18 research outputs found

    Thalassemia intermedia in HbH-CS disease with compound heterozygosity for β-thalassemia: challenges in hemoglobin analysis and clinical diagnosis

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    Co-inheritance of α-thalassemia with homozygosity or compound heterozygosity for β-thalassemia may ameliorate β-thalassemia major. A wide range of clinical phenotypes is produced depending on the number of α-thalassemia alleles (-α/αα --/αα, --/-α). The co-inheritance of β-thalassemia with α-thalassemia with a single gene deletion (-α/αα) is usually associated with thalassemia major. In contrast, the co-inheritance of β-thalassemia with two α-genes deleted in cis or trans (--/αα or -α/-α) generally produces β-thalassemia intermedia. In Southeast Asia, the most common defect responsible for α-thalassemia is the Southeast Asian (SEA) deletion of 20.5 kilobases. The presence of the SEA deletion with Hb Constant Spring (HbCS) produces HbH-CS disease. Co-inheritance of HbH-CS with compound heterozygosity for β-thalassemia is very rare. This study presents a Malay patient with HbH-CS disorder and β° /β +-thalassemia. The SEA deletion was confirmed in the patient using a duplex-PCR. A Combine-Amplification Refractory Mutation System (C-ARMS) technique to simultaneously detect HbCS and Hb Quong Sze confirmed HbCS in the patient. Compound heterozygosity for CD41/ 42 and Poly A was confirmed using the ARMS. This is a unique case as the SEA α-gene deletion in cis (-- SEA/αα) is generally not present in the Malays, who more commonly posses the two α-gene deletion in trans (-α/-α). In addition, the β-globin gene mutation at CD41/42 is a common mutation in the Chinese and not in the Malays. The presence of both the SEA deletion and CD41/42 in the mother of the patient suggests the possible introduction of these two defects into the family by marriage with a Chinese

    Abstracts from the Food Allergy and Anaphylaxis Meeting 2016

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    Role of HDB in the 21st century.

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    HDB roles and achievements over the social and housing aspects of Singapore can be seen in the housing policies and housing they provided. Its future role will be of an even more challenging one with the rising housing expectations of Singaporeans and our ageing population. This research studies two new major projects (the Executive Condominium scheme and the Studio Apartment scheme) done by the HDB

    Relationship between Intracellular Magnesium Level, Lung Function, and Level of Asthma Control in Children with Chronic Bronchial Asthma

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    Background: This study aimed to determine the intracellular (red blood cell (RBC)) magnesium levels in children with chronic bronchial asthma and to determine the relationship between the magnesium level and peak expiratory flow rate (PEFR), type of asthma treatment, and level of asthma control. Methods: A cross-sectional study was conducted at the Paediatric Clinic, Sarawak General Hospital. A total of 100 children, aged 6–12 years with chronic bronchial asthma, were recruited according to the study criteria. Venous blood samples were obtained to measure the intracellular (RBC) magnesium level using the GBC Avanta Flame Atomic Absorption Spectrophotometer. Results: Mean age was 8.57 (SD 1.18) years, and 63% of the participants were male. Mean duration of asthma was 62.2 (SD 32.3) months. A normal intracellular magnesium level was found in 95% of the participants, with a mean of 2.27 (SD 0.33) mmol/L. Two-thirds of the participants had a normal peak flow expiratory rate (> 80% of predicted value). About 85% were using both reliever and controller. Almost half of the participants (49%) had chronic asthma that was well-controlled. No significant relationship was found between magnesium level and age (r = –0.089, P = 0.379), gender (t = 0.64, P = 0.52), duration of asthma (r = –0.03, P = 0.74), PEFR (t = 0.41, P = 0.68), current level of asthma control (t = 0.02, P = 0.97), and current treatment (t = 0.414, P = 0.680). Conclusions: There was no significant intracellular magnesium deficiency in children with chronic bronchial asthma. There was no significant relationship between therapeutic medications used for treatment of children with chronic asthma and intracellular magnesium level

    Prevalence of undernutrition and associated factors in young children in Malaysia: A nationwide survey

