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Biomedical subjects

A Piankijagum

Publications and source records attributed to A Piankijagum.

At least 19 recordsLinked to original sources

Screening for the carriers of thalassemias and abnormal hemoglobins at the community level.

Thalassemia and abnormal hemoglobins are common genetic disorders in Southeast Asia. Thalassemia is not only an important public health problem but also a socio-economic problem of many countries in the region. The approach to deal with the thalassemic problem is to prevent and control births of the new cases. This requires an accurate identification of couple at high risk to have a thalassemic child. The diagnosis of thalassemia carriers need several tests that are not practical for screening the population at large. In this study we used two simple laboratory tests to screen for potential thalassemia carriers and hemoglobin E individuals. Three-hundred pregnant women and 40 spouses were recruited in this study. The methods were the red cell osmotic fragility (OF) screening test with 0.36% NaCl and the dichlorophenolindophenol (DCIP) precipitation test to detect Hb E and unstable hemoglobins. Standard methods for red cell indices, hemoglobin analysis and detection of alpha-thalassemia by immunological method were also performed to confirm genotypes of thalassemia. The results showed that 98 women (32.7%) were carriers of thalassemias and hemoglobin E. The number of false positive by OF test was 3.2% and by DCIP test was 0.6%. Sensitivity and specificity of OF test were 89.5% and 93.3%, respectively whereas those of DCIP test were 100%. Of the 40 couples investigated, one was found to be at risk of having beta-thalassemia/Hb E fetus. Screening techniques including one tube osmotic fragility and DCIP precipitation tests are sensitive and specific for the detection of thalassemia and unstable hemoglobins such as Hb E. The techniques are also simple, economic, rapid, and give minimal false negative result.

Adult↗

Regional patterns in the incidence of aplastic anemia in Thailand. The Aplastic Anemia Study Group.

The annual incidence of aplastic anemia has been determined in a rigorous and standardized epidemiologic study conducted in Thailand. A total of 374 cases were identified over a period of 3-6 years in three geographically defined and distinct regions of the country; Bangkok, Khonkaen in the northeast, and Songkla in the south. The incidence was 3.9 cases per million persons in Bangkok, 3.0 per million in Songkla, and 5.0 per million in Khonkaen. These rates are as high or higher than in any region of Europe or Israel as reported in the International Agranulocytosis and Aplastic Anemia Study, in which the methods and case definition were the same. Rates were stable over the course of the study. There were marked differences in incidence between northern and southern rural regions of Thailand, and among Bangkok suburbs. These differences, together with an unusual peak in the incidence among young people in Bangkok, suggest the possibility of occupational and environmental factors in the etiology of aplastic anemia.

Adolescent↗

Agranulocytosis in Bangkok, Thailand: a predominantly drug-induced disease with an unusually low incidence. Aplastic Anemia Study Group.

Agranulocytosis, a syndrome characterized by a marked reduction in circulating granulocytes, is strongly associated with medical drug use in Europe and the United States. Unregulated use of common pharmaceutical agents in developing countries has been suspected of causing large numbers of cases of agranulocytosis and deaths, especially among children. To elucidate the incidence and etiology of agranulocytosis in Thailand, a population-based case-control study of symptomatic agranulocytosis that resulted in hospital admission was conducted in Bangkok from 1990 to 1994. An attempt was also made to study the disease in Khonkaen (in northeastern Thailand) and Songkla (in southern Thailand), but there were insufficient cases in the latter regions, and the analysis was confined to subjects from Bangkok. In that region, the overall incidence of agranulocytosis was 0.8 per million per year; there were no deaths. As expected, the incidence was higher in females (0.9 per million), and it increased with age (4.3 per million beyond age 60). Among 25 cases and 529 controls the relative risk estimate for a combined category of all suspect drugs was 9.2 (95% confidence interval = 3.9-21), and the proportion of cases that could be attributed to drug use was 68%. For individual drugs and drug classes the data were sparse; within these limitations, the strongest association appeared to be with antithyroid drugs. One case and three controls were exposed to dipyrone, a drug known to cause agranulocytosis; with such scanty data the risk could not be evaluated. Exposure to pesticides or solvents was not associated with an increased risk. This is the first formal epidemiologic study of agranulocytosis in a developing country. As in the West, most cases are attributable to medical drug use. However, the incidence of agranulocytosis in Bangkok, and apparently, in Thailand as a whole, is unusually low, and the disease does not pose a public health risk.

