A5032277792- Hemoglobinopathies and Related Disorders
- Iron Metabolism and Disorders
- Hemophilia Treatment and Research
- Platelet Disorders and Treatments
- Blood Coagulation and Thrombosis Mechanisms
- Blood groups and transfusion
- Venous Thromboembolism Diagnosis and Management
- Myeloproliferative Neoplasms: Diagnosis and Treatment
- Hemostasis and retained surgical items
- Parvovirus B19 Infection Studies
- Chronic Myeloid Leukemia Treatments
- Neonatal Health and Biochemistry
- Acute Ischemic Stroke Management
- Prenatal Screening and Diagnostics
- Immunodeficiency and Autoimmune Disorders
- Cancer-related gene regulation
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Enzyme function and inhibition
- Gastrointestinal Bleeding Diagnosis and Treatment
- Antiplatelet Therapy and Cardiovascular Diseases
- Eicosanoids and Hypertension Pharmacology
- Bone and Joint Diseases
- Acute Lymphoblastic Leukemia research
- Atrial Fibrillation Management and Outcomes
- Lipoproteins and Cardiovascular Health
Ramathibodi Hospital
2016-2025
Mahidol University
2016-2025
Bumrungrad International Hospital
2016
Centre for Nursing Innovation
2014
We describe a 10-year-old severe hemophilia B boy with stop codon mutation of exon 2 in the factor IX gene who developed high inhibitor 70 Bethesda units (BU) from 12 months age after exposure to prothrombin complex concentrate for 14 days. The spontaneously disappeared within 3 months. patient, however, exhibited anaphylactic reaction administration and at ages 15 23 months, respectively. Although recombinant activated VII was alternatively given, he suffered progressive hemophilic...
<b><i>Objective:</i></b> To study the efficacy of combined treatment with oral and subcutaneous iron chelators. <b><i>Material Methods:</i></b> 50-100 mg/kg/day deferiprone (DFP) 40 mg/kg/dose s.c. desferrioxamine (DFO) twice weekly were given to transfusion-dependent β-thalassemia children. <b><i>Results:</i></b> Enrolled patients (9 major 33 hemoglobin E), ranging from 3 18 years in age, divided into groups; group 1...
Data on the use of deferiprone in young children with iron overload are limited.To study safety profile a liquid formulation chelating transfusion-induced overload.A daily dose 50-100 mg/kg BW three divided doses oral was given to patients who had received at least ten packed red cell transfusions and achieved serum ferritin level >1000 μg/L during 12-month period from 2011 2012.Nine (four males) diagnosed various types thalassaemia (n = 8) hereditary spherocytosis 1) were enrolled. Their...
<b><i>Aims:</i></b> To compare insulin sensitivity, β-cell function and iron status biomarkers in non-transfusion-dependent thalassaemia (NTDT) with excess during pre- post-iron chelation. <b><i>Methods:</i></b> Subjects NTDT, aged older than 10 years, serum ferritin >300 ng/ml, were included. Iron chelation deferasirox (10 mg/kg/day) was prescribed daily for 6 months. <b><i>Results:</i></b> Ten patients a median age...
Hemoglobin (Hb) H is generally recognized as mild thalassemia, despite its actual phenotypic diversity. A disease severity scoring system to guide initiation of regular transfusion among severely affected pediatric patients has not previously been reported.Patients with HbH were classified into transfusion-dependent thalassemia (TDT) and non-transfusion-dependent (NTDT) a surrogate for severity. Alpha-globin genotypes relevant clinical parameters associated TDT identified. Univariate...
Abstract Background Reticulocyte hemoglobin equivalent (Ret‐He), a direct measure of the (Hb) in young red blood cells, has been reported to be useful diagnosis iron deficiency anemia ( IDA ) but may have some limitations thalassemia trait. This study evaluated differences Ret‐He school‐aged children, and assessed diagnostic value identifying thalassemia‐prevalent area. Methods Blood samples underwent complete count analysis, including Ret‐He, ferritin, serum total binding capacity. also Hb...
MRI imaging is an alternative to serum ferritin for assessing iron overload in patients with thalassaemia disease.To correlate liver concentration (LIC) determined by and clinical biochemical parameters.An study using cardiovascular magnetic resonance (CMR) tools determine cardiac was undertaken adolescents disease.Eighty-nine (48 males) disease were enrolled. Seventy had been transfusion-dependent since a mean (SD) age of 3.8 (3.0) years, 19 not transfusiondependent. Mean haematocrit 27.3...