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    Undernutrition in young children is a significant public health problem globally. We determined the prevalence of and factors predisposing to stunting and underweight in children aged 1 to 5 years in Malaysia.Data were extracted from a cross-sectional nationwide campaign involving healthy children aged 1–5 years conducted over a 4-month period in 2019. We obtained information on demography, parental height and risk factors of undernutrition and anthropometric measurements (height and weight) of children enrolled. Age and sex-specific z-score for length/height-for-age (HAZ), weight-for-age (WAZ), body mass index (BMI) z-score (BAZ) and weight-for-height/length (WFH) z-score (WFHZ) were obtained using World Health Organization growth standards. The following definitions were used: (a) HAZ < −2 SD as stunted and −2 to −1 SD as at risk of stunting; (b) WFHZ < −3 SD as severe, −3 to < −2 SD as moderate wasting, and −2 to < +1 SD as normal; (c) WAZ −2 to −1 as at risk of underweight; (d) BAZ +1 to < +2 SD as at risk of and > +2 SD as overweight.Of the 15,331 children surveyed, prevalence of stunting and at risk of stunting were 16.1 and 20.0%, severe and moderate wasting were 4.0 and 6.1%, while 21.1% was at risk of underweight. Prevalence of at risk of and overweight 14.2 and 7.3%, respectively. One in fifth (25.0%) children had at least one form of undernutrition (stunting and/or underweight/wasting). Of the 1,412 (13.2%) children reported to have risk factors of undernutrition, 47.2% had feeding difficulties, 44.8% had poor dietary intake and 8.0% had both. Boys, paternal height < 156 cm and poor dietary intake were significantly associated with stunting and/or wasting. Compared with children with no risk factors, children with feeding difficulties were more likely to be wasted (AOR: 1.48, 95% CI: 1.18–1.85), and had at least one form of undernutrition (AOR: 1.45, 95% CI: 1.25–1.69).In Malaysian children aged 1 to 5 years, dual burden of under- and overnutrition are common. Poor dietary intake and feeding difficulties were risk factors for undernutrition

    The value of open-source clinical science in pandemic response: lessons from ISARIC

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    The value of open-source clinical science in pandemic response: lessons from ISARIC

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    ISARIC-COVID-19 dataset: A Prospective, Standardized, Global Dataset of Patients Hospitalized with COVID-19

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    The International Severe Acute Respiratory and Emerging Infection Consortium (ISARIC) COVID-19 dataset is one of the largest international databases of prospectively collected clinical data on people hospitalized with COVID-19. This dataset was compiled during the COVID-19 pandemic by a network of hospitals that collect data using the ISARIC-World Health Organization Clinical Characterization Protocol and data tools. The database includes data from more than 705,000 patients, collected in more than 60 countries and 1,500 centres worldwide. Patient data are available from acute hospital admissions with COVID-19 and outpatient follow-ups. The data include signs and symptoms, pre-existing comorbidities, vital signs, chronic and acute treatments, complications, dates of hospitalization and discharge, mortality, viral strains, vaccination status, and other data. Here, we present the dataset characteristics, explain its architecture and how to gain access, and provide tools to facilitate its use

    Thrombotic and hemorrhagic complications of COVID-19 in adults hospitalized in high-income countries compared with those in adults hospitalized in low- and middle-income countries in an international registry

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    Background: COVID-19 has been associated with a broad range of thromboembolic, ischemic, and hemorrhagic complications (coagulopathy complications). Most studies have focused on patients with severe disease from high-income countries (HICs). Objectives: The main aims were to compare the frequency of coagulopathy complications in developing countries (low- and middle-income countries [LMICs]) with those in HICs, delineate the frequency across a range of treatment levels, and determine associations with in-hospital mortality. Methods: Adult patients enrolled in an observational, multinational registry, the International Severe Acute Respiratory and Emerging Infections COVID-19 study, between January 1, 2020, and September 15, 2021, met inclusion criteria, including admission to a hospital for laboratory-confirmed, acute COVID-19 and data on complications and survival. The advanced-treatment cohort received care, such as admission to the intensive care unit, mechanical ventilation, or inotropes or vasopressors; the basic-treatment cohort did not receive any of these interventions. Results: The study population included 495,682 patients from 52 countries, with 63% from LMICs and 85% in the basic treatment cohort. The frequency of coagulopathy complications was higher in HICs (0.76%-3.4%) than in LMICs (0.09%-1.22%). Complications were more frequent in the advanced-treatment cohort than in the basic-treatment cohort. Coagulopathy complications were associated with increased in-hospital mortality (odds ratio, 1.58; 95% CI, 1.52-1.64). The increased mortality associated with these complications was higher in LMICs (58.5%) than in HICs (35.4%). After controlling for coagulopathy complications, treatment intensity, and multiple other factors, the mortality was higher among patients in LMICs than among patients in HICs (odds ratio, 1.45; 95% CI, 1.39-1.51). Conclusion: In a large, international registry of patients hospitalized for COVID-19, coagulopathy complications were more frequent in HICs than in LMICs (developing countries). Increased mortality associated with coagulopathy complications was of a greater magnitude among patients in LMICs. Additional research is needed regarding timely diagnosis of and intervention for coagulation derangements associated with COVID-19, particularly for limited-resource settings
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