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Biomedical subjects

S Fucharoen

Publications and source records attributed to S Fucharoen.

234 records · Page 13Linked to original sources
PubMed1984

Cardiac pathology in 47 patients with beta thalassaemia/haemoglobin E.

Autopsy protocols and heart slides of 47 patients with beta thalassaemia/haemoglobin E disease were reviewed. All but 1 patient had cardiac hypertrophy, accompanied by dilatation in 17; 5 of 9 patients (56%) with right ventricular and 11 of 22 patients (50%) with biventricular hypertrophy had chronic pulmonary thromboembolism. Cardiac iron deposition while present in 15 patients (32%) was very slight, in contrast to the amount of iron in their liver and pancreas. Four patients had fibrinous pericarditis, 2 with rheumatic heart disease. Twelve patients had chronic pericardial changes, 7 with adhesive pericarditis. The effects of cardiac pathology on the morbidity and mortality of patients with beta thalassaemia/haemoglobin E disease were discussed.

Adolescent↗
PubMed1984

Cholecystitis associated with Enterobacter agglomerans.

A case report of chronic cholecystitis due to Enterobacter agglomerans, occurred in a 54-year old female with homozygous beta-thalassemia. The patient responded successfully to cholecystectomy and sulfamethoxazole + trimetroprim therapy. The source of the infection was not known, however, cystic duct obstruction and immune deficit were thought to be the predisposing causes.

Cholecystectomy↗
PubMed1982

Heterogeneity of beta thalassaemia in Thailand.

Beta thalassaemia in Thailand is heterogeneous. Clinical, genetical, haematological and globin chain biosynthetic studies were performed in seven beta-thalassaemia families. The results showed different gene combinations. These were alpha-thalassaemia/homozygous beta 0-thalassaemia, questionable double heterozygosity between a beta-thalassaemia and a silent beta-thalassaemia genes with low Hb F of unexplained cause, silent beta-thalassaemia/beta+ - or beta 0-thalassaemia, high Hb A2 high Hb F-beta-thalassaemia - a new mutant, mild beta+-thalassaemia/Hb E, and beta 0-thalassaemia/heterocellular HPFH or delta beta-thalassaemia associated with alpha-thalassaemia. Most of variability of clinical and haematological findings in these families is due to heterogeneity of the beta-thalassaemia and related genes.

Adolescent↗
PubMed1981

Homozygous haemoglobin Constant Spring: a need for revision of concept.

Twenty-two patients with mild haemolytic anaemia and haemoglobin (Hb) Constant Spring (CS) of around 6% were studied because they were suspected of having homozygous Hb CS. Family studies revealed Hb CS trait in both parents of eight patients, supporting that they were homozygous for Hb CS. The other patients were included because they had clinical and haematological features similar to the diagnosed cases of homozygous Hb CS. Heterozygosity and homozygosity for Hb CS are clearly distinguishable in that the former is asymptomatic but the latter is associated with overt haemolytic anaemia, and the levels of Hb CS in the two conditions of less than 1% and around 6%, respectively, do not overlap. The findings in homozygous Hb CS contradict prediction. There are four alpha-structural genes per normal human diploid genome. Hb CS trait is believed to be almost equivalent to alpha-thalassaemia 2 or a loss of one alpha-gene because HB CS, and alpha-variant, is barely or not detectable. Homozygosity for Hb CS has thus been predicted to be equivalent to alpha-thalassaemia 1 or a loss of two genes. The latter is asymptomatic and associated with microcytic-hypochromic red cells. However, Hb CS homozygosity presents with mild overt haemolytic anaemia and normal sized red cells. Pathogenesis associated with Hb CS inheritance is more complex than originally believed. There is a possibility that the unstable alpha CS mRNAs precipitate and aggregate leading to pathology of red cells and to the basophilic stippling appearance, so striking in this syndrome.

Adolescent↗
PubMed1981

Ineffective erythropoiesis in haemoglobin E beta -thalassaemia: an electron microscope study.