The p.R147W mutation, the c.C6152T in exon 7, causing a change amino acid from arginine to tryptophan of PROC gene has been reported as common mutation Taiwanese populations with venous thromboembolism (VTE). present study aimed identify prevalence Thai population and children TE risk developing TE. Patients aged ≤18 years diagnosed were enrolled. was amplified by polymerase chain reaction using specific primer 7. restriction fragment length polymorphism designed MwoI enzyme. A total 184...
This study aimed to explore real-world evidence on health resource use (HRU) spending patients with haemophilia and inhibitor.Medical records from 1990 2019 of inhibitor three comprehensive treatment centres were retrospectively retrieved.In all, 31 (A = 30, B 1) ≥5 BU included. The mean initial 95.4 was detected at the age 6.7 years. number annual hospitalisations 3.9. A total 795 bleeding episodes (major =125, minor =670) evaluated. included bypassing agents or plasma exchange before...
Background Haemophilia is a lifelong X‐linked recessive inherited bleeding disorder. Since the haemophilia management in economically less‐developed countries inadequately provided, prevention of new cases essentially required. Subjects and methods A total 42 pregnancies 37 women at risk for severe moderate (A = 33, B 4) were enrolled. The prenatal diagnostic ( PND ) procedure was performed 32 women, while 10 refused further after knowing their foetuses female n 8) male 2). foetal specimen...
We report a haemophilia A boy with high inhibitor titre (170 BU) who experienced five life-threatening bleeding episodes during one-year period from 9 to 21 months. At the age of 22 months, he received rituximab (375 mg m(-2) per dose) at one- and three-week intervals, three courses each alternative daily treatment factor VIII concentrate doses 100 units kg(-1) for 24 weeks 50 following 28 weeks. Although pretreatment level 4.5 BU showed an anamnestic response reaching maximum 200 9th week...
Background:MRI imaging is an alternative to serum ferritin for assessing iron overload in patients with thalassaemia disease.Aims:To correlate liver concentration (LIC) determined by MRI and clinical biochemical parameters.Methods:An study using cardiovascular magnetic resonance (CMR) tools determine cardiac was undertaken adolescents disease.Results:Eighty-nine (48 males) disease were enrolled. Seventy had been transfusion-dependent since a mean (SD) age of 3.8 (3.0) years, 19 not...
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 11 x cm2 at the abdominal wall and 14 left trunk were covered sterile sponges embedded multiple-hole drain tube transparent plastic film. The exposed end was then connected suction apparatus create -120 mmHg. changed every 2 days. Within 4-6 weeks, completely healed skin grafting not required.
High factor VIII levels and a risk of arterial ischemic stroke is controversial. Levels depend on ethnicity, age, sex. This report included 24 Thai children with cryptogenic 41 controls, mean (standard deviation) age 11.5 (6.3) 9.0 (4.7), respectively. The study was performed during the post–arterial events to avoid acute phase reaction von Willebrand factor. activity antigen in patients were not significantly different compared controls (119.8 ± 41.3% vs 138.0 48.7%, P = .29 92.8 29.8%...
A retrospective evaluation of growth in 112 patients (68 males, 44 females) with Hb E (HBB: c.79G>A)/β-thalassemia (β-thal), classified as 88 transfusion-dependent thalassemia (TDT) and 24 non (NTDT), is reported. Patients TDT have received regular transfusions red blood cells (RBCs) 15 mL/kg every 4 weeks to maintain pre transfusion hemoglobin (Hb) levels at least 9.0 g/dL were categorized according age initiation RBC subgroup 1, <4 years; 2, 4-10 years, 3, >10 years. Iron chelation was...
Objective:The study aimed to report a 3-decade successive establishment of care for women/girls from families with haemophilia.Methods: A retrospective analysis was conducted on 462 243 1987 2021.Results: Combining phenotypic coagulation factor and genotypic either linkage or mutation detection confirmed the status all obligate haemophilia carriers (A118, B19).For potential carrier, 159 proven (A130, B29) 146 noncarrier (A126, B20) were diagnosed except 20 (A16, B4).Only 54 prenatal...