No abstract available.
beta-Thalassemia* ; Thalassemia*

No abstract available.
beta-Thalassemia*

No abstract available.
beta-Thalassemia*

The rate of general population carries the gene of Beta-thalassemia in some countries was high such as North of Italia (20%), Sardinia (11-34%), and Cyprus (5-15%). The frequency of gene carrier of Beta-thalassemia was quite common such as Laos (9.6%), Thailand (6%), and South of China and Cambodia. This rate in Kinh Nation of Vietnam was 1.5-2% higher than other minorities. It was estimated that there were 1.17-1.56 millions of Vietnamese who carry the gene of Beta-thalassemia.
beta-Thalassemia ; epidemiology

50 samples of blood from patients diagnosed as betathalassemia in the center for blood transfussion and cryopreservation at minus 20oC and studied in the Mahidol University, where, DNA extracted by phenol and chloroform and the mutation analysed by technique of PCR and RDBH. The investigation of mutation that caused thalassemia help to establish the Center for the prenatal diagnosis, prevention and control of thalassemia in Vietnam.
beta-Thalassemia ; diagnosis ; mutation

No abstract available.
beta-Thalassemia ; Hematologic Neoplasms

Genotype ; Humans ; beta-Thalassemia

Background: \u03b2-thalassemia is a hereditary disease caused by disorder in \u03b2-globin chain synthesis process. In this research, multiplex-PCR was used in combination with blood chemistry assays and clinical symptoms to detect \u03b2-globin mutations. Objectives: (1) to identify the \u03b2-thalassemia mutation spectrum in the North of Vietnam; (2) to determine the relation between biochemistry values and types of mutations. Subject and methods: Blood samples collected from 60 pediatric patients were used in screening assays (hemoglobin counting, red blood cell counting, hematocrit\ufffd? and multiplex-PCR to detect 6 point mutations with high prevalence in the region. Results: \r\n', u'(1) Of 60 blood samples collected from pediatric patients, 30 (50%) had mutation in codon 17 (A\u2192T), 6 (10%) had a frameshift mutation in codons 41/42 and 4 (6%) had both types of these mutations; (2) The average onset time in patients with FS 41/42 mutation was earlier than that of patients with codon 17 (A\u2192T) mutation, whereas transfusion interval did not differ significantly among these patients; (3) Mean corpuscular volume (MCV) was lower in patients with homozygous mutations (\u03b2o) (average 64.8) than in those with heterozygous mutations (\u03b2+) (average 72.7). Conclusions: (1) multiplex-PCR is an effective technique in identifying the mutation spectrum of \u03b2-globin gene in the North of Vietnam; (2) Biochemistry assays should be associated with molecular techniques in diagnose of \u03b2-thalassemia\r\n', u'\r\n', u'\r\n', u'
beta-Thalassemia ; Thalassemia ; Genetic Diseases ; Inborn ; Mutation

Hemoglobins, Abnormal ; Humans ; alpha-Thalassemia ; complications ; beta-Thalassemia ; complications

INTRODUCTION: Pyoderma gangrenosum is a rare neutrophilic dermatoses in children of unknown etiology. Its occurrence may be associated with a systemic condition. In most cases, diagnosis is late and treatment is sometimes refractory to conventional therapies especially if the underlying disorder is undetected. CASE REPORT: A 3-year-old Filipino male presented with a one-year history of extensive ulcers over the nape, chest, back and lower extremities which started as a solitary painful pustule over the right gluteal area. Central healing was observed with cribriform scarring. Patient was previously treated as a case of skin infection without success. Significant laboratory findings showed severe anemia, neutrophilia, increased ferritin and increased ESR. Initial hemoglobin electrophoresis showed normal profile with mild microcytic anemia. Bacterial culture and ANA (anti-nuclear antigen) were negative. A course of oral prednisone (1-2mkd) and dapsone (2mkd) were given for a month with no improvement of the lesions. Folic acid alone was started at 5mg per day for anemia. After 2 weeks, lesions significantly improved. Three years later, upon repeat hemoglobin electrophoresis test, patient was diagnosed with alpha-thalassemia trait. Folic acid was given as mainstay therapy. Lesions healed with characteristic cribriform scarring. CONCLUSION: This is a rare case of a Filipino child who initially manifested with pyoderma gangrenosum and eventually diagnosed with alpha-thalassemia trait. Due to serious cosmetic sequelae of pyoderma gangrenosum, it is important to find and treat the underlying systemic disorder to stop progression of this debilitating and disfiguring dermatosis
Pyoderma Gangrenosum ; alpha-Thalassemia ; Ulcer ; beta-Thalassemia ; Family