiocytosis (LCH, previously called Histiocytosis-X). Nineteen cases were Class II: Infection-Associated Hemophagocytic Syndrome (IAHS) and Sinus Histiocytosis. Forty-seven cases were class III which included acute monocytic leukemia, juvenile CML; malignant histiocytosis, HMR, and histiocytic lymphoma. Excellent prognosis (cure) was seen in all cases of Hand-Schuller Christian Disease, eosinophilic granuloma, sinus histiocytosis with massive lymphadenopathy and many cases of Class II (except IAHS). The worse prognosis (100% mortality rate) was seen in HMR and juvenile CML. The intermediate prognosis (50%, 54%, 58.8% and 66.7% mortality rate) was seen in MH, HL, LSD and IAHS respectively. To differentiate between IAHS and MH/HMR, the prominent bone marrow findings in 12 cases of IAHS revealed that every case showed prominent hemophagocytosis by the promono-histiocytes/histiocytes, the maximal total erythroblasts (TE) were only 7.5 per cent except for 3 cases; in which one case with agranulocytosis from co-trimoxazole had 84 per cent erythroid cells, one case with prior co-trimoxazole treatment had 37 per cent TE, the last one had 40 per cent TE with massive GI bleeding while in the recovery stage of DHF. All 5 cases of HMR had prominent hemophagocytosis and increased TE (> 22%).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

Thrombotic risk of children with thalassemia.

Pulmonary microthromboembolism is one of the serious complications found in patients with thalassemia. The pathogenesis is undetermined. The thrombotic risk in 44 patients (26 males, 18 females) with beta-thalassemia/hemoglobin E disease and without clinical symptoms of thrombosis were studied. The age ranged from 3-19 yr (X +/- SD = 10 +/- 4). Neither of them had chronic hepatitis B infection. They were divided into three groups according to clinical manifestations as follows: (1) Mild form (n = 12). They did not require blood transfusion. The mean +/- SD of hematocrit was 23.3 per cent +/- 2.3; (2) Severe form (n = 19). They required frequent blood transfusion. The mean +/- SD of hematocrit was 17.7 per cent +/- 1.5; (3) Severe form with splenectomy (n = 13). They seldom required blood transfusion. The mean +/- SD of hematocrit was 21.8 per cent +/- 3.5. Most of the patients had delayed growth. They had high serum ferritin reflecting iron overload status which was prominent in the severe groups (group 2 & 3). The prothrombin time and serum albumin were slightly decreased, and the serum alanine transaminase were slightly increased; all of which reflected mild alteration of liver function. The plasma AT III, PC and PS antigen in the three groups were similar. The mean +/- SD of AT III antigen was 106.7 per cent +/- 22.2 which is normal. The mean +/- SD of PC antigen was 44.2 per cent +/- 14.2 and PS antigen level was 77.2 per cent +/- 17.8.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

Chemotherapy in patients with retinoblastoma.

Chemotherapy composed of vincristine, cyclophosphamide, procarbazine and intrathecal thiotepa/methotrexate (in the indicated ones) were given for 1.5 years in the doubtful and very unfavorable (Reese-Ellsworth classification) cases of retinoblastoma. There were 58 new cases treated at the Department of Ophthalmology and the Department of Pediatrics, Ramathibodi Hospital from October 1, 1985 to December 31, 1991. Eight cases had refused the surgery prior to admission. Nine cases were not given chemotherapy due to the very advanced cases and refusal. Forty-nine cases received chemotherapy. Eighteen cases were not followed. Among 31 evaluable cases, three cases had progression of the disease. Twenty-eight cases (90%) were clinically well 2 months-6 years after discontinuation of chemotherapy. The good result could be attributable to spontaneous regression (minimal percentage), surgery, radiation therapy or chemotherapy. However, obvious chemotherapeutic effects were seen in some cases. Even though 28 cases seemed to have good result including three of the 8 cases who had refused surgery before admission, we should stress that retinoblastoma is a curable disease provided that early diagnosis and treatment are rendered. Ophthalmologic examination in first degree relatives should be done in the bilaterally involved cases.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1993

Response of hemophilia A with bleeding to fresh dry plasma.