Adolescent↗

Standardization on laboratory diagnosis of thalassemia and abnormal hemoglobin.

Thalassemia is one of the most common single gene disorders. The geographic distribution of thalassemia and abnormal hemoglobin has been known for many years. A worldwide significant spread of these abnormal genes, especially from Southeast Asia, occurred in the last two decades. This has resulted in a dramatic increase of Hb E disorders and various Southeast Asian thalassemia genotypes, which means that requests for hemoglobinopathy investigations are likely to increase in many laboratories worldwide. Hemoglobinopathy screening and diagnosis may need to be undertaken antenatally, neonatally and in certain hematological situations. The introduction of automation for hemoglobinopathy screening, including the automated cell counting and HPLC system, is an important advance in technology for hematology laboratories. The instruments need to be calibrated and standardized to get an accurate data for interpretation. Internal and external control samples are also needed. Combination of test results is usually required to achieve a proper diagnosis, which in turn, provide a self-check for each laboratory test.

Adult↗

Malignant lymphoma in Thailand: changes in the frequency of malignant lymphoma determined from a histopathologic and immunophenotypic analysis of 425 cases at Siriraj Hospital.

BACKGROUND: Analysis of malignant lymphoma in a single institution at different periods of time can determine the changing status of the disease in the region. METHODS: To compare with the large series of 1095 lymphoma cases reported between 1957-1971 at Siriraj Hospital, the largest hospital in Thailand, a similar study was performed through histopathologic evaluation of 425 lymphoma cases diagnosed consecutively at the same institution between August 1993 and October 1995. Phenotypic analysis was performed by paraffin section-immunoperoxidase studies. RESULTS: A striking increase in lymphoma cases was noted from 73 cases/year in the first series to 189 cases/year in the second series (an increase of 158.9%). Lymphoma occurred in all age groups, with a peak incidence at the seventh decade of life. The male to female ratio decreased from 2:1 in 1957-1971 to 1.3:1 in the more recent series. The incidence of Hodgkin's disease (HD) was found to have decreased from 28.9% to 8.5%. There were 36 cases (8.5%) of HD and 389 cases (91.5%) of non-Hodgkin's lymphoma (NHL) reported in the second series. The subtypes of HD included 16 cases of mixed cellularity, 13 cases of nodular sclerosis, 6 cases of lymphocyte depletion, and 1 case of lymphocyte predominance. According to the Working Formulation, the 389 NHL cases included low grade (14.1%), intermediate grade (57.3%), high grade (11.3%), and miscellaneous groups (17.2%). They were classified as small lymphocytic (9.5%), follicular (11.1%), diffuse (50.9%), immunoblastic (4.1%), small noncleaved (4.4%), lymphoblastic (2.8%), anaplastic large cell (9.0%), mycosis fungoides (1.8%), hairy cell leukemia (0.3%), true histiocytic (0.5%), and extramedullary plasmacytoma (1.0%). The immunophenotypes of the 359 NHL cases available for paraffin section-immunoperoxidase studies were B-cell (71.0%), T-cell (24.5%), histiocyte (0.6%), and undetermined phenotypes (3.9%). CONCLUSIONS: The incidence of malignant lymphoma is increasing in Thailand, with a high frequency of intermediate to high grade NHL of B-cell phenotype reported.

Adolescent↗

Prenatal and postnatal diagnoses of thalassemias and hemoglobinopathies by HPLC.

The conventional approach to qualitative and quantitative analyses of hemoglobin (Hb) molecules for the diagnoses of hemoglobinopathies requires a combination of tests. We used an automated HPLC (VARIANT) system to study alpha-thalassemia and beta-thalassemia syndromes in Thailand. The beta-thalassemia short program is applicable to the diagnosis of alpha-thalassemia and beta-thalassemia disorders, including Hb H, EA Bart's disease, and EF Bart's disease, in adults, newborns, and fetuses. The system cannot quantify accurately certain Hb molecules, such as Hb H and Hb Bart's. The alpha-thalassemia short program was therefore developed and used to quantify Hb Bart's to detect alpha-thalassemia genotypes in cord blood. This automated HPLC system is an alternative approach to the diagnosis of complicated thalassemia syndromes in Thailand and Southeast Asia.