Electron microscope studies have been performed on the bone marrow cells of two non-splenectomized patients and the circulating erythroblasts and reticulocytes of three splenectomized patients with HbE/beta-thalassaemia. Some intracellular precipitates (probably consisting of alpha-chains) and mild dyserythropoietic changes were found in the early polychromatic erythroblasts within the bone marrow. Larger quantities of precipitate and more marked dyserythropoietic changes were found in the late polychromatic erythroblasts and reticulocytes both within the marrow and within the circulation. The bone marrow macrophages contained phagocytosed erythroblasts within their cytoplasm. These data indicate that the anaemia in HbE/beta-thalassaemia results largely from dyserythropoiesis and ineffective erythropoiesis. The ultrastructural abnormalities encountered in the cases of HbE/beta-thalassaemia were qualitatively and quantitatively similar to those seen in homozygous beta-thalassaemia.

Adult↗
PubMed1981

Morphology and kinetics of erythropoiesis in haemoglobin H disease.

Some aspects of erythroblast function have been investigated in two Thai patients with haemoglobin H disease. Electron microscope and high resolution autoradiographic studies revealed that (1) some erythroblasts and marrow reticulocytes contained branching intracytoplasmic inclusions and (2) the presence of such inclusions did not necessarily prevent the entry of cells either from the G1 phase into the S phase or from the G2 phase into mitosis. The distribution of early polychromatic erythroblasts in the different stages of the cell cycle was determined by the technique of combined Feulgen microspectrophotometry and 3H-thymidine autoradiography and found to be essentially normal. It is concluded that although the presence of an excess of alpha-chains in the beta-thalassaemia syndromes has been previously shown to be associated with a gross impairment of erythroblast proliferation, the presence of an excess beta-chains in HbH disease is not.

Adult↗
PubMed1981

Hypoxaemia and the effect of aspirin in thalassaemia.

Blood gas analysis of arterial blood was performed in 34 patients with beta o-thalassaemia/haemoglobin E disease and homozygous beta o-thalassaemia. Among the 22 splenectomised patients 19 had PaO2 lower than the normal expected value, and 18 of these had PaO2 lower than 80 mmHg. Of the 12 non-splenectomised patients 5 had PaO2 lower than the expected normal value but only in one case it was lower than 80 mmHg. After aspirin or Persantin administration there was a definite rise in the PaO2 in 10 out of 12 patients. The hypoxaemia is believed to occur from increased platelet aggregation leading to pulmonary artery occlusion. The rise of the arterial PaO2 after aspirin administration indicates that the observed hypoxaemia is due to reversible platelet aggregation in the majority of cases.

Adolescent↗
PubMed1981

Increased circulating platelet aggregates in thalassaemia.

Examination for circulating platelet aggregates according to Wu and Hoak revealed increased circulating platelet aggregates in 71% of splenectomized and 35% of nonsplenectomized patients with beta (0)-thalassaemia/Hb E disease. This may be causally related to the newly observed high incidences of pulmonary artery thrombosis and hypoxaemia in splenectomized thalassaemic patients. It is recommended that anti-platelet aggregation drugs such as aspirin and/or dipyridamole are given to thalassaemic patients after splenectomy.

Humans↗
PubMed1980

Pulmonary artery obstruction in thalassaemia.

A new feature has been encountered in review of a large species of autopsy materials of beta-thalassaemia/Hb E disease. Among 43 patients pulmonary arterial obstructive lesions were found in 19 (44%), of which 17 were splenectomised cases. The pulmonary arterial thromboembolism may have been due to circulating platelet aggregates. This newly discovered pathology may be an additional factor contributing toward dyspnoea and heart failure in thalassaemia besides anaemia and cardiac iron deposition. If it is proven that this pulmonary arterial thromboembolism is indeed due to circulating platelet aggregates, preventive measure by administration of drugs reducing platelet aggregation such as aspirin and Persantin may be indicated, especially after splenectomy.

Adolescent↗
PubMedDate not supplied

Fatal arteritis due to Pythium insidiosum infection in patients with thalassaemia.

Six thalassaemic patients had a distinct clinical syndrome characterized by progressive ischemia of the lower extremities, with ascending arteritis and thrombosis of the main arteries of the lower limbs. With periodic acid Schiff and Gomori's methenamine silver staining a large number of hyphae were revealed in the arterial wall and the outer part of the thrombus. Pythium insidiosum was isolated from 3 patients. The clinical course of the disease was progressive gangrene of the extremities and the patients invariably died when the infectious process reached the bifurcation of the aorta. There is no effective antimicrobial agent for the syndrome and radical amputation was the only method to ensure survival of the patients. P. insidiosum infection should be considered in thalassaemic patients with leg ulcers or arterial occlusion of the lower limbs.