Fresh dry plasma (FDP) is a lyophilized form of fresh frozen plasma (FFP) which can be stored at 4 degrees C for one year. One bottle of FDP is prepared from 220 ml of FFP and contains FVIII: C 0.75 +/- 0.3 U/ml (mean +/- 1 SD). This study describes the clinical and laboratory response in 7 severe and 4 moderate hemophilia A patients. The age ranged from 7-17 years (mean +/- SD = 11.7 +/- 2.9 years). Either 12.4 ml/kg. FDP or 12.2 ml/kg FFP was transfused to the patients when they had bleeding episodes such as hemarthrosis, hematoma. 16 episodes of FDP and 12 episodes of FFP transfusion were studied. The bleeding could be effectively controlled by FDP or FFP except one patient who had physical therapy at 8 hours post FDP transfusion. The increment of FVIII:C was 14.1 +/- 5.3% at 30 minutes after FDP transfusion and 12.1 +/- 3.7% at 30 minutes after FFP transfusion. The recovery rate was 83.2 +/- 32.6% in FDP and 65.3 +/- 22.7% in FFP transfusion. The FVIII:C was decreased to 78.9 +/- 12.3%, 55.6 +/- 13% and 16.3% of the initial level at 2, 8, 24 hours after FDP transfusion respectively which were not statistically significantly different from FFP transfusion. No serious complication was found. FDP will replace FFP for the treatment of coagulation disorders such as hemophilia A. It is an useful alternative therapy which can be provided to the hemophiliac patients in the rural area in developing countries.

Adolescent↗
PubMed1993

Response of patients with bleeding disorder to DDAVP administration.

DDAVP has been shown to provide hemostasis in patients with bleeding disorder. Thirty-one episodes of intravenous DDAVP administration (0.3-0.4 microgram/kg) in 22 patients with bleeding disorder were studied. There were 13 patients with hemophilia A, 1 with type I vWD and 8 with inherited and acquired platelet dysfunction. The age ranged from 2.3-26 yrs (mean +/- SD = 10 +/- 4.8). None of the 3 severe hemophilia A patients responded to the treatment. Two out of five episodes in 4 moderate hemophilia A patients responded clinically and had minute increments of F VIII:C. Ten out of eleven episodes (91%) in 6 mild hemophilia A patients had good responses. The dental procedures for these patients were successfully performed without blood component transfusion. The increments of F VIII:C ranged from 1.5-6.8 folds over the baseline levels (mean +/- SD = 2.5 +/- 1.4). In addition, two episodes of epistaxis in a vWD patient responded excellently and one dental procedure was successfully performed by giving DDAVP. The increments of F VIII:C and vWF:Ag ranged from 2.8-12.5 and 2.9-8 fold over the baseline levels respectively. The prolonged bleeding times were shorten to 6.5-7 minutes. Only three out of eight episodes in 8 inherited and acquired platelet dysfunction patients showed temporary responses. The bleeding time responses did not correlate with in vitro platelet aggregation.

Adolescent↗
PubMed1993

HIV seroprevalence in hematologic patients other than hemophiliacs at Ramathibodi Hospital.

Recently there have been increasing reports of HIV infection acquired through transfusion of HIV seronegative blood in Thailand due to high incidence of HIV new infection in blood donors. Blood or blood components (BC) prepared from HIV seronegative blood donation pose significant hazards to recipients because of the risk of viremia during the "window period" of HIV infection. This paper presents the HIV seroprevalence in hematologic patients other than hemophiliacs who received multiple blood transfusion at Ramathibodi Hospital. The retrospective analysis was done on 167 patients: 132 thalassemia, 19 leukemia, 5 aplastic anemia, 5 ITP, 2 pure red cell aplasia, 2 congenital non spherocytic hemolytic anemia, 1 hereditary spherocytosis and 1 autoimmune hemolytic anemia patients, who received blood transfusion during January 1, 1987 till February 29, 1992 at the Department of Pediatrics, Ramathibodi Hospital. The number of blood or BC transfused in each patient was 1-154 units with the average of 23 units per patient per 5 years with a total 4,000 units. All were HIV sero-negative. Anti-HIV screening was performed periodically in these patients about 1-2 times per year or as necessary. The results were HIV seronegative in all cases. The reason for negative results cannot be explained clearly. It should be noted that our thalassemic patients receive leukocyte poor blood and avoid a hypertransfusion program. Patients with other blood diseases received both whole blood and BC. The HIV contaminated blood in the window period was estimated to be 1:10,000 in Thailand which showed HIV antigen positive but antibody negative. These patients may be fortunately received HIV non contaminated blood.

Adolescent↗
PubMed1993

HIV seroconversion in Thai hemophiliacs up to 1991.

In Thailand, the anti-HIV screening in the donor blood was started in 1987 and was compulsory nationwide in February 1989. Sixty-six hemophilia A and 10 hemophilia B patients who received approximately six million units of factor VIII and IX in the form of fresh frozen plasma, frozen cryoprecipitate, cryoprecipitate removed plasma, fresh dry plasma and factor concentrate during 1976 to 1991 were tested for anti-HIV since 1987. The age ranged from 1-39 year (mean +/- SD = 15 +/- 7.3). The anti-HIV test was performed by ELISA and/or gel agglutination and confirmed by Western blot analysis. The patients would be checked 1-2 times per year and as necessary. A total of 174 tests for the first, second, third, fourth, fifth and sixth tests were studied in 76, 49, 27, 14, 5 and 3 patients respectively during 1987 to 1991. The prevalence of HIV seroconversion in the year 1987, 1988, 1989, 1990 and 1991 was 2.2% (1/45), 1.9% (1/53), 1.6% (1/63), 1.5% (1/67) and 3.9% (3/76) respectively. Three HIV seroconversion were found in the first, fourth and fifth anti-HIV test in 3 hemophilia A patients who received massive infusion of blood components during orthopedic corrective surgery. One case of HIV seroconversion found in 1987 was transmitted by HIV unscreened blood while 2 cases in 1991 by anti-HIV seronegative blood whose donors were in the window period of HIV infection. The prevalence of HIV seroconversion in Thai hemophiliacs is much lower than those in western countries.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

Plasmapheresis for the preparation of HIV free cryoprecipitate.

Single donor cryoprecipitate was prepared by blood cell separator for treatment of hemophilia A and von Willebrand patients to reduce a risk of transfusion associated HIV infection. A total of 7 plasmapheresis (range 1-1.5 plasma volume) was performed in 4 donors. Then fresh frozen plasma (FFP) was processed to cryoprecipitate and cryoprecipitate removed plasma (CRP). Donors were replaced with 0.9% normal saline solution (NSS) and 5% albumin for the first donation or their own CRP and 5% albumin for the second and third donation. After plasmapheresis total protein, albumin, IgG, IgA and IgM were below normal level in 71.43% (5/7), 14.3% (1/7), 28.57% (2/7), 14.3% (1/7) and 28.57% (2/7), respectively. All of these parameters returned to normal level within 3 days. Factor VIII:C was decreased after plasmapheresis in all donors and the low level of F VIII:C returned to normal within 24 hours. The donor was not exposed to any harmful effect. Donor reactions observed were mild. One donor was chilled due to unwarmed replacement solution. When this donor donated for the second time and was replaced with prewarmed replacement solution, no reaction was observed. We conclude that a preparation of single donor cryoprecipitate by plasmapheresis is safe and can reduce a risk of transfusion associated HIV infection. The donors are not at risk as the result of changes in the measured plasma protein and factor VIII:C level following plasmapheresis.

Adult↗
PubMed1993

The safe blood components for a hemophilia patient: a case report.

This study comments on safe blood components prepared for a 6 year old boy with hemophilia A (F VIII:C 1.8%). He has required blood transfusion since 10 months of age and started home therapy since 3 years of age. The utilized blood components were fresh dry plasma (FDP) prepared from 8-10 selected donors who were in good health and had no risk of blood-transmitted diseases. They were parents, relatives and friends with the age ranging from 30-45 years old. The FDP would be used after the donors had at least two subsequent negative tests for anti-HIV. In addition, cryoprecipitate collected from parents by plasmapheresis was started in 1990. Twelve to fifteen bags of cryoprecipitate were collected each plasmapheresis. The blood components prepared from the selected donors is an additional management to obtain the better quality of blood. Moreover, the role of parents taking the major responsibility in the recruitment of donors will minimize the shortage of blood donors.

Blood Component Transfusion↗
PubMed1993

Immunoglobulins in acquired platelet dysfunction with eosinophilia.

Acquired platelet dysfunction with eosinophilia (APDE) is acquired bleeding diathesis associated with platelet dysfunction causing prolonged bleeding time. Most of them had eosinophilia. Various reports of the isolated cases had normal level of immunoglobulins but few had increased IgE. This report is different from others in that among 41 cases of APDE, it was found that most of them had normal levels of IgG, IgA, IgE but there was increased IgM in 95.1% of the cases. Increased in IgE was noticed in 7/24 cases (29.2%) and only half (3/6 cases) corresponded with eosinophilia.

Adolescent↗