Adult↗

Low drug attributability of aplastic anemia in Thailand. The Aplastic Anemia Study Group.

From 1989 to 1994, a population-based, case-control study of aplastic anemia was conducted in Thailand, including the regions of Bangkok, Khonkaen in the northeast, and Songkla in the south. An annual incidence in Bangkok of 3.7 cases per million population, about twice as high as in Western countries, has been reported. To evaluate the etiologic role of drugs, 253 subjects were compared with 1,174 hospital controls. With multivariate adjustment for confounding, a significant association was identified for exposure 2 to 6 months before admission to thiazide diuretics (relative risk estimate 7.7; 1.5 to 40). There were crude associations with sulfonamides (relative risk estimate, 7.9; P = 0.004) and mebendazole (6.3; P = 0.03) (there were insufficient data for multivariate adjustment). Excess risks for the three drugs were in the range of 9 to 12 cases per million users. There was no significant association with chloramphenicol, although the multivariate relative-risk estimate was elevated (2.7; 0.7 to 10). Other drugs that have been reported to increase the risk of aplastic anemia, such as nonsteroidal anti-inflammatory drugs and anticonvulsants, were not commonly used. There were no associations with commonly used drugs, including benzodiazepines, antihistamines, oral contraceptives, and herbal preparations. For all associated drugs, the overall etiologic fraction (the proportion of cases attributable to an exposure) was 5%, compared with 25% in Europe and Israel. Drugs are uncommon causes of aplastic anemia in Thailand, and their use does not explain the relatively high incidence of the disease in that country.

Adolescent↗

Association of seropositivity for hepatitis viruses and aplastic anemia in Thailand.

Aplastic anemia is more common in the Orient than in western countries, with an incidence in Thailand that is 2- to 3-fold higher than in Europe. Aplastic anemia after hepatitis is a well characterized clinical entity, and clinical hepatitis is also prevalent in the Far East. We performed a prospective case-control study to determine risk factors for aplastic anemia in Bangkok and two rural regions during 1989 to 1994. A total of 375 cases were identified, along with 1,174 hospital controls matched for age and sex. Historical data were collected by trained interviewers. Sera from a subset of cases (N = 177) and controls (N = 183) were tested for antibodies to hepatitis viruses A, B, and C and hepatitis B surface antigen. There was no evidence of association of aplastic anemia with hepatitis B or hepatitis C. Previous exposure to hepatitis A, as determined by immunoglobulin G (IgG) seropositivity, was significantly associated with aplastic anemia: the relative risk adjusted for confounding was 2.9 (95% confidence interval 1.2-6.7). The same association also existed for persons under age 25 years, in whom the prevalence of hepatitis A IgG was lower than in the total population. However, no patients showed evidence of recent infection with hepatitis A (immunoglobulin M [IgM] seropositivity). These results indicate that exposure to a hepatitis virus is a risk indicator for aplastic anemia in Thailand, and while itself unlikely to be etiologic, hepatitis A may be a surrogate marker for another enteric microbial agent.

Adult↗

Use of household pesticides and the risk of aplastic anaemia in Thailand. The Aplastic Anemia Study Group.

BACKGROUND: Aplastic anaemia is a severe blood dyscrasia that is more common in Thailand than in Western countries. Its a etiology remains poorly understood. METHODS: A case-control study was conducted in Bangkok and two rural regions of Thailand. The effect of household pesticides was evaluated among 253 incident cases of aplastic anaemia and 1174 hospital controls. RESULTS: A total of 54% of the cases and 61% of the controls were exposed 1-6 months previously. For most individual household pesticides and for groups classified according to chemical type (organophosphates, pyrethrins, and organochlorines), the relative risk (RR) estimates approximated 1.0; upper 95% confidence limits were below 2.0 for many comparisons. A significant association was observed for exposure to combination products containing dichlorvos and propoxur, with an overall RR estimate of 1.7 (95% confidence interval [CI]: 1.1-2.6); the estimate for regular use was 1.6 (95% CI: 0.9-2.9). CONCLUSIONS: The absence of a higher risk for the regular use of dichlorvos/propoxur reduces the credibility of the apparent association, which could well have been an artefact of multiple comparisons. We conclude that most household pesticides used in Thailand do not appear to increase the risk of aplastic anaemia.

Adult↗

Relation of haemolytic anaemia and erythrocyte-bound IgG in alpha- and beta-thalassaemic syndromes.

There has been evidence that IgG-mediated phagocytosis plays some part in destruction of erythroid cells and subsequent anaemia in thalassaemia. In this study using direct immunofluorescence and flow cytometry, erythrocyte-bound IgG was studied in 53 and 33 beta-thal/Hb E and Hb H patients, respectively. The mean percentages of IgG-positive erythrocytes in both nonsplenectomized (4.4%) and splenectomized (24%) beta-thal/Hb E patients were significantly higher than that in normal subjects (1.0%); p < 0.05 and < 0.001, respectively. Splenectomized beta-thal/Hb E patients had significantly higher percentages of IgG-positive erythrocytes than the nonsplenectomized patients (p < 0.001). The mean percentages of IgG-positive erythrocytes in Hb H patients were comparable to those from normal controls. However, patients with alpha-thal 1/Hb CS had significantly higher IgG-positive erythrocytes than those with alpha-thal 1/alpha-thal 2 (p = 0.008). Splenectomized Hb H patients also had higher IgG-positive erythrocytes than nonsplenectomized Hb H patients (p < 0.039). During haemolytic crisis in some Hb H patients, high percentages of IgG-positive erythrocytes were also demonstrated. This study demonstrates the presence of increased IgG on erythrocytes in patients with beta-thal/Hb E and in those with Hb H disease following splenectomy or in haemolytic crisis.

Adult↗

Hydroxyurea increases hemoglobin F levels and improves the effectiveness of erythropoiesis in beta-thalassemia/hemoglobin E disease.

Hydroxyurea (HU) is one of several agents that have been shown to enhance hemoglobin (Hb) F levels in patients with sickle cell disease and may be useful as a therapy for beta-globinopathies. However, limited information exists on the effects of HU in patients with thalassemia. Accordingly, we examined the hematologic effects of orally administered HU in 13 patients with beta-thalassemia/Hb E, including four patients who had been splenectomized. These patients were treated with escalating doses (final range, 10 to 20 mg/kg/d) for 5 months and were observed in the outpatient hematology clinic every 2 to 4 weeks. Complete blood counts including reticulocyte counts, amounts of Hb E and Hb F, G gamma:A gamma and alpha:non-alpha globin biosynthetic ratios were evaluated before and during treatment. Almost all patients responded with an average increase of 33% in Hb F levels, from a mean (+/- SD) of 42% +/- 11% to 56% +/- 8% (P < .0001), and a reciprocal decline in the percentage of Hb E from 59% +/- 9% to 49% +/- 8% (P < .001). Reticulocytosis was decreased from a mean (+/- SD) of 18.0% +/- 15.6% to 11.7% +/- 9.1% (P < .05); there was also a slight (10%) but statistically significant increase in hemoglobin levels and an improved balance in alpha:non-alpha globin chains ratios. The side effects were minimal in most patients, although these patients tended to tolerate a lower dose of HU before significant myelosuppression than has been our previous experience in sickle cell disease. One splenectomized patient died of sepsis during the trial. We conclude that increased Hb F production in beta-thalassemia/Hb E patients, with an improvement in the alpha:non-alpha globin ratios and, probably, the effectiveness of erythropoiesis, can be achieved using HU. Longer trials of HU in this population, including at other doses and in combination with other agents, appear warranted.

Adolescent↗

Collection of cord blood stem cells for transplantation in thalassemic patients.

Thalassemia is widely distributed throughout the world and is one of the major public health problems. The use of bone marrow transplantation, the only curative therapy for thalassemia, is limited because less than 30% of the patients have unaffected and HLA-identical siblings as donors. Cord blood stem cells, an alternative source of stem cells for transplantation, have been successfully transplanted into patients with several diseases after myeloablative therapy. Twenty cord blood samples from unaffected neonates whose siblings had severe thalassemia were collected. The median volume was 80 ml. The median number of cells and colony forming units-granulocyte-macrophage in cord blood was 9.2 x 10(8) and 3.4 x 10(5), respectively. Four of 20 cord blood samples had HLA-matched to the affected siblings. One patient underwent cord blood transplantation with success; one patient is waiting for transplantation.

Blood Specimen Collection↗

An association of aplastic anaemia in Thailand with low socioeconomic status. Aplastic Anemia Study Group.

The relationship of socioeconomic status to the risk of aplastic anaemia was evaluated in a case-control study conducted in Bangkok and two rural regions of Thailand (Khonkaen and Songkla). Among 152 cases and 921 controls there were significant trends of increasing risk with decreasing years of education (P = 0.01) and total household income (P = 0.0001), after control for confounding. The relative risk estimate for those with monthly incomes of < 1500 baht (about $60 U.S.) was 3.9 (95% confidence interval 2.1-7.3) compared to those with monthly incomes of at least 5000 baht (about $200). The pattern of increasing risk with decreasing income was observed in all three regions, with significant trends in Bangkok (P = 0.004) and Khonkaen (P = 0.003). This finding may partly explain the high incidence of aplastic anaemia in Thailand. Low socioeconomic status may be a surrogate for one or more environmental factors that could cause aplastic anaemia, such as infectious pathogens or toxic exposures.

Adolescent↗

Glomerulonephritis in beta-thalassemia Hb-E disease: clinical manifestations, histopathologic studies and outcome.

We presented 8 patients with beta-thal/Hb E with glomerular diseases. Diverse glomerular lesions were seen, but diffuse endocapillary glomerulonephritis was the most common. The clinical manifestations of acute glomerulonephritis in beta-thal/Hb E differed from typical cases in the older age group, female preponderance, longer duration of edema, less hypertension, marked proteinuria, hypoalbuminemia and hypertriglyceridemia and also a longer period of recovery but their outcome was still favorable despite many risk factors of renal injury. Renal biopsy was necessary in doubtful cases to detect the correct diagnosis and give proper management. The association and mechanism of glomerulonephritis in these patients require further prospective study.

Adolescent↗

Effect of pyrexia in the formation of intraerythrocytic inclusion bodies and vacuoles in haemolytic crisis of haemoglobin H disease.

Clinical aspects and in vivo formation of inclusion bodies and vacuoles in red cells of patients with haemoglobin H disease were studied and compared during steady state and after pyrexia of acute infections. During period of pyrexia, there were haemolytic crises with significant drops of the steady-state haemoglobin levels of 8.6 +/- 0.9 g/dl to 5.6 +/- 1.7 g/dl. The phenomenon was observed with splenic enlargement and significant increase of inclusion body formation. The findings suggested that inclusion body formation in red cells during pyrexia of acute infections played an important role in haemolytic crisis of haemoglobin H disease, probably by causing these cells to be sequestered and destroyed in the spleen.

Acute Disease↗

Significance of blood coagulation and platelet profiles in relation to pulmonary thrombosis in beta-thalassemia/Hb E.

In beta-Thalassemia hemoglobin E (beta-thal Hb E), hypoxemia with abnormal lung function was described and postmortem examination in these patients showed organized pulmonary trombi with thickened arterial wall, particularly in post-splenectomized cases. Coagulation and platelet profiles were studied in 58 beta-thal Hb E patients. In 35 cases with intact spleen, the fibrinolytic activity was significantly decreased with high antithrombin III activity, while coagulation tests revealed mild abnormality. The platelet aggregation to ADP, adrenaline, collagen and ristocretin were defective and platelet 5-hydroxytryptamine content was lower than normal. Twenty-three patients who had been splenectomized for 5-18 years, decreased fibrinolytic activity and high antithrombin III activity were also observed. The coagulation profiles and platelet aggregation in response to ADP, adrenaline and collagen showed better results. Fourteen cases exhibited thrombocytosis and their thrombin generation was in the hypercoagulable range. Platelet aggregation in response to ristocetin remained defective and platelet 5-hydroxytryptamine content was lower than in cases with intact spleens. Defective aggregation to ristocetin would indicate abnormal von Willebrand's factor (vWF). Decreased fibrinolysis should very likely have a role in the occurrence of thrombosis and the better hemostatic profiles in post-splenectomized cases would contribute to the more frequent thrombotic incidence in these cases.

Adolescent↗