Adolescent↗
PubMedDate not supplied

Clinical manifestation of beta-thalassemia/hemoglobin E disease.

PURPOSE: To review the clinical manifestation and changes in hematologic parameters of patients with beta-thalassemia/hemoglobin (Hb) E. MATERIALS AND METHODS: Retrospective analysis of the clinical manifestation of 378 patients with beta-thalassemia/Hb E attending the hematology clinic at Siriraj Hospital between 1957 and 1982. RESULTS: A wide spectrum of clinical phenotypes has been observed. Most patients show clinical symptoms by 10 years of age. The majority of patients survive with or without occasional transfusion. Splenectomy was performed in 26.5% of patients. Patients come to the hospital because of anemia, fever, abdominal mass, and jaundice. Gastrointestinal tract disturbances are the most common presenting symptoms (34.6%), especially abdominal pain (10%) and cholecystitis (5.1%). Respiratory tract infections were found in 21.8% of patients and cardiovascular complications, including congestive heart failure, occurred in 11.9%. Other less common symptoms and complications included bone pain, chronic leg ulcers, paraplegia and hypertension-associated convulsions, and cerebral hemorrhage after multiple blood transfusion. Patients usually die between 20 to 40 years of age (67%), mainly from congestive heart failure and septicemia. Septicemia was often caused by Gram-negative bacteria. CONCLUSION: These clinical features observed in patients with beta-thalassemia/Hb E are probably the results of chronic anemia and iron overload. The study of the life history and clinical courses of patients with beta-thalassemia/Hb E should provide important information for the better management of these patients.

Adult↗
PubMedDate not supplied

Genetic factors affecting clinical severity in beta-thalassemia syndromes.

PURPOSE: Heterogeneity in the clinical manifestation of beta-thalassemic diseases may occur from the nature of beta-globin gene mutations, alpha-thalassemia gene interaction, or differences in the amount of hemoglobin (Hb) F production. This study was conducted to determine whether these genetic determinant factors can predict phenotypic severity of patients with beta-thalassemia and to assess the relationship between the genotype and phenotype of the disease. MATERIALS AND METHODS: A total of 144 patients with beta-thalassemia were divided into mild (46 patients), intermediate (55 patients), and severe groups (43 patients). DNA analysis based on polymerase chain reaction technique was performed to characterize types of beta-thalassemia mutation, interaction of alpha-thalassemia, and XmnI polymorphism 5' to Ggamma-globin gene. RESULTS: Two alleles of mild beta-thalassemia mutation (beta+/beta+-thalassemia or beta+-thalassemia/Hb E) resulted in a mild clinical symptom whereas two alleles of severe beta-thalassemia mutation (betao/betao) produced a severe clinical phenotype. Compound heterozygosity for mild and severe alleles of beta-thalassemia (betao/ beta+-thalassemia or betao-thalassemia/Hb E) led to variable severity of anemia. Coinheritance of alpha-thalassemia alleviated the severity of beta-thalassemia disease in those patients with at least one allele of the mild beta-thalassemia genotype. DNA polymorphism at position-158 nt 5' to the Ggamma-globin gene was demonstrated by XmnI restriction enzyme. Homozygote of the XmnI site, +/+, was found to have a strong linkage with high Hb F levels and high hemoglobin production in two patients who had mild clinical symptoms. However, some patients who had XmnI site -/- also had mild clinical symptoms because the XmnI- was found to be associated with beta+-thalassemia mutation. CONCLUSION: Types of beta-thalassemia mutation and coinheritance of alpha-thalassemia in the patient who has at least one allele of the mild beta-thalassemia genotype are predictive for the clinical severity of the disease. However, a mild clinical symptom in some patients with betao/beta+-thalassemia or betao-thalassemia/Hb E who do not have a detectable alpha-thalassemia haplotype and no linkage with XmnI++ suggests that there are other confounding factors responsible for the severity differences of the disease.

Age of Onset